Transient hypogammaglobulinemia of infancy; analysis and follow-up

Abstract

Rationale Transient Hypogammaglobulinemia of Infancy is considered a primary immune deficiency, but is not well defined, and long term follow-up not reported. The purpose of this study is to: 1) Characterize infants with recurrent infections and low immunoglobulins 2) Provide long-term follow-up. Methods Forty-two patients were evaluated between 1977-2003 who: 1) Were younger than 24 months, 2) Had one or more immunoglobulin classes 2 SD below normal means, 3) Produced antibody normally to diphtheria and tetanus, 4) Had evidence of intact cell mediated immunity, and 5) Lacked features of other immunodeficiency syndromes. The following were collected: Quantitative immunoglobulins at presentation and follow-up, recurrent infection information, atopic development, age at presentation and immunoglobulin normalization, sex, and gender. Results 1) Infants were 27/42 (64%) male. 2) At presentation, immunoglobulins showed decreased: IgA (95%), IgG (74%), then IgM (5%). 3) OM's, bronchitis, or both in 88%. Other infections were: thrush, pneumonia, and bacteremia. 4) 43% of study patients developed atopy. 5) Infections decreased as age increased. 6) Not all immunoglobulins normalized during observation. All with IgM deficiency normalized by age 3 years, but 80% had low IgA and 10% low IgG at 6-11 years and 2 children had selective IgA deficiency. Conclusions Infants who present exclusively with diminished immunoglobulins without other immunologic abnormalities are predominantly male and suffer from recurrent infections. With age, the frequency and severity of infections decrease as immunoglobulins normalize, but, many develop atopy and some IgA deficiency. Children with THI represent a heterogeneous group whose immunogical outcome varies. Close monitoring is indicated.