Pseudoxanthoma elasticum: a report of 11 cases

INTRODUCTION: Pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue with many systemic manifestations PATIENTS AND METHODS: We performed a retrospective study from all the patients diagnosed with PXE at the department of dermatology of La Rabta hospital of Tunis, between 1986 and 2003. RESULTS: During the observation period, we identified 11 patients with PXE, 5 males and 6 females with a mean age of 28 years (10-47 years). Family history was found in 5 patients. Exhibit yellowish, pigskin, and popular lesions on the sides of the neck were observed in all cases. Systematic ophthalmologic examination revealed angio?d streaks in 4 patients. No abnormalities were found in cardiovascular and metabolic explorations. DISCUSSION: Diagnosis of PXE is based on clinical, histological and genetic criteria. Ocular and cardiovascular damage make all the gravity of the disease, from where interest of an ophthalmologic and cardiovascular examination systematic.     

Bronchopulmonary aspergillosis: study of 17 cases

Aspergillosis is a fungic infection depending on the local or general physiologic and immunologic state of the host. We report the result of retrospective five year study (1995-1999) about 17 cases in the laboratory of Parasitology-Mycology of Rabta hospital in Tunis. Six aspergillomas were observed, they occurred after a pulmonary tuberculosis, two cases of allergic broncho-pulmonary aspergillosis described in two asthmatic patients, nine cases of invasive pulmonary aspergillosis complicating two cancers, one leukaemia, six chronic granulomatous disease. Aspergillus fumigatus is the most frequent species (67%). The clinical and biological characteristic of those will be studied, and compared with those of the literature.     

The results of anterior cruciate ligament reconstrution by bone--patellar tendon--bone technique. A series of 40 cases

We reviewed retrospectively 40 patients treated surgically for rupture of anterior cruciate ligament. The technique used was arthroscopy bone patellar tendon reconstruction. The mean age of patients was 27 years. The operative delay was 27.2 months in average. Functional and anatomic result was evaluated with a mean follow up of 24 months. 80% of patients were classified IKDC A or B. Sports activity was resumed after 6.6 months in average. Results depented meniscus lesions and operative delay. Reconstruction of anterior cruciate ligament with bone patellar tendon bone is a reliable technique. Operative delay should be as short as possible to prevent meniscus lesion and aggravation of laxity.     

Role of mitochondrial dysfunction in non-alcoholic steatohepatitis

The non alcoholic steatohepatitis is a more and more frequent disease. Great progress in the physiopathologic mechanisms has been recently observed. The mitochondrial dysfunction seems to be the main mechanism implied in the necroticoinflammatory genesis of the non alcoholic steatohepatitis lesions. When this one is of metabolic origin, this dysfunction occurs on a vulnerable to aggressions steatosis liver. Several implied factors leads to the increased liberation of free radicals that will activate the lipidic peroxydation. This one is considered as the main generator of necroticoinflammatory lesions and fibrosis. The future therapeutic alternatives depends on a better comprehension of mitochondrial dysfunction.     

Epidemiological and clinical features of giant cell arteritis in Tunisia

BackgroundGiant cell arteritis (GCA) is a systemic vasculitis of the elderly that could result in vision loss or even be life threatening. Unlike western countries, this disease is considered exceptional in Tunisia.ObjectiveThe aims of this study were to determine epidemiological and clinical features of GCA in Tunisian population and to identify management difficulties.Patients and methodsA multicentric study of 96 patients in whom GCA was diagnosed between 1986 and 2003. All patients fulfilled the ACR criteria for classification of GCA.ResultsThe majority of cases (77%) were diagnosed since 1994. The male/female ratio was 0.88 and the mean age at the time of diagnosis was 70.8 ± 7.7 years. Clinical features were characterized by gradual onset in 64.4% of cases. The most frequent clinical manifestations were headache (91.7%), abnormalities in temporal arteries (85.4%), severe ischemic manifestations (80.2%), constitutional symptoms (75%), and polymyalgia rheumatica (56.3%). Biological inflammatory syndrome was noted in all patients. Temporal artery biopsy established histological diagnosis in 73% of cases. All patients were treated by corticosteroids. Remission was obtained in 45.6%. Relapses occurred in 40.4% of cases and 30 patients were still receiving corticosteroids at the time of study. Four patients died and irreversible ischemic complications were noted in 15.6% of cases. Steroid adverse effects occurred in 56 patients.ConclusionGCA is not exceptional to Tunisia. It occurs amongst elderly patients with no female predominance noticed. Clinical features are similar to those reported in other series.