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Minimal Change Disease

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Proteinuria: Basic Mechanisms, Pathophysiology and Clinical Relevance

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Abstract

Minimal Change Disease (MDC) is the most common type of nephrotic syndrome in childhood. Currently, advancements in podocyte biology suggest that podocyte may play a pathogenic role in the development of proteinuria in this disease. In the majority of children, MCD presents a relapsing pattern that may persist to adulthood. Steroids are considered the drug of choice to control proteinuria. Recently, new therapeutic modalities including ACTH and rituximab have been tried with variable results. There is a need for multicenter, randomized, controlled studies to define the efficacy of these drugs in MCD.

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Abbreviations

ACTH:

Adrenocorticotrophic hormone

Angptl-4 :

Angiopoietin-like 4

APN:

Arbeitsgemeinschaft für Pädiatrische Nephrologie

ASMase:

Acid-sphingomyelinase

BUN:

Blood urea nitrogen

CCT:

Cortical collecting tubule

CD80 :

Cluster of differentiation 80

CNI:

Calcineurin inhibitor

CTLA-4:

Cytotoxic T-lymphocyte-associated protein 4.

ENaC:

Epithelial sodium channel

FDA:

Food and Drug Administration

FP:

Foot processes

FR:

Frequent relapsing

FR:

Frequent relapsing

FSGS:

Focal segmental glomerulosclerosis

g:

Grams

GBM :

Glomerular basement mkembrane

HSP:

Heparan-sulfate proteoglycans

IFN:

Interferon

Ig:

Immunoglobulin.

IL:

Interleukin

INS:

Idiopathic nephrotic syndrome

ISKDC :

International Study of Kidney Disease of Children

IV:

Intravenous

kDa:

Kilodalton

KDIGO:

Kidney Disease: Improving Global Outcomes

kg:

Kilograms

LPS:

Polysaccharide

ManNAc:

Acetylated N-acetylmannosamine

MC1R:

Mineralcorticoid-1 receptor

MCD:

Minimal Change Disease

mEq:

Milliequivalents

mg:

Milligrams

MMF:

Mycophenolate mofetil

PA:

Puromycin aminoglycoside

PBMC:

Peripheral mononuclear cells

PDGF:

Platelet-derived growth factor

PHN:

Passive Heymann nephritis

pI:

Isoelectric point

Poly:IC:

Polyinosinic:polycytidylic acid

RCT:

Randomized controlled trial

SCID:

Severe combined immunodeficient

SD:

Steroid-dependent

SMPDL-3b:

Sphingomyelin phosphodiesterase acid-like 3 b protein

TG:

Transgenic

TLR:

Toll-like receptor

TNF:

Tumor necrosis factor

URI:

Upper respiratory infection.

VEGF:

Vascular endothelial growth factor

Vs:

Versus

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Authors and Affiliations

  1. Division of Pediatric Nephrology, Department of Pediatrics, University of Florida, 1600 SW Archer Road, HD214, Gainesville, FL, 32607, USA

    Gabriel M. Cara-Fuentes & Eduardo H. Garin

  2. Division of Renal Diseases and Hypertension, Department of Medicine, University of Colorado, Denver, CO, USA

    Richard J. Johnson

Authors
  1. Gabriel M. Cara-Fuentes
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  2. Richard J. Johnson
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  3. Eduardo H. Garin
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Correspondence to Eduardo H. Garin .

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Editors and Affiliations

  1. Division of Renal Diseases and Hypertension, University of Colorado Denver, Aurora, Colorado, USA

    Judith Blaine

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Cara-Fuentes, G.M., Johnson, R.J., Garin, E.H. (2016). Minimal Change Disease. In: Blaine, J. (eds) Proteinuria: Basic Mechanisms, Pathophysiology and Clinical Relevance. Springer, Cham. https://doi.org/10.1007/978-3-319-43359-2_6

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