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Immunoglobulin replacement therapy contribution in agammaglobulinemia: 10 case reports

Abstract : Intravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions. Patients and methods. - Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immuno fluorescence labeling by monoclonal antibodies. T-cell fonctions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy. Results. - Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/ year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection /year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment. Conclusion. - Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis. (c) 2006 Elsevier SAS. Tous droits reserves.
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https://hal-riip.archives-ouvertes.fr/pasteur-01375264
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Submitted on : Monday, December 19, 2016 - 3:06:25 PM
Last modification on : Wednesday, April 24, 2019 - 4:30:21 PM

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H. Ksouri, F. Mellouli, R. Barbouch, A. Ben Hassen, M. Béjaoui. Immunoglobulin replacement therapy contribution in agammaglobulinemia: 10 case reports. Archives de Pédiatrie, Elsevier, 2006, 13 (7), pp.1034 - 1039. ⟨10.1016/j.arcped.2006.03.152⟩. ⟨pasteur-01375264⟩

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