1974
DOI: 10.1016/s0140-6736(74)90004-x
|View full text |Cite
|
Sign up to set email alerts
|

Is an Acute Disturbance in Hepatic Transport of Bile-Acids the Primary Cause of Cholestasis in Benign Recurrent Intrahepatic Cholestasis?

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
1
1
1
1

Citation Types

0
8
0
1

Year Published

1976
1976
2010
2010

Publication Types

Select...
7
2

Relationship

0
9

Authors

Journals

citations
Cited by 32 publications
(9 citation statements)
references
References 5 publications
0
8
0
1
Order By: Relevance
“…13,14 BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS TYPE 1 PFIC type 1 is an autosomal homozygous disease. Heterozygotes with a mutation of the ATP8B1 gene or homozygotes with a mutation that only partly affects the function of the protein may present with benign recurrent intrahepatic cholestasis (BRIC) type 1 or Tygstrup, 15 or Summerskill and Walshe cholestasis. 16 This disease usually becomes manifest in adolescence and is characterized by episodes of cholestasis with severe pruritus and jaundice.…”
Section: Therapymentioning
confidence: 99%
“…13,14 BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS TYPE 1 PFIC type 1 is an autosomal homozygous disease. Heterozygotes with a mutation of the ATP8B1 gene or homozygotes with a mutation that only partly affects the function of the protein may present with benign recurrent intrahepatic cholestasis (BRIC) type 1 or Tygstrup, 15 or Summerskill and Walshe cholestasis. 16 This disease usually becomes manifest in adolescence and is characterized by episodes of cholestasis with severe pruritus and jaundice.…”
Section: Therapymentioning
confidence: 99%
“…This disease is characterized by multiple episodes of cholestasis without extrahepatic bile duct obstruction. At the onset of symptoms, serum bile acids are elevated and patients complain of severe pruritus and anorexia [Van Berge Henegouwen et al, 1974;Summerfield et al, 1981;Bijleveld et al, 1989al. This prodromal phase is generally followed by cholestatic jaundice.…”
Section: Introductionmentioning
confidence: 99%
“…The concentration of serum bile acids increases during the period of pruritus, continues to increase as patients develop jaundice and scleral icterus, and then returns to normal levels as the attack resolves. [16][17][18][19] A correlation has not been found between the severity of the pruritus and the concentration of bile acids. 20,21 The hepatic metabolism of organic anions appears to be affected during attacks of BRIC; after attacks, the secretion of these anions either returns to normal levels or remains slightly higher than seen in unaffected persons.…”
Section: Discussionmentioning
confidence: 99%