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Transient hypogammaglobulinemia of infancy

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Disease definition

A rare primary immunodeficiency characterized by a delay in the maturation of immunoglobulin production, leading to prolongation of the physiologic hypogammaglobulinemia of the newborn period beyond six months of age. Patients present recurrent respiratory infections, otitis media, bronchitis, gastroenteritis, or allergic symptoms in the first two to four years of life, before the condition resolves spontaneously. Some children may remain asymptomatic, and severe or life-threatening infections are rare. The capacity to synthesize specific antibodies in response to vaccines is usually normal.

ORPHA:169139

Classification level: Disorder

Prevalence: -

Inheritance: -

Age of onset:

ICD-10: D80.7

ICD-11: 4A01.03

UMLS: C0272238

MedDRA: 10044388

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Deutsch,
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2020) - J Clin Immunol Logo ERN
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