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A Tohme

Publications and source records attributed to A Tohme.

16 recordsLinked to original sources

[Behçet disease. Genetic factors, immunologic aspects and new therapeutic methods].

PATHOGENESIS: The pathogenesis of Behcetp3disease is still unknown, although a genetic predisposition appears to play an important role with a strong association with the MICA gene located between the HLA-B and TNF genes rather than HLA B51. Abnormal immune responses affect especially cellular immunity and significant T-cell proliferative responses by the gamma ô subset of T cells are shown after stimulation with heat shock protein peptides. Systemic levels of the soluble TNF R-75 and IL 12 could be the best biological markers of disease activity. NEW THERAPEUTIC APPROACHES: Systemic colchicine being implicated in polynuclear neutrophil over-production of toxic super-oxides, its prescription for controlling eye involvement should be reevaluated. Steroids and immunosuppressive drugs are still the treatment of choice for severe manifestations of the disease. Beneficial effects of cyclosporine are established in uveitis. Tacrolimus (FK 506) and pentoxifylline may be valuable.

Adrenal Cortex Hormones↗

[Werner's syndrome].

BACKGROUND: Werner's syndrome associates early aging in young adults, small height, cataract, glucose intolerance, hypogonadism, skin ulcers, vascular calcifications and osteoporosis. CASE REPORT: We report a new case of Werner's syndrome in a 34-year-old man with suggestive alterations of the skin and endocrine anomalies in addition to hypospadias, urethral stenosis, bilateral mega-ureter and chronic renal failure. DISCUSSION: The diagnosis of Werner's syndrome in our patient was unquestionable because of the clinical presentation and the familial context. However, the urology anomalies have not been reported in this syndrome. A simple coincidence cannot be excluded.

Adult↗

[Neurologic manifestations in Behçet's disease. 16 cases in a cohort of 110 patients].

OBJECTIVE: To discuss neurological involvement of Behçet's disease and therapeutical possibilities. PATIENTS: Among 110 patients with Behçet's disease, 16 were found to have neurological involvement. All these patients fulfilled Japanese and International Study Group criteria for diagnosis of Behçet's disease. RESULTS: Central nervous system involvement was found in 15 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were the most frequent features (69%) followed by tumor-like manifestations (13%); cerebral venous thrombosis was identified in 1 patient with occlusion of the left lateral sinus. Focal deficits were the major presenting signs and cranial paralysis were present in 19% of patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. Cerebral CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 4, showed abnormal signals distributed over hemispheric white matter, the brainstem and the thalamus in one patient, an occlusion of the left lateral sinus in the second one and a tumor-like lesion in the third. MRI abnormalities were associated with concording clinical deficits. CONCLUSION: Neurological involvement in Behçet's disease con be classified into 3 clinical aspects: meningoencephalitis (and/or myelitis), cerebral venous thrombosis and tumor-like features. Corticosteroids, when prescribed early, are useful and are associated with better prognosis.

Adult↗

[Scleroderma with anomalies of the thyroid function. 7 cases].

Thyroid function, studied in 36 scleroderma patients revealed 7 abnormal cases: 6 hypothyroid patients secondary to autoimmune thyroiditis and 1 hyperthyroidism secondary to Graves' disease. In the hypothyroid subgroup, 3 cases presented a localized systemic sclerosis and the 3 others presented a diffuse systemic sclerosis; Sjögren syndrome was found in 2 of these patients. The hyperthyroid patient presented a diffuse systemic sclerosis. Because of the association between scleroderma and thyroid diseases, we suggest to perform thyroid screening regularly for all patients with systemic sclerosis.

Adult↗

Behçet's disease in Lebanon: report of 100 cases.

PURPOSE: To present clinical features of Behçet's disease in Lebanon and to evaluate the efficacy of treatment. PATIENTS AND METHODS: The 100 patients are seen at Hotel-Dieu de France Hospital of Beirut between 1980 and 1992. Diagnostic criteria are those of the Behçet Syndrome Research Committee of Japan. RESULTS: Recurrent oral ulcers are present in 95% of cases, genital ulcers in 78%, ocular manifestations are more frequent than those of several studies whereas skin lesions and vascular signs are less frequent. Joint involvement are recorded in 65% of cases and neurological one in 14%. The effectiveness of colchicine therapy is confirmed for mucocutaneous lesions and arthritis. Immunosuppressive agents are more beneficial than steroids alone on serious uveitis. An aggressive treatment with corticosteroids and immunosuppressive drugs improve the prognosis of meningoencephalitis. CONCLUSION: Behçet's disease doesn't have any specific characteristics in Lebanon despite some variation in the frequency of lesions.

Adolescent↗

[Bullous pemphigoid and multiple sclerosis].

The occurrence of bullous pemphigoid (BP) in patients with multiple sclerosis (MS) is uncommon. The aetiology is not clear and several abnormalities of the immune system are reported in both pathologies. We report one additional case in a 40-year-old woman with a 15-year history of MS. BP was confirmed histologically and immunologically. The therapy with steroids was successful and no recurrence of BP was observed after a six month follow-up.

Adult↗

Familial macroglobulinemia in a Lebanese family with two sisters presenting Waldenström's disease.

We report a non-consanguineous family with ten children, in which two sisters were found to have Waldenström's disease with light chain IgM monoclonal components. Immunoglobulins were examined in four siblings and revealed high serum IgM concentrations with no monoclonal component. This additional case of familial Waldenström's macroglobulinemia stresses the usefulness of screening family members of patients with monoclonal gammopathy since they may be at high risk of developing the disease.

Adolescent↗

[Unusual presentation of typhoid fever. Apropos of 3 cases].

Typhoid fever is still an endemic disease in Lebanon with a risk of mortality especially in the immunosuppressed patients. We report 3 atypical observations and discuss cardiopulmonary, neurological, hematologic and urinary manifestations of this disease.

Adult↗

[Vertebral actinomycosis: case report and review of the literature].

In this article, the case of a 32-year-old man with a paravertebral actinomycosis is discussed. Initially, the diagnosis was not obvious but it was confirmed later with the repetitive radiologic procedures, the elimination of other etiologies (purulent, mycobacterial or mycotic infections and neoplasia) and the biopsy. Treatment with penicillin initially and then with tetracycline for a long term led to a very good outcome at a 3-year follow-up with a radiologic remission. Following the discussion of the case, a review of the literature concerning the paravertebral actinomycosis, its diagnostic clues and treatment is undertaken.

Actinomycosis↗

[Neonatal lupus erythematosis and atrial-ventricular block. A case report and review of the literature].

Neonatal lupus erythematosus is a rare syndrome. It is characterized by a transient lupus dermatitis and congenital heart block. The immunopathogenesis of the disease has been linked to the presence of the SSA antibody. In this report, we describe a symptomatic congenital heart block in a 13-year-old male whose mother had documented systemic lupus erythematosus. We also discuss how to manage a woman with lupus erythematosus and positive antiSSA/Ro antibodies. We conclude that patients with neonatal lupus and their mothers should be observed closely before delivery and for prolonged periods for signs of active disease.

Adolescent↗