Transient hypogammaglobulinemia of infancy

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Transient hypogammaglobulinemia of infancy
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Transient hypogammaglobulinemia of infancy is a form of hypogammaglobulinemia appearing after birth, leading to a reduction in the level of IgG, and also sometimes IgA [1] [2] and IgM. [3]

Contents

It can result in increased infections, but it can also present without symptoms. [4]

Signs and symptoms

Allergy disorders are the second most common feature, with upper and lower respiratory tract infections accounting for the majority of cases. In addition to developmental delay and cardiac defects, [5] rare manifestations include gastroenteritis, invasive infections, and urinary tract infections. [6] [7] [8]

Causes

It is unknown what specifically causes infantile transient hypogammaglobulinemia. The following are some of the hypothesized mechanisms: 1) defective T cells that prevent B cells from stimulating the proper synthesis of antibodies; 2) maternal IgG suppresses the production of IgG; 3) low levels of vital cytokines; and 4) genetic variations in families predisposed to immunodeficiency. [9] [10] [11]

Mechanism

Normally, a newborn's immunoglobulins come from the mother during pregnancy and wane after birth until 3–6 months of age, when the infant begins to start to produce their own IgG. However, in transient hypogammaglobulinemia of infancy, the IgG synthesis is delayed, and the hypogammaglobulinemia is prolonged beyond age 6 months. [12]

Diagnosis

Transient hypogammaglobulinemia of infancy can be identified if the mean age-specific reference values for serum IgG levels are decreased by more than two standard deviations. [13]

Epidemiology

Transient hypogammaglobulinemia of infancy is thought to affect between 0.061 and 1.1 out of every 1000 live births. [14] [15]

See also

References

  1. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 818. ISBN   978-1-4160-2999-1.
  2. ↑ "Transient Hypogammaglobulinemia of Infancy: Immunodeficiency Disorders: Merck Manual Professional" . Retrieved 2008-03-01.
  3. ↑ Kiliç SS, Tezcan I, Sanal O, Metin A, Ersoy F (2000). "Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases". Pediatr Int. 42 (6): 647–50. doi:10.1046/j.1442-200x.2000.01301.x. PMID   11192522. S2CID   25895833.
  4. ↑ Hsueh KC, Chiu HH, Lin HC, Hsu CH, Tsai FJ (2005). "Transient hypogammaglobulinemia of infancy presenting as Staphylococcus aureus sepsis with deep neck infection". J Microbiol Immunol Infect. 38 (2): 141–4. PMID   15843860.
  5. ↑ Dorsey, Morna J.; Orange, Jordan S. (2006). "Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy". Annals of Allergy, Asthma & Immunology. 97 (5). Elsevier BV: 590–595. doi:10.1016/s1081-1206(10)61085-x. ISSN   1081-1206. PMID   17165264.
  6. ↑ Qian, Ji-hong; Zhu, Jian-xing; Zhu, Xiao-dong; Chen, Tong-xin (2009-08-20). "Clinical features and follow-up of Chinese patients with symptomatic hypogammaglobulinemia in infancy". Chinese Medical Journal. 122 (16): 1877–1883. ISSN   2542-5641. PMID   19781364.
  7. ↑ Kutukculer, Necil; Gulez, Nesrin (November 2009). "The outcome of patients with unclassified hypogammaglobulinemia in early childhood". Pediatric Allergy and Immunology. 20 (7): 693–698. doi:10.1111/j.1399-3038.2008.00845.x. ISSN   1399-3038. PMID   19196447. S2CID   25506883.
  8. ↑ Artac, Hasibe; Kara, Reyhan; Gokturk, Bahar; Reisli, Ismail (November 2013). "Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy". Clinical and Experimental Medicine. 13 (4): 257–263. doi:10.1007/s10238-012-0200-y. ISSN   1591-9528. PMID   22820757. S2CID   26184302.
  9. ↑ Wang, Ling-Jen; Yang, Yao-Hsu; Lin, Yu-Tsan; Chiang, Bor-Luen (March 2004). "Immunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan" (PDF). Asian Pacific Journal of Allergy and Immunology. 22 (1): 25–31. PMID   15366655 . Retrieved 7 February 2024.
  10. ↑ Whelan, M. A.; Hwan, W. H.; Beausoleil, J.; Hauck, W. W.; Mcgeady, S. J. (2006). "Infants Presenting with Recurrent Infections and Low Immunoglobulins: Characteristics and Analysis of Normalization". Journal of Clinical Immunology. 26 (1). Springer Science and Business Media LLC: 7–11. doi:10.1007/s10875-006-8144-1. ISSN   0271-9142. S2CID   24144434.
  11. ↑ Wang, Helen C.; Whelan, Meg A.; McGeady, Stephen J.; Yousef, Ejaz (2006). "A 5-month-old boy with recurrent respiratory infections, failure to thrive, and borderline elevated sweat chloride levels". Allergy and Asthma Proceedings. 27 (3): 285–288. doi:10.2500/aap.2006.27.2847. ISSN   1088-5412. PMID   16913275.
  12. ↑ Vaillant, Angel A. Justiz; Wilson, Allecia M. (September 4, 2023). "Transient Hypogammaglobulinemia of Infancy". StatPearls Publishing. PMID   31335076 . Retrieved February 7, 2024.
  13. ↑ Duse, M.; Iacobini, M.; Leonardi, L.; Smacchia, P.; Antonetti, L.; Giancane, G. (January 1, 2010). "Transient hypogammaglobulinemia of infancy: intravenous immunoglobulin as first line therapy". International Journal of Immunopathology and Pharmacology. 23 (1): 349–353. doi:10.1177/039463201002300134. ISSN   0394-6320. PMID   20378022. S2CID   31079239.
  14. ↑ Walker, A M; Kemp, A S; Hill, D J; Shelton, M J (March 1, 1994). "Features of transient hypogammaglobulinaemia in infants screened for immunological abnormalities". Archives of Disease in Childhood. 70 (3). BMJ: 183–186. doi:10.1136/adc.70.3.183. ISSN   0003-9888. PMC   1029737 . PMID   8135560.
  15. ↑ Tiller, Thomas L.; Buckley, Rebecca H. (1978). "Transient hypogammaglobulinemia of infancy: Review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up". The Journal of Pediatrics. 92 (3). Elsevier BV: 347–353. doi:10.1016/s0022-3476(78)80417-x. ISSN   0022-3476. PMID   632973.

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