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Kwape L, Gabriel S, Abdelsalem A, Rose P, Bathobakae L, Peterson D, Moodley D, Parker M, Moolla S, Parker A, Siamisang K, Van Rensburg C, Fredericks E. Evaluation of Noninvasive Tools for Predicting Esophageal Varices in Patients With Cirrhosis at Tygerberg Hospital, Cape Town. Int J Hepatol 2024; 2024:9952610. [PMID: 39296589 PMCID: PMC11410406 DOI: 10.1155/2024/9952610] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2023] [Revised: 07/23/2024] [Accepted: 08/06/2024] [Indexed: 09/21/2024] Open
Abstract
Background: In patients with cirrhosis, esophageal variceal hemorrhage (EVH) is a devastating consequence of portal hypertension (PH). Upper endoscopy is considered the gold standard for the detection and diagnosis of esophageal varices (EVs), despite being invasive and costly. This study was aimed at identifying and evaluating the diagnostic accuracy of noninvasive tools in predicting EVs in patients with compensated cirrhosis. Methods: This cross-sectional study included 50 patients with compensated cirrhosis at the Tygerberg Hospital Gastroenterology Clinic in Cape Town between November 2022 and May 2023. We collected clinical, anthropometric, and laboratory data from patients' physical and electronic charts. All patients underwent an abdominal ultrasound, vibration-controlled transient elastography (VCTE) to assess liver and splenic stiffness, and upper endoscopy. In this comparative study, we evaluated the diagnostic accuracy of different noninvasive tools in detecting EVs in patients with compensated cirrhosis. Results: Of the 50 patients included in the study, 30 (60%) were female and 20 (40%) were male. The patients' age ranged from 18 to 83, with a mean age of 46.6 years. Cirrhosis was mainly due to alcohol use (n = 11, 22%), hepatitis B virus (HBV) infection (n = 11, 22%), and autoimmune hepatitis (n = 10, 20%). The patients included in the study were divided into two subgroups: with (n = 34, 68%) or without (n = 16, 32%) EVs. Statistically significant differences were detected between groups in platelet count (PC), liver stiffness measurement (LSM), spleen stiffness measurement (SSM), portal vein diameter (PVD), bipolar spleen diameter (SBD), aspartate aminotransferase-to-platelet ratio index (APRI), fibrosis-4 index (FIB-4), platelet/bipolar spleen diameter ratio (PSR), liver stiffness-spleen size-platelet ratio (LSPS), liver stiffness-spleen stiffness-platelet ratio score (LS3PS), and spleen stiffness-spleen size-platelet ratio score (SSPS) (p < 0.001). The highest diagnostic precision was observed with SSM (96%), SSPS (96%), LS3PS (94%), LSPS (94%), PSR (94%), and PC (92%). SBD (88%), LSM (86%), APRI (82%), and FIB-4 (82%) had the lowest diagnostic accuracy. Conclusion: SSM and SSPS have the highest diagnostic accuracy for predicting the presence of EVs in patients with compensated cirrhosis. LSPS, LS3PS, and PSR come second at 94%. We recommend SSM and SSPS in institutions with transient elastography equipped with the software necessary to measure splenic stiffness. We introduce and propose LS3PS as a novel composite score for predicting the presence of EVs in patients with compensated cirrhosis. Large-sample-size studies are needed to validate these prediction scores and to allow direct comparison with Baveno VII. These prediction tools can help clinicians avoid unnecessary endoscopic procedures in patients with compensated cirrhosis, especially in developing countries with limited resources such as South Africa.
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Affiliation(s)
- Lawrence Kwape
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Shiraaz Gabriel
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Ahmad Abdelsalem
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Penelope Rose
- Department of Paediatrics and Child Health Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Lefika Bathobakae
- Internal Medicine St. Joseph's University Medical Center, Paterson, New Jersey, USA
| | - Dale Peterson
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Desiree Moodley
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Mohammed Parker
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Saadiq Moolla
- Division of Pulmonology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Arifa Parker
- Unit for Infection Prevention and Control Department of Medicine Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
- Division of Infectious Diseases Department of Medicine Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Keatlaretse Siamisang
- Department of Family Medicine and Public Health University of Botswana, Gaborone, Botswana
| | - Christoffel Van Rensburg
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
| | - Ernst Fredericks
- Division of Gastroenterology and Hepatology Department of Medicine Faculty of Medicine and Health Sciences Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa
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Longitudinal analysis of transplant candidates with primary sclerosing cholangitis in an Asian liver transplant center. Eur J Gastroenterol Hepatol 2023; 35:480-487. [PMID: 36719819 DOI: 10.1097/meg.0000000000002516] [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: 02/01/2023]
Abstract
BACKGROUND Primary sclerosing cholangitis (PSC) is a rare disease in Asia, and few studies have investigated the disease in this ethnicity, particularly in wait-listed patients for liver transplantation (LT). We aimed to investigate the prognostic factors and outcomes of wait-listed patients with PSC in an Asian transplant center. METHODS Survival was retrospectively analyzed. RESULTS Eighteen (10 male and 8 female) wait-listed patients with PSC, with a median age at diagnosis of 44.5 years, were included. Compared with men, women had significantly higher aspartate aminotransferase to platelet ratio index scores (3.28 vs. 1.13; P = 0.012) and bilirubin levels (7.68 vs. 4.03 mg/dl; P = 0.043) and more often presented with decompensating events, including ascites [5 (63%) vs. 1 (10%); P = 0.043] and splenomegaly [8 (100%) vs. 4 (40%); P = 0.013]. Compared with the non-LT group, the LT group exhibited a superior survival rate for women ( P = 0.004) but not for men. In the univariable analysis, significant risk factors associated with overall survival included malignancies with a hazard ratio (95% confidence interval) of 5.53 (1.00-30.51) and esophageal varices (EV) [4.18 (1.05-16.61)], whereas female gender [25.00 (1.49-500.00)], LT [0.09 (0.01-0.80)] and EV [39.03 (2.92-521.96)] were indicated in the multivariable analysis. CONCLUSIONS For Asian wait-listed patients with PSC, EV and female gender were the risk factors related to overall survival, and LT was the protective factor. Our experiences suggested that LT brings more benefits in female patients. Strategies are needed to provide equivalent transplant benefits.
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Chazouilleres O, Beuers U, Bergquist A, Karlsen TH, Levy C, Samyn M, Schramm C, Trauner M. EASL Clinical Practice Guidelines on sclerosing cholangitis. J Hepatol 2022; 77:761-806. [PMID: 35738507 DOI: 10.1016/j.jhep.2022.05.011] [Citation(s) in RCA: 152] [Impact Index Per Article: 50.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2022] [Accepted: 05/16/2022] [Indexed: 02/07/2023]
Abstract
Management of primary or secondary sclerosing cholangitis is challenging. These Clinical Practice Guidelines have been developed to provide practical guidance on debated topics including diagnostic methods, prognostic assessment, early detection of complications, optimal care pathways and therapeutic (pharmacological, endoscopic or surgical) options both in adults and children.
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Isoura Y, Yamamoto A, Cho Y, Ehara E, Jogo A, Suzuki T, Amano-Teranishi Y, Kioka K, Hamazaki T, Murakami Y, Tokuhara D. Platelet count and abdominal dynamic CT are useful in predicting and screening for gastroesophageal varices after Fontan surgery. PLoS One 2021; 16:e0257441. [PMID: 34618830 PMCID: PMC8496823 DOI: 10.1371/journal.pone.0257441] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2021] [Accepted: 09/02/2021] [Indexed: 12/12/2022] Open
Abstract
OBJECTIVE Patients who undergo Fontan surgery for complex cardiac anomalies are prone to developing liver and gastrointestinal complications. In particular, gastroesophageal varices (GEVs) can occur, but their prevalence is unknown. We aimed to elucidate the occurrence of GEVs and the predicting parameters of GEVs in these patients. MATERIALS AND METHODS Twenty-seven patients (median age, 14.8 years; median time since surgery, 12.9 years) who had undergone the Fontan surgery and were examined by abdominal dynamic computed tomography (CT) for the routine follow-up were included in the study. Radiological findings including GEVs and extraintestinal complications were retrospectively evaluated by experienced radiologists in a blinded manner. Relationships between blood-biochemical and demographic parameters and the presence of GEVs were statistically analyzed. RESULTS Dynamic CT revealed gastric varices (n = 3, 11.1%), esophageal varices (n = 1, 3.7%), and gastrorenal shunts (n = 5, 18.5%). All patients with gastric varices had gastrorenal shunts. All gastric varices were endoscopically confirmed as being isolated and enlarged, with indications for preventive interventional therapy. A platelet count lower than 119 × 109 /L was identified as a predictor of GEV (area under the receiver operating curve, 0.946; sensitivity, 100%; and specificity, 87%). CONCLUSIONS GEVs are important complications that should not be ignored in patients who have undergone a Fontan procedure. Platelet counts lower than 119 × 109 /L may help to prompt patient screening by using abdominal dynamic CT to identify GEVs and their draining collateral veins in these patients.
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Affiliation(s)
- Yoshiharu Isoura
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yuki Cho
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Eiji Ehara
- Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Tsugutoshi Suzuki
- Department of Pediatric Electrophysiology, Osaka City General Hospital, Osaka, Japan
| | | | - Kiyohide Kioka
- Department of Hepatology, Osaka City General Hospital, Osaka, Japan
| | - Takashi Hamazaki
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yosuke Murakami
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
- Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan
| | - Daisuke Tokuhara
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
- * E-mail:
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Nair AV, Macdonald DB, Kelly EM, Satheesh S, Venugopalan P, Soman DK. Utility of MRCP in surveillance of primary sclerosing cholangitis associated hepatobiliary malignancy: 15 year experience at a single institution in Ontario, Canada. Clin Imaging 2021; 81:47-53. [PMID: 34598005 DOI: 10.1016/j.clinimag.2021.08.007] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2021] [Revised: 08/18/2021] [Accepted: 08/23/2021] [Indexed: 11/03/2022]
Abstract
OBJECTIVE Magnetic resonance cholangiopancreatography (MRCP) is used for the surveillance of primary sclerosing cholangitis (PSC) and its associated complications. The time interval gap for subsequent follow-up MRCP is variable depending on clinical practice patterns, therefore this study was done to assess the MRCP follow-up strategy used in our institution for screening PSC-associated hepatobiliary malignancies. MATERIALS AND METHODS This retrospective observational cohort included MRCP studies in adult patients, with clinical and radiological diagnosis of PSC over the past 15-year period between January 1, 2003 to December 31, 2018. The study population was grouped based on the presence and absence of PSC-associated malignancy. The frequency of MRCP follow-up was compared between the groups to look for MRI ordering trends in surveillance for PSC-associated complications. RESULTS The overall median interval follow-up with MRCP was 14 months. The median follow-up interval in cases with PSC-associated malignancy was 6.0 months, compared to 13.1 months in the PSC group without malignancy (p 0.013). During the study period, the PSC-associated malignancy group had a median number of 7.5 scans, while the no malignancy group had a median number of 4 scans. Three patients (3/10, 30%) developed hepatobiliary malignancies within the first year of clinical diagnosis of PSC. The most common malignancy associated with PSC was cholangiocarcinoma (4.6%,7/10). Other PSC-associated malignancies included carcinoma gallbladder (1.3%,2/10), and hepatocellular carcinoma (0.6%,1/10). The median age of PSC associated malignancies was 56 (IQR 15) and higher compared to median age of PSC group without malignancies 46 (IQR 25.5), p 0.035. CONCLUSION The median interval for subsequent follow-up MRCP in our study cohort was 14 months. One-third of PSC-associated hepato-biliary malignancies developed within the first year of clinical diagnosis of PSC, and the risk of PSC-associated hepato-biliary malignancy is constant after the first year.
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Affiliation(s)
| | - D Blair Macdonald
- Dept of Medical Imaging, The Ottawa Hospital Research Institute, Ottawa, Canada
| | - Erin M Kelly
- Dept of Gastro-enterology, The Ottawa Hospital, University of Ottawa, Canada
| | - Soumya Satheesh
- Dept of Pathology, VPS Lakeshore Hospital, Kochi, Kerala, India
| | - Prasanna Venugopalan
- Dept of Obstetrics and Gynaecology, Travancore Medical College, Kollam, Kerala, India
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Mahmood K, Haider I, Adil SO, Ubaid M, Talib A. Non-invasive assessment of large esophageal varices with liver cirrhosis ; a study conducted in Pakistan. THE JOURNAL OF MEDICAL INVESTIGATION 2020; 66:248-251. [PMID: 31656283 DOI: 10.2152/jmi.66.248] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
Abstract
The assessment of non-invasive parameters for the prediction of large esophageal varices among patients with liver cirrhosisis is of utmost importance. In this study, non-invasive parameters for prediction of large esophageal varices were retrospectively evaluated. The presence of esophageal varices grade III and IV was classified as large esophageal varices positive while no varices or grade I and II were classified as large esophageal varices negative. There were 473 (90.09%) patients with ascites [mild 38 (8.03%), moderate 257 (54.33%) and severe 178 (37.63%)]. Frequency of esophageal varices was found to be higher (n=415, 79.04%). Whereas, large esophageal varices were found in 251 (47.81%) patients. The sensitivity, specificity, positive predicted value, negative predicted value and test accuracy of thrombocytopenia in predicting large esophageal varices were found to be 88.05%, 59.85%, 66.77%, 84.54% and 73.33% respectively. A significant association for large esophageal varices was observed for low platelet counts (AOR : 0.98, 95% CI : 0.97-0.99), high bilirubin level (AOR : 1.22, 95% CI : 1.07-1.39), ascites (AOR : 1.98, CI : 1.02-3.85) and Child score A (AOR : 0.26, 95% CI : 0.09-0.75) and Child Score B (AOR : 0.42, 95% CI : 0.28-0.61). In conclusion, low platelet count, high bilirubin level and ascites are found to be non-invasive predictive factor for large esophageal varices. J. Med. Invest. 66 : 248-251, August, 2019.
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Affiliation(s)
- Khalid Mahmood
- Former Dean, Department of Medicine and Allied Sciences, Dow University of Health Sciences, Karachi, Pakistan
| | - Iftikhar Haider
- Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan
| | - Syed Omair Adil
- Department of Research, Dow University of Health Sciences, Karachi, Pakistan
| | | | - Abu Talib
- Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan
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Schwarzer R, Reiberger T, Mandorfer M, Kivaranovic D, Hametner S, Hametner S, Paternostro R, Scheiner B, Schneeweiss-Friedl J, Trauner M, Schoefl R, Maieron A. The von Willebrand Factor antigen to platelet ratio (VITRO) score predicts hepatic decompensation and mortality in cirrhosis. J Gastroenterol 2020; 55:533-542. [PMID: 31832759 DOI: 10.1007/s00535-019-01656-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/10/2019] [Accepted: 11/30/2019] [Indexed: 02/04/2023]
Abstract
BACKGROUND The ratio of von Willebrand Factor to platelets (VITRO) reflects the severity of fibrosis and portal hypertension and might thus hold prognostic value. METHODS Patients with compensated cirrhosis were recruited. VITRO, Child-Pugh score (CPS) and MELD were determined at study entry. Hepatic decompensation was defined as variceal bleeding, ascites or hepatic encephalopathy. Liver transplantation and death were recorded. RESULTS One hundred and ninety-four patients with compensated cirrhosis (CPS-A 89%, B 11%; 56% male; median age 56 years; 50% with varices) were included. During a median follow-up of 45 months (IQR 29-61), decompensation occurred in 35 (18%) patients and 14 (7%) patients deceased. The risk of hepatic decompensation was significantly increased in the n = 88 (45%) patients with a VITRO ≥ 2.5 (p < 0.001). Patients with a VITRO ≥ 2.5 had a higher probability of decompensation at 1-year 9% (95% CI 3-16) vs. 0% (95% CI 0-0) and at 2-years 18% (95% CI 10-27%), vs. 4% (95% CI 0-8%) as compared to patients with VITRO < 2.5. Patients with VITRO ≥ 2.5, the estimated 1-year/2-year survival rates were at 98% (95% CI 95-100%) and 94% (95% CI 88-99%) as compared to 100% (95% CI 100-100%) both in the patients with a VITRO < 2.5 (p < 0.001). After adjusting for age, albumin and MELD, VITRO ≥ 2.5 remained as significant predictor of transplant-free mortality (HR 1.38, CI 1.09-1.76; p = 0.007). Patients with compensated cirrhosis and VITRO > 2.1 after hepatitis C eradication remained at significantly increased risk for decompensation (p = 0.033). CONCLUSIONS VITRO is a valuable prognostic tool for estimating the risk of decompensation and mortality in patients with compensated cirrhosis-including the setting after hepatitis C eradication.
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Affiliation(s)
- Rémy Schwarzer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | - Danijel Kivaranovic
- Department of Statistics and Operations Research, University of Vienna, Oskar-Morgenstern-Platz 1, 1090, Vienna, Austria
| | - Silvia Hametner
- Division of Gastroenterology and Hepatology, Ordensklinikum Linz, Fadingerplatz 1, 4020, Linz, Austria
| | - Stephanie Hametner
- Division of Gastroenterology and Hepatology, Ordensklinikum Linz, Fadingerplatz 1, 4020, Linz, Austria
| | - Rafael Paternostro
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
- Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | | | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria
| | - Rainer Schoefl
- Division of Gastroenterology and Hepatology, Ordensklinikum Linz, Fadingerplatz 1, 4020, Linz, Austria
| | - Andreas Maieron
- Division of Gastroenterology and Hepatology, Ordensklinikum Linz, Fadingerplatz 1, 4020, Linz, Austria.
- Internal Medicine II, Gastroenterology and Hepatology, Karl Landsteiner University of Health Sciences, University Hospital of St. Pölten, Dunant Platz 1, 3100, St. Pölten, Austria.
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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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Chapman MH, Thorburn D, Hirschfield GM, Webster GGJ, Rushbrook SM, Alexander G, Collier J, Dyson JK, Jones DE, Patanwala I, Thain C, Walmsley M, Pereira SP. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut 2019; 68:1356-1378. [PMID: 31154395 PMCID: PMC6691863 DOI: 10.1136/gutjnl-2018-317993] [Citation(s) in RCA: 178] [Impact Index Per Article: 29.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2018] [Revised: 02/21/2019] [Accepted: 03/24/2019] [Indexed: 12/11/2022]
Abstract
These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.
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Affiliation(s)
- Michael Huw Chapman
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Liver Unit, Royal Free London NHS Foundation Trust, London, UK
| | | | - Gideon M Hirschfield
- Toronto Centre for Liver Disease, University Health Network and University of Toronto, Toronto, Canada
| | | | - Simon M Rushbrook
- Department of Hepatology, Norfolk and Norwich University Hospitals NHS Trust, Norwich, UK
| | | | | | - Jessica K Dyson
- Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - David Ej Jones
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - Imran Patanwala
- Gastroenterology, Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK
- University of Liverpool, Liverpool, UK
| | | | | | - Stephen P Pereira
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Institute for Liver & Digestive Health, University College London, London, UK
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Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management. Dig Dis Sci 2019; 64:632-642. [PMID: 30725292 DOI: 10.1007/s10620-019-05484-y] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2018] [Accepted: 01/19/2019] [Indexed: 02/08/2023]
Abstract
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterized by progressive idiopathic stricturing of the biliary system, typically leading to cirrhosis, end-stage liver disease, and colonic or hepatobiliary malignancy. Its presentation is often that of asymptomatic alkaline phosphatase elevation. When symptoms are present, they typically include fatigue, pruritus, or jaundice. The diagnosis can be confirmed via cholangiography, either magnetic resonance cholangiography (MRCP) or endoscopic retrograde cholangiography if the former is inconclusive. The clinical course is marked by progressive liver disease leading to cirrhosis with its attendant complications of portal hypertension, often including recurrent episodes of cholangitis. Greater elevation in alkaline phosphatase or liver stiffness is associated with worse clinical outcomes. Management includes endoscopic treatment of symptomatic biliary strictures and evaluation of dominant strictures as no adequate medical treatment is available. Multiple medical therapies are under evaluation. Ultimately, liver transplantation may be necessary for management of decompensated cirrhosis or disabling symptoms. There is also a markedly increased risk of cancer, notably including cholangiocarcinoma and gallbladder and colorectal cancers (particularly in patients with colitis). Cancer screening can be done with semi-annual liver imaging (MRCP or ultrasound) and colonoscopy every 1-2 years in those with colitis.
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Marya NB, Tabibian JH. Role of endoscopy in the management of primary sclerosing cholangitis. World J Gastrointest Endosc 2019; 11:84-94. [PMID: 30788027 PMCID: PMC6379747 DOI: 10.4253/wjge.v11.i2.84] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2018] [Revised: 01/15/2019] [Accepted: 01/30/2019] [Indexed: 02/06/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) is a rare but prominent fibroinflammatory cholangiopathy which can affect individuals of essentially any age. It carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent risk for biliary tract and other complications, and the paucity of effective pharmacotherapies, endoscopy plays a major role in the care of many patients with this disorder. In this review, we discuss the endoscopic management of PSC, including established and evolving approaches to the diagnosis and treatment of its benign as well as malignant sequelae. Owing to the rarity of PSC and dearth of high-quality evidence, we propose pragmatic approaches based on both currently available data and expert opinion.
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Affiliation(s)
- Neil Bharat Marya
- Vatche and Tamar Manoukian Division of Digestive Diseases, UCLA Gastroenterology Fellowship Training Program, Los Angeles, CA 90095, United States
| | - James H Tabibian
- Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States
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Isayama H, Tazuma S, Kokudo N, Tanaka A, Tsuyuguchi T, Nakazawa T, Notohara K, Mizuno S, Akamatsu N, Serikawa M, Naitoh I, Hirooka Y, Wakai T, Itoi T, Ebata T, Okaniwa S, Kamisawa T, Kawashima H, Kanno A, Kubota K, Tabata M, Unno M, Takikawa H. Clinical guidelines for primary sclerosing cholangitis 2017. J Gastroenterol 2018; 53:1006-1034. [PMID: 29951926 PMCID: PMC8930933 DOI: 10.1007/s00535-018-1484-9] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2018] [Accepted: 06/11/2018] [Indexed: 02/04/2023]
Abstract
BACKGROUND Primary sclerosing cholangitis (PSC) is relatively rare disease and pathogenesis and methods of treatments were still not established. Then, we had conducted the making clinical guidelines to manage patients with PSC based on the literature review and expert opinions. These clinical guidelines were made for the medical doctors on the management of PSC, except child case of PSC. METHODS We had employed modified Delphi method. The production committee decided guidelines, strength of recommendations and evidence level after reviewed literatures systematically, and The Expert panel evaluated those. The Scientific Committee of the Japan Biliary Association (JBA) evaluated revised guidelines, and the Public comments were collected on web site of JBA. RESULTS We had made 16 guidelines about epidemiology/pathophysiology, diagnostics, therapy and prognosis. Also, we had made both diagnostic and therapeutic flow chart. CONCLUSIONS We hope that these guidelines will contribute to the improvement and development of the medical care of PSC.
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Affiliation(s)
- Hiroyuki Isayama
- Department of Gastroenterology, Graduate School of Medicine, Juntendo University, Tokyo, Japan
| | - Susumu Tazuma
- Department of General Internal Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University Hospital, 1-2-3, Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
| | - Norihiro Kokudo
- Department of Surgery, National Center for Global Health and Medicine, Tokyo, Japan
| | - Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Toshio Tsuyuguchi
- Department of Medicine and Gastroenterology, Chiba University, Chiba, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan
| | - Kenji Notohara
- Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Suguru Mizuno
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Nobuhisa Akamatsu
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masahiro Serikawa
- Department of Gastroenterology and Metabolism, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan
| | - Yoshiki Hirooka
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan
| | - Toshifumi Wakai
- Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Tomoki Ebata
- Division of Surgical Oncology, Department of Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Shinji Okaniwa
- Department of Gastroenterology, Iida Municipal Hospital, Nagano, Japan
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Komagome Metropolitan Hospital, Tokyo, Japan
| | - Hiroki Kawashima
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Atsushi Kanno
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
| | - Keiichi Kubota
- Second Department of Surgery, Dokkyo Medical University, Tochigi, Japan
| | - Masami Tabata
- Department of Surgery, Matsusaka Central General Hospital, Matsusaka, Mie, Japan
| | - Michiaki Unno
- Department of Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
| | - Hajime Takikawa
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
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Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M. Primary sclerosing cholangitis. Lancet 2018; 391:2547-2559. [PMID: 29452711 DOI: 10.1016/s0140-6736(18)30300-3] [Citation(s) in RCA: 282] [Impact Index Per Article: 40.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2016] [Revised: 11/24/2017] [Accepted: 01/03/2018] [Indexed: 12/13/2022]
Abstract
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance strategies for early cholangiocarcinoma detection are limited. No single drug has been proven to improve transplant-free survival. Liver transplantation is effective for advanced disease but at least 25% of patients develop recurrent disease in the graft.
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Affiliation(s)
- Jessica K Dyson
- Department of Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust and Institute of Cellular Medicine, Newcastle University, Newcastle, UK.
| | - Ulrich Beuers
- Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands
| | - David E J Jones
- Department of Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust and Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - Ansgar W Lohse
- Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - Mark Hudson
- Department of Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust and Institute of Cellular Medicine, Newcastle University, Newcastle, UK
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Ferrari F, Ranucci G, Aloi M, Della Volpe L, Viola F, Miele E, Cucchiara S, Iorio R. A promising medium-term follow-up of pediatric sclerosing cholangitis: Mild phenotype or early diagnosis? Hepatol Res 2018; 48:556-565. [PMID: 29316057 DOI: 10.1111/hepr.13059] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2017] [Revised: 01/02/2018] [Accepted: 01/05/2018] [Indexed: 12/24/2022]
Abstract
AIM Sclerosing cholangitis (SC) is a chronic cholestatic liver disease that is being increasingly diagnosed in childhood. The long-term course and prognosis of pediatric SC are poorly described. METHODS We reviewed data of pediatric SC patients, followed in two referral centers, during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS Among 45 patients (median age, 10.4 years; male patients, 73.4%) 29 (64.4%) were asymptomatic at presentation. Twenty patients (44%) had a concomitant inflammatory bowel disease (SC/IBD). Autoimmune features were found in 20 patients (44%). Liver biopsy showed severe fibrosis or cirrhosis in 32% of cases. Patients with SC alone had a higher rate of interface hepatitis at liver biopsy than SC/IBD patients. All children received ursodeoxycholic acid at diagnosis, and 17 received steroids and/or azathioprine. After a mean follow-up of 8.7 ± 5.6 years, all patients were alive and seven developed at least one liver-related complication. At the end of follow-up, 10 patients stopped immunosuppressants and two had no therapy. Only two patients underwent liver transplantation. Complication-free survival did not differ between SC/IBD and SC patients, but survival was longer in patients without autoimmune features. CONCLUSIONS In our early diagnosed cohort, the 9-year survival with native liver was better than that reported in other studies. Approximately 15% of patients developed liver-related disease complications, less than previously reported. The long-term course of SC was negatively influenced by the presence of autoimmune features, but not by concomitant IBD.
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Affiliation(s)
- Federica Ferrari
- Pediatric Gastroenterology and Liver Unit, Department of Pediatrics, Sapienza University of Rome, Italy
| | - Giusy Ranucci
- Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy
| | - Marina Aloi
- Pediatric Gastroenterology and Liver Unit, Department of Pediatrics, Sapienza University of Rome, Italy
| | - Luca Della Volpe
- Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy
| | - Franca Viola
- Pediatric Gastroenterology and Liver Unit, Department of Pediatrics, Sapienza University of Rome, Italy
| | - Erasmo Miele
- Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy
| | - Salvatore Cucchiara
- Pediatric Gastroenterology and Liver Unit, Department of Pediatrics, Sapienza University of Rome, Italy
| | - Raffaele Iorio
- Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy
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16
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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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Gossard AA, Gores GJ. Primary Sclerosing Cholangitis: What the Gastroenterologist and Hepatologist Needs to Know. Clin Liver Dis 2017; 21:725-737. [PMID: 28987259 DOI: 10.1016/j.cld.2017.06.004] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic biliary tract disease characterized by segmental strictures. The disease is progressive with no proven treatments and may eventually lead to cirrhosis and end-stage liver disease. Abrupt changes in liver biochemistries, pain, and/or cholangitis may suggest a dominant stricture amenable to endoscopic therapy or the development of cholangiocarcinoma. Patients with PSC are at increased risk of cholangiocarcinoma. There is a strong association with inflammatory bowel disease, and an associated increased risk of colorectal cancer. Colonoscopy every 1 to 2 years is appropriate.
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Affiliation(s)
- Andrea A Gossard
- Division of Gastroenterology and Hepatology, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55901, USA.
| | - Gregory J Gores
- Division of Gastroenterology and Hepatology, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55901, USA
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18
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Deneau MR, El-Matary W, Valentino PL, Abdou R, Alqoaer K, Amin M, Amir AZ, Auth M, Bazerbachi F, Broderick A, Chan A, Cotter J, Doan S, El-Youssef M, Ferrari F, Furuya KN, Gottrand M, Gottrand F, Gupta N, Homan M, Kamath BM, Kim KM, Kolho KL, Konidari A, Koot B, Iorio R, Ledder O, Mack C, Martinez M, Miloh T, Mohan P, O'Cathain N, Papadopoulou A, Ricciuto A, Saubermann L, Sathya P, Shteyer E, Smolka V, Tanaka A, Varier R, Venkat V, Vitola B, Vos MB, Woynarowski M, Yap J, Jensen MK. The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration. Hepatology 2017; 66:518-527. [PMID: 28390159 DOI: 10.1002/hep.29204] [Citation(s) in RCA: 145] [Impact Index Per Article: 18.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2016] [Revised: 02/07/2017] [Accepted: 04/04/2017] [Indexed: 12/14/2022]
Abstract
UNLABELLED There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long-term outcome. We identified 781 patients, median age 12 years, with 4,277 person-years of follow-up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event-free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC-inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5-0.9, and 0.7, 95% confidence interval 0.5-0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long-term outcome. CONCLUSION PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC-inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518-527).
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Affiliation(s)
| | | | | | - Reham Abdou
- State University of New York Buffalo, Buffalo, NY
| | - Khaled Alqoaer
- Prince Salman North West Armed Forces Hospital, Tabuk, Saudi Arabia
| | - Mansi Amin
- University of California San Francisco, San Francisco, CA, and Texas Children's Hospital, Houston, TX
| | - Achiya Z Amir
- The Dana-Dwek Children's Hospital, The Tel-Aviv Medical Center, Tel-Aviv University, Tel Aviv, Israel
| | - Marcus Auth
- Alder Hey Children's Hospital, Liverpool, UK
| | | | | | - Albert Chan
- University of Rochester Medical Center, Rochester, NY
| | | | | | | | | | - Katryn N Furuya
- Mayo Clinic, Rochester, MN
- Nemours Alfred I duPont Hospital For Children, Wilmington, DE
| | | | | | - Nitika Gupta
- Emory University School of Medicine, Atlanta, GA
| | | | | | | | | | - Anastasia Konidari
- University of Liverpool, Liverpool, and University of Manchester, Manchester, UK
| | - Bart Koot
- Academic Medical Centre, Amsterdam, The Netherlands
| | | | - Oren Ledder
- Shaare Zedek Medical Center, Jerusalem, Israel
| | - Cara Mack
- University of Colorado School of Medicine, Aurora, CO
| | - Mercedes Martinez
- Columbia University College of Physicians and Surgeons, New York, NY
| | - Tamir Miloh
- Texas Children's Hospital, Houston, TX, and Phoenix Children's Hospital, Phoenix, AZ
| | | | | | | | | | | | - Pushpa Sathya
- Memorial University, St. John's, Newfoundland and Labrador, Canada
| | | | | | | | - Raghu Varier
- Northwest Pediatric Gastroenterology LLC, Portland, OR
| | - Veena Venkat
- University of Pittsburgh Medical Center, Pittsburgh, PA
| | | | - Miriam B Vos
- Emory University School of Medicine, Atlanta, GA
| | | | - Jason Yap
- University of Alberta, Edmonton, Alberta, Canada
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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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Abstract
PURPOSE OF REVIEW Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. RECENT FINDINGS PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis. The incidence of PSC appears to be increasing, the reasons for which are unclear. The time from diagnosis to liver transplant appears to be longer in more recent studies compared with earlier studies, suggesting a better overall prognosis than previously believed. In addition, with an increasing number of patients undergoing liver transplantation for PSC, the frequency of death because of liver failure has decreased, whereas cancer-related deaths have increased among patients with PSC. SUMMARY PSC is a heterogeneous disease with a variety of clinical outcomes, both fatal and nonfatal. The progression of liver fibrosis in an individual patient is difficult to predict and may vary from a relatively benign, nonprogressive form to a rapidly progressive form with the need for liver transplantation.
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Suárez Ferrer C, Llop Herrera E, Calvo Moya M, Vera Mendoza MI, González Partida I, González Lama Y, Matallana Royo V, Calleja Panero JL, Abreu García L. Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2017; 108:79-83. [PMID: 26838489 DOI: 10.17235/reed.2015.3954/2015] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
INTRODUCTION The possibility of developing idiopathic portal hypertension has been described with thiopurine treatment despite compromises the prognosis of these patients, the fact its true prevalence is unknown. MATERIAL AND METHODS A cross-sectional study was conducted in a cohort of inflammatory bowel disease (IBD) patients followed at our unit, to determine the prevalence of diagnosis of idiopathic portal hypertension (IPH) and its relationship with thiopurine treatment. RESULTS At the time of the analysis, 927/1,419 patients were under treatment with thiopurine drugs (65%). A total of 4 patients with IBD type Crohn's disease with idiopathic portal hypertension probably related to the thiopurine treatment were identified (incidence of 4.3 cases per 1,000). Seventy-five percent of patients started with signs or symptoms of portal hypertension. Only one patient was asymptomatic but the diagnosis of IPH because of isolated thrombocytopenia is suspected. However, note that all patients had thrombocytopenia previously. Abdominal ultrasound with fibroscan, hepatic vein catheterization and liver biopsy were performed on all of them as part of the etiology of portal hypertension. In the abdominal ultrasound, indirect portal hypertension data were observed in all patients (as splenomegaly) cirrhosis was also ruled out. The fibroscan data showed significant liver fibrosis (F2-F3). CONCLUSION Idiopathic portal hypertension following thiopurine treatment in IBD patients is a rare occurrence, but it must be borne in mind in the differential diagnosis for early diagnosis, especially in patients undergoing thiopurine treatment over a long period. The presence of thrombocytopenia is often the only predictor of its development in the preclinical stage.
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Affiliation(s)
| | - Elba Llop Herrera
- GASTROENTEROLOGIA , Hospital Universitario Puerta de Hierro Majadahonda. Madrid, ESPAÑA
| | - Marta Calvo Moya
- GASTROENTEROLOGIA , Hospital Universitario Puerta de Hierro Majadahonda. Madrid, españa
| | | | | | - Yago González Lama
- Servicio de Gastroenterología y Hepatología, Hospital Universitario Puerta de Hierro, España
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Liberal R, Grant CR. Cirrhosis and autoimmune liver disease: Current understanding. World J Hepatol 2016; 8:1157-1168. [PMID: 27729952 PMCID: PMC5055585 DOI: 10.4254/wjh.v8.i28.1157] [Citation(s) in RCA: 43] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/03/2016] [Revised: 05/14/2016] [Accepted: 08/08/2016] [Indexed: 02/06/2023] Open
Abstract
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues. Patients with cirrhosis have higher risks or morbidity and mortality, and that in the decompensated phase, complications of portal hypertension and/or liver dysfunction lead to rapid deterioration. Accurate diagnosis and monitoring of cirrhosis is, therefore of upmost importance. Liver biopsy is currently the gold standard technique, but highly promising non-invasive methodology is under development. Liver transplantation (LT) is an effective therapeutic option for the management of end-stage liver disease secondary to AIH, PBC and PSC. LT is indicated for AILD patients who have progressed to end-stage chronic liver disease or developed intractable symptoms or hepatic malignancy; in addition, LT may also be indicated for patients presenting with acute liver disease due to AIH who do not respond to steroids.
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Tse Y, Narula N, Uhanova J, Sirpal S, Marotta P, Chandok N. When to perform gastroscopy in the PSC patient. Ann Hepatol 2016; 15:135-6. [PMID: 26626651 DOI: 10.5604/16652681.1184294] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Affiliation(s)
- Yvonne Tse
- Division of Gastroenterology, University of Western Ontario, London, Ontario, Canada
| | - Neeraj Narula
- Division of Gastroenterology, University of Western Ontario, London, Ontario, Canada
| | - Julia Uhanova
- Section of Hepatology, Department of Medicine, University of Manitoba, Winnipeg, Manitoba. Canada
| | - Sanjeev Sirpal
- Department of Medicine, William Osler Health Centre, Brampton, Ontario, Canada
| | - Paul Marotta
- Division of Gastroenterology, University of Western Ontario, London, Ontario, Canada
| | - Natasha Chandok
- Division of Gastroenterology, University of Western Ontario, London, Ontario, Canada
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Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol 2015; 110:646-59; quiz 660. [PMID: 25869391 DOI: 10.1038/ajg.2015.112] [Citation(s) in RCA: 330] [Impact Index Per Article: 33.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2014] [Accepted: 03/02/2015] [Indexed: 12/11/2022]
Abstract
Primary sclerosing cholangitis is a chronic cholestatic liver disease that can shorten life and may require liver transplantation. The cause is unknown, although it is commonly associated with colitis. There is no approved or proven therapy, although ursodeoxycholic acid is used by many on an empiric basis. Complications including portal hypertension, fat-soluble vitamin deficiency, metabolic bone diseases, and development of cancers of the bile duct or colon can occur.
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Affiliation(s)
- Keith D Lindor
- 1] College of Health Solutions, Arizona State University, Phoenix, Arizona, USA [2] Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, Arizona, USA
| | - Kris V Kowdley
- Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, Washington, USA
| | - M Edwyn Harrison
- Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, Arizona, USA
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Peñaloza-Posada MA, Pérez-Torres E, Pérez-Hernández JL, Higuera-de la Tijera F. Non-invasive parameters as predictors of high risk of variceal bleeding in cirrhotic patients. REVISTA MÉDICA DEL HOSPITAL GENERAL DE MÉXICO 2014. [DOI: 10.1016/j.hgmx.2014.09.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
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Detection of risky esophageal varices by two-dimensional ultrasound: when to perform endoscopy. Am J Med Sci 2014; 347:28-33. [PMID: 23267234 DOI: 10.1097/maj.0b013e3182750ce8] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
OBJECTIVE Esophageal varices are a consequence of portal hypertension in cirrhotic patients. Current guidelines recommend that all cirrhotic patients undergo screening endoscopy at diagnosis to identify patients with varices at high risk of bleeding who will benefit from primary prophylaxis. This practice increases costs, involves a degree of invasiveness and discomfort and places a heavy burden on endoscopy units. Several studies have evaluated possible noninvasive predictors of esophageal varices, but most of these studies remain controversial. METHODS The intra-abdominal portion of the esophagus in 673 patients who presented with liver cirrhosis and portal hypertension was examined using standard 2-dimensional (2D) ultrasound. A direct relationship between the degree of varices observed on upper endoscopy and the intra-abdominal esophageal wall thickness was detected using 2D ultrasound. RESULTS The mean thicknesses of the esophageal wall were 3.7 ± 0.5 mm (mean ± standard deviation) in normal individuals, 7.3 ± 3.3 mm in those with esophageal varices and 8.65 ± 1.98 mm in those with risky esophageal varices. The overall accuracy of 2D ultrasound was 95%. CONCLUSIONS The intra-abdominal esophagus should be observed during abdominal ultrasound examination in patients with liver cirrhosis. Two-dimensional ultrasound can play an important role in screening for esophageal varices.
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González-Ojeda A, Cervantes-Guevara G, Chávez-Sánchez M, Dávalos-Cobián C, Ornelas-Cázares S, Macías-Amezcua MD, Chávez-Tostado M, Ramírez-Campos KM, Ramírez-Arce ADR, Fuentes-Orozco C. Platelet count/spleen diameter ratio to predict esophageal varices in Mexican patients with hepatic cirrhosis. World J Gastroenterol 2014; 20:2079-2084. [PMID: 24616574 PMCID: PMC3934478 DOI: 10.3748/wjg.v20.i8.2079] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/26/2013] [Revised: 10/25/2013] [Accepted: 11/05/2013] [Indexed: 02/07/2023] Open
Abstract
AIM: To validate whether the platelet count/spleen size ratio can be used to predict the presence of esophageal varices in Mexican patients with hepatic cirrhosis.
METHODS: This was an analytical cross-sectional study to validate the diagnostic test for hepatic cirrhosis and was performed between February 2010 and December 2011. Patients with a diagnosis of hepatic cirrhosis were included and stratified using their Child-Pugh score. Biochemical parameters were evaluated, and ultrasound was used to measure the longest diameter of the spleen. The platelet count/spleen diameter ratio was calculated and analyzed to determine whether it can predict the presence of esophageal varices. Upper gastrointestinal endoscopy was used as the gold standard. Sensitivity and specificity, positive and negative predictive values, and positive and negative likelihood ratios were determined, with the cutoff points determined by receiver-operating characteristic curves.
RESULTS: A total of 91 patients were included. The mean age was 53.75 ± 12 years; 50 (54.9%) were men, and 41 (45.0%) women. The etiology of cirrhosis included alcohol in 48 (52.7%), virally induced in 24 (26.3%), alcoholism plus hepatitis C virus in three (3.2%), cryptogenic in nine (9.8%), and primary biliary cirrhosis in seven (7.6%). Esophageal varices were present in 73 (80.2%) patients. Child-Pugh classification, 17 (18.6%) patients were classified as class A, 37 (40.6%) as class B, and 37 (40.6%) as class C. The platelet count/spleen diameter ratio to detect esophageal varices independent of the grade showed using a cutoff value of ≤ 884.3, had 84% sensitivity, 70% specificity, and positive and negative predictive values of 94% and 40%, respectively.
CONCLUSION: Our results suggest that the platelet count/spleen diameter ratio may be a useful tool for detecting esophageal varices in patients with hepatic cirrhosis.
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Procopet B, Berzigotti A. New Tools for the Noninvasive Assessment of Cirrhosis. CURRENT HEPATOLOGY REPORTS 2014. [DOI: 10.1007/s11901-014-0215-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/04/2025]
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Abstract
Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease characterized by multifocal strictures of intra and extrahepatic bile ducts. PSC occurs more commonly in men and is often associated with inflammatory bowel disease. At present, there is no effective medical therapy for PSC. Current management of patients with PSC is centered on endoscopic therapy of biliary strictures, management of complications of chronic cholestasis and of progressive liver disease, and close clinical monitoring for development of cholangiocarcinoma, as well as for timely referral for liver transplantation.
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Affiliation(s)
- Claudia O Zein
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, 9500 Euclid Avenue, A31, Cleveland, OH 44195, USA.
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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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Berzigotti A, Seijo S, Arena U, Abraldes JG, Vizzutti F, García-Pagán JC, Pinzani M, Bosch J. Elastography, spleen size, and platelet count identify portal hypertension in patients with compensated cirrhosis. Gastroenterology 2013; 144:102-111.e1. [PMID: 23058320 DOI: 10.1053/j.gastro.2012.10.001] [Citation(s) in RCA: 383] [Impact Index Per Article: 31.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/03/2012] [Revised: 09/27/2012] [Accepted: 10/02/2012] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Noninvasive methods are needed to identify clinically significant portal hypertension (CSPH) and esophageal varices (EVs) in patients with compensated cirrhosis. We looked for markers of the presence of CSPH and EVs in patients with cirrhosis. METHODS We performed a cross-sectional study that included a training set of 117 patients with compensated cirrhosis, confirmed by histology, from a tertiary referral center. Spleen diameter was measured by ultrasound, and liver stiffness (LS) was measured by transient elastography; endoscopy was used as the standard for detection of EVs, and measurements of hepatic venous pressure gradient were used as the standard for identifying CSPH. We assessed the ability of platelet count, spleen diameter, LS, and combinations of these factors (ie, ratio of platelet count to spleen size, and LS × spleen size/platelet count [LSPS]) to identify patients with CSPH and EV. The analysis included 2 new statistical models: the PH risk score and the varices risk score. Results were validated using an independent series of 56 patients with compensated patients from another center. RESULTS LS was the best single noninvasive variable for identifying patients with CSPH (area under the receiver operating characteristic, 0.883; 95% confidence interval [CI], 0.824-0.943; P < .0001). The area under the receiver operating characteristic value increased when LS was combined with platelet count and spleen size, either as LSPS (0.918; 95% CI, 0.872-0.965; P < .0001) or PH risk score (0.935; 95% CI, 0.893-0.977; P < .0001). More than 80% of patients were accurately classified using LSPS and PH risk score. Analyses of the varices risk score and LSPS were superior to all other noninvasive tests for identifying patients with EVs (area under the receiver operating characteristic, 0.909; 95% CI, 0.841-0.954 and 0.882; 95% CI, 0.810-0.935, respectively); they correctly classified 85% of patients in the training set and 75% in the validation set. CONCLUSIONS Combined data on LS, spleen diameter, and platelet count can be used to identify patients with compensated cirrhosis most likely to have CSPH and EV.
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Affiliation(s)
- Annalisa Berzigotti
- Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, University of Barcelona, Spain
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Lazaridis KN, Gores GJ. Primary Sclerosing Cholangitis. SHACKELFORD'S SURGERY OF THE ALIMENTARY TRACT 2013:1405-1416. [DOI: 10.1016/b978-1-4377-2206-2.00112-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Management of primary sclerosing cholangitis: conventions and controversies. CANADIAN JOURNAL OF GASTROENTEROLOGY = JOURNAL CANADIEN DE GASTROENTEROLOGIE 2012; 26:261-8. [PMID: 22590699 DOI: 10.1155/2012/426430] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Primary sclerosing cholangitis (PSC) is a chronic inflammatory cholangiopathy that results in fibrotic strictures and dilations of the intra- and extrahepatic bile ducts. PSC is uncommon, occurs predominantly in males and has a strong association with inflammatory bowel disease. While the pathogenesis of PSC has not been fully elucidated, emerging evidence supports roles for the innate and adaptive immune systems, and genome-wide analyses have identified several genetic associations. Using the best available evidence, the present review summarizes the current understanding of the diagnosis, pathogenesis and management of PSC. Despite its rarity, there is an urgent need for collaborative research efforts to advance therapeutic options for PSC beyond liver transplantation.
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Min YW, Bae SY, Gwak GY, Paik YH, Choi MS, Lee JH, Paik SW, Yoo BC, Koh KC. A clinical predictor of varices and portal hypertensive gastropathy in patients with chronic liver disease. Clin Mol Hepatol 2012; 18:178-84. [PMID: 22893868 PMCID: PMC3415878 DOI: 10.3350/cmh.2012.18.2.178] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2011] [Revised: 02/14/2012] [Accepted: 02/14/2012] [Indexed: 12/13/2022] Open
Abstract
Background/Aims The aim of this study was to identify the parameters that could noninvasively predict the presence of esophageal/gastric varices and portal hypertensive gastropathy (PHG) in patients with chronic liver disease (CLD), and to determine the accuracy of those parameters. Methods We retrospectively analyzed 232 patients with CLD who underwent both upper endoscopy and liver CT within an interval of 3 months. The multidimensional index (M-Index) for spleen volume was obtained from the multiplication of splenic length, width, and thickness, as measured by computer tomography. Results The multivariate analysis revealed that platelet, albumin, and M-Index were independently associated with the presence of varices and PHG. We combined three independent parameters, and developed a varices and portal hypertensive gastropathy (VAP) scoring system (=[platelet count (/mm3)×albumin (g/dL)]/[M-Index (cm3)]). The area under the receiver operating characteristic curve of the VAP score was 0.850 (95% confidence interval, 0.801-0.899). The VAP cut-off value of 861 had a sensitivity of 85.3%, a positive likelihood ratio of 3.17, and a negative predictive value of 86.4%. For predicting high-risk lesions for bleeding, with a cut-off value of 861 the sensitivity was 92.0%, the positive likelihood ratio was 2.20, and the negative predictive value was 96.4%. Conclusions The VAP score can predict the presence of varices and PHG in patients with CLD and may increase the cost-benefit of screening endoscopy in the clinical practice setting. A prospective validation study is necessary in the future.
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Affiliation(s)
- Yang Won Min
- Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
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Bosch J, Abraldes JG, Albillos A, Aracil C, Bañares R, Berzigotti A, Calleja JL, de la Peña J, Escorsell A, García-Pagán JC, Genescà J, Hernández-Guerra M, Ripoll C, Planas R, Villanueva C. Hipertensión portal: recomendaciones para su evaluación y tratamiento. GASTROENTEROLOGIA Y HEPATOLOGIA 2012; 35:421-50. [DOI: 10.1016/j.gastrohep.2012.02.009] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/09/2012] [Accepted: 02/15/2012] [Indexed: 12/16/2022]
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Mangone M, Moretti A, Alivernini F, Papi C, Orefice R, Dezi A, Amadei E, Aratari A, Bianchi M, Tornatore V, Koch M. Platelet count/spleen diameter ratio for non-invasive diagnosis of oesophageal varices: is it useful in compensated cirrhosis? Dig Liver Dis 2012; 44:504-7. [PMID: 22321622 DOI: 10.1016/j.dld.2011.12.016] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2011] [Revised: 12/14/2011] [Accepted: 12/18/2011] [Indexed: 12/11/2022]
Abstract
AIM To assess the diagnostic accuracy of the platelet count/spleen diameter ratio for identification of oesophageal varices and/or hypertensive gastropathy in patients with compensated cirrhosis. METHODS Platelet count/spleen diameter ratio was calculated in 87 consecutive patients with compensated cirrhosis. A new cut-off with the highest sensitivity and specificity for the presence/absence of oesophageal varices and/or hypertensive gastropathy was identified. Performance of the platelet count/spleen diameter ratio considering previously reported cut-off values were then tested in our population. RESULTS A platelet count/spleen diameter ratio <936.4 had the best sensitivity and specificity for the diagnosis of oesophageal varices and for all endoscopic findings of portal hypertension. A value lower than 936.4 allowed identification of 64.5% of patients with oesophageal varices and 66.7% of patients with any sign of portal hypertension; a value higher than 936.4 excluded oesophageal varices in 64.3% of patients and any sign of portal hypertension in 68.6% of patients. CONCLUSIONS In patients with compensated cirrhosis, the platelet count/spleen diameter ratio is not a useful parameter to avoid unnecessary upper endoscopy, independently of the cut-off.
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Affiliation(s)
- Manuela Mangone
- Gastroenterology and Hepatology Unit, San Filippo Neri Hospital, Rome, Italy.
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Chawla S, Katz A, Attar BM, Gupta A, Sandhu DS, Agarwal R. Platelet count/spleen diameter ratio to predict the presence of esophageal varices in patients with cirrhosis: a systematic review. Eur J Gastroenterol Hepatol 2012; 24:431-436. [PMID: 22410714 DOI: 10.1097/meg.0b013e3283505015] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Esophageal variceal bleeding remains the leading cause of acute mortality in patients with cirrhosis. Platelet count to spleen diameter (PC/SD) ratio less than 909 is one of several parameters proposed for the noninvasive prediction of esophageal varices. The aim of this study is to systematically review the evidence on the diagnostic accuracy of the 909 ratio. METHODS We identified relevant studies from a MEDLINE search and performed a meta-analysis to estimate the pooled sensitivity, specificity, and positive and negative likelihood ratios (LRs) using Meta-Disc software. RESULTS Eight studies met the inclusion criteria and included a total of 1275 patients. Meta-analysis yielded a pooled sensitivity of 89% [95% confidence interval (CI) 87-92%; I2 statistic 92.9%] and a pooled specificity of 74% (95% CI 70-78%; I2 statistic 94.5%). The pooled positive LR was 3.5 (95% CI 1.92-6.25; I2 statistic 94.0%) and the pooled negative LR was 0.12 (95% CI 0.05-0.32; I2 statistic 90.8%). The quality of the evidence as assessed by the GRADE methodology was low. CONCLUSION In its present form, the test characteristics of PC/SD ratio of 909 may not be adequate to completely replace esophagogastroduodenoscopy as a noninvasive screening tool for esophageal varices, given the low grade of evidence. However, it may be potentially useful as part of a prediction rule incorporating other clinical characteristics or varying PC/SD cutoffs. When compared with other noninvasive predictor tools, the PC/SD ratio is elegant, simple, and inexpensive. With some minor modifications, it may become a helpful tool to limit the number of endoscopies in primary prophylaxis to be performed in patients with portal hypertension.
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Affiliation(s)
- Saurabh Chawla
- Division of Gastroenterology, Department of Medicine, Cook County-John H Stroger Jr Hospital, Illinois, USA.
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Nguyen DL, LaRusso NF, Lazaridis KN. Primary sclerosing cholangitis. BLUMGART'S SURGERY OF THE LIVER, PANCREAS AND BILIARY TRACT 2012:603-614.e3. [DOI: 10.1016/b978-1-4377-1454-8.00041-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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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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Violi F, Basili S, Raparelli V, Chowdary P, Gatt A, Burroughs AK. Patients with liver cirrhosis suffer from primary haemostatic defects? Fact or fiction? J Hepatol 2011; 55:1415-27. [PMID: 21718668 DOI: 10.1016/j.jhep.2011.06.008] [Citation(s) in RCA: 124] [Impact Index Per Article: 8.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2011] [Revised: 06/20/2011] [Accepted: 06/21/2011] [Indexed: 02/06/2023]
Abstract
Patients with cirrhosis can have abnormalities in laboratory tests reflecting changes in primary haemostasis, including bleeding time, platelet function tests, markers of platelet activation, and platelet count. Such changes have been considered particularly relevant in the bleeding complications that occur in cirrhosis. However, several studies have shown that routine diagnostic tests, such as platelet count, bleeding time, PFA-100, thromboelastography are not clinically useful to stratify bleeding risk in patients with cirrhosis. Moreover, treatments used to increase platelet count or to modulate platelet function could potentially do harm. Consequently the optimal management of bleeding complications is still a matter of discussion. Moreover, in the last two decades there has been an increased recognition that not only bleeding but also thrombosis complicates the clinical course of cirrhosis. Thus, we performed a literature search looking at publications studying both qualitative and quantitative aspects of platelet function to verify which primary haemostasis defects occur in cirrhosis. In addition, we evaluated the contribution of qualitative and quantitative aspects of platelet function to the clinical outcome in cirrhosis and their therapeutic management according to the data available in the literature. From the detailed analysis of the literature, it appears clear that primary haemostasis may not be defective in cirrhosis, and a low platelet count should not necessarily be considered as an automatic index of an increased risk of bleeding. Conversely, caution should be observed in patients with severe thrombocytopenia where its correction is advised if bleeding occurs and before invasive diagnostic and therapeutic procedures.
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Affiliation(s)
- F Violi
- Divisione di I Clinica Medica, Sapienza-University of Rome, Rome, Italy.
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Abstract
One of typical examples of liver-gut cross talk is the interaction and impact of inflammatory bowel disease (IBD) and hepatobiliary (HB) abnormalities on each other's disease course. There are several layers of association between IBD and HB diseases: (i) HB diseases and IBD share pathogenetic mechanisms; (ii) HB diseases parallel structural and pathophysiological changes seen with IBD; and (iii) hepatic toxicity is associated with medical therapy for IBD. Interdisciplinary approach, involving gastroenterologists, hepatologists and, in advanced cases, general, colorectal, and liver transplant surgeons, is advocated.
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Affiliation(s)
- Preethi Gk Venkatesh
- Department of Gastroenterology/Hepatology, Digestive Disease Institute, the Cleveland Clinic Foundation, Cleveland, Ohio, USA
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Abstract
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder that most commonly affects middle-aged men. PSC is strongly associated with IBD, and in this setting the risk of colorectal cancer is markedly increased. Cholangiocarcinoma, and its devastating consequences, is another well-recognized complication of PSC. This condition tends to progress to end-stage liver disease, and patients with PSC have reduced survival rates compared with the general population. Despite significant research efforts in this field, the pathogenetic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. Effective medical therapy is lacking; liver transplantation is the only curative treatment modality, with excellent outcomes in this patient population.
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Affiliation(s)
- Flavia Mendes
- Department of Medicine, University of Miami Miller School of Medicine, Miami VA Medical Center, 1201 NW 16th Street Miami, FL 33125, USA
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Navaneethan U, Shen B. Hepatopancreatobiliary manifestations and complications associated with inflammatory bowel disease. Inflamm Bowel Dis 2010; 16:1598-619. [PMID: 20198712 DOI: 10.1002/ibd.21219] [Citation(s) in RCA: 145] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Diseases involving the hepatopancreatobiliary (HPB) system are frequently encountered in patients with inflammatory bowel disease (IBD). Hepatobiliary manifestations constitute some of the most common extraintestinal manifestations of IBD. They appear to occur with similar frequency in patients with Crohn's disease or ulcerative colitis. HPB manifestations may occur in following settings: 1) disease possibly associated with a shared pathogenetic mechanism with IBD including primary sclerosing cholangitis (PSC), small-duct PSC/pericholangitis and PSC/autoimmune hepatitis overlap, acute and chronic pancreatitis related to IBD; 2) diseases which parallel structural and physiological changes seen with IBD, including cholelithiasis, portal vein thrombosis, and hepatic abscess; and 3) diseases related to adverse effects associated with treatment of IBD, including drug-induced hepatitis, pancreatitis (purine-based agents), or liver cirrhosis (methotrexate), and reactivation of hepatitis B, and biologic agent-associated hepatosplenic lymphoma. Less common HPB manifestations that have been described in association with IBD include autoimmune pancreatitis (AIP), IgG4-associated cholangitis (IAC), primary biliary cirrhosis (PBC), fatty liver, granulomatous hepatitis, and amyloidosis. PSC is the most significant hepatobiliary manifestation associated with IBD and poses substantial challenges in management requiring a multidisciplinary approach. The natural disease course of PSC may progress to cirrhosis and ultimately require liver transplantation in spite of total proctocolectomy with ileal-pouch anal anastomosis. The association between AIP, IAC, and elevated serum IgG4 in patients with PSC is intriguing. The recently reported association between IAC and IBD may open the door to investigate these complex disorders. Further studies are warranted to help understand the pathogenesis of HPB manifestations associated with IBD, which would help clinicians better manage these patients. An interdisciplinary approach, involving gastroenterologists, hepatologists, and, in advanced cases, general, colorectal, and transplant surgeons is advocated.
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Treeprasertsuk S, Kowdley KV, Luketic VAC, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Keach J, Schmoll J, Hoskin T, Thapa P, Enders F, Lindor KD. The predictors of the presence of varices in patients with primary sclerosing cholangitis. Hepatology 2010; 51:1302-1310. [PMID: 20044810 PMCID: PMC2898188 DOI: 10.1002/hep.23432] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
UNLABELLED The predictors for developing varices in patients with primary sclerosing cholangitis (PSC) have not been well studied prospectively. We sought to define the predictors for the presence of varices at baseline and for newly developing varices in patients with PSC. We used prospectively collected data from a multicenter randomized trial of high dose ursodeoxycholic acid for PSC. All 150 patients enrolled were reviewed for predictors of varices and we excluded 26 patients who had esophageal varices at baseline so that predictors of newly developing varices could be determined. Clinical examination, blood tests, and upper endoscopy were done before randomization, at 2 years and after 5 years. Liver biopsy was performed at entry and at 5 years. The median age (interquartile range) of patients was 45.9 years (35.8, 54.9). In a multivariable logistic regression, a higher Mayo risk score (> or =0.87) or a higher aspartate/alanine aminotransferase (AST/ALT) ratio (> or =1.12) were significantly associated with the presence of varices at initial endoscopy (odds ratio = 1.9 and 3.9). By the end of the study, 25 patients had new varices (20.2%). In a Cox model, after adjustment for baseline variables lower platelet count and higher total bilirubin at 2 years were significantly associated with the presence of new varices. The platelet count of 205 (x 10(9)/L) and the total bilirubin level of 1.7 mg/dL were the best cutoff values for the detection of new varices. CONCLUSION A higher Mayo risk score and higher AST/ALT ratio were significantly associated with the presence of varices at initial endoscopy. Lower platelet count and higher total bilirubin at 2 years were significantly associated with an increased risk of developing new varices in patients with PSC.
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Affiliation(s)
- Sombat Treeprasertsuk
- Division of Gastroenterology and Hepatology; Mayo Clinic Rochester
- Division of Gastroenterology; Chulalongkorn University, Thailand
| | | | - Velimir A. C. Luketic
- Division of Gastroenterology, Hepatology and Nutrition; Virginia Commonwealth University School of Medicine
| | | | | | - Alex S. Befeler
- Division of Gastroenterology and Hepatology; Saint Louis University
| | - Denise Harnois
- Division of Gastroenterology and Hepatology; Mayo Clinic Jacksonville
| | | | - Jan Petz
- Division of Gastroenterology and Hepatology; Mayo Clinic Rochester
| | - Jill Keach
- Division of Gastroenterology and Hepatology; Mayo Clinic Rochester
| | - Jeff Schmoll
- Biostatistics and Health Sciences Research; Mayo Clinic Rochester
| | - Tanya Hoskin
- Biostatistics and Health Sciences Research; Mayo Clinic Rochester
| | - Prabin Thapa
- Biostatistics and Health Sciences Research; Mayo Clinic Rochester
| | - Felicity Enders
- Biostatistics and Health Sciences Research; Mayo Clinic Rochester
| | - Keith D. Lindor
- Division of Gastroenterology and Hepatology; Mayo Clinic Rochester
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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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Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51:660-78. [PMID: 20101749 DOI: 10.1002/hep.23294] [Citation(s) in RCA: 833] [Impact Index Per Article: 55.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
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Zein CO, Lindor KD. Latest and emerging therapies for primary biliary cirrhosis and primary sclerosing cholangitis. Curr Gastroenterol Rep 2010; 12:13-22. [PMID: 20425480 DOI: 10.1007/s11894-009-0079-2] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults. In PBC, therapy with ursodeoxycholic acid (UDCA) is safe and has been associated with tangible biochemical, histologic, and survival benefits. However, a need for different or adjuvant therapies remains for specific subsets of PBC patients, including those who do not respond to UDCA and those who have advanced histologic disease at presentation. Similarly, beneficial therapies for disease-related symptoms that do not typically respond to UDCA (eg, fatigue and pruritus) are still needed. In contrast to PBC, no medical therapy of proven benefit has been identified for patients with PSC. In PBC and PSC, adequate management of complications of chronic cholestasis is important. For both diseases, liver transplantation is the only curative option.
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Affiliation(s)
- Claudia O Zein
- Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA
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Sarangapani A, Shanmugam C, Kalyanasundaram M, Rangachari B, Thangavelu P, Subbarayan JK. Noninvasive prediction of large esophageal varices in chronic liver disease patients. Saudi J Gastroenterol 2010; 16:38-42. [PMID: 20065573 PMCID: PMC3023101 DOI: 10.4103/1319-3767.58767] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
BACKGROUND/AIM Esophageal varices (EVs) are a serious consequence of portal hypertension in patients with liver diseases. Several studies have evaluated possible noninvasive markers of EVs to reduce the number of unnecessary endoscopies in patients with cirrhosis but without varices. This prospective study was conducted to evaluate noninvasive predictors of large varices (LV). PATIENTS AND METHODS The study analyzed 106 patients with liver diseases from January 2007 to March 2008. Relevant clinical parameters assessed included Child-Pugh class, ascites and splenomegaly. Laboratory parameters like hemoglobin level, platelet count, prothrombin time, serum bilirubin, albumin and ultrasonographic characteristics like splenic size, splenic vein size, portal vein diameter were assessed. Univariate and multivariate analysis was done on the data for predictors of large EVs. RESULTS Incidence of large varices was seen in 41%. On multivariate analysis, independent predictors for the presence of LV were palpable spleen, low platelet count, spleen size> 13.8 mm, portal vein> 13 mm, splenic vein> 11.5 mm. The receiver operating characteristic (ROC) curve showed 0.883 area under curve. Platelet spleen diameter ratio 909 had a sensitivity and specificity of 88.5%, 83% respectively. CONCLUSION Thrombocytopenia, large spleen size, portal vein size and platelet spleen diameter ratio strongly predicts large number of EVs.
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Affiliation(s)
- Arulprakash Sarangapani
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India.
| | - Chitra Shanmugam
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India
| | - Muthukumaran Kalyanasundaram
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India
| | - Balamurali Rangachari
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India
| | - Pugazhendhi Thangavelu
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India
| | - Jeevan Kumar Subbarayan
- Department of Digestive Health and Diseases, Government Peripheral Hospital, Kilpauk Medical College, Chennai - 102, India
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Sgouros SN, Vasiliadis KV, Pereira SP. Systematic review: endoscopic and imaging-based techniques in the assessment of portal haemodynamics and the risk of variceal bleeding. Aliment Pharmacol Ther 2009; 30:965-76. [PMID: 19735231 DOI: 10.1111/j.1365-2036.2009.04135.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
BACKGROUND Invasive measurement of the hepatic venous pressure gradient (HVPG) is regarded as the gold standard for risk stratification and the evaluation of pharmaceutical agents in patients with portal hypertension. AIM To review the techniques for endoscopic and imaging-based assessment of portal haemodynamics, with particular emphasis on trials where the results were compared with HVPG or direct portal pressure measurement. METHODS Systematic search of the MEDLINE electronic database with keywords: portal hypertension, variceal bleeding, variceal pressure, endoscopic ultrasound, Doppler ultrasonography, magnetic resonance angiography, CT angiography, hepatic venous pressure gradient. RESULTS Computed tomography angiography and endoscopic ultrasound (EUS) have been both employed for the diagnosis of complications of portal hypertension and for the evaluation of the efficacy of endoscopic therapy. Colour Doppler ultrasonography and magnetic resonance angiography has given discrepant results. Endoscopic variceal pressure measurements either alone or combined with simultaneous EUS, correlate well with HVPG and risk of variceal bleeding and have a low interobserver variability. CONCLUSIONS Endoscopic and imaging-based measurements of portal haemodynamics provide an alternate means for the assessment of complications of portal hypertension. Further studies are required to validate their use in risk stratification and the evaluation of drug therapies in patients with portal hypertension.
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Affiliation(s)
- S N Sgouros
- Department of Gastroenterology, University College London Hospitals NHS Foundation Trust, UK
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