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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

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
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
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

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
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
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

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
Hypogammaglobulinemia in neonates: illustrative cases and review of the literature.
Darmawan D, Raychaudhuri S, Lakshminrusimha S, Dimitriades VR
J Perinatol 2024 Jul;44(7):929-934. Epub 2023 Sep 4 doi: 10.1038/s41372-023-01766-6. PMID: 37667006
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
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
Study of patients with Hyper-IgM type IV phenotype who recovered spontaneously during late childhood and review of the literature.
Karaca NE, Durandy A, Gulez N, Aksu G, Kutukculer N
Eur J Pediatr 2011 Aug;170(8):1039-47. Epub 2011 Jan 28 doi: 10.1007/s00431-011-1400-2. PMID: 21274562
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
Study of patients with Hyper-IgM type IV phenotype who recovered spontaneously during late childhood and review of the literature.
Karaca NE, Durandy A, Gulez N, Aksu G, Kutukculer N
Eur J Pediatr 2011 Aug;170(8):1039-47. Epub 2011 Jan 28 doi: 10.1007/s00431-011-1400-2. PMID: 21274562
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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