Liddle’s-Like Syndrome Associated With Nephrotic Syndrome in a Pediatric Patient

This is the authors' abstract. We don't add key points for this paper.

Nephrotic syndrome, especially the steroid-resistant form, is often complicated by hypertensionin childhood. The syndrome is common in black children. However, the histopathologicalfinding of membranous nephropathy in children with nephrotic syndrome is rare. Liddle’ssyndrome is a rare genetic abnormality that presents with salt-sensitive hypertension causedby constitutive activation of the amiloride-sensitive epithelial sodium channel. The epithelialsodium channel comprises homologous α, β, and γ subunits that share similar structures.Gene mutations associated with Liddle’s syndrome occur in either the β or γ subunits anddisturb or truncate a conserved proline-rich sequence (i.e., PY motif), leading to constitutiveactivation of the epithelial sodium channel. The association of nephrotic syndrome withLiddle’s syndrome has only been described in an adult patient. We present here the first caseof these two syndromes (nephrotic syndrome and Liddle’s-like syndrome) co-existing in achild with membranous nephropathy in his histopathological finding on kidney biopsy.