Abstract | BACKGROUND:
Bardet-Biedl syndrome is an autosomal recessive disorder characterized by obesity, polydactyly of hands and feet, retinitis pigmentosa, hypogenitalism, various degrees of intellectual impairment and renal anomalies. Other clinical features include speech disorder, brachydactyly, developmental delay, polyuria/ polydipsia, ataxia, poor coordination/clumsiness, diabetes mellitus, left ventricular hypertrophy, hepatic fibrosis, and renal hypoplasia/dysplasia. If spasticity and mental retardation present, it fulfills the criteria for Laurence-Moon-Bardet-Biedl syndrome. METHODS: Between July 1985 and January 2005, 2,282 kidney transplantations were performed at Labbafinejad Medical Center, of whom 262 were children under 15 years of age (61% males). Among children, five (four females) had Bardet-Biedl syndrome who were transplanted preemptively. Kidneys were taken from living unrelated donors. RESULTS: CONCLUSION:
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Authors | Mostafa Sharifian, Masood Dadkhah-Chimeh, Behzad Einollahi, Mohsen Nafar, Nasser Simforoush, Abbass Basiri, Hassan Otukesh |
Journal | Archives of Iranian medicine
(Arch Iran Med)
Vol. 10
Issue 3
Pg. 339-42
(Jul 2007)
ISSN: 1029-2977 [Print] Iran |
PMID | 17604471
(Publication Type: Journal Article)
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Topics |
- Adolescent
- Bardet-Biedl Syndrome
(surgery)
- Body Mass Index
- Child
- Cohort Studies
- Female
- Humans
- Iran
- Kidney
(abnormalities)
- Kidney Function Tests
- Kidney Transplantation
- Male
- Retrospective Studies
- Treatment Outcome
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