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Increased circulating IgG3 level

MedGen UID:
1687845
Concept ID:
C5139254
Finding
HPO: HP:0032297

Definition

An abnormally increased concentration of the IgG3 subtype in the blood circulation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased circulating IgG3 level

Conditions with this feature

Combined immunodeficiency due to STIM1 deficiency
MedGen UID:
440575
Concept ID:
C2748557
Disease or Syndrome
Immunodeficiency-10 (IMD10) is an autosomal recessive primary immunodeficiency characterized by onset of recurrent infections in childhood due to defective T- and NK-cell function, although the severity is variable. Affected individuals may also have hypotonia, hypohidrosis, or dental enamel hypoplasia consistent with amelogenesis imperfecta (summary by Parry et al., 2016).
Immunodeficiency 95
MedGen UID:
1802205
Concept ID:
C5676929
Disease or Syndrome
Immunodeficiency-95 (IMD95) is an autosomal recessive disorder characterized predominantly by the onset of recurrent and severe viral respiratory infections in infancy or early childhood. Affected individuals often require hospitalization or respiratory support for these infections, which include human rhinovirus (HRV) and RSV. Immunologic workup is usually normal, although some mild abnormalities may be observed. The disorder results from a loss of ability of the innate immune system to sense viral genetic information, which causes a lack of interferon (IFN) production, poor response to viral and immunologic stimulation, and failure to control viral replication (summary by Lamborn et al., 2017, Asgari et al., 2017, Cananzi et al., 2021).

Professional guidelines

PubMed

Sloan EE, Kmetova K, NaveenKumar SK, Kluge L, Chong E, Hoy CK, Yalavarthi S, Sarosh C, Baisch J, Walters L, Nassi L, Fuller J, Turnier JL, Pascual V, Wright TB, Madison JA, Knight JS, Zia A, Zuo Y
Clin Immunol 2024 Apr;261:109926. Epub 2024 Feb 13 doi: 10.1016/j.clim.2024.109926. PMID: 38355030
Fedele G, Schiavoni I, Trentini F, Leone P, Olivetta E, Fallucca A, Fiore S, Di Martino A, Abrignani S, Baldo V, Baldovin T, Bandera A, Clerici P, De Paschale M, Diaco F, Domnich A, Fortunato F, Giberti I, Gori A, Grifantini R, Lazzarotto T, Lodi V, Mastroianni CM, Prato R, Restivo V, Vitale F, Brusaferro S, Merler S, Palamara AT, Stefanelli P; Study Group for the Immunological Monitoring post Covid19 vaccination
Front Immunol 2023;14:1272119. Epub 2023 Nov 20 doi: 10.3389/fimmu.2023.1272119. PMID: 38077369Free PMC Article
Handler MZ, Schwartz RA
J Eur Acad Dermatol Venereol 2014 Nov;28(11):1418-23. Epub 2014 May 20 doi: 10.1111/jdv.12541. PMID: 24841497

Recent clinical studies

Etiology

Xiao N, He X, Niu H, Yu H, Cui N, Li H, Yan L, Shao Z, Xing L, Wang H
J Immunol Res 2021;2021:4302515. Epub 2021 Sep 30 doi: 10.1155/2021/4302515. PMID: 34631897Free PMC Article
Shilova N, Huflejt ME, Vuskovic M, Obukhova P, Navakouski M, Khasbiullina N, Pazynina G, Galanina O, Bazhenov A, Bovin N
Top Curr Chem 2015;366:169-81. doi: 10.1007/128_2013_469. PMID: 24037491
Handler MZ, Schwartz RA
J Eur Acad Dermatol Venereol 2014 Nov;28(11):1418-23. Epub 2014 May 20 doi: 10.1111/jdv.12541. PMID: 24841497
Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T, González-Peralta RP, Kelly D, Mohan N, Shah U, Murray KF
J Pediatr Gastroenterol Nutr 2009 Aug;49(2):158-64. doi: 10.1097/MPG.0b013e3181a1c265. PMID: 19561543
Cohen AJ, Kessler CM
Baillieres Clin Haematol 1996 Jun;9(2):331-54. doi: 10.1016/s0950-3536(96)80067-9. PMID: 8800509

Diagnosis

Aranda CJ, Gonzalez-Kozlova E, Saunders SP, Fernandes-Braga W, Ota M, Narayanan S, He JS, Del Duca E, Swaroop B, Gnjatic S, Shattner G, Reibman J, Soter NA, Guttman-Yassky E, Curotto de Lafaille MA
Allergy 2023 Mar;78(3):752-766. Epub 2022 Dec 19 doi: 10.1111/all.15601. PMID: 36445014Free PMC Article
Shilova N, Huflejt ME, Vuskovic M, Obukhova P, Navakouski M, Khasbiullina N, Pazynina G, Galanina O, Bazhenov A, Bovin N
Top Curr Chem 2015;366:169-81. doi: 10.1007/128_2013_469. PMID: 24037491
Handler MZ, Schwartz RA
J Eur Acad Dermatol Venereol 2014 Nov;28(11):1418-23. Epub 2014 May 20 doi: 10.1111/jdv.12541. PMID: 24841497
Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T, González-Peralta RP, Kelly D, Mohan N, Shah U, Murray KF
J Pediatr Gastroenterol Nutr 2009 Aug;49(2):158-64. doi: 10.1097/MPG.0b013e3181a1c265. PMID: 19561543
Cohen AJ, Kessler CM
Baillieres Clin Haematol 1996 Jun;9(2):331-54. doi: 10.1016/s0950-3536(96)80067-9. PMID: 8800509

Therapy

Szepanowski F, Warnke C, Meyer Zu Hörste G, Mausberg AK, Hartung HP, Kleinschnitz C, Stettner M
CNS Drugs 2021 Nov;35(11):1173-1188. Epub 2021 Oct 16 doi: 10.1007/s40263-021-00863-4. PMID: 34657228Free PMC Article
Zhang Y, Fan C, Zhang L, Ma X
Prog Mol Biol Transl Sci 2019;163:471-485. Epub 2019 Mar 25 doi: 10.1016/bs.pmbts.2019.03.004. PMID: 31030759
Handler MZ, Schwartz RA
J Eur Acad Dermatol Venereol 2014 Nov;28(11):1418-23. Epub 2014 May 20 doi: 10.1111/jdv.12541. PMID: 24841497
Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T, González-Peralta RP, Kelly D, Mohan N, Shah U, Murray KF
J Pediatr Gastroenterol Nutr 2009 Aug;49(2):158-64. doi: 10.1097/MPG.0b013e3181a1c265. PMID: 19561543
Cohen AJ, Kessler CM
Baillieres Clin Haematol 1996 Jun;9(2):331-54. doi: 10.1016/s0950-3536(96)80067-9. PMID: 8800509

Prognosis

Xia C, Liu C, Liu Y, Long Y, Xu L, Liu C
J Immunol Res 2020;2020:3757015. Epub 2020 Nov 27 doi: 10.1155/2020/3757015. PMID: 33313326Free PMC Article
Alesaeidi S, Darvishi M, Dabiri S, GhasemiAdl M, Jafarieh A, Tavakolpour S
Curr Rheumatol Rev 2020;16(4):257-266. doi: 10.2174/1573397116666200331130919. PMID: 32228425
Handler MZ, Schwartz RA
J Eur Acad Dermatol Venereol 2014 Nov;28(11):1418-23. Epub 2014 May 20 doi: 10.1111/jdv.12541. PMID: 24841497
Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T, González-Peralta RP, Kelly D, Mohan N, Shah U, Murray KF
J Pediatr Gastroenterol Nutr 2009 Aug;49(2):158-64. doi: 10.1097/MPG.0b013e3181a1c265. PMID: 19561543
Cohen AJ, Kessler CM
Baillieres Clin Haematol 1996 Jun;9(2):331-54. doi: 10.1016/s0950-3536(96)80067-9. PMID: 8800509

Clinical prediction guides

McQuiston A, Scott D, Nord D, Langerude L, Pelaez A, Machuca T, Mehta A, Drake RR, Christie JD, Angel P, Atkinson C
Transpl Immunol 2022 Apr;71:101491. Epub 2021 Nov 10 doi: 10.1016/j.trim.2021.101491. PMID: 34767945
Xia C, Liu C, Liu Y, Long Y, Xu L, Liu C
J Immunol Res 2020;2020:3757015. Epub 2020 Nov 27 doi: 10.1155/2020/3757015. PMID: 33313326Free PMC Article
Alesaeidi S, Darvishi M, Dabiri S, GhasemiAdl M, Jafarieh A, Tavakolpour S
Curr Rheumatol Rev 2020;16(4):257-266. doi: 10.2174/1573397116666200331130919. PMID: 32228425
Kamekura R, Takahashi H, Ichimiya S
Curr Opin Rheumatol 2019 Jan;31(1):9-15. doi: 10.1097/BOR.0000000000000558. PMID: 30422824Free PMC Article
Shilova N, Huflejt ME, Vuskovic M, Obukhova P, Navakouski M, Khasbiullina N, Pazynina G, Galanina O, Bazhenov A, Bovin N
Top Curr Chem 2015;366:169-81. doi: 10.1007/128_2013_469. PMID: 24037491

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