Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors.
We evaluated 131 children (M=88, F=43) with hypogammaglobulinemia. Data was analyzed mainly for delineating predictor factors for outcome. The distance from the lower limit of normal (-2SD) for any single measurement of immunoglobulins (Ig) was calculated and transformed into Ig scores. Mean age and duration of follow-up were 5.06 ± 4.05 and 3.7 ± 3.03 years, respectively. The diagnoses were: 22 CVID, 16 IgA deficiency, 33 transient hypogammaglobulinemia of childhood (THC), 3 selective IgM deficiency and 57 unclassified hypogammaglobulinemia (UCH). Low IgA scores (<-0.124) at presentation were indicative of subsequent development of IgA deficiency or CVID, whereas low IgM score (<-0.038) pointed towards more severe and persistent phenotypes. Combination of low IgM score between 2 and 5 years, impaired antibody response and low B cell counts enabled us to predict persistence of hypogammaglobulinemia beyond 5 years (specificity=90.5% and PPV=94.9%) and chronic lung disease (sensitivity=90.4% and specificity=68.3%). The set of criteria including low IgM scores, impaired antibody response and low B cell counts provided a high predictive value in detecting those with persistent hypogammaglobulinemia.
Ozen
Ahmet
A
Division of Pediatric Allergy/Immunology, Yeditepe University Faculty of Medicine, Turkey. ahmetozen_md@yahoo.com
Baris
Safa
S
Karakoc-Aydiner
Elif
E
Ozdemir
Cevdet
C
Bahceciler
Nerin Nadir
NN
Barlan
Isil Berat
IB
D000293:Adolescent; D000361:Agammaglobulinemia / Q000175:diagnosis* / Q000276:immunology*; D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000097:blood*; D008297:Male; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies
D015415:Biomarkers; D007136:Immunoglobulins