1/37. 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- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/37. hypertension due to renal artery occlusion in a patient with antiphospholipid syndrome.We report an unusual case of renovascular hypertension in a 16-year-old boy with primary antiphospholipid syndrome (PAPS), admitted to our clinic for severe drug-resistant hypertension and hypokalemia. Hormonal investigation revealed secondary aldosteronism and positive captopril test for renovascular disease. aortography confirmed the occlusion of the left renal artery. After nephrectomy, normalization of blood pressure and secondary aldosteronism occurred. Presently the patient remains in good health, receiving warfarin anticoagulant therapy. PAPS is defined by the presence of antiphospholipid antibodies and recurrent thrombosis. Arterial thrombosis (29%) appears to be less prevalent than venous thrombosis. Thrombotic microangiopathy of the kidney is frequently observed but renal artery occlusion, as seen in our patient, is unusual.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/37. Repeated transient anuria following losartan administration in a patient with a solitary kidney.We report the case of a 70-year-old hypertensive man with a solitary kidney and chronic renal insufficiency who developed two episodes of transient anuria after losartan administration. He was hospitalized for a myocardial infarction with pulmonary edema, treated with high-dose diuretics. Due to severe systolic dysfunction losartan was prescribed. Surprisingly, the first dose of 50 mg of losartan resulted in a sudden anuria, which lasted eight hours despite high-dose furosemide and amine infusion. One week later, by mistake, losartan was prescribed again and after the second dose of 50 mg, the patient developed a second episode of transient anuria lasting 10 hours. During these two episodes, his blood pressure diminished but no severe hypotension was noted. Ultimately, an arteriography showed a 70-80% renal artery stenosis. In this patient, renal artery stenosis combined with heart failure and diuretic therapy certainly resulted in a strong activation of the renin-angiotensin system (RAS). Under such conditions, angiotensin ii receptor blockade by losartan probably induced a critical fall in glomerular filtration pressure. This case report highlights the fact that the angiotensin ii receptor antagonist losartan can cause serious unexpected complications in patients with renovascular disease and should be used with extreme caution in this setting.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/37. Severe hypertension in children with renovascular disease.Renovascular disease is an important cause of hypertension in children and is associated with considerable morbidity and mortality risks. Secondary hypertension is more common in children than in adults, with children accounting for 75% to 80% of cases. In 70% of secondary hypertension in children, the cause is fibromuscular hyperplasia. Other associated conditions are aorto-aortitis, the midaortic syndrome, and Williams-Bueren syndrome. Imaging techniques have an important role in the early discovery of renal artery stenosis. Although renal arteriography remains the definitive method, noninvasive and less invasive radiographic procedures such as ultrasonography with duplex Doppler scanning and radionuclide scintigraphy have been used as adjunct diagnostic tools in children. The authors describe three young children with renovascular hypertension in whom dynamic radionuclide scintigraphy with Tc-99m MAG3 played an essential role in the diagnosis of renovascular hypertension.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
5/37. moyamoya disease associated with renovascular disease in a young African-Brazilian patient.Moyamoya is a rare disease characterized by fibrous dysplasia of the internal carotid and proximal cerebral arteries, which has been described mainly in young Japanese. We present a case of moyamoya disease with renal artery involvement in a young male patient with an African origin. A 15-year-old boy was referred to our hospital due to uncontrolled blood pressure, headache, somnolence, cognitive deficit and multiple lacunar infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular anatomy at the level of the circle of willis. The intracranial vessels presented severe stenosis or were occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriography showed a tight stenosis of the ostium and proximal segment of right main artery, which was only partially relieved by balloon angioplasty. A saphenous bypass from aorta to the right renal artery re-established the renal blood flow. blood pressure dropped after surgery and was controlled with low doses of diuretic and beta-blocker. After arteriography he presented right hemiplegia, with partial recovering in the following months. In conclusion, we described the first case of moyamoya disease with concomitant renovascular disease in a young adult of African origin, an association that may be more frequent than usually suspected in clinical practice.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
6/37. renal artery stenosis and aneurysmatic dilatation of arteria carotis interna in tuberous sclerosis complex.hypertension in children with neurocutaneous disorders (phacomatoses) is a well-recognized complication of these diseases and the cause of hypertension is fairly specific within each group. In patients with neurofibromatosis, hypertension is mainly caused by renovascular disease, whereas in tuberous sclerosis (TSC) reasons for hypertension are renoparenchymal lesions, such as angiomyolipoma or cysts. We report on a girl with TSC and hypertension due to unilateral renal artery stenosis associated with aneurysmatic changes of internal carotid artery. This unusual combination of symptoms in our patient supports the importance of thorough and complete investigation of hypertension in children with phacomatoses.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/37. Unilateral stent implantation for renal function in bilateral atherosclerotic renovascular hypertension--a case report.renal artery stenting improves or preserves renal function in patients with bilateral renovascular disease and chronic renal insufficiency. An 80-year-old male was admitted to the hospital for elevated blood pressure accompanied by congestive heart failure. He had renal insufficiency and severe hypertension secondary to bilateral atherosclerotic renal artery stenosis. Unilateral renal artery stenting in the left kidney resulted in the recovery of renal function, whereas renal artery stenting in the right kidney was technically difficult due to a tortuous aorta. After the left unilateral stent implantation, the serum creatinine concentration decreased from 2.0 to 1.3 mg/dL, and control of his blood pressure required fewer antihypertensive drugs, namely a calcium antagonist, an angiotensin-converting enzyme inhibitor, and diuretics. Fifteen months after stenting, renal scintigraphy demonstrated improved function of the right kidney, despite severe renal artery stenosis, as well as improved function of the left kidney. Renal angioplasty or stenting should be attempted in bilateral atherosclerotic renovascular hypertension with renal insufficiency, even though it may only be successful unilaterally.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/37. Renovascular hypertension secondary to spontaneous renal artery dissection and treatment with stenting.Renovascular disease is the cause of less than 1% of patients with hypertension; within this group aneurysm and dissection in renal artery are even rarer causes. It may be not only iatrogenic or traumatic, but may also develop spontaneously from fibromuscular disease or atherosclerotic lesions. We present a 26-year-old male patient with recently developed and progressive hypertension in whom renal angiography showed aneurysmal dilatation and dissection of the right renal artery. With the implantation of two stents in the true lumen, normal renal blood flow and thrombosis of the aneurysmal sac was established. As a result, stent implantation to renal artery dissection is effective, reliable and easy and can be an alternative to surgical treatment.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/37. Percutaneous angioplasty for renovascular hypertension due to fibromuscular dysplasia.Because the cause of hypertension is reversible in only 5 percent of patients, extensive initial work-up should only be considered in selected cases. Secondary causes should be suspected in patients whose hypertension begins before age 30 or after age 50 and in patients whose hypertension suddenly worsens after a long period of good control, becomes severe or malignant, or remains refractory to maximal medical therapy. A sudden reduction in renal function in a hypertensive patient and the discovery of a unilateral small kidney may also raise suspicion of a secondary cause. Renovascular disease, one of the most common secondary causes of hypertension, is usually the result of atherosclerosis in older patients and the result of fibromuscular dysplasia in younger patients. Physical examination seldom contributes to the diagnosis. The classic upper abdominal or flank bruit occurs in only 30 to 50 percent of patients with renovascular disease, and is not uncommon in patients with essential hypertension. The gold standard for diagnosis of renovascular disease remains the arteriogram. Transluminal renal angioplasty may be performed during arteriography if a high-grade stenosis is identified. Other management options include medical therapy and surgical revascularization with grafts.- - - - - - - - - - ranking = 3keywords = vascular disease (Clic here for more details about this article) |
10/37. captopril-induced pancytopenia in a premature newborn.A preterm infant with renovascular hypertension who developed significant trilineage bone marrow suppression after receiving captopril is reported. captopril-associated pancytopenia is a very rare complication that was thought to be dose-related and usually caused by accumulation of the drug through defective renal tubular excretion. In our patient, it appears that the combination of renal artery stenosis and renal tubular dysfunction of prematurity might have led to pancytopenia. captopril should be used with caution especially in premature babies and newborns with underlying renal or renovascular disease even if they do not have overt renal dysfunction. Monitorization of these babies with creatinine clearance or free serum captopril levels may help to establish accumulation of the drug before causing pancytopenia.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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