Results 61 to 70 of about 15,869 (231)

Interferon‐gamma blocking as a promising treatment for severe liver dysfunction in secondary hemophagocytic lymphohistiocytosis after liver transplantation

JPGN Reports, EarlyView.
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a life‐threatening hyperinflammatory syndrome that can occur after solid organ transplantation but remains underrecognized in this setting. The diagnosis is often delayed due to overlapping clinical manifestations with infection, rejection, or malignancy, and management becomes particularly ...
Chen Chen, Tao Zhou, Jing Jin, Yi Luo, Lijing Shen, Qiang Xia, Yongbing Qian   +6 more
wiley   +1 more source

Expanding the genotypic spectrum of PCSK1 deficiency: A novel mutation in severe neonatal diarrhea

JPGN Reports, EarlyView.
Abstract Among congenital diarrhea and enteropathies (CODEs), proprotein convertase subtilisin/kexin type 1 (PCSK1) deficiency is a rare monogenic disorder, associated with severe neonatal diarrhea and polyendocrinopathies. We report an 18‐day‐old male neonate, born to consanguineous parents, presenting with persistent watery diarrhea, metabolic ...
Eleonora Saraceno, Angelo Corso Faini, Silvia Deaglio, Marianna Pellegrini, Raffaele Buganza, Francesca Giuliani, Pier Luigi Calvo, Michele Pinon   +7 more
wiley   +1 more source

Physical development and cognitive performance in a monozygotic twins for biliary atresia: Report of a case and literature reviewing

Journal of Pediatric Surgery Case Reports, 2016
A case of monozygotic twins, one of whom suffered from biliary atresia and the other one was apparently healthy, is described herein. Children with biliary atresia appear to be vulnerable to developmental lags in the physical and cognitive performance ...
Yang Chen, Jianghua Zhan
doaj   +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

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

A Simplified Risk Assessment Tool to Predict Post Deceased Donor Liver Transplantation Outcomes: A Single, Highly Experienced Medical Center in Taiwan

The Kaohsiung Journal of Medical Sciences, EarlyView.
ABSTRACT Liver transplantation (LT) is the standard treatment for end‐stage liver disease, yet the gap between the demand for organs and their availability is widening. In Taiwan, the scarcity of deceased donor organs highlights the need for optimized utilization strategies.
Jie‐Lan Jhang, Hao‐Chien Hung, Yin Lai, Jin‐Chiao Lee, Yu‐Chao Wang, Chih‐Hsien Cheng, Tsung‐Han Wu, Chen‐Fang Lee, Ting‐Jung Wu, Hong‐Shiue Chou, Kun‐Ming Chan, Wei‐Chen Lee   +11 more
wiley   +1 more source

Human Cyclophilins—An Emerging Class of Drug Targets

Medicinal Research Reviews, EarlyView.
ABSTRACT Cyclophilins are a family of enzymes with peptidyl‐prolyl isomerase activity found in all cells of all organisms. To date, 17 cyclophilin isoforms have been identified in the human body, participating in diverse biological processes. Consequently, cyclophilins have emerged as promising targets for drug development to address a wide array of ...
Katarina Jurkova, Hana Navratilova, Kamil Musilek, Ondrej Benek   +3 more
wiley   +1 more source

Epidemiology of hypospadias in China: A nationwide surveillance‐based study, 2010–2020

Andrology, EarlyView.
Abstract Background The prevalence of hypospadias varied internationally. However, epidemiological data on hypospadias in contemporary China remain limited. Objectives We aim to examine the epidemiological characteristics of hypospadias in Chinese population.
Chen Zhiyu, Gao Yuyang, Xu Wenli, Li Wenyan, Liu Zhen, Zhou Jiayuan, Zhu Jun, Dai Li   +7 more
wiley   +1 more source

Intestinal Atresia in Finland: Maternal Risk Factors, Prevalence, Associated Anomalies and Survival

Acta Paediatrica, EarlyView.
ABSTRACT Aim We aimed to investigate prevalence, associated anomalies and survival of congenital intestinal atresia and to examine maternal risk factors for jejunoileal atresia (JIA). Methods All children born with, or pregnancies terminated because of, JIA or colonic atresia (CA) in Finland during 1987–2019 were identified from the Finnish Register of
Esko Tahkola, Arimatias Raitio, Ilkka Helenius, Johanna Syvänen, Teemu Kemppainen, Eliisa Löyttyniemi, Maarit K. Leinonen, Mika Gissler, Topi Luoto, Mikko P. Pakarinen   +9 more
wiley   +1 more source

Moderate increase of MET in hepatocytes protects against cholestatic liver injury by promoting an effective antioxidant response

The FEBS Journal, EarlyView.
Moderate overexpression of wild‐type Met in hepatocytes (Alb‐R26Met mouse model) boosts a strong antioxidant response dependent on the glutathione system while impairing TGF‐β signaling in the liver. This leads to improved liver regeneration and protects against DDC‐induced injury, a model for cholestatic disease sharing features with primary ...
Carlos González‐Corralejo, Juan García‐Sáez, María Figueroa‐Fuentes, Annalisa Addante, Cesáreo Roncero, Nerea Lazcanoiturburu, Beatriz Pacheco, Julián Sanz, Cristina Gato, Eva Fernández‐Calderón, María de la O López, Águeda González‐Rodríguez, Almudena Porras, Flavio Maina, Isabel Fabregat, Blanca Herrera, Aránzazu Sánchez   +16 more
wiley   +1 more source