[Common variable immunodeficiency (CVID): clinical and immunological features of 29 Algerian patients]. / Déficit immunitaire commun variable (DICV) : caractéristiques cliniques et immunologiques de 29 patients algériens.

PURPOSE: Common variable immunodeficiency (CVID) is the commonest symptomatic primary immunodeficiency. It is characterized by a defect of antibody production, recurrent respiratory tract infections and increased occurrence of auto-immune discords and lymphoproliferative disease. METHODS: This retrospective study was conducted on 29 patients fulfilling the classical CVID definition. Blood tests included immunoglobulin measurement and lymphocyte subpopulations phenotyping. RESULTS: This study includes 29 patients. The mean age at diagnosis was 23years. Recurrent upper and lower bacterial respiratory tract infections were common in almost all patients. Five patients developed auto-immune conditions and six had lymphoproliferative disease. Decreased IgG was found in almost all patients. Low IgA and IgM levels were found in 89.6 % and 65.5 % of cases respectively. Abnormal T and/or B phenotype was found in 75 % of cases; the most common abnormalities were decreased circulating B (54.2 %) and T CD4+ (41.7 %) cells and inversion of the CD4/CD8 ratio (70.8 %). Patients with decreased circulating B and T CD4+ cells were significantly more likely to have auto-immune cytopenias and lymphoproliferative disease. CONCLUSIONS: Our study confirms the heterogeneity of CVID. A patient's classification is necessary to define homogeneous groups of patients and to characterize specific molecular abnormalities in each group.

Foreign body aspiration in children: experience from 2624 patients.

OBJECTIVES: The objective of this study is to analyze the epidemiological, clinical, radiological and endoscopic characteristics of pediatric foreign body aspiration in Algeria. METHODS: In this retrospective study, the results of 2624 children younger than 18 years admitted in our department for respiratory foreign body removal between 1989 and 2012, were presented. Most of them had an ambulatory rigid bronchoscopy. RESULTS: The children (62.34% males and 37.65% females) were aged 4 months to 18 years with 66% between 1 and 3 years. Choking was related in 65% of cases. The delay between aspiration and removal was 2-8 days in 65.8% and within 24 h in 9.2%. In the most cases, the children arrived with cough, laryngeal or bronchial signs and unilateral reduction of vesicular murmur. The examination was normal in 13%. The most common radiologic finding was pulmonary air trapping (40.7%). The aspirated bodies were organic in 66.7%, dominated by peanuts, while sunflower seeds, beans and ears of wheat were the most dangerous. In the other cases, they were metallic or plastic as pen caps and recently scarf pins. The endoscopic removal by rigid bronchoscopy was successful and complete in 97%. Cases with extraction failure (3%) limited to certain FBs, all of them inorganic were assigned to surgery. The complications related to the endoscopic procedure were 0.29% with a mortality of 0.26%. CONCLUSION: Foreign body aspiration is a real public health problem in Algeria. The best way to manage it is an early diagnosis and a rigid bronchoscopy removal under general anesthesia used by fully trained staff. The prevention of this domestic accident should consider the population lifestyle and cultural habits to be more effective.

Analysis of the complete coding region of the CFTR gene in ten Algerian cystic fibrosis families.

The spectrum of cystic fibrosis (CF) mutations in the North African population remains poorly known. In order to offer an effective diagnostic service and to determine accurate risk estimates, we decided to identify the CF mutations in 10 Algerian CF families. We carried out a chemical-clamp denaturing gradient gel electrophoresis analysis of the CFTR gene and automated direct DNA sequencing. We identified 5 mutations and we characterized 60% of the CF chromosomes. Taking advantage of the homogeneity of the sample, we report clinical features of homozygous CF patients.

Chemoprophylaxis of tuberculosis in children.

PIP: The situations when prophylactic antibiotics should be given to children in developing countries where BCG vaccination is common are explained. In most countries, 80-90% of children have been vaccinated with BCG by the age of 1. Since BCG makes a tuberculin (PPD) test moot, because all BCG-vaccinated persons have positive PPD tests, the only children in developing countries who can be diagnoses with TB are those who are vaccinated and live in close contact with a TB infected person. The only prophylactic medication recommended for children is daily isoniazid, 5 mg/kg for 6 months. Preventive medication is never given to healthy BCG-vaccinated children. The multi-drug regimen is not appropriate because young children have relatively few Mycobacterium bacilli, and developing countries cannot even afford to treat infected adults with these regimens. The only situations for treating children prophylactically are: 1) healthy children, under 5, not BCG vaccinated, and tuberculin positive; 2) healthy children, age 5-14, tuberculin positive with documented negative PPD test within 2 years, and 3) age 1, not BCG vaccinated, recently diagnosed as tuberculin positive. In an infant living in close contact with an infected source, a PPD test is done after 3 months of treatment with isoniazid, and if positive, treatment is continued for another 3 months. If the PPD test is still negative, the BCG vaccination is given.^ieng

An Algerian child homozygous for the M470V polymorphism and for a deletion of two nucleotides in exon 10 of the CFTR gene, shows severe cystic fibrosis symptoms.

When screening for the presence of major cystic fibrosis mutations in Algerian cystic fibrosis families by heteroduplex formation, aberrant heteroduplexes were observed for exon 10 in one family. Here we describe the clinical and molecular findings in a severely affected child of this family, homozygous for the 1609delCA and for the M470V polymorphism.

Elastic behavior of the lungs in healthy children determined by means of an exponential function.

In order to evaluate the elastic properties of the lungs in children, a new exponential sigmoid curve fitting model was developed with the function VL = Vm + (Vm/1 + b X e-K X Pst), where VL is the lung volume, Pst the static recoil pressure, VM and Vm the upper and the lower asymptotes (limits of lung volume) and K and b are specific constants. Pressure-volume (PV) data obtained from tidal breathing cycles at different inflation and deflation levels from functional residual capacity (FRC) up to approximately 90% total lung capacity (TLC) in 16 healthy children have been evaluated by this model. K in relation to age, calculated by the least square nonlinear regression analysis decreased during childhood, whereas in b increased. It seems that K (a volume-independent index of compliance and alveolar distensibility) is influenced by the increasing number of elastic fiber bundles, changes in the surface/volume ratio and finally by changes in the composition of the surfactant. It can be seen that this model is not only restricted to higher lung volumes, the lower limb of the S-shaped curve is also represented.

[Cerebral abscess and pulmonary arteriovenous fistula. A clinical and respiratory function study]. / Abcès cérébral et fistule artérioveineuse pulmonaire. Etude clinique et fonctionnelle respiratoire.

Liz... Josiane, a 9 year old girl, was admitted with a 24 hours history of severe headache and vomiting. On admission she was conscious, irritable and complained of a severe headache. Clinical examination revealed a right hemiparesis with cyanosis of the lips and extremities and clubbing of the fingers, all consistent with chronic hypoxia. Cardiovascular examination was normal apart from a systolic murmur which could be heard posteriorly under the left scapula. There were no angiomatous or telangiectatic lesions of the skin. A blood examination revealed a raised ESR, a marked polycythaemia with a decreased arterial oxygen tension. Chest x-rays showed the presence of an irregular well delineated opacity in the posterior basal segment of the left lower lobe. This opacity was confluent with the ipsilateral hilum and was suggestive of a pulmonary arteriovenous fistula. An intracranial space occupying lesion in the left temporal region of the brain was revealed by electroencephalographic and CT scan investigations; this proved to be an abscess which was surgically removed with no subsequent complications. Further radiological investigations of the chest revealed the pulmonary lesion was an arteriovenous aneurysm occupying the whole left inferior lobe. This was removed at thoracotomy three months after the acute neurological event. The results of respiratory function and regional isotopic investigations before and after surgery will be discussed.

[Comparison of reversed phase passive hemagglutination test and the electro-immunodiffusion technic for the detection of HBs antigen]. / Etude comparée de l'hémagglutination passive inversée et de l'électro-immunodiffusion dans le dépistage de l'antigène HBs.

In this study, were compared two methods used in case-finding of the australian antigen (Ag. HBs) : the electro-immunodiffusion technique (E.I.D.) and the passive reverse hemagglutination tests. All the tests were carried out on couple of sera obtained from mothers and her newborns, giving on the whole 717 sera. The results we obtained led us to maintain the E.I.D. for the biological diagnosis of B virus hepatitis. The passive reverse hemagglutination test has been kept back for the titration of the Ag. HBs in the sera found positive by E.I.D. With regard to the vertical transmission (mother-newborn) of Ag. HBs, we could not draw any good conclusion.