1. World Kidney Day 2016: Kidney Disease & Children
World Kidney Day 2016: Kidney Disease & Children. Act Early to Prevent It!

2. World Kidney Day 2016: Kidney Disease & Children
World Kidney Day 2016: Kidney Disease & Children. Act Early to Prevent It!
Kidney disease affects millions of people worldwide, including many children who may be at risk at an early age. It is therefore crucial that we encourage and facilitate education, early detection and a healthy life style in children, to fight the increase of preventable kidney diseases and to treat children with inborn and acquired disorders of the kidneys worldwide.

3. Pediatric Kidney Disease – An Update
Edited by the ASPN Clinical Affairs Committee

4. Nephrotic Syndrome (NS)
Juan Kupferman, MD
Albert Einstein School of Medicine

5. NS: Background
NS is defined by four clinico- biochemical characteristics
Nephrotic-range proteinuria
Hypoalbuminemia
Edema (not always present)
Hyperlipidemia (not always present)
NS is caused by renal diseases that increase the permeability across the glomerular filtration barrier
Normal
Fusion of podocytes

6. NS: Background NS can be primary or secondary to a systemic disease
Idiopathic NS (INS) is the most common form in childhood and include 3 histologic variants
minimal change disease (most common)
focal segmental glomerulosclerosis (worse prognosis)
mesangial proliferation
It is not known whether these variants are separate disorders or spectrum of a single disease
Minimal change disease
Focal segmental sclerosis
Mesangial proliferation

7. NS: Treatment
Response to steroid therapy is useful to predict long-term prognosis (avoiding the need for an initial kidney biopsy in the majority of patients)
Children with NS can be classified based on their response to steroids
Steroid-responsive
Mostly children with minimal change disease
Better prognosis
Steroid-resistant: will undergo kidney biopsy for histologic diagnosis
Mostly children with FSGS
Worse outcome, many progress to end-stage kidney disease over time

8. NS: Treatment Treatment goals
Induce and maintain remission of proteinuria and its complications
Avoid/minimize steroid toxicity
If steroid toxicity: consider steroid-sparing agents (i.e. alkylating agents, mycophenolate mofetil, calcineurin inhibitors, levamisole, or lastly, rituximab)
If steroid resistance: consider alternative tx (i.e. calcineurin inhibitors, mycophenolate mofetil) + low-dose steroids + angiotensin converting enzyme inhibitors

9. NS: New developments
Urine biomarkers, such as NGAL (neutrophil gelatinase- associated lipocalin) may distinguish steroid sensitivity in idiopathic NS
Bennett et al. Urine biomarkers. Pediatr Nephrol 2012
Novel therapies for FSGS, such as modulators of immune system/inflammation (i.e. rituximab, ACTH, abatacept, adalimumab), antagonists of circulating factors (i.e. galactose, inhibitors of suPAR), anti-fibrotic agents (i.e. TGF-β inhibitors), and stem cell therapy, are in pre-clinical or clinical evaluation
Malaga-Dieguez et al. Novel therapies. Adv Chron Kid Dis 2015
References:
Gipson et al. Nephrotic syndrome. Pediatrics 2009
KDIGO guidelines at
Bennett et al. Urine biomarkers. Pediatr Nephrol 2012
Lombel et al. KDIGO. Pediatr Nephrol 2013
Malaga-Dieguez et al. Novel therapies. Adv Chron Kid Dis 2015
Ravani et al. Rituximab in children. JASN 2015
Niaudet. UpToDate 2016

10. NS: Summary Response to steroids still the major prognostic factor
Goals of therapy are cessation of proteinuria and to minimize toxicity from treatment medications
Novel biomarkers and medications are being developed to enhance our ability to diagnose, predict prognosis, and treat.

11. Happy World Kidney Day 2016!
Your pediatric nephrology community continues to work hard to improve clinical care, foster education, and advance the science regarding kidney disease in children! We appreciate your support and all you do for children’s health care!