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Decreased circulating IgG4 level

MedGen UID:
1704015
Concept ID:
C5139156
Finding
Synonym: Decreased IgG4 level in blood
 
HPO: HP:0032138

Definition

A reduction in immunoglobulin levels of the IgG4 subclass in the blood circulation. [from HPO]

Conditions with this feature

Immunodeficiency 61
MedGen UID:
337462
Concept ID:
C1845903
Disease or Syndrome
Immunodeficiency-61 (IMD61) is an X-linked recessive primary immunodeficiency characterized by onset of recurrent infections in early childhood due to impaired antibody production. Affected individuals have normal numbers of circulating B and T cells, but B cells have an intrinsic defect in antibody production (summary by Keller et al., 2018). For a general phenotypic description of X-linked agammaglobulinemia, see 300755.

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
El-On J
Acta Trop 2003 Feb;85(2):243-52. doi: 10.1016/s0001-706x(02)00217-6. PMID: 12606103

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
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
Bajzik V, DeBerg HA, Garabatos N, Rust BJ, Obrien KK, Nguyen QA, O'Rourke C, Smith A, Walker AH, Quinn C, Gersuk VH, Farrington M, Jeong D, Vickery BP, Adelman DC, Wambre E
Allergy 2022 Aug;77(8):2534-2548. Epub 2022 Mar 14 doi: 10.1111/all.15276. PMID: 35266148Free PMC Article
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
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

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
Bajzik V, DeBerg HA, Garabatos N, Rust BJ, Obrien KK, Nguyen QA, O'Rourke C, Smith A, Walker AH, Quinn C, Gersuk VH, Farrington M, Jeong D, Vickery BP, Adelman DC, Wambre E
Allergy 2022 Aug;77(8):2534-2548. Epub 2022 Mar 14 doi: 10.1111/all.15276. PMID: 35266148Free PMC Article
Chen J, Ye H, Xiao W, Mao Y, Ai S, Chen R, Lian X, Shi L, Wang X, Bi S, Yang S, Ji X, Zhang T, Yang H
Front Immunol 2021;12:634847. Epub 2021 May 3 doi: 10.3389/fimmu.2021.634847. PMID: 34012433Free 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

Therapy

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
Bajzik V, DeBerg HA, Garabatos N, Rust BJ, Obrien KK, Nguyen QA, O'Rourke C, Smith A, Walker AH, Quinn C, Gersuk VH, Farrington M, Jeong D, Vickery BP, Adelman DC, Wambre E
Allergy 2022 Aug;77(8):2534-2548. Epub 2022 Mar 14 doi: 10.1111/all.15276. PMID: 35266148Free PMC Article
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
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

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
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
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
El-On J
Acta Trop 2003 Feb;85(2):243-52. doi: 10.1016/s0001-706x(02)00217-6. PMID: 12606103

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
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
Bajzik V, DeBerg HA, Garabatos N, Rust BJ, Obrien KK, Nguyen QA, O'Rourke C, Smith A, Walker AH, Quinn C, Gersuk VH, Farrington M, Jeong D, Vickery BP, Adelman DC, Wambre E
Allergy 2022 Aug;77(8):2534-2548. Epub 2022 Mar 14 doi: 10.1111/all.15276. PMID: 35266148Free 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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