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Nephrotic syndrome of childhood - steroid sensitive

MedGen UID:
588368
Concept ID:
C0403396
Disease or Syndrome
Synonyms: Nephrotic Syndrome, Steroid-Sensitive; Steroid Sensitive Nephrotic Syndrome; Steroid-Sensitive Nephrotic Syndrome; Steroid-Sensitive Nephrotic Syndromes; Syndrome, Steroid-Sensitive Nephrotic
SNOMED CT: Steroid sensitive nephrotic syndrome of childhood (445119005); Steroid-sensitive nephrotic syndrome (236380004); Steroid-responsive nephrotic syndrome (236380004); SSNS - Steroid-sensitive nephrotic syndrome (236380004)
 
HPO: HP:0025647
Monarch Initiative: MONDO:0044781

Definition

A type of nephrotic syndrome in which complete remission (albumin urine dipstick negative/trace or proteinuria <4mg/m2/d or urinary protein to creatinine ratio <200mg/g (<20 mg/mmol) for 3 consecutive days) is achieved within initial 4 weeks of corticosteroid therapy. [from HPO]

Professional guidelines

PubMed

Barratt TM, Bercowsky A, Osofsky SG, Soothill JF
Lancet 1975 Jan 11;1(7898):55-8. doi: 10.1016/s0140-6736(75)91068-5. PMID: 46019

Recent clinical studies

Etiology

Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
Politano SA, Colbert GB, Hamiduzzaman N
Prim Care 2020 Dec;47(4):597-613. Epub 2020 Sep 26 doi: 10.1016/j.pop.2020.08.002. PMID: 33121631
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Noone DG, Iijima K, Parekh R
Lancet 2018 Jul 7;392(10141):61-74. Epub 2018 Jun 14 doi: 10.1016/S0140-6736(18)30536-1. PMID: 29910038
Iijima K, Sako M, Nozu K
Clin Exp Nephrol 2017 Apr;21(2):193-202. Epub 2016 Jul 15 doi: 10.1007/s10157-016-1313-5. PMID: 27422620Free PMC Article

Diagnosis

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
Politano SA, Colbert GB, Hamiduzzaman N
Prim Care 2020 Dec;47(4):597-613. Epub 2020 Sep 26 doi: 10.1016/j.pop.2020.08.002. PMID: 33121631
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
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

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Noone DG, Iijima K, Parekh R
Lancet 2018 Jul 7;392(10141):61-74. Epub 2018 Jun 14 doi: 10.1016/S0140-6736(18)30536-1. PMID: 29910038
Kodner C
Am Fam Physician 2016 Mar 15;93(6):479-85. PMID: 26977832

Prognosis

Rodriguez-Ballestas E, Reid-Adam J
Pediatr Rev 2022 Feb 1;43(2):87-99. doi: 10.1542/pir.2020-001230. PMID: 35102405
AbuMaziad AS, Abusaleh R, Bhati S
J Perinatol 2021 Dec;41(12):2704-2712. Epub 2022 Jan 4 doi: 10.1038/s41372-021-01279-0. PMID: 34983935
Keri KC, Blumenthal S, Kulkarni V, Beck L, Chongkrairatanakul T
Postgrad Med J 2019 Jan;95(1119):23-31. Epub 2019 Jan 25 doi: 10.1136/postgradmedj-2018-135729. PMID: 30683678
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
Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, Anarat A, Melk A, Azocar M, Oh J, Saeed B, Gheisari A, Caliskan S, Gellermann J, Higuita LMS, Jankauskiene A, Drozdz D, Mir S, Balat A, Szczepanska M, Paripovic D, Zurowska A, Bogdanovic R, Yilmaz A, Ranchin B, Baskin E, Erdogan O, Remuzzi G, Firszt-Adamczyk A, Kuzma-Mroczkowska E, Litwin M, Murer L, Tkaczyk M, Jardim H, Wasilewska A, Printza N, Fidan K, Simkova E, Borzecka H, Staude H, Hees K, Schaefer F; PodoNet Consortium
J Am Soc Nephrol 2017 Oct;28(10):3055-3065. Epub 2017 May 31 doi: 10.1681/ASN.2016101121. PMID: 28566477Free PMC Article

Clinical prediction guides

Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
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
Veltkamp F, Rensma LR, Bouts AHM; LEARNS consortium
Pediatrics 2021 Jul;148(1) Epub 2021 Jun 30 doi: 10.1542/peds.2020-029249. PMID: 34193618
Sanchez-Rodriguez E, Southard CT, Kiryluk K
Clin J Am Soc Nephrol 2021 Mar 8;16(3):458-466. Epub 2020 Jul 17 doi: 10.2215/CJN.14031119. PMID: 32680915Free PMC Article
Warejko JK, Tan W, Daga A, Schapiro D, Lawson JA, Shril S, Lovric S, Ashraf S, Rao J, Hermle T, Jobst-Schwan T, Widmeier E, Majmundar AJ, Schneider R, Gee HY, Schmidt JM, Vivante A, van der Ven AT, Ityel H, Chen J, Sadowski CE, Kohl S, Pabst WL, Nakayama M, Somers MJG, Rodig NM, Daouk G, Baum M, Stein DR, Ferguson MA, Traum AZ, Soliman NA, Kari JA, El Desoky S, Fathy H, Zenker M, Bakkaloglu SA, Müller D, Noyan A, Ozaltin F, Cadnapaphornchai MA, Hashmi S, Hopcian J, Kopp JB, Benador N, Bockenhauer D, Bogdanovic R, Stajić N, Chernin G, Ettenger R, Fehrenbach H, Kemper M, Munarriz RL, Podracka L, Büscher R, Serdaroglu E, Tasic V, Mane S, Lifton RP, Braun DA, Hildebrandt F
Clin J Am Soc Nephrol 2018 Jan 6;13(1):53-62. Epub 2017 Nov 10 doi: 10.2215/CJN.04120417. PMID: 29127259Free PMC Article

Recent systematic reviews

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Hedin E, Bijelić V, Barrowman N, Geier P
Pediatr Nephrol 2022 Aug;37(8):1747-1757. Epub 2022 Mar 3 doi: 10.1007/s00467-021-05358-4. PMID: 35239032
Lee TH, Kuo G, Chang CH, Huang YT, Yen CL, Lee CC, Fan PC, Chen JJ
PLoS One 2021;16(12):e0260312. Epub 2021 Dec 1 doi: 10.1371/journal.pone.0260312. PMID: 34851962Free PMC Article
Gertz MA, Dispenzieri A
JAMA 2020 Jul 7;324(1):79-89. doi: 10.1001/jama.2020.5493. PMID: 32633805
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article

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