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

Nephrotic syndrome, especially the steroid-resistant form, is often complicated by hypertension in childhood. The syndrome is common in black children. However, the histopathological finding of membranous nephropathy in children with nephrotic syndrome is rare. Liddle’s syndrome is a rare genetic abnormality that presents with salt-sensitive hypertension caused by constitutive activation of the amiloride-sensitive epithelial sodium channel. The epithelial sodium 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 and disturb or truncate a conserved proline-rich sequence (i.e., PY motif), leading to constitutive activation of the epithelial sodium channel. The association of nephrotic syndrome with Liddle’s syndrome has only been described in an adult patient. We present here the first case of these two syndromes (nephrotic syndrome and Liddle’s-like syndrome) co-existing in a child with membranous nephropathy in his histopathological finding on kidney biopsy.