[An uncommon cause of venous thrombosis].
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Biomedical subjects
Publications and source records attributed to H Lévesque.
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The aim of this retrospective, study was to assess the tolerance and therapeutic effect of a stable prostacyclin (iloprost) analog in severe forms of permanent lower limb ischemia. Ninety consecutive unselected patients, in Leriche and Fontaine stages III or IV, turned down for vascular surgery after angiography and treated with iloprost for 28 days were enrolled in the study. Patients were followed up clinically (ischemic pain, trophic changes, walking distance) and with transcutaneous oxymetry (D28). Long-term assessment (mean 2 years) was expressed as rates of death, major amputation and "patients alive with limb". There were no manifestations of intolerance to iloprost. At two months, 42 out of 90 patients (47%) were considered as responders because of a lack (n=36) or significant decrease (n=6) in pain, reduction of trophic lesions and conservative walking. At long term (6 months, one and two years) we observed that 10 (11%), 17 (20%) and 22 (25%) patients respectively had died, 24 (27%), 26 (30%) and 28 (32%) patients underwent major amputation, but 60 (68%), 54 (62%) and 49 (56%) patients still alive with their limb and conservative walking. No predictive factors were noted, but diabetic patients without microangiopathy or recent bypass occlusions (respectively 43% and 56% out of patients were alive with limb at 6 months) were associated with bad results. This retrospective study, despite its limitations, underlines the good tolerance to, and effectiveness of iloprost in non surgical chronic critical ischemia. However, no predictive criterion of long-term effectiveness could be established, except initial clinical severity and clinical change one month after treatment.
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INTRODUCTION: Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. CURRENT KNOWLEDGE AND KEY POINTS: The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment. FUTURE PROSPECTS AND PROJECTS: Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.
INTRODUCTION: Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complications exist, including lung cancer, alveolar hemorrhage, and in particular isolated pulmonary arterial hypertension, which is still considered the bête noire as regards this disease. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of pulmonary arterial hypertension has been reported to range from 5 to 60% in cases of systemic sclerosis; isolated pulmonary arterial hypertension has been principally observed in subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific respiratory clinical manifestations, the diagnosis is made at a much later stage in the course of the disease. The diagnostic method of choice is echocardiography-doppler, which should be performed during the preliminary investigation, and at follow-up. The prognosis is poor, and patient survival rate at 2 years after onset of symptoms amounts to 40%. To date, no curative therapy for pulmonary arterial hypertension has yet been found. FUTURE PROSPECTS AND PROJECTS: A knowledge of the mechanisms involved in the development of isolated pulmonary arterial hypertension is essential to the determination of new and relevant therapeutic strategies. Vasodilatory treatment, notably calcium channel blockers, prostacyclin and analogs such as iloprost, may be effective at an early stage of the disease before the appearance of permanent vascular damage.
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INTRODUCTION: Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure. EXEGESIS: We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest. CONCLUSION: Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.
INTRODUCTION: Though impairment of the gastrointestinal tract is commonly encountered in patients with systemic sclerosis, the most frequent abnormalities are esophageal and anorectal disorders. Involvement of the small intestine is also common, reaching a 40-80% prevalence. It often leads to life-threatening complications. CURRENT KNOWLEDGE AND KEY POINTS: The occurrence of small intestine impairment and its potential relationships with other organ impairment is still unknown. However, it rarely indicates the existence of the disease (10%) which remains asymptomatic for a long period. As clinical symptoms are non-specific and radiological tests (upper intestinal tract barium meal, gastrointestinal transit times of radiolabeled meal, computerized tomography scan) not sensitive enough to detect the symptoms, diagnosis of small intestine impairment is delayed, i.e., when severe complications such as malabsorption or pseudo-obstruction are present. The physiopathology of small intestine disorders is still unclear, leading to both collagenous fibrosis and atrophy of muscle fibers. As well, its treatment is difficult. FUTURE PROSPECTS AND PROJECTS: Knowledge of the mechanisms at the origin of small intestine impairment in the course of systemic sclerosis is important for the development of efficacious therapies. Manometry of the small intestine would be a useful tool to assess the various motor abnormalities that may occur in patients presenting with systemic sclerosis associated with either malabsorption or pseudo-obstruction. It would also provide a useful test in selecting patients whose treatment require somatostatin analogs.
PURPOSE: Interferon alpha treatment for virus C hepatitis may be responsible for autoimmune thyroiditis. Relationships between thyroiditis and virus C infection are still debated. The aim of this study was to evaluate the existence of this association. METHODS: The prevalence of autoimmune thyroiditis in 58 patients (35 male and 23 female patients, mean age 52.6) with untreated virus C hepatitis was compared to that of 56 alcoholic patients (41 male and 15 female patients, mean age 53.8). Autoimmune thyroiditis was defined as the association of abnormal TSH and an increase in antithyroid antibodies. RESULTS: We did not find any statistical difference in either autoimmune thyroiditis or antithyroid antibodies prevalences. CONCLUSION: Both our results and a literature review suggest that the few reported cases of related autoimmune thyroiditis and virus C infection are probably coincidental.
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Lupus-like anticoagulant is commonly encountered in human immunodeficiency virus infection although thromboembolic manifestations are rare in HIV patients. We report the case of an HIV patient who developed gangrene of both forefeet associated with anticardiolipin antibodies. A 42-year-old woman had a 12-year history of HIV infection (stage B2). She presented with painful gangrene involving the forefeet of 4-day duration. Doppler ultrasonography, electromyography and nailfold capillaroscopy were normal. Skin biopsy revealed intracapillary thrombi and severe necrosis within the hypodermis; there was no evidence of vasculitis. Laboratory findings showed a marked inflammatory syndrome and the presence of anticardiolipin antibodies (IgG: 22 GPL U/ml). Several cutaneous manifestations are known to be associated with antiphospholipid syndrome, such as livedo reticularis, ulcers and gangrene of the extremities. Skin biopsy often shows noninflammatory thrombosis of small vessels within the dermis. Microcirculation damages have also been described in HIV infection, mainly vasculitis. In the present case report, the absence of both vasculitis and other causes suggest that anticardiolipin could be the culprit. But, it is possible that painful gangrene of the forefeet was secondary to HIV infection.
Marfan syndrome is an hereditary condition which primarily affects conjunctive tissue with predominant vascular lesions, aortic insufficiency and aortic dissection which condition vital prognosis. Until further progress is made in the genetic determination of the disease, the diagnosis is currently based on the association of clinical criteria, which enables multidisciplinary management. This approach should lead to specific medical and surgical treatment--which may reduce Marfan morbidity and mortality. We report the case of a 45 year-old patient with suspected Marfan syndrome during adolescence. The presence of a cardiovascular lesion and a recently reported abnormality i.e. a sacral erosion by a dural ectasia, enabled us to confirm the diagnosis. We reviewed the current criteria in Marfan diagnosis and their specific management.
Palpable purpura is the hallmark of cutaneous vasculitis. Small-vessel vasculitis is a common vasculitis manifestation associated with acute or chronic infection. It is also characteristic of a systemic disease whether infectious or not. The pathogenic mechanisms appear to be complex: immune complex formation, vessel damage or altered vessel function mediated directly by infectious agents, humoral or cellular immunologic response. It is also a reaction to mixed cryoglobulinemia. Diagnosis of cutaneous vasculitis is simple (palpable purpuric eruption, nodules, vesiculobullous lesions, ulcerations), but etiological investigation is often difficult because the infectious origin is only rarely demonstrated. This type of purpura occurs in bacterial endocarditis and therefore blood cultures must be performed in any febrile patient particularly in the presence of a cardiac murmur. In fact the viral, parasitic or bacterial infectious origin is demonstrated in less than 30% of the cases of leucocytoclastic vasculitis. While focal sepsis is often found and its eradication should be followed-up, its role has not been proven particularly as antibiotics alone themselves can cause hypersensitivity vasculitis. Finally, mention must be made of virus induced vasculitis (B and C hepatitis, cytomegalovirus, parvovirus), antiviral treatment which permits better control of vasculitis.