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Middle aortic syndrome (MAS) is a clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. MAS may be acquired, caused by Takayasu's or temporal arteritis (giant cell arteritides), neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or congenital, ascribed to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas. Segmental aortic stenosis may be located at the suprarenal, inter-renal or infrarenal aorta, with a high propensity for concomitant stenoses in both the renal (63%) and visceral (33%) arteries. Hypertension proximal to the aortic stenosis, and relative hypotension distal to it, are characteristic findings in MAS. Typical manifestations include headache, early fatigue on exertion, and bilateral lower-limb claudication. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. As a great proportion of patients with MAS are children or teenagers, the clinical benefits of early surgical intervention to reverse refractory hypertension have to be weighed against the repercussions pertaining to the insult of surgery on the developing aorta. Open surgery is the primary treatment of tubular aortic narrowing (MAS) associated with renovascular hypertension and visceral artery stenosis. This entails aortoaortic bypass of the diseased segment or, less often, patch aortoplasty and usually bypass grafting of the stenosed renal and visceral arteries performed with autologous conduits, particularly in the youngest of patients. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenoses that do not encompass the mesenteric and renal arteries. Hypertension is thus improved or cured in more than 70% of patients. Prognosis after uncompromised surgical reconstruction is rewarding in the mid and long term in patients with congenital aortic coarctation but deteriorates in patients with aortoarteritis and recurrent inflammatory activity.
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PMID:Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. 1627 55

Intermittent claudication (IC) is defined by leg muscle pain, cramping and fatigue brought on by ambulation/exercise; relieved on rest; and caused by inadequate blood supply and is the primary symptom of peripheral arterial disease (PAD). PAD has a detrimental effect on the quality of life. PAD is a debilitating atherosclerotic disease of the lower limbs and is associated with an increased risk of cardiovascular morbidity and mortality. IC is an extremely important marker of atheroma. Up to 60% patients with IC have significant underlying coronary and/or carotid disease and 40% of all patients suffering from IC die or suffer a stroke within 5 years of presentation. The therapeutic intervention of IC essentially aims at providing symptomatic relief and reducing the systemic cardiovascular complications. Although exercise therapy is one of the most efficacious conservative treatments for claudication, the pharmacotherapeutic goals can be best achieved through an increase in the walking capacity to improve quality of life and a decrease in rates of amputation. In the development of treatment for IC, an aggressive non-pharmacological intervention and pharmacological treatment of the risk factors associated with IC are considered. In the next 2 years, the results of major trials of drugs that stabilize and regress atherosclerosis such as statins and angiotensin converting enzyme inhibitors, and anti-platelet agents, recombinant growth factors and immune modulators will be available for IC. Levocarnitine (l-carnitine) and a derivative, propionyl levocarnitine, are emerging agents that increase the pain-free walking and improve the quality of life in IC patients by working at the metabolism and exercise performance of ischemic muscles. This article provides a comprehensive review of the pathophysiology involved, diagnosis of IC and existing and emerging pharmacotherapies with rationale for their use in its treatment.
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PMID:Intermittent claudication: an overview. 1638 60

Takayasu's arteritis is a systemic disease, presenting as chronic inflammation of the main arteries. It usually affects the aorta and its large branches. General symptoms often include fatigue, subfebrile temperatures and weight loss. Complaints due to perfusion disorders are, for example, muscle pain, dizziness or claudication. Takayasu's arteritis is a rare disease which generally occurs in female patients under 40 years of age. We report on a patient with primary involvement of the mesenteric arteries. The disease process was fulminant and refractory to all therapeutic strategies. According to our knowledge this is the first case report from Germany of Takayasu's arteritis with mesenteric infarction.
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PMID:[Fulminant course of a Takayasu's arteritis and rare mesenteric arterial maninfestion]. 1655 Mar 22

Giant cell arteritis is a systemic disease of unknown origin. Vasculitis involves large and medium-sized vessels. Frequent clinical manifestations include characteristic headache in the temporal area, jaw or tongue claudication, apathy, fatigue, weight loss. The incidence of ocular involvement is reported in up to 70% patients. The most common and serious ophthalmic presentation is arteritic anterior ischemic optic neuropathy, which can lead to irreversible visual loss. Only early and aggressive steroid therapy may prevent this dangerous complication. The authors presented a case of a 68-years-old woman with giant cell arteritis. The main visual manifestation of this disease was anterior ischemic optic neuropathy.
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PMID:[Giant cell arteritis--case report]. 1866 91

Takayasu's arteritis (TA) is characterized by a chronic inflammatory arteriopathy with unknown etiology affecting large vessels. As TA occurs mostly in young females, elderly males with this disease may be under-diagnosed. A 65-year-old Chinese male with a 6-week history of fever, fatigue, low appetite, night sweats, and a 5-kg weight loss did not experience symptoms of jaw claudication, localized headache, sudden visual change, upper or lower extremity claudication, arthralgia, and rashes. On physical examination and laboratory findings, there was no evidence of infectious disease or malignancy. (18)F-Fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT) was performed which suggested TA. The patient was treated with methylprednisolone and clopidogrel. His symptoms subsided gradually and hematological examination showed decreases of erythrocyte sedimentation rate and high-sensitivity C-reactive protein. We reported here a patient who presented with fever of unknown origin and was diagnosed with TA using PET/CT. Although elderly males are beyond the predominant population of TA, TA should be considered when there is no evidence of infectious disease and malignancy.
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PMID:Diagnosis of a 65-year-old male patient with Takayasu's arteritis by 18F-FDG PET/CT. 1982 33

Fever of unknown origin (FUO) refers to prolonged fevers of > or = 101 degrees F and that persists for > 3 weeks that remain undiagnosed after an intensive in-hospital/outpatient workup. The most common FUO categories of are infectious, neoplastic, rheumatic/inflammatory, and miscellaneous causes. Malignancies have supplanted infectious diseases as the most common cause of FUOs in the adult population. Rheumatic/inflammatory causes of FUO are relatively less common than previously because of the introduction over the years of sophisticated diagnostic tests for most rheumatic diseases. The rheumatic/inflammatory disorders that remain important causes of FUO today are those that cannot be readily diagnosed by readily available/noninvasive tests, for example, adult Still's disease and temporal arteritis (TA). In older patients with FUO, TA can be a difficult diagnosis when the characteristic findings (ie, scalp tenderness, jaw claudication) are not present. Patients with TA presenting as FUO often have only headaches that may be accompanied by bilateral jaw discomfort. Endocrine causes of FUOs are rare. The most common endocrine disorder rarely presenting as an FUO is de Quervain's subacute thyroiditis. As in TA, subacute thyroiditis may present with headache and pain at the angle of the jaw. Both TA and subacute thyroiditis may be accompanied by fatigue, weight loss, and night sweats. We present a case of 55-year-old woman who presented with an FUO with clinical and laboratory findings suggesting TA. However, the absence of thrombocytosis and a normal alkaline phosphatase argued against the diagnosis of TA. Also against the diagnosis of TA was weight loss without loss of appetite and a slightly increased pulse. After nonspecific laboratory test results suggested that TA was not the cause of her FUO, additional tests were ordered. Thyroid function test results suggested the possibility of de Quervain's subacute thyroiditis as the cause of her FUO. To the best of our knowledge, this is the first case of de Quervain's subacute thyroiditis presenting as an FUO with elevated ferritin levels.
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PMID:Fever of unknown origin (FUO): de Quervain's subacute thyroiditis with highly elevated ferritin levels mimicking temporal arteritis (TA). 2010 88

The aim of this study was to determine oxidative stress status as well as blood lead (Pb) and zinc (Zn) levels and clinical markers in workers of a Zn-Pb mine. A comparative cross-sectional analysis was performed in 67 mine workers who have been in contact with Zn and Pb in comparison to a control group containing 67 healthy subjects with the same age and sex. Lipid peroxidation, superoxide dismutase, catalase, glutathione reductase, myleoperoxidase, DNA damage, total antioxidant capacity, Zn, and Pb levels were measured in blood of workers and controls. Clinical examination was accomplished to record any abnormal sign or symptoms. Comparing with controls, the workers showed higher blood levels of superoxide dismutase, myleoperoxidase, glutathione reductase, lipid peroxidation, Pb, and Zn. Workers showed lower DNA-damage as compared with controls. Workers showed clinical symptoms such as memory impairment, less of concentration, insomnia, headache, claudication, epigasteric, inappetence, agitation, tremor, decreasing of reflection of deep tendon, conduction deafness of ear, and fatigue. The workers had extra normal levels of Pb (0.9-3 microg/dL) and showed oxidative stress. Taken together, the results indicate that exposure to combination of Pb and Zn in mine elevates total antioxidant capacity of body in a reflex to overcome to oxidative stress. Especially, in the present case, it seems that toxic effect of Pb has been greater than positive effects of Zn, but the combination exposure has resulted in not such a critical toxicity situation.
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PMID:Study on clinical and biochemical toxicity biomarkers in a zinc-lead mine workers. 2037 35

Giant cell arteritis is a systemic, inflammatory, and vascular syndrome that requires early diagnosis and immediate management because of the risk of loss of vision. Local symptoms include headache, scalp tenderness, jaw claudication, visual disturbances, and scalp necrosis. Systemic symptoms include weight loss, fever, malaise, fatigue, and polymyalgia rheumatica. We describe a case that was identified histologically as an incidental finding after excision of a basal cell carcinoma from the parietal area of the scalp. A search of PubMed and Medline using the keywords "giant cell arteritis", and "incidental histopathological diagnosis" returned no similar previously published cases in the head and neck. We present this as an unusual and interesting case.
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PMID:Giant cell arteritis--an incidental finding. 2178 97

Up to 60 percent of adults report that they have had nocturnal leg cramps. The recurrent, painful tightening usually occurs in the calf muscles and can cause severe insomnia. The exact mechanism is unknown, but the cramps are probably caused by muscle fatigue and nerve dysfunction rather than electrolyte or other abnormalities. Nocturnal leg cramps are associated with vascular disease, lumbar canal stenosis, cirrhosis, hemodialysis, pregnancy, and other medical conditions. Medications that are strongly associated with leg cramps include intravenous iron sucrose, conjugated estrogens, raloxifene, naproxen, and teriparatide. A history and physical examination are usually sufficient to differentiate nocturnal leg cramps from other conditions, such as restless legs syndrome, claudication, myositis, and peripheral neuropathy. Laboratory evaluation and specialized testing usually are unnecessary to confirm the diagnosis. Limited evidence supports treating nocturnal leg cramps with exercise and stretching, or with medications such as magnesium, calcium channel blockers, carisoprodol, or vitamin B(12). Quinine is no longer recommended to treat leg cramps.
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PMID:Nocturnal leg cramps. 2296 30

Polymyalgia rheumatica affects proximal muscles and joints, causing disability in older adults. Giant cell arteritis affects medium and large arteries and can result in blindness. These conditions overlap significantly, often occurring together. Despite the similarities, each has distinct symptoms, corticosteroid dosing requirements, and prognosis. The hallmark of both conditions is inflammation. Polymyalgia rheumatica primarily affects the shoulders, neck, and hips with prominent bilateral pain. Systemic findings such as fatigue and weight loss are common, and there is no definitive diagnostic test. Moderate-dose corticosteroid therapy with a slow taper rapidly resolves symptoms. Management of patients responding to treatment can occur in the primary care setting, if there is no concomitant giant cell arteritis. The clinical presentation of giant cell arteritis varies widely, from new-onset headache and constitutional symptoms, to jaw claudication, to less common isolated visual changes and upper extremity claudication. Treatment requires higher dosages of corticosteroids and urgent referral to a rheumatologist. Relapse is common in both diseases. Surveillance is important, as is monitoring for long-term complications of corticosteroid use. Osteoporosis management and gastrointestinal ulcer prophylaxis should be initiated. The primary care physician's coordination of care with a rheumatologist and with other subspecialists, if needed, is essential in the management of giant cell arteritis.
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PMID:Recognition and management of polymyalgia rheumatica and giant cell arteritis. 2436 92


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