Open AccessGlomerulocystic disease, a rare cause of renal cysts in infants: A series of three cases
Author(s) -
Nelofar Islam,
Biplab Mandal,
Goutam Bera,
Suchandra Mukherjee,
Uttara Chatterjee
Publication year - 2019
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_643_17
Subject(s) - medicine , disease , polycystic kidney disease , genetic counseling , pathology , autosomal dominant polycystic kidney disease , biopsy , kidney , pediatrics , biology , genetics
Glomerulocystic kidney disease (GCKD) is an uncommon type of cystic renal disease affecting children. It has both sporadic and familial occurrence and is characterized by cortical microcysts associated with dilatation of Bowman's spaces. In some instances, GCKD is an early manifestation of autosomal dominant polycystic kidney disease. Here, we present three cases of GCKD, two in infants and one in a perinatal postmortem. The first one is a case of GCKD with unilateral involvement, diagnosed on surgical biopsy. GCKD is a morphological expression of several hereditary and nonhereditary disorders that differ vastly in their management and long-term outcome. Hence, accurate morphological diagnosis of this entity is important for prognostication and genetic counseling.