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Sporadic idiopathic steroid-resistant nephrotic syndrome
Steroid-resistant, sporadic idiopathic nephrotic syndrome, is a heterogeneous entity. Nephrotic syndrome is characterised by marked proteinuria, with reduced plasmatic levels of albumin, and potentially with oedema.
- Sporadic idiopathic nephrosis
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Childhood
- ICD-10: N04.1 N04.3 N04.8
- OMIM: 612551
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
Its prevalence in the general population is unknown.
Familial forms are in most cases related to podocyte protein structural anomalies. Other forms result from a totally different mechanism, which has not yet been elucidated. One of the proposed hypotheses suggests that cells from the immune system could produce one or several circulating factors, which would increase the glomerular permeability to proteins. Current research aims at identifying the circulating factors which could be responsible for the nephrotic syndrome.
Management and treatment
In some cases, immunosuppressive drugs, in particular cyclosporin, can prove beneficial.
However, the progression to end-stage renal failure is frequent. In this case, there is a risk of nephrotic syndrome recurrence after kidney transplant.