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Kølle IS, Hesthaven AS, Davidsen L, Hagn-Meincke R, Drewes AM, Pedersen IS, Ejstrud P, Henriksen SD, Olesen SS. Diagnostic yield of second-line aetiological workup in patients with presumed idiopathic acute pancreatitis: a retrospective cohort study. Scand J Gastroenterol 2025; 60:485-493. [PMID: 40160120 DOI: 10.1080/00365521.2025.2485135] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/21/2025] [Revised: 03/14/2025] [Accepted: 03/22/2025] [Indexed: 04/02/2025]
Abstract
BACKGROUND After an aetiological (first-line) workup, the cause of acute pancreatitis remains unidentified in a significant proportion of cases, a condition known as idiopathic acute pancreatitis (IAP). METHODS Retrospective cohort study involving patients with presumed IAP referred for second-line aetiological workup. The completion of first-line aetiological evaluations was assessed upon referral, and the diagnostic outcomes of second-line investigations were evaluated. Over a one-year follow-up period, we documented acute pancreatitis recurrence and patient mortality. Recurrence risk was analysed using an age-adjusted Cox regression model, stratified by treatable versus non-treatable aetiologies. RESULTS We identified 161 patients with presumed IAP, among whom 81 (50%) had recurrent acute pancreatitis. In total, 115 patients (71%) had a complete first-line aetiological workup. The overall diagnostic yield of the second-line aetiological workup was 25% (95% confidence interval [CI] 18-32%). Among second-line tests, the highest diagnostic yield was found for endoscopic ultrasound (34%, 95% CI 20-50%) and genetic testing (37%, 95% CI 22-53%). The most frequent aetiologies identified were biliary pancreatitis (16 patients [10%]) and pancreatitis with a genetic mutation (15 patients [9%]). Neoplasia was identified in two patients. A treatable aetiology was associated with a numerically reduced pancreatitis recurrence risk (Hazard Ratio 0.50, 95% CI 0.07-3.85, p = 0.51). No patient died during the follow-up period. CONCLUSION A second-line aetiological workup can identify the aetiology in 25% of patients with presumed IAP. The most frequent aetiologies are biliary pancreatitis and pancreatitis with a genetic mutation.
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Affiliation(s)
- Ida Saksenborg Kølle
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
| | - Andreas Svenstrup Hesthaven
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
| | - Line Davidsen
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
| | - Rasmus Hagn-Meincke
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
| | - Asbjørn Mohr Drewes
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
| | - Inge Søkilde Pedersen
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
- Department of Molecular Diagnostics, Aalborg University Hospital, Aalborg, Denmark
| | - Per Ejstrud
- Department of Gastrointestinal Surgery, Aalborg University Hospital, Aalborg, Denmark
| | - Stine Dam Henriksen
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
- Department of Gastrointestinal Surgery, Aalborg University Hospital, Aalborg, Denmark
| | - Søren Schou Olesen
- Centre for Pancreatic Diseases and Mech-Sense, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark
- Department of Clinical Medicine, Aalborg University, Aalborg, Denmark
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Duggal S, Akahara O, Didia C. Chronic Pancreatitis and Pancreas Divisum: A Case Report of Recurrent Management Challenges. Cureus 2025; 17:e82313. [PMID: 40376315 PMCID: PMC12080951 DOI: 10.7759/cureus.82313] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2024] [Accepted: 04/15/2025] [Indexed: 05/18/2025] Open
Abstract
Pancreas divisum, resulting from incomplete fusion of the pancreatic ducts during development, disrupts normal drainage and can lead to recurrent acute and chronic pancreatitis. This report presents a case of a 46-year-old male with chronic necrotizing pancreatitis secondary to pancreas divisum. The patient experienced multiple hospital admissions and underwent a cholecystectomy before the underlying etiology, pancreas divisum, was identified after six hospitalizations. This case highlights the diagnostic challenges of recurrent pancreatitis, emphasizing the importance of considering congenital pancreatic anomalies in patients with unexplained or refractory disease. It also underscores the need for a systematic approach to evaluating recurrent pancreatitis to avoid delays in diagnosis and unnecessary interventions. Pancreas divisum is associated with recurrent pancreatitis in a subset of patients. While endoscopic retrograde cholangiopancreatography remains the gold standard for diagnosis and intervention, non-invasive imaging such as magnetic resonance cholangiopancreatography is preferred for initial diagnosis. Endoscopic treatment, including minor papilla papillotomy and stenting, is typically effective for symptomatic cases. However, surgery may be necessary when these methods fail.
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Affiliation(s)
- Shivangini Duggal
- Internal Medicine, Texas Tech University of Health Sciences, El Paso, USA
| | - Ozioma Akahara
- Internal Medicine, Texas Tech University Health Sciences Center El Paso Paul L. Foster School of Medicine, El Paso, USA
| | - Claudia Didia
- Internal Medicine, Texas Tech University Health Sciences Center El Paso Paul L. Foster School of Medicine, El Paso, USA
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Testoni PA, Testoni S. Endoscopic Management of Recurrent Acute Pancreatitis. J Clin Med 2025; 14:2150. [PMID: 40217601 PMCID: PMC11989922 DOI: 10.3390/jcm14072150] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2025] [Revised: 02/28/2025] [Accepted: 03/05/2025] [Indexed: 04/14/2025] Open
Abstract
This review aims to summarize the role of endoscopic therapy in the management and outcomes of recurrent acute pancreatitis (RAP). RAP is a clinical entity characterized by repeated episodes of acute pancreatitis in the setting of a normal gland or chronic pancreatitis (CP). The aetiology of RAP can be identified in about 70% of cases; for the remaining cases, the term "idiopathic" (IRAP) is used. However, advanced diagnostic techniques may reduce the percentage of IRAP to 10%. Recognized causes of RAP are gallstone disease, including microlithiasis and biliary sludge, sphincter of Oddi dysfunction (SOD), pancreatic ductal abnormalities (either congenital or acquired) interfering with pancreatic juice or bile outflow, genetic mutations, and alcohol consumption. SOD, as a clinical entity, was recently revised in the Rome IV consensus, which only recognized type 1 dysfunction as a true pathological condition, while type 2 SOD was defined as a suspected functional biliary sphincter disorder requiring the documentation of elevated basal sphincter pressure to be considered a true clinical entity and type 3 was abandoned as a diagnosis and considered functional pain. Endoscopic therapy by retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS) has been proven effective when a mechanical obstruction is found and can be removed. If an obstruction is not documented, few treatment options are available to prevent the recurrence of pancreatitis and progression toward chronic disease. In gallstone disease, endoscopic biliary sphincterotomy (EBS) is effective when a dilated common bile duct or biliary sludge/microlithiasis is documented. In type 1 SOD, biliary or dual sphincterotomy is generally successful, while in type 2 SOD, endotherapy should be reserved for patients with documented sphincter dysfunction. However, in recent years, doubts have been expressed about the real efficacy of sphincterotomy in this setting. When sphincter dysfunction is not confirmed, endotherapy should be discouraged. In pancreas divisum (PD), minor papilla sphincterotomy is effective when there is a dilated dorsal duct, and the success rate is the highest in RAP patients. In the presence of obstructive conditions of the main pancreatic duct, pancreatic endotherapy is generally successful if RAP depends on intraductal hypertension. However, despite the efficacy of endotherapy, progression toward CP has been shown in some of these patients, mainly in the presence of PD, very likely depending on underlying genetic mutations. In patients with IRAP, the real utility of endotherapy still remains unclear; this is because several unknown factors may play a role in the disease, and data on outcomes are few, frequently contradictory or uncontrolled, and, in general, limited to a short period of time.
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Affiliation(s)
- Pier Alberto Testoni
- Gastroenterology and Gastrointestinal Endoscopy, La Madonnina Clinic, Vita-Salute San Raffaele University, 20100 Milan, Italy
| | - Sabrina Testoni
- Unit of Gastroenterology and Gastrointestinal Endoscopy, IRCCS Policlinico San Donato, Vita-Salute San Raffaele University, 20100 Milan, Italy;
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4
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Aseni P, Fanetti I, Ganguzza E, Bosco S, Fontana P, Armellino A, Gambitta P. Role of Endoscopic Ultrasound in Diagnosis and Management of Pancreas Divisum: A Case Study and Literature Review. APPLIED SCIENCES 2025; 15:2948. [DOI: 10.3390/app15062948] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/21/2025]
Abstract
The long-term efficacy of endoscopic treatment of pancreas divisum is controversial. This review focuses on recent literature on the role of endoscopic ultrasonography (EUS) as effective clinical support in the diagnosis and management of pancreas divisum. A challenging case study in a patient with pancreas divisum affected by recurrent acute pancreatitis and chronic pain is also reported. Our methodology was developed from a search strategy based on the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. A comprehensive electronic search across multiple databases, including Medline/PubMed, EMBASE, Medline/Metacrawler and the Cochrane Library, to identify relevant publications for this systematic review was conducted. A total of 308 articles were found. According to EMBASE grouping criteria, 31 articles were considered major clinical studies and were analysed, reporting for each study the relevant clinical features. In the majority of studies examined, EUS proved useful in diagnosing a pancreatic divisum. The EUS diagnostic yield was reported to have a sensitivity ranging from 51% to 95% and high diagnostic accuracy up to 97%. EUS has shown high sensitivity and specificity in diagnosing pancreas divisum. Studies indicate that EUS can achieve sensitivity rates ranging from 80% to 100% and specificity rates around 97% to 100% for detecting pancreas divisum. Based on these figures, EUS is the most reliable imaging system in terms of diagnostic capability compared with other imaging systems.
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Affiliation(s)
- Paolo Aseni
- Dipartimento di Emergenza Urgenza, ASST Grande Ospedale Metropolitano Niguarda Hospital, 20162 Milan, Italy
| | - Ilaria Fanetti
- Department of Gastroenterology, Ospedale Civile di Legnano, ASST-Ovest Milanese, 20025 Legnano, Italy
| | - Enrico Ganguzza
- Department of Gastroenterology, Ospedale Civile di Legnano, ASST-Ovest Milanese, 20025 Legnano, Italy
- Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy
| | - Sofia Bosco
- Department of Gastroenterology, Ospedale Civile di Legnano, ASST-Ovest Milanese, 20025 Legnano, Italy
- Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy
| | - Paola Fontana
- Department of Gastroenterology, Ospedale Civile di Legnano, ASST-Ovest Milanese, 20025 Legnano, Italy
| | - Antonio Armellino
- Endoscopy Division, Ospedale San Leopoldo Mandic di Merate, ASST Lecco, 23807 Lecco, Italy
| | - Pietro Gambitta
- Department of Gastroenterology, Ospedale Civile di Legnano, ASST-Ovest Milanese, 20025 Legnano, Italy
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5
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Rodriguez J, Coté GA. Clinical and Investigative Approach to Recurrent Acute Pancreatitis. Gastroenterol Clin North Am 2025; 54:113-127. [PMID: 39880522 DOI: 10.1016/j.gtc.2024.09.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2025]
Abstract
Recurrent acute pancreatitis (RAP) is a complex syndrome that presents variably, with many cases remaining idiopathic after thorough diagnostics. For evaluating structural etiologies, endoscopic ultrasound and MR cholangiopancreatography are preferred over endoscopic retrograde cholangiopancreatography (ERCP) given their more favorable risk profile and sensitivity. The diagnostic work-up remains paramount since treatment should focus on addressing underlying causes such as early cholecystectomy for gallstone pancreatitis. As more etiologic factors are uncovered, such as genetic susceptibility, causality becomes more nuanced. Earlier enthusiasm for endoscopic sphincterotomy as a treatment for idiopathic RAP has been tempered by less favorable studies in recent years.
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Affiliation(s)
- Jennifer Rodriguez
- Division of Gastroenterology and Hepatology, Oregon Health & Science University, Portland, OR, USA
| | - Gregory A Coté
- Division of Gastroenterology and Hepatology, Oregon Health & Science University, Portland, OR, USA.
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6
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Nagahama M, Takano Y, Niiya F, Tamai N, Noda J, Yamawaki M, Azami T. Early Chronic Pancreatitis Findings by Endoscopic Ultrasonography (EUS) in Asymptomatic Patients with Pancreas Divisum. Diagnostics (Basel) 2025; 15:253. [PMID: 39941183 PMCID: PMC11817992 DOI: 10.3390/diagnostics15030253] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2024] [Revised: 01/11/2025] [Accepted: 01/21/2025] [Indexed: 02/16/2025] Open
Abstract
Background/Objectives: Pancreas divisum (PD) is a congenital malformation associated with chronic and recurrent acute pancreatitis. Although PD often presents asymptomatically, the extent to which early chronic pancreatitis (ECP) changes occur in asymptomatic patients with PD remains unclear. This study aimed to evaluate endoscopic ultrasonography (EUS) findings indicative of ECP in asymptomatic patients with PD and investigate the relationship between these findings and background factors, such as age, sex, main pancreatic duct diameter, and alcohol intake. Methods: We retrospectively analyzed 17 asymptomatic patients diagnosed with PD at the Showa University Fujigaoka Hospital between January 2016 and May 2024. EUS was used to assess the pancreatic parenchyma and ductal features, and the findings were classified according to the Rosemont Classification (RC) and the Japan Pancreas Society (JPS) 2019 criteria for ECP (JDCECP2019). Statistical analyses were performed to examine the association between EUS findings and patient background factors. Results: EUS findings of ECP were observed in 8 of 17 asymptomatic patients with PD (47%) according to both the RC and the JDCECP2019 criteria. Hyperechoic foci without shadowing or strands were the most common findings, present in 82% of the patients. No significant associations were found between EUS findings and the background factors of age, sex, main pancreatic duct diameter, or alcohol intake. Conclusions: A considerable proportion of asymptomatic patients with PD exhibited EUS findings suggestive of early chronic pancreatitis. These findings indicate that early changes in chronic pancreatitis may be accompanied by asymptomatic PD.
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Affiliation(s)
- Masatsugu Nagahama
- Division of Gastroenterology, Department of Internal Medicine, Showa University Fujigaoka Hospital, 1-30 Fujigaoka, Aoba-ku, Yokohama 227-8501, Kanagawa, Japan; (Y.T.); (F.N.); (N.T.); (J.N.); (M.Y.); (T.A.)
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7
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Gaitanidis A, Christensen MA, Breen KA, Kambadakone AR, Joshipura ND, Fernandez-Del Castillo C, Hernandez-Barco YG, Kaafarani HMA, Velmahos GC, Farhat MR, Fagenholz PJ. A Genome-Wide Association Study Reveals a Novel Susceptibility Locus for Pancreas Divisum at 3q29. J Surg Res 2024; 303:287-294. [PMID: 39393116 DOI: 10.1016/j.jss.2024.09.028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2023] [Revised: 05/17/2024] [Accepted: 09/12/2024] [Indexed: 10/13/2024]
Abstract
INTRODUCTION Pancreas divisum (PD) is a common congenital anomaly of the pancreas, but its genetic basis remains unknown. The purpose of this genome-wide association study was to identify genetic loci associated with PD. METHODS Using the Mass General Brigham Biobank, patients diagnosed with PD were identified. Quality control and imputation were performed using standard approaches. Single nucleotide polymorphisms (SNPs) with minor allele frequency (MAF) ≥ 5% were tested for association with PD using mixed linear model-based association analysis. The significance threshold was set at 5 × 10-8. RESULTS A total of 13,940 subjects were included, of which 251 (1.8%) were diagnosed with PD. A genetic locus in chromosome 3q29 was found to be associated with PD (lead SNP rs3850646, MAFPD = 34.6% vs. MAFcontrols = 26.4%, beta = 0.0106, P = 1.47 × 10-8). The identified locus is located in the phosphatidylinositol glycan anchor biosynthesis class Xand p21 activated kinase 2genes. The heritability of PD was estimated at 27.5%. (Expression quantitative trait loci) and chromatin interaction analysis found 12 genes whose expression may be regulated by SNPs in this genomic locus. CONCLUSIONS The results of this study suggest that a genetic locus at 3q29 is associated with PD. This locus is in the phosphatidylinositol glycan anchor biosynthesis class X and p21 activated kinase 2 genes. Twelve candidate genes were identified whose expression may be regulated by this locus. These findings may help us understand both normal and aberrant pancreatic development and may aid in clinical evaluation and genetic counseling of patients with PD and associated diseases, such as acute pancreatitis.
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Affiliation(s)
- Apostolos Gaitanidis
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts
| | - Mathias A Christensen
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts; Department of Anesthesia, Center of Head and Orthopedics, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
| | - Kerry A Breen
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts
| | - Avinash R Kambadakone
- Department of Radiology, Abdominal Imaging, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
| | - Nencyben D Joshipura
- Department of Radiology, Abdominal Imaging, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
| | | | - Yasmin G Hernandez-Barco
- Division of Gastroenterology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
| | - Haytham M A Kaafarani
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts
| | - George C Velmahos
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts
| | - Maha R Farhat
- Department of Biomedical Informatics, Harvard Medical School, Boston, Massachusetts; Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, Massachusetts
| | - Peter J Fagenholz
- Division of Trauma, Emergency Surgery and Surgical Critical Care, Massachusetts General Hospital, Boston, Massachusetts.
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Zouaki I, Jarti M, Sadik A, Nacir O, Lairani F, Aiterrami A, Oubaha S, Samlani Z, Krati K. Pancreas divisum unmasked: a case report. Pan Afr Med J 2024; 49:48. [PMID: 39867551 PMCID: PMC11760208 DOI: 10.11604/pamj.2024.49.48.45004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2024] [Accepted: 10/13/2024] [Indexed: 01/28/2025] Open
Abstract
Pancreas divisum occurs during development when the ventral and dorsal buds of the pancreas do not fuse. It is the most common congenital anomaly of the pancreas. Patients with this condition are usually asymptomatic, but almost 25% of these patients experience recurrent pancreatitis, which may progress to chronic pancreatitis. This is a case of a 16-year-old male with a significant history of recurrent pancreatitis. The patient underwent a computed tomography scan which revealed pancreatitis. Further magnetic resonance cholangiopancreatography supported the diagnosis of pancreatic divisum. This case highlights the importance of considering even rare etiologies such as pancreas divisum in unexplained cases of recurrent pancreatitis. After ruling out the obvious etiologies (gallstones, alcohol intake, metabolic disorders), it is recommended to demand a magnetic resonance cholangiopancreatography (MRCP) to better analyze the anatomy of the biliary and the pancreatic ductal systems. Early confirmation of the underlying etiology and aggravating factors can improve patient outcomes and prevent the recurrence of pancreatitis.
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Affiliation(s)
- Imane Zouaki
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Mariama Jarti
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Asmaa Sadik
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Oussama Nacir
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Fatimezzahra Lairani
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Adil Aiterrami
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Sofia Oubaha
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Zouhour Samlani
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
| | - Khadija Krati
- Department of Hepato-Gastroenterology, University Hospital of Mohammed VI, Marrakech, Morocco
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9
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Gómez Pérez A, Aparicio Serrano A, Serrano Ruiz FJ. Etiological diagnosis of recurrent acute pancreatitis. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2024; 116:399-403. [PMID: 38685884 DOI: 10.17235/reed.2024.10404/2024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/02/2024]
Abstract
Acute pancreatitis is the leading cause of inpatient care among gastrointestinal conditions. Despite early intervention, one-third of patients experience recurrent acute pancreatitis (RAP). A comprehensive diagnostic approach is warranted to identify and treat underlying factors in order to prevent recurrence. RAP is most frequent among men aged 30-40, smokers, and in those with excessive alcohol consumption. To identify the etiology is paramount to stratify patients according to their individual risk of RAP and for predicting an eventual evolution to chronic pancreatitis. Although the initial management of acute pancreatitis is widely homogeneous according to established guidelines, there are no defined protocols to investigate RAP. In the present editorial article we propose a structured algorithm with precise recommendations to investigate the etiology RAP as part of routine clinical practice. Although there are relevant knowledge gaps in this disease, we believe that our guidance would contribute for a more homogenous diagnostic approach of RAP in clinical practice.
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Affiliation(s)
- Alberto Gómez Pérez
- Gastroenterology Clinical Management Unit, Hospital Universitario Reina Sofía, España
| | - Ana Aparicio Serrano
- Gastroenterology Clinical Management Unit, Hospital Universitario Reina Sofía, España
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10
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Kundra A, Strand DS, Shami VM. Endoscopic Management of Pain due to Chronic Pancreatitis. Gastrointest Endosc Clin N Am 2024; 34:433-448. [PMID: 38796291 DOI: 10.1016/j.giec.2024.02.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/28/2024]
Abstract
Pain secondary to chronic pancreatitis is a poorly understood and complex phenomenon. Current endoscopic treatments target pancreatic duct decompression secondary to strictures, stones, or inflammatory and neoplastic masses. When there is refractory pain and other treatments have been unsuccessful, one can consider an endoscopic ultrasound-guided celiac plexus block. Data on the latter are underwhelming.
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Affiliation(s)
- Arjun Kundra
- Department of Gastroenterology and Hepatology, University of Virginia, Charlottesville, VA, USA
| | - Daniel S Strand
- Department of Gastroenterology, University of Virginia, Charlottesville, VA, USA
| | - Vanessa M Shami
- Department of Medicine, University of Virginia, Charlottesville, VA, USA.
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11
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Destro F, Durante E, Salerno R, Campari A, Meroni M, Diotto V, Brunero M, Pelizzo G. Case Report: Chronic pancreatitis in children as the cumulative effect of bilio-pancreatic abnormalities and genetic mutations. Front Pediatr 2024; 12:1393891. [PMID: 38978842 PMCID: PMC11228964 DOI: 10.3389/fped.2024.1393891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Accepted: 05/24/2024] [Indexed: 07/10/2024] Open
Abstract
Pancreatitis, in general, is a high-morbidity condition. Genetic conditions and anatomic variants are sometimes seen, especially in children, where biliary etiologies and alcohol are less common than in adults. The decision to intervene, the combined operative-endoscopic strategy, and the timing pose unique challenges. We report the case of a 10-year-old boy with PRSS1 mutation and pancreatic duct duplication, discussing the management and reviewing the recent reports in the Literature.
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Affiliation(s)
- Francesca Destro
- Department of Pediatric Surgery, Buzzi Children's Hospital, Milan, Italy
| | - Eleonora Durante
- Department of Pediatric Surgery, Buzzi Children's Hospital, Milan, Italy
| | - Raffaele Salerno
- Gastroenterology and Digestive Endoscopy Unit, ASST Fatebenefratelli Sacco, Milan, Italy
| | - Alessandro Campari
- Department of Pediatric Radiology, Buzzi Children's Hospital, Milan, Italy
| | - Milena Meroni
- Department of Pediatric Surgery, Buzzi Children's Hospital, Milan, Italy
| | - Veronica Diotto
- Pediatric Anesthesia and Intensive Care Unit, Buzzi Children's Hospital, Milan, Italy
| | - Marco Brunero
- Department of Pediatric Surgery, Buzzi Children's Hospital, Milan, Italy
| | - Gloria Pelizzo
- Department of Pediatric Surgery, Buzzi Children's Hospital, Milan, Italy
- Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy
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12
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Nikpanah M, Morgan DE. Magnetic resonance imaging in the evaluation and management of acute pancreatitis: a review of current practices and future directions. Clin Imaging 2024; 107:110086. [PMID: 38262258 DOI: 10.1016/j.clinimag.2024.110086] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2023] [Revised: 12/18/2023] [Accepted: 01/10/2024] [Indexed: 01/25/2024]
Abstract
Acute pancreatitis is a condition marked by inflammation of the pancreas and surrounding tissues. While the majority of cases of acute pancreatitis are mild, a minority of severe cases are the primary contributors to the morbidity and mortality attributed to this condition. Retroperitoneal morphologic changes can be detected by utilization of various imaging modalities, and their accurate evaluation is crucial for effective management. Acute pancreatitis is commonly diagnosed using computed tomography (CT). However, there are certain clinical scenarios where magnetic resonance imaging (MRI) may have superiority over CT. In particular, MRI is useful in cases where patients cannot receive iodinated CT contrast, or where there is a need to investigate the underlying cause of acute pancreatitis. Additionally, MRI can be utilized to evaluate ductal disconnection and guide interventions for necrotic collections. The unique features of MRI can be particularly useful, including its ability to provide superior contrast resolution and to offer greater functional information through techniques such as diffusion-weighted imaging. The aim of this review is to discuss the MRI assessment of individuals with acute pancreatitis. Additionally, the recent advances in MRI for evaluation of acute pancreatitis will also be introduced.
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Affiliation(s)
- Moozhan Nikpanah
- Department of Radiology, University of Alabama at Birmingham, Birmingham, AL, USA.
| | - Desiree E Morgan
- Department of Radiology, University of Alabama at Birmingham, Birmingham, AL, USA
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13
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Hines OJ, Pandol SJ. Management of chronic pancreatitis. BMJ 2024; 384:e070920. [PMID: 38408777 DOI: 10.1136/bmj-2023-070920] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/28/2024]
Abstract
Chronic pancreatitis results from repeated episodes of pancreatic inflammation and associated fibrosis leading to the loss of functional exocrine and endocrine pancreatic function. The disease is manifested by abdominal pain, deterioration in quality of life, food maldigestion and malabsorption, diabetes, and an increased risk for pancreatic adenocarcinoma. This review summarizes the latest evidence on the diagnosis and management of chronic pancreatitis and its manifestations. In particular, this review discusses advances in understanding of the role of genetic disorders in the mechanisms of the disease and surgical options for patients refractory to medical therapy. Furthermore, clinical trials are under way to develop medical therapeutics.
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Affiliation(s)
- O Joe Hines
- Department of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
| | - Stephen J Pandol
- Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA
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14
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Schepis T, Pafundi PC, Tringali A, Carcagnì A, Familiari P, Landi R, Boskoski I, Perri V, Spada C, Costamagna G. Endoscopic minor papilla sphincterotomy in patients with complete pancreas divisum and acute recurrent pancreatitis: a metanalysis. Scand J Gastroenterol 2024; 59:225-231. [PMID: 37795553 DOI: 10.1080/00365521.2023.2266079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2023] [Revised: 09/22/2023] [Accepted: 09/28/2023] [Indexed: 10/06/2023]
Abstract
BACKGROUND AND AIMS Pancreas divisum (PD) is a congenital variant of the pancreatic ductal system and a potential cause of acute recurrent pancreatitis (ARP). Endoscopic minor papilla sphincterotomy (MiES) is the most common procedure performed in the management of PD-related ARP. The aim of this study is to perform a meta-analysis estimating the efficacy and the safety of MiES in the management of patients with PD-related ARP. METHODS A research was performed in Pubmed, EMBASE and Web of science, the studies were reviewed and selected according to inclusion and exclusion criteria. Evaluation of Heterogeneity and publication bias was performed, and a random effect model was used to estimate the effect size of each study. RESULTS One hundred and thirteen articles were selected and reviewed, 13 met the inclusion criteria. All the studies were retrospective with a mean follow-up duration of 45.9 months. A total of 323 patients with PD-related ARP treated with MiES were included in the meta-analysis. The overall clinical success rate of MiES (defined as no further episodes of ARP, reduction of episodes of ARP, or improvement in quality of life) was of 77% (95%CI: 72%-81%; p = 0.30). Evaluating only the studies with clinical success rate defined as "no further AP in the follow-up" the clinical success rate was of 69.8% (95%CI: 61.3%-77.2%; p = 0.57), while evaluating the studies with other definitions (reduction of episodes of ARP or improvement in quality of life) the clinical success rate was of 81.2% (95%CI: 75.2%-86.1%; p = 0.45). The common fixed effects model disclosed a 25.5% overall adverse events rate (95%CI: 19.3%-32.8%; p = 0.42): acute pancreatitis in 14.3% (95%CI: 9.7%-20.6%; p = 0.36), bleeding in 5.6% (95%CI: 2.9%-10.4%; p = 0.98), and other adverse events in 5.6% (95%CI: 2.9%-10.4%; p = 0.67). CONCLUSION MiES is an effective and relatively safe treatment in the management of PD-related ARP. The retrospective nature of the studies selected is the main limitations of this metanalysis. Prospective trials are needed to confirm these data.
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Affiliation(s)
- T Schepis
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - P C Pafundi
- Facility of Epidemiology and Biostatistics, Gemelli Generator, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
| | - A Tringali
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - A Carcagnì
- Facility of Epidemiology and Biostatistics, Gemelli Generator, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
| | - P Familiari
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - R Landi
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - I Boskoski
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - V Perri
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - C Spada
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
| | - G Costamagna
- Digestive Endoscopy Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Centre for Endoscopic Research Therapeutics and Training (CERTT), Università Cattolica del Sacro Cuore, Rome, Italy
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15
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Khan E, Chakrabarty S, Shariff S, Bardhan M. Genetics and Genomics of Chronic Pancreatitis with a Focus on Disease Biology and Molecular Pathogenesis. Glob Med Genet 2023; 10:324-334. [PMID: 38025192 PMCID: PMC10665123 DOI: 10.1055/s-0043-1776981] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2023] Open
Abstract
Chronic pancreatitis is a long-term fibroinflammatory condition of the pancreas with varying incidences across countries. The recent increase in its occurrence implies the involvement of genetic, hereditary, and unconventional risk factors. However, there is a lack of updated literature on recent advances in genetic polymorphisms of chronic pancreatitis. Therefore, this review aims to present recent findings on the genetic implications of chronic pancreatitis based on individual gene mechanisms and to discuss epigenetics and epistasis involved in the disease. Four mechanisms have been implicated in the pathogenesis of chronic pancreatitis, including premature activation of proteases, endoplasmic reticulum stress, ductal pathway dysfunction, and inflammatory pathway dysfunction. These mechanisms involve genes such as PRSS1, PRSS2, SPINK, CEL, PNLIP, PNLIPRP2, CFTR, CaSR, CLDN2, Alpha 1 antitrypsin, and GGT1 . Studying genetic polymorphisms on the basis of altered genes and their products may aid clinicians in identifying predispositions in patients with and without common risk factors. Further research may also identify associations between genetic predispositions and disease staging or prognosis, leading to personalized treatment protocols and precision medicine.
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Affiliation(s)
- Erum Khan
- Department of Neurology, Alzheimer's Disease Research Center, The university of Alabama at Birmingham, Birmingham, United States
| | - Soura Chakrabarty
- Department of Pathology, University of Cambridge, Cambridge, United Kingdom
| | | | - Mainak Bardhan
- Department of Medical Oncology, Miami Cancer Institute, Baptist Health South Florida, Miami, Florida, United States
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16
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Singh A, Bush N, Bhullar FA, Faghih M, Moreau C, Mittal R, Seo JH, Talukdar R, Lakhtakia S, Singh VK, Akshintala VS. Pancreatic duct pressure: A review of technical aspects and clinical significance. Pancreatology 2023; 23:858-867. [PMID: 37798192 DOI: 10.1016/j.pan.2023.09.141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2023] [Revised: 06/12/2023] [Accepted: 09/28/2023] [Indexed: 10/07/2023]
Abstract
Pancreatic duct pressure (PDP) dynamics comprise an intricately modulated system that helps maintain homeostasis of pancreatic function. It is affected by various factors, including the rate of pancreatic fluid secretion, patency of the ductal system, sphincter of Oddi function, and pancreatic fluid characteristics. Disease states such as acute and chronic pancreatitis can alter the normal PDP dynamics. Ductal hypertension or increased PDP is suspected to be involved in the pathogenesis of pancreatic pain, endocrine and exocrine pancreatic insufficiency, and recurrent pancreatitis. This review provides a comprehensive appraisal of the available literature on PDP, including the methods used in the measurement and clinical implications of elevated PDP.
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Affiliation(s)
- Anmol Singh
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States
| | - Nikhil Bush
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States
| | - Furqan A Bhullar
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States
| | - Mahya Faghih
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States
| | - Chris Moreau
- Division of Gastroenterology, University of Texas Health San Antonio, San Antonio, TX, United States
| | - Rajat Mittal
- Department of Mechanical Engineering, Johns Hopkins University, Baltimore, MD, United States
| | - Jung-Hee Seo
- Department of Mechanical Engineering, Johns Hopkins University, Baltimore, MD, United States
| | - Rupjyoti Talukdar
- Department of Gastroenterology, Asian Institute of Gastroenterology, Hyderabad, India
| | - Sundeep Lakhtakia
- Department of Gastroenterology, Asian Institute of Gastroenterology, Hyderabad, India
| | - Vikesh K Singh
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States
| | - Venkata S Akshintala
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, United States.
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17
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Inamdar S, Cote GA, Yadav D. Endotherapy for Pancreas Divisum. Gastrointest Endosc Clin N Am 2023; 33:789-805. [PMID: 37709411 DOI: 10.1016/j.giec.2023.04.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/16/2023]
Abstract
Pancreas divisum (PD) is a common anatomic variant of the pancreatic duct. Causal association between PD and pancreatitis has been debated for many years. Minor papilla sphincterotomy (miES) is offered in clinical practice to patients with idiopathic acute recurrent pancreatitis (iRAP) and PD. However, available data originate mainly from observational studies with many limitations. An ongoing international, multicenter, sham-controlled trial is evaluating the efficacy of miES in iRAP and PD. Endoscopic therapy for pain relief has limited to no benefit in patients with chronic abdominal pain or chronic pancreatitis who have PD and is not recommended.
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Affiliation(s)
- Sumant Inamdar
- Division of Gastroenterology and Hepatology, Department of Medicine, University of Arkansas for Medical Sciences, Shorey Building, 8th Floor, 4301 West Markham Street, Little Rock, AR 72205, USA.
| | - Gregory A Cote
- Division of Gastroenterology, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Mail Code L461, Portland, OR, USA
| | - Dhiraj Yadav
- Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburg Medical Center, 200 Lothrop Street, M2, C-wing, Pittsburgh, PA 15213, USA
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18
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Li BB, Zheng H, Lou YD, Zhang WW, Zheng S. Periampullary tumors in a patient with pancreatic divisum and neurofibromatosis type 1: a case report. Hered Cancer Clin Pract 2023; 21:18. [PMID: 37773168 PMCID: PMC10540396 DOI: 10.1186/s13053-023-00262-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2023] [Accepted: 09/20/2023] [Indexed: 10/01/2023] Open
Abstract
INTRODUCTION We present a case of a male patient with neurofibromatosis type 1 diagnosed with pancreatic divisum and several gastrointestinal tumors. A 55-year-old man was admitted to the hospital with recurrent chronic pancreatitis, indicating a large mass in the ampulla. In addition, genetic testing revealed two unique germline mutations in the neurofibromin (NF1) gene, and their potential interaction in promoting cancer was further investigated. CONCLUSION The first similar case was reported in 2020. The current case was distinct from other cases since an additional two NF1 mutations were found in the patient. In conjunction with prior case reports, our findings imply that genetic testing in patients diagnosed with neurofibromatosis type 1 could be helpful in the development of effective treatments.
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Affiliation(s)
- Bin-Bin Li
- The Fourth School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, 310053, Zhejiang, China
- Department of Medical Oncology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China
| | - Hui Zheng
- Department of Medical Oncology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China
| | - Yi-Dan Lou
- Department of Medical Oncology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China
- Zhejiang University School of Medicine, Hangzhou, China
| | - Wen-Wei Zhang
- The Fourth School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, 310053, Zhejiang, China
- Department of Medical Oncology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China
| | - Song Zheng
- The Fourth School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, 310053, Zhejiang, China.
- Department of Medical Oncology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China.
- Department of Medical Oncology, Affiliated Hangzhou Cancer Hospital, Zhejiang University School of Medicine, Hangzhou, 310000, Zhejiang, China.
- Key Laboratory of Clinical Cancer Pharmacology and Toxicology Research of Zhejiang Province, Hangzhou, 310000, Zhejiang, China.
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19
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Orellana-Donoso M, Milos-Brandenberg D, Benavente-Urtubia A, Guerra-Loyola J, Bruna-Mejias A, Nova-Baeza P, Becerra-Farfán Á, Sepulveda-Loyola W, Luque-Bernal RM, Valenzuela-Fuenzalida JJ. Incidence and Clinical Implications of Anatomical Variations in the Pancreas and Its Ductal System: A Systematic Review and Meta-Analysis. Life (Basel) 2023; 13:1710. [PMID: 37629567 PMCID: PMC10455790 DOI: 10.3390/life13081710] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2023] [Revised: 07/27/2023] [Accepted: 08/03/2023] [Indexed: 08/27/2023] Open
Abstract
OBJECTIVE This systematic review analyzes the anatomical variants in the pancreas and its ductal system to report on their association with pancreatic pathologies. METHODS We conducted a search of the MEDLINE, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS databases from their inception to July 2023. The methodological quality was assessed with the Anatomical Quality Assessment (AQUA) tool. Finally, the pooled prevalence was estimated using a random effects model. RESULTS 55 studies were found that met the eligibility criteria. The overall prevalence of pancreas divisum (PD) was 18% (95% CI = 15-21%). The prevalence of PD associated with pancreatitis was 30% (95% CI = 1-61%). CONCLUSIONS An anatomical variant of the pancreas such as PD may be the cause of bile duct obstruction, resulting in various clinical complications, such as pancreatitis. Hence, knowing this variant is extremely important for surgeons, especially for those who treat the gastroduodenal region.
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Affiliation(s)
- Mathias Orellana-Donoso
- Escuela de Medicina, Universidad Finis Terrae, Santiago 7500000, Chile;
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
| | - Daniel Milos-Brandenberg
- Escuela de Medicina, Facultad Ciencias de la Salud, Universidad del Alba, Santiago 8320000, Chile;
| | - Andoni Benavente-Urtubia
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
| | - Javier Guerra-Loyola
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
| | - Alejandro Bruna-Mejias
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
| | - Pablo Nova-Baeza
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
| | - Álvaro Becerra-Farfán
- Departamento de Ciencias Química y Biológicas, Facultad de Ciencias de la Salud, Universidad Bernardo O’Higgins, Santiago 8370993, Chile;
| | - Walter Sepulveda-Loyola
- Faculty of Health and Social Sciences, Universidad de las Américas, Santiago 8370040, Chile;
| | - Ricardo Miguel Luque-Bernal
- Unidad de Anatomía, Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá 111221, Colombia;
| | - Juan José Valenzuela-Fuenzalida
- Departamento de Morfología, Facultad de Medicina, Universidad Andrés Bello, Santiago 8370146, Chile; (A.B.-U.); (J.G.-L.); (A.B.-M.); (P.N.-B.)
- Department of Morphology and Function, Faculty of Health and Social Sciences, Universidad de Las Américas, Santiago 8370040, Chile
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20
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Hirota M, Shimosegawa T. Idiopathic and Rare Causes of Chronic Pancreatitis. THE PANCREAS 2023:404-411. [DOI: 10.1002/9781119876007.ch49] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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21
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Grover AS, Gugig R, Barakat MT. Endoscopy and Pediatric Pancreatitis. Gastrointest Endosc Clin N Am 2023; 33:363-378. [PMID: 36948751 DOI: 10.1016/j.giec.2022.11.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/24/2023]
Abstract
Children and adolescents are increasingly impacted by pancreatic disease. Interventional endoscopic procedures, including endoscopic retrograde cholangiopancreatography) and endoscopic ultrasonography, are integral to the diagnosis and management of many pancreatic diseases in the adult population. In the past decade, pediatric interventional endoscopic procedures have become more widely available, with invasive surgical procedures now being replaced by safer and less disruptive endoscopic interventions.
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Affiliation(s)
- Amit S Grover
- Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA 02115, USA
| | - Roberto Gugig
- Division of Pediatric Gastroenterology, Lucille Packard Children's Hospital at Stanford University Medical Center, Stanford, CA 94305, USA
| | - Monique T Barakat
- Division of Pediatric Gastroenterology, Lucille Packard Children's Hospital at Stanford University Medical Center, Stanford, CA 94305, USA; Division of Gastroenterology, Stanford University Medical Center, Stanford, CA 94305, USA.
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22
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Barakat MT, Husain SZ, Gugig R. Safety and efficacy of minor papillotomy in children and adolescents with pancreas divisum. Pancreatology 2023; 23:171-175. [PMID: 36641286 DOI: 10.1016/j.pan.2022.12.009] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Revised: 12/06/2022] [Accepted: 12/13/2022] [Indexed: 01/16/2023]
Abstract
INTRODUCTION Pancreas Divisum (PD) is a common pancreatic ductal variant which is twice as common in pediatric patients with acute recurrent pancreatitis (ARP) relative to the general population (14% vs. 7%). Endoscopic retrograde cholangiopancreatography (ERCP) with minor papillotomy has been performed to facilitate drainage of pancreatic juice from the diminutive minor papilla to prevent pancreatitis and pancreatic damage. METHODS We searched our prospectively-maintained endoscopy databases for patients 18 and younger who underwent ERCP with minor papillotomy between 2009 and 2019. Demographic data, indications, procedural interventions and findings, as well as available clinical outcomes data were analyzed. RESULTS 54 ARP/PD patients underwent ERCP with minor papillotomy. Median age was 14 (range 7-18) years, and 26 (48.1%) patients were female. Post-ERCP pancreatitis developed in 10/54 patients (18.5%). 12-month post-ERCP clinical trajectory was available in 47/54 (87%) patients and most patients (38/47, 80.8%) improved clinically after minor papillotomy, with 9/47 (19.1%) experiencing resolution of pancreatitis episodes and none indicated worsening severity or frequency of pancreatitis episodes following ERCP. CONCLUSION The majority of children and adolescents with PD and ARP who underwent ERCP with minor papillotomy experienced subjective improvement in their symptoms following the intervention. These data suggest that ERCP with minor papillotomy for pediatric patients with PD and ARP is beneficial and may be curative in a subset of patients-higher rates of improvement than have been previously reported in adults.
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Affiliation(s)
- Monique T Barakat
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Stanford University School of Medicine, Stanford, CA 94305, United States; Division of Pediatric Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, United States
| | - Sohail Z Husain
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, United States
| | - Roberto Gugig
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, United States.
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23
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Zhang JY, Deng ZH, Gong B. Clinical characteristics and endoscopic treatment of pancreatitis caused by pancreaticobiliary malformation in Chinese children. J Dig Dis 2022; 23:651-659. [PMID: 36630003 DOI: 10.1111/1751-2980.13152] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2022] [Revised: 10/01/2022] [Accepted: 10/17/2022] [Indexed: 01/12/2023]
Abstract
OBJECTIVES We aimed to describe the clinical characteristics of pediatric patients with pancreatitis caused by pancreaticobiliary malformation and to evaluate the efficacy and safety of magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of the disease. METHODS Medical records of pediatric patients with pancreatitis related to pancreaticobiliary malformation diagnosed by ERCP and treated in our hospital between April 2008 and December 2020 were retrospectively reviewed. Clinical manifestations, laboratory indicators, genetic testing results and imaging findings including MRCP were collected. RESULTS Of the 148 patients with pancreaticobiliary malformation-related pancreatitis, 90 (60.8%) had pancreaticobiliary maljunction (PBM), 52 (35.1%) had pancreatic divisum (PD), and six (4.1%) had annular pancreas (AnnP). Compared with the PD group, patients with PBM were younger (P < 0.001), and were more likely to have jaundice (P < 0.001) and fever (P = 0.034). Genetic mutation was found in 51.6% of patients with PD, 50.0% with AnnP, and 15.0% with PBM. Diagnostic rate of PBM, PD, and AnnP using MRCP was 46.7%, 15.4%, and 100%, respectively. In total, 87.8% of patients had symptomatic improvement after endoscopic treatment. ERCP-related complications were observed in 28 out of the 260 procedures, including post-ERCP pancreatitis (7.7%), infection (2.3%), and gastrointestinal bleeding (0.8%). CONCLUSIONS PBM should be considered when jaundice and fever occur in pediatric patients. Genetic testing is recommended for those with PD and AnnP. The role of MRCP is limited in identifying pancreaticobiliary malformation in children. ERCP is effective and safe for the diagnosis and treatment for pediatric pancreatitis caused by pancreaticobiliary malformation.
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Affiliation(s)
- Jia Yu Zhang
- Department of Pediatric Gastroenterology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Zhao Hui Deng
- Department of Pediatric Gastroenterology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Biao Gong
- Department of Gastroenterology, Shanghai Shuguang Hospital, Shanghai University of Chinese Medicine, Shanghai, China
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24
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Strand DS, Law RJ, Yang D, Elmunzer BJ. AGA Clinical Practice Update on the Endoscopic Approach to Recurrent Acute and Chronic Pancreatitis: Expert Review. Gastroenterology 2022; 163:1107-1114. [PMID: 36008176 DOI: 10.1053/j.gastro.2022.07.079] [Citation(s) in RCA: 30] [Impact Index Per Article: 10.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2022] [Revised: 06/08/2022] [Accepted: 07/01/2022] [Indexed: 02/07/2023]
Abstract
DESCRIPTION The purpose of this American Gastroenterological Association (AGA) Clinical Practice Update Expert Review is to provide practical, evidence-based guidance to clinicians regarding the role of endoscopy for recurrent acute and chronic pancreatitis. METHODS This expert review was commissioned and approved by the AGA Institute Clinical Practice Updates Committee and the AGA Governing Board to provide guidance on a topic of clinical importance to the AGA membership, underwent internal peer review by the Clinical Practice Updates Committee (CPUC), and external peer review through standard procedures of Gastroenterology. This review is framed around the 8 best practice advice points agreed upon by the authors, based on the results of randomized controlled trials, observational studies, systematic reviews and meta-analyses, as well expert consensus in this field. Best Practice Advice Statements BEST PRACTICE ADVICE 1: After an unrevealing initial evaluation, endoscopic ultrasound is the preferred diagnostic test for unexplained acute and recurrent pancreatitis. Magnetic resonance imaging with contrast and cholangiopancreatography is a reasonable complementary or alternative test to endoscopic ultrasound, based on local expertise and availability. BEST PRACTICE ADVICE 2: The role of endoscopic retrograde cholangiopancreatography (ERCP) for reducing the frequency of acute pancreatitis episodes in patients with pancreas divisum is controversial, but minor papilla endotherapy may be considered, particularly for those with objective signs of outflow obstruction, such as a dilated dorsal pancreatic duct and/or santorinicele. There is no role for ERCP to treat pain alone in patients with pancreas divisum. BEST PRACTICE ADVICE 3: The role of ERCP for reducing the frequency of pancreatitis episodes in patients with unexplained recurrent acute pancreatitis and standard pancreatic ductal anatomy is controversial and should only be considered after a comprehensive discussion of the uncertain benefits and potentially severe procedure-related adverse events. When pursued, ERCP with biliary sphincterotomy alone may be preferable to dual sphincterotomy. BEST PRACTICE ADVICE 4: Surgical intervention should be considered over endoscopic therapy for long-term treatment of patients with painful obstructive chronic pancreatitis. Endoscopic intervention is a reasonable alternative to surgery for suboptimal operative candidates or those who favor a less invasive approach, assuming they are clearly informed that the best practice advice primarily favors surgery. BEST PRACTICE ADVICE 5: When ERCP is pursued, small (≤5mm) main pancreatic duct stones can be treated with pancreatography and conventional stone extraction maneuvers. For larger stones, extracorporeal shockwave lithotripsy and/or pancreatoscopy with intraductal lithotripsy may be required. BEST PRACTICE ADVICE 6: When ERCP is pursued, prolonged stent therapy (6-12 months) is effective for treating symptoms and remodeling main pancreatic duct strictures. The preferred approach is to place and sequentially add multiple plastic stents in parallel (upsizing); emerging evidence suggests that fully covered self-expanding metal stents may have a role for this indication, but additional research is necessary. BEST PRACTICE ADVICE 7: ERCP with stent insertion is the preferred treatment for benign biliary stricture due to chronic pancreatitis. FCSEMS placement is favored over multiple plastic stents whenever feasible, given similar efficacy but significantly reduced need for stent exchange procedures during the treatment course. BEST PRACTICE ADVICE 8: Celiac plexus block should not be routinely performed for the management of pain due to chronic pancreatitis. The decision to proceed with celiac plexus block in selected patients with debilitating pain in whom other therapeutic measures have failed can be considered on a case-by-case basis, but only after discussion of the unclear outcomes of this intervention and its procedural risks.
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Affiliation(s)
- Daniel S Strand
- Division of Gastroenterology and Hepatology, University of Virginia Health System, Charlottesville, Virginia
| | - Ryan J Law
- Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
| | - Dennis Yang
- Center for Interventional Endoscopy, AdventHealth, Orlando, Florida.
| | - B Joseph Elmunzer
- Division of Gastroenterology and Hepatology, Medical University of South Carolina, Charleston, South Carolina
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Zdanowicz K, Uscinowicz M, Rakowska M, Wertheim-Tysarowska K, Rygiel AM, Oracz G, Lebensztejn DM. Chronic pancreatitis caused by a Homozygous SPINK1 c.194 + 2T > C variant and Pancreas Divisum in a 3-year-old child—case report. J Pediatr Genet 2022; 11:232-235. [DOI: 10.1055/s-0040-1717109] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2020] [Accepted: 08/21/2020] [Indexed: 10/23/2022]
Abstract
AbstractChronic pancreatitis (CP) is a rare disease in children. We describe the first case of a 3-year-old Caucasian patient with CP with the presence of a homozygous pathogenic variant c.194 + 2T > C in serine protease inhibitor, Kazal type 1 (SPINK1) and pancreas divisum.
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Affiliation(s)
- Katarzyna Zdanowicz
- Department of Pediatrics, Gastroenterology, Hepatology, Nutrition and Allergology, Medical University of Bialystok, Bialystok, Poland
| | - Miroslawa Uscinowicz
- Department of Pediatrics, Gastroenterology, Hepatology, Nutrition and Allergology, Medical University of Bialystok, Bialystok, Poland
| | - Magdalena Rakowska
- Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Children's Memorial Health Institute, Warsaw, Poland
| | | | | | - Grzegorz Oracz
- Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Children's Memorial Health Institute, Warsaw, Poland
| | - Dariusz Marek Lebensztejn
- Department of Pediatrics, Gastroenterology, Hepatology, Nutrition and Allergology, Medical University of Bialystok, Bialystok, Poland
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Sun Y, Jin J, Zhu A, Hu H, Lu Y, Zeng Y, Jing D. Risk Factors for Recurrent Pancreatitis After First Episode of Acute Pancreatitis. Int J Gen Med 2022; 15:1319-1328. [PMID: 35173470 PMCID: PMC8841459 DOI: 10.2147/ijgm.s344863] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2021] [Accepted: 12/30/2021] [Indexed: 12/12/2022] Open
Abstract
Background Methods Results Conclusion
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Affiliation(s)
- Yingying Sun
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
| | - Jie Jin
- Department of Geriatrics, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200080, People’s Republic of China
| | - Aying Zhu
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
| | - Hong Hu
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
| | - Yingying Lu
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
| | - Yue Zeng
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
| | - Dadao Jing
- Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 201600, People’s Republic of China
- Correspondence: Dadao Jing, Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, NO. 650 Xinsongjiang Road, Shanghai, 201600, People’s Republic of China, Tel +86-13816958050, Email
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Al Droubi B, Altamimi E. Acute Pancreatitis in Jordanian Children: A Single Center Experience. Front Pediatr 2022; 10:908472. [PMID: 35844741 PMCID: PMC9283568 DOI: 10.3389/fped.2022.908472] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2022] [Accepted: 06/10/2022] [Indexed: 11/30/2022] Open
Abstract
BACKGROUND There is still much to understand and discover regarding pediatric pancreatitis. The etiology, clinical presentation, and prognosis of pancreatitis differs considerably between young children and adults. The incidence of pancreatitis has been increasing; it is no longer as rare in children as previously thought and could cause significant morbidity and mortality when severe. METHODS In this retrospective study conducted at a tertiary care hospital in Jordan, we present a cohort of children with 64 episodes of acute pancreatitis. RESULTS While abdominal pain was the most common presenting complaint in our cohort (97%), the classical features of radiation to the back and relief by the forward-lean position were observed in only one-third of our patients. Compared to serum amylase, serum lipase had a higher sensitivity for detecting pancreatitis (98 vs. 67%). Abdominal ultrasound is a non-invasive, widely available imaging modality; when performed, it revealed an enlarged pancreas in almost 60% of the patients. However, abdominal ultrasonography is often limited by the presence of excessive bowel gas. Anatomical abnormalities were the most common etiologies of pancreatitis (29%), followed by idiopathic pancreatitis (21%), and biliary causes (21%). CONCLUSION In our cohort, serum lipase was a better diagnostic tool compared to serum amylase. Congenital biliary-pancreatic abnormalities were the most common causes of acute pancreatitis in our cohort. Almost half of these patients developed recurrent acute pancreatitis. The prevalence of pancreatic pseudocysts was 16.7%, and nearly half of them required an intervention.
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Affiliation(s)
- Belal Al Droubi
- Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan
| | - Eyad Altamimi
- Department of Pediatrics, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan
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Zeng J, Zhang J, Hu Y, Wang X, Deng Z. Risk factors for the progression from acute recurrent to chronic pancreatitis among children in China. Front Pediatr 2022; 10:908347. [PMID: 35958176 PMCID: PMC9357905 DOI: 10.3389/fped.2022.908347] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2022] [Accepted: 07/07/2022] [Indexed: 11/17/2022] Open
Abstract
BACKGROUND Risk factors for progression from acute recurrent pancreatitis (ARP) to chronic pancreatitis (CP) in children are poorly understood. AIM To summarize the clinical characteristics of children with ARP and CP, identify the risk factors of CP, and investigate the factors associated with rapid progression from initial onset of ARP to CP. METHODS The following variables were included in the risk factor analysis: sex, age at onset, family history, pancreas or biliary tract structural abnormalities, and genetic variations. Univariate and multivariate logistic regression analyses were used to assess the risk factors of CP. The Kaplan-Meier curves of the ARP progression to CP for various risk factor groupings were constructed and compared using the log-rank test. The Cox proportional hazard regression model was fitted to estimate the hazard ratio (HR) of progression to CP for each risk variable. RESULTS In total, 276 children were studied, of whom 136 progressed to CP. Among them, 41 had pancreatic duct obstructive disease; 105 underwent genetic testing, of whom 68 were found to have genetic variations. Among the remaining 140 patients who did not progress to CP, 61 had biliary obstructions. Forty-three of these children underwent genetic testing, and 15 were found to have genetic variations. Risk factor analysis showed that children with gene mutations were at a higher risk of progressing to CP [odds ratio (OR) = 3.482; 95% confidence interval (CI): 1.444-8.398; P = 0.005]; children with pancreas divisum (PD) had a higher risk of CP than those without (OR = 8.665; 95% CI: 1.884, 9.851; P = 0.006). Further, children whose first ARP occurred at an older age might develop CP faster (HR = 1.070; 95% CI: 1.003, 1.141; P = 0.039). Children with gene mutations had a faster rate of progression to CP after onset than children without gene mutations (HR = 1.607; 95% CI: 1.024, 2.522; P = 0.039), PRSS1 gene mutations were more associated (P = 0.025). There was no difference in the rate of progression from ARP to CP in children with PD (P = 0.887); however, endoscopic retrograde cholangiopancreatography (ERCP) intervention delayed the progression to CP in ARP patients with PD (P = 0.033). CONCLUSION PRSS1 gene mutations and PD are key risk factors for ARP progression to CP in children. PD itself does not affect the disease progression rate, but therapeutic ERCP can be beneficial to patients with ARP with symptomatic PD and delay the progression to CP.
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Affiliation(s)
- Jingqing Zeng
- Department of Pediatric Digestive, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Jiayu Zhang
- Department of Pediatric Digestive, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yabin Hu
- Department of Clinical Epidemiology and Biostatistics, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Xiumin Wang
- Department of Pediatric Endocrinology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Zhaohui Deng
- Department of Pediatric Digestive, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
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Abstract
Hereditary pancreatitis (HP) is a rare inherited chronic pancreatitis (CP) with strong genetic associations, with estimated prevalence ranging from 0.3 to 0.57 per 100,000 across Europe, North America, and East Asia. Apart from the most well-described genetic variants are PRSS1, SPINK1, and CFTR, many other genes, such as CTRC, CPA1, and CLDN2 and CEL have been found to associate with HP, typically in one of the 3 main mechanisms such as altered trypsin activity, pancreatic ductal cell secretion, and calcium channel regulation. The current mainstay of management for patients with HP comprises genetic testing for eligible individuals and families, alcohol and tobacco cessation avoidance, pain control, and judicious screening for complications, including exocrine and endocrine insufficiency and pancreatic cancer.
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Affiliation(s)
- Yichun Fu
- Henry D. Janowitz Division of Gastroenterology, One Gustave L. Levy Place, Box 1069, New York, NY 10029, USA; Samuel Bronfman Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, USA
| | - Aimee L Lucas
- Henry D. Janowitz Division of Gastroenterology, One Gustave L. Levy Place, Box 1069, New York, NY 10029, USA; Samuel Bronfman Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, USA.
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Gurakar M, Jalaly NY, Faghih M, Boortalary T, Azadi JR, Khashab MA, Fan C, Kalloo AN, Zaheer A, Singh VK, Afghani E. Impact of genetic testing and smoking on the distribution of risk factors in patients with recurrent acute and chronic pancreatitis. Scand J Gastroenterol 2022; 57:91-98. [PMID: 34663162 PMCID: PMC9278560 DOI: 10.1080/00365521.2021.1984573] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
OBJECTIVE The aim of the present study is to assess the impact of smoking dose and duration on the distribution of risk factor(s) in patients with RAP and CP, and the impact of genetic testing on the distribution of risk factor(s) in patients with idiopathic RAP and CP. METHODS All adult patients with RAP and CP referred to a multidisciplinary pancreatitis clinic between 2010 and 2017 were evaluated. Risk factors included alcohol and smoking, hypertriglyceridemia, biliary, and other etiologies. Genetic testing was only pursued in patients with idiopathic RAP or CP. RESULTS Among the 1770 patients evaluated, 167 had RAP and 303 had CP. After genetic testing and smoking, the most common risk factors for RAP and CP were pathogenic variant(s) (23%) and the combination of alcohol and smoking (23%), respectively. Genetic testing and smoking assessment decreased the proportion of patients with alcoholic RAP from 17% to 5%, alcoholic CP from 33% to 10%, idiopathic RAP from 49% to 12%, and idiopathic CP from 54% to 14%. Pathogenic CFTR variants were the most common variant in patients with RAP (51%) and CP (43%). Among the 68 patients with pancreas divisum, other risk factor(s) were identified in 72%. CONCLUSION Genetic testing and a detailed assessment of smoking dose and duration reduce the proportion of patients with alcoholic and idiopathic pancreatitis. Other risk factor(s) for pancreatitis are found in the majority of patients with pancreas divisum further questioning its role as an independent risk factor.1. What is the current knowledge?Approximately 30% of patients with pancreatitis have no clear risk factor(s) and are categorized as having an idiopathic etiology.Pathogenic variant(s) as well as smoking dose and duration are well-established risk factors for recurrent acute and chronic pancreatitis but are not widely recognized or incorporated into clinical practice.2. What is new here?Genetic testing and a detailed assessment of smoking dose and duration reduced the proportion of patients with alcoholic and idiopathic acute recurrent and chronic pancreatitis.Approximately three-fourths of patients with pancreas divisum have a risk factor for pancreatitis.
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Affiliation(s)
- Merve Gurakar
- Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | | | - Mahya Faghih
- Pancreatitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Tina Boortalary
- Department of Medicine, Thomas Jefferson University, Philadelphia, PA, USA
| | - Javad R. Azadi
- Division of Abdominal Imaging, Department of Radiology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Mouen A. Khashab
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Christopher Fan
- Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Anthony N. Kalloo
- Pancreatitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA,Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Atif Zaheer
- Pancreatitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA,Division of Abdominal Imaging, Department of Radiology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Vikesh K. Singh
- Pancreatitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA,Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | - Elham Afghani
- Pancreatitis Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA,Division of Gastroenterology, Johns Hopkins Medical Institutions, Baltimore, MD, USA
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Abstract
Pediatric pancreatitis describes a spectrum covering acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis, each with varying clinical manifestations and risk factors requiring a tailored diagnostic approach. We emphasize management strategies based on age, risk factors, recurrence, and complications. A discussion of the role of therapeutic endoscopy is reviewed and highlights the growing role of endoscopic ultrasound and endoscopic retrograde cholangiopancreatography in children with pancreatitis. Particular diagnostic challenges in autoimmune pancreatitis are reviewed with an emphasis on differentiating this entity from alternate pancreaticobiliary pathologies. Finally, we explore a multidisciplinary approach to acute recurrent and chronic pancreatitis.
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Affiliation(s)
- Reuven Zev Cohen
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Emory University School of Medicine, Children's Healthcare of Atlanta, 1400 Tullie Road Northeast, Atlanta, GA 30329, USA.
| | - A Jay Freeman
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Emory University School of Medicine, Children's Healthcare of Atlanta, 1400 Tullie Road Northeast, Atlanta, GA 30329, USA
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Mann R, Boregowda U, Vyas N, Gajendran M, Umapathy CP, Sayana H, Echavarria J, Patel S, Saligram S. Current advances in the management of chronic pancreatitis. Dis Mon 2021; 67:101225. [PMID: 34176572 DOI: 10.1016/j.disamonth.2021.101225] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Chronic pancreatitis is characterized by irreversible destruction of pancreatic parenchyma and its ductal system resulting from longstanding inflammation, leading to fibrosis and scarring due to genetic, environmental, and other risk factors. The diagnosis of chronic pancreatitis is made based on a combination of clinical features and characteristic findings on computed tomography or magnetic resonance imaging. Abdominal pain is the most common symptom of chronic pancreatitis. The main aim of treatment is to relieve symptoms, prevent disease progression, and manage complications related to chronic pancreatitis. Patients who do not respond to medical treatment or not a candidate for surgical treatment are usually managed with endoscopic therapies. Endoscopic therapies help with symptoms such as abdominal pain and jaundice by decompression of pancreatic and biliary ducts. This review summarizes the risk factors, pathophysiology, diagnostic evaluation, endoscopic treatment of chronic pancreatitis, and complications. We have also reviewed recent advances in endoscopic retrograde cholangiopancreatography and endoscopic ultrasound-guided therapies for pancreatic duct obstruction due to stones, strictures, pancreatic divisum, and biliary strictures.
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Affiliation(s)
- Rupinder Mann
- Department of Internal Medicine, Saint Agnes Medical Center, 1303 E Herndon Ave, Fresno, CA 93720, USA
| | - Umesha Boregowda
- Department of Internal Medicine, Bassett Healthcare Network, Columbia Bassett Medical School, 1 Atwell Road, Cooperstown, NY 13326, USA
| | - Neil Vyas
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
| | - Mahesh Gajendran
- Department of Internal Medicine, Texas Tech University Health Science Center El Paso, 2000B Transmountain Road, El Paso, TX 79911, USA
| | - Chandra Prakash Umapathy
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
| | - Hari Sayana
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
| | - Juan Echavarria
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
| | - Sandeep Patel
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
| | - Shreyas Saligram
- Department of Gastroenterology and Advanced endoscopy, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA.
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Ammer-Herrmenau C, Ellenrieder V, Neesse A. [Chronic pancreatitis: update diagnostic and therapeutic concepts]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2021; 60:1131-1138. [PMID: 34798674 DOI: 10.1055/a-1659-4636] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
INTRODUCTION Chronic pancreatitis (CP) is a frequent cause for hospitalization and is associated with impaired quality of life and reduced overall survival. The German Society for Gastroenterology (DGVS) has recently completed the S3-Guideline "Pancreatitis" that summarizes key findings on epidemiology, diagnostic and therapeutic concepts for acute and chronic pancreatitis. Here, we recapitulate the most relevant findings for clinicians regarding CP. RESULTS The most common cause of CP is chronic alcohol abuse, other causes are hereditary pancreatitis, autoimmune pancreatitis, hyperparathyroidism and idiopathic forms. Apart from the classical hereditary pancreatitis (PRSS1 mutation), a number of genetic associations have been discovered over the last years that are associated with an increased risk to develop idiopathic CP. The conservative management of CP is focused on the appropriate management of exocrine and endocrine insufficiency, and the prevention and treatment of secondary complications such as osteoporosis, vitamin deficiencies and malnutrition. Local complications (bile duct stenosis, duodenal stenosis, pseudocysts and chronic pain) should be managed in multidisciplinary teams in specialized pancreas centres with expert surgeons, radiologists and gastroenterologists. Infected or symptomatic pseudocysts should be primarily addressed by endoscopic drainage. In contrast, patients with chronic pain, dilated pancreas duct and opioid use should be considered for early surgical intervention. CONCLUSION Chronic pancreatitis is associated with increased morbidity and mortality and often leads to hospital admissions. The clinical management of complex patients with local complications requires an interdisciplinary approach to tailor available therapeutic modalities depending on the stage of the disease and pre-existing comorbidities.
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Affiliation(s)
- Christoph Ammer-Herrmenau
- Klinik für Gastroenterologie, gastrointestinale Onkologie und Endokrinologie, Universitätsmedizin Göttingen, Göttingen, Germany
| | - Volker Ellenrieder
- Klinik für Gastroenterologie, gastrointestinale Onkologie und Endokrinologie, Universitätsmedizin Göttingen, Göttingen, Germany
| | - Albrecht Neesse
- Klinik für Gastroenterologie, gastrointestinale Onkologie und Endokrinologie, Universitätsmedizin Göttingen, Göttingen, Germany
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Comparison of idiopathic recurrent acute pancreatitis [IRAP] and recurrent acute pancreatitis with genetic mutations. Dig Liver Dis 2021; 53:1294-1300. [PMID: 33972190 DOI: 10.1016/j.dld.2021.04.013] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 04/13/2021] [Accepted: 04/14/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND Idiopathic recurrent acute pancreatitis (IRAP) describes frequent episodes of pancreatitis without an etiology found using current testing. We compared the natural history of IRAP with recurrent acute pancreatitis with genetic mutations. METHODS Retrospective cohort of patients with recurrent acute pancreatitis (≥2 episodes) and negative conventional testing. All patients had ≥1 episode after cholecystectomy and completed genetic testing. Primary outcomes were chronic pancreatitis incidence, pancreatic cancer, and mortality. Secondary outcomes included opioid and ERCP utilization. RESULTS 128 patients met criteria for presumed IRAP. 35 patients met criteria for true IRAP. 12 patients had recurrent acute pancreatitis with gene mutations. Chronic pancreatitis developed in 27 (77.1%) IRAP patients over a median of 6 years. Chronic pancreatitis incidence was similar in IRAP and CFTR mutation carriers; but developed later in SPINK1 carriers. No patients developed pancreatic cancer or died from pancreatic-related causes. Patients were frequently treated with oral opioids and ERCP, without significant differences within or between groups. CONCLUSION IRAP and pancreatitis in mutation carriers is associated with chronic pancreatitis. Important differences in natural history were observed, but no association was found with cancer or pancreas-related mortality. Efforts to understand the genetic contributions to IRAP, minimize opioids and unnecessary ERCPs are encouraged.
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Pancreas Divisum Increases the Risk of Recurrent Acute Pancreatitis in Patients with rs12338 Polymorphism in the Cathepsin B Gene. Dig Dis Sci 2021; 66:2283-2290. [PMID: 32754840 DOI: 10.1007/s10620-020-06517-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2020] [Accepted: 07/22/2020] [Indexed: 12/21/2022]
Abstract
OBJECTIVES Pancreas divisum (PD) as a cause of pancreatitis has been debated. In this study, we report the association of multiple gene polymorphisms on the risk of RAP in the presence of PD. DESIGN We enrolled 687 individuals (167 IRAP, 276 ICP, and 244 unrelated healthy controls) from May 2015 to September 2016. Patients were divided into those with/without PD. Associations between the significantly prevalent SNPs and IRAP/ICP in the presence of PD were evaluated. Clinical data were analyzed using Mann-Whitney U/Chi-square test. Effect size of association of SNPs with IRAP/ICP was expressed as odds ratio (OR) (95% CI). Gene-gene interaction was assessed by transheterozygosity analyses. Bonferroni-corrected two-tailed "p" value of ≤ 0.01 was considered statistically significant. RESULTS Thirty-three (19.8%) and 82 (29.7%) patients with IRAP and ICP, respectively, had PD. Among the patients with IRAP, duration of disease was significantly shorter in those with PD compared to those without (mean [95% CI] duration: 1.6 (1.3-1.9) vs 2.7 (2.3-3.1) years; p = 0.005). There were no differences in gender, race, and diabetes among patients with/without PD in IRAP/ICP groups. Mean (95% CI) pancreatic duct diameter (mm) was significantly higher in the presence of PD in patients with both IRAP [1.6 (1.4-1.9) v/s 1.29 (1.2-1.4); p = 0.03)] and ICP [5.2 (4.5-5.9) v/s 4.5 (3.9-5.1); p = 0.02]. CTSB (rs12338) polymorphisms were significantly associated with IRAP [OR (95% CI) 2.44 (1.41-4.22); p = 0.001] among patients with PD. No association was observed with ICP. Transheterozygosity analysis did not show any significant associations of combination of SNPs with IRAP in the presence of PD. CONCLUSION Risk of RAP due to PD increases in patients with rs12338 polymorphism in the cathepsin B gene.
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Chronic pancreatitis for the clinician. Part 1: Etiology and diagnosis. Interdisciplinary position paper of the Societat Catalana de Digestologia and the Societat Catalana de Pàncrees. GASTROENTEROLOGIA Y HEPATOLOGIA 2021; 45:231-248. [PMID: 34157366 DOI: 10.1016/j.gastrohep.2021.05.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Revised: 04/13/2021] [Accepted: 05/07/2021] [Indexed: 11/24/2022]
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Conti Bellocchi MC, Crinò SF, Pin F, di Stefano S, Le Grazie M, Bernardoni L, Ruffini M, Frulloni L, Gabbrielli A. Reappraisal of factors impacting the cannulation rate and clinical efficacy of endoscopic minor papilla sphincterotomy. Pancreatology 2021; 21:805-811. [PMID: 33745788 DOI: 10.1016/j.pan.2021.01.019] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2020] [Revised: 01/15/2021] [Accepted: 01/25/2021] [Indexed: 12/13/2022]
Abstract
BACKGROUND We aimed to assess factors impacting the endoscopic minor papilla sphincterotomy (EMPS) success rate, clinical efficacy, and safety in a large cohort of patients with symptomatic pancreas divisum (PD). METHODS Retrospective study including patients with PD referred to the Pancreas Institute of Verona from May 2009 to May 2020 to undergo EMPS. The whole population was analyzed to assess EMPS technical success, defined as the rate of deep cannulation of the dorsal duct. Patients treated for recurrent pancreatitis (RP) with a minimum follow-up of 1 year were included to evaluate the clinical efficacy, defined as resolution or significant reduction of acute pancreatitis (AP) episodes. Safety was defined as the rate of procedure-related adverse events (AEs) according to an international lexicon. The effects of the main determinants on study outcomes were evaluated. RESULTS Overall, 106 patients were evaluated. Technical success was obtained in 87 (82.1%). The presence of pancreatic calcifications was associated with failure (p < 0.0001). Clinical efficacy was evaluated in 59 patients. Resolution/reduction of AP episodes after EMPS was observed in 93% of patients over a median follow-up of 49 months (IQR 37-92). Smoking habit was associated with AP recurrence (p = 0.026). The overall AE rate was 14.9%, with post-ERCP pancreatitis as the most common complication (12.6%). CONCLUSIONS In our study, performed at a tertiary center, EMPS showed satisfactory technical success and an acceptable safety profile. If confirmed by prospective multicenter studies, EMPS could become the standard of care for the treatment of RP in PD.
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Affiliation(s)
- Maria Cristina Conti Bellocchi
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy.
| | - Stefano Francesco Crinò
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Federico Pin
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Serena di Stefano
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Marco Le Grazie
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Laura Bernardoni
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Martina Ruffini
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Luca Frulloni
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
| | - Armando Gabbrielli
- Department of Medicine, Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, G.B. Rossi University Hospital, Verona, Italy
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Hereditary pancreatitis in childhood: course of disease and complications. Wien Klin Wochenschr 2021; 133:669-673. [PMID: 33909107 DOI: 10.1007/s00508-021-01869-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2020] [Accepted: 03/31/2021] [Indexed: 10/21/2022]
Abstract
BACKGROUND Hereditary pancreatitis is rare. Pain therapy for juvenile symptom onset, child development and the risk of pancreatic carcinoma in adulthood must be considered. PATIENTS, MATERIAL AND METHODS Data from a cohort of 11 patients with disease onset in childhood (< 16 years) were analyzed retrospectively. The gene encoding cationic trypsinogen (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1) and cystic fibrosis transmembrane conductance regulator (CFTR) genes were investigated as genetic factors. Treatment concept and complications were registered. Prognosis, treatment success and quality of life were objectified using the chronic pancreatitis prognosis score and a standardized questionnaire (KIDSCREEN-10 index). RESULTS The mean age of symptom onset was 7.5 ±4.2 years. The PRSS1 and SPINK1 mutations each occurred with 36.4%, 3 patients had a pancreas divisum and 2 a long common channel. The course of pancreatitis was obstructive in 90.9%. Exocrine pancreatic insufficiency occurred in seven patients so far (mean age 12.5 years). Stenting was performed in 72.7% and 18.2% needed pancreatic surgery. Currently the chronic prognosis score is on average 7.5 points, pain on numerical rating scale 0 (no pain). The mean KIDSCREEN‑T score of 66.9 confirms a very good quality of life. CONCLUSION Patients with genetically caused chronic pancreatitis are rare. Their care ranges from pain therapy in childhood and adolescence to questions concerning family planning and pancreatic cancer prevention from mid-adulthood onward. The disease is challenging for the interdisciplinary cooperation. We found the step-up strategy to be a good option for pain therapy. A national registry monitored by scientific societies with active recruitment for screening examinations will further improve and ensure care in the long term.
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Andersson R, Löhr JM. Swedish national guidelines for chronic pancreatitis. Scand J Gastroenterol 2021; 56:469-483. [PMID: 33617407 DOI: 10.1080/00365521.2021.1881815] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2020] [Revised: 01/11/2021] [Accepted: 01/20/2021] [Indexed: 02/04/2023]
Abstract
Chronic pancreatitis (CP) should be suspected in the case of recurrent upper abdominal pain of unknown origin and/or clinical signs of exocrine pancreatic insufficiency (EPI). Alcohol is the most common etiological factor associated with CP, others being smoking, male gender, and hereditary forms. CP is often associated with recurrent episodes of acute exacerbations.As of today, there is no accepted clinical definition of CP. However, irreversible morphological changes within the pancreas often occur, including dilatation of the main and branch pancreatic ducts, calcifications in ducts and parenchyma, parenchymal atrophy, and development of pseudocysts, though less so in the early phase of CP.
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Clinical Outcomes Following Therapeutic Endoscopic Retrograde Cholangiopancreatography in Children With Pancreas Divisum. J Pediatr Gastroenterol Nutr 2021; 72:300-305. [PMID: 33230075 DOI: 10.1097/mpg.0000000000002996] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
OBJECTIVES Pancreas divisum (PD) is a risk factor in children for the development of acute pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) with minor papilla endoscopic sphincterotomy (mPES) may be of clinical benefit, however, the clinical outcomes from endotherapy remain unclear. We sought to review the outcomes and safety of therapeutic ERCP in children with PD. METHODS We performed a retrospective chart of children with PD who underwent an ERCP between February 2012 and December 2018. Pertinent patient, clinical and procedure information was collected including procedure-related adverse events. A follow-up questionnaire of the parent was conducted to determine the clinical impact from endotherapy. RESULTS Fifty-eight ERCPs were performed in 27 patients (14 boys; mean age: 9.7 years, range 2-19) with PD. All patients underwent a successful mPES. A genetic variant was identified in 19/26 (73%) tested patients. Post-ERCP pancreatitis (PEP) was the only observed adverse event; 21% (12/58). Median follow-up interval from first ERCP intervention to questionnaire completion was 31.5 months (range: 4--72 months). Of the 20 questionnaire responders, 13 reported clinical improvement from endotherapy. CONCLUSIONS The majority of children from our PD cohort possessed at least 1 genetic variant. Most questionnaire responders had a favorable response to endotherapy. PEP rate was comparable with that of prior reports in adult patients.
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Suzuki M, Minowa K, Isayama H, Shimizu T. Acute recurrent and chronic pancreatitis in children. Pediatr Int 2021; 63:137-149. [PMID: 32745358 DOI: 10.1111/ped.14415] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2020] [Revised: 06/27/2020] [Accepted: 07/28/2020] [Indexed: 12/14/2022]
Abstract
Acute recurrent pancreatitis (ARP) is defined as two distinct episodes of acute pancreatitis (AP), whereas chronic pancreatitis (CP) is caused by persistent inflammation of the pancreas. In children they are caused by genetic mutations, autoimmune pancreatitis, congenital pancreatic abnormalities, and other conditions. Acute recurrent pancreatitis is frequently a precursor to CP, and both are thought to be on the same disease continuum. In particular, genetic factors are associated with early progression of ARP to CP. The diagnosis of CP, as in AP, is based on clinical findings, biochemical tests, and imaging studies. Findings of exocrine pancreatic dysfunction are also important in the diagnosis of CP. A step-up strategy has become increasingly standard for the treatment of patients with CP. This strategy starts with endoscopic treatment, such as pancreatic sphincterotomy and stenting, and progresses to surgery should endoscopic therapy fail or prove technically impossible. Non-opioid (e.g. ibuprofen / naproxen) and opioid (e.g. oxycodone) forms of analgesia are widely used in pediatric patients with AP or CP, whereas pancreatic enzyme replacement therapy may be beneficial for patients with abdominal pain, steatorrhea, and malnutrition. Despite the disparity in the age of onset, pediatric CP patients display some similarities to adults in terms of disease progress. To reduce the risk of developing pancreatic exocrine inefficiency, diabetes and pancreatic cancer in the future, clinicians need to be aware of the current diagnostic approach and treatment methods for ARP and CP and refer them to a pediatric gastroenterologist in a timely manner.
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Affiliation(s)
- Mitsuyoshi Suzuki
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
| | - Kei Minowa
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
| | - Hiroyuki Isayama
- Department of Gastroenterology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Toshiaki Shimizu
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
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Pan G, Yang K, Gong B, Deng Z. Analysis of the Efficacy and Safety of Endoscopic Retrograde Cholangiopancreatography in Children With Symptomatic Pancreas Divisum. Front Pediatr 2021; 9:761331. [PMID: 34796156 PMCID: PMC8592933 DOI: 10.3389/fped.2021.761331] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/19/2021] [Accepted: 10/12/2021] [Indexed: 12/11/2022] Open
Abstract
Background: Endoscopic retrograde cholangiopancreatography (ERCP) has been increasingly performed in children with symptomatic pancreas divisum (PD). Aim: To investigate the safety and efficacy of ERCP in the treatment of children with symptomatic PD. Methods: We performed a retrospective analysis on children with PD who were treated with ERCP at Shanghai Children's Medical Center between June 2015 and May 2020. Pertinent patient, clinical and procedural data were collected to assess the therapeutic effects and identify the risk factors for post-ERCP pancreatitis (PEP). Results: Overall, 114 ERCPs were performed in 46 children with PD. With a median follow-up of 28.5 months (12-71 months), 40 (87.0%) children achieved clinical remission, the median number of acute pancreatitis episodes decreased from four times per year pre-operatively to once per year post-operatively (P < 0.001), and the nutritional score improved post-operatively (P = 0.004). The incidence of PEP was 7.9%, and female sex, stone extraction, and gene mutations were identified as possible risk factors for PEP on univariate analysis. However, there was no statistical significance on multivariate analysis (P > 0.05). Conclusion: Therapeutic ERCP is an effective and safe intervention for children with symptomatic PD.
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Affiliation(s)
- Guixian Pan
- Department of Gastroenterology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Kaihua Yang
- Department of Gastroenterology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Biao Gong
- Department of Gastroenterology, Shanghai Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Zhaohui Deng
- Department of Gastroenterology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
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Sugawa C, Culver A, Sundaresan N, Lucas CE. Congenital pancreaticobiliary anomalies in an urban medical center in the United States. JGH OPEN 2020; 4:1176-1182. [PMID: 33319053 PMCID: PMC7731818 DOI: 10.1002/jgh3.12418] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/27/2020] [Accepted: 09/06/2020] [Indexed: 11/17/2022]
Abstract
Background and Aim Pancreaticobiliary anomalies are rare and often present with cryptic signs and symptoms, thus delaying appropriate treatment. Methods Endoscopic retrograde cholangiopancreatography (ERCP) was used to define pancreaticobiliary anomalies. A retrospective review was performed of 5522 ERCPs conducted at a tertiary care center from 1972 to 2015. Results There were 249 (4.5%) patients with pancreaticobiliary anomalies, including 179 patients with pancreas divisum (PD), 44 patients with choledochal cyst (CC) (Todani's classification Type I: extrahepatic cyst 31, Type III; choledochocele 9, Type V: Caroli's disease 4), 20 patients with anomalous pancreaticobiliary ductal union (APDU), and 6 patients with other abnormalities. Of 179 patients with pancreas divisum, 8 (4.5%) required minor sphincterotomies for multiple unexplained acute pancreatitis. Of the 31, 15 (48%) Type I CC patients underwent an operation. In patients with Type III CC (choledochocele), seven of the nine were treated by endoscopic sphincterotomy, and two patients were treated by surgery. Four patients with Type V CC (Caroli's disease) were managed nonoperatively. Of the 20 patients with APDU, 8 (40%) required operative intervention. Six patients were found to have other anomalies: two with pancreas bifidum, one with a duplication of the gallbladder, one with a cystic duct diverticulum, one with an annular pancreas, and one with an abnormal cystic duct origin. These patients were treated based on their etiology. Conclusion Pancreaticobiliary anomalies are rare and can be defined using ERCP. The appreciation of these abnormalities is important for the proper diagnosis and treatment of these rare biliary and pancreatic disorders.
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Affiliation(s)
- Choichi Sugawa
- Michael and Marian Ilitch Department of Surgery Wayne State University School of Medicine Detroit, Michigan USA
| | - Ashley Culver
- Michael and Marian Ilitch Department of Surgery Wayne State University School of Medicine Detroit, Michigan USA
| | - Naresh Sundaresan
- Michael and Marian Ilitch Department of Surgery Wayne State University School of Medicine Detroit, Michigan USA
| | - Charles E Lucas
- Michael and Marian Ilitch Department of Surgery Wayne State University School of Medicine Detroit, Michigan USA
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Abstract
The evolution of advanced pancreaticobiliary endoscopy in the past 50 years is remarkable. Endoscopic retrograde cholangiopancreatography (ERCP) has progressed from a diagnostic test to an almost entirely therapeutic procedure. The endoscopist must have a clear understanding of the indications for ERCP to avoid unnecessary complications, including post-ERCP pancreatitis. Endoscopic ultrasound initially was used as a diagnostic tool but now is equipped with accessary channels allowing endoscopic ultrasound-guided interventions in various pancreaticobiliary conditions. This review discusses the endoscopic management of common pancreatic and biliary diseases along with the techniques, indications, outcomes, and complications of pancreaticobiliary endoscopy.
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Affiliation(s)
- Catherine F Vozzo
- Department of Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue / A30, Cleveland, OH 44195, USA
| | - Madhusudhan R Sanaka
- Department of Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue / Q30, Cleveland, OH 44195, USA.
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Affiliation(s)
| | - Karla Au Yeung
- Department of Pediatric Gastroenterology and Nutrition, and
| | - Brian Pugmire
- Department of Radiology, Valley Children's Hospital, Madera, CA
| | - Roberto Gugig
- Department of Pediatric Gastroenterology and Nutrition, and
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Nabi Z, Talukdar R, Venkata R, Aslam M, Shava U, Reddy DN. Genetic Evaluation of Children with Idiopathic Recurrent Acute Pancreatitis. Dig Dis Sci 2020; 65:3000-3005. [PMID: 31900721 DOI: 10.1007/s10620-019-06026-2] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2019] [Accepted: 12/22/2019] [Indexed: 12/09/2022]
Abstract
OBJECTIVES Several genetic risk factors have been identified in adults with idiopathic acute recurrent pancreatitis (IARP). However, the literature regarding the genetics of IARP is sparse in children. In this study, we aimed to analyze the genetic risk factors in children with IARP. METHODS All children (< 18 years) with ARP from January 2015 to May 2018 were prospectively enrolled in the study. Children with a known cause of ARP like obstructive, toxic/metabolic, and autoimmune were excluded from the final analysis. Children with IARP underwent genetic testing for mutations/polymorphisms in genes known to predispose to pancreatitis including cationic trypsinogen protease serine 1 (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1), cystic fibrosis transmembrane conductance regulator gene (CFTR), chymotrypsin C (CTRC), claudin-2 (CLDN2) and cathepsin B (CTSB). RESULTS A total of 239 children (116 boys, 10.3 ± 3.7 years) were enrolled during the study period. Of these, 204 (85.35%) children were identified as IARP. The mean age of symptom onset and the number of pancreatitis episodes were 8.3 ± 3.7 years and 3.3 ± 1.8, respectively. A family history of pancreatitis was noted in 4.6% children. Mutations/polymorphisms in at least 1 gene were identified in 89.5% (129/144) children including SPINK1 in 41.9%, PRSS1 (rs10273639) in 58.2%, CTRC in 25.6%, CTSB in 54.9%, CLDN2 in 72.9%, and CFTR in 2.3%. There was no significant incidence of genetic mutations/polymorphisms in IARP with or without pancreas divisum (95.7 vs 88.4%; p = 0.467). CONCLUSIONS Genetic alterations are present in the majority of the children with IARP. The incidence of genetic mutations is similar in children with or without pancreas divisum.
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Affiliation(s)
- Zaheer Nabi
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India.
| | - Rupjyoti Talukdar
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India
| | - Ravikanth Venkata
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India
| | - Mohsin Aslam
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India
| | - Upender Shava
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India
| | - D Nageshwar Reddy
- Asian Institute of Gastroenterology, 6-3-661, Hyderabad, 500082, India
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Hérin E, Sauvanet A, Cros J, Koulakian H, Lévy P, Vullierme MP. Recurrent acute pancreatitis caused by combined gastric and pancreatic duplications: value of preoperative CT and MRCP and conservative surgery. J Surg Case Rep 2020; 2020:rjaa175. [PMID: 32874535 PMCID: PMC7450821 DOI: 10.1093/jscr/rjaa175] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2020] [Revised: 05/10/2020] [Accepted: 06/06/2020] [Indexed: 12/14/2022] Open
Abstract
We present a series of patients with recurrent acute pancreatitis caused by a duplicated pancreatic head connected to a gastric duplication and successfully treated by conservative surgery. This retrospective study included consecutive adult patients referred to our institution for recurrent acute pancreatitis. All patients underwent a preoperative non-invasive imaging examination including contrast-enhanced computed tomography and magnetic resonance cholangiopancreatography (MRCP). The final diagnosis of this developmental anomaly was based on surgical and pathological examinations. The four patients in this study had the same typical imaging pattern including a duplicated duct. There was no recurrent acute pancreatitis after surgical treatment, which involved atypical resection of the duplicated pancreatic head and segmental gastric resection, without a Whipple procedure. The discovery of an accessory pancreatic head with a duct terminating in a cyst identified on MRCP in a patient with recurrent acute pancreatitis could suggest this rare and surgically treatable cause of acute pancreatitis.
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Affiliation(s)
- Edouard Hérin
- Department of Radiology, Beaujon Hospital, Clichy, France
| | | | - Jérome Cros
- Department of Pathology, Beaujon Hospital, Clichy, France
| | | | - Philippe Lévy
- Department of Pancreatology, Beaujon Hospital, Clichy, France
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Dutta S, Sowmiya SV, Chapa UK, Jain A, Reddy A, Nelamangala Ramakrishnaiah VP. Pancreatic divisum with chronic pancreatitis of the ventral pancreas. ANZ J Surg 2020; 91:E126-E127. [PMID: 32648986 DOI: 10.1111/ans.16152] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2020] [Revised: 06/27/2020] [Accepted: 07/01/2020] [Indexed: 11/30/2022]
Affiliation(s)
- Souradeep Dutta
- Division of GI and HPB Surgery, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Sathyamangalam V Sowmiya
- Division of GI and HPB Surgery, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Uday K Chapa
- Division of GI and HPB Surgery, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Ankit Jain
- Division of GI and HPB Surgery, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Abhinaya Reddy
- Division of GI and HPB Surgery, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
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Aljiffry M, Abbas M, Wazzan MAM, Abduljabbar AH, Aloufi S, Aljahdli E. Biliary anatomy and pancreatic duct variations: A cross-sectional study. Saudi J Gastroenterol 2020; 26:285019. [PMID: 32461381 PMCID: PMC7580731 DOI: 10.4103/sjg.sjg_573_19] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2019] [Revised: 02/22/2020] [Accepted: 03/09/2020] [Indexed: 01/25/2023] Open
Abstract
BACKGROUND/AIM Biliary tree and pancreatic duct can appear in different variations whose proper understanding is obligatory for surgeons. Magnetic resonance cholangiopancreatography (MRCP) is considered a safe and accurate tool for evaluating biliary tree and pancreatic duct. Typical anatomy for right hepatic duct (RHD) and left hepatic duct (LHD) is reported as 57% and 63%, respectively. The most common (4-10%) pancreatic anomaly is divisum. In the present study, we evaluated and determined the prevalence of biliary tree and pancreatic duct variations among patients at a university hospital. MATERIALS AND METHODS The MRCP records of 370 patients from 2015 to 2017 were obtained for cross-sectional study. Images were retrospectively reviewed for variations by two independent senior radiologists. Demographic data were obtained for all the patients. Huang et al. classification was used for RHD and LHD variations. The cystic duct was reported based on its course and insertion pattern. The pancreatic duct was observed for the presence of divisum, its course, and configuration. RESULTS Three hundred and twenty-five patients were included in the final study. Most commonly observed variant for RHD were A1 (34.2%) and A2 (32.2%). For LHD, B1 (71.4%) was the most common variant. Cystic duct insertion was commonly seen as right lateral insertion (27.7%). Pancreatic divisum was observed in 0.6% of cases. Nationality, origin, and gender-specific variations were obtained. CONCLUSION Variations in biliary anatomy and pancreatic duct are very diverse and extend from the intrahepatic biliary system down to the pancreas. Performing a similar study on a larger population is mandatory to illustrate the range of variations present within the community.
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Affiliation(s)
- Murad Aljiffry
- Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Mohammad Abbas
- Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Mohammad A. M. Wazzan
- Department of Radiology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Ahmed H. Abduljabbar
- Department of Radiology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Safiyah Aloufi
- Department of Radiology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Emad Aljahdli
- Department of Medicine, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
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Abstract
Computed tomography (CT) is the most commonly performed imaging test for acute pancreatitis. Nevertheless, magnetic resonance (MRI) imaging is useful in many specific situations. These include evaluating patients with acute pancreatitis who cannot receive iodinated CT contrast, elucidating the underlying cause of acute pancreatitis, assessing ductal disconnection and for guiding intervention of necrotic collections. Non-contrast MRI is superior to non-contrast CT and MRI for the diagnosis of acute pancreatitis. We discuss these specific uses of MRI in acute pancreatitis. We highlight the future advances in MRI including faster, free-breathing scans that allow MRI to be completed within 10 min.
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