Transient Hypogammaglobulinemia of Infancy

Preferred Label : Transient Hypogammaglobulinemia of Infancy;

NCIt synonyms : THI;

NCIt definition : A rare, primary humoral immunodeficiency of childhood characterized by decreasing serum levels of immunoglobulin G (IgG) as maternal antibodies clear the circulation while serum levels of immunoglobulin A and immunoglobulin M remain normal or are slightly decreased. Diagnosis may be suspected after the age of six months when a child's own synthesis of IgG should accelerate but it must be confirmed retrospectively after normalization of all serum immunoglobulin levels is seen by ages 2-6. This disorder may be caused by inadequate activation of progenitor B cells, defective class-switching or may even represent a maturational variant. Typically, a normal response to protein antigens is found while there is a notably diminished response to viral and bacterial polysaccharide antigens. Clinical presentation may include recurrent infections especially those of the respiratory tract. Despite increased susceptibility to infection in childhood, this disorder is self-limited with minimal implications for a normal life span.;

Alternative definition : NICHD: Transitory decrease in the concentration of IgG below established reference ranges, in an infant or child less than 6 months of age. IgA and IgM concentrations may be decreased as well.;

Details

Origin ID

C27071;

UMLS CUI

C0272238;

Currated CISMeF NLP mapping
- Transient hypogammaglobulinaemia of infancy [ICD-11 Sub-sub category]
- Transient hypogammaglobulinaemia of infancy [ICD-10 Sub-category (who)]
- Transient hypogammaglobulinaemia of infancy [MedDRA Preferred Term]
- Transient hypogammaglobulinemia of infancy [HPO term]
- Transient hypogammaglobulinemia of infancy [ICD-10 Sub-category]
- Transient hypogammaglobulinemia of infancy [ORDO Disease]
- Transient hypogammaglobulinemia of infancy [MedDRA LLT]
- Transient hypogammaglobulinemia of infancy (disorder) [SNOMED CT concept]
- transient hypogammaglobulinemia of infancy [DO Concept]
- transient hypogammaglobulinemia of infancy [Disease (Nov.)]
- transient hypogammaglobulinemia of infancy [SNOMED Notion]
DO Cross reference
- transient hypogammaglobulinemia of infancy [DO Concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Transient hypogammaglobulinaemia of infancy [ICD-11 Sub-sub category]
- Transient hypogammaglobulinaemia of infancy [ICD-10 Sub-category (who)]
- Transient hypogammaglobulinaemia of infancy [MedDRA Preferred Term]
- Transient hypogammaglobulinemia of infancy [MedDRA LLT]
- Transient hypogammaglobulinemia of infancy [ORDO Disease]
- Transient hypogammaglobulinemia of infancy [ICD-10 Sub-category]
- Transient hypogammaglobulinemia of infancy [HPO term]
- Transient hypogammaglobulinemia of infancy (disorder) [SNOMED CT concept]
- transient hypogammaglobulinemia of infancy [DO Concept]
- transient hypogammaglobulinemia of infancy [Disease (Nov.)]
- transient hypogammaglobulinemia of infancy [SNOMED Notion]
concept_is_in_subset
- NICHD Terminology [NCIt concept]
- Pediatric Adverse Events Terminology [NCIt concept]
- Pediatric Adverse Events Terminology Mapped to MedDRA [NCIt concept]
- Perinatal Terminology [NCIt concept]

Keyword's position in hierarchy(ies) :

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