Results 41 to 50 of about 89,189 (328)

∆4-3-oxo-5β-reductase deficiency: favorable outcome in 16 patients treated with cholic acid

Orphanet Journal of Rare Diseases, 2023
Background Oral cholic acid therapy is an effective therapy in children with primary bile acid synthesis deficiencies. Most reported patients with this treatment have 3β-hydroxy-Δ5-C27-steroid oxidoreductase deficiency.
Antoine Gardin, Mathias Ruiz, Jan Beime, Mara Cananzi, Margarete Rathert, Barbara Rohmer, Enke Grabhorn, Marion Almes, Veena Logarajah, Luis Peña-Quintana, Thomas Casswall, Amaria Darmellah-Remil, Ana Reyes-Domínguez, Emna Barkaoui, Loreto Hierro, Carolina Baquero-Montoya, Ulrich Baumann, Björn Fischler, Emmanuel Gonzales, Anne Davit-Spraul, Sophie Laplanche, Emmanuel Jacquemin   +21 more
doaj   +1 more source

Changes in activity of metalloproteinases 2 and 9 in rat liver in experimental model of cholestasis taking into account its duration and assessment of selected features

Annales Academiae Medicae Silesiensis, 2023
INTRODUCTION: Cholestasis, otherwise known as bile stasis, occurs when the flow of bile is blocked. There are currently two types of cholestasis: extrahepatic and intrahepatic.
Krzysztof Suszyński, Natalia Białoń, Dariusz Górka, Anna Dolińska, Mikołaj Górka   +4 more
doaj   +1 more source

Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2

ACG Case Reports Journal, 2020
ABSTRACT Benign recurrent intrahepatic cholestasis represents a rare class of autosomal recessive chronic cholestasis disorders, usually presenting with recurrent episodes of intense pruritus and jaundice. We report a 27-year-old woman presenting with benign recurrent intrahepatic cholestasis type 2 due to heterozygosity in ABCB11 ...
Arthur Lorio, Eric, Valadez, David, Alkhouri, Naim, Loo, Nicole   +3 more
openaire   +2 more sources

Urinary porphyrin excretion in hepatitis C infection [PDF]

, 1999
A high prevalence of hepatitis C virus infection in porphyria cutanea tarda in some populations suggests a close link between viral hepatitis and alteration of porphyrin metabolism.
Jacob, Karl, Vogeser, Michael, Zachoval, Reinhart   +2 more
core   +1 more source

DIFFERENCES OF BIRTH WEIGHT AND ONSET OF ACHOLIC STOOL BETWEEN EXTRAHEPATIC AND INTRAHEPATIC CHOLESTASIS

Indonesian Midwifery and Health Sciences Journal
Background: Biliary atresia (extrahepatic cholestasis) and neonatal hepatitis (intrahepatic cholestasis) are two main causes of cholestasis. It is important to distinguish the type of cholestasis for determine management.
Bagus Setyoboedi, Lasmauli Situmorang, Rendi Aji Prihaningtyas, Sjamsul Arief   +3 more
doaj   +1 more source

The impact of elevation of total bilirubin level and etiology of the liver disease on serum N-glycosylation patterns in mice and men [PDF]

, 2010
The GlycoFibroTest and GlycoCirrhoTest are noninvasive alternatives for liver biopsy that can be used as a follow-up tool for fibrosis patients and to diagnose cirrhotic patients, respectively.
BLOMME, BRAM, Callewaert, Nico, Colle, Isabelle, Van Steenkiste, Christophe, Van Vlierberghe, Hans, Vanhuysse, Jacques   +5 more
core   +1 more source

Severe drug-induced liver injury associated with prolonged use of Linezolid [PDF]

, 2010
This study aims to describe a patient developing concomitant severe liver failure and lactic acidosis after long-term treatment with linezolid. A 55-year-old Caucasian woman developed concomitant severe liver failure and lactic acidosis after a treatment
Benoit, Dominique, De Bus, Liesbet, Depuydt, Pieter, Libbrecht, Louis, NOLLET, JOKE, Van Vlierberghe, Hans, Vandekerckhove, Linos, Vogelaers, Dirk   +7 more
core   +1 more source

New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications

Canadian Journal of Gastroenterology and Hepatology, 2018
Cholestasis is characterised by impaired bile secretion and accumulation of bile salts in the organism. Hereditary cholestasis is a heterogeneous group of rare autosomal recessive liver disorders, which are characterised by intrahepatic cholestasis ...
Eva Sticova, Milan Jirsa, Joanna Pawłowska   +2 more
doaj   +1 more source

Cholestasis Reveals Severe Cortisol Deficiency in Neonatal Pituitary Stalk Interruption Syndrome. [PDF]

PLoS ONE, 2016
Cholestasis has been reported during the course of congenital hypothalamic-pituitary deficiency, but crucial information is lacking regarding both its origin and prognosis. We aimed to characterize the course of cholestasis and factors contributing to it
Francois-Xavier Mauvais, Emmanuel Gonzales, Anne Davit-Spraul, Emmanuel Jacquemin, Raja Brauner   +4 more
doaj   +1 more source

Dolphin Morbillivirus Associated with a Mass Stranding of Sperm Whales, Italy [PDF]

, 2017
In September 2014, 7 sperm whales stranded along the Adriatic Italian coastlines. Postmortem investigations on 3 dead females dead and in 1 fetus harbored by the largest one revealed molecular and immunoistochemical evidences of dolphin morbillivirus ...
Cardeti, Giusy, Casalone, Cristina, CENTELLEGHE, CINZIA, Cersini, Antonella, Di Guardo, Giovanni, Fichi, Gianluca, Francesco, Cristina Esmeralda Di, MAZZARIOL, SANDRO, Mignone, Walter, Petrella, Antonio, Provvido, Andrea Di, Renzo, Ludovica Di   +11 more
core   +2 more sources