title: Diffuse bilateral glomerulocystic disease of the kidneys and multiple cardiac rhabdomyomas in a newborn. Relationship with tuberous sclerosis and review of the literature.
abstract: A case of glomerulocystic disease of the kidneys in a newborn is described. The kidneys were very large and diffusely cystic. Histologically, all portions of the nephron were involved and some of the cysts were lined by a prominent hyperplastic eosinophilic epithelium. This, together with the presence of numerous cardiac rhabdomyomas, a nodular haemangiomatous harmartoma and histiocytoid cell nests in the spleen were strongly suggestive of tuberous sclerosis. The relation between glomerulocystic kidney disease and tuberous sclerosis is discussed.
authors.affiliation: Department of Pathology, Faculty of Medicine, Geneva, Switzerland.
authors.forename: M H
authors.initials: MH
authors.lastname: Saguem
authors.forename: M
authors.initials: M
authors.lastname: Laarif
authors.forename: S
authors.initials: S
authors.lastname: Remadi
authors.forename: C
authors.initials: C
authors.lastname: Bozakoura
authors.forename: J N
authors.initials: JN
authors.lastname: Cox
mesh_terms: D003937:Diagnosis, Differential; D006338:Heart Neoplasms / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D052177:Kidney Diseases, Cystic / Q000473:pathology*; D008297:Male; D012207:Rhabdomyoma / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000473:pathology*