Biomedical subjects
Publications and source records attributed to M Miled.
INTRODUCTION: The antiphospholipid syndrome had been rarely reported as a cause of mesenteric venous thrombosis. CASE REPORT: We report two exceptional cases of primary antiphospholipid syndrome associated with mesenteric venous thrombosis in 51 years old man and 27 years old women. DISCUSSION: Identifying the underlying process is very important to try to propose a specific treatment.
INTRODUCTION: Adult onset Still's disease is a systemic inflammatory disorder of unknown etiology characterized by the association of a high spiking fever, an evanescent skin rash, arthritis, and hyperleukocytosis. Pericarditis is amongst the most common systemic manifestations of adult onset Still's disease. EXEGESIS: We report on two patients with a pericardial tamponade revealing an adult onset Still's disease in a 52-year-old female and a 31-year-old male. Pericardial fluid was bloody in the two cases, and histopathology only disclosed non specific inflammatory changes. Both patients received corticosteroids and outcome was uneventful with a follow-up of 8 years and 12 months, respectively. CONCLUSION: Pericardial tamponade is an uncommon clinical feature of adult-onset Still's disease and usually occurs at disease onset. It makes the diagnosis of adult-onset Still's disease difficult as the other disease manifestations are commonly neglected. Adult onset Still's disease should be added to the differential of acute pericarditis and tamponade.
INTRODUCTION: Arterial involvement in Behçet's disease is rare, present in 2-8% of cases. Aortic aneurysms represent the most frequent arterial lesions encountered in this disease and are associated with high mortality. CASE REPORT: We report the exceptional observation of an abdominal aortic aneurysm ruptured to the duodenum, responsible of a massive gastro intestinal bleeding in a patient with Behçet's disease requiring an emergent surgical intervention. The outcome was favorable. DISCUSSION: The clinical presentation of abdominal aortic aneurysms in Behçet's disease is often atypical leading to an important diagnosis delay and favours the rupture of the aneurysm.
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Behçet's disease is a multi systemic vasculitis of unknown origin characterized by recurrent oral and genital ulcerations, uveitis and skin lesions. Vascular involvement mainly interesting the venous system is present in about 7 to 38% of patients. Arterial involvement is present in 2 to 8% of patients and is associated with a high mortality. It may present as aneurysms, pseudo aneurysms or arterial occlusions. Pseudo aneurysm formation has been reported to be sometimes due to invasive imaging techniques, it may also follow arterial repair. We report two exceptional localizations of iatrogenic pseudo aneurysms due to accidental arterial puncture, involving respectively the internal carotid artery and the humeral artery. We discuss the clinical characteristics and therapeutic modalities of this complication.
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OBJECTIVE: To investigate a possible pathogenic role of cytokines in Behçet's disease (BD) by focusing on the analysis of cytokine gene expression within mucocutaneous BD lesions. METHODS: The study group comprised 20 patients with active BD. In this group, a set of chemokines as well as Th1 and Th2 cytokines in biopsy specimens obtained from oral and genital ulcers, pseudofolliculitis lesions, and lesions at the site of pathergy testing were studied using real-time quantitative reverse transcriptase-polymerase chain reaction. RESULTS: We observed important increases in the expression of interleukin-8 (IL-8) ( approximately 700-fold), monocyte chemoattractant protein 1 ( approximately 65-fold), interferon-gamma ( approximately 71-fold), and IL-12 ( approximately 69-fold) messenger RNA in BD lesions compared with normal skin. Except for IL-10 ( approximately 75-fold increase), Th2 cytokines (i.e., IL-4 and IL-13) were absent. CONCLUSION: Our data suggest a direct role of Th1 lymphocytes in the pathogenesis of mucocutaneous BD lesions.
UNLABELLED: Gaucher's disease is one of the rare lysosomial disease that could receive substitutive enzymatic treatment which may improve considerably the prognosis of certain forms. The purpose of this work is to study the epidemiology of the disease in Tunisia, to highlight the diagnostic and therapeutic difficulties and also to precise our subsequent needs for substitutive medication. PATIENTS AND METHODS: We have conducted a retrospective survey of the hospital wards that were susceptible to take care of patients having Gaucher's disease. These wards are the paediatric, neonatology, internal medicine, haematology, neurology and cardiology wards. RESULTS: In this study we have observed 27 cases of Gaucher's disease over a period of 18 years (1983-2001). The age at onset ranges from birth to 73 years of age, with an average age of 14.5 years. According to the age at onset and the clinical presentation, we classify our patients into: 20 cases of type 1 (74%), three cases of type 2 (12%), and three cases of type 3 (12%), and one case of unspecified type.Gaucher's disease type 1: The age at onset ranged from 10 months to 73 years with an average of 19 years. The main clinical signs that we have observed were splenomegaly, hepatomegaly, pallor, haemorrhagic appearance and also osteoporosis and bone pain observed in 40% of the cases. The diagnosis was based on histology showing the Gaucher's cells in various tissues while the diagnosis obtained by the dosage of glucocerebrosidase took place only in 50% of the cases. The treatment has always been symptomatic (analgesics, transfusion). A splenectomy was performed in 47% of the cases and none of the patients received a specific treatment. The follow-up period ranged from 1 month to 18 years with an average follow-up of 4 years. Among the 12 patients having a follow-up of at least 1 year, we have noticed an improvement after splenectomy in three cases, a stability in three cases and two worsening cases dealing mainly with bone problems. One patient aged 73 died from respiratory problem and three were lost to follow-up. Gaucher's disease type 2: We have observed three cases of Gaucher's disease type 2 diagnosed at 1 day, 45 days and 3 months of age. The visceral manifestations were serious and the neurological features included seizures, hypertony, ocular-nerve palsies and psychometric decline. The three patients died. Gaucher's disease type 3: Three patients were probably suffering from Gaucher's disease type 3 with visceral manifestations observed at the ages of 9 months, 1 year and 3 years, and also neurological signs observed at respective ages of 2.5 and 3 years. Two patients died and the remaining one was lost to follow-up. CONCLUSION: Gaucher's disease is not exceptional in Tunisia. Type 1 is by far the most common one. We have noticed some insufficiency in the diagnosis as the glucocerebrosidase enzymatic dosage was performed only in 50% of the cases as well as therapeutic insufficiency with no prescription of the specific treatment.
Cardiac tIssue is a very rare localization for benign lipoma. Interseptal lipoma and lipomatous hypertrophy of the interatrial septum are two distinguished entities. We report the case of a 58-year-old patient who developed lipomatous hypertrophy of the interatrial septum which was revealed by arrhythmia. Diagnosis was suspected at magnetic resonance imaging and was confirmed intraoperatively and histologically.
There is a wide variation in the natural history of systemic lupus erythematosus (SLE) among different ethnic and geographical groups. Studies in Arabs are few and those in North Africans and especially in the Tunisian population do not exist. This study aims to demonstrate the demographic, clinical and laboratory characteristics of SLE Tunisian patients and to identify those at high risk for renal and neuropsychiatric involvements. One hundred patients with SLE (American College of Rheumatology criteria), seen at the Department of Internal Medicine of the University Hospital La Rabta in Tunisia over a 15-year period (1987 to 2001) were retrospectively enrolled. There were 92 women and eight men with an average age at the onset of disease of 32 years. Nineteen patients were aged over 50 years at the moment of SLE diagnosis (late-onset SLE). Of the patients, 78% had articular involvement, 53% photosensitivity and 63% malar rash. Serositis occurred in 45 patients of whom 16 had pericarditis and 29 had pleuritis. Nephritis was diagnosed in 43% of the cases and consisted always of glomerular nephritis, in three cases of which tubulointerstitial lesions were also observed. Comparison of patients with and without renal involvement showed that lupus nephritis was significantly associated with pericarditis (P = 0.03), arterial blood hypertension (P < 0.0001), cryoglobulinemia (P = 0.07) and antiphospholipid syndrome (P = 0.03). The SLEDAI at SLE diagnosis was significantly higher for lupus nephritis patients. Twelve patients with lupus nephritis died compared with three patients in the remaining group (P < 0.0001). Neuropsychiatric manifestations were observed in 25% of the cases. The mean age at SLE onset was significantly lower, the mean SLEDAI at SLE diagnosis and the mortality were significantly higher in the neuropsychiatric group than in the remaining group. Immunological features included antinuclear antibodies (100%), anti-DNA antibodies (56%), anti-Sm antibodies (61%), anticardiolipin antibodies (62%), anti-beta2GP1 (13%) anti-Rnp (23%) and hypocomplementemia (48%). The frequencies of pulmonary hypertension (25 versus 2%, P < 0.00001) and vascular thrombosis (25 versus 2%, P < 0.00001) were significantly higher in patients with positive anti beta2GP1 antibodies. The five-year survival rate in our series was 86%. The most frequent causes of death were active SLE and infections.
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INTRODUCTION: The concomitant unexpected arrival of a breast tuberculosis and cerebral tuberculomas to a same immunocompetent patient has not been ever described in the literature. EXEGESIS: We report the case of an non-HIV-infected woman who presented a pulmonary, breast tuberculosis and intracranial tuberculomas. Investigation for acid-fast bacilli in sputum and cerebrospinal fluid was negative. The patient received an antituberculous therapy for 15 months, which led to the disappearance of the cerebral lesions. CONCLUSION: Our case was particular by the affected organs, the absence of immunodeficiency and the favorable outcome.
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OBJECTIVES: We present a case report of Rosai-Dorfman disease with multiple extranodal involvement and favorable outcome after steroid therapy. MATERIAL AND METHODS: Rosai-Dorfman disease was diagnosed in a 17-year-old man with orbital, lacrymal, and rhinopharyngeal localizations. Histopathologic examination of lymphadenopathy biopsy revealed the diagnostic of Rosai-Dorfman disease. We present the histological findings leading to diagnosis and radiological data. RESULTS: Conservative treatment with oral steroids resulted in the resolution of cervical lymphadenopathy and extranodal lesions. The patient was free of problems nine mouths after discontinuation of his treatment, demonstrating its efficacy and safety. CONCLUSION: Rosai-Dorfman disease is a benign disorder and steroid treatment must be initiated in cases with significant cosmetic deformity.
PURPOSE: To describe epidemiological and clinical characteristics of neurological involvement in Behçet's disease (BD) and to determine a subgroup of patients at high risk for this complication. PATIENTS AND METHODS: The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behçet's disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests. RESULTS: Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 +/- 8.79 years. Nineteen patients (70.3%) had meningoparenchymal "MP" central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis: 1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behçet "CAB" (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both "MP" and "CAB" involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1 case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in "CAB" subgroups than in subgroup 2. CONCLUSION: Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behçet appears to be a protector factor against uveitis.