1977
DOI: 10.1056/nejm197703032960901
|View full text |Cite
|
Sign up to set email alerts
|

Failure of Complete Bile Diversion and Oral Bile Acid Therapy in the Treatment of Homozygous Familial Hypercholesterolemia

Abstract: Two patients with nomozygous familial hypercholesterolemia, refractory to medical therapy, underwent complete bile diversion by common-duct ligation and cholecystostomy, in an attempt to arrest the progression of their xanthomatosis and atherosclerosis by depletion of body cholesterol. Clofibrate was given after operation to one patient, and cholic acid to both, in an effort to enhance further the negative sterol balance. Bile diversion produced an increase of six to eight times in gastrointestinal sterol outp… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
2
1
1
1

Citation Types

1
19
0

Year Published

1978
1978
2011
2011

Publication Types

Select...
8
1
1

Relationship

0
10

Authors

Journals

citations
Cited by 53 publications
(20 citation statements)
references
References 21 publications
1
19
0
Order By: Relevance
“…First, cholestyramine treatment increases fecal bile acid excretion in FH homozygotes to the same degree as it does in normal subjects (40). Second, complete biliary diversion increased bile acid excretion markedly in two FH homozygotes without altering the plasma cholesterol level (41). Third, feeding bile acids to patients with FH does not materially influence the plasma cholesterol level (42).…”
Section: Resultsmentioning
confidence: 99%
“…First, cholestyramine treatment increases fecal bile acid excretion in FH homozygotes to the same degree as it does in normal subjects (40). Second, complete biliary diversion increased bile acid excretion markedly in two FH homozygotes without altering the plasma cholesterol level (41). Third, feeding bile acids to patients with FH does not materially influence the plasma cholesterol level (42).…”
Section: Resultsmentioning
confidence: 99%
“…Partial ileal bypass may be complicated by malabsorptive gastrointestinal side effects, whereas portacaval shunting may lead to hepatic encephalopathy. 22 Liver transplantation is restricted by a lack of donor organs and the need for continuous postoperative immunosuppression. 23 LDL apheresis significantly lowers LDL-C and is considered the standard of care for patients with HoFH.…”
Section: Discussionmentioning
confidence: 99%
“…Cholestyramine treatment has been shown to reduce plasma LDL levels in individuals with heterozygous familial hypercholesterolemia by increasing the receptor-mediated elimination of LDL (44). However, plasma LDL levels in patients with homozygous (receptor-negative) familial hypercholesterolemia are not lowered even by treatment with a total bile fistula (45). In addition, suppression of bile acid biosynthesis in man, which occurs during treatment with the primary bile acid chenodeoxycholic acid (46,47), results in a slight, but significant, increase in plasma LDL levels (48,49).2 Chenodeoxycholic acid treatment in man is associated with decreased hepatic cholesterol synthesis (50), a reduced production rate of VLDL (51) (the precursor of human plasma LDL), and possibly a reduction in LDL catabolism.…”
Section: Discussionmentioning
confidence: 99%