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M Bejaoui

Publications and source records attributed to M Bejaoui.

33 records · Page 2Linked to original sources

[Epidemiological pattern of the resistance of 153 Salmonella strains (S. typhi excluded) isolated in a Tunisian pediatric unit from 1985 to 1990].

Since 1985 to 1990, 153 isolates of non typhoidic Salmonella were recovered from pediatric unit of the Charles Nicolle Hospital in Tunis. The epidemiological profile of these isolates was established according to the biochemical and serological characterizations, the antibiotic susceptibility patterns (disk diffusion method) to betalactams and aminoglycosides. 127 isolates of Salmonella spp were from stools and 22 from blood cultures. The main serotypes were S. ser. Wien (108 isolates) and S. ser. Typhimurium (21). A sample of 50 isolates was selected among the different resistance phenotypes for determination of MICs and betalactamase identification by isolelectro-focusing (36 isolates). All of them were resistant to ampicillin and carbenicillin but variable for cephaloridine. Since 1986 appeared the resistance to cefotaxime and in 1990 all the S. ser. Wien isolates were resistant to the 4 betalactams tested including cefotaxime associated to streptomycin, kanamycin, gentamicin, tobramycin and amikacin. All of them had a high level of resistance to amoxicillin and gentamicin (mean MICs > 2048 and > 512 mg/l). The mean MIC of cefotaxime was 43.2 mg/l for S. ser. Wien, 64mg/l for S. ser. Typhimurium and decreased to 1.9 and 0.12 mg/l in combination with 1 mg/l of clavulanic acid. The beta-lactam resistance was mediated to enzymatic mechanism, at first the betalactamase of TEM-1 type alone, then associated to extended-spectrum betalactamase, mainly type SHV-2 since 1986, and other new types in 1990. They were under the control of plasmids with different sizes.

Aminoglycosides↗

[Lymphoid hyperplasia of the intestine in children. 15 cases].

Fifteen cases of nodular lymphoid hyperplasia (NLH) of the bowel in patients aged 17 months to 15 years are reported. Fourteen patients had NLH confined to the small bowel and one had involvement of both the small bowel and colon. Mean age at diagnosis was 10 years. The most common presenting symptoms were intestinal manifestations (86%). Diagnosis was suspected upon roentgenographic studies in one case and digestive endoscopy in ten cases. Histologic confirmation was obtained in all fifteen patients. Immunohistochemical studies, done in 8 patients, demonstrated a paucity of IgA plasmocytes in one patient with an immune deficiency and a polyclonal plasmocyte population with mainly IgA plasmocytes in the seven other patients. Five patients had a deficiency in humoral immunity, with variable expression hypogammaglobulinemia in three patients and IgA deficiency in two; intestinal giardiasis was found in eight patients. Histologic outcome was documented in five cases; evidence of NLH disappeared in only one patient. Metronidazole improved clinical symptoms in most instances.

Adolescent↗

[Syndrome associating: osteopetrosis, tubular acidosis, mental retardation and cerebral calcifications due to carbonic anhydrase II deficiency. Apropos of 3 cases in the siblings].

Three new cases of a syndrome including osteopetrosis, tubular acidosis, mental retardation and cerebral calcifications are reported in the same sibship. This rare syndrome has an autosomal recessive inheritance and is due to carbonic anhydrase II deficiency. Fractures and mild sensorial manifestations were noticed in 2 cases. Mental deficiency and otitis were present in the 3 cases. Blood count and phosphocalcic metabolites were normal. A proximal tubular acidosis was present in each case. The radiological features were specific: cerebral calcifications were present in all cases since the age of 2 1/2 years; osteocondensation and bone modeling were predominant in long bones metaphyses. Alcalinization improved the height growth.

Acidosis, Renal Tubular↗

[The accelerated phase of Chediak-Higashi syndrome].

We have retrospectively analysed the clinical and biological features as well as the outcome of 18 accelerated phases having occurred in 11 patients with the Chediak-Higashi syndrome. This complication is very frequent and is characterized by a multi-visceral lymphohistiocytic infiltration with hemophagocytosis leading to pancytopenia, a bleeding disorder secondary to low fibrinogen level, hypertriglyceridemia and hemodilution. The accelerated phase of the Chediak-Higashi syndrome is identical to the manifestations of familial erythrophagocytic lymphohistiocytosis and of the viral-associated hemophagocytic syndrome. The outcome was invariably fatal before the use of etoposide (VP 16) in association with steroids and intrathecal methotrexate. Complete remission with this management regimen was observed in 7/7 cases. However, remissions were only transient. HLA identical bone marrow transplantation appeared to be the only therapeutic strategy capable of curing the disease (3/3 patients). Non transplanted patients relapsed and died as well as one patient who received a HLA non identical bone marrow transplantation. Due to the frequency and the severity of the accelerated phase of the Chediak-Higashi syndrome, HLA identical bone marrow transplantation should be proposed as early as possible after the onset of the accelerated phase.

Adrenal Cortex Hormones↗

Genetic and immunological assessment of a bone marrow transplantation in a patient with a primary immune defect: leukocyte adhesion deficiency.

Leukocyte adhesion deficiency (LAD) was suspected in a three weeks old girl from a family with an established history of LAD with a lack (less then 1%) of the beta 2 integrins CD 11a, b/CD 18 expression at the leukocytes surface, was engrafted with her mother HLA identical bone marrow at the age of 14 months. Repeated post transplantation (up to 22 months). Immunological assessments showed a good engraftment with 97% of the lymphocytes expressing CD11a/CD18. Cells proliferated normally in response to PHA and to Tetanus toxoïd after revaccination. The level of serum immunoglobulins was normal. Investigation of the CD18 intragenic polymorphic marker Avall before and after bone marrow transplantation (BMT) showed a transition from the Avall +/+ genotype to the mother's Avall +/- genotype. Similarly DNA fingerprints obtained with the patient genomic DNA, prepared from PBMC, prior and after transplantation, showed that the patient's DNA fingerprints pattern matched the mother's one. These findings are consistent with the good engraftment observed clinically. This study emphasizes the usefulness of the molecular techniques to evaluate the degree of chimerism in monitoring the outcome of bon marrow transplantation.

Bone Marrow Transplantation↗

[Recessive osteopetrosis. Identification of a form of medium severity].

BACKGROUND: Several distinct forms of osteopetrosis have been identified. Some of the autosomally recessive inherited forms are benign, much like the autosomal dominant form. Others are more malignant. PATIENTS: The clinical data, skeletal radiographs, histological features and histories of 32 children with osteopetrosis were analyzed retrospectively. RESULTS: The 32 patients, belonging to 20 sibships were divided into two groups. The first group included 24 patients, aged 1 day-11 months (mean 4.5 months), suffering from hepatosplenomegaly, anemia, thrombocytopenia and optic atrophy in early infancy. They also had a generalized increase in bone density, abnormal bone remodeling, rachitic lesions and a "bone-within-bone" appearance. Biopsies showed severe bone resorption and myelofibrosis. 19 of the 20 patients whose outcomes were known died during the first year of life. The second group included 8 patients, aged 40 days-3 years (mean: 11 months). Hepatosplenomegaly appeared later, anemia was less severe and thrombocytopenia occurred in only 1 patient. However, all 8 patients suffered from optic atrophy and 3 were deaf. Radiographs showed bone growth without rachitic lesions. Biopsies from 2 patients showed bone resorption, but no myelofibrosis. The outcome was less severe: 6 patients, now aged 8 months to 8 years, have survived, 3 of them for over 5 years. Genetic investigation showed patterns compatible with autosomal recessive inheritance in both groups, with similar sets of features within each sibship. CONCLUSION: This study reveals a new type of recessively inherited osteopetrosis. It can be classified as an intermediate form, distinct from both the malignant and the benign forms, and also distinct from osteopetrosis with carbonic anhydrase II deficiency.

Child, Preschool↗

Failure of immunosuppressive therapy and high-dose intravenous immunoglobulins in four transfusion-dependent, steroid-unresponsive Blackfan-Diamond anemia patients.

Blackfan-Diamond anemia (BDA) is a rare hypoproliferative anemia occurring in infancy or in early childhood. Patients who fail on usual doses of steroids did not achieve remission with other pharmacological agents. Claims that other molecules such as cyclosporin A (CSA) or antithymocyte globulin (ATG) are effective require substantiation. We treated four transfusion-dependent, steroid-unresponsive BDA patients with ATG and methylprednisolone (MP). Only a transient response was obtained in one case. None of these patients responded to high-dose intravenous immunoglobulins (HDIg) or CSA.

Antilymphocyte Serum↗