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Multisystem inflammatory syndrome in children

MedGen UID:
1713342
Concept ID:
C5391534
Disease or Syndrome
Synonyms: MIS-C associated with COVID-19; MISC associated with COVID-19; multi-system inflammatory disease, pediatric, COVID-19 related; multi-system inflammatory syndrome, pediatric, COVID-19 related; multisystem inflammatory disease, pediatric, COVID-19 related; multisystem inflammatory syndrome in children MIS-C associated with COVID-19; multisystem inflammatory syndrome, pediatric, COVID-19 related; pediatric multi-system inflammatory disease, COVID-19 related; pediatric multi-system inflammatory syndrome, COVID-19 related; pediatric multi-system inflammatory syndrome, SARS-CoV-2 related; pediatric multisystem inflammatory disease, COVID-19 related; pediatric multisystem inflammatory syndrome, COVID-19 related; pediatric multisystem inflammatory syndrome, SARS-CoV-2 related; PIMS-TS
SNOMED CT: Pediatric multisystem inflammatory syndrome (895448002); MIS-C - multisystem inflammatory syndrome in children (895448002); Multisystem inflammatory syndrome in children (895448002); Multi-system inflammatory syndrome in children (895448002); PIMS-TS - pediatric inflammatory multisystem syndrome temporally associated with COVID-19 (895448002); PIMS - pediatric inflammatory multisystem syndrome (895448002)

Definition

A rare syndrome temporally associated with COVID-19 in children, marked by persistent fever, inflammation (neutrophilia, elevated C-reactive protein (CRP), and lymphopenia), poor function in one or more organs, and other specific clinical and laboratory features not attributable to other infections. The characteristics of this syndrome appear to be similar to toxic shock syndrome and Kawasaki disease. The following variable signs and symptoms have been most commonly reported to date: coagulopathy, cardiac dysfunction, diarrhea, abdominal distension, other GI symptoms (with some children having positive stool tests for SARS-CoV-2), and acute kidney injury. Respiratory symptoms are not always a prominent feature in these cases. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Multisystem inflammatory syndrome in children

Professional guidelines

PubMed

Kuo HC
Int J Mol Sci 2023 Sep 11;24(18) doi: 10.3390/ijms241813948. PMID: 37762250Free PMC Article
Santos MO, Gonçalves LC, Silva PAN, Moreira ALE, Ito CRM, Peixoto FAO, Wastowski IJ, Carneiro LC, Avelino MAG
J Pediatr (Rio J) 2022 Jul-Aug;98(4):338-349. Epub 2021 Dec 3 doi: 10.1016/j.jped.2021.08.006. PMID: 34863701Free PMC Article
Kabeerdoss J, Pilania RK, Karkhele R, Kumar TS, Danda D, Singh S
Rheumatol Int 2021 Jan;41(1):19-32. Epub 2020 Nov 21 doi: 10.1007/s00296-020-04749-4. PMID: 33219837Free PMC Article

Recent clinical studies

Etiology

Rao S, Gross RS, Mohandas S, Stein CR, Case A, Dreyer B, Pajor NM, Bunnell HT, Warburton D, Berg E, Overdevest JB, Gorelik M, Milner J, Saxena S, Jhaveri R, Wood JC, Rhee KE, Letts R, Maughan C, Guthe N, Castro-Baucom L, Stockwell MS
Pediatrics 2024 Mar 1;153(3) doi: 10.1542/peds.2023-062570. PMID: 38321938Free PMC Article
Kuo HC
Int J Mol Sci 2023 Sep 11;24(18) doi: 10.3390/ijms241813948. PMID: 37762250Free PMC Article
Zhang HP, Sun YL, Wang YF, Yazici D, Azkur D, Ogulur I, Azkur AK, Yang ZW, Chen XX, Zhang AZ, Hu JQ, Liu GH, Akdis M, Akdis CA, Gao YD
Allergy 2023 Feb;78(2):369-388. Epub 2022 Dec 5 doi: 10.1111/all.15593. PMID: 36420736Free PMC Article
Nikolopoulou GB, Maltezou HC
Arch Med Res 2022 Jan;53(1):1-8. Epub 2021 Jul 6 doi: 10.1016/j.arcmed.2021.07.002. PMID: 34311990Free PMC Article
Cabler SS, French AR, Orvedahl A
Trends Mol Med 2020 Dec;26(12):1078-1085. Epub 2020 Sep 30 doi: 10.1016/j.molmed.2020.09.012. PMID: 33051104Free PMC Article

Diagnosis

Kuo HC
Int J Mol Sci 2023 Sep 11;24(18) doi: 10.3390/ijms241813948. PMID: 37762250Free PMC Article
Noval Rivas M, Arditi M
Rheum Dis Clin North Am 2023 Aug;49(3):647-659. Epub 2023 Mar 17 doi: 10.1016/j.rdc.2023.03.002. PMID: 37331738Free PMC Article
Long A, Kleiner A, Looney RJ
J Allergy Clin Immunol 2023 Jan;151(1):70-80. doi: 10.1016/j.jaci.2022.11.001. PMID: 36608984
Patel JM
Curr Allergy Asthma Rep 2022 May;22(5):53-60. Epub 2022 Mar 22 doi: 10.1007/s11882-022-01031-4. PMID: 35314921Free PMC Article
Hoste L, Van Paemel R, Haerynck F
Eur J Pediatr 2021 Jul;180(7):2019-2034. Epub 2021 Feb 18 doi: 10.1007/s00431-021-03993-5. PMID: 33599835Free PMC Article

Therapy

Zhang HP, Sun YL, Wang YF, Yazici D, Azkur D, Ogulur I, Azkur AK, Yang ZW, Chen XX, Zhang AZ, Hu JQ, Liu GH, Akdis M, Akdis CA, Gao YD
Allergy 2023 Feb;78(2):369-388. Epub 2022 Dec 5 doi: 10.1111/all.15593. PMID: 36420736Free PMC Article
Zachariah P
Infect Dis Clin North Am 2022 Mar;36(1):1-14. Epub 2021 Nov 15 doi: 10.1016/j.idc.2021.11.002. PMID: 35168704Free PMC Article
Son MBF, Murray N, Friedman K, Young CC, Newhams MM, Feldstein LR, Loftis LL, Tarquinio KM, Singh AR, Heidemann SM, Soma VL, Riggs BJ, Fitzgerald JC, Kong M, Doymaz S, Giuliano JS Jr, Keenaghan MA, Hume JR, Hobbs CV, Schuster JE, Clouser KN, Hall MW, Smith LS, Horwitz SM, Schwartz SP, Irby K, Bradford TT, Maddux AB, Babbitt CJ, Rowan CM, McLaughlin GE, Yager PH, Maamari M, Mack EH, Carroll CL, Montgomery VL, Halasa NB, Cvijanovich NZ, Coates BM, Rose CE, Newburger JW, Patel MM, Randolph AG; Overcoming COVID-19 Investigators
N Engl J Med 2021 Jul 1;385(1):23-34. Epub 2021 Jun 16 doi: 10.1056/NEJMoa2102605. PMID: 34133855Free PMC Article
Kabeerdoss J, Pilania RK, Karkhele R, Kumar TS, Danda D, Singh S
Rheumatol Int 2021 Jan;41(1):19-32. Epub 2020 Nov 21 doi: 10.1007/s00296-020-04749-4. PMID: 33219837Free PMC Article
Feldstein LR, Rose EB, Horwitz SM, Collins JP, Newhams MM, Son MBF, Newburger JW, Kleinman LC, Heidemann SM, Martin AA, Singh AR, Li S, Tarquinio KM, Jaggi P, Oster ME, Zackai SP, Gillen J, Ratner AJ, Walsh RF, Fitzgerald JC, Keenaghan MA, Alharash H, Doymaz S, Clouser KN, Giuliano JS Jr, Gupta A, Parker RM, Maddux AB, Havalad V, Ramsingh S, Bukulmez H, Bradford TT, Smith LS, Tenforde MW, Carroll CL, Riggs BJ, Gertz SJ, Daube A, Lansell A, Coronado Munoz A, Hobbs CV, Marohn KL, Halasa NB, Patel MM, Randolph AG; Overcoming COVID-19 Investigators; CDC COVID-19 Response Team
N Engl J Med 2020 Jul 23;383(4):334-346. Epub 2020 Jun 29 doi: 10.1056/NEJMoa2021680. PMID: 32598831Free PMC Article

Prognosis

Santos MO, Gonçalves LC, Silva PAN, Moreira ALE, Ito CRM, Peixoto FAO, Wastowski IJ, Carneiro LC, Avelino MAG
J Pediatr (Rio J) 2022 Jul-Aug;98(4):338-349. Epub 2021 Dec 3 doi: 10.1016/j.jped.2021.08.006. PMID: 34863701Free PMC Article
Nikolopoulou GB, Maltezou HC
Arch Med Res 2022 Jan;53(1):1-8. Epub 2021 Jul 6 doi: 10.1016/j.arcmed.2021.07.002. PMID: 34311990Free PMC Article
Ramaswamy A, Brodsky NN, Sumida TS, Comi M, Asashima H, Hoehn KB, Li N, Liu Y, Shah A, Ravindra NG, Bishai J, Khan A, Lau W, Sellers B, Bansal N, Guerrerio P, Unterman A, Habet V, Rice AJ, Catanzaro J, Chandnani H, Lopez M, Kaminski N, Dela Cruz CS, Tsang JS, Wang Z, Yan X, Kleinstein SH, van Dijk D, Pierce RW, Hafler DA, Lucas CL
Immunity 2021 May 11;54(5):1083-1095.e7. Epub 2021 Apr 13 doi: 10.1016/j.immuni.2021.04.003. PMID: 33891889Free PMC Article
Hoste L, Van Paemel R, Haerynck F
Eur J Pediatr 2021 Jul;180(7):2019-2034. Epub 2021 Feb 18 doi: 10.1007/s00431-021-03993-5. PMID: 33599835Free PMC Article
Feldstein LR, Rose EB, Horwitz SM, Collins JP, Newhams MM, Son MBF, Newburger JW, Kleinman LC, Heidemann SM, Martin AA, Singh AR, Li S, Tarquinio KM, Jaggi P, Oster ME, Zackai SP, Gillen J, Ratner AJ, Walsh RF, Fitzgerald JC, Keenaghan MA, Alharash H, Doymaz S, Clouser KN, Giuliano JS Jr, Gupta A, Parker RM, Maddux AB, Havalad V, Ramsingh S, Bukulmez H, Bradford TT, Smith LS, Tenforde MW, Carroll CL, Riggs BJ, Gertz SJ, Daube A, Lansell A, Coronado Munoz A, Hobbs CV, Marohn KL, Halasa NB, Patel MM, Randolph AG; Overcoming COVID-19 Investigators; CDC COVID-19 Response Team
N Engl J Med 2020 Jul 23;383(4):334-346. Epub 2020 Jun 29 doi: 10.1056/NEJMoa2021680. PMID: 32598831Free PMC Article

Clinical prediction guides

Rao S, Gross RS, Mohandas S, Stein CR, Case A, Dreyer B, Pajor NM, Bunnell HT, Warburton D, Berg E, Overdevest JB, Gorelik M, Milner J, Saxena S, Jhaveri R, Wood JC, Rhee KE, Letts R, Maughan C, Guthe N, Castro-Baucom L, Stockwell MS
Pediatrics 2024 Mar 1;153(3) doi: 10.1542/peds.2023-062570. PMID: 38321938Free PMC Article
Kuo HC
Int J Mol Sci 2023 Sep 11;24(18) doi: 10.3390/ijms241813948. PMID: 37762250Free PMC Article
Godfred-Cato S, Abrams JY, Balachandran N, Jaggi P, Jones K, Rostad CA, Lu AT, Fan L, Jabbar A, Anderson EJ, Kao CM, Hunstad DA, Rosenberg RB, Zafferani MJ, Ede KC, Ballan W, Laham FR, Beltran Y, Bryant B, Meng L, Hammett TA, Oster ME, Bamrah Morris S, Belay ED
Pediatr Infect Dis J 2022 Apr 1;41(4):315-323. doi: 10.1097/INF.0000000000003449. PMID: 35093995Free PMC Article
Nikolopoulou GB, Maltezou HC
Arch Med Res 2022 Jan;53(1):1-8. Epub 2021 Jul 6 doi: 10.1016/j.arcmed.2021.07.002. PMID: 34311990Free PMC Article
Feldstein LR, Rose EB, Horwitz SM, Collins JP, Newhams MM, Son MBF, Newburger JW, Kleinman LC, Heidemann SM, Martin AA, Singh AR, Li S, Tarquinio KM, Jaggi P, Oster ME, Zackai SP, Gillen J, Ratner AJ, Walsh RF, Fitzgerald JC, Keenaghan MA, Alharash H, Doymaz S, Clouser KN, Giuliano JS Jr, Gupta A, Parker RM, Maddux AB, Havalad V, Ramsingh S, Bukulmez H, Bradford TT, Smith LS, Tenforde MW, Carroll CL, Riggs BJ, Gertz SJ, Daube A, Lansell A, Coronado Munoz A, Hobbs CV, Marohn KL, Halasa NB, Patel MM, Randolph AG; Overcoming COVID-19 Investigators; CDC COVID-19 Response Team
N Engl J Med 2020 Jul 23;383(4):334-346. Epub 2020 Jun 29 doi: 10.1056/NEJMoa2021680. PMID: 32598831Free PMC Article

Recent systematic reviews

Piechotta V, Siemens W, Thielemann I, Toews M, Koch J, Vygen-Bonnet S, Kothari K, Grummich K, Braun C, Kapp P, Labonté V, Wichmann O, Meerpohl JJ, Harder T
Lancet Child Adolesc Health 2023 Jun;7(6):379-391. Epub 2023 Apr 18 doi: 10.1016/S2352-4642(23)00078-0. PMID: 37084750Free PMC Article
Arantes Junior MAF, Conegundes AF, Branco Miranda BC, Radicchi Campos ASR, França Vieira AL, Faleiro MD, Campos MA, Kroon EG, Bentes AA
Rev Med Virol 2023 May;33(3):e2432. Epub 2023 Feb 18 doi: 10.1002/rmv.2432. PMID: 36807958
Watanabe A, Kani R, Iwagami M, Takagi H, Yasuhara J, Kuno T
JAMA Pediatr 2023 Apr 1;177(4):384-394. doi: 10.1001/jamapediatrics.2022.6243. PMID: 36689319Free PMC Article
Santos MO, Gonçalves LC, Silva PAN, Moreira ALE, Ito CRM, Peixoto FAO, Wastowski IJ, Carneiro LC, Avelino MAG
J Pediatr (Rio J) 2022 Jul-Aug;98(4):338-349. Epub 2021 Dec 3 doi: 10.1016/j.jped.2021.08.006. PMID: 34863701Free PMC Article
Hoste L, Van Paemel R, Haerynck F
Eur J Pediatr 2021 Jul;180(7):2019-2034. Epub 2021 Feb 18 doi: 10.1007/s00431-021-03993-5. PMID: 33599835Free PMC Article

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