[An anterior thoracic painful cord].
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Biomedical subjects
Publications and source records attributed to N Cailleux.
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We report a case of Susac's syndrome (retinocochleocerebral vasculopathy) that was initially successfully treated with steroids. The patient had a recurrence of the disorder 2 years after the onset of the first clinical manifestations; this was treated with steroids and both calcium channel blockers and aspirin. Our findings underline the importance of recognizing patients with retinocochleocerebral vasculopathy at an early stage so that it may be accurately managed and, therefore, result in both decreased morbidity and disability related to severe sequelae. We also stress the need for patients with Susac's syndrome to have both close and long-term follow-up in order to disclose both symptomatic and asymptomatic recurrences of the disorder.
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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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We report the case of a patient with Whipple's disease (WD) who developed hypertrophic osteoarthropathy (HOA) characterized by digital clubbing, periostosis of the tubular bones, and polysynovitis. The HOA disclosed the recurrence of the patient's WD, since polymerase chain reaction (PCR) analysis clearly demonstrated the presence of Tropheryma whippelii in the synovial fluid from the patient's left knee. Initiation of appropriate antibiotic therapy resulted in complete healing of all clinical rheumatologic manifestations within 2 months and in disappearance of radiographic bone changes at 7-month followup. We suggest that HOA be included within the spectrum of rheumatologic manifestations of WD, and that an evaluation for WD should be considered in patients, especially middle-aged men, presenting with HOA even without gastrointestinal symptoms. PCR analysis may be useful in accurate diagnosis and management of early WD with unusual clinical manifestations, and may contribute to decreased morbidity and mortality.
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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.
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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.
Esophageal involvement is considered a major cause of morbidity and an indicator of poor prognosis in polymyositis (PM) and dermatomyositis (DM). We describe 3 patients with steroid resistant PM/DM with life threatening esophageal involvement, resulting in impossible oral feeding and enteral nutrition with a gastric tube. All patients had both dramatic and rapid improvement of all clinical manifestations after initiation of intravenous immunoglobulin (IVIG) therapy. Swallowing disorders completely disappeared after the second infusion of IVIG, which permitted normal oral feeding and ablation of the gastric tube. Our findings suggest IVIG should be considered the treatment of choice in such cases.