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2018 ; 6
(ä): 72
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Treatment of Genetic Forms of Nephrotic Syndrome
#MMPMID29632851
Kemper MJ
; Lemke A
Front Pediatr
2018[]; 6
(ä): 72
PMID29632851
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Idiopathic steroid-resistant nephrotic syndrome (SRNS) is most frequently
characterized by focal segmental glomerulosclerosis (FSGS) but also other
histological lesions, such as diffuse mesangial sclerosis. In the past two
decades, a multitude of genetic causes of SRNS have been discovered raising the
question of effective treatment in this cohort. Although no controlled studies
are available, this review will discuss treatment options including pharmacologic
interventions aiming at the attenuation of proteinuria in genetic causes of SRNS,
such as inhibitors of the renin-angiotensin-aldosterone system and indomethacin.
Also, the potential impact of other interventions to improve podocyte stability
will be addressed. In this respect, the treatment with cyclosporine A (CsA) is of
interest, since a podocyte stabilizing effect has been demonstrated in various
experimental models. Although clinical response to CsA in children with genetic
forms of SRNS is inferior to sporadic SRNS, some recent studies show that partial
and even complete response can be achieved even in individual patients inherited
forms of nephrotic syndrome. Ideally, improved pharmacologic and molecular
approaches to induce partial or even complete remission will be available in the
future, thus slowing or even preventing the progression toward end-stage renal
disease.
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