Pathogenic role of glomerulo-tubular junction stenosis in glomerulocystic disease.
Glomerulocystic disease is an uncommon cystic renal condition characterized by cystic dilatation forming a glomerular cyst. The pathogenesis of this familial disease is unknown. We performed a serial section study using a biopsy specimen of a 16-year old female patient with glomerular cystic disease who had a family history of end stage renal failure. A total of 14 different glomeruli were analyzed, four of which exhibited a cystic appearance. Five glomerulotubular junctions were observed by serial sections, two of which had a stenotic appearance where glomerular cystic changes and periglomerular fibrosis were observed concomitantly. There were no such cystic glomerular changes in the other three glomeruli with non-stenotic glomerulo-tubular junctions. These findings suggest that the glomerular cystic lesion develops as a consequence of glomerulo-tubular junctional stenosis probably caused by periglomerular fibrosis.
Hotta
O
O
Department of Nephrology, Sendai Shakaihoken Hospital, Japan.
Sato
M
M
Furuta
T
T
Taguma
Y
Y
D000293:Adolescent; D001707:Biopsy, Needle; D003251:Constriction, Pathologic / Q000150:complications / Q000473:pathology; D005260:Female; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000209:etiology / Q000473:pathology*; D007678:Kidney Glomerulus / Q000473:pathology*; D007684:Kidney Tubules / Q000473:pathology