Clinical Evaluation of Nephrotic Syndrome Manifesting in the First Year of Life
Journal of the Korean Society of Pediatric Nephrology
;
: 161-169, 2009.
Artigo
em Coreano
| WPRIM
| ID: wpr-78751
ABSTRACT
PURPOSE:
This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life.METHODS:
We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007.RESULTS:
Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease.CONCLUSION:
Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Prognóstico
/
Esclerose
/
Biópsia
/
Glomerulosclerose Segmentar e Focal
/
Estudos Retrospectivos
/
Transplante de Rim
/
Sepse
/
Síndrome de Denys-Drash
/
Parto
/
Diálise
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Feminino
/
Humanos
/
Masculino
Idioma:
Coreano
Revista:
Journal of the Korean Society of Pediatric Nephrology
Ano de publicação:
2009
Tipo de documento:
Artigo
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