Diffuse bilateral glomerulocystic disease of the kidneys and multiple cardiac rhabdomyomas in a newborn. Relationship with tuberous sclerosis and review of the literature.
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.
Saguem
M H
MH
Department of Pathology, Faculty of Medicine, Geneva, Switzerland.
Laarif
M
M
Remadi
S
S
Bozakoura
C
C
Cox
J N
JN
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*