Results 51 to 60 of about 116,093 (277)

∆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

THREE CASES WITH BURKITT LYMPHOMA PRESENTING WITH CHOLESTASIS

Hematology, Transfusion and Cell Therapy, 2021
Case report: Cholestasis secondary to neoplasm is rare in children. It is also rare in Burkitt lymphoma and may be cause to treatment delay. We report 3 cases diagnosed with Burkitt lymphoma with cholestasis.
Fatma Tuba YILDIRIM, Derya ÖZYÖRÜK, Arzu YAZAL ERDEM, Selma ÇAKMAKCI, Neriman SARI, Sonay İNCESOY, İnci İLHAN   +6 more
doaj   +1 more source

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

Liver Transplantation for Alagille's Syndrome [PDF]

, 1993
Twenty-three children with Alagille's syndrome and end-stage liver disease underwent liver transplantation with cyclosporine and low-dose steroid immunosuppression. Two to 9 years (mean, 4.4 years) after surgery, 13 (57%) of the children were still alive,
Reyes, J, Starzl, TE, Tepetes, K, Todo, S, Tzakis, AG, Tzoracoleftherakis, V   +5 more
core   +1 more source

Decreased expression of breast cancer resistance protein in the duodenum in patients with obstructive cholestasis [PDF]

, 2006
Background/Aims: The expression of transporters involved in bile acid homeostasis is differentially regulated during obstructive cholestasis. Since the drug efflux transporter breast cancer resistance protein (BCRP) is known to transport bile acids, we ...
Adachi Y, Brendel C, Christian Zimmermann, Christoph Beglinger, Denk GU, Denson LA, Dietrich CG, Dietrich CG, Ee PL, Frank Lehmann, Freeman LA, Geier A, Gutmann H, Heike Gutmann, Hruz P, Imai Y, Janvilisri T, Jonker JW, Juergen Drewe, Jung D, Jung D, Kamisako T, Klucken J, Li N, Lown KS, Lu TT, Luigi Terracciano, Maliepaard M, Miyake K, Petr Hruz, Plebani M, Popowski K, Schellens JH, Smith M, Stedman CA, Stellaard F, Suzuki M, Taipalensuu J, Tanaka Y, Tanaka Y, Trauner M, Ulrich Beuers, van Herwaarden AE, Volk EL, Wagner M, Xie W, Yu L, Zimmermann C, Zollner G   +48 more
core   +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

Baboon-to-human liver transplantation [PDF]

, 1993
Our ability to control both the cellular and humoral components of xenograft rejection in laboratory experiments, together with an organ shortage that has placed limits on clinical transplantation services, prompted us to undertake a liver ...
A Tzakis, A Zeevi, A Zeevi, A.J Demetris, A.J Demetris, Alper, Asonuma, Bailey, Barnard, Cano, Crawford, Essex, Francavilla, H Doyle, Hasan, Hitchcock, I.R Marino, Iwaki, J Fung, Jacobson, Karpas, Kelly, M Michaels, M Trucco, M Trucco, Moran, Porter, S Kusne, S Kusne, S Todo, Starzl, Starzl, Starzl, Starzl, Starzl, T.E Starzl, Takaya, Todo, Tzakis, V Warty, V Warty, W.A Rudert, W.A Rudert, Wolpl   +43 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

Tauroursodeoxycholic acid exerts anticholestatic effects by a cooperative cPKC alpha-/PKA-dependent mechanism in rat liver. [PDF]

, 2008
Objective: Ursodeoxycholic acid (UDCA) exerts anticholestatic effects in part by protein kinase C (PKC)-dependent mechanisms. Its taurine conjugate, TUDCA, is a cPKCa agonist.
Beuers, U., Denk, G.U., Hohenester, S., Pusl, T., Rust, C., Wimmer, R.   +5 more
core   +1 more source

Glycochenodeoxycholate Promotes Liver Fibrosis in Mice with Hepatocellular Cholestasis

Cells, 2020
Hydrophobic bile salts are considered to promote liver fibrosis in cholestasis. However, evidence for this widely accepted hypothesis remains scarce. In established animal models of cholestasis, e.g., by Mdr2 knockout, cholestasis and fibrosis are both ...
S. Hohenester, V. Kanitz, A. Kremer, C. Paulusma, Ralf Wimmer, Helen Kuehn, G. Denk, D. Horst, R. O. Oude Elferink, U. Beuers   +9 more
semanticscholar   +1 more source