Lipoprotein glomerulopathy: a rare cause of steroid-resistant nephrotic syndrome in a child.
CEN Case Rep
; 13(5): 403-407, 2024 Oct.
Article
en En
| MEDLINE
| ID: mdl-38438730
ABSTRACT
Lipoprotein glomerulopathy (LPG) is a rare condition of renal lipidosis characterized by lipoprotein thrombi in glomeruli, an abnormal plasma lipoprotein profile, and a marked increase in serum apolipoprotein E (apo E) levels. It is a monogenic disorder with autosomal dominant inheritance and the average age of presentation is 32 years (4-69 years). It is rare in children. The presentation can be nephrotic syndrome, hematuria, or progressive renal failure. Here we report the first described case of LPG in an Indian 7.5-year-old boy who presented with steroid-resistant nephrotic syndrome with normal renal function. A renal biopsy was suggestive of lipoprotein glomerulopathy. The detection of a pathogenic variant in apo E, Kyoto type, by exome sequencing, confirmed the diagnosis of lipoprotein glomerulopathy. Complete response was achieved with Angiotensin-converting Enzyme inhibitor and fenofibrates.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Síndrome Nefrótico
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
CEN Case Rep
/
CEN case reports
Año:
2024
Tipo del documento:
Article
País de afiliación:
India