Transient hypogammaglobulinemia of infancy

Specialty| Immunology

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] (The ratios of immunoglobulins vary rapidly in all infants, and the term dysgammaglobulinemia, although theoretically applicable, is not usually used in this context.)

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

## Contents

* 1 Signs and symptoms
* 2 Pathophysiology
* 3 Diagnosis
* 4 Treatment
* 5 See also
* 6 References
* 7 External links

## Signs and symptoms[edit | edit source]

The clinical presentation of this condition is as follows:[5]

* Pyodermitis
* Purulent conjunctivitis
* Aphthous stomatitis
* Diarrhea
* Bacteremia
* Otitis media
* Septicemia

## Pathophysiology[edit | edit source]

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 [6]

## Diagnosis[edit | edit source]

CT-massive pleural effusion (collapsed left lung)

The diagnosis for Transient hypogammaglobulinemia of infancy is based on the following:[5]

* B-cell quantitative assessment
* Antibody testing
* Immunophenotyping
* DNA test
* Blood culture
* Stool culture
* Sputum exam
* Chest x-ray
* Diagnostic ultrasound
* CT scan

## Treatment[edit | edit source]

The management of this condition, Transient hypogammaglobulinemia of infancy, is done via antibiotics, IVIG, as well as antihistamines if needed[5]

## See also[edit | edit source]

* List of cutaneous conditions

## References[edit | edit source]

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". Archived from the original on 2008-03-09. Retrieved 2008-03-01. `{{cite web}}`: More than one of `|archivedate=` and `|archive-date=` specified (help); More than one of `|archiveurl=` and `|archive-url=` specified (help)
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. Archived from the original on 2009-09-23. Retrieved 2021-06-16. `{{cite journal}}`: More than one of `|accessdate=` and `|access-date=` specified (help); More than one of `|archivedate=` and `|archive-date=` specified (help); More than one of `|archiveurl=` and `|archive-url=` specified (help)
5. ↑ 5.0 5.1 5.2 Justiz Vaillant, Angel A.; Wilson, Allecia M. (2021). "Transient Hypogammaglobulinemia of Infancy". StatPearls. StatPearls Publishing. Archived from the original on 30 October 2021. Retrieved 29 October 2021. `{{cite book}}`: More than one of `|archivedate=` and `|archive-date=` specified (help); More than one of `|archiveurl=` and `|archive-url=` specified (help)
6. ↑ Justiz Vaillant, AA; Wilson, AM (January 2021). "Transient Hypogammaglobulinemia of Infancy". PMID 31335076. `{{cite journal}}`: Cite journal requires `|journal=` (help)

## External links[edit | edit source]

Classification|

* ICD-10: D80.7
* ICD-9-CM: 279.09

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External resources|

* eMedicine: ped/2280

* v
* t
* e

Lymphoid and complement disorders causing immunodeficiency

Primary|

| Antibody/humoral
(B)|

| Hypogammaglobulinemia|

* X-linked agammaglobulinemia
* Transient hypogammaglobulinemia of infancy

|

Dysgammaglobulinemia|

* IgA deficiency
* IgG deficiency
* IgM deficiency
* Hyper IgM syndrome (1
* 2
* 3
* 4
* 5)
* Wiskott–Aldrich syndrome
* Hyper-IgE syndrome

Other|

* Common variable immunodeficiency
* ICF syndrome

T cell deficiency
(T)|

* thymic hypoplasia: hypoparathyroid (Di George's syndrome)
* euparathyroid (Nezelof syndrome
* Ataxia–telangiectasia)

peripheral: Purine nucleoside phosphorylase deficiency

* Hyper IgM syndrome (1)

Severe combined
(B+T)|

* x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
* Omenn syndrome
* ZAP70 deficiency
* Bare lymphocyte syndrome

Acquired|

* HIV/AIDS

Leukopenia:
Lymphocytopenia|

* Idiopathic CD4+ lymphocytopenia

Complement
deficiency|

* C1-inhibitor (Angioedema/Hereditary angioedema)
* Complement 2 deficiency/Complement 4 deficiency
* MBL deficiency
* Properdin deficiency
* Complement 3 deficiency
* Terminal complement pathway deficiency
* Paroxysmal nocturnal hemoglobinuria
* Complement receptor deficiency

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