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Síndrome nefrótico idiopático en niños
Damien G Noone1, Kazumoto Iijima2, Rulan Parekh3
1Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
Lancet (London, England)
|June 19, 2018
Resumen
El síndrome nefrótico idiopático (NS) en niños tiene una incidencia variable y causas desconocidas, que a menudo involucran problemas inmunológicos o factores genéticos. Mientras que el NS que responde a los esteroides tiene un excelente pronóstico, la resistencia a los esteroides indica un mayor riesgo de enfermedad renal.
Área de la Ciencia:
- Nefrología pediátrica
- Inmunología
- La genética
Sus antecedentes:
- El síndrome nefrótico idiopático (NS) afecta a 1.15-16.9 por cada 100.000 niños, con una incidencia que varía según la etnia y la región.
- La patogénesis es compleja, potencialmente involucrando desregulación inmune, factores circulantes o anomalías de podocitos.
- Los factores genéticos están más implicados en la NS resistente a los esteroides.
Objetivo del estudio:
- Para resumir el entendimiento actual del síndrome nefrótico idiopático en niños.
- Para resaltar aspectos clave de su patogénesis, tratamiento, complicaciones y pronóstico.
Principales métodos:
- Revisión de la literatura existente sobre el síndrome nefrótico idiopático.
- Análisis de datos epidemiológicos, mecanismos patógenos, enfoques terapéuticos y factores de pronóstico.
Principales resultados:
- El tratamiento principal es la prednisona para los casos que responden a los esteroides.
- Las recaídas frecuentes pueden requerir agentes inmunosupresores alternativos.
- Las principales complicaciones incluyen infección, tromboembolismo venoso y lesión renal aguda.
Conclusiones:
- El NS que responde a los esteroides generalmente tiene un excelente resultado renal a largo plazo.
- La resistencia a los esteroides es un factor determinante para el riesgo futuro de enfermedad renal crónica o terminal.
- Se necesitan más investigaciones sobre la patogénesis y el tratamiento de los casos resistentes a los esteroides.
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