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1/8. Solitary renal myofibromatosis: an unusual cause of infantile hypertension.

    INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4 protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren
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2/8. Percutaneous transluminal coronary angioplasty for renovascular hypertension in a child: a case report.

    Since its introduction, percutaneous transluminal coronary angioplasty has become an alternative therapeutic modality to surgical and medical treatment for renovascular hypertension. We report the case of a nine-year-old boy who had hypertension caused by renal arterial stenosis. The patient's high blood pressure was 164/100 mmHg, which was discovered incidentally during a physical check-up. A selective renal angiography showed a severe short-segment stenosis with post-stenotic dilatation of the left renal artery. A 4-mm balloon catheter was advanced through the stenotic area and was inflated five times to dilate the stenosis. After the procedure, the selective renal angiography showed a significant increase in the diameter of the left renal artery. blood pressure decreased to normal immediately after the procedure. During the one-year follow-up period, the patient remained normotensive without the use of antihypertensive drugs.
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3/8. aortic coarctation suspected by Doppler echocardiography of renal arteries in hypertensive patients referred to a hospital outpatient hypertension clinic.

    Coarctation of the aorta is the fourth most frequent form of congenital cardiovascular disease, which is diagnosed by the presence of higher blood pressures in the arms than in the legs. In this report we describe 3 cases of aortic coarctation, in which the correct diagnosis was suspected only months or years after the detection of hypertension, when a renal ultrasound examination was requested, despite the fact that the hallmarks of the disease were present at the physical examination in all patients. A marked reduction in renal flow velocities was suggestive of proximal aortic stenosis in all 3 cases. We conclude that the diagnosis of aortic coarctation, an uncommon but not so rare form of secondary hypertension, by renal ultrasonography rather than by a complete physical examination, reflects a commitment failure of physicians in everyday management of hypertension.
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4/8. renal artery aneurysm secondary to fibromuscular dysplasia in a young patient.

    An 8-year-old male was found on routine physical examination to have a blood pressure of 220/110. Renal angiography demonstrated bilateral renal artery stenosis and an aneurysm of the distal left renal artery with branch involvement. At operation, the left renal artery stenosis and aneurysm was repaired by ex vivo arterial reconstruction and autotransplantation of the kidney. Pathologic evaluation of the resected aneurysm confirmed the diagnosis of fibromuscular dysplasia. fibromuscular dysplasia is the most common cause of renal artery stenosis in children over 1 year of age and can in rare cases be associated with the development of renal artery aneurysms. In complex cases of renal artery stenosis with involvement of renal artery branches, ex vivo repair and orthotopic autotransplantation is an excellent approach for surgical management.
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5/8. renal artery dissection after angiographic evaluation of lower extremities.

    We describe a case of renovascular hypertension that was caused by renal artery dissection after an angiographic evaluation of the lower extremities. Retention of contrast medium in the affected kidney even 1 day after the procedure caused us to suspect renal artery dissection. magnetic resonance angiography revealed irregular streaks in the lumen of the affected artery, a sign of dissection. Treatment by a transluminal angioplasty with stent insertion improved renal function and reduced systemic blood pressure with normalization of plasma renin activity. In patients whose systemic blood pressure increases suddenly after any conceivable physical traction on the abdominal aorta, evaluation of the renal arteries should be considered. Appropriate endovascular treatment can preserve renal function and reduce systemic blood pressure.
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6/8. Primary intimal fibroplasia of the renal artery.

    Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.
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7/8. renal artery stenosis in children.

    Because high blood pressure in children is rare and most of these patients are asymptomatic, many are overlooked until they present with a hypertensive crisis or irreparable damage. Most children with renal artery stenosis are asymptomatic and the hypertension is detected only by blood pressure recording during physical examination. Angiography is the most helpful diagnostic study. It is generally agreed that renal artery lesions in children should be considered for surgical correction. An illustrated case is described.
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8/8. Abdominal symptoms as presentation of hypertensive crisis.

    OBJECTIVE--To describe a series of patients in hypertensive crisis who presented primarily with abdominal symptoms. DESIGN--Patient reports. SETTING--Referral center after initial presentation in the general community. PARTICIPANTS--Three children, aged 10 months to 4 years, in hypertensive crisis who presented with abdominal symptoms that promptly resolved and did not recur with blood pressure control. CONCLUSIONS--blood pressure should be measured in all children undergoing physical examination; particular attention should be paid to the blood pressure of patients with unexplained abdominal symptoms before extensive diagnostic testing is pursued.
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