Treatment of primary FSGS in adults

SM Korbet - Journal of the American Society of Nephrology, 2012 - journals.lww.com
Over the last 20 years, primary FSGS has emerged as one of the leading causes of
idiopathic nephrotic syndrome in adults, particularly among African Americans. In nephrotic …

Genetic causes of focal segmental glomerulosclerosis: implications for clinical practice

IM Rood, JKJ Deegens… - Nephrology Dialysis …, 2012 - academic.oup.com
Focal segmental glomerulosclerosis (FSGS) is a common cause of steroid-resistant
nephrotic syndrome in children and adults. Although FSGS is considered a podocyte …

Mutation-dependent recessive inheritance of NPHS2-associated steroid-resistant nephrotic syndrome

K Tory, DK Menyhárd, S Woerner, F Nevo… - Nature …, 2014 - nature.com
Monogenic disorders result from defects in a single gene. According to Mendel's laws, these
disorders are inherited in either a recessive or dominant fashion. Autosomal-recessive …

[HTML][HTML] Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis

JKJ Deegens, HBPM Dijkman, GF Borm… - Kidney international, 2008 - Elsevier
Podocyte foot process effacement is characteristic of proteinuric renal diseases. In minimal
change nephrotic syndrome (MCNS) foot processes are diffusely effaced whereas the extent …

[HTML][HTML] A risk allele for focal segmental glomerulosclerosis in African Americans is located within a region containing APOL1 and MYH9

G Genovese, SJ Tonna, AU Knob, GB Appel, A Katz… - Kidney international, 2010 - Elsevier
Genetic variation at the MYH9 locus is linked to the high incidence of focal segmental
glomerulosclerosis (FSGS) and non-diabetic end-stage renal disease among African …

[HTML][HTML] Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant

E Machuca, A Hummel, F Nevo, J Dantal, F Martinez… - Kidney international, 2009 - Elsevier
Mutations of NPHS2, encoding podocin, are the main cause of autosomal recessive steroid-
resistant nephrotic syndrome (NS) presenting in childhood. Adult-onset steroid-resistant NS …

[HTML][HTML] TRPC channels: Regulation, dysregulation and contributions to chronic kidney disease

SE Dryer, H Roshanravan, EY Kim - Biochimica et Biophysica Acta (BBA) …, 2019 - Elsevier
Mutations in the gene encoding canonical transient receptor potential-6 (TRPC6) channels
result in severe nephrotic syndromes that typically lead to end-stage renal disease. Many …

The glomerular permeability factors in idiopathic nephrotic syndrome

JC Davin - Pediatric nephrology, 2016 - Springer
It is currently postulated that steroid-sensitive idiopathic nephrotic syndrome (SSNS) and
steroid-resistant idiopathic nephrotic syndrome (SRNS), which are not related to the …

Clinical and pathological phenotype of genetic causes of focal segmental glomerulosclerosis in adults

N Lepori, L Zand, S Sethi… - Clinical kidney …, 2018 - academic.oup.com
Focal segmental glomerulosclerosis (FSGS) is a histologic lesion resulting from a variety of
pathogenic processes that cause injury to the podocytes. Recently, mutations in more than …

[HTML][HTML] Genetic screening in adolescents with steroid-resistant nephrotic syndrome

BS Lipska, P Iatropoulos, R Maranta, G Caridi… - Kidney international, 2013 - Elsevier
Genetic screening paradigms for congenital and infantile nephrotic syndrome are well
established; however, screening in adolescents has received only minor attention. To help …