[Immunoglobulin replacement therapy contribution in agammaglobulinemia: 10 case reports]. / Apport du traitement substitutif par les immunoglobulines au cours des agammaglobulinémies: à propos de 10 cas.

UNLABELLED: 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 immunofluorescence labeling by monoclonal antibodies. T-cell functions 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.

[Chronic septic granulomatosis in a young girl manifesting as invasive pulmonary aspergillosis and flaccid paraplegia]. / Granulomatose septique chronique chez une fillette revelee par une aspergilose pulmonaire invasive et une paraplegie flasque.

UNLABELLED: The invasive Pulmonary Aspergillosis (IPA) is the principal cause of death in patients with chronic granulomatosis disease (CGD). It can happen before age of one and can reveal the (CGD). Usually, the transmission of GSD is linked to x in 65% of cases. We report, the case of a girl aged 3 years issued from consanguine marriage referred to hospital for hemoptysia. We note that at 40 days she presents an suppurate adenitis. She is hypotrophic at--3DS without respiratory problem, she have a splenomegaly. The radiography chest note an alveolar and interstitial opacity bilateral, apical right and basal left. There is a biological inflammatory syndrome. During the evolution, the child presents a paraplegia secondary to compression myelitis. A D5 laminectomy in urgency was done. The vertebral Biopsia, the serology and the culture isolate fumigatus aspergillus the GCD is evoqued confirmed by the NBT test who was negative. IN CONCLUSION: --We have to look for GCD in front of pulmonary aspergillus.--The vertebra lesion is secondary to pulmonary lesion.--Our observation is noticeable by the recessive autosomal transmission who was seen in 35% of cases, and by the neurologic complication rarely reported.