Progressive familial intrahepatic cholestasis: Partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients

American Journal of Gastroenterology

Volumn 95, Issue 12, 2000, Pages 3522-3528

  Melter, Michael ^a   Rodeck, Burkhard ^b   Kardorff, Rüdiger ^b   Hoyer, Peter F ^b   Petersen, Claus ^b   Ballauff, Antje ^b   Brodehl, Johannes ^b  

^a BOSTON CHILDREN S HOSPITAL   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

APOLIPOPROTEIN A1; BILE ACID; CHOLESTEROL; CHOLINESTERASE; DEETHYLLIDOCAINE; GAMMA GLUTAMYLTRANSFERASE; HIGH DENSITY LIPOPROTEIN;

ARTICLE; BILIARY TRACT SURGERY; CATCH UP GROWTH; CHILD; CLINICAL ARTICLE; ENZYME ACTIVITY; FEMALE; GROWTH DISORDER; HEPATOMEGALY; HUMAN; INTRAHEPATIC CHOLESTASIS; JAUNDICE; LIPID METABOLISM; LIVER FUNCTION; MALE; PRIORITY JOURNAL; PRURITUS;

ADULT; BILIARY TRACT SURGICAL PROCEDURES; CHILD, PRESCHOOL; CHOLESTASIS, INTRAHEPATIC; FEMALE; FOLLOW-UP STUDIES; GALLBLADDER; GROWTH; GROWTH DISORDERS; HUMANS; JEJUNUM; LIPIDS; LIVER CIRRHOSIS; LIVER FUNCTION TESTS; MALE; TIME FACTORS;

EID: 0033638340     PISSN: 00029270     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0002-9270(00)02163-8     Document Type: Article

References (60)

1. Orthotopic liver transplantation for Byler disease
2. Progressive familial intrahepatic cholestasis (Byler's disease)
3. Familial intrahepatic cholestasis syndromes
4. Fatal familial cholestatic syndrome in Greenland Eskimo children
5. Byler disease - Fatal familial intrahepatic cholestasis in an Amish kindred
6. Histologic pathology of the liver in progressive familial intrahepatic cholestasis
7. Progressive intrahepatic cholestasis; A defect in apolipoprotein A-1 synthesis?
8. Parameters of lipoprotein metabolism and cholestasis in healthy and cholestatic infants and children
9. Progressive intrahepatic cholestasis
10. Progressive familial intrahepatic cholestasis
11. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis
12. Locus heterogeneity in progressive familial intrahepatic cholestasis
13. Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): Evidence for heterogeneity
14. Progressive familial intrahepatic cholestasis (PFIC): Evidence for genetic heterogeneity by exclusion of linkage to chromosome 18q21-q22
15. Identification of a locus for progressive familial intrahepatic cholestasis PFIC2 on chromosome 2q24
16. Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region
17. Defect of multidrug-resistance 3 gene expression in a subtype of progressive familial intrahepatic cholestasis
18. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis
19. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis
20. Longitudinale Korperentwicklung gesunder Kinder von 0 bis 18 Jahren
21. Langfristige Ursodeoxycholsaurebehandlung cholestatischer Lebererkrankungen des Kindesalters - Klinische und biochemische Effekte
22. Direct spectrophotometry of total bile acids in serum
23. Enzymatic determination of lipoprotein-X, a specific serum cholestasis marker
24. Fortschritte in der Analytik des Lipoproteinmusters
25. Semi-automated enzyme-linked immunosorbent assay (ELISA) for the quantification of apolipoprotein B using monoclonal antibodies
26. Competitive enzyme-linked immunosorbent assey (ELISA) for the quantitation of apolipoprotein A-1 using a monoclonal antibody
27. Monoethylglycinexylidide formation kinetics: A novel approach to assessment of liver function
28. Procedures for the chromatographic determination of amino acids on four percent cross-linked sulfonated polystrene resins
29. Vergleich verschiedener Leberfunktionsteste bei chronisch leberkranken Kindern
30. The plasma amino acid profile and its relationships to standard quantities of liver function in infants and children with extrahepatic biliary atresia and preterminal liver cirrhosis
31. Plasma amino acids in patients with hepatic encephalopathy. Effect of amino acid infusions
32. Clinical and biochemical findings in progressive familial intrahepatic cholestasis
33. The effects of phenobarbital on bile salts and bilirubin in patients with intrahepatic and extrahepatic cholestasis
34. Cholestyramine therapy in patients with a paucity of intrahepatic bile ducts
35. Rifampicin relieves pruritus in children with chronic cholestatic liver disease
36. Long term outcome in pediatric liver transplant recipients with Byler's disease
37. Morbus Byler (Progressive familiare intrahepatische Cholestase)-Klinik, Diagnostik und Therapie
38. Treatment of progressive familial intrahepatic cholestasis: Liver transplantation or partial external biliary diversion
39. Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus
40. Clinical case report: Chronic pruritus and cholestasis
41. Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease)
42. Byler disease: Fatal familial intrahepatic cholestasis in an Amish kindred
43. Kleinwuchsdiagnostik bei M. Byler
44. Growth hormone secretion dynamics, IGF-1, IGF BP- and GHBP in children with familial intrahepatic cholestasis (Bylers disease)
45. High density lipoprotein deficiency with xanthomas. A defect in reverse cholesterol transport caused by a point mutation in the apolipoprotein A-I gene
46. Reduced plasma cholesterol and increased fecal sterol loss in multidrug resistance gene 2 P-glycoprotein-deficient mice
47. Bile diversion lowers apolipoprotein A-I and A-IV mRNA levels in rat ileum
48. Apolipoprotein A-I is required for cholesteryl ester accumulation in steroidogenic cells and for normal adrenal steroid production
49. Hepatic expression of genes regulating lipid metabolism in rabbits
50. Hepatic lipase promotes the selective uptake of high density lipoprotein-cholesteryl esters via the scavenger receptor Bl
51. Hepatic lipase is localized at the parenchymal cell microvilli in rat liver
52. Reverse cholesterol transport - A review of the process and its clinical implications
53. Cholesteryl ester transfer activity in liver disease and cholestasis, and its relation with fatty acid composition of lipoprotein lipids
54. Lipoproteins, HDL-apolipoproteins, activities of hepatic lipase and lecithin-cholesterol acyltransferase in the plasma of patients with post-alcoholic end-stage liver cirrhosis
55. Hepatic lipase and lipoprotein lipase in postheparin plasma in liver disease. Relations to plasma proteins
56. Lipoprotein abnormalities in primary biliary cirrhosis. Association with hepatic lipase inhibition as well as altered cholesterol esterification
57. Role of hepatic lipase in the uptake and processing of chylomicron remnants in rat liver
58. Mild dyslipidemia in mice following targeted inactivation of the hepatic lipase gene
59. Effects of intravenous infusion of lipid-free apo A-I in humans
60. The influence of apolipoproteins on the hepatic lipase-mediated hydrolysis of high density lipoprotein phospholipid and triacylglycerol