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Congenital Nephrotic Syndrome - Focal Segmental Glomerulosclerosis

MedGen UID:
882036
Concept ID:
C4055193
Disease or Syndrome
Synonym: Congenital FSGS

Definition

Nephrotic syndrome within the first three months of life, characterized by scarring of the glomerulus, in which only part of the glomerulus is involved, and less than 50% of the glomeruli are affected. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital Nephrotic Syndrome - Focal Segmental Glomerulosclerosis

Professional guidelines

PubMed

Song X, Fang X, Tang X, Cao Q, Zhai Y, Chen J, Liu J, Zhang Z, Xiang T, Qian Y, Wu B, Wang H, Zhou W, Liu C, Shen Q, Xu H, Rao J
Mol Genet Genomic Med 2020 Aug;8(8):e1360. Epub 2020 Jun 16 doi: 10.1002/mgg3.1360. PMID: 32543055Free PMC Article
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167
Lovric S, Ashraf S, Tan W, Hildebrandt F
Nephrol Dial Transplant 2016 Nov;31(11):1802-1813. Epub 2015 Oct 27 doi: 10.1093/ndt/gfv355. PMID: 26507970Free PMC Article

Recent clinical studies

Etiology

Dadgar K, Xue Y, Chung J, Sangar S, Bhatt M, Chan AKC, Geddie H, Chanchlani R
Pediatr Res 2023 May;93(6):1463-1469. Epub 2022 Sep 20 doi: 10.1038/s41390-022-02302-6. PMID: 36127405
Rood IM, Deegens JKJ, Lugtenberg D, Bongers EMHF, Wetzels JFM
Am J Kidney Dis 2019 Mar;73(3):400-403. Epub 2018 Sep 18 doi: 10.1053/j.ajkd.2018.06.034. PMID: 30241959
Lovric S, Ashraf S, Tan W, Hildebrandt F
Nephrol Dial Transplant 2016 Nov;31(11):1802-1813. Epub 2015 Oct 27 doi: 10.1093/ndt/gfv355. PMID: 26507970Free PMC Article
Tasic V, Gucev Z, Polenakovic M
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2015;36(3):5-12. doi: 10.1515/prilozi-2015-0073. PMID: 27442391
Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, Marasà M, Chianca A, Rubis N, Ene-Iordache B, Rudnicki M, Pollastro RM, Capasso G, Pisani A, Pennesi M, Emma F, Remuzzi G; Rituximab in Nephrotic Syndrome of Steroid-Dependent or Frequently Relapsing Minimal Change Disease Or Focal Segmental Glomerulosclerosis (NEMO) Study Group
J Am Soc Nephrol 2014 Apr;25(4):850-63. Epub 2014 Jan 30 doi: 10.1681/ASN.2013030251. PMID: 24480824Free PMC Article

Diagnosis

Campbell RE, Thurman JM
Clin J Am Soc Nephrol 2022 Dec;17(12):1823-1834. Epub 2022 Oct 5 doi: 10.2215/CJN.07180622. PMID: 36198505Free PMC Article
Kostovska I, Trajkovska KT, Topuzovska S, Cekovska S, Labudovic D, Kostovski O, Spasovski G
Adv Clin Chem 2022;108:1-36. Epub 2021 Sep 10 doi: 10.1016/bs.acc.2021.08.001. PMID: 35659057
da Silva Filha R, Burini K, Pires LG, Brant Pinheiro SV, Simões E Silva AC
Curr Pediatr Rev 2022;18(4):251-264. doi: 10.2174/1573396318666220314142713. PMID: 35289253
Harita Y
Clin Exp Nephrol 2018 Jun;22(3):491-500. Epub 2017 Jul 27 doi: 10.1007/s10157-017-1449-y. PMID: 28752288Free PMC Article
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167

Therapy

Dadgar K, Xue Y, Chung J, Sangar S, Bhatt M, Chan AKC, Geddie H, Chanchlani R
Pediatr Res 2023 May;93(6):1463-1469. Epub 2022 Sep 20 doi: 10.1038/s41390-022-02302-6. PMID: 36127405
El-Reshaid K, Al-Bader S, Madda JP
Saudi J Kidney Dis Transpl 2022 Jul-Aug;33(4):509-515. doi: 10.4103/1319-2442.388185. PMID: 37929543
Song X, Fang X, Tang X, Cao Q, Zhai Y, Chen J, Liu J, Zhang Z, Xiang T, Qian Y, Wu B, Wang H, Zhou W, Liu C, Shen Q, Xu H, Rao J
Mol Genet Genomic Med 2020 Aug;8(8):e1360. Epub 2020 Jun 16 doi: 10.1002/mgg3.1360. PMID: 32543055Free PMC Article
Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, Marasà M, Chianca A, Rubis N, Ene-Iordache B, Rudnicki M, Pollastro RM, Capasso G, Pisani A, Pennesi M, Emma F, Remuzzi G; Rituximab in Nephrotic Syndrome of Steroid-Dependent or Frequently Relapsing Minimal Change Disease Or Focal Segmental Glomerulosclerosis (NEMO) Study Group
J Am Soc Nephrol 2014 Apr;25(4):850-63. Epub 2014 Jan 30 doi: 10.1681/ASN.2013030251. PMID: 24480824Free PMC Article
Benchimol C
Curr Opin Pediatr 2003 Apr;15(2):171-80. doi: 10.1097/00008480-200304000-00006. PMID: 12640274

Prognosis

Kostovska I, Trajkovska KT, Topuzovska S, Cekovska S, Labudovic D, Kostovski O, Spasovski G
Adv Clin Chem 2022;108:1-36. Epub 2021 Sep 10 doi: 10.1016/bs.acc.2021.08.001. PMID: 35659057
da Silva Filha R, Burini K, Pires LG, Brant Pinheiro SV, Simões E Silva AC
Curr Pediatr Rev 2022;18(4):251-264. doi: 10.2174/1573396318666220314142713. PMID: 35289253
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167
Park E, Kang HG, Choi YH, Lee KB, Moon KC, Jeong HJ, Nagata M, Cheong HI
Pediatr Nephrol 2017 Sep;32(9):1547-1554. Epub 2017 Apr 12 doi: 10.1007/s00467-017-3657-9. PMID: 28405841
Tasic V, Gucev Z, Polenakovic M
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2015;36(3):5-12. doi: 10.1515/prilozi-2015-0073. PMID: 27442391

Clinical prediction guides

Dadgar K, Xue Y, Chung J, Sangar S, Bhatt M, Chan AKC, Geddie H, Chanchlani R
Pediatr Res 2023 May;93(6):1463-1469. Epub 2022 Sep 20 doi: 10.1038/s41390-022-02302-6. PMID: 36127405
Campbell RE, Thurman JM
Clin J Am Soc Nephrol 2022 Dec;17(12):1823-1834. Epub 2022 Oct 5 doi: 10.2215/CJN.07180622. PMID: 36198505Free PMC Article
da Silva Filha R, Burini K, Pires LG, Brant Pinheiro SV, Simões E Silva AC
Curr Pediatr Rev 2022;18(4):251-264. doi: 10.2174/1573396318666220314142713. PMID: 35289253
Tasic V, Gucev Z, Polenakovic M
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2015;36(3):5-12. doi: 10.1515/prilozi-2015-0073. PMID: 27442391
Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, Marasà M, Chianca A, Rubis N, Ene-Iordache B, Rudnicki M, Pollastro RM, Capasso G, Pisani A, Pennesi M, Emma F, Remuzzi G; Rituximab in Nephrotic Syndrome of Steroid-Dependent or Frequently Relapsing Minimal Change Disease Or Focal Segmental Glomerulosclerosis (NEMO) Study Group
J Am Soc Nephrol 2014 Apr;25(4):850-63. Epub 2014 Jan 30 doi: 10.1681/ASN.2013030251. PMID: 24480824Free PMC Article

Recent systematic reviews

Dadgar K, Xue Y, Chung J, Sangar S, Bhatt M, Chan AKC, Geddie H, Chanchlani R
Pediatr Res 2023 May;93(6):1463-1469. Epub 2022 Sep 20 doi: 10.1038/s41390-022-02302-6. PMID: 36127405
Alwahaibi NY, Al Issaei HK, Al Dhahli BS
Saudi J Kidney Dis Transpl 2019 Jan-Feb;30(1):15-23. PMID: 30804262

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