scholarly journals Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency

Hepatology ◽  
2021 ◽  
Author(s):  
Daan B.E. van Wessel ◽  
Richard J. Thompson ◽  
Emmanuel Gonzales ◽  
Irena Jankowska ◽  
Benjamin L. Shneider ◽  
...  
Keyword(s):  
Bile Acids ◽  
Serum Bile Acids ◽  
Biliary Diversion ◽  
Native Liver ◽  
Native Liver Survival

scholarly journals Partial Biliary Diversion May Promote Long-Term Relief of Pruritus and Native Liver Survival in Children with Cholestatic Liver Diseases

2020 ◽  
Author(s):  
Kristin Bjørnland ◽  
Maria Hukkinen ◽  
Vladimir Gatzinsky ◽  
Henrik Arnell ◽  
Mikko P. Pakarinen ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Diseases ◽  
Serum Levels ◽  
Cholestatic Liver Disease ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Biliary Diversion ◽  
Native Liver ◽  
Native Liver Survival

Abstract Introduction Rare cholestatic liver diseases may cause debilitating pruritus in children. Partial biliary diversion (PBD) may relieve pruritus and postpone liver transplantation which is the only other alternative when conservative treatment fails. The aim was to report long-term outcome after PBD in a population of 26 million people during a 25-year period. Materials and Methods This is an international, multicenter retrospective study reviewing medical journals. Complications were graded according to the Clavien–Dindo classification system. Results Thirty-three patients, 14 males, underwent PBD at a median of 1.5 (0.3–13) years at four Nordic pediatric surgical centers. Progressive familial intrahepatic cholestasis was the most common underlying condition. Initially, all patients got external diversion, either cholecystojejunostomy (25 patients) or button placed in the gallbladder or a jejunal conduit. Early complications occurred in 14 (42%) patients, of which 3 were Clavien–Dindo grade 3. Long-term stoma-related complications were common (55%). Twenty secondary surgeries were performed due to stoma problems such as prolapse, stricture, and bleeding, or conversion to another form of PBD. Thirteen children have undergone liver transplantation, and two are listed for transplantation due to inefficient effect of PBD on pruritus. Serum levels of bile acids in the first week after PBD construction were significantly lower in patients with good relief of pruritus than in those with poor effect (13 [2–192] vs. 148 [5–383] μmol/L; p = 0.02). Conclusion PBD may ensure long-term satisfactory effect on intolerable pruritus and native liver survival in children with cholestatic liver disease. However, stoma-related problems and reoperations are common.

scholarly journals Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis

2020 ◽  
Vol 13 (6) ◽  
pp. e234185
Author(s):  
Christoph Slavetinsky ◽  
Ekkehard Sturm
Keyword(s):  
Bile Acid ◽  
Bile Acids ◽  
Intrahepatic Cholestasis ◽  
Cholestatic Liver Disease ◽  
Serum Bile Acid ◽  
Open Label ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Serum Bile Acids ◽  
Biliary Diversion

Untreated progressive familial intrahepatic cholestasis (PFIC) type 2, or bile salt exporter protein deficiency, frequently leads to severe pruritus, impaired growth and progressive liver fibrosis with risk of organ failure. We describe a 15-month-old male patient with severe pruritus diagnosed with PFIC type 2 enrolled in an open-label phase 2 study who received 4 weeks of treatment with odevixibat, an ileal bile acid transporter inhibitor under development for cholestatic liver disease treatment. The patient experienced reductions in serum bile acids and improvement in itching and sleep scores, and odevixibat was well tolerated. After the odevixibat study, symptoms returned and the patient underwent partial external biliary diversion (PEBD). Odevixibat treatment and PEBD produced similar normalisation of serum bile acid levels and improvements in pruritus and sleep disruptions. Thus, odevixibat appeared to be as effective as invasive PEBD in treating serum bile acids and cholestatic pruritus in this patient.

Gut permeability and serum bile acids in metabolic syndrome

2013 ◽  
Vol 51 (05) ◽  
Author(s):  
B Leber ◽  
N Tripolt ◽  
A Horvath ◽  
S Lemesch ◽  
T Stojakovic ◽  
...  
Keyword(s):  
Metabolic Syndrome ◽  
Bile Acids ◽  
Gut Permeability ◽  
Serum Bile Acids

scholarly journals Measurement of human serum bile acids by gas–liquid chromatography

1965 ◽  
Vol 6 (2) ◽  
pp. 182-192 ◽  
Author(s):  
D.H. Sandberg ◽  
J. Sjövall ◽  
K. Sjövall ◽  
D.A. Turner
Keyword(s):  
Liquid Chromatography ◽  
Human Serum ◽  
Bile Acids ◽  
Gas Liquid Chromatography ◽  
Serum Bile Acids

scholarly journals Isotope derivative assay of human serum bile acids.

1981 ◽  
Vol 22 (4) ◽  
pp. 725-729
Author(s):  
J F Pageaux ◽  
B Duperray ◽  
M Dubois ◽  
H Pacheco
Keyword(s):  
Human Serum ◽  
Bile Acids ◽  
Serum Bile Acids

Investigation of serum bile acids; seven patients with Alagille syndrome

1985 ◽  
Vol 144 (3) ◽  
pp. 236-239 ◽  
Author(s):  
K. Obinata ◽  
N. Nakatsu ◽  
T. Watanabe ◽  
S. Niijima ◽  
O. Arisaka ◽  
...  
Keyword(s):  
Bile Acids ◽  
Alagille Syndrome ◽  
Serum Bile Acids
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