Conkar, Secil Ege Üniverstesi Tıp Fakültesi - Çocuk Nefroloji BD, İzmir , Yılmaz, Ebru Ege Üniverstesi Tıp Fakültesi - Çocuk Nefroloji BD, İzmir , Özdemir, Kadriye Ege Üniverstesi Tıp Fakültesi - Çocuk Nefroloji BD, İzmir , Bulut, İpek Kaplan Ege Üniverstesi Tıp Fakültesi - Çocuk Nefroloji BD, İzmir , Bozkaya, Halil Ege Üniverstesi Tıp Fakültesi, Radyoloji BD, İzmir , Mir, Sevgi Ege Üniverstesi Tıp Fakültesi - Çocuk Nefroloji BD, İzmir

Neurofibromatosis type 1 (NF1): as a cause of hypertension

23557

Neurofibromatosis type 1 (NF1), an autosomal dominant disorder, is characterized by various clinical manifestations as a result of dysplasia of neuroectodermal and mesodermal tissues. Cafe-au-lait spots, axillary and inguinal freckling, cutaneous neurofibromas with a variable clinical expression, iris Lisch nodules, and multiple tumors of central and peripheral nervous system, also other systems may be involved. NF1 patients develop hypertension due to renovascular diseases, mid-aortic syndrome, or pheochromocytoma. In pediatric NF1 patients elevated blood pressure is usually due to renal artery stenosis, generally involving the origin or the proximal tract of the vessel, and is associated in 25% of patients with coarctation of the abdominal aorta.Several patients with NF1 and hypertension have been reported in the literature, showing the extreme variability in anatomical lesions. Although NF1 is a rare disease, it is an important cause of renovasculer hypertension in childhood. Abdominal bruit is an alerting physical finding in diagnosis of renal artery stenosis. We here presented our two cases diagnosed with hypertension while being screened for NF1 to emphasize the importance of blood pressure measurement in children with NF1 screening. Also we suggest that hypertensive NF1 patients should be examined in terms of stenosis in the major branch vessels of the aorta and renal arteries.

259

Neurofibromatosis , hypertension , children

Pamukkale Medical Journal

4

262

2015