The cause of death in Laurence-Moon-Bardet-Biedl syndrome.

The certificates of death of 14 deceased patients with Laurence-Moon-Bardet-Biedl (LMBB) syndrome, in which retinal dystrophy is dominant feature, were review. Death occurred at a considerably younger age than in the general population. Renal disease was noted as primary or contributing cause of death in 7 cases with the diagnoses: cyst of the kidney, renal sclerosis, renal failure, proteinuria, renal disease unspecified and malignant hypertension with renal involvement. It is concluded that renal involvement is characteristic of individuals with LMBB syndrome and seems to reduce life expectancy considerably.