2000, Number 2
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Alerg Asma Inmunol Pediatr 2000; 9 (2)
Gammaglobuline in the treatment of humoral immunodeficiencies
Jiménez-Córdoba I, Correa-Bautista Y, Berrón-Pérez R, Espinosa-Rosales F, Hernández-Bautista V, Almendarez-Flores C
Language: Spanish
References: 31
Page: 50-56
PDF size: 658.62 Kb.
ABSTRACT
Introduction: Primary humoral immunodeficiency syndromes (PHID) are group of congenital or acquired disorders in which recurrent infections with encapsulated bacterial pathogens are the clinical hallmark. Over the past 30 years, intramuscular administration of immunoglobulins has been the treatment more frequently used in antibody deficient patients seen at our hospital, because of the high cost of the IVIG and because this therapeutic modality is not part of the basic stuck of drugs in our Institute. The purpose of this work was to investigate the age at which PHID diagnosis is made in our patients, their clinical evolution, number and consequences of infections before and after diagnosis; and finally the life span in patients treated with different methods of immunoglobulin replacement.
Method: We reviewed the medical records of patients with X-linked agammaglobulinemia, common variable immunodeficiency and hyperimmunoglobulin M (hyper-IgM syndrome) treated in our department between 1970 and 1999. We investigated the following variables: Age at which recurrent infections started and when diagnosis was confirmed. The number of infections episodes severe infections, infections per year per patient, the need for hospitalization, complications and sequelae previous and after diagnosis. Patients were assigned to one of four groups based on the method (IV versus IM), dose and regularity of immunoglobulin replacement.
Results: We included a total of 25 patients, twenty with X-linked agammaglobulinemia, 2 with hyper-IgM syndrome, and 3 with common variable immunodeficiency. The median age at diagnosis was 5.5 years (range 0.75-14 years). The median age when the infectious episodes began was 12.8 months and disease evolution had a median of 3.8 years (range 1 month-10 years) with recurrent infections before diagnosis was confirmed. The whole group had 502 infectious episodes prior to the diagnosis. Upper airway infections were the most frequently reported (50%) followed by pneumonia (20.3%), acute and chronic gastroenteritis (18.7%), and other. Infections per year per patients decreased dramaticly after IVIG was started in the group 3 patients when compared with the rest of the patients. Severe infections per patient per year decreased in group 3 and 4 patients after immunoglobulin replacement. Five/25 patients included in this study developed new long-term clinical consequences: 11 of the twelfth patients whose had bronchiectasis developed chronic pulmonar obstructive disease with cardiac insufficiency. The need for hospitalization showed a significant reduction in patients receiving IVIG (p = 0.000001). Only 10/ 25 patients, are currently being followen.
Conclusion: The only recognized effective therapy for PHID at this time is IVIG, because this modality is the only one capable of modify the natural history of these entities. In our series we have found this to be true by proving that patients treated with IVI showed a significant decrease in the number and severity of infections, and the long term complications and sequelae.
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