title: Prominent medial hypertrophy of renal arterioles in an infant with hyporeninemic hypoaldosteronism.
abstract: We describe an 11-month-old boy who presented clinically with hyperkalemic renal tubular acidosis due to hyporeninemic hypoaldosteronism. Persistent hyperchloremic acidosis and mild azotemia were present. All abnormal laboratory values were corrected by the administration of fludrocortisone. Renal biopsy showed prominent medial hypertrophy of renal arterioles and interstitial fibrosis, which closely resemble those of the gene-targeted mice with disruption of the renin angiotensin system. This is the first case report raising the possibility that a defective renin angiotensin system in infancy may lead to tubulointerstitial damage with medial hypertrophy of intrarenal arterioles.
authors.affiliation: Department of Pediatrics and Laboratory Medicine, Fukui Medical University, Matsuoka, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Tsuchida
authors.forename: Y
authors.initials: Y
authors.lastname: Hara
authors.forename: C
authors.initials: C
authors.lastname: Hori
authors.forename: M
authors.initials: M
authors.lastname: Hiraoka
authors.forename: S
authors.initials: S
authors.lastname: Suzuki
authors.forename: M
authors.initials: M
authors.lastname: Mayumi
mesh_terms: D001160:Arterioles / Q000473:pathology*; D001706:Biopsy; D006801:Humans; D006947:Hyperkalemia / Q000209:etiology; D006984:Hypertrophy / Q000473:pathology; D006994:Hypoaldosteronism / Q000150:complications / Q000473:pathology*; D007223:Infant; D007668:Kidney / Q000098:blood supply* / Q000473:pathology*; D008297:Male; D014511:Uremia / Q000209:etiology