[Minimal change disease and focal segmental glomerulosclerosis]
- PMID: 30887070
- DOI: 10.1007/s00108-019-0590-y
[Minimal change disease and focal segmental glomerulosclerosis]
Abstract
Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults. Both disease entities resemble each other and can sometimes only be discriminated on the basis of their clinical courses. MCD and FSGS display two classical examples that share a common pathophysiology in which the glomerular podocyte and the cytoskeleton of its foot processes play important roles. Therefore, the term "podocytopathy" was introduced for both diseases. In this article, we compare their differences and similarities, and summarized new data on pathophysiology and treatment. In adults, only a renal biopsy including electron microscopy allows for the discrimination of MCD and FSGS and other differential diagnoses. The identification of a primary or secondary form of the disease is based on the clinical course. Data from studies on the treatment are sparse; hence, treatment is still based on high-dose steroids followed by additional immunosuppressive agents. In secondary forms, treatment of the underlying disease is elementary.
Keywords: Biopsy, kidney; Immunosuppression; Nephrotic syndrome; Podocytes; Steroids.
Similar articles
-
[Nephrotic syndrome in minimal change disease (kidney), focal segmental glomerulosclerosis, and focal mesangioproliferative glomerulonephritis].Ter Arkh. 2002;74(6):31-3. Ter Arkh. 2002. PMID: 12136479 Russian.
-
Indications for kidney biopsy in idiopathic childhood nephrotic syndrome.Pediatr Nephrol. 2017 Oct;32(10):1897-1905. doi: 10.1007/s00467-017-3687-3. Epub 2017 May 24. Pediatr Nephrol. 2017. PMID: 28540445
-
Ezrin--a useful factor in the prognosis of nephrotic syndrome in children: an immunohistochemical approach.J Clin Pathol. 2006 Sep;59(9):916-20. doi: 10.1136/jcp.2005.031732. Epub 2006 Mar 7. J Clin Pathol. 2006. PMID: 16522749 Free PMC article.
-
Emerging therapeutic strategies for minimal change disease and focal and segmental glomerulosclerosis.Expert Opin Investig Drugs. 2018 Nov;27(11):839-879. doi: 10.1080/13543784.2018.1540587. Epub 2018 Oct 30. Expert Opin Investig Drugs. 2018. PMID: 30360670 Review.
-
Renal disease. II. The treatment of minimal change nephropathy and focal segmental glomerulosclerosis.J R Coll Physicians Lond. 1997 Mar-Apr;31(2):137-41. J R Coll Physicians Lond. 1997. PMID: 9131509 Free PMC article. Review. No abstract available.
Cited by
-
Mycophenolate mofetil and telmisartan for the treatment of proteinuria secondary to minimal change disease podocytopathy in a dog.J Vet Intern Med. 2022 Nov;36(6):2187-2190. doi: 10.1111/jvim.16534. Epub 2022 Sep 24. J Vet Intern Med. 2022. PMID: 36151875 Free PMC article.
-
Understanding the podocyte immune responses in proteinuric kidney diseases: from pathogenesis to therapy.Front Immunol. 2024 Jan 15;14:1335936. doi: 10.3389/fimmu.2023.1335936. eCollection 2023. Front Immunol. 2024. PMID: 38288116 Free PMC article. Review.
-
Minimal Change Disease: Pathogenetic Insights from Glomerular Proteomics.Int J Mol Sci. 2024 May 21;25(11):5613. doi: 10.3390/ijms25115613. Int J Mol Sci. 2024. PMID: 38891801 Free PMC article.
-
Podocyte Sphingolipid Signaling in Nephrotic Syndrome.Cell Physiol Biochem. 2021 Apr 17;55(S4):13-34. doi: 10.33594/000000356. Cell Physiol Biochem. 2021. PMID: 33861526 Free PMC article. Review.
-
Exploring the therapeutic mechanism of Yuebi decoction on nephrotic syndrome based on network pharmacology and experimental study.Aging (Albany NY). 2024 Sep 20;16(18):12623-12650. doi: 10.18632/aging.206116. Epub 2024 Sep 20. Aging (Albany NY). 2024. PMID: 39311772 Free PMC article.
References
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources