Liddle’s-Like Syndrome Associated With Nephrotic Syndrome in a Pediatric Patient
Journal of Pediatric Nephrology,
Vol. 10 No. 1 (2022),
20 April 2022
,
Page 39-46
https://doi.org/10.22037/jpn.v10i1.36352
Abstract
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.
- Children
- Liddle’s syndrome
- Nephrotic syndrome
- Hypertension
How to Cite
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