Liver-Kidney Transplantation to Cure Atypical Hemolytic Uremic Syndrome

Atypical hemolytic uremic syndrome is often associated with mutations in genes encoding complement regulatory proteins and secondary disorders of complement regulation. Progression to kidney failure and recurrence with graft loss after kidney transplantation are frequent. The most common mutation is...

Full description

Saved in:
Bibliographic Details
Published in:Journal of the American Society of Nephrology 2009-05, Vol.20 (5), p.940-949
Main Authors: SALAND, Jeffrey M, RUGGENENTI, Piero, REMUZZI, Giuseppe
Format: Article
Language:English
Subjects:
Anemia, Hemolytic - surgery
Biological and medical sciences
Combined Modality Therapy
Complement Factor H - genetics
Hematologic and hematopoietic diseases
Hemolytic-Uremic Syndrome - genetics
Hemolytic-Uremic Syndrome - surgery
Humans
Kidney Diseases - surgery
Kidney Transplantation - methods
Liver Transplantation - methods
Medical sciences
Mutation
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
Platelet diseases and coagulopathies
Practice Guidelines as Topic
Renal failure
Thrombocytopenia - surgery
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Atypical hemolytic uremic syndrome is often associated with mutations in genes encoding complement regulatory proteins and secondary disorders of complement regulation. Progression to kidney failure and recurrence with graft loss after kidney transplantation are frequent. The most common mutation is in the gene encoding complement factor H. Combined liver-kidney transplantation may correct this complement abnormality and prevent recurrence when the defect involves genes encoding circulating proteins that are synthesized in the liver, such as factor H or I. Good outcomes have been reported when surgery is associated with intensified plasma therapy. A consensus conference to establish treatment guidelines for atypical hemolytic uremic syndrome was held in Bergamo in December 2007. The recommendations in this article are the result of combined clinical experience, shared research expertise, and a review of the literature and registry information. This statement defines groups in which isolated kidney transplantation is extremely unlikely to be successful and a combined liver-kidney transplant is recommended and also defines those for whom kidney transplant remains a viable option. Although combined liver-kidney or isolated liver transplantation is the preferred therapeutic option in many cases, the gravity of risk associated with the procedure has not been eliminated completely, and assessment of risk and benefit requires careful and individual attention.
ISSN:1046-6673
1533-3450
DOI:10.1681/asn.2008080906