Results 71 to 80 of about 102,708 (355)

Autosomal Dominant Erythrocytosis Caused by Non‐Renal Erythropoietin (EPO) Due to EPO c.‐136 G>A Germline Mutation

American Journal of Hematology, EarlyView.
A novel erythropoietin (EPO) promoter mutation (c.‐136 G>A) causes autosomal dominant erythrocytosis via non‐renal expression of EPO. ABSTRACT We previously reported a five‐generation kindred with autosomal dominant erythrocytosis associated with a novel germline promoter variant in the erythropoietin (EPO) gene (EPO c.‐136 G>A).
Lucie Lanikova, Dusan Hrckulak, Veronika Zimolova, Felipe R. Lorenzo, Jihyun Song, Katerina Vecerkova, Olga Babosova, Linda Berkova, Vladimir Korinek, Steve Elliott, Josef T. Prchal   +10 more
wiley   +1 more source

Magnetic resonance cholangiopancreatography (MRCP) using new negative per-oral contrast agent based on superparamagnetic iron oxide nanoparticles for extrahepatic biliary duct visualization in liver cirrhosis

Biomedical Papers, 2016
Background and Aims: Magnetic resonance cholangiopancreatography (MRCP) is often used for imaging of the biliary tree and is required by surgeons before liver transplantation.
Katerina Polakova, Ingrid Mocikova, Dana Purova, Pavel Tucek, Pavel Novak, Katerina Novotna, Izak Niko, Radoslav Bielik, Radek Zboril, Miroslav Herman   +9 more
doaj   +1 more source

Cyclosporine Absorption Following Orthotopic Liver Transplantation [PDF]

, 1986
Blood concentrations of cyclosporine were determined in adult and pediatric patients following orthotopic liver transplantation to quantitate cyclosporine blood clearance and oral absorption.
Beveridge, Burckart, Burckart, Calne, Calne, Holt, Kahan, Kennedy, Malatack, Miles, Ptachcinski, Ptachcinski, Ptachcinski, Sawchuk, Starzl, Starzl, Starzl, Venkataramanan, Venkataramanan, Venkataramanan, Wood   +20 more
core   +2 more sources

Variants in AKR1D1 and Infant Mortality: Should Bile Acid Screening be a Routine Part of Newborn Screening?

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Biallelic pathogenic variants in AKR1D1 cause Δ4‐3‐oxosteroid 5β‐reductase deficiency, disrupt bile acid synthesis, and result in Congenital Bile Acid Synthesis defect type 2 (CBAS2). CBAS2 presents in infancy with cholestasis, coagulopathy, and failure to thrive.
Jade Hudson, Stephanie Hyunh, Bojana Rakic, Cornelius Boerkoel   +3 more
wiley   +1 more source

Evaluation of epidemiologic and demographic aspects of primary biliary cirrhosis in Isfahan population from 2009 - 2017

Majallah-i Dānishgāh-i ’Ulūm-i Pizishkī-i Shahīd Ṣadūqī Yazd, 2018
Introdution: Primary biliary cirrhosis is a chronic cholestatic disease of the liver, characterized by the inflammation plus progressive and non-purulent destruction of the small biliary ducts, and the presence of auto antibodies against mitochondrial ...
babak tamizi far, sayed mohamad javad sajadi, vahid sebghatollahi   +2 more
doaj  

Colangiopatia auto-imune.

Acta Médica Portuguesa, 2001
We report two patients with mixed characteristics of primary biliary cirrhosis and autoimmune hepatitis. They are two female patients in their fifties (54 and 58 years-old) one of them with autoimmune phenomenon, rheumatoid arthritis and Sjögren syndrome.
R Marinho, H Graça, F Ramalho, A Costa, A Batista, M C De Moura   +5 more
doaj   +1 more source

Characterization of inflammatory pseudotumors in a large animal model of liver cancer

Animal Models and Experimental Medicine, EarlyView.
CT images in arterial and portal venous phases of liver lesions in Oncopig. Abstract Background The development of relevant and robust large animal models of hepatocellular carcinoma is needed to test new therapeutic strategies for this disease. Transgenic approaches hold promise in addressing this complex problem. One such model, the Oncopig, has been
Erik N. K. Cressman, Samantha Hicks, Natalie W. Fowlkes, Danielle L. Stolley, Maria Sophia Stenkamp   +4 more
wiley   +1 more source

An Asymptomatic Male with Coexisting Celiac Disease and Primary Biliary Cirrhosis

Canadian Journal of Gastroenterology, 1991
Both primary biliary cirrhosis and celiac disease may be asymptomatic in many patients for extended periods of time. Rarely the disorders have been reported to occur together, in which case symptomatic malabsorption and diarrhea are virtually always ...
Franzjosef Schweiger, Jasim Radhi
doaj   +1 more source

A Randomized, Controlled, Phase 2 Study of Maralixibat in the Treatment of Itching Associated With Primary Biliary Cholangitis. [PDF]

, 2019
Primary biliary cholangitis (PBC) is typically associated with elevated serum bile acid levels and pruritus, but pruritus is often refractory to treatment with existing therapies.
Apostol, George, Bacon, Bruce, Bowlus, Christopher L, Dorenbaum, Alejandro, Gu, Joan, Hirschfield, Gideon M, Jones, David, Kennedy, Ciara, Levy, Cynthia, Luketic, Velimir, Mayo, Marlyn J, Medendorp, Sharon, Novak, Patricia, Patanwala, Imran, Pockros, Paul J, Vuppalanchi, Raj   +15 more
core   +2 more sources

Long‐term impact of Elexacaftor/Tezacaftor/ivacaftor on pulmonary, nutritional and metabolic outcomes in homozygous F508del cystic fibrosis patients: A real‐world cohort study

British Journal of Clinical Pharmacology, EarlyView.
Aim Elexacaftor/tezacaftor/ivacaftor (ETI) has markedly improved cystic fibrosis (CF) outcomes. However, its long‐term impact on nutrition, metabolism and liver health remains underexplored. We assessed 30‐month changes in pulmonary, nutritional, metabolic and inflammatory markers in people with CF (PwCF) homozygous for F508del.
Nicola Perrotta, Luigi Angelo Fiorito, Roberta Vescovo, Anna Virgilio, Giulia Amato, Giuseppe Cimino   +5 more
wiley   +1 more source