Effect of center practices on the choice of the first dialysis modality for children and young adults

Hogan, Julien; Ranchin, Bruno; Fila, Marc; Harambat, Jérôme; Krid, Saoussen; Vrillon, Isabelle; Roussey, G.; Fischbach, Michel; Couchoud, Cécile

doi:10.1007/s00467-016-3538-7

Cited by 10 publications

(1 citation statement)

References 43 publications

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“…For patients with ESKD, dialysis (peritoneal dialysis, centre haemodialysis or home-haemodialysis) negatively affects the quality of life 235 . However, when patients reach ESKD, oedema and other symptoms of nephrotic syndrome typically improve because urinary protein losses decline and finally cease with progressive oligo-anuria.…”

Section: Cushingoid Featuresmentioning

confidence: 99%

Podocytopathies

Kopp

Anders

Suszták

et al. 2020

Nat Rev Dis Primers

312

270

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Podocytopathies are kidney diseases in which direct or indirect podocyte injury drives proteinuria or nephrotic syndrome. In children and young adults, genetic variants in >50 podocyte-expressed genes, syndromal non-podocyte-specific genes and phenocopies with other underlying genetic abnormalities cause podocytopathies associated with steroid-resistant nephrotic syndrome or severe proteinuria. A variety of genetic variants likely contribute to disease development. Among genes with non-Mendelian inheritance, variants in APOL1 have the largest effect size. In addition to genetic variants, environmental triggers such as immune-related, infection-related, toxic and haemodynamic factors and obesity are also important causes of podocyte injury and frequently combine to cause various degrees of proteinuria in children and adults. Typical manifestations on kidney biopsy are minimal change lesions and focal segmental glomerulosclerosis lesions. Standard treatment for primary podocytopathies manifesting with focal segmental glomerulosclerosis lesions includes glucocorticoids and other immunosuppressive drugs; individuals not responding with a resolution of proteinuria have a poor renal prognosis. Renin-angiotensin system antagonists help to control proteinuria and slow the progression of fibrosis. Symptomatic management may include the use of diuretics, statins, infection prophylaxis and anticoagulation. This Primer discusses a shift in paradigm from patient stratification based on kidney biopsy findings towards personalized management based on clinical, morphological and genetic data as well as pathophysiological understanding.

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Section: Cushingoid Featuresmentioning

confidence: 99%