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Transient hypogammaglobulinemia of infancy

MedGen UID:
124421
•Concept ID:
C0272238
•
Disease or Syndrome
Synonyms: Immunoglobulin maturational delay; Newborn gammaglobulin deficiency; THI; THI - Transient hypogammaglobulinemia of infancy; Transient Hypogammaglobulinemia of Infancy; transient hypogammaglobulinemia of infancy
SNOMED CT: Transient hypogammaglobulinemia of infancy (88714009); Immunoglobulin maturational delay (88714009); THI - Transient hypogammaglobulinemia of infancy (88714009)
 
HPO: HP:0005432
Monarch Initiative: MONDO:0015698
Orphanet: ORPHA169139

Definition

At birth, newborns are endowed with maternal antibodies. IgG production normally begins at the age of two months. A delay in recovery from this physiological hypogammaglobulinemia between the 3rd and the 6th month of life, and of recovery period between 18 and 36 months defines transient newborn hypogammaglobulinemia. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTransient hypogammaglobulinemia of infancy
Follow this link to review classifications for Transient hypogammaglobulinemia of infancy in Orphanet.

Conditions with this feature

Say-Barber-Miller syndrome
MedGen UID:
343258
•Concept ID:
C1855078
•
Disease or Syndrome
Say-Barber-Miller syndrome is characterised by the association of unusual facial features, microcephaly, developmental delay, and severe postnatal growth retardation.
See: Condition Record
Say-Barber-Miller syndrome

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

Recent clinical studies

Etiology

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
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
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

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
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

BCGitis in a patient with transient hypogammaglobulinemia of infancy.
Lynch M, O'Loughlin A, Devaney D, O'Donnell B
Pediatr Dermatol 2014 Nov-Dec;31(6):750-1. Epub 2012 Dec 18 doi: 10.1111/pde.12036. PMID: 23253103
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
Massive empyema associated with transient hypogammaglobulinemia of infancy and IgA deficiency.
Park K, Lee KY, Lee MH, Lee JS, Kim JC
J Korean Med Sci 2009 Apr;24(2):357-9. Epub 2009 Apr 20 doi: 10.3346/jkms.2009.24.2.357. PMID: 19399287Free PMC Article
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
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 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: 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

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
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: 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

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