Format

Send to

Choose Destination

See 1 citation found using an alternative search:

Clin J Am Soc Nephrol. 2017 Feb 7;12(2):332-345. doi: 10.2215/CJN.05000516. Epub 2016 Dec 9.

Minimal Change Disease.

Author information

1
Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; and marina.vivarelli@opbg.net.
2
Division of Nephrology and Dialysis, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; and.
3
Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", IRCCS - Istituto di Ricerche Farmacologiche Mario Negri, Ranica, Bergamo, Italy.

Abstract

Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses. Therefore, in this setting steroid-sensitive NS can be considered synonymous with MCD. The pathologic hallmark of disease is absence of visible alterations by light microscopy and effacement of foot processes by electron microscopy. Although the cause is unknown and it is likely that different subgroups of disease recognize a different pathogenesis, immunologic dysregulation and modifications of the podocyte are thought to synergize in altering the integrity of the glomerular basement membrane and therefore determining proteinuria. The mainstay of therapy is prednisone, but steroid-sensitive forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immunosuppression. The outcome is variable, but forms of MCD that respond to steroids usually do not lead to chronic renal damage, whereas forms that are unresponsive to steroids may subsequently reveal themselves as FSGS. However, in a substantial number of patients the disease is recurrent and requires long-term immunosuppression, with significant morbidity because of side effects. Recent therapeutic advances, such as the use of anti-CD20 antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis.

KEYWORDS:

nephrotic syndrome; pathology; pediatric nephrology; podocyte; proteinuria; renal

PMID:
27940460
PMCID:
PMC5293332
DOI:
10.2215/CJN.05000516
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for HighWire Icon for PubMed Central
Loading ...
Support Center