Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.
نویسندگان
چکیده
Two children with Laurence-Moon-Biedl syndrome had radiographic evidence of cysts in the renal medulla, and one had impaired renal function and infection. The frequency of cystic disease in this syndrome implies that intravenous urography should be carried out on all patients with this syndrome. Children with renal abnormalities should be followed to avoid further damage from urinary infection, or from dehydration in those with a concentrating defect. Regular renal function tests should be carried out on other children. and full investigation should follow if abnormalities are found in order that the natural history of the often fatal renal lesion can be clarified.
منابع مشابه
Glomerulonephropathy of Laurence-Moon-Biedl syndrome.
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Laurence-Moon-Bardet -Biedl syndrome is a rare congenital disorder, inherited in the autosomal recessive mode. The syndrome is characterized by five cardinal features, namely obesity (83% of cases), mental retardation (80%), polydactyly (75%), retinitis pigmentosa (68%) and hypogeni tali sm (60%).1 Renal invo lvement wi th progressive deterioration in renal function was recognized as a major ca...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 52 12 شماره
صفحات -
تاریخ انتشار 1977