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Increased circulating IgG2 level

MedGen UID:
1704424
Concept ID:
C5139256
Finding
HPO: HP:0032299

Definition

An abnormally increased concentration of the IgG2 subtype in the blood circulation. [from HPO]

Term Hierarchy

Conditions with this feature

Neutropenia, severe congenital, 11, autosomal dominant
MedGen UID:
1846394
Concept ID:
C5882742
Disease or Syndrome
Autosomal dominant severe congenital neutropenia-11 (SCN11) is characterized by the onset of recurrent infections, mainly bacterial, in early childhood. Laboratory studies show severe neutropenia due to maturation arrest and impaired development of myeloid cells. Other leukocyte subsets, including B cells and NK cells, may also be subtly affected. Patients should be followed for possible renal dysfunction (Van Nieuwenhove et al., 2020). For discussion of genetic heterogeneity of severe congenital neutropenia, see SCN1 (202700).

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: 38355030Free PMC Article
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

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
Zuo Y, Navaz S, Liang W, Li C, Ayers CR, Rysenga CE, Harbaugh A, Norman GL, Solow EB, Bermas B, Akinmolayemi O, Rohatgi A, Karp DR, Knight JS, de Lemos JA
JAMA Netw Open 2023 Apr 3;6(4):e236530. doi: 10.1001/jamanetworkopen.2023.6530. PMID: 37014642Free PMC Article
Kishnani PS, Rockman-Greenberg C, Rauch F, Bhatti MT, Moseley S, Denker AE, Watsky E, Whyte MP
Bone 2019 Apr;121:149-162. Epub 2018 Dec 18 doi: 10.1016/j.bone.2018.12.011. PMID: 30576866
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
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

Shirai Y, Miura K, Ishizuka K, Ando T, Kanda S, Hashimoto J, Hamasaki Y, Hotta K, Ito N, Honda K, Tanabe K, Takano T, Hattori M
Kidney Int 2024 Mar;105(3):608-617. Epub 2023 Dec 16 doi: 10.1016/j.kint.2023.11.022. PMID: 38110152
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
Kishnani PS, Rockman-Greenberg C, Rauch F, Bhatti MT, Moseley S, Denker AE, Watsky E, Whyte MP
Bone 2019 Apr;121:149-162. Epub 2018 Dec 18 doi: 10.1016/j.bone.2018.12.011. PMID: 30576866
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
Cohen AJ, Kessler CM
Baillieres Clin Haematol 1996 Jun;9(2):331-54. doi: 10.1016/s0950-3536(96)80067-9. PMID: 8800509

Prognosis

Marafini I, Laudisi F, Salvatori S, Lavigna D, Venuto C, Giannarelli D, Monteleone G
Dig Liver Dis 2024 Jan;56(1):55-60. Epub 2023 Jul 6 doi: 10.1016/j.dld.2023.06.024. PMID: 37407314
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
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

Bruschi M, Angeletti A, Prunotto M, Meroni PL, Ghiggeri GM; Zeus consortium, Moroni G, Sinico RA, Franceschini F, Fredi M, Vaglio A, Cavalli A, Scapozza L, Patel JJ, Tan JC, Lo KC, Cavagna L, Petretto A, Pratesi F, Migliorini P, Locatelli F, Pazzola G, Pesce G, Giannese D, Manfredi A, Ramirez GA, Esposito P, Murdaca G, Negrini S, Bui F, Trezzi B, Emmi G, Cavazzana I, Binda V, Fenaroli P, Pisan I, Montecucco C, Santoro D, Scolari F, Mescia F, Volpi S, Mosca M, Tincani A, Ravelli A, Murtas C, Candiano G, Caridi G, La Porta E, Verrina E
Autoimmun Rev 2024 May;23(5):103535. Epub 2024 Mar 27 doi: 10.1016/j.autrev.2024.103535. PMID: 38552995
Bradford HF, Haljasmägi L, Menon M, McDonnell TCR, Särekannu K, Vanker M, Peterson P, Wincup C, Abida R, Gonzalez RF, Bondet V, Duffy D, Isenberg DA, Kisand K, Mauri C
Cell Rep Med 2023 Jan 17;4(1):100894. doi: 10.1016/j.xcrm.2022.100894. PMID: 36652906Free PMC Article
Kishnani PS, Rockman-Greenberg C, Rauch F, Bhatti MT, Moseley S, Denker AE, Watsky E, Whyte MP
Bone 2019 Apr;121:149-162. Epub 2018 Dec 18 doi: 10.1016/j.bone.2018.12.011. PMID: 30576866
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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