Objectives and studies: The aim of this retrospective study is to assess the prevalence of hypertension in a population of 69 pediatric patients with NF1 and to confirm the relationship between NF1 and secondary form of hypertension. Methods: In the Pediatric Centre NF1 of the Second University of Naples we take care of 312 patients. We checked blood pressure with Office Monitoring Method (OBPM) and then we performed ABPM on 69 patients (36 males and 33 females) with a medium(mean) age of 11 years (5–25 years). We considered hypertensive patients who showed two ABPM with mean SBP24-h and/or mean DBP24-h above age and sex specific 95th percentile. Diagnosis of renovascular hypertension is suspected in patients with poorly controlled blood pressure on at least two antihypertensive agents and confirmed by PRA-ALDO, echo-color Doppler of the renal artery and \“pulse wave velocity test”, Renal scintigraphy performed before and after administration of an angiotensin-converting enzyme inhibitor, MR angiography of renal artery and DSA . The urinary assay of catecholamines, VMA, 5-HIAA, HVA and metanephrines can verify diagnosis of pheochromocytoma. Aortic coarctation can be confirmed by chest Rx, echo-color-Doppler and MR Angiography. Results: Hypertension was diagnosed in 14 patients, with a medium age of 10 years and 4 months (5–14 years) at the time of diagnosis. Results: suspected renovascular hypertention in 2 patients at present in therapy with two drugs, bilateral renal artery stenosis in one patient, pheochromocytoma in 3 patients, aortic coarctation in one patient and essential hypertension with abnormalities of the course of both renal arteries in one patient. Six patients were lost during the follow-up. Conclusions: The prevalence of Hypertension in the NF1 patients is 20,29% and the prevalence of secondary hypertension is 87.5%.
Neurofibromatosis type 1 as an example of high prevalence of secondary hypertension
PELLINO, Giuditta;
2011
Abstract
Objectives and studies: The aim of this retrospective study is to assess the prevalence of hypertension in a population of 69 pediatric patients with NF1 and to confirm the relationship between NF1 and secondary form of hypertension. Methods: In the Pediatric Centre NF1 of the Second University of Naples we take care of 312 patients. We checked blood pressure with Office Monitoring Method (OBPM) and then we performed ABPM on 69 patients (36 males and 33 females) with a medium(mean) age of 11 years (5–25 years). We considered hypertensive patients who showed two ABPM with mean SBP24-h and/or mean DBP24-h above age and sex specific 95th percentile. Diagnosis of renovascular hypertension is suspected in patients with poorly controlled blood pressure on at least two antihypertensive agents and confirmed by PRA-ALDO, echo-color Doppler of the renal artery and \“pulse wave velocity test”, Renal scintigraphy performed before and after administration of an angiotensin-converting enzyme inhibitor, MR angiography of renal artery and DSA . The urinary assay of catecholamines, VMA, 5-HIAA, HVA and metanephrines can verify diagnosis of pheochromocytoma. Aortic coarctation can be confirmed by chest Rx, echo-color-Doppler and MR Angiography. Results: Hypertension was diagnosed in 14 patients, with a medium age of 10 years and 4 months (5–14 years) at the time of diagnosis. Results: suspected renovascular hypertention in 2 patients at present in therapy with two drugs, bilateral renal artery stenosis in one patient, pheochromocytoma in 3 patients, aortic coarctation in one patient and essential hypertension with abnormalities of the course of both renal arteries in one patient. Six patients were lost during the follow-up. Conclusions: The prevalence of Hypertension in the NF1 patients is 20,29% and the prevalence of secondary hypertension is 87.5%.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
