Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022116 (ischemia)
91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fibromuscular dysplasia of renal arteries was the cause of hypertension in four consecutive children with renal artery stenosis. Two were asymptomatic, the third had had hypertension for seven years but had not been treated, and the fourth, a 9-month-old infant, presented with cardiac failure. Heart enlargement and left ventricular hypertrophy were present in all. Rapid sequence urograms demonstrated a smaller kidney and delayed appearance and disappearance of the contrast medium on the affected side in all. Angiograms showed left RAS in all. Peripheral plasma renin activity was elevated in only three of the four patients. Antihypertensive and diuretic drugs were not very effective therapeutically. Ischemia of the ipsilateral kidney probably prevented normal growth and led to shrinkage of the kidney in one patient. Following nephrectomy the BP has remained normal without any therapy for 24 to 64 months. With normalization of BP, accelerated growth ensued, the cardiomegaly regressed and the hypertensive retinopathy resolved. These patients demonstrate that: (1) FMD is an important cause of RAS. (2) the well-known radiologic feature of FMD, the beaded appearance, is usually not seen in children. (3) control of BP leads to normalization of linear growth, usually impaired in severe hypertension, and (4) target organ complications such as cardiomegaly, LVH, and hypertensive retinopathy are reversible in one to 10 months.
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PMID:Fibromuscular dysplasia of renal arteries: an important cause of renovascular hypertension in children. 15 54

A 74-year-old woman was admitted to the hospital with sudden onset of a blue, painful right index finger, and the condition progressed to digital ulceration. The ulcer gradually healed over a period of 2 months. Peripheral pulses, Doppler-derived brachial and radial systolic pressures, and results of an echocardiogram were normal. Digital pulse volume recordings showed obstructive waveforms in all digits of the right hand. Arteriography showed a normal arch and normal innominate, subclavian, and axillary arteries. The midbrachial artery was markedly abnormal and had alternating areas of stenosis and aneurysm formation. Multiple occlusions involved the palmar arch and proper digital arteries. The abnormal brachial artery segment was excised and replaced with an autogenous reversed saphenous-vein conduit. Histologic examination confirmed the lesion to be medial fibromuscular dysplasia. Fibromuscular dysplasia that involves upper extremity arteries is extremely uncommon and rarely presents with digital artery embolization. This case emphasizes the importance of exclusion of proximal reconstructible arterial occlusive disease by means of complete arteriographic examination of patients who were admitted with unilateral finger or hand ischemia.
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PMID:Digital artery embolization as a result of fibromuscular dysplasia of the brachial artery. 186 35

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily the renal and internal carotid arteries and less often the vertebral, iliac, subclavian, and visceral arteries. Although its pathogenesis is not completely understood, humoral, mechanical, and genetic factors as well as mural ischemia may play a role. The natural history is relatively benign, with progression occurring in only a minority of the patients. Typical clinical manifestations are renovascular hypertension, stroke, subarachnoid hemorrhage, abdominal angina, or claudication of the legs or arms. In patients with symptoms, percutaneous transluminal angioplasty has emerged as the treatment of choice in most involved vascular beds.
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PMID:Arterial fibromuscular dysplasia. 330 88

Two typical cases of fibromuscular dysplasia of the cervicocephalic arteries in two women of 61 and 48 years of age are reported. The angiograms revealed bilateral affectation of the internal carotid artery and of the right vertebral artery in one case, and of both vertebral and renal arteries in the other. The patients presented neurological symptoms corresponding to ischemia of the vertebro-basilar territory. The first case was treated with anti-platelet aggregates with positive results. An extensive review of 70 similar published cases is presented. Several characteristics are studied such as: age, sex, localization, symptoms, clinical course and treatment. The quantitative evaluation of these factors agrees with those of other reviews carried out by some other authors. Fibromuscular dysplasia is an arteriopathy of unknown etiology which has a predominant incidence among middle age females (83 percent approximately). The disease usually involves the renal arteries and the cervical segment (adjacent to C1-C2 interspace) of the carotid arteries. There was an association with single or multiple intracranial aneurisms in 22.86 percent of the cases. Vertebral arteries were affected in 28.57 percent of the cases, although vertebral angiograms were not performed in 35.7 percent of them. Transient episodes of cerebral ischemia is the most frequent clinical manifestation (42.85 percent of the cases.).
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PMID:[Fibromuscular dysplasia of the cervicocephalic arteries. Report of two cases and review of the literature (author's transl)]. 736 81

Fibromuscular dysplasia is an uncommon angiopathy that is principally observed in the renal and carotid arteries. Digital ischemia resulting from fibromuscular dysplasia of the forearm arteries is a rare occurrence. This article describes a case of distal radial and ulnar artery fibromuscular dysplasia presenting as paresthesia, claudication, and finger ulceration. Angiography was diagnostic in visualizing the characteristic "string of beads" appearance. In addition to the typical histological findings, we also observed a previously undescribed pathological finding. Surgical management involved resection of the diseased segment and primary anastomosis.
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PMID:Fibromuscular dysplasia of distal radial and ulnar arteries: uncommon cause of digital ischemia. 788 60

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that usually involves medium- and small-sized arteries. It is most commonly observed in the renal, carotid, and intracerebral arteries, although it has been reported in other arterial beds. The most common form is characterized by medial fibrosis, with or without smooth muscle cell hyperplasia, which can result in luminal narrowing and turbulent flow. There is often a secondary aneurysmal degeneration of the artery, which may or may not be associated with thrombosis or obstruction of flow. This accounts for the typical "string-of-beads" appearance seen on arteriography. We describe a patient who presented with ischemia of the right hand secondary to fibromuscular dysplasia of the brachial artery. Subsequent studies also demonstrated fibromuscular dysplasia in the other brachial artery as well as mild involvement of the right renal artery. The patient was treated on the symptomatic side with dilatation of proximal lesions, resection of the thrombosed segment, and reconstruction with a reversed saphenous vein graft. Distal pulses were fully restored postoperatively. Pathologic examination confirmed the arteriographic and clinical diagnosis of fibromuscular dysplasia. The salient features of this case are reviewed in addition to the other cases reported in the literature.
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PMID:Bilateral brachial artery fibromuscular dysplasia. 826 96

Fibromuscular dysplasia (FMD) commonly involves the renal and carotid arteries. However, visceral arterial involvement is rare. We report a 39-yr-old male who developed ischemic bowel disease twice (once in the jejunal artery and once in the sigmoid artery) as a result of FMD. First ischemic change occurred in the sigmoid colon with manifestation of melena and abdominal pain, and the second occurred in the jejunum, with severe abdominal pain and intestinal stenosis. Angiography revealed the "string of beads" appearance, characteristic of FMD, in those two arteries responsible for the ischemic lesions. Fasting therapy with intravenous hyperalimentation improved both lesions, but secondary stenosis caused by ischemia occurred in the jejunum. Surgical resection of the stenotic jejunum was required. Although the abnormal arteries that had been imaged as a "string of beads" on angiography were not resected, the postoperative course has been uneventful and satisfactory.
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PMID:Fibromuscular dysplasia of the visceral arteries. 875 76

An 8-year-old girl presented with fibromuscular dysplasia of the intracranial vessels manifesting as ischemic stroke. Neuroimaging showed infarction of the right putamen and ipsilateral frontal white matter. Angiography revealed "string of beads" sign involving the terminal portion of the right internal carotid artery and the horizontal segment of the ipsilateral middle cerebral artery. She was treated conservatively. Magnetic resonance angiography at 2 months post ictus showed similar findings in the middle cerebral artery but improvement of the stenosis of the internal carotid artery. Her neurological deficits had almost resolved. Fibromuscular dysplasia should be part of the differential diagnosis of ischemia in children.
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PMID:Childhood ischemic stroke caused by fibromuscular dysplasia of the intracranial artery--case report. 925 54

Fibromuscular dysplasia is a rare nonatherosclerotic, noninflammatory angiopathy of uncertain etiology and high morbidity. Because of its propensity to affect medium-sized vessels in a variety of locations, presenting symptoms may vary substantially, resulting in a delayed or missed diagnosis. We describe a 57-year-old woman who, on multiple occasions, presented with progressive gastrointestinal symptoms and eventually underwent surgical revascularization for celiac and superior mesenteric artery stenosis of uncertain etiology. Her postoperative course was complicated by bowel ischemia, multiple organ failure, and death. Autopsy findings proved useful in determining the underlying disease process and cause of death. This case report and a review of the literature illustrate the high morbidity and mortality that are caused by mesenteric fibromuscular dysplasia, the challenge in establishing a correct diagnosis, and the importance of early detection and treatment.
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PMID:Fatal mesenteric fibromuscular dysplasia: a case report and review of the literature. 1515 74

Fibromuscular dysplasia is a multifactorial arteriopathy most commonly affecting the renal and carotid arteries. In this report we present a case of visceral artery involvement, causing occlusion of the superior mesenteric artery and celiac trunk and resulting in visceral ischemia. Treatment consisted of superior mesenteric artery reimplantation. Visceral artery FMD can present as occlusive or aneurysmal disease and treatment depends on patient characteristics and symptoms.
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PMID:Fibromuscular dysplasia of the superior mesenteric artery--case report and review of the literature. 1631 39


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