Results 11 to 20 of about 24,959 (186)
Multi-omics analysis of host-microbiome interactions in a mouse model of congenital hepatic fibrosis [PDF]
BMC MicrobiologyBackground Congenital hepatic fibrosis (CHF) caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene is a rare genetic disorder with poorly understood pathogenesis.
Mengfan Jiao +8 more
doaj +2 more sources
Hepatic abscess associated with ductal plate malformation in a dog [PDF]
BMC Veterinary ResearchBackground Hepatic abscesses are an uncommon but potentially life-threatening condition in dogs. Various predisposing factors have been reported, including biliary tract disease, systemic infection, endocrine disorders, and congenital hepatic ...
Won-Joung Jaung +3 more
doaj +2 more sources
Congenital hepatic fibrosis in the Franches-Montagnes horse is associated with the polycystic kidney and hepatic disease 1 (PKHD1) gene. [PDF]
PLoS ONE, 2014 Congenital hepatic fibrosis has been described as a lethal disease with monogenic autosomal recessive inheritance in the Swiss Franches-Montagnes horse breed.
Michaela Drögemüller +15 more
doaj +1 more source
Hepatology, EarlyView., 2022 Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid +20 more
wiley +1 more source
Hepatology, EarlyView., 2022 A deleterious variant of FCHSD1 results in mTOR pathway overactivation and may cause porto‐sinusoidal vascular disorder (PSVD). The pedigree of the family demonstrated an autosomal dominant disease with variable expressivity. Whole‐genome sequencing and Sanger sequencing both validated the existence of the FCHSD1 variant and the heterozygosity of c ...
Jingxuan Shan +19 more
wiley +1 more source
Congenital hepatic fibrosis (a case report)
Zaporožskij Medicinskij Žurnal, 2021 Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder of intrauterine morphogenesis of the portobiliary system, characterized by periportal fibrosis and defective remodeling of the bile ducts.
O. O. Popovych +4 more
doaj +1 more source
First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan
Трансплантология (Москва), 2017 Congenital hepatic fibrosis is a rare autosomal recessive disease. Only a few such cases have been described worldwide, but the exact incidence of the disease is unknown.
R. A. Mamedov, N. Yu. Bayramov
doaj +1 more source
Congenital hepatic fibrosis leading to cirrhosis and hepatocellular carcinoma: a case report
Journal of Medical Case Reports, 2011 Introduction Congenital hepatic fibrosis is an uncommon cause of portal hypertension. Despite the presence of portal hypertension, hepatocellular and renal function are usually well preserved.
Bagheri Mohammad +2 more
doaj +1 more source
Congenital hepatic fibrosis associated with ascites in a nellore calf [PDF]
Ciência Rural, 2020 : Congenital hepatic fibrosis (CHF) is an autosomal recessive malformation characterized by a variable degree of fibrosis and bile duct proliferation, mainly described in people and rarely reported in bovine European breeds. In addition to liver fibrosis,
Daniela Dantas de Gois +7 more
doaj +1 more source
Congenital hepatic fibrosis in a child with Prader-Willi syndrome: a novel association
Annals of Saudi Medicine, 2014 Prader–Willi syndrome (PWS) is a rare genetic disorder caused by deletion or unexpression of the chromosome 15 (q 11-13). Symptomatologies include hypotonia, hyperphagia, cognitive impairment, and characteristic dysmorphic profile.
Ahmed Al Sarkhy +4 more
doaj +1 more source