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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

Etiology

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
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
Severe atopic dermatitis and transient hypogammaglobulinemia in children.
Wang AS, Liang MG, Schneider LC
Pediatr Dermatol 2012 Jan-Feb;29(1):73-8. Epub 2011 Aug 19 doi: 10.1111/j.1525-1470.2011.01477.x. PMID: 21854413
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
Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases.
Kiliç SS, Tezcan I, Sanal O, Metin A, Ersoy F
Pediatr Int 2000 Dec;42(6):647-50. doi: 10.1046/j.1442-200x.2000.01301.x. PMID: 11192522

Diagnosis

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
Diagnostic approach of hypogammaglobulinemia in infancy.
Plebani A, Palumbo L, Dotta L, Lougaris V
Pediatr Allergy Immunol 2020 Feb;31 Suppl 24:11-12. doi: 10.1111/pai.13166. PMID: 32017208
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
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
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

Therapy

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 after antiepileptic drug hypersensitivity.
Okumura A, Tsuge I, Kamachi Y, Negoro T, Watanabe K
Pediatr Neurol 2007 May;36(5):342-4. doi: 10.1016/j.pediatrneurol.2006.12.015. PMID: 17509469
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
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
Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases.
Kiliç SS, Tezcan I, Sanal O, Metin A, Ersoy F
Pediatr Int 2000 Dec;42(6):647-50. doi: 10.1046/j.1442-200x.2000.01301.x. PMID: 11192522

Clinical prediction guides

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
Immune regulation: what immunodeficiency disease has taught us.
Wara DW
J Invest Dermatol 1985 Jul;85(1 Suppl):149s-154s. doi: 10.1111/1523-1747.ep12275690. PMID: 2989376

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