Results 131 to 140 of about 82,056 (314)

Liver disease in Shwachman‐Diamond syndrome: A comprehensive characterization across the age spectrum

Journal of Pediatric Gastroenterology and Nutrition, EarlyView.
Abstract Objectives Shwachman‐Diamond syndrome (SDS) is an inherited bone marrow failure disorder, and its hepatic phenotype is poorly defined. Our objective was to systematically characterize the prevalence, features, and outcomes of liver injury in a multicenter SDS cohort. Methods Retrospective registry study of 171 patients with biallelic Shwachman‐
Jane Koo, Diana Schwarz, Meghan Haney, Juan Putra, Jaeson Kim, Leah Cheng, Elizabeth Korn, Sarah Steltz, Lois Schwarz, Sara Loveless, Richard Cooper, Claire Dusa, Adam Lane, Kasiani C. Myers, Akiko Shimamura, Amit S. Grover, Andrew Wehrman   +16 more
wiley   +1 more source

Sentinel events in pediatric hepatology: A pilot simulation curriculum

JPGN Reports, EarlyView.
Abstract Objectives Sentinel hepatology events require robust prior experience to accurately diagnose and manage. Given the rarity of complex pediatric liver disease, gastroenterology (GI) fellows do not uniformly get exposure to these patients.
Anne Lyon, Alan Leichtner, Donna Luff, Scott Elisofon, Andrew Wehrman, Katherine Sweeny, Janet MacDonald, Christine K. Lee   +7 more
wiley   +1 more source

Efficacy of hepatic transplantation in patients with primary sclerosing cholangitis [PDF]

, 1993
Controlled trials to assess the therapeutic benefit of orthotopic hepatic transplantation (OHTx) for primary sclerosing cholangitis (PSC) cannot be justified in view of improvement of patient survival after this operation since 1981.
Abu-Elmagd, KM, Demetris, AJ, Dickson, ER, Fung, JJ, Krom, RAF, Langworthy, AL, Malinchoc, M, Murtaugh, PA, Starzl, TE, Van Thiel, DH   +9 more
core  

Diagnostic dilemma of cystic biliary atresia: A series of two cases and brief review of the diagnostic modalities

JPGN Reports, EarlyView.
Abstract Cystic biliary atresia (CBA) is a rare variant of biliary atresia that closely resembles choledochal cyst (CC), complicating diagnosis and potentially delaying critical surgical intervention. We report two cases of CBA that were difficult to diagnose.
Hamza Hassan Khan, Leslie Hirsig Spence, Nagraj Kasi   +2 more
wiley   +1 more source

Itching for a diagnosis: Dysesthesias as an atypical presentation of Wilson disease in an adolescent—Case report

JPGN Reports, EarlyView.
Abstract Wilson disease (WD) is an autosomal recessive disorder of hepatic copper metabolism with varied clinical presentations. We describe a 15‐year‐old male referred for elevated aminotransferases, burning facial pruritis, scalp dysesthesias, and chronic bilateral lower extremity edema.
Tierra L. R. Mosher, John Hicks, Krupa R. Mysore   +2 more
wiley   +1 more source

Contrast-enhanced MR cholangiography with Gd-EOB-DTPA in patients with liver cirrhosis: visualization of the biliary ducts in comparison with patients with normal liver parenchyma [PDF]

, 2018
The purpose of this study was to assess the quality of biliary duct visualization using Gd-EOB-DTPA-enhanced magnetic resonance cholangiography (EOB-MRC) in patients with liver cirrhosis. Forty adult patients with liver cirrhosis (cirrhosis group) and 20
Kakales, Irini, Marincek, Borut, Petrowsky, Henrik, Struwe, Anja, Tschirch, Frank, Weishaupt, Dominik   +5 more
core  

Delayed diagnosis of 3β‐HSD7 deficiency in adolescence: Two case reports and review of the literature

JPGN Reports, EarlyView.
ABSTRACT Congenital bile acid synthesis defects (BASD), the most common of which is 3β‐hydroxy‐Δ5‐C27‐steroid dehydrogenase oxidoreductase (3β‐HSD7) deficiency, are a rare cause of fat‐soluble vitamin malabsorption. We describe a 14‐year‐old girl who presented at 14 months with a left distal femur fracture and failure to thrive.
Samantha Pendleton, Mojdeh Mostafavi, Jamie Mathew, Kayla Ganzburg, Barry Hirsch, Nadia Ovchinsky, Wael Sayej   +6 more
wiley   +1 more source

Liver Stiffness Characterization of OGTLKO Mouse Model of Progressive Liver Fibrosis

Journal of Ultrasound in Medicine, EarlyView.
Objectives O‐GlcNAcylation plays a key regulatory role in hepatic physiology, and its disruption leads to fibrosis in liver‐specific OGT knockout mice (OGTLKO), making this model valuable for studying advanced metabolic dysfunction‐associated steatohepatitis (MASH).
Gilles Renault, Lenin Chinchilla, Lourdes Massengo, Pierre Monbernard, Fadila Benhamed, Marion Regnier, Raphael Denis, Catherine Postic, Anthony Novell, Jean‐Luc Gennisson   +9 more
wiley   +1 more source

A Study on the Characteristics of Sonazoid Contrast‐Enhanced Ultrasound in the Transitional Phase for Common Focal Liver Lesions and Its Value in Differential Diagnosis

Journal of Ultrasound in Medicine, EarlyView.
Objective To investigate the value of transitional phase characteristics on Sonazoid contrast‐enhanced ultrasound (S‐CEUS) in the differential diagnosis of common focal liver lesions (FLLs). Materials and Methods This prospective multicenter study enrolled 122 participants with FLLs from 8 medical centers between December 2023 and March 2025. The study
Yunhao Luo, Danlin Wen, Jing Zhang, Zhengrong Song, Yang Liu, Yan Liu, Qiang Mao, Yunquan Zhou, Liwen Ma, Congcong Yu, Lang Qiao   +10 more
wiley   +1 more source

Phase IIb Trial for the Palliative Treatment of Patients With Primary Hepatic Malignancy Unable to Receive Curative Treatment: Efficacy of Colchicine

The Kaohsiung Journal of Medical Sciences, EarlyView.
ABSTRACT This trial was to evaluate the efficacy and the safety of colchicine for the palliative treatment of patients with primary hepatic malignancy unable to receive curative treatment. Forty hepatocellular carcinoma (HCC) patients and two intrahepatic cholangiocarcinoma (ICC) patients signed the informed consents.
Zu‐Yau Lin, Ming‐Lun Yeh, Po‐Cheng Liang, Shinn‐Cherng Chen, Chung‐Feng Huang, Jee‐Fu Huang, Chia‐Yen Dai, Ming‐Lung Yu, Wan‐Long Chuang   +8 more
wiley   +1 more source