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Biomedical subjects

H Braham

Publications and source records attributed to H Braham.

7 recordsLinked to original sources

[Prevalence of Epstein-Barr virus in Sjögren's syndrome in Tunisia].

PURPOSE: The cause of Sjögren's syndrome is unclear. Several studies suggested the role of Epstein-Barr virus (EBV) in the pathogenesis of this syndrome, but this always remains a subject of numerous controversies. The purpose of this study was to evaluate the prevalence of EBV in Sjögren's syndrome in Tunisia. METHODS: A series of 31 paraffin-embedded biopsies of salivary glands from patients with Sjögren's syndrome were studied in comparison with 19 control glands. EBV was investigated by PCR, EBERs in situ hybridization and by immunohistochemistry for the detection of LMP1, EBNA2 and ZEBRA. RESULTS: EBV DNA was detected by PCR in 3 of 22 PCR beta-globin positive Sjögren's syndrome cases (13.6%) and in 2 of 17 PCR beta-globin positive control glands (11.7%); in situ hybridization positivity was noted in rare lymphocytes in the 3 EBV positive cases of Sjögren's syndrome, but not in control glands; immunohistochemical study was negative in all cases. CONCLUSION: EBV infection does not appear to play a significant role in the pathogenesis of Sjögren's syndrome in Tunisia.

Adult↗

[Werner's syndrome and endocrine disorders].

Werner's syndrome is a rare autosomal recessive disease caused by the mutation of DNA helicase gene (WRN), characterized by the premature onset of multiple age-related disorders and skin changes similar to those observed in scleroderma. Some endocrinologic and metabolic disorders have been described in patients with Werner's syndrome. We report one case in a 41-year-old man issuing from consanguineous parents, who presented for exploration of hypoglycemic episodes and sexual impotence. Werner's syndrome was diagnosed on the basis of his characteristic clinical appearance. Metabolic disorders were insulin-requiring diabetes and hypertriglyceridemia. Endocrinologic investigation revealed nodular goiter, sub clinical primary hypothyroidism, hypergonadotrophic hypogonadism,adrenal cortical hypofunction and GH deficiency. Pathology examination of the skin biopsy showed a scleroderma-like aspect. Finally, osteoporosis, atherosclerosis and sub-capsular cataract were associated. Thus, in Werner's syndrome metabolic and endocrinologic investigation is necessary in order to treat these disorders and improve the patient's prognosis and life.

Adrenal Insufficiency↗

The primary hereditary form of distal renal tubular acidosis: clinical and genetic studies in 60-member kindred.

A distal (type 1) renal tubular acidosis (RTA-1) has been studied in 60 of 69 living members of a large family "HK" and two unrelated small families. The "HK" family, including 28 RTA-1 subjects, presents the first large family with only primary RTA-1 reported to date. The genetic situation in this family confirms the autosomal dominant transmission of the hereditary primary RTA-1 suggested previously on the basis of a few small families. Our data show that, in contrast to the secondary hereditary form, RTA-1 in its primary hereditary form is always complete and often tolerated (asymptomatic). It occurs in non-hypercalciuric families with no clinical variants observed in family members without RTA-1. In our series some clinical abnormalities commonly associated with RTA-1, such as nephrocalcinosis and growth retardation, appeared only in three cases among offspring when both parents were affected. The appearance of such abnormalities, taken as consequences of chronic acidosis in RTA-1, could be favored by the genetic background and/or the homozygosity for the RTA-1 gene. Linkage studies between RTA-1 and 10 genetic markers have been carried out. Results show that only ABO, MNS, GM and RH loci are informative for linkage analysis and none of these loci can be suggested as linked to RTA-1 locus.

Acid-Base Imbalance↗

[Malignant otitis externa (2 cases)].

Malignant external otitis is a progressive necrotizing otitis. It's a rare severe and evolutive clinical entities, old diabetics are the most victim. Pseudomonas aeruginosa is the bacteria responsible in the most cases. Prognostic vital can be affected, treatment must be energic, rapid and well adapted. The authors report two old diabetic women presenting a malignant external otitis and discuss etiopathogenic, physiopathologic, diagnosis and treatment of this illness.

Anti-Bacterial Agents↗

[Meningococcosis in Tunisia. Apropos of 80 cases].

Meningococcosis are nowadays an health problem because their incidence rate (38 cases/100,000 people in 1986 in Mahdia region) and a high death rate (40%) due to fulminating forms. At the occasion of a prospective study during the first six months of 1987, the authors report the emergence of C serogroup (21%), the smallness of A serogroup (6.50%) and the predominance of B serogroup (68%). Improving level of living, good prophylaxy based on spiramycin, development of use of meningococcal vaccine are advisable means to control epidemic outbreaks.

Adolescent↗