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Decreased circulating IgG3 level

MedGen UID:
1686383
Concept ID:
C5139155
Finding
Synonym: Decreased IgG3 level in blood
 
HPO: HP:0032137

Definition

A reduction in immunoglobulin levels of the IgG3 subclass in the blood circulation. [from HPO]

Conditions with this feature

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

Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article
Zhou J, Zhou Y, Wen J, Sun X, Zhang X
Braz J Med Biol Res 2017 Feb 16;50(2):e5637. doi: 10.1590/1414-431X20165637. PMID: 28225866Free PMC Article
Morselli-Labate AM, Pezzilli R
J Gastroenterol Hepatol 2009 Jan;24(1):15-36. Epub 2008 Dec 2 doi: 10.1111/j.1440-1746.2008.05676.x. PMID: 19067780

Recent clinical studies

Etiology

Podestà MA, Trillini M, Portalupi V, Gennarini A, Tomatis F, Villa A, Perna A, Rubis N, Remuzzi G, Ruggenenti P
Am J Kidney Dis 2024 Mar;83(3):340-349.e1. Epub 2023 Sep 29 doi: 10.1053/j.ajkd.2023.08.010. PMID: 37777061
Graça D, Brglez V, Allouche J, Zorzi K, Fernandez C, Teisseyre M, Cremoni M, Benzaken S, Pradier C, Seitz-Polski B
J Clin Immunol 2023 Nov;43(8):1724-1739. Epub 2023 Aug 22 doi: 10.1007/s10875-023-01558-9. PMID: 37606852Free PMC Article
Cianci R, Mancino G, Galli E, Serone E, Massoud R, D'Addabbo P, Poscia A, Borghetti A, Porzio O, Marmo R, Gambassi G, Frezza D
Gene 2023 Apr 30;862:147254. Epub 2023 Feb 9 doi: 10.1016/j.gene.2023.147254. PMID: 36764340
Li X, Wan X, Liu J, Wang H, Li A, Ke C, Tang S, Zhao W, Cai S, Wan C
Microbiol Spectr 2022 Apr 27;10(2):e0149621. Epub 2022 Mar 21 doi: 10.1128/spectrum.01496-21. PMID: 35311573Free PMC Article
Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article

Diagnosis

Greene SE, Huang Y, Curtis KC, King CL, Fischer PU, Weil GJ
PLoS Negl Trop Dis 2023 Jun;17(6):e0011364. Epub 2023 Jun 7 doi: 10.1371/journal.pntd.0011364. PMID: 37285374Free PMC Article
Jamialahmadi T, Baratzadeh F, Reiner Ž, Mannarino MR, Cardenia V, Simental-Mendía LE, Pirro M, Watts GF, Sahebkar A
Oxid Med Cell Longev 2022;2022:7850659. Epub 2022 Aug 1 doi: 10.1155/2022/7850659. PMID: 35958018Free PMC Article
Li X, Wan X, Liu J, Wang H, Li A, Ke C, Tang S, Zhao W, Cai S, Wan C
Microbiol Spectr 2022 Apr 27;10(2):e0149621. Epub 2022 Mar 21 doi: 10.1128/spectrum.01496-21. PMID: 35311573Free PMC Article
Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article
Lin W, Zhang P, Chen H, Chen Y, Yang H, Zheng W, Zhang X, Zhang F, Zhang W, Lipsky PE
Arthritis Res Ther 2017 Feb 10;19(1):25. doi: 10.1186/s13075-017-1231-2. PMID: 28183334Free PMC Article

Therapy

Podestà MA, Trillini M, Portalupi V, Gennarini A, Tomatis F, Villa A, Perna A, Rubis N, Remuzzi G, Ruggenenti P
Am J Kidney Dis 2024 Mar;83(3):340-349.e1. Epub 2023 Sep 29 doi: 10.1053/j.ajkd.2023.08.010. PMID: 37777061
Jamialahmadi T, Baratzadeh F, Reiner Ž, Mannarino MR, Cardenia V, Simental-Mendía LE, Pirro M, Watts GF, Sahebkar A
Oxid Med Cell Longev 2022;2022:7850659. Epub 2022 Aug 1 doi: 10.1155/2022/7850659. PMID: 35958018Free PMC Article
Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article
Leborgne C, Barbon E, Alexander JM, Hanby H, Delignat S, Cohen DM, Collaud F, Muraleetharan S, Lupo D, Silverberg J, Huang K, van Wittengerghe L, Marolleau B, Miranda A, Fabiano A, Daventure V, Beck H, Anguela XM, Ronzitti G, Armour SM, Lacroix-Desmazes S, Mingozzi F
Nat Med 2020 Jul;26(7):1096-1101. Epub 2020 Jun 1 doi: 10.1038/s41591-020-0911-7. PMID: 32483358
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

Prognosis

Liang P, Huang Q, Xu Y, Chen L, Li J, Xu A, Yang Q
Clin Rheumatol 2023 Apr;42(4):1069-1076. Epub 2022 Dec 30 doi: 10.1007/s10067-022-06457-9. PMID: 36585530
Beran O, Matuskova K, Bartovska Z, Bartos O, Holub M
Bratisl Lek Listy 2022;123(4):244-247. doi: 10.4149/BLL_2022_039. PMID: 35294209
Long Y, Xia C, Sun Y, Ma Y, Xu L, Song Y, Liu C
Immunol Lett 2021 May;233:2-10. Epub 2021 Mar 3 doi: 10.1016/j.imlet.2021.03.001. PMID: 33675861
Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article
Ward ES, Devanaboyina SC, Ober RJ
Mol Immunol 2015 Oct;67(2 Pt A):131-41. Epub 2015 Mar 9 doi: 10.1016/j.molimm.2015.02.007. PMID: 25766596Free PMC Article

Clinical prediction guides

Kuang H, Shen CR, Jia XY, Tan M, Yang XF, Cui Z, Borza DB, Zhao MH
Kidney Int 2023 Dec;104(6):1124-1134. Epub 2023 Aug 19 doi: 10.1016/j.kint.2023.07.023. PMID: 37598856Free PMC Article
Williams LD, Shen X, Sawant SS, Akapirat S, Dahora LC, Tay MZ, Stanfield-Oakley S, Wills S, Goodman D, Tenney D, Spreng RL, Zhang L, Yates NL, Montefiori DC, Eller MA, Easterhoff D, Hope TJ, Rerks-Ngarm S, Pittisuttithum P, Nitayaphan S, Excler JL, Kim JH, Michael NL, Robb ML, O'Connell RJ, Karasavvas N, Vasan S, Ferrari G, Tomaras GD; RV305 study team
PLoS Pathog 2023 May;19(5):e1011359. Epub 2023 May 31 doi: 10.1371/journal.ppat.1011359. PMID: 37256916Free PMC Article
Monzon-Posadas WO, Zorn J, Peters K, Baum M, Proksch H, Schlüter CB, Menting T, Pušnik J, Streeck H
Front Immunol 2023;14:1066123. Epub 2023 Jan 19 doi: 10.3389/fimmu.2023.1066123. PMID: 36742295Free PMC Article
Inoue T, Tanaka Y
Clin Mol Hepatol 2020 Jul;26(3):261-279. Epub 2020 Jun 15 doi: 10.3350/cmh.2020.0032. PMID: 32536045Free PMC Article
Ward ES, Devanaboyina SC, Ober RJ
Mol Immunol 2015 Oct;67(2 Pt A):131-41. Epub 2015 Mar 9 doi: 10.1016/j.molimm.2015.02.007. PMID: 25766596Free PMC Article

Recent systematic reviews

Jamialahmadi T, Baratzadeh F, Reiner Ž, Mannarino MR, Cardenia V, Simental-Mendía LE, Pirro M, Watts GF, Sahebkar A
Oxid Med Cell Longev 2022;2022:7850659. Epub 2022 Aug 1 doi: 10.1155/2022/7850659. PMID: 35958018Free PMC Article
Morselli-Labate AM, Pezzilli R
J Gastroenterol Hepatol 2009 Jan;24(1):15-36. Epub 2008 Dec 2 doi: 10.1111/j.1440-1746.2008.05676.x. PMID: 19067780

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