[Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome. Apropos of 3 cases].
Three cases of Laurence-Moon-Bardet-Biedl (LMBB) syndrome with renal involvement characterized by tubulo-interstitial lesions are reported. Caliceal deformations were noted on urography. From 55 additional cases found in the literature the main characteristics of the renal disease in LMBB are defined : impairment of renal concentration is present in 36 % of the cases, and urographic abnormalities, mainly dysplasic features and multiple cystic formations on the calices, in 90 %; tubulo interstitial lesions are the most frequent histological finding. The renal involvement lead to chronic renal insufficiency responsible for the death of 60 % of the patients. Renal disease appears therefore as the sixth cardinal features of the LMBB syndrome and makes this syndrome as an entity close to other hereditary disorders with renal abnormalities as Alström syndrome.
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D007849:Laurence-Moon Syndrome / Q000473:pathology* / Q000503:physiopathology; D008297:Male
L'atteinte rénale dans le syndrome de Laurence-Moon-Bardet-Biedl. A propos de trois observations.