Mauriac syndrome: three cases with retinal angiofluorescein study.

Three children with features of the Mauriac syndrome have been studied. Ocular complications, mainly characterized by increased capillary permeability have been demonstrated by fluorescein angiography. A decreased proximal tubular beta 2-microglobulin reabsorption has been found in one patient, and in another one, there was a slowed sensory nerve condition velocity. A liver biopsy, performed in one patient, has shown numerous fat filled cells and intralysosomal lipofuscin storage. No hormonal disturbance, which could explain the growth reardation, has been found. Bad metabolic control of diabetes could be responsible for all the anomalies of Mauriac syndrome.