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Rapelly SS, Singh S, Verma N, Bhattacharya S, Rungta S. Non-invasive predictors to grade esophageal varices in liver cirrhosis patients. J Family Med Prim Care 2024; 13:1232-1237. [PMID: 38827661 PMCID: PMC11141952 DOI: 10.4103/jfmpc.jfmpc_792_23] [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: 05/11/2023] [Revised: 07/08/2023] [Accepted: 07/12/2023] [Indexed: 06/04/2024] Open
Abstract
Background Portal hypertension commonly occurs due to liver cirrhosis, and esophageal varices (EV) is one of the major complications associated with it. The most common cause of death in liver cirrhosis is EV bleeding. Hence, GE screening for EV is required, which is an invasive procedure. Regular use of endoscopy results in low compliance due to cost and discomfort for patients. Hence, identifying non-invasive markers that could grade EV provides a useful screening tool for family physicians and primary health centers (PHCs) by referring the patient to higher centers for definitive treatment, which could reduce mortality due to variceal bleeding in cirrhotic patients. Aims To assess non-invasive predictors of grade EV in patients diagnosed with liver cirrhosis. Settings and Design Cross-sectional study. Methods and Material A total of 109 patients with liver cirrhosis underwent clinical and biochemical evaluation, USG abdomen with spleen bipolar diameter, ascitic fluid analysis, and upper GE with a grade of EV are recorded. Statistical Analysis Used SPSS software with Student t-test, Chi-square t-test, analysis of variance, receiver operator characteristic (ROC) curves, and Spearman correlation with 95% CI is used. P <0.05 is considered significant. Results Aminotransferase to Platelet count Ratio Index (APRI) score >1.815, PC/SD ≤909, and SAAG >1.1g/dl showed EV in liver cirrhosis (P < 0.05). The order of prediction with ROC curves shows APRI score > PC/SD > SAAG. In grading EV, APRI scores of 1.9-2.5 and >2.5 showed small and large EV, respectively (P < 0.05). Conclusions APRI score may be used in PHC as an early intervention to grade EV and refer the patient to higher centers for definitive treatment. This would prevent the progression of varices to rupture and reduce mortality due to variceal bleeds in liver cirrhosis patients.
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Affiliation(s)
- Sushma S. Rapelly
- Department of Physiology, King George’s Medical University, Lucknow, Uttar Pradesh, India
| | - Shraddha Singh
- Department of Physiology, King George’s Medical University, Lucknow, Uttar Pradesh, India
| | - Narsingh Verma
- Department of Physiology, King George’s Medical University, Lucknow, Uttar Pradesh, India
| | - Sandeep Bhattacharya
- Department of Physiology, King George’s Medical University, Lucknow, Uttar Pradesh, India
| | - Sumit Rungta
- Department of Medical Gastroenterology, King George’s Medical University, Lucknow, Uttar Pradesh, India
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Mathur N, Patel H, Mangalia R, Mehta A, Mathur M, Verma A, Patyal A. Correlation of non-invasive parameters with upper gastrointestinal endoscopy findings for presence and grading of gastroesophageal varices in liver cirrhosis patients. J Family Med Prim Care 2024; 13:1347-1353. [PMID: 38827664 PMCID: PMC11141970 DOI: 10.4103/jfmpc.jfmpc_702_23] [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: 04/26/2023] [Revised: 11/27/2023] [Accepted: 11/30/2023] [Indexed: 06/04/2024] Open
Abstract
Background Development of esophageal varices is one of the major complications of liver cirrhosis, and endoscopy is used to see the presence, grading, and long-term monitoring of esophageal varices which is an invasive and unpleasant procedure. There is no adequate data available showing noninvasive methods can be used for the same. Methods Seventy patients with liver cirrhosis participated in the study. Factors like portal vein diameter, spleen size, platelet count, serum bilirubin, Child-Pugh score, prothrombin time (PT), and PT INR were observed and correlated endoscopically with the presence and grading of esophageal varices in all patients. Results The platelet count, portal vein diameter, serum bilirubin, spleen bipolar diameter, and PT had statistically significant correlations with the presence of varices. Among them, platelet count, portal vein diameter, and serum bilirubin also had statistically significant correlations with the grading of varices. Monitoring of these noninvasive parameters can help in monitoring variceal growth. Conclusions Noninvasive parameters can be used effectively to predict the presence and grading of esophageal varices and at the same time keep the rate of undiagnosed varices acceptably low. By using noninvasive parameters, patients can be benefited by decreasing the requirement of repeated endoscopic evaluation which is an unpleasant procedure and availability is also limited.
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Affiliation(s)
- Navgeet Mathur
- Department of General Medicine, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
| | - Harsh Patel
- Department of General Medicine, GMERS Medical College and Hospital, Sola, Ahmedabad, Gujarat, India
| | - Ravi Mangalia
- Department of General Medicine, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
| | - Amit Mehta
- Department of General Medicine, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
| | - Medha Mathur
- Department of Community Medicine, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
| | - Anjana Verma
- Department of Community Medicine, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
| | - Ashish Patyal
- Department of Anesthesiology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
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Ravindranath A, Yachha SK. An Approach to Investigations of Chronic Liver Disease. Indian J Pediatr 2024; 91:262-269. [PMID: 37702974 DOI: 10.1007/s12098-023-04751-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/08/2023] [Accepted: 06/23/2023] [Indexed: 09/14/2023]
Abstract
Chronic liver disease (CLD) in children is more diverse compared to adults with respect to the etiology, progression and response to therapy. After history and clinical examination, the first step is to confirm the presence of CLD with basic blood investigations and ultrasonography. Markers of portal hypertension are splenomegaly, increased portal vein diameter, thrombocytopenia and presence of varices on endoscopy. The next step is to evaluate the etiology of CLD which will depend on the age of the child and needs targeted investigations as metabolic and inherited causes predominate in early childhood. CLD progression ought to be monitored regularly and several non-invasive markers are available but they have to be evaluated further in children. Since CLD progresses, complications have to be detected early not only to initiate appropriate treatment but also to prognosticate.
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Affiliation(s)
- Aathira Ravindranath
- Department of Pediatric Gastroenterology, Institute of Gastrointestinal Sciences, Apollo BGS Hospitals, Kuvempunagar, Mysore, Karnataka, 570023, India
| | - Surender Kumar Yachha
- Department of Pediatric Gastroenterology, Hepatology and Liver Transplantation, Sakra World Hospital, Bengaluru, Karnataka, 560103, India.
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Poddar U, Samanta A, Sarma MS, Kumar B, Lal R, Srivastava A, Upadhyaya VD, Yachha SK, Mandelia A. How to suspect the presence of high-risk esophageal varices and when to start endoscopic surveillance in children with biliary atresia? J Gastroenterol Hepatol 2023; 38:1610-1617. [PMID: 37407246 DOI: 10.1111/jgh.16267] [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: 04/13/2023] [Revised: 05/30/2023] [Accepted: 06/07/2023] [Indexed: 07/07/2023]
Abstract
BACKGROUND AND AIM Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA. METHODS From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices. RESULTS A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices. CONCLUSION Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding.
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Affiliation(s)
- Ujjal Poddar
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Basant Kumar
- Pediatric Surgical Specialty, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Richa Lal
- Pediatric Surgical Specialty, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Anshu Srivastava
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Vijay Datta Upadhyaya
- Pediatric Surgical Specialty, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Surender Kumar Yachha
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
| | - Ankur Mandelia
- Pediatric Surgical Specialty, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
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A Model for Early Endoscopic Detection of High-Risk Gastroesophageal Varices in Children With Biliary Atresia. J Pediatr Gastroenterol Nutr 2022; 74:643-650. [PMID: 34984987 DOI: 10.1097/mpg.0000000000003375] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVE In children with biliary atresia and portal hypertension, progression to gastroesophageal varices carrying a risk of bleeding depends on age, total serum bilirubin concentration and initial endoscopic features. We report an attempt to use these factors for early detection of high-risk varices (HRVs). METHODS Based on different combinations of these factors, a model was set to estimate the probabilities of emergence of HRVs at various time intervals. A 10% probability was chosen to set the date of the next endoscopy in children who did not display HRVs initially. A total of 113 children without HRVs who underwent their first endoscopy before age 8 in 2013-2020 were included. A comparison was made with children seen during the period 1990-2012 when this model was not used. RESULTS In all, 65 of the 113 children underwent one to five additional endoscopies at dates set according to the model. The emergence of HRVs was recorded in 22 children after a mean interval of 14 months and was managed by endoscopic primary prophylaxis in all but one who underwent liver transplantation. Three other children bled before the next planned endoscopy. Compared with 175 children of the same age ranges without HRVs in the period 1990-2012, the use of the model was associated with a faster detection of HRVs with a lower number of endoscopic procedures (P = 0.0022 and P = 0.023, respectively). CONCLUSION The results suggest that the model reported may be a useful tool for the early detection of HRVs to allow primary prophylaxis of bleeding.
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Overview of Albumin Physiology and its Role in Pediatric Diseases. Curr Gastroenterol Rep 2021; 23:11. [PMID: 34213692 DOI: 10.1007/s11894-021-00813-6] [Citation(s) in RCA: 27] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/31/2021] [Indexed: 01/15/2023]
Abstract
PURPOSE OF REVIEW Albumin plays a critical role in a wide range of disease processes; however, the role of albumin in pediatric patients has not been well described. This article aims to review albumin physiology and kinetics in children, albumin's impact on pediatric diseases, and the utility of albumin as a predictor of clinical outcome. RECENT FINDINGS Hypoalbuminemia is seen in a wide range of conditions, including protein-losing enteropathy, hepatic synthetic failure, malnutrition, inflammatory states, and renal disease. While the impact of hypoalbuminemia has been more extensively studied in adult patients, there is a relative paucity of literature in the pediatric population. Hypoalbuminemia is a marker of poor outcome in critically ill children and those undergoing a wide range of medical interventions. Albumin infusions may be an effective therapy for fluid resuscitation and for patients with severe hypoalbuminemia.
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Non-invasive Predictors of Esophageal Varices With a High Risk of Bleeding in Pediatric Cirrhotic Patients. J Pediatr Gastroenterol Nutr 2021; 72:802-806. [PMID: 33399326 DOI: 10.1097/mpg.0000000000003039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
OBJECTIVES To evaluate non-invasive predictive factors of varices with a high risk of bleeding in pediatric cirrhotic patients. METHODS This retrospective, cross-sectional study included data from 158 children with cirrhosis, median age of 5.38 years (interquartile [IQ] 2.08-11.52 years), and no history of upper gastrointestinal bleeding. Patients underwent an endoscopy to screen for esophageal varices. Varices with a high risk of bleeding were defined as those with a medium to large caliber, presence of red spots, or the presence of gastric varices and identified as high-risk varices (HRV). Laboratory and clinical factors were evaluated as possible predictors of HRV. RESULTS HRV were detected in 30 children (19%) after the first endoscopy. In the multivariate analysis, only the risk score (RS), as described by Park et al, and the aspartate aminotransferase-to-platelet ratio index (APRi) were predictive of HRV. The best non-invasive predictor of HRV was the RS with an area under the receiver operating characteristic curve of 0.764. When used a cut-off point of -1.2, the sensitivity of the RS was 90% and specificity was 53%. The use of RS or APRi correctly identified 96% of children with HRV. CONCLUSIONS The described predictors allow the correct identification of patients with HRV. The association of RS >-1.2 or APRi >1.4 has a good sensitivity to identify HRV and to prevent unnecessary endoscopy in about one-third of children with no HRV.
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Rahmani P, Farahmand F, Heidari G, Sayarifard A. Noninvasive markers for esophageal varices in children with cirrhosis. Clin Exp Pediatr 2021; 64:31-36. [PMID: 32718149 PMCID: PMC7806413 DOI: 10.3345/cep.2019.01599] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2019] [Accepted: 06/15/2020] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND The diagnosis of esophageal varices (EV) is based on the findings of esophagogastroduodenoscopy (EGD), biopsy, and serum markers. Thus, noninvasive cost-effective tests through which high-risk EV children can be diagnosed are needed. PURPOSE This cross-sectional study aimed to identify the noninvasive markers for EV in children with liver cirrhosis. METHODS A total of 98 children with liver cirrhosis were evaluated in this study. The spleen size, platelet count, serum albumin, liver function test results, and risk scores were evaluated prior to endoscopy. The endoscopic investigations aimed to identify the presence of EV and red signs, and determine varices sizes. RESULTS Endoscopy revealed varices in 43 subjects (43.9%). The spleen size, platelet count, international normalized ratio, aspartate aminotransferase to platelet ratio index (APRI), platelet count to spleen size ratio, and risk score differed significantly between patients with and without EV on univariate analysis; however, the logistic regression analysis showed no differences, indicating that none of these parameters were independently associated with the presence of EV. CONCLUSION Platelet count, risk score, platelet count to spleen size, and APRI can be useful tools for the identification of highrisk patients with EV and might reduce the need for invasive methods like EGD.
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Affiliation(s)
- Parisa Rahmani
- Pediatric Gastroenterology and Hepatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Fatemeh Farahmand
- Pediatric Gastroenterology and Hepatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
| | - Ghobad Heidari
- Department of Pediatrics, Faculty of Medicine, Lorestan University of Medical Sciences, Khorramabad
| | - Azadeh Sayarifard
- Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran
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Garlapati P, Rahman EU, Gayam V, Chobufo MD, Fatima F, Khan AM, Suliman M, Thompson EA, El-Hamdani M, Aronow WS. Outcomes of oesophageal variceal bleeding among patients with atrial fibrillation: a propensity-matched analysis of a nationwide inpatient sample. Arch Med Sci Atheroscler Dis 2020; 5:e255-e262. [PMID: 33305064 PMCID: PMC7717439 DOI: 10.5114/amsad.2020.98949] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 08/23/2020] [Indexed: 11/27/2022] Open
Abstract
INTRODUCTION We aimed to determine the influence of atrial fibrillation (AF) on mortality, morbidity, length of hospital stay, and resource utilisation in patients with oesophageal variceal bleeding (OVB). MATERIAL AND METHODS The National Inpatient Sample database (2016 and 2017) was used for data analysis using the International Classification of Diseases, Tenth Revision codes to identify patients with the principal diagnosis of OVB and AF. We assessed the all-cause in-hospital mortality, morbidity, predictors of mortality, length of hospital stay (LOS), and total costs between propensity-matched groups of OVB with AF vs. OVB alone. RESULTS We identified 80,325 patients with OVB, of whom 4285 had OVB with AF, and 76,040 had OVB only. The in-hospital mortality was higher in OVB with AF (OR = 1.4, 95% CI: 1.09-1.83; p < 0.001). OVB with AF had higher odds of sepsis (OR = 1.4, 95% CI: 1.1-1.8; p = 0.007), acute kidney injury (OR = 1.2, 95% CI: 1.12-1.32; p < 0.001), and mechanical ventilation (OR = 1.2, 95% CI: 1.12-1.32; p < 0.001). Advanced age (OR = 1.06, 95% CI: 1.05-1.07; p < 0.001), congestive heart failure (OR = 1.7, 95% CI: 1.3-2.3; p < 0.001), coronary artery disease (OR = 1.4, 95% CI: 1.03-1.92; p = 0.02), and sepsis (OR = 1.3, 95% CI: 1.06-1.70; p = 0.01) were identified as predictors of mortality in OVB with AF. Mean LOS (7.5 ±7.4 vs. 6.0 ±7.2, p < 0.001) and mean total costs ($25,452 vs. $21,109, p < 0.001) were also higher. CONCLUSIONS In this propensity-matched analysis, OVB with AF was associated with higher odds of in-hospital mortality, sepsis, acute kidney injury, and mechanical ventilation.
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Affiliation(s)
| | | | | | | | - Farah Fatima
- Deccan College of Medical Sciences, Hyderabad, India
| | | | | | | | | | - Wilbert S. Aronow
- Westchester Medical Centre and New York Medical College, Valhalla, NY, USA
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GonÇalves MB, Alves BDQ, Marianelli BF, Polizelli MU, Azevedo RAD, Moraes NSBD. VALUE OF CLINICAL, LABORATORY PARAMETERS AND ANALYSIS OF RETINAL VASCULARIZATION IN PEDIATRIC PATIENTS WITH PORTAL HYPERTENSION. ARQUIVOS DE GASTROENTEROLOGIA 2020; 57:121-125. [PMID: 32609163 DOI: 10.1590/s0004-2803.202000000-41] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/29/2019] [Accepted: 04/27/2020] [Indexed: 11/21/2022]
Abstract
BACKGROUND Portal hypertension is one of the complications of cirrhosis and is associated with numerous systemic manifestations, including renal, brain, pulmonary, cardiac and vascular changes. In routine ophthalmological examinations performed at our service, we observed that some children diagnosed with portal hypertension had increased retinal vascular tortuosity. OBJECTIVE 1. To evaluate the presence of retinal vascular abnormalities (vascular tortuosity) in children diagnosed with portal hypertension; 2. To investigate the association between retinal vascular tortuosity and the presence of gastroesophageal varices in these children; 3. To evaluate the use of clinical and laboratory parameters to predict the presence of gastroesophageal varices in children with portal hypertension. METHODS This was a cross-sectional and observational study that included patients aged <18 years with a diagnosis of portal hypertension. The participants included were submitted to dilated fundus examination and fundus photography with Visucam (Carl Zeiss Meditec AG) device. Besides, clinical and laboratorial data were collected from the patients' medical records. RESULTS A total of 72 patients were included in this study, and 36% of them had an increase in retinal vascular tortuosity. Platelet count (P=0.001), bilirubin dosage (P=0.013) and aspartate transaminase dosage (AST) (P=0.042) were associated with the presence of gastroesophageal varices in digestive endoscopy. There was no association between retinal vascular tortuosity and the presence of gastroesophageal varices (P=0.498). CONCLUSION The results of this study suggest that platelet count, bilirubin dosage, and aspartate transaminase dosage were associated with the presence of gastroesophageal varices in digestive endoscopy. Regarding the retinal findings, we found that there was an increase in retinal vascular tortuosity in 36% of pediatric patients, but no association was found with the presence of gastroesophageal varices.
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Affiliation(s)
- Mariana Batista GonÇalves
- Universidade Federal de São Paulo, Departamento de Oftalmologia e Ciências Visuais, Divisão de Retina e Vítreo, São Paulo, SP, Brasil
| | - Bruno de Queiroz Alves
- Universidade Federal de São Paulo, Departamento de Oftalmologia e Ciências Visuais, Divisão de Retina e Vítreo, São Paulo, SP, Brasil
| | - Bruna Ferraço Marianelli
- Universidade Federal de São Paulo, Departamento de Oftalmologia e Ciências Visuais, Divisão de Retina e Vítreo, São Paulo, SP, Brasil
| | - Murilo Ubukata Polizelli
- Universidade Federal de São Paulo, Departamento de Oftalmologia e Ciências Visuais, Divisão de Retina e Vítreo, São Paulo, SP, Brasil
| | - Ramiro Anthero de Azevedo
- Universidade Federal de São Paulo, Divisão de Cirurgia Pediátrica, São Paulo, SP, Brasil.,Universidade Federal de São Paulo, Divisão de Gastroenterologia Pediátrica, São Paulo, SP, Brasil
| | - Nilva Simeren Bueno de Moraes
- Universidade Federal de São Paulo, Departamento de Oftalmologia e Ciências Visuais, Divisão de Retina e Vítreo, São Paulo, SP, Brasil
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Yuldashev RZ, Aliev MM, Shokhaydarov SI, Tursunova DB. Spleen stiffness measurement as a non-invasive test to evaluate and monitor portal hypertension in children with extrahepatic portal vein obstruction. Pediatr Surg Int 2020; 36:637-641. [PMID: 32206893 DOI: 10.1007/s00383-020-04648-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/05/2020] [Indexed: 02/08/2023]
Abstract
PURPOSE The aim of the study was to test the feasibility of spleen stiffness measurement (SSM) by two-dimensional shear wave elastography (2D-SWE) and compare data on its diagnostic use with upper gastrointestinal endoscopy in children with extrahepatic portal hypertension (EHPH) before and after surgery. METHODS A total of 44 children were included in this study [34 children with EHPH (main group)] and 10 controls (7.57 ± 1.22 years), who underwent ultrasonography including SSM by 2D-SWE. Patients in the main group also underwent upper gastrointestinal endoscopy (UGE) and CT angiography. The main group was divided into three subgroups: Group A: patients with EHPH without large spontaneous portosystemic shunts (n = 15); Group B: patients with EHPH with large spontaneous portosystemic shunts (n = 9); Group C: patients with EHPVO and after surgical portosystemic shunts (n = 10). RESULTS According to UGE, children in group A had significantly higher grades of esophageal varices (EV) (2.3 ± 0.14; p < 0.001) compared to those in groups B and C. After surgical shunting procedures (in group C), the grade of EV declined to 0.37 ± 0.14. There was significant difference (p < 0.001) in the mean SS of children in group A (70 ± 4.64 kPa) compared to those in group B (37.04 ± 4.62 kPa) and group C (26.3 ± 2.9 kPa). After surgery, SS decreased but remained elevated compared with controls (26.3 ± 2.9 vs 17.85 ± 1.3 kPa; p = 0.016). The SS showed a small but significant correlation with grades of EV (r = 0.56, p = 0.002). CONCLUSIONS The SS measured by 2D-SWE is feasible in children with EHPH and the results reflect the presence or degree of EV, thus elastography of spleen is useful in monitoring portal hypertension before and after shunt surgeries.
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Affiliation(s)
- R Z Yuldashev
- Department of Pediatric Surgery, Republican Specialized Scientific Practical Medical Center of Pediatrics, Tashkent, Uzbekistan. .,Department of Pediatric Surgery, Tashkent Pediatric Medical Institute, Tashkent, Uzbekistan.
| | - M M Aliev
- Department of Pediatric Surgery, Tashkent Pediatric Medical Institute, Tashkent, Uzbekistan
| | - Sh I Shokhaydarov
- Department of Pediatric Surgery, Republican Specialized Scientific Practical Medical Center of Pediatrics, Tashkent, Uzbekistan
| | - D B Tursunova
- Department of Radiology, Republican Specialized Scientific Practical Medical Center of Pediatrics, Tashkent, Uzbekistan
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Gunda DW, Kilonzo SB, Mamballah Z, Manyiri PM, Majinge DC, Jaka H, Kidenya BR, Mazigo HD. The magnitude and correlates of esophageal Varices among newly diagnosed cirrhotic patients undergoing screening fibre optic endoscope before incident bleeding in North-Western Tanzania; a cross-sectional study. BMC Gastroenterol 2019; 19:203. [PMID: 31783802 PMCID: PMC6884911 DOI: 10.1186/s12876-019-1123-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2018] [Accepted: 11/19/2019] [Indexed: 01/09/2023] Open
Abstract
BACKGROUND Bleeding esophageal varices is a deadly complication of liver cirrhosis. Guidelines recommend an early diagnosis of esophageal varices before incident bleeding by screening all patients diagnosed with liver cirrhosis. Though it has been reported elsewhere that the presence of esophageal varices varies widely among cirrhotic patients this has not been assessed in Tanzania since endoscopy is not readily available for routine use in our setting. This study was designed to determine the prevalence of esophageal varices and assess the utility of clinical parameters in predicting the presence of varices among cirrhotic patients in northwestern Tanzania. METHODS A cross-sectional analysis of adult patients with liver cirrhosis was done at Bugando Medical Centre. Demographic, clinical, laboratory and endoscopic data were collected and analyzed using STATA 13. The presence of esophageal varices was detected using endoscopic examination and associated factors were assessed by logistic regression. The predictive value of clinical predictors was also assessed by calculating sensitivity and specificity. RESULTS A total of 223 patients were enrolled, where 88 (39.5%; 95%CI: 33.0-45.9) had esophageal varices. The varices were independently associated with increased age (OR: 1.02; 95%CI: 1.0-1.04; p = 0.030); increased splenic diameter (OR:1.3; 95%CI:1.2-1.5; p < 0.001), increased portal vein diameter (OR:1.2; 95%CI: 1.07-1.4; p = 0.003), having ascites (OR: 3.0; 95%CI: 1.01-8.7; p = 0.046), and advanced liver disease (OR: 2.9; 95%CI: 1.3-6.7; p = 0.008). PSDR least performed in predicting varices, (AUC: 0.382; 95%CI: 0.304-0.459; cutoff: < 640; Sensitivity: 58.0%; 95%CI: 46.9-68.4; specificity: 57.0%; 95%CI: 48.2-65.5). SPD had better prediction; (AUC: 0.713; 95%CI: 0.646-0.781; cut off: > 15.2 cm; sensitivity: 65.9%; (95% CI: 55-75.7 and specificity:65.2%; 95%CI: 56.5-73.2), followed by PVD, (AUC: 0.6392; 95%CI: 0.566-0.712;cutoff: > 1.45 cm; sensitivity: 62.5%; 95CI: 51.5-72.6; specificity: 61.5%; 95%CI: 52.7-69.7). CONCLUSION Esophageal varices were prevalent among cirrhotic patients, most of which were at risk of bleeding. The non-invasive prediction of varices was not strong enough to replace endoscopic diagnosis. However, the predictors in this study can potentially assist in the selection of patients at high risk of having varices and prioritize them for endoscopic screening and appropriate management.
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Affiliation(s)
- Daniel W. Gunda
- Department of medicine, Weill Bugando School of Medicine, P.O Box 1464, Mwanza, Tanzania
- Department of medicine, Bugando medical center, 1370 Mwanza, Tanzania
| | - Semvua B. Kilonzo
- Department of medicine, Weill Bugando School of Medicine, P.O Box 1464, Mwanza, Tanzania
- Department of medicine, Bugando medical center, 1370 Mwanza, Tanzania
| | - Zakhia Mamballah
- Department of medicine, Weill Bugando School of Medicine, P.O Box 1464, Mwanza, Tanzania
| | | | - David C. Majinge
- Department of medicine, Bugando medical center, 1370 Mwanza, Tanzania
| | - Hyasinta Jaka
- Department of medicine, Bugando medical center, 1370 Mwanza, Tanzania
- Lake Zone Health Training institute, 11351 Bugando Mwanza, Tanzania
| | - Benson R. Kidenya
- Department of Biochemistry and Molecular Biology, Weill Bugando School of Medicine, Mwanza, Tanzania
| | - Humphrey D. Mazigo
- Department of Parasitology, Weill Bugando School of Medicine, 1464 Mwanza, Tanzania
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Abstract
Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are prototype noncirrhotic causes of portal hypertension (PHT), characterized by normal hepatic venous pressure gradient, variceal bleeds, and moderate to massive splenomegaly with preserved liver synthetic functions. Infections, toxins, and immunologic, prothrombotic and genetic disorders are possible causes in IPH, whereas prothrombotic and local factors around the portal vein lead to EHPVO. Growth failure, portal biliopathy, and minimal hepatic encephalopathy are long-term concerns in EHPVO. Surgical shunts and transjugular intrahepatic portosystemic shunt resolve the complications secondary to PHT. Meso-Rex shunt is now the standard-of-care surgery in children with EHPVO.
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Affiliation(s)
- Rajeev Khanna
- Department of Pediatric Hepatology, Institute of Liver & Biliary Sciences (ILBS), D-1, Vasant Kunj, New Delhi 110 070, India
| | - Shiv Kumar Sarin
- Department of Hepatology, Institute of Liver & Biliary Sciences (ILBS), D-1, Vasant Kunj, New Delhi 110 070, India.
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14
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Non-endoscopic predictors of esophageal varices in children with chronic liver disease and their utility in resource-constrained countries. Indian J Gastroenterol 2019; 38:310-316. [PMID: 31423562 DOI: 10.1007/s12664-019-00960-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2018] [Accepted: 04/30/2019] [Indexed: 02/04/2023]
Abstract
BACKGROUND Although endoscopy is the standard diagnostic screening test to identify esophageal varices in patients with chronic liver disease (CLD), selective endoscopy in patients who are at higher risk of having varices may be cost-effective in a resource-constrained country. The aim of this prospective study was to identify non-endoscopic parameters that may predict the presence of varices, especially high-risk esophageal varices in children with CLD. METHODS From January 2016 through March 2018, consecutive children with CLD without a history of variceal bleeding were prospectively included. Esophagogastroduodenoscopy was done in all the children to detect and to grade esophageal varices. Both univariate and multivariate logistic regression analyses were done using SPSS version 22 to identify factors associated with esophageal varices. RESULTS The mean age of 84 children was 9.7 ± 3.2 years (male 44). Esophageal varices were present in 71.4% of children and 55% of them had large varices. On univariate analysis, low platelet count (< 100,000/mm3) and splenomegaly were found to be associated with the presence of esophageal varices (p = 0.006 and 0.001, respectively) and large varices (p = 0.03 and 0.01, respectively). On multivariate analysis, both low platelet count and splenomegaly were independent predictors for the presence of esophageal varices (respectively, OR 11.21, 95% CI 1.2-96.9; and OR 11.39, 95% CI 3.19-40.59). CONCLUSIONS Splenomegaly and low platelet count independently predict the presence of any grade of esophageal varices and can be used as screening tests to select children for endoscopy. This strategy may help in relieving medical, social, and economic costs in resource-constrained countries.
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15
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Pittman ME, Kierans AS, Rao D, Yantiss RK, Samstein B, Jessurun J. Portal Cavernoma Cholangiopathy: Histologic Features and Differential Diagnosis. Am J Clin Pathol 2019; 151:255-262. [PMID: 30357333 DOI: 10.1093/ajcp/aqy132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Objectives Portal cavernoma cholangiopathy (formerly portal biliopathy) is a type of biliary injury that occurs in association with a portal vein thrombus or cavernoma. Although the radiographic features of portal cavernoma cholangiopathy have been enumerated in the literature, its histologic features have not been described in detail. Methods We describe the histologic findings in liver specimens from three patients with radiologically confirmed portal cavernoma cholangiopathy. Results Of the three patients, one underwent surgical resection due to a clinical suspicion for cholangiocarcinoma, one had a liver biopsy sample obtained for evaluation of possible cirrhosis, and one had a clinically suspicious "hilar mass" at the time of orthotopic liver transplant. Histologic features common among the three liver specimens included portal venous abnormalities, where the portal veins were obliterated or small relative to the portal tract size, and obstructive biliary changes, such as ductular reaction and reactive epithelial atypia accompanied by a mixed inflammatory cell infiltrate with neutrophils. Conclusions This case series provides clinicopathologic characteristics of portal cavernoma cholangiopathy. Histologic changes are reminiscent of hepatoportal sclerosis and/or bile duct obstruction. Attention to portal veins can provide helpful diagnostic clues, especially when biopsy samples are obtained from patients with a known portal vein thrombus or cavernoma.
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Affiliation(s)
- Meredith E Pittman
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY
| | | | - Deepthi Rao
- Department of Pathology and Anatomical Sciences, University of Missouri, Columbia
| | - Rhonda K Yantiss
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY
| | - Benjamin Samstein
- Center for Liver Disease and Transplantation, Department of Surgery, Weill Cornell Medicine, New York, NY
| | - Jose Jessurun
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY
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16
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Chiou FK, Ong C, Low Y, Chiang LW, Phua KB. Non-Invasive Predictors for the First Variceal Hemorrhage in Children With Biliary Atresia After Kasai Portoenterostomy. J Clin Exp Hepatol 2019; 9:581-587. [PMID: 31695248 PMCID: PMC6823700 DOI: 10.1016/j.jceh.2019.03.005] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/03/2018] [Accepted: 03/17/2019] [Indexed: 12/12/2022] Open
Abstract
BACKGROUND/AIM : Portal hypertension and variceal hemorrhage (VH) are significant complications in biliary atresia (BA). The study aims to evaluate risk factors and noninvasive markers that predict actual VH for the first time in children with BA without prior endoscopic surveillance or treatment. METHODS Retrospective review was performed of patients diagnosed with BA from 1989 to 2016 at a single center. Primary outcome was the first episode of VH. Patients were stratified into VH and non-VH groups according to the development of VH, and laboratory and ultrasonographic data were analyzed at 2 time points: pre-VH and the last follow-up. Existing indices, varices prediction rule (VPR), and aspartate aminotransferase (AST)-platelet ratio index (APRI) were also applied retrospectively to evaluate their performance in prediction of VH in our cohort. RESULTS Seventy-two patients were included; 16 patients developed the first VH at median age of 5.5 years. On univariate analysis, serum albumin (P = 0.034), AST (P = 0.017), hemoglobin (P = 0.019), platelet count (P = <0.001), spleen size Z-score (P = <0.001), and rate of splenic enlargement (P = 0.006) were associated with VH. On multivariable regression analysis, only platelet count was independently predictive (P = 0.041). The optimal cutoff values for prediction of the first VH were platelet count ≤100 × 109/L (sensitivity 75.0%, specificity 80.4%, positive predictive value [PPV] 52.2%, negative predictive value [NPV] 91.8%), VPR ≤3.0 (sensitivity 81.3%, specificity 85.7%, PPV 61.9%, NPV 94.1%), and APRI ≥3.0 (sensitivity 81.3%, specificity 76.8%, PPV 50.0%, NPV 93.5%). CONCLUSIONS Platelet count <100 × 109/L and VPR <3.0 are simple, reproducible and effective noninvasive markers in predicting the first episode of acute VH in children with BA and may be used in pediatrics for the selection of patients to undergo primary prophylactic endoscopic therapy.
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Key Words
- ALT, alanine aminotransferase
- APRI, aspartate aminotransferase-platelet ratio index
- AST, aspartate aminotransferase
- AUROC, area under receiver operating characteristic curve
- BA, biliary atresia
- EV, esophageal varices
- KP, Kasai portoenterostomy
- NPV, negative predictive value
- OR, odds ratio
- PPV, positive predictive value
- ROC, receiver operating characteristic
- VH, variceal hemorrhage
- VPR, varices prediction rule
- esophageal varices
- hypersplenism
- liver cirrhosis
- pediatrics
- portal hypertension
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Affiliation(s)
- Fang K. Chiou
- Gastroenterology, Hepatology & Nutrition Service, Paediatric Medicine, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore,Address for correspondence: Fang K. Chiou, Paediatric Gastroenterologist, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore.
| | - Christina Ong
- Gastroenterology, Hepatology & Nutrition Service, Paediatric Medicine, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore
| | - Yee Low
- Paediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore
| | - Li W. Chiang
- Paediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore
| | - Kong B. Phua
- Gastroenterology, Hepatology & Nutrition Service, Paediatric Medicine, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, 229899, Singapore
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Nicholls L, Montez J, Pelayo-Katsanis L, Katsanis E. Esophageal Varices in Adolescent and Young Adult Males with Acute Lymphocytic Leukemia. J Adolesc Young Adult Oncol 2018; 8:217-220. [PMID: 30335538 DOI: 10.1089/jayao.2018.0098] [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: 11/13/2022] Open
Abstract
Hepatic late effects are not commonly reported in survivors of childhood leukemia. Four young male patients with acute lymphoblastic leukemia (ALL) were diagnosed with bleeding esophageal varices (EVs) during or shortly after completion of maintenance chemotherapy. EVs were identified from 0 to 60 months after completion of leukemia therapy. All four patients were men between 20 to 24 years old. Hematemesis was the most common presenting symptom. Associated features included splenomegaly, cytopenias, azole therapy, alcohol use, and hepatic iron overload. EVs may be an under-recognized complication of ALL therapy, with adolescent and young adult males at highest risk.
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Affiliation(s)
- Lauren Nicholls
- 1 Department of Pediatrics, University of Arizona, Tucson, Arizona.,2 Department of Pediatric Hematology/Oncology, University of Arizona Cancer Center, University of Arizona , Tucson, Arizona.,3 Department of Pediatric Hematology/Oncology, Banner University Medical Center , Tucson, Arizona
| | - Jana Montez
- 1 Department of Pediatrics, University of Arizona, Tucson, Arizona.,3 Department of Pediatric Hematology/Oncology, Banner University Medical Center , Tucson, Arizona
| | - Luz Pelayo-Katsanis
- 1 Department of Pediatrics, University of Arizona, Tucson, Arizona.,3 Department of Pediatric Hematology/Oncology, Banner University Medical Center , Tucson, Arizona
| | - Emmanuel Katsanis
- 1 Department of Pediatrics, University of Arizona, Tucson, Arizona.,2 Department of Pediatric Hematology/Oncology, University of Arizona Cancer Center, University of Arizona , Tucson, Arizona.,3 Department of Pediatric Hematology/Oncology, Banner University Medical Center , Tucson, Arizona.,4 Department of Immunobiology, University of Arizona, Tucson, Arizona.,5 Department of Medicine, and University of Arizona, Tucson, Arizona.,6 Department of Pathology, University of Arizona, Tucson, Arizona
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18
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Authors' Response. J Pediatr Gastroenterol Nutr 2018; 67:e41. [PMID: 29746343 DOI: 10.1097/mpg.0000000000002026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
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Non-invasive Markers of Portal Hypertension: Appraisal of Adult Experience and Potential Utilisation in Children. J Pediatr Gastroenterol Nutr 2018; 66:559-569. [PMID: 29287004 DOI: 10.1097/mpg.0000000000001882] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Portal hypertension (PHT) is a significant cause of morbidity and mortality in children with chronic liver disease and portal vein obstruction. Increased portal pressure results in variceal formation along the gastrointestinal (GI) tract resulting in major bleeding. Identifying children with significant PHT who are more likely to suffer GI bleeding has been challenging and the role of surveillance upper GI endoscopy has been debated. This review analyses research done on serum biomarkers and imaging techniques as possible predictors of significant PHT. We evaluated the research performed on adult population, as well as the limited work done on children, to identify promising areas for future research. A literature search was conducted on "PubMed." Several search terms were used including "portal hypertension," "paediatric portal hypertension," "non-invasive markers of portal hypertension," "spleen stiffness," "liver stiffness," "elastography," and "endothelial damage." The articles included were selected based on their relevance to the purpose of our review. The research suggests a combination of several biomarkers, in addition to an imaging technique such as transient elastography or magnetic resonance elastography, would allow for the best prediction of significant varices. The most promising indicators would be those that are applicable in both intra- and extra-hepatic causes of PHT. Further research on these predictors in children with PHT is required to determine their potential role as selection criteria for PHT and stratification of surveillance GI endoscopies.
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Noninvasive Methods of Predicting Large Esophageal Varices in Children With Intrahepatic Portal Hypertension. J Pediatr Gastroenterol Nutr 2018; 66:442-446. [PMID: 29176477 DOI: 10.1097/mpg.0000000000001841] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVE Esophageal variceal bleeding is a severe complication of portal hypertension. The standard diagnostic screening test and therapeutic procedure for esophageal varices (EV) is endoscopy, which is invasive in pediatric patients. This study aimed to evaluate the role of noninvasive parameters as predictors of large varices in children with intrahepatic portal hypertension. METHODS Participants included in this cross-sectional study underwent a screening endoscopy. Variceal size, red marks, and portal gastropathy were assessed and rated. Patients were classified into two groups: Group 1 (G1) with small or no varices and Group 2 (G2) with large varices. The population consisted of 98 children with no history of gastrointestinal (GI) bleeding, with a mean age of 8.9 ± 4.7 years. The main outcome evaluated was the presence of large varices. RESULTS The first endoscopy session revealed the presence of large varices in 32 children. The best noninvasive predictors for large varices were platelets (Area under the ROC Curve [AUROC] 0.67; 95% CI 0.57-0.78), the Clinical Prediction Rule (CPR; AUROC 0.65; 95% CI 0.54-0.76), and risk score (AUROC 0.66; 95% CI 0.56-0.76). The logistic regression model showed that children with a CPR value under 114 were 8.59 times more likely to have large varices. Risk scores higher than -1.2 also increased the likelihood of large varices (OR 6.09; P = 0.014), as did a platelet count/spleen size z score lower than 25 (OR 3.99; P = 0.043). The combination of these three tests showed a high negative predictive value. CONCLUSIONS The CPR, the risk score, and the platelet count/spleen size z score could be helpful in identifying cirrhotic children who may be eligible for endoscopy.
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Khanna R, Sarin SK. Idiopathic portal hypertension and extrahepatic portal venous obstruction. Hepatol Int 2018; 12:148-167. [PMID: 29464506 DOI: 10.1007/s12072-018-9844-3] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2017] [Accepted: 01/19/2018] [Indexed: 02/06/2023]
Abstract
BACKGROUND Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO. CONCLUSION This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.
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Affiliation(s)
- Rajeev Khanna
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Shiv Kumar Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India.
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A review of long-term outcome and quality of life of patients after Kasai operation surviving with native livers. Pediatr Surg Int 2017; 33:1283-1287. [PMID: 28940041 DOI: 10.1007/s00383-017-4158-4] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/05/2017] [Indexed: 02/07/2023]
Abstract
Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy. These morbidities impose a huge impact on the quality of life of the patients and their families. Herein, we performed a comprehensive review on the clinical status and quality of life of long-term survivors of biliary atresia with their native livers, to facilitate meticulous longitudinal follow-up of these patients, and alert caregivers the probable complications to be aware of.
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Colli A, Gana JC, Yap J, Adams‐Webber T, Rashkovan N, Ling SC, Casazza G, Cochrane Hepato‐Biliary Group. Platelet count, spleen length, and platelet count-to-spleen length ratio for the diagnosis of oesophageal varices in people with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev 2017; 4:CD008759. [PMID: 28444987 PMCID: PMC6478276 DOI: 10.1002/14651858.cd008759.pub2] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND Current guidelines recommend screening of people with oesophageal varices via oesophago-gastro-duodenoscopy at the time of diagnosis of hepatic cirrhosis. This requires that people repeatedly undergo unpleasant invasive procedures with their attendant risks, although half of these people have no identifiable oesophageal varices 10 years after the initial diagnosis of cirrhosis. Platelet count, spleen length, and platelet count-to-spleen length ratio are non-invasive tests proposed as triage tests for the diagnosis of oesophageal varices. OBJECTIVES Primary objectives To determine the diagnostic accuracy of platelet count, spleen length, and platelet count-to-spleen length ratio for the diagnosis of oesophageal varices of any size in paediatric or adult patients with chronic liver disease or portal vein thrombosis, irrespective of aetiology. To investigate the accuracy of these non-invasive tests as triage or replacement of oesophago-gastro-duodenoscopy. Secondary objectives To compare the diagnostic accuracy of these same tests for the diagnosis of high-risk oesophageal varices in paediatric or adult patients with chronic liver disease or portal vein thrombosis, irrespective of aetiology.We aimed to perform pair-wise comparisons between the three index tests, while considering predefined cut-off values.We investigated sources of heterogeneity. SEARCH METHODS The Cochrane Hepato-Biliary Group Controlled Trials Register, the Cochrane Hepato-Biliary Group Diagnostic Test Accuracy Studies Register, the Cochrane Library, MEDLINE (OvidSP), Embase (OvidSP), and Science Citation Index - Expanded (Web of Science) (14 June 2016). We applied no language or document-type restrictions. SELECTION CRITERIA Studies evaluating the diagnostic accuracy of platelet count, spleen length, and platelet count-to-spleen length ratio for the diagnosis of oesophageal varices via oesophago-gastro-duodenoscopy as the reference standard in children or adults of any age with chronic liver disease or portal vein thrombosis, who did not have variceal bleeding. DATA COLLECTION AND ANALYSIS Standard Cochrane methods as outlined in the Cochrane Handbook for Diagnostic Test of Accuracy Reviews. MAIN RESULTS We included 71 studies, 67 of which enrolled only adults and four only children. All included studies were cross-sectional and were undertaken at a tertiary care centre. Eight studies reported study results in abstracts or letters. We considered all but one of the included studies to be at high risk of bias. We had major concerns about defining the cut-off value for the three index tests; most included studies derived the best cut-off values a posteriori, thus overestimating accuracy; 16 studies were designed to validate the 909 (n/mm3)/mm cut-off value for platelet count-to-spleen length ratio. Enrolment of participants was not consecutive in six studies and was unclear in 31 studies. Thirty-four studies assessed enrolment consecutively. Eleven studies excluded some included participants from the analyses, and in only one study, the time interval between index tests and the reference standard was longer than three months. Diagnosis of varices of any size. Platelet count showed sensitivity of 0.71 (95% confidence interval (CI) 0.63 to 0.77) and specificity of 0.80 (95% CI 0.69 to 0.88) (cut-off value of around 150,000/mm3 from 140,000 to 150,000/mm3; 10 studies, 2054 participants). When examining potential sources of heterogeneity, we found that of all predefined factors, only aetiology had a role: studies including participants with chronic hepatitis C reported different results when compared with studies including participants with mixed aetiologies (P = 0.036). Spleen length showed sensitivity of 0.85 (95% CI 0.75 to 0.91) and specificity of 0.54 (95% CI 0.46 to 0.62) (cut-off values of around 110 mm, from 110 to 112.5 mm; 13 studies, 1489 participants). Summary estimates for detection of varices of any size showed sensitivity of 0.93 (95% CI 0.83 to 0.97) and specificity of 0.84 (95% CI 0.75 to 0.91) in 17 studies, and 2637 participants had a cut-off value for platelet count-to-spleen length ratio of 909 (n/mm3)/mm. We found no effect of predefined sources of heterogeneity. An overall indirect comparison of the HSROCs of the three index tests showed that platelet count-to-spleen length ratio was the most accurate index test when compared with platelet count (P < 0.001) and spleen length (P < 0.001). Diagnosis of varices at high risk of bleeding. Platelet count showed sensitivity of 0.80 (95% CI 0.73 to 0.85) and specificity of 0.68 (95% CI 0.57 to 0.77) (cut-off value of around 150,000/mm3 from 140,000 to 160,000/mm3; seven studies, 1671 participants). For spleen length, we obtained only a summary ROC curve as we found no common cut-off between studies (six studies, 883 participants). Platelet count-to-spleen length ratio showed sensitivity of 0.85 (95% CI 0.72 to 0.93) and specificity of 0.66 (95% CI 0.52 to 0.77) (cut-off value of around 909 (n/mm3)/mm; from 897 to 921 (n/mm3)/mm; seven studies, 642 participants). An overall indirect comparison of the HSROCs of the three index tests showed that platelet count-to-spleen length ratio was the most accurate index test when compared with platelet count (P = 0.003) and spleen length (P < 0.001). DIagnosis of varices of any size in children. We found four studies including 277 children with different liver diseases and or portal vein thrombosis. Platelet count showed sensitivity of 0.71 (95% CI 0.60 to 0.80) and specificity of 0.83 (95% CI 0.70 to 0.91) (cut-off value of around 115,000/mm3; four studies, 277 participants). Platelet count-to-spleen length z-score ratio showed sensitivity of 0.74 (95% CI 0.65 to 0.81) and specificity of 0.64 (95% CI 0.36 to 0.84) (cut-off value of 25; two studies, 197 participants). AUTHORS' CONCLUSIONS Platelet count-to-spleen length ratio could be used to stratify the risk of oesophageal varices. This test can be used as a triage test before endoscopy, thus ruling out adults without varices. In the case of a ratio > 909 (n/mm3)/mm, the presence of oesophageal varices of any size can be excluded and only 7% of adults with varices of any size would be missed, allowing investigators to spare the number of oesophago-gastro-duodenoscopy examinations. This test is not accurate enough for identification of oesophageal varices at high risk of bleeding that require primary prophylaxis. Future studies should assess the diagnostic accuracy of this test in specific subgroups of patients, as well as its ability to predict variceal bleeding. New non-invasive tests should be examined.
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Affiliation(s)
- Agostino Colli
- A Manzoni Hospital ASST LeccoDepartment of Internal MedicineVia dell'Eremo, 9/11LeccoItaly23900
| | - Juan Cristóbal Gana
- Division of Pediatrics, Escuela de Medicina, Pontificia Universidad Católica de ChileGastroenterology and Nutrition Department85 LiraSantiagoRegion MetropolitanaChile8330074
| | - Jason Yap
- University of AlbertaDivision of Pediatric Gastroenterology, Hepatology and Nutrition, Dept. of Pediatrics, Stollery Children's Hospital, Faculty of MedicineAberhart Centre 111402 University AveEdmontonABCanadaT6G 2J3
| | | | - Natalie Rashkovan
- Sunnybrook Health Sciences CentreDepartment of Neurology2075 Bayview ave., room A448TorontoONCanadaM4N 3M5
| | - Simon C Ling
- The Hospital for Sick ChildrenDivision of Gastroenterology, Hepatology and Nutrition555 University AvenueTorontoONCanadaM5G 1X8
| | - Giovanni Casazza
- Università degli Studi di MilanoDipartimento di Scienze Biomediche e Cliniche "L. Sacco"via GB Grassi 74MilanItaly20157
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King's Variceal Prediction Score: A Novel Noninvasive Marker of Portal Hypertension in Pediatric Chronic Liver Disease. J Pediatr Gastroenterol Nutr 2017; 64:518-523. [PMID: 27749613 DOI: 10.1097/mpg.0000000000001423] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND AIMS Variceal haemorrhage can be a life-threatening complication of chronic liver disease in children. There is limited evidence about the optimal prophylactic management and selection criteria of children who will benefit from upper gastrointestinal endoscopy. METHODS Children presenting in our centre with suspected portal hypertension or gastrointestinal bleeding and undergoing their first oesophagogastroduodenoscopy between 2005 and 2012 were included. Clinical, biochemical, and radiological data were collected. A separate validation cohort from May 2013 to October 2014 was obtained. RESULTS Data on 124 treatment-naïve patients were collected; 50% had biliary atresia. Thirty-five (28%) children presented with gastrointestinal bleeding and overall 79 (64%) had clinically significant (grade II-III) varices. Clinical prediction rule, aspartate aminotransferase-platelet ratio index, and varices prediction rule had at optimal cut-off sensitivity and specificity of 76% and 59%, 60% and 55%, and 80% and 59%, respectively. Logistic regression yielded a new prediction rule of (3 × albumin ([g/dL]) - (2 - equivalent adult spleen size [cm]). This King's variceal prediction score had a favourable areas under the curve of 0.772 (0.677-0.867) compared to clinical prediction rule 0.732 (0.632-0.832). At the optimal cut-off of 76 this yielded a sensitivity and specificity of 72% and 73% and a positive and negative predictive value of 82% and 60%, respectively. In the validation cohort areas under the curve was 0.818 (0.654-0.995) with sensitivity and specificity of 78% and 73%, respectively. CONCLUSIONS Our new prediction score may be a useful tool in the selection of children with clinically significant varices eligible for a screening endoscopy.
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Pimenta JR, Ferreira AR, Fagundes EDT, Queiroz TCN, Baptista RAN, de Araújo Moreira EG, de Resende CB, Bittencourt PFS, Carvalho SD, Neto JAF, Penna FJ. Factors Associated With Bleeding Secondary to Rupture of Esophageal Varices in Children and Adolescents With Cirrhosis. J Pediatr Gastroenterol Nutr 2017; 64:e44-e48. [PMID: 27496799 DOI: 10.1097/mpg.0000000000001362] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
OBJECTIVE Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. The present study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out. The esophageal and gastric varices were classified, and the portal hypertensive gastropathy, laboratory findings, and Child-Pugh classification were measured. RESULTS Factors observed in univariate analysis to be associated with UGIB were the presence of esophageal varices of medium caliber or larger, portal hypertensive gastropathy, presence of red spots on esophageal varices, Child-Pugh class B or C, and hypoalbuminemia (P < 0.05). After multivariate logistic regression analysis, the significant factors were the presence of red spots on esophageal varices and the presence of gastric varices. When separated the autoimmune hepatitis, nonbiliary atresia patients (all patients except the patients with biliary atresia), and biliary atresia groups the findings in the univariate analysis were the presence of esophageal varices of medium or larger caliber, presence of red spots on varices, and presence of gastric varices in the autoimmune hepatitis patients and nonbiliary atresia patients and presence of red spots on esophageal varices, presence of gastric varices, and Child-Pugh classification B or C in biliary atresia group (P < 0.05). After multivariate logistic regression analysis, no statistical significance was found for any factor analyzed in any groups. CONCLUSIONS The presence of gastric varices and red spots on esophageal varices were related to episodes of UGIB secondary to rupture of esophageal varices. When these findings are observed, indications for endoscopic primary prophylaxis should be evaluated. More studies are, however, necessary to better understand this problem.
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Affiliation(s)
- Júlio R Pimenta
- *Pediatric Gastroenterology Group, Hospital das Clínicas da UFMG †School of Medicine of UFMG ‡Department of Pediatrics, Faculdade de Medicina da UFMG, Belo Horizonte, Brazil
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Duché M, Ducot B, Ackermann O, Guérin F, Jacquemin E, Bernard O. Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding. J Hepatol 2017; 66:320-327. [PMID: 27663417 DOI: 10.1016/j.jhep.2016.09.006] [Citation(s) in RCA: 62] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2016] [Revised: 09/09/2016] [Accepted: 09/12/2016] [Indexed: 12/11/2022]
Abstract
BACKGROUND & AIMS Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. METHODS From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices. Two hundred forty-six children bled spontaneously and 182 underwent primary prophylaxis. The results of primary prophylaxis were reviewed as well as bleed-free survival, overall survival and life-threatening complications of bleeding. RESULTS High-risk varices were found in 96% of children who bled spontaneously and in 11% of children who did not bleed without primary prophylaxis (p<0.001), regardless of the cause of portal hypertension. Life-threatening complications of bleeding were recorded in 19% of children with cirrhosis and high-risk varices who bled spontaneously. Ten-year probabilities of bleed-free survival after primary prophylaxis in children with high-risk varices were 96% and 72% for non-cirrhotic causes and cirrhosis respectively. Ten-year probabilities of overall survival after primary prophylaxis were 100% and 93% in children with non-cirrhotic causes and cirrhosis respectively. CONCLUSION In children with portal hypertension, bleeding is linked to the high-risk endoscopic pattern reported here. Primary prophylaxis of bleeding based on this pattern is fairly effective and safe. LAY SUMMARY In children with liver disease, the risk of bleeding from varices in the esophagus is linked to their large size, the presence of congestion on their surface and their expansion into the stomach but not to the child's age nor to the cause of portal hypertension. Prevention of the first bleed in children with high-risk varices can be achieved by surgery or endoscopic treatment, and decreases mortality and morbidity.
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Affiliation(s)
- Mathieu Duché
- Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France; Radiologie Pédiatrique, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre 94275, France.
| | - Béatrice Ducot
- Santé Publique et Èpidémiologie, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre 94275, France; Inserm, CESP Centre for Research in Epidemiology and Population Health, U1018, Epidemiology of Reproduction and Children Development Team, 94276 Le Kremlin-Bicêtre, France; Université Paris-Sud 11, UMRS 1018, 94276 Le Kremlin-Bicêtre, France
| | - Oanez Ackermann
- Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France
| | - Florent Guérin
- Chirurgie Pédiatrique Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France
| | - Emmanuel Jacquemin
- Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France; Inserm U 1174, Hepatinov, Université Paris-Sud 11, Orsay 91405, France
| | - Olivier Bernard
- Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Hôpital Bicêtre, AP-HP and Université Paris-Sud 11, Le Kremlin-Bicêtre 94275, France
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Sezer OB, Çelik D, Tutar N, Özçay F. Can platelet count/spleen diameter ratio be used for cirrhotic children to predict esophageal varices? World J Hepatol 2016; 8:1466-1470. [PMID: 27957245 PMCID: PMC5124718 DOI: 10.4254/wjh.v8.i33.1466] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/19/2016] [Revised: 09/10/2016] [Accepted: 10/09/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To determine the laboratory and radiologic parameters, including the platelet count (PC)-to-spleen diameter (SD) ratio as a non-invasive marker that may predict the presence of esophageal varices (EV) in children with cirrhosis.
METHODS Eighty-nine patients with cirrhosis, but without a history of variceal bleeding were prospectively included. The children were grouped into 6-12 and 12-18 years of age groups. These groups were also divided into 2 sub-groups (presence and absence of EV). All of the patients underwent a complete biochemical and radiologic evaluation. The PC (n/mm3)-to-SD (mm) ratio was calculated for each patient.
RESULTS Sixty-nine of 98 (70.4%) patients had EV. The presence of ascites in all age groups was significantly associated with the presence of EV. There were no differences in serum albumin levels, PC, SD and the PC-to-SD ratio between the presence and absence of EV groups in both age groups (P > 0.05).
CONCLUSION Laboratory and radiologic parameters, including the PC-to-SD ratio as a non-invasive marker (except for the presence of ascites), was inappropriate for detecting EV in children with cirrhosis.
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Outcome of the Pediatric Patients with Portal Cavernoma: The Retrospective Study for 10 Years Focusing on Recurrent Variceal Bleeding. Gastroenterol Res Pract 2016; 2016:7953870. [PMID: 26949386 PMCID: PMC4754495 DOI: 10.1155/2016/7953870] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2015] [Revised: 12/06/2015] [Accepted: 01/05/2016] [Indexed: 02/07/2023] Open
Abstract
Background. Portal cavernoma (PC) is the most critical condition with risk or variceal hemorrhage in pediatric patients. We retrospectively investigated the patients with PC focusing on the predictors for recurrent variceal bleeding. Methods. Between July 2003 and June 2013, we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of PC without abdominal malignancy or liver cirrhosis. The primary endpoint of this observational study was recurrent variceal bleeding. Independent predictors of recurrent variceal bleeding were identified using the logistic regression model. Results. A total of 157 patients were enrolled in the study. During the follow-up period, 24 patients exhibited onset of recurrent variceal bleeding. Acute variceal bleeding was subjected to conservative symptomatic treatment and emergency endoscopic sclerotherapy. Surgical procedure selection was based on the severity of vascular dilation and collateral circulation. Multivariate logistic regression analysis demonstrated that the presence of ascites, collateral circulation, and portal venous pressure were independent prognostic factors of recurrent variceal bleeding for patients with portal cavernoma. Conclusions. The presence of ascites, collateral circulation, and portal venous pressure evaluation are important and could predict the postsurgical recurrent variceal bleeding in patients with portal cavernoma.
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Lirio RA. Management of Upper Gastrointestinal Bleeding in Children: Variceal and Nonvariceal. Gastrointest Endosc Clin N Am 2016; 26:63-73. [PMID: 26616897 DOI: 10.1016/j.giec.2015.09.003] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Upper gastrointestinal (UGI) bleeding is generally defined as bleeding proximal to the ligament of Treitz, which leads to hematemesis. There are several causes of UGI bleeding necessitating a detailed history to rule out comorbid conditions, medications, and possible exposures. In addition, the severity, timing, duration, and volume of the bleeding are important details to note for management purposes. Despite the source of the bleeding, acid suppression with a proton-pump inhibitor has been shown to be effective in minimizing rebleeding. Endoscopy remains the interventional modality of choice for both nonvariceal and variceal bleeds because it can be diagnostic and therapeutic.
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Affiliation(s)
- Richard A Lirio
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, UMass Memorial Children's Medical Center University Campus, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA 01655, USA.
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Bozic MA, Puri K, Molleston JP. Screening and Prophylaxis for Varices in Children with Liver Disease. Curr Gastroenterol Rep 2015; 17:27. [PMID: 26122248 DOI: 10.1007/s11894-015-0450-4] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/04/2023]
Abstract
Esophageal varices in children with portal hypertension are quite common. Bleeding from these varices frequently occurs. Prophylactic measures to prevent such bleeding can be undertaken either before ("primary," prompted by a screening endoscopy) or after ("secondary") an initial variceal bleed. There are no clear pediatric guidelines for primary or secondary prophylaxis of esophageal varices. Adult studies clearly support the use of pharmacologic (beta blockers) and endoscopic (endoscopic band ligation, EBL) management for both primary and secondary prophylaxis of esophageal varices in patients with portal hypertension. Pediatric studies are limited. There are inadequate data to recommend use of beta blockers to prevent variceal bleeding or rebleeding in children with portal hypertension. There is very limited support for EBL for primary prophylaxis in children and more compelling support for EBL for secondary prophylaxis. Further randomized controlled studies are needed but are difficult to implement in this vulnerable population.
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Affiliation(s)
- Molly A Bozic
- Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Indiana University School of Medicine, 705 Riley Hospital Drive, ROC 4210, Indianapolis, IN, 46202, USA
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Abstract
Portal hypertension is one of the most serious complications of childhood liver disease, and variceal bleeding is the most feared complication. Most portal hypertension results from cirrhosis but extra hepatic portal vein obstruction is the single commonest cause. Upper gastrointestinal endoscopy endoscopy remains necessary to diagnose gastro-esophageal varices. Families of children with portal hypertension should be provided with written instructions in case of gastrointestinal bleeding. Children with large varices should be considered for primary prophylaxis on a case-by-case basis. The preferred method is variceal band ligation. Children with acute bleeding should be admitted to hospital and treated with antibiotics and pharmacotherapy before urgent therapeutic endoscopy. All children who have bled should then receive secondary prophylaxis. The preferred method is variceal band ligation and as yet there is little evidence to support the use of β-blockers. Children with extrahepatic portal vein obstruction should be assessed for suitability of mesoportal bypass.
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Pinto RB, Schneider ACR, da Silveira TR. Cirrhosis in children and adolescents: An overview. World J Hepatol 2015; 7:392-405. [PMID: 25848466 PMCID: PMC4381165 DOI: 10.4254/wjh.v7.i3.392] [Citation(s) in RCA: 61] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2014] [Revised: 12/10/2014] [Accepted: 01/09/2015] [Indexed: 02/06/2023] Open
Abstract
Several conditions, especially chronic liver diseases, can lead to cirrhosis in children and adolescents. Most cases in clinical practice are caused by similar etiologies. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic diseases, while in older children, it tends to result from autoimmune hepatitis, Wilson's disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis. The symptoms of cirrhosis in children and adolescents are similar to those of adults. However, in pediatric patients, the first sign of cirrhosis is often poor weight gain. The complications of pediatric cirrhosis are similar to those observed in adult patients, and include gastrointestinal bleeding caused by gastroesophageal varices, ascites and spontaneous bacterial peritonitis. In pediatric patients, special attention should be paid to the nutritional alterations caused by cirrhosis, since children and adolescents have higher nutritional requirements for growth and development. Children and adolescents with chronic cholestasis are at risk for several nutritional deficiencies. Malnutrition can have severe consequences for both pre- and post-liver transplant patients. The treatment of cirrhosis-induced portal hypertension in children and adolescents is mostly based on methods developed for adults. The present article will review the diagnostic and differential diagnostic aspects of end-stage liver disease in children, as well as the major treatment options for this condition.
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Affiliation(s)
- Raquel Borges Pinto
- Raquel Borges Pinto, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Porto Alegre 91340 480, Rio Grande do Sul, Brazil
| | - Ana Claudia Reis Schneider
- Raquel Borges Pinto, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Porto Alegre 91340 480, Rio Grande do Sul, Brazil
| | - Themis Reverbel da Silveira
- Raquel Borges Pinto, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Porto Alegre 91340 480, Rio Grande do Sul, Brazil
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Spleen stiffness measurement by transient elastography to diagnose portal hypertension in children. J Pediatr Gastroenterol Nutr 2014; 59:197-203. [PMID: 24732027 DOI: 10.1097/mpg.0000000000000400] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
BACKGROUND The development of esophageal varices is a late complication of chronic liver disease (LD) in children. The diagnosis is presently limited to invasive procedures such as endoscopy. Noninvasive tools to diagnose the presence and degree of esophageal varices would alter management decisions and support indications for invasive procedures in affected children. The aim of the study was to test the feasibility of spleen stiffness measurement (SSM) by transient elastography (TE; FibroScan) in children and compare data on its diagnostic use with established markers of liver fibrosis and parameters of portal hypertension. METHODS A total of 99 children (62 with chronic LD, 6 after liver transplantation, 31 controls) underwent SSM by TE. Fibrotest was determined in 37 children, 45 children had an additional liver stiffness measurement, and 19 underwent upper endoscopy. RESULTS SSM by FibroScan is feasible. Spleen size significantly determined success rate (90.5% in patients with splenomegaly vs 70.2% in patients without, P = 0.02). Spleen stiffness was significantly higher in patients with splenomegaly (62.96 vs 18.4 kPa, P < 0.001), in patients with varices (75 vs 24 kPa, P < 0.01), and in patients with a history of variceal hemorrhage (75 vs 50.25 kPa, P < 0.05). Variceal hemorrhage did not occur in patients with SSM results <60 kPa. Spleen stiffness decreased after liver transplantation, but remained elevated compared with controls (27.5 vs 16.3 kPa). Liver stiffness measurements and Fibrotest did not reflect the presence or degree of esophageal varices. CONCLUSIONS SSM by TE is feasible in children and the results reflect the degree and occurrence of complications. A prospective follow-up study with larger patient numbers and performance of screening endoscopies appears justified and desirable.
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Abstract
Non-cirrhotic portal hypertension (NCPH) encompasses a wide range of disorders, primarily vascular in origin, presenting with portal hypertension (PHT), but with preserved liver synthetic functions and near normal hepatic venous pressure gradient (HVPG). Non-cirrhotic portal fibrosis/Idiopathic PHT (NCPF/IPH) and extrahepatic portal venous obstruction (EHPVO) are two prototype disorders in the category. Etiopathogenesis in both of them centers on infections and prothrombotic states. Presentation and management strategies focus on repeated well tolerated episodes of variceal bleed and moderate to massive splenomegaly and other features of PHT. While the long-term prognosis is generally good in NCPF, portal biliopathy and parenchymal extinction after prolonged PHT makes outcome somewhat less favorable in EHPVO. While hepatic schistosomiasis, congenital hepatic fibrosis and nodular regenerative hyperplasia have their distinctive features, they often present with NCPH.
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Affiliation(s)
- Shiv K Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India.
| | - Rajeev Khanna
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India
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[Hepatic involvement in hereditary alpha-1-antitrypsin deficiency]. Rev Mal Respir 2014; 31:357-64. [PMID: 24750955 DOI: 10.1016/j.rmr.2013.10.651] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2011] [Accepted: 10/21/2013] [Indexed: 01/18/2023]
Abstract
Apha-1-antitrypsin deficiency is an autosomal recessive genetic disorder seen in all races. The molecular defect is a specific mutation of the SERPINA1 gene leading to synthesis of an abnormal protein (alpha-1-antitrypsin Z) that cannot be secreted and polymerizes in the endoplasmic reticulum of hepatocytes. The inter-individual variability in the responses to intracellular stress induced by the accumulation of abnormal polymers and the mechanisms allowing their degradation is, without doubt, responsible for the different clinical manifestations of the disease. The disease affects the liver where the abnormal protein is synthesized and the lung, which is its place of action. Liver involvement is well recognized in homozygous infants of the phenotype ZZ. In this situation the disease may present a varying picture from neonatal cholestasis (about 15% of neonatal defects) to cirrhosis. However, evolution towards cirrhosis affects less than 3% of infants with the ZZ phenotype and it is preceded in 80% of cases by neonatal cholestasis. In adolescents or adults the manifestations associated with alpha-1-antitrypsin deficiency are usually limited to biochemical abnormalities but may lead to cirrhosis or hepatocellular carcinoma. The hepatic disorder and its complications are treated symptomatically though the pulmonary involvement may benefit from substitution treatment. More specific treatments targeting the molecular and cellular abnormalities are the subject of research.
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Khanna R, Sarin SK. Non-cirrhotic portal hypertension - diagnosis and management. J Hepatol 2014; 60:421-41. [PMID: 23978714 DOI: 10.1016/j.jhep.2013.08.013] [Citation(s) in RCA: 259] [Impact Index Per Article: 23.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/20/2013] [Revised: 08/07/2013] [Accepted: 08/19/2013] [Indexed: 02/06/2023]
Abstract
NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.
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Affiliation(s)
- Rajeev Khanna
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Shiv K Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
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Abstract
OBJECTIVE The aim of this study was to identify ultrasonographic predictors of esophageal varices (EVs) in children and adolescents with chronic liver disease (CLD) and extrahepatic portal venous obstruction (EHPVO). METHODS This study evaluates 53 patients younger than 20 years with CLD or EHPVO and no history of bleeding or prophylactic EVs treatment. They were divided into 2 groups: group I (35 with CLD) and group II (18 with EHPVO). Splenorenal shunt (SS), gallbladder wall varices, gallbladder wall thickening (GT), and lesser omental thickness (LOT) were compared with the presence of EVs, gastric varices, and portal hypertensive gastropathy (PHG). Univariate (χ² test, Fisher exact test, and Wilcoxon signed rank test) and multivariate (logistic regression) analyses were performed. The area under the receiver operating curve was calculated. RESULTS EVs were observed in 48.5% of patients with CLD and in 83.3% of patients with EHPVO. SS (P = 0.0329) and LOT (P = 0.0151) predicted EV among patients with CLD. A median of 5.3 mm of LOT was considered a predictor of EVs among these patients. Multivariate analysis showed SS as an independent predictor of EVs in patients with EHPVO (odds ratio 15). Gallbladder varices (P = 0.0245) and GT (P = 0.0289) predicted EVs among patients with EHPVO. PHG occurred more often among patients with CLD who had SS (P = 0.0384) and greater LOT (P = 0.0226). CONCLUSIONS SS and a greater LOT were indicative of EV among children and adolescents with CLD. Gallbladder varices and GT were indicative of EVs among patients with EHPVO. SS and a greater LOT were indicative of PHG among patients with CLD.
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Affiliation(s)
- Jean Pappas Molleston
- Department of Pediatrics, Pediatric Gastroenterology, Hepatology and Nutrition, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, Indiana.
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Flores-Calderón J. [Pediatric hepatology]. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO 2013; 78 Suppl 1:117-9. [PMID: 24041081 DOI: 10.1016/j.rgmx.2013.06.042] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/06/2013] [Accepted: 06/28/2013] [Indexed: 11/17/2022]
Affiliation(s)
- J Flores-Calderón
- UMAE Hospital de Pediatría, Departamento de Gastroenterología, Centro Médico Nacional SXXI. Instituto Mexicano del Seguro Social.
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Adami MR, Ferreira CT, Kieling CO, Hirakata V, Vieira SMG. Noninvasive methods for prediction of esophageal varices in pediatric patients with portal hypertension. World J Gastroenterol 2013; 19:2053-2059. [PMID: 23599624 PMCID: PMC3623982 DOI: 10.3748/wjg.v19.i13.2053] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2012] [Revised: 12/19/2012] [Accepted: 01/12/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate clinical and laboratory parameters for prediction of bleeding from esophageal varices (EV) in children with portal hypertension.
METHODS: Retrospective study of 103 children (mean age: 10.1 ± 7.7 years), 95.1% with intrahepatic portal hypertension. All patients had no history of bleeding and underwent esophagogastroduodenoscopy for EV screening. We recorded variceal size (F1, F2 and F3), red-color signs and portal gastropathy, according to the Japanese Research Society for Portal Hypertension classification. Patients were classified into two groups: with and without EV. Seven noninvasive markers were evaluated as potential predictors of EV: (1) platelet count; (2) spleen size z score, expressed as a standard deviation score relative to normal values for age; (3) platelet count to spleen size z score ratio; (4) platelets count to spleen size (cm) ratio; (5) the clinical prediction rule (CPR); (6) the aspartate aminotransferase to platelet ratio index (APRI); and (7) the risk score.
RESULTS: Seventy-one children had EV on first endoscopy. On univariate analysis, spleen size, platelets, CPR, risk score, APRI, and platelet count to spleen size z score ratio showed significant associations. The best noninvasive predictors of EV were platelet count [area under the receiver operating characteristic curve (AUROC) 0.82; 95%CI: 0.73-0.91], platelet: spleen size z score (AUROC 0.78; 95%CI: 0.67-0.88), CPR (AUROC 0.77; 95%CI: 0.64-0.89), and risk score (AUROC 0.77; 95%CI: 0.66-0.88). A logistic regression model was applied with EV as the dependent variable and corrected by albumin, bilirubin and spleen size z score. Children with a CPR < 114 were 20.7-fold more likely to have EV compared to children with CPR > 114. A risk score > -1.2 increased the likelihood of EV (odds ratio 7.47; 95%CI: 2.06-26.99).
CONCLUSION: Children with portal hypertension with a CPR below 114 and a risk score greater than -1.2 are more likely to have present EV. Therefore, these two tests can be helpful in selecting children for endoscopy.
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Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction (EHPVO) in children. Ann Hepatol 2013; 12 Suppl 1:S3-S24. [PMID: 31207845 DOI: 10.1016/s1665-2681(19)31403-6] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2012] [Accepted: 10/15/2012] [Indexed: 02/04/2023]
Abstract
INTRODUCTION Extrahepatic portal vein obstruction is an important cause of portal hypertension among children. The etiology is heterogeneous and there are few evidences related to the optimal treatment. AIM AND METHODS To establish guidelines for the diagnosis and treatment of EHPVO in children, a group of gastroenterologists and pediatric surgery experts reviewed and analyzed data reported in the literature and issued evidence-based recommendations. RESULTS Pediatric EHPVO is idiopathic in most of the cases. Digestive hemorrhage and/or hypersplenism are the main symptoms. Doppler ultrasound is a non-invasive technique with a high degree of accuracy for the diagnosis. Morbidity is related to variceal bleeding, recurrent thrombosis, portal biliopathy and hypersplenism. Endoscopic therapy is effective in controlling acute variceal hemorrhage and it seems that vasoactive drug therapy can be helpful. For primary prophylaxis of variceal bleeding, there are insufficient data for the use of beta blockers or endoscopic therapy. For secondary prophylaxis, sclerotherapy or variceal band ligation is effective; there is scare evidence to recommend beta-blockers. Surgery shunt is indicated in children with variceal bleeding who fail endoscopic therapy and for symptomatic hypersplenism; spleno-renal or meso-ilio-cava shunting is the alternative when Mesorex bypass is not feasible due to anatomic problems or in centers with no experience. CONCLUSIONS Prospective control studies are required for a better knowledge of the natural history of EHPVO, etiology identification including prothrombotic states, efficacy of beta-blockers and comparison with endoscopic therapy on primary and secondary prophylaxis.
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Shneider BL, Abel B, Haber B, Karpen SJ, Magee JC, Romero R, Schwarz K, Bass LM, Kerkar N, Miethke AG, Rosenthal P, Turmelle Y, Robuck PR, Sokol RJ, for the Childhood Liver Disease Research and Education Network (ChiLDREN). Portal hypertension in children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr 2012; 55:567-73. [PMID: 22903006 PMCID: PMC3483444 DOI: 10.1097/mpg.0b013e31826eb0cf] [Citation(s) in RCA: 75] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
OBJECTIVE Biliary atresia (BA) frequently results in portal hypertension (PHT), complications of which lead to significant morbidity and mortality. The Childhood Liver Disease Research and Education Network was used to perform a cross-sectional multicentered analysis of PHT in children with BA. METHODS Subjects with BA receiving medical management at a Childhood Liver Disease Research and Education Network site were enrolled. A priori, clinically evident PHT was defined as "definite" when there was either history of a complication of PHT or clinical findings consistent with PHT (both splenomegaly and thrombocytopenia). PHT was denoted as "possible" if one of the findings was present in the absence of a complication, whereas PHT was "absent" if none of the criteria were met. RESULTS A total of 163 subjects were enrolled between May 2006 and December 2009. At baseline, definite PHT was present in 49%, possible in 17%, and absent in 34% of subjects. Demographics, growth, and anthropometrics were similar amongst the 3 PHT categories. Alanine aminotransferase, γ-glutamyl transpeptidase, and sodium levels were similar, whereas there were significant differences in aspartate aminotransferase (AST), AST/alanine aminotransferase, albumin, total bilirubin, prothrombin time, white blood cell count, platelet count, and AST/platelet count between definite and absent PHT. Thirty-four percent of those with definite PHT had either prothrombin time >15 seconds or albumin <3 g/dL. CONCLUSIONS Clinically definable PHT is present in two-thirds of North American long-term BA survivors with their native livers. The presence of PHT is associated with measures of hepatic injury and dysfunction, although in this selected cohort, the degree of hepatic dysfunction is relatively mild and growth is preserved.
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Affiliation(s)
| | - Bob Abel
- University of Michigan, Ann Arbor, MI, USA
| | - Barbara Haber
- Children’s Hospital of Philadelphia, Philadelphia, PA, USA
| | | | | | | | | | - Lee M. Bass
- Children’s Memorial Hospital, Chicago, IL, USA
| | - Nanda Kerkar
- Mount Sinai School of Medicine, New York, NY, USA
| | | | | | | | - Patricia R. Robuck
- National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
| | - Ronald J. Sokol
- University of Colorado and Children’s Hospital Colorado, Aurora, CO, USA
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Gana JC, Turner D, Mieli-Vergani G, Davenport M, Miloh T, Avitzur Y, Yap J, Morinville V, Brill H, Ling SC. A clinical prediction rule and platelet count predict esophageal varices in children. Gastroenterology 2011; 141:2009-16. [PMID: 21925123 DOI: 10.1053/j.gastro.2011.08.049] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/12/2011] [Revised: 08/25/2011] [Accepted: 08/29/2011] [Indexed: 12/13/2022]
Abstract
BACKGROUND & AIMS The validation of noninvasive tests to diagnose esophageal varices is a priority in children because repeated endoscopic evaluations are too invasive. We measured the ability of a previously developed noninvasive clinical prediction rule (CPR) to predict the presence of esophageal varices in children. METHODS We analyzed data from 108 children, younger than age 18, who received endoscopies at 8 centers, to assess portal hypertension from chronic liver disease or portal vein obstruction. Blood test and abdominal ultrasound scan results were obtained within 4 months of endoscopy. Grading of varices identified by endoscopy was confirmed by independent blinded review. Spleen size, based on data from the ultrasound scan, was expressed as a standard deviation score relative to normal values for age. RESULTS Of the children studied, 74 had esophageal varices (69%), including 35 with large varices (32%). The best noninvasive predictors of esophageal varices of any size were as follows: platelet:spleen size z-score ratio (area under the receiver operating characteristic curve [AUROC], 0.84; 95% confidence interval [CI] 0.75-0.93), CPR (AUROC, 0.80; 95% CI, 0.70-0.91), and platelet count (AUROC, 0.79; 95% CI, 0.69-0.90). The positive predictive values for the CPR and platelet count were 0.87 and 0.86, the negative predictive values were 0.64 and 0.63, the positive likelihood ratios were 3.06 and 2.76, and the negative likelihood ratios were 0.64 and 0.63, respectively. Based on positive and negative predictive values, the most accurate noninvasive tests were the CPR and platelet counts. CONCLUSIONS Noninvasive tests such as CPR and platelet count can assist in triaging children for endoscopy to identify esophageal varices.
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Affiliation(s)
- Juan Cristóbal Gana
- Division of Pediatrics, Gastroenterology, Hepatology and Nutrition Unit, Pontificia Universidad Católica de Chile, Santiago, Chile.
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Zhang MM, Pu CL, Li YC, Guo CB. Sixty-four-slice computed tomography in surgical strategy of portal vein cavernous transformation. World J Gastroenterol 2011; 17:4334-8. [PMID: 22090790 PMCID: PMC3214709 DOI: 10.3748/wjg.v17.i38.4334] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2010] [Revised: 11/23/2010] [Accepted: 11/30/2010] [Indexed: 02/06/2023] Open
Abstract
AIM: To investigate the role of 64-slice computed tomography (CT) in portal vein cavernous transformation to determine surgical strategy.
METHODS: The site of lesions and extent of collateral circulation in 12 pediatric cases of cavernous transformation of the portal vein with surgical treatment were analyzed.
RESULTS: Eleven of 12 children had esophageal varices and were treated with lower esophageal and gastric devascularization and splenectomy, and the other case was only treated with splenectomy. There were eight cases with spontaneous spleen/stomach-renal shunt, four with Retzius vein opening, which was reserved during surgery. Three cases of lesions involving the intrahepatic portal vein (PV) were treated with living donor liver transplantation. One patient died from PV thrombosis after liver transplantation, and the rest had no significant complications.
CONCLUSION: The PV, its branches and collateral circulation were clearly seen by 64-slice spiral CT angiography, which helped with preoperative surgical planning.
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Colecchia A, Di Biase AR, Scaioli E, Predieri B, Iughetti L, Reggiani MLB, Montrone L, Ceccarelli PL, Vestito A, Viola L, Paolucci P, Festi D. Non-invasive methods can predict oesophageal varices in patients with biliary atresia after a Kasai procedure. Dig Liver Dis 2011; 43:659-63. [PMID: 21596631 DOI: 10.1016/j.dld.2011.04.006] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2010] [Revised: 01/28/2011] [Accepted: 04/10/2011] [Indexed: 02/07/2023]
Abstract
BACKGROUND After a Kasai procedure, 70% of patients with biliary atresia develop chronic liver disease with portal hypertension and oesophageal varices. AIMS To investigate the role of new non-invasive parameters in predicting the presence of varices in patients with biliary atresia after a Kasai procedure and to identify the cut-off values of these parameters in predicting the presence of varices. METHODS 31 patients with biliary atresia who had undergone a Kasai portoenterostomy were studied. Clinical, biochemical and abdominal ultrasound examination, liver stiffness measurement (LSM), LSM-spleen diameter to platelet ratio score (LSPS) and upper digestive endoscopy were performed. RESULTS 15 (47%) patients had oesophageal varices (Group A) and 16 had no varices (Group B). Median values of LSM (kPa) and LSPS were significantly higher in Group A than in Group B (LSM: 17.0 vs. 7.5, respectively; p=0.0001; LSPS: 19.62 vs. 2.94, respectively; p=0.0001). The optimal cut-offs for predicting oesophageal varices were: LSM>10.6 kPa (sensitivity: 87%, specificity: 87.5%, PPV: 87%, NPV: 87.5%, and AUC: 0.92) and LSPS ≥9.2 (sensitivity: 91%, specificity: 92%, PPV: 91%, NPV: 92%, and AUC: 0.96). CONCLUSIONS Non-invasive methods can predict the presence of oesophageal varices in patients with biliary atresia; the sequential use of two non-invasive methods improves accuracy.
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Chongsrisawat V, Vejapipat P, Siripon N, Poovorawan Y. Transient elastography for predicting esophageal/gastric varices in children with biliary atresia. BMC Gastroenterol 2011; 11:41. [PMID: 21501480 PMCID: PMC3089784 DOI: 10.1186/1471-230x-11-41] [Citation(s) in RCA: 61] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2011] [Accepted: 04/18/2011] [Indexed: 12/30/2022] Open
Abstract
BACKGROUND Transient elastography (TE) is an innovative, noninvasive technique to assess liver fibrosis by measuring liver stiffness in patients with chronic liver diseases. The purpose of this study has been to explore the accuracy of TE and clinical parameters in predicting the presence of esophageal/gastric varices in children with biliary atresia (BA) following portoenterostomy. METHODS Patients with BA status post portoenterostomy and normal children were recruited. Splenomegaly and presence of EV/GV were determined by physical examination and endoscopy, respectively. Aspartate transaminase to platelet ratio index (APRI) was used as a serum fibrosis marker. TE was performed by using FibroScan. Data was expressed as mean ± SD. RESULTS Seventy-three BA patients (male:female = 32:41; age 9.11 ± 5.64 years) and 50 normal controls (male:female = 19:31; age 11.00 ± 3.31 years) were enrolled. The liver stiffness score of BA patients was significantly higher than that of normal controls (27.37 ± 22.48 and 4.69 ± 1.03 kPa; p < 0.001). Patients with EV/GV had significantly higher liver stiffness score and APRI than those without EV/GV. As for EV/GV diagnosis, the areas under the receiver operating characteristic curve were 0.89 (95% CI 0.80 to 0.98) for TE and 0.87 (95% CI 0.78 to 0.96) for APRI, respectively. The sensitivity (and specificity) of TE (using a cut-off value of 12.7 kPa) and APRI (using a cut-off value of 1.92) in predicting EV/GV were 84% (77%) and 84% (83%), respectively, whereas the sensitivity (and specificity) of splenomegaly in predicting EV/GV were 92% (85%). CONCLUSIONS Transient elastography is a useful tool for predicting the presence of EV/GV. In addition, basic physical examination, routine biochemical and hematological tests, are still worthwhile and correlate well with the presence of EV/GV in patients with BA post portoenterostomy.
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Affiliation(s)
- Voranush Chongsrisawat
- Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Paisarn Vejapipat
- Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Nipaporn Siripon
- Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Yong Poovorawan
- Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
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Primary prophylaxis of variceal hemorrhage in children with portal hypertension: a framework for future research. J Pediatr Gastroenterol Nutr 2011; 52:254-61. [PMID: 21336158 PMCID: PMC3728696 DOI: 10.1097/mpg.0b013e318205993a] [Citation(s) in RCA: 73] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Nonselective β-blocker therapy and endoscopic variceal ligation reduce the incidence of variceal hemorrhage in cirrhotic adults, but their use in children is controversial. There are no evidence-based recommendations for the prophylactic management of children at risk of variceal hemorrhage due to the lack of appropriate randomized controlled trials. In a recent gathering of experts at the American Association for the Study of Liver Diseases annual meeting, significant challenges were identified in attempting to design and implement a clinical trial of primary prophylaxis in children using either of these therapies. These challenges render such a trial unfeasible, primarily due to the large sample size required, inadequate knowledge of appropriate dosing of β-blockers, and difficulty in recruiting to a trial of endoscopic variceal ligation. Pediatric research should focus on addressing questions of natural history and diagnosis of varices, prediction of variceal bleeding, optimal approaches to β-blocker and ligation therapy, and alternative study designs to explore therapeutic efficacy in children.
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Hong WD, Dong LM, Jiang ZC, Zhu QH, Jin SQ. Prediction of large esophageal varices in cirrhotic patients using classification and regression tree analysis. Clinics (Sao Paulo) 2011; 66:119-24. [PMID: 21437447 PMCID: PMC3044565 DOI: 10.1590/s1807-59322011000100021] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2010] [Accepted: 10/24/2010] [Indexed: 12/13/2022] Open
Abstract
OBJECTIVES Recent guidelines recommend that all cirrhotic patients should undergo endoscopic screening for esophageal varices. That identifying cirrhotic patients with esophageal varices by noninvasive predictors would allow for the restriction of the performance of endoscopy to patients with a high risk of having varices. This study aimed to develop a decision model based on classification and regression tree analysis for the prediction of large esophageal varices in cirrhotic patients. METHODS 309 cirrhotic patients (training sample, 187 patients; test sample 122 patients) were included. Within the training sample, the classification and regression tree analysis was used to identify predictors and prediction model of large esophageal varices. The prediction model was then further evaluated in the test sample and different Child-Pugh classes. RESULTS The prevalence of large esophageal varices in cirrhotic patients was 50.8%. A tree model that was consisted of spleen width, portal vein diameter and prothrombin time was developed by classification and regression tree analysis achieved a diagnostic accuracy of 84% for prediction of large esophageal varices. When reconstructed into two groups, the rate of varices was 83.2% for high-risk group and 15.2% for low-risk group. Accuracy of the tree model was maintained in the test sample and different Child-Pugh classes. CONCLUSIONS A decision tree model that consists of spleen width, portal vein diameter and prothrombin time may be useful for prediction of large esophageal varices in cirrhotic patients.
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Affiliation(s)
- Wan-dong Hong
- Department of Gastroenterology and Hepatology, Wenzhou Medical College, Wenzhou, China
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Derivation of a clinical prediction rule for the noninvasive diagnosis of varices in children. J Pediatr Gastroenterol Nutr 2010; 50:188-93. [PMID: 19966576 DOI: 10.1097/mpg.0b013e3181b64437] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND OBJECTIVES Identification of children who are at high risk for having varices using noninvasive tests would enable the selection of children for future studies of primary prophylaxis of variceal hemorrhage, but this has been inadequately studied. The objective of the study was to derive a noninvasive clinical prediction rule that is able to identify children with esophageal varices. METHODS Fifty-one consecutive children with liver disease or portal hypertension who underwent endoscopy were included in the present retrospective study. At endoscopy, variceal size was graded on a 4-point Likert scale. Results of physical examination, blood tests, and abdominal ultrasound scan (USS) were recorded. Spleen length on USS was expressed as a standard deviation score (z score). A descriptive univariate analysis was performed on variables that were potentially associated with esophageal varices and multivariate logistic regression was then modeled to derive a clinical prediction rule. RESULTS Esophageal varices were found in 17 of the 51 children (33%). Variables found to differ significantly between children with and without varices included platelet/spleen-length z score ratio (P < 0.001), platelet count (P < 0.001), international normalized ratio (P = 0.001), aspartate aminotransferase/alanine aminotransferase ratio (P = 0.002), and albumin (P = 0.003). Using multivariate logistic regression, a model with platelet count, spleen length z score, and albumin as the independent variables had the best fit. Area under the receiver operating characteristic curve for this clinical prediction rule was 0.93 (95% confidence interval 0.85-0.99), sensitivity 94%, specificity 81%, positive predictive value 0.83, negative predictive value 0.94, positive likelihood ratio 5, and negative likelihood ratio 0.06. CONCLUSIONS This clinical prediction rule is a simple noninvasive measure that may identify children at high risk for esophageal varices. A prospective validation study is in progress.
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Mattioli G, Avanzini S, Pini Prato A, Asquasciati C, Rapuzzi G, Costanzo S, Basile A, Rossi V, Bisio G, Boeri E, Montobbio G, Jasonni V. Spleen surgery in pediatric age: seven-year unicentric experience. J Laparoendosc Adv Surg Tech A 2009; 19:437-41. [PMID: 19245317 DOI: 10.1089/lap.2008.0289] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
BACKGROUND Either "open" and laparoscopic spleen surgery in pediatric age are well known and performed with ease in children. Yet, few data regarding follow-up and outcome are discussed in the international literature. MATERIALS AND METHODS Clinical notes of all patient who underwent spleen surgery in a single center between 2000 and 2007 were reviewed and a specific follow-up questionnaire was administered, aiming to evaluate pre- and postoperative data, especially considering underlying disease, cosmetic results, and quality of life after surgery. RESULTS Fifty-one patients underwent spleen surgery in our series, 33 of whom returned a complete follow-up questionnaire and were included in the study. Splenectomy was performed in 26 patients, whereas 7 patients underwent a partial splenectomy; 19 cases (57.6%) were approached laparoscopically. A total of 4 complications (12%) occurred in our series, none of them being intraoperative. Hospital stay resulted as significantly shorter in laparoscopic cases (5.5 +/- 2.9 vs. 8.7 +/- 4.8 days; P < 0.01), with better results in terms of cosmetics. Quality of life is strictly related to underlying disease, as well as long-term survival. CONCLUSIONS Whatever surgical approach is adopted, spleen surgery is safe, effective, and reproducible. When feasible, the laparoscopic approach should be preferred to the traditional open approach, as far as efficacy and safety are similar, in order to reduce hospital stay, abdominal wall traumatism, and consequently, improve postoperative pain control and cosmetic results.
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Affiliation(s)
- Girolamo Mattioli
- Department of Pediatric Surgery, G. Gaslini Children's University Institute, Genoa, Italy., University of Genoa, Genoa, Italy.
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