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Biomedical subjects

A Bouguerra

Publications and source records attributed to A Bouguerra.

At least 19 recordsLinked to original sources
PubMed1997

[Primary immunodeficiency in Tunisia: study of 152 cases].

BACKGROUND: Primary immunodeficiencies are rare immunopathological disorders. A multidisciplinary study group was set up in Tunis in 1988 and has since identified 152 cases of such diseases. We herein present our series and compare it to the international registries. POPULATION AND METHODS: Over a period of 8 years (April 1988-April 1996), 295 children suffering from recurrent infections were investigated; primary immunodeficiency was confirmed in 152 out of them. The immunological investigation included a study of specific and/or non specific humoral and cellular immunity. RESULTS: These 152 patients belonged to 129 families among which 70 were consanguine (54%). Familial primary immunodeficiency occurred in 23 of them. In 39 families (30%), one or more deaths occurred during early childhood. In more than half of the cases (89 cases), the immunological investigations revealed a cellular or combined immunodeficiency with a majority of ataxia-telangiectasia syndromes (53 cases), T cell activation immunodeficiencies (12 cases) and HLA class II deficiency (nine cases). A predominant antibody defect was observed in 35 patients with a majority of agammaglobulinemia (11 cases) and hyper-IgM syndromes (11 cases). A defect of non specific cellular immunity was found in 18 cases (11.8%) including seven cases of chronic granulomatous disease and five cases of leukocyte adhesion deficiency. Three children (1.9%) were deficient in the complement system. Deaths occurred so far in 37 patients (24.3%). CONCLUSIONS: Primary immunodeficiencies are relatively frequent in Tunisia, probably because of the high rate of consanguinity among the general population. The distribution of the different groups of primary immunodeficiencies is characterized by high frequency of ataxia-telangiectasia and hyper-IgM syndrome and scarcity of severe combined immunodeficiencies and Wiskott-Aldrich syndrome.

Child↗
PubMed1994

[Successful treatment of chronic active hepatitis with high-dose intravenous immunoglobulins in agammaglobulinemia].

BACKGROUND: Rapid progression of chronic active hepatitis can occur in patients with hypogammaglobulinemia. This report describes the successful use of i.v. immunoglobulins to treat chronic hepatitis in a child with agammaglobulinemia. CASE REPORT: A 17 month-old boy was admitted because he had suffered from recurrent infections since the age of 6 months. His family history was normal. Clinical and laboratory investigations showed hepatomegaly, agammaglobulinemia with absence of IgG, IgA, IgM and IgE, absence of beta cells, normal T cells, normal T cell proliferation and normal levels of complement, elevated ALAT (70 and 200 IU/ml) and ASAT (60 and 188 IU/ml). Liver biopsy showed typical features of chronic active hepatitis. The cause of this hepatitis (B and C virus, EBV, autoimmune markers) was not found. The patient was first given gammaglobulins (80 mg/kg) every week, subcutaneously, for 9 weeks, which did not change his transaminasemia. A second course of gamma-globulins, 400 mg/kg every 3 weeks, intravenously, for 6 months, resulted in a transient normalization of transaminases for 3 months. Definitive normalization was only obtained when the patient was given i.v. gammaglobulins (400 mg/kg/week) which gave a residual level of blood IgG of 10 g/l. This apparent cessation of hepatitis activity was confirmed by a second liver biopsy. The patient is now given i.v. gammaglobulins, twice a month, producing a residual blood IgG concentration of 5 g/l. CONCLUSIONS: The activity of this chronic hepatitis is closely correlated with the residual blood IgG concentration. Gammaglobulins could help neutralize virus extra-cellularly, although the viral origin of this hepatitis has not been-demonstrated.

Agammaglobulinemia↗
PubMed1992

Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins.

Human alpha heavy chain disease is characterized by the production of abnormally short alpha IgH chains. In previously published cases it has been found that the malignant cells produce abnormal alpha mRNA, lacking VH and CH1 sequences and composed of a leader sequence peptide, sequences of variable length (69 to 84 bp) and of unknown origin, followed by normal CH2 and CH3 sequences. In this study we established the nucleotide sequence of alpha mRNA for six cases of alpha heavy chain disease. We observed that all six alpha mRNA lack the VH and CH1 sequences as do those previously described. They also contain in-frame inserts of unknown origin between the leader peptide and the normal CH2 and CH3 coding sequences. These inserts are of variable length (42 to 105 bp) and they are unrelated. These results suggest the existence of a common mechanism defect leading to deletions/insertions in alpha heavy chain disease rather than a specific interaction between alpha 1 IgH gene with a unique defined molecular species.

Amino Acid Sequence↗
PubMed1991

A human monoclonal IgG reactive with a private idiotope of a monoclonal IgM with autoantibody activity against myelin-associated glycoprotein.

One hundred and thirteen sera from patients with monoclonal IgG were tested for reactivity against a panel of 13 human monoclonal IgM having various autoantibody activities: 6 to myelin-associated glycoprotein (MAG), 2 to vimentin intermediate filament protein and 5 to red blood cell antigens [cold agglutinins with specificity directed to I antigen (3 cases), i antigen (1 case) or Pr antigen (1 case)]. One IgG was found to react with a monoclonal IgM with anti-MAG activity. This reactivity was characterized as idiotypic and directed against a private idiotope of the monoclonal IgM. This work provides further evidence for the existence of anti-idiotypic antibody activity of monoclonal Ig occurring in human B cell neoplasias.

Antibodies, Anti-Idiotypic↗
PubMed1989

Treatment of alpha chain disease. Results of a prospective study in 21 Tunisian patients by the Tunisian-French intestinal Lymphoma Study Group.

Between 1981 and 1985, the authors studied 21 Tunisian patients with alpha chain disease. Twenty of 21 underwent laparotomy. According to Galian et al. six patients were classified Stage A, two Stage B, and 13 Stage C. The therapeutic regimen included the following: (1) Antibiotics: In the case of intestinal bacterial overgrowth (IBO), antibiotics selected by their antibiograms were delivered; in absence of IBO, metronidazole plus ampicillin were first given. The antibiotic treatment was changed in case of therapeutic failure. (2) Chemotherapy: From 1981 to 1983 a cyclophosphamide, Adriamycin (doxorubicin), teniposide (VM-26), prednisone (CHVP) protocol (Adriamycin 35 mg/m2, teniposide 50 mg/m2 day 2, cyclophosphamide 300 mg/m2 days 2 through 4, prednisone 40 mg/m2 days 1 through 10) was used. After 1983 bleomycin 15 mg, Adriamycin 30 mg, vinblastine 10 mg were given on day 15. Serum immunoelectrophoresis and immunohistochemical study of duodenojejunal specimens were made on a 3-month and 6-month basis, respectively. Survival curve analysis was made according to Kaplan and Meier. Results were as follows: (1) Stage A: Six patients were first treated by antibiotics alone; two complete responses (CR) persisting 42 and 55 months later were observed, respectively. The four antibiotic failures were submitted to further chemotherapy with four subsequent failures and two deaths. (2) Stage B-C: Chemotherapy led to nine CR with one precocious relapse, a salvage chemotherapy allowing to one more CR. (3) All stages mixed, percentage of survival reached 90 +/- 12% at 2 years and 67 +/- 25% at 3 years, all patients alive beyond 3.5 years being disease-free.

Actuarial Analysis↗
PubMed1985

[Primary, exclusively gastric localization of alpha-chain disease: anatomo-clinical and immunohistochemical study].

We report the first case of heavy alpha-chain disease exclusively located in the stomach and in the perigastric nodes in a thirty year old Tunisian patient. Both the small bowel and the mesenteric nodes were evaluated histologically and immunochemically and were found to be free of disease. Complete remission was obtained using chemotherapy. The reasons for which the small intestinal mucosa and the mesenteric nodes, nearly constantly involved, in alpha-chain disease were spared in our case, are discussed.

Adult↗
PubMed1979

P phenotype observed in two generations of Tunisian family with a high rate of inbreeding.

A p phenotype was discovered in a Tunisian woman and in three of her seven children. This paradoxical result challenges the known hypotheses on this phenotype, which generally recognise that it is determined by the existence of a very rare allele in a double dose. The computation of the coefficient of inbreeding of these subjects showed that repeated intermarriages increased the coefficient of kinship of the propositus and her husband to 0.116 (if unknown women of the ancestry were assumed to be different for each child) through 0,1362 (if unknown women of the ancestry were assumed to be the same for sibs). Therefore inbreeding was proved to be so high as to induce the presence of a recessively defined phenotype in two successive generations. Moreover, the search for probability of origin of genes of the last generation showed that the BCH ancestor was probably the carrier of the p allele.

Alleles↗
PubMedDate not supplied

Seroepidemiology of hepatitis B, C and delta viruses in Tunisia.

Serum samples from 33,363 healthy people in Tunisia have been tested for serological markers of hepatitis B, C and delta viruses (HBV, HCV and HDV). Hepatitis B surface antigen (HBsAg) was detected in 6.5% of sera. The overall seroprevalence of HBV was 37.5%. Vertical and perinatal transmission of HBV in the first 3 months of life occurred in only 0.4% of 177 mother and child pairs. HBV seroprevalence was 10.7% in infants under 5 years old and increased with age rapidly till 25 years of age and then more slowly in adulthood, reaching 54% for people aged over 40 years. HBsAg seropositivity varied throughout the country, ranging from 3% to 13% with higher prevalences in the south and central-west regions. Overall seroprevalences for HDV and HCV were 17.7% and 0.4%, respectively. HDV superinfection occurred later than HBV and increased with age in parallel with HBV. Overall, HCV and HBV infections had different geographical distributions throughout the country. The study confirmed the high prevalence of HBV infection in Tunisia; it occurs mainly in children and teenagers, and vertical and perinatal transmission of HBV does not appear to be significant. HDV superinfection is quite common in Tunisia and occurs in almost 44% of individuals infected with HBV. In contrast, seroprevalence of HCV in the Tunisian general population was low (0.4%). These results indicate differences in the distribution of the viruses and/or different routes of transmission.

Adolescent↗