Vol 19: Renal artery stenosis due to neurofibromatosis type 1: case report and literature review.Reportar como inadecuado



 Vol 19: Renal artery stenosis due to neurofibromatosis type 1: case report and literature review.


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This article is from European Journal of Medical Research, volume 19.AbstractBackground: Neurofibromatosis type 1 NF1 is a relatively common autosomal dominant disorder. The most common vascular abnormality in patients with NF1 is bilateral or unilateral renal artery stenosis. Case report: A 16-year-old boy presented with a headache of 4-year duration and was found to be moderately hypertensive. On physical examination, axillary freckling and multiple café-au-lait spots were revealed over the trunk, while numerous small nodules were palpable on the limbs. Biopsy of subcutaneous nodule showed neurofibroma. Lisch nodules were identified on slit-lamp examination and grade I hypertensive retinopathy was present on fundoscopy. Clinical laboratory investigations revealed that renal and liver function tests, blood cells count, urinalysis, serum electrolytes, serum levels of renin and aldosterone, and 24-hour urine levels of catecholamines were all within normal ranges. Abdominal ultrasound and CT were normal. Both kidneys were of normal size. CT angiography showed right renal artery stenosis 90% at the ostium. The final diagnosis of NF1 with right renal artery stenosis and secondary hypertension was then made. The patient was treated with Procardin 30 mg-d and improved with a significant decline in blood pressure. The main outcomes were to control blood pressure without necessarily proceeding with PTRA. We also present a review of the literature. Conclusions: NF1 may present with hypertension due to renal artery stenosis in children. All young patients



Autor: Duan, Lian; Feng, Kai; Tong, Anli; Liang, Zhiyong

Fuente: https://archive.org/



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