Gene/Protein Disease Symptom Drug Enzyme Compound
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Authors describe a rare cause, diagnostic difficulties and successful therapy of renovascular hypertension in a 12 year-old girl caused by anular stenosis of the intrarenal arterial branch. Activation of the system renin-angiotensin (RAS) is found in all forms of renovascular hypertension at the beginning. Etiologically, stenosis in childhood is caused mostly by renal artery dysplasia, affecting mostly media, and fibromuscular dysplasia. Fibromuscular dysplasia affects middle and distal third of renal artery in 60%, more frequently on right, only in 10% of cases affects segmental branches; one quarter of patients are affected bilaterally. This disease is found predominantly in young women. During clinical course, typical signs include sudden onset of severe and poorly controlled hypertension, renal insufficiency, proteinuria and hypertensive retinopathy. From non-invasive diagnostic approaches, color duplex ultrasound, NMR and CT angiography are important, from invasive ones, digital subtractive angiography and the measurement of plasma rennin activity in renal veins. In therapy, it is percutaneous transluminal renal angioplasty, associated with low mortality and morbidity. The net result of angioplasty is dilation of stenosis, complete restoration of artery lumen and flow and decrease of blood pressure. The best results can be achieved in young patients with fibromuscular dysplasia, more then half can recover completely. Using this method, also our patient has recovered (Tab. 2, Fig. 2, Ref. 7).
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PMID:A successful therapy of renovascular hypertension. 1705 4

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease, commonly involving the renal arteries. Here we report a case of a 16-year-old Chinese male who was found to have severe hypertension with proteinuria for 2 years. Computed tomography showed absence of the left kidney and enlargement of the right kidney. Subsequent angiography confirmed the above findings and revealed narrowing of both the upper and lower branches of the right renal artery caused by FMD. These combined lesions are very rare, and individuals affected are at increased risk of renal dysfunction if left untreated. Treatment with percutaneous balloon angioplasty is the first choice in such a patient and usually results in optimal outcomes.
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PMID:Fibromuscular dysplasia affecting a two-branched renal artery in a patient with a solitary kidney: case presentation. 2413 76

Background: Fibromuscular dysplasia (FMD) is one of the important etiologies of renovascular hypertension in children. It is usually resistant to multiple antihypertensive agents and can cause extreme elevation in blood pressures, which can lead to end organ damage if not promptly diagnosed and treated. Treatment options include medical management with antihypertensive agents, balloon or stent angioplasties, surgical revascularization, and nephrectomy. The aim of the study was to review the efficacy of antihypertensive therapy only in the management of FMD in a very young child. Methods: This is a retrospective chart study with review of literature. Results: Here, we report a 22-month-old toddler who presented with severe resistant hypertension and cardiomyopathy who was found to have focal FMD of the right renal artery. She also presented with proteinuria, hyponatremia that was probably secondary to pressure natriuresis, hypokalemia, hyperaldosteronism, and elevated plasma renin activity. The stabilization of blood pressures was done medically with the usage of antihypertensive medications only, without the need for angioplasty or surgical revascularization. Conclusions: We demonstrate that surgical intervention may not always be necessary in the treatment of all cases of FMD, especially in a small child where such intervention may be technically challenging and lead to potential complications. Hence, medical management alone may be sufficient, at least for the short-term, in small children with controlled hypertension and normal renal function, with surgical intervention reserved for FMD with medication-refractory hypertension and/or compromised renal function.
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PMID:Efficacy of Antihypertensive Therapy in a Child with Unilateral Focal Fibromuscular Dysplasia of the Renal Artery: A Case Study and Review of Literature. 3209 71