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Rev Esp Enferm Dig. 2000 Sep;92(9):601-8.

Role of orthotopic liver transplant in the treatment of homozygous familial hypercholesterolemia.

[Article in English, Spanish]

Author information

  • 1Liver Unit Transplantation, Hospital Universitario Reina Sofía, Córdoba, Spain. jacccg@teleline.es

Abstract

Homozygous familial hypercholesterolemia is an inherited metabolic disease that leads to decreased catabolism of low-density lipoprotein cholesterol. As a result coronary artery disease ensues by the first or second decade. Because most low-density lipoprotein receptors (50-75%) are located in the liver, liver transplantation has been introduced as a therapeutic option in this disorder.

AIMS:

To report our experience in the treatment of homozygous familial hypercholesterolemia with ortothopic liver transplantation. We evaluated metabolic results and patient survival.

METHOD:

We treated two affected siblings. One of them received a sequenced heart-liver transplantation because of cardiac failure due to severe coronary disease.

RESULTS:

The operative and postoperative course was favourable in both patients, with a decrease in cholesterol levels to normal values. Four years later both were alive and both had normal liver and heart functions. Neither patient needed cholesterol-lowering drugs, and the disease had not progressed.

CONCLUSIONS:

As shown by our results and those reported by others, liver transplantation is the treatment of choice for homozygous familial hypercholesterolemia until gene therapy becomes a viable option. Transplantation should be done before of cardiovascular complications develop.

PMID:
11138242
[PubMed - indexed for MEDLINE]
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