Cases reported "Disease Progression"

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1/52. Vanishing pulmonary hypertension in mixed connective tissue disease.

    A 29-year-old woman with mixed connective tissue disease presented with signs of progressive pulmonary hypertension. After admission to the hospital her condition worsened rapidly and she developed a cardiac arrest resistant to cardiopulmonary resuscitation. Therefore, emergency extracorporeal assist was performed. No pulmonary embolism was found. Right heart catheterisation showed severe pulmonary hypertension, which was treated with nitric oxide ventilation. She was weaned from the extracorporeal assist with high doses of inotropic agents. Because of suspicion of exacerbation of her underlying disease, which led to pulmonary hypertension, immunosuppressive treatment was started with high doses of corticosteroids and plasma exchange. This resulted in slow recovery over the next four weeks. Control echocardiography showed complete normalisation of cardiac function without signs of pulmonary hypertension. Two months after admission she was discharged from the hospital in good condition.
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ranking = 1
keywords = embolism
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2/52. Ulcerated plaque in the ulnar artery causing distal seeding of microemboli: a possible early stage of ulnar artery thrombosis.

    angiography of a patient presenting with ischemia of the ring and small fingers, but with patent arterial inflow through the ulnar artery on Allen's test, demonstrated stenosis of the ulnar artery just distal to Guyon's canal. Surgical exploration revealed a discrete ulcerated plaque with an overlying loosely adherent thrombus. An ulcerated plaque in one of the main inflow arteries to the hand may produce ischemia of the fingers by seeding microemboli distally into the digital arteries. This pathophysiologic mechanism may represent a very early phase of incipient or impending thrombosis of the ulnar artery and may eventually progress to complete thrombosis. Early recognition and treatment by resection of the involved arterial segment and microsurgical reconstruction may prevent the evolution to complete ulnar artery thrombosis.
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ranking = 25.39044468574
keywords = thrombosis
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3/52. Giant unruptured aneurysm of the thoracic aorta--a case report.

    An asymptomatic 88-year-old woman underwent a screening medical examination. The chest x-ray film showed a large mediastinal mass with calcification. Both chest computed tomography and nuclear magnetic resonance imaging revealed an unruptured aortic aneurysm, predominantly affecting the ascending aorta and the proximal part of the aortic arch. Its maximum diameter was 10.5 cm. An ascending aortic aneurysm more than 10 cm in diameter is very rare. She died of acute pulmonary embolism unrelated to the aneurysm, and autopsy indicated that the etiology of the aneurysm was atherosclerotic degeneration. Retrospectively, the natural progression of the aneurysm was able to be followed on a series of chest x-ray films obtained over 18 years.
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ranking = 1
keywords = embolism
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4/52. Extensive radiculopathy: a manifestation of intracranial hypertension.

    We report two patients with severe radiculopathy due to elevated intracranial pressure (ICP) resulting from idiopathic intracranial hypertension (IHH) in one, and cerebral venous sinus thrombosis (CVT) in the other. Our aim is to document this unique association, which escaped diagnosis in both patients.
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ranking = 3.6272063836772
keywords = thrombosis
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5/52. Aortic thrombosis after lumbar spine surgery.

    A case of distal aortic occlusion after circumferential lumbar spine surgery is presented. The paradoxical clinical findings are discussed.
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ranking = 14.508825534709
keywords = thrombosis
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6/52. Pulmonary hypertension.

    A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1) pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia; (2) pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary arterial hypertension; and (5) pulmonary hypertension due to disorders directly affecting the pulmonary vasculature. Pulmonary arterial hypertension includes individuals with primary pulmonary hypertension, congenital heart disease, connective tissue disease, and liver disease. These heterogeneous diseases have similar characteristic pathological changes, including in situ thrombosis, smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization is essential to confirm diagnosis, determine prognosis, and assign therapy. A minority of patients have a favorable response to an acute vasodilator trial and long-term benefit with calcium channel blocker therapy. Continuous intravenous epoprostenol improves symptoms and survival in patients with advanced primary pulmonary hypertension and has potential benefit in other forms of pulmonary arterial hypertension. lung transplantation remains an important option for individuals in whom maximal medical therapy fails. The recent discovery of the gene for familial primary pulmonary hypertension and the increase in new drugs undergoing clinical trials are encouraging developments.
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ranking = 3.6272063836772
keywords = thrombosis
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7/52. Progression of primary APS (Hughes syndrome) into serological SLE: case report.

    An Arab woman presented with a history of multiple foetal losses and spontaneous venous thromboembolism, which recurred on several occasions. The presence of antiphospholipid antibodies in the absence of other clinical and serological features of systemic lupus erythematosus (SLE), including negative antinuclear antibodies (ANA), confirmed the diagnosis of primary antiphospholipid syndrome (PAPS). More than 15 years after the beginning of clinical events and 10 years after diagnosis, she progressed into the immunological domain of SLE without concurrent clinical features. The patient exhibited weakly positive ANA of a speckled pattern, strongly positive anti (ds) dna antibodies and false positive VDRL. lymphopenia has not been observed at any stage of the follow-up. Although the evolution of PAPS into SLE has been infrequently reported, this seems to be another case suggesting that PAPS in some patients may be an early manifestation of lupus.
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ranking = 1
keywords = embolism
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8/52. Potential therapeutic effect of simvastatin on progressive renal failure and nephrotic-range proteinuria caused by renal cholesterol embolism.

    We report our experience with a 62-year-old Japanese man with cholesterol crystal embolism after angiographic procedures performed because of intermittent claudication. In addition to progressive renal failure and nephrotic-range proteinuria, cutaneous ischemia, consisting of livedo reticularis in the lower limbs and digital necrosis at the tip of the right toe, and fundoscopic findings showing several white spots in the branches of retinal artery were also observed. Progressive renal failure and nephrotic-range proteinuria were halted just after treatment with simvastatin. Thus, simvastatin can exert a beneficial therapeutic effect on renal cholesterol embolism.
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ranking = 6
keywords = embolism
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9/52. Evolution of incidentally-discovered fusiform aneurysms of the vertebrobasilar arterial system: neuroimaging features suggesting progressive aneurysm growth.

    This study investigated the natural history and biological behavior of incidental fusiform aneurysms in four patients with incidental fusiform aneurysms of the vertebrobasilar arterial system who had been followed up for more than 3 years (mean 3.5 years). Two lesions remained the same size, and two lesions gradually grew. angiography showed the non-growing fusiform aneurysms as a circumferentially or unilaterally fusiform dilatation of a short segment of the vertebral artery with smooth walls and a steep slope of the dilatation, and the growing fusiform aneurysms as unilaterally fusiform involving a long segment of the vertebral artery or basilar artery with irregular walls and a gentle slope of dilatation. Magnetic resonance (MR) imaging demonstrated the non-growing fusiform aneurysms as a signal-void area, and the growing fusiform aneurysms as high and intermediate signals in addition to the normal flow void. The heterogeneous MR intensities probably correspond to turbulent flow, laminar flow, thrombosis, or intramural hematoma. Differentiation of growing and non-growing fusiform aneurysms is very difficult at the initial diagnosis. However, enlargement of the fusiform aneurysms is consistent with hemorrhage into the aneurysmal wall, which is confirmed by MR imaging. Fusiform aneurysms with the characteristics of the growing aneurysms cannot be overlooked because of the potential to develop into giant fusiform aneurysms which are very difficult to manage therapeutically.
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ranking = 3.6272063836772
keywords = thrombosis
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10/52. calciphylaxis in two patients with end-stage renal disease.

    Fatal calciphylaxis (CPX) occurred in two 71-year-old females both requiring haemodialysis for end-stage renal disease. Case 2 also had an associated follicular lymphoplasmocytoid lymphoma. Although laboratory tests disclosed normal coagulation parameters, this woman had a striking cutaneous histological picture of vessel thrombosis and finally died of disseminated intravascular coagulation. CPX is a rare but potentially life-threatening complication of renal failure. The clinical picture is primarily characterized by livedoid purpura with subsequent cutaneous ischaemia and painful ulcerations. Cutaneous ischaemic phenomena are sustained by a progressive process of vascular calcification and thrombosis involving small to medium size arteries of the dermis and subcutis. Although not yet clearly explained, the pathogenetic role of a predisposing hypercoagulability state is currently the most frequently considered hypothesis.
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ranking = 7.2544127673544
keywords = thrombosis
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