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Transient hypogammaglobulinemia

MedGen UID:
167815
•Concept ID:
C0859960
•
Disease or Syndrome
Synonyms: Transient Hypogammaglobulinemia; transient hypogammaglobulinemia
 
Monarch Initiative: MONDO:0003827

Definition

A broad classification for humoral immunodeficiencies. These disorders may be caused by inadequate activation of progenitor B cells, defective class-switching or the effects of medications. Despite the potential for increased susceptibility to infection, these disorders are self-limited with eventual normalization of serum antibody levels. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTransient hypogammaglobulinemia

Professional guidelines

PubMed

All Patients With Common Variable Immunodeficiency Disorders (CVID) Should Be Routinely Offered Diagnostic Genetic Testing.
Ameratunga R, Lehnert K, Woon ST
Front Immunol 2019;10:2678. Epub 2019 Nov 22 doi: 10.3389/fimmu.2019.02678. PMID: 31824486Free PMC Article
The Natural History of Untreated Primary Hypogammaglobulinemia in Adults: Implications for the Diagnosis and Treatment of Common Variable Immunodeficiency Disorders (CVID).
Ameratunga R, Ahn Y, Steele R, Woon ST
Front Immunol 2019;10:1541. Epub 2019 Jul 17 doi: 10.3389/fimmu.2019.01541. PMID: 31379811Free PMC Article

Recent clinical studies

Therapy

Transient hypogammaglobulinemia of infancy and unclassified syndromic immunodeficiencies are highly common in oesophageal atresia patients.
Ulman H, Aygün A, Çağlar D, Dökümcü Z, Topyıldız E, Erdener A, Aksu G, Karaca NE, Özcan C, Kütükçüler N
Scand J Immunol 2024 Feb;99(2):e13338. Epub 2023 Nov 12 doi: 10.1111/sji.13338. PMID: 38981850
Evolution of hypogammaglobulinemia in premature and full-term infants.
Ricci G, Piccinno V, Giannetti A, Miniaci A, Specchia F, Masi M
Int J Immunopathol Pharmacol 2011 Jul-Sep;24(3):721-6. doi: 10.1177/039463201102400318. PMID: 21978704
Transient hypogammaglobulinemia of infancy: intravenous immunoglobulin as first line therapy.
Duse M, Iacobini M, Leonardi L, Smacchia P, Antonetti L, Giancane G
Int J Immunopathol Pharmacol 2010 Jan-Mar;23(1):349-53. doi: 10.1177/039463201002300134. PMID: 20378022
The four most common pediatric immunodeficiencies.
Stiehm RE
Adv Exp Med Biol 2007;601:15-26. doi: 10.1007/978-0-387-72005-0_2. PMID: 17712988
Transient hypogammaglobulinemia of infancy.
Hong R
J Pediatr 1978 Mar;92(3):521-2. doi: 10.1016/s0022-3476(78)80484-3. PMID: 75969

Prognosis

Regulatory T and B cells in transient hypogammaglobulinemia of infancy.
Emsen A, Uçaryılmaz H, Güler T, Artaç H
Turk J Pediatr 2022;64(2):228-238. doi: 10.24953/turkjped.2021.83. PMID: 35611411
Selective IgM deficiency: Follow-up and outcome.
Caka C, Cimen O, Kahyaoğlu P, Tezcan İ, Cagdas D
Pediatr Allergy Immunol 2021 Aug;32(6):1327-1334. Epub 2021 Mar 29 doi: 10.1111/pai.13497. PMID: 33706406
B lymphocyte subsets and outcomes in patients with an initial diagnosis of transient hypogammaglobulinemia of infancy.
Eroglu FK, Aerts Kaya F, Cagdas D, Özgür TT, Yılmaz T, Tezcan İ, Sanal Ö
Scand J Immunol 2018 Oct;88(4):e12709. doi: 10.1111/sji.12709. PMID: 30152873
Transient hypogammaglobulinemia of infancy: predictive factors for late recovery.
Sütçü M, Aktürk H, Salman N, Özçeker D, Gülümser-Şişko S, Acar M, Somer A
Turk J Pediatr 2015 Nov-Dec;57(6):592-598. PMID: 27735798
The four most common pediatric immunodeficiencies.
Stiehm RE
Adv Exp Med Biol 2007;601:15-26. doi: 10.1007/978-0-387-72005-0_2. PMID: 17712988

Clinical prediction guides

Transcriptome profiling of regulatory T cells from children with transient hypogammaglobulinemia of infancy.
Rutkowska-Zapała M, Grabowska A, Lenart M, Kluczewska A, Szaflarska A, Kobylarz K, Pituch-Noworolska A, Siedlar M
Clin Exp Immunol 2023 Dec 13;214(3):275-288. doi: 10.1093/cei/uxad116. PMID: 37936298Free PMC Article
Regulatory T and B cells in transient hypogammaglobulinemia of infancy.
Emsen A, Uçaryılmaz H, Güler T, Artaç H
Turk J Pediatr 2022;64(2):228-238. doi: 10.24953/turkjped.2021.83. PMID: 35611411
Transient hypogammaglobulinemia of infancy: predictive factors for late recovery.
Sütçü M, Aktürk H, Salman N, Özçeker D, Gülümser-Şişko S, Acar M, Somer A
Turk J Pediatr 2015 Nov-Dec;57(6):592-598. PMID: 27735798
Evolution of hypogammaglobulinemia in premature and full-term infants.
Ricci G, Piccinno V, Giannetti A, Miniaci A, Specchia F, Masi M
Int J Immunopathol Pharmacol 2011 Jul-Sep;24(3):721-6. doi: 10.1177/039463201102400318. PMID: 21978704
Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'similarities and differences'.
Keles S, Artac H, Kara R, Gokturk B, Ozen A, Reisli I
Pediatr Allergy Immunol 2010 Aug;21(5):843-51. Epub 2010 Jul 1 doi: 10.1111/j.1399-3038.2010.01010.x. PMID: 20609138

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