Dataset Information

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

ABSTRACT: Our patient with primary biliary cholangitis, previously termed as primary biliary cirrhosis, presented with an unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices; they should be considered in the differential in the etiology of hematemesis in primary biliary cholangitis and thoroughly searched for during an endoscopic procedure for early treatment and subsequent secondary prevention.

SUBMITTER: Kolli S

PROVIDER: S-EPMC6188036 | biostudies-other | 2018 Oct

REPOSITORIES: biostudies-other

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Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

Kolli Sindhura S Mori Amit A Moshenyat Isaac I

Gastroenterology research 20181001 5

Our patient with primary biliary cholangitis, previously termed as primary biliary cirrhosis, presented with an unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices; they should be considered in the differential in the etiology of hematemesis in primary biliary cholangitis and thoroughly searched for during an endoscopic procedu ...[more]

PMID: 30344813

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Project description:Esophageal variceal bleeding is a severe complication of portal hypertension with significant morbidity and mortality. Although traditional screening and grading of esophageal varices has been performed by endogastroduodenoscopy (EGD), wireless video capsule endoscopy provides a minimally invasive alternative that may improve screening and surveillance compliance.The aim of the study was to perform a systematic review and structured meta-analysis of all eligible studies to evaluate the efficacy of wireless capsule endoscopy for screening and diagnosis of esophageal varices among patients with portal hypertension.Searches of PubMed, EMBASE, Web of Science, and the Cochrane Library databases were performed through December 2015. Bivariate and hierarchical models were used to compute the pooled sensitivity and specificity, and to plot the summary receiver operating characteristics curve with summary point and corresponding 95% confidence region. Bias of included studies was assessed using the quality assessment of diagnostic accuracy studies-2.Seventeen studies from 2005 to 2015 were included in this meta-analysis (n=1328). The diagnostic accuracy of wireless capsule endoscopy in the diagnosis of esophageal varices was 90% [95% confidence interval (CI), 0.88-0.93]. The diagnostic pooled sensitivity and specificity were 83% (95% CI, 0.76-0.89) and 85% (95% CI, 0.75-0.91), respectively. The diagnostic accuracy of wireless capsule endoscopy for the grading of medium to large varices was 92% (95% CI, 0.90-0.94). The pooled sensitivity and specificity were 72% (95% CI, 0.54-0.85) and 91% (95% CI, 0.86-0.94), respectively, for the grading of medium to large varices. The use of capsule demonstrated only mild adverse events. A sensitivity analysis limited to only high quality studies revealed similar results.Wireless esophageal capsule endoscopy is well tolerated and safe in patients with liver cirrhosis and suspicion of portal hypertension. The sensitivity of capsule endoscopy is not currently sufficient to replace EGD as a first exploration in these patients, but given its high accuracy, it may have a role in cases of refusal or contraindication to EGD.

Project description:We investigated noninvasive follow-up markers for histologic liver fibrosis and portal hypertension (PH) in patients with biliary atresia after successful portoenterostomy (PE). Among children with bilirubin <20 µmol/L after PE (n = 39), Metavir fibrosis stage was evaluated at PE and in follow-up protocol liver biopsies (n = 83). PH was defined as endoscopically confirmed esophageal varices or thrombocytopenia associated with splenomegaly. The accuracy of liver biochemistry and stiffness in detecting liver fibrosis and PH was analyzed by the area under the receiving operating characteristic curve (AUROC) and multiple regression models. During a median native liver survival of 8.3 years (interquartile range 2.5-10.8 years), cirrhosis (Metavir F4) had developed in 51% of patients and PH in 54% of patients. Cirrhosis was equally common in all age tertiles of 1.2-2.1 years (n = 10/27), 3.9-5.8 years (n = 12/28), and 9.0-14 years (n = 12/28). In the two oldest age tertiles, histologic liver fibrosis had progressed further in patients with PH than without PH (P < 0.001). PH was accurately predicted by the aspartate aminotransferase-to-platelet ratio index (APRI) (cutoff, 0.70; AUROC, 0.92), bile acids (cutoff, 49 µmol/L; AUROC, 0.91), and liver stiffness (cutoff, 16.9 kPa; AUROC, 0.89; P < 0.001 each) across all age tertiles. Liver stiffness was the most accurate predictor of cirrhosis overall (AUROC, 0.82; P < 0.001), whereas bilirubin was >11 µmol/L in the youngest tertile (AUROC, 0.91; P < 0.001), bile acids was >80 µmol/L in the middle tertile (AUROC, 0.81; P = 0.009), and liver stiffness was >24 kPa in the oldest age tertile (AUROC, 0.96; P = 0.002). Conclusion: After successful PE, development of PH associates with progression of liver fibrosis and can be accurately detected by APRI and stiffness. Liver stiffness most accurately identified cirrhosis in older children, whereas biochemical markers of cholestasis closely reflected histologic cirrhosis in younger children.

Dataset Information

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

Publications

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

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