Results 61 to 70 of about 9,482 (191)

Preclinical Efficacy and Safety Evaluation of a Novel Controlled‐Expansion ePTFE‐Covered TIPS Stent in a Swine Model

Portal Hypertension &Cirrhosis, EarlyView.
This preclinical study evaluated a novel controlled‐expansion expanded polytetrafluoroethylene (ePTFE)‐covered transjugular intrahepatic portosystemic shunt (TIPS) stent in 11 swine, demonstrating 100% technical success, excellent deployability, and durable 6‐month angiographic patency without thrombosis, migration, fracture, or device‐related toxicity.
Yi Xiang, Qi Zhang, Chuan Liu, Jiawei Zhang, Qingqiao Zhang, Changsheng Shi, Chunze Zhou, Yudong Zhang, Hongliang He, Hao Xie, Hui Shi, Haijian Guo, Wenjing Liu, Muqing Lin, Huajie Yu, Xiaolong Qi   +15 more
wiley   +1 more source

Gene Therapy for Treatment of Chronic Hyperammonemia in a Rat Model of Hepatic Encephalopathy

Annals of Hepatology, 2018
Introduction and aim. Hepatic encephalopathy (HE), caused by hyperammonemia resulting from liver disease, is a spectrum of neuropsychiatric and motor disorders that can lead to death. Existing therapies are deficient and alternative treatments are needed.
Plácido Espíritu-Ramírez, Nancy Y. Ortega-Balderas, Laura Sevilla-Tapia, Ana G. Montiel-Martínez, Ana R. Pastor-Flores, Laura A. Palomares, Miguel A. Torres-Vega   +6 more
doaj   +1 more source

Asparaginase-associated hyperammonemia [PDF]

Haematologica
Asparaginase is an essential drug in the treatment of acute lymphoblastic leukemia, and discontinuation of asparaginase therapy due to clinical toxicity or silent inactivation may lead to reduced event-free survival. Common toxicities include hypersensitivity reactions, acute pancreatitis, thrombosis, hepatotoxicity, and hyperlipidemia.
Raja, Raheel Altaf, Als-Nielsen, Bodil, Lund, Allan M., Vilstrup, Hendrik, Dalhoff, Kim Peder, Schmiegelow, Kjeld   +5 more
openaire   +4 more sources

Consensus definition for developmental regression during childhood

Developmental Medicine &Child Neurology, EarlyView.
In this study, 41 expert interdisciplinary clinicians participated in two rounds of a Delphi survey to reach a consensus agreement on the developmental domains and duration of regression. Reaching an agreed working definition is a crucial first step towards the earlier and consistent identification of children experiencing developmental regression ...
Gauravi Gawade, Ellaina P Andersson, Ava Nielsen, Katrina Williams, Kirsten Furley   +4 more
wiley   +1 more source

Carglumic acid as a treatment for persistent hyperammonemia in carnitine-acylcarnitine translocase deficiency: A case study

Molecular Genetics and Metabolism Reports
Carnitine-acylcarnitine translocase deficiency (CACTD) is a rare autosomal recessive fatty acid oxidation disorder resulting in energy deficiency due to impaired mitochondrial long-chain fatty acid transport.
Hanım Babazade, Tanyel Zubarioglu, Esma Uygur, Mehmet Şerif Cansever, Ertuğrul Kiykim, Çiğdem Aktuğlu Zeybek   +5 more
doaj   +1 more source

Hyperammonemia in azotemic cats

Journal of Feline Medicine and Surgery, 2020
Objectives Hyperammonemia occurs in cats with hepatobiliary and nutritional (cobalamin and arginine deficiency) disorders, and has also been documented in four cats with renal azotemia. We hypothesized that in cats with renal azotemia, fasting hyperammonemia would correlate with indices of worsening kidney ...
Lauren Carvalho, Denise Kelley, Mary Anna Labato, Cynthia RL Webster   +3 more
openaire   +2 more sources

Hypermanganesemia in Children With Liver and Portosystemic Circulation Disorders: A Systematic Review

Journal of Paediatrics and Child Health, EarlyView.
ABSTRACT Aim Children with chronic liver disease and portosystemic circulation disorders may have long‐term neurocognitive problems. Hypermanganesemia, reported in this group, could be a contributor. This systematic review aimed to characterise liver and portosystemic circulation disorders associated with hypermanganesemia and reported neurocognitive ...
Helena J. Kim, Susan M. Siew, Ji Hyun Moon, Tracey A. Williams, S. Murthy Chennapragada, Gordon Thomas, Shekeeb S. Mohammad   +6 more
wiley   +1 more source

Response of an Infant With Presumed Multiple Acyl‐CoA Dehydrogenase Deficiency (MADD) to Ketone Supplementation

American Journal of Medical Genetics Part A, Volume 200, Issue 9, Page 2140-2150, September 2026.
ABSTRACT Multiple Acyl‐CoA Dehydrogenase Deficiency (MADD) is an autosomal recessive inborn error of metabolism caused by biallelic pathogenic variants in one of three known genes: ETFA, ETFB, and ETFDH. It can cause multisystem dysfunction, including cardiomyopathy in severe cases.
Yutaka Furuta, Kaitlyn N. Bloom, Jerry Vockley, Angela R. Grochowsky, Neena S. Agrawal, Ellen W. Strickler, Natalie N. Owen, Erica T. Gray, B. Lakshitha A. Perera, Eric R. Gamazon, Lynette C. Rives, Hua‐Chang Chen, Qi Liu, Rizwan Hamid, Joy D. Cogan, John A. Phillips III, Thomas A. Cassini, Bryce A. Schuler   +17 more
wiley   +1 more source

Hyperammonemia Post Lung Transplantation: A Review

Clinical Medicine Insights: Circulatory, Respiratory and Pulmonary Medicine, 2020
Hyperammonemia is the pathological accumulation of ammonia in the blood, which can occur in many different clinical settings. Most commonly in adults, hyperammonemia occurs secondary to hepatic dysfunction; however, it is also known to be associated with
Robert F Leger, Matthew S Silverman, Ellen S Hauck, Ksenia D Guvakova   +3 more
doaj   +1 more source

From Common Pathway to Divergent Diseases: Metabolic Aspects of Inborn Errors of CoA Biosynthesis

Journal of Inherited Metabolic Disease, Volume 49, Issue 5, September 2026.
ABSTRACT Coenzyme A (CoA) biosynthesis is a conserved, dynamically regulated pathway essential for mitochondrial energy production, fatty acid oxidation, lipid biosynthesis and protein acylation. Biallelic variants in PANK2, PPCS, PPCDC, and COASY cause rare inborn errors of CoA biosynthesis, associated with markedly different clinical phenotypes ...
Ivano Di Meo, Yair Anikster, Valeria Tiranti, Arcangela Iuso   +3 more
wiley   +1 more source