Results 31 to 40 of about 24,959 (186)

Congenital hepatic fibrosis in Holando Argentino calf: first report in Argentina

Brazilian Journal of Veterinary Pathology, 2019
A case of congenital hepatic fibrosis in a 10-month-old calf is described. The calf had been raised in a feed lot for the past 173 days and exhibited loss of body condition and depression, followed by death.
Mirentxu Indart, María G. de Yaniz, Jorge P. García, Ángel R. Bence, Marcial S. Negrette   +4 more
doaj   +1 more source

Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report

Journal of International Medical Research, 2020
Caroli’s disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts.
Song Wang, Mei Xiao, Liqun Hua, Yong Jia, Si Chen, Kaiguang Zhang   +5 more
doaj   +1 more source

Recurrent Cholangitis with Congenital Hepatic Fibrosis and Pancreaticobiliary Maljunction after Roux-en-Y Reconstruction

European Journal of Pediatric Surgery Reports, 2013
A 1-year-old girl had pancreaticobiliary maljunction, a choledochal cyst, and polycystic kidney. At the age of 4 years, she was treated by resection of the choledochal cyst and Roux-en-Y reconstruction because of the cyst's risk of cancer.
Tokio Sugiura, Takeshi Endo, Koichi Ito, Kenji Goto, Yoko Sato, Satoshi Kondo, Tatsuya Suzuki, Takashi Hashimoto   +7 more
doaj   +1 more source

SPSB1 Promotes Subcutaneous Adipose Hyperplasia in Facial Port‐Wine Stains by Controlling HDAC1 Degradation and Stability Through Two Distinct Proteolytic Pathways

Advanced Science, EarlyView.
In PWS‐ASPCs, FOSL1 drives the expression of SPSB1. SPSB1, as part of the ESC complex, further binds to HDAC1 and promotes K29‐linked and K48‐linked polyubiquitination of HDAC1. These modifications facilitate the degradation of HDAC1 through the ALP and UPS pathways, respectively.
Hongrui Chen, Bin Sun, Yajing Qiu, Chen Hua, Xiaoxi Lin, Wei Gao   +5 more
wiley   +1 more source

Congenital Hepatic Fibrosis as a Cause of Recurrent Cholangitis: A Case Report and Review of the Literature

Livers, 2021
Rare liver diseases caused by ductal plate malformation, such as congenital hepatic fibrosis (CHF), Caroli syndrome, and polycystic liver disease, can have clinical manifestations such as recurrent cholangitis—frequently involving multidrug-resistant ...
Emanuele Palomba, Marco Maggioni, Giulia Viero, Davide Mangioni, Rosa Lombardi, Barbara Antonelli, Daniele Dondossola, Massimo Iavarone, Anna Ludovica Fracanzani, Alessandra Bandera, Andrea Gori, Andrea Lombardi   +11 more
doaj   +1 more source

Intrasplenic Thymus Organogenesis from Injectable Tissue Fragments Restores Functional T‐Cell Immunity

Advanced Science, EarlyView.
Clinical intramuscular thymus transplantation yields only short‐lived efficacy and marginal therapeutic benefits. Benefiting from the spleen's intrinsic strengths—rapid vascular perfusion, abundant developmental factors, and resident progenitors—the intrasplenic thymic grafts achieve robust thymic regeneration and substantial T‐cell reconstitution ...
Shaocong Wang, Zijian Zhang, Fulin Dai, Zhijie Yin, Zhen Xing, Yurong Li, Haochong Lei, Junfeng Zhang, Chunyan Liu, Yan Li, Lei Dong   +10 more
wiley   +1 more source

Mitochondria‐Targeted Nanotherapeutics: A Promising Strategy in Modulating Mitochondrial Function, Transfer, and Transplantation

Advanced Science, EarlyView.
This review summarizes the pathogenic role of mitochondria in diseases and highlights mitochondrial transfer and transplantation as emerging therapeutic strategies. It systematically discusses how nanomaterials are engineered to facilitate these processes, and critically examines the current challenges and future perspectives for their clinical ...
Yuanyuan Su, Zhiyan Gong, Jiao Mu, Lili Huang, Pengfei Yang, Chenli Zhang, Lingshan Zhao, Liling Yang, Jun Deng, Xiaohui Liao   +9 more
wiley   +1 more source

An uncommon cause of portal hypertension in a toddler

International Journal of Advanced Medical and Health Research, 2017
Autosomal recessive polycystic kidney disease (ARPKD) affects the liver and the kidney. Renal involvement presents early in life, whereas hepatic involvement manifests slightly later with portal hypertension.
Sudipta Mohakud, Saraswata Das, Pradeep Sharma, Lipsa Das, Suprava Naik, N Deep   +5 more
doaj   +1 more source

Congenital hepatic fibrosis as a rare cause of non-cirrhotic portal hypertension

Annals of Hepatology, 2022
Introduction and Objective: a case of congenital hepatic fibrosis is presented Case report: A 19-year-old female with a medical story relevant only to epistaxis of 3 years long was referred to the liver clinic because of thrombocytopenia and ...
MO Santana Montes, VM Páez Zayas, MF Higuera De la Tijera, JL Pérez-Hernández, LG Ruíz-Fabian, D Montante-Montes de Oca, VH Méndez Cano   +6 more
doaj   +1 more source

Expanding the Phenotypic Spectrum of TXNDC15‐Related Ciliopathies to Include Joubert Syndrome

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Biallelic loss‐of‐function variants in TXNDC15 are a known cause of the perinatally lethal ciliopathy Meckel syndrome (MKS). TXNDC15 encodes an endoplasmic reticulum (ER)‐resident thioredoxin‐domain protein required for ciliary transition zone integrity.
Zachary T. Sentell, Robert Geraghty, Juliana E. Arcila‐Galvis, Praveen Dhondurao‐Sudhindar, Sergio Foullerat Cañada, Fulvio D'Abrusco, Bosanka Jocic‐Jakubi, Miguel Barroso‐Gil, Valentina Serpieri, Thomas M. Kitzler, Kate E. Chandler, Eleanor G. Seaby, Gabrielle Wheway, Jennifer C. Dempsey, Dan Doherty, Enza M. Valente, John A. Sayer   +16 more
wiley   +1 more source