Symptomatic hypogammaglobulinemia in infancy and childhood - clinical outcome and in vitro immune responses.
BACKGROUND
Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferative and secretory responses with recovery of immunoglobulin values and clinical resolution.


METHODS
Children, older than 1 year of age, referred to our specialist clinic because of recurrent infections and serum immunoglobulin (Ig) levels 2 SD below the mean for age, were followed for a period of 8 years. Patient with any known familial, clinical or laboratory evidence of cellular immunodeficiency or other immunodeficiency syndromes were excluded from this cohort. Evaluation at 6- to 12-months intervals continued up to 1 year after resolution of symptoms. In a subgroup of patients, in vitro lymphocyte proliferation and Ig secretion in response to mitogens was performed.


RESULTS
32 children, 24 (75%) males, 8 (25%) females, mean age 3.4 years fulfilled the inclusion criteria.


METHODS
ENT infections 69%, respiratory 81%, diarrhea 12.5%. During follow-up, 17 (53%) normalized serum Ig levels and were diagnosed as transient hypogammaglobulinemia of infancy (THGI). THGI patients did not differ clinically or demographically from non-transient patients, both having a benign clinical outcome. In vitro Ig secretory responses, were lower in hypogammaglobulinemic, compared to normal children and did not normalize concomitantly with serum Ig's in THGI patients.


CONCLUSIONS
The majority of children with SHIC in the first decade of life have THGI. Resolution of symptoms as well as normalization of Ig values may be delayed, but overall the clinical outcome is good and the clinical course benign.
Kidon
Mona Iancovici
MI
Department of Pediatrics, Kaplan Medical Center, Rehovot, Israel. drkidon@bezeqint.net
Handzel
Zeev T
ZT
Schwartz
Rivka
R
Altboum
Irit
I
Stein
Michael
M
Zan-Bar
Israel
I
D000361:Agammaglobulinemia / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D000367:Age Factors; D000917:Antibody Formation; D049109:Cell Proliferation; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000453:epidemiology / Q000276:immunology*; D004198:Disease Susceptibility / Q000276:immunology; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000276:immunology; D006801:Humans; D017099:IgG Deficiency / Q000175:diagnosis / Q000276:immunology; D007136:Immunoglobulins / Q000096:biosynthesis / Q000097:blood* / Q000172:deficiency; D066298:In Vitro Techniques; D007223:Infant; D007557:Israel / Q000453:epidemiology; D008213:Lymphocyte Activation / Q000276:immunology; D008297:Male; D008934:Mitogens / Q000276:immunology; D012008:Recurrence; D012075:Remission, Spontaneous; D012141:Respiratory Tract Infections / Q000453:epidemiology / Q000276:immunology*
D007136:Immunoglobulins; D008934:Mitogens