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Clinical and Laboratory Features of 184 Italian Pediatric Patients Affected with Selective IgA Deficiency (SIgAD): a Longitudinal Single-Center Study.

Lougaris, Vassilios; Sorlini, Annamaria; Monfredini, Chiara; Ingrasciotta, Giulia; Caravaggio, Andrea; Lorenzini, Tiziana; Baronio, Manuela; Cattalini, Marco; Meini, Antonella; Ruggeri, Laura; Salpietro, Annamaria; Pilotta, Alba; Grazzani, Livia; Prandi, Elena; Felappi, Barbara; Gualdi, Giulio; Fabiano, Antonella; Fuoti, Maurizio; Ravelli, Alberto; Villanacci, Vincenzo; Soresina, Annarosa; Badolato, Raffaele; Plebani, Alessandro.
J Clin Immunol; 39(5): 470-475, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31129864

PURPOSE:

Selective IgA deficiency (SIgAD) is the most common humoral primary immunodeficiency. Long-term follow-up data in large cohort of pediatric patients are scarce.

METHODS:

We report on a single-center cohort of 184 pediatric patients affected with selective IgA deficiency and describe the characteristics at diagnosis and during follow-up.

RESULTS:

Respiratory infections were the most common clinical finding leading to the initial diagnosis (62%). Positive family history for antibody deficiencies (selective IgA deficiency, common variable immunodeficiency) led to SIgAD diagnosis in 16% of cases. During follow-up, while the incidence of respiratory infections was not particularly high, gastrointestinal symptoms were reported in 27% of patients. Allergic manifestations were found in 23% at diagnosis and an additional 16% of patients during follow-up, leading to a prevalence of atopy of 39% among SIgAD patients. Autoimmune manifestations, excluding celiac disease, were found in 9% of affected patients during follow-up. Celiac disease was found in a high prevalence (14%). Increase of serum IgA levels to partial deficiency (9%) and normal serum levels for age (4%) was observed during follow-up. A small percentage of patients (2%) progressed to common variable immunodeficiency (CVID).

CONCLUSIONS:

In conclusion, this is the first study to describe a large single-center pediatric cohort of patients affected with SIgAD, revealing that overall most patients do well with regard to infections. Many develop CD, at a rate much higher than the general population. A few normalize their IgA levels. A few progress to CVID. Thus, careful follow-up is suggested to diagnose and treat potential complications earlier for avoiding potential morbidities.
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