Blood pressure and cardiovascular involvement in children with neurofibromatosis type1

We evaluated blood pressure in a sample of patients with neurofibromatosis type 1 (NF1), using ambulatory blood pressure monitoring (ABPM), to determine whether ABPM, when compared with casual BP recordings, allowed the detection of a higher risk for hypertension. We also evaluated the correlation b...

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Published inPediatric nephrology (Berlin, West) Vol. 19; no. 4; pp. 413 - 418
Main Authors Lama, Giuliana, Graziano, Luisa, Calabrese, Elvira, Grassia, Carolina, Rambaldi, Pier Francesco, Cioce, Fabrizio, Tedesco, Michele Adolfo, Di Salvo, Giovanni, Esposito-Salsano, Maria
Format Journal Article
LanguageEnglish
Published Germany Springer Nature B.V 01.04.2004
Subjects
Adolescent
Adult
Blood Pressure
Blood Pressure Monitoring, Ambulatory
Child
Child, Preschool
Coronary Vessels - pathology
Echocardiography
Electrocardiography
Female
Humans
Hypertension - diagnosis
Hypertension - etiology
Male
Myocardium - pathology
Neurofibromatosis 1 - complications
Risk Factors
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Summary:We evaluated blood pressure in a sample of patients with neurofibromatosis type 1 (NF1), using ambulatory blood pressure monitoring (ABPM), to determine whether ABPM, when compared with casual BP recordings, allowed the detection of a higher risk for hypertension. We also evaluated the correlation between BP and vascular abnormalities. We studied 69 NF1 patients (36 males and 33 females) with a mean age of 11+/-4 years, divided into group A, with 24-h mean systolic blood pressure (SBP) or diastolic blood pressure (DBP) <95th percentile, and group B, with mean SBP or DBP >95th percentile. Standard electrocardiography and M-mode, two-dimensional echocardiography were performed and all patients were in sinus rhythm. ABPM identified 11 hypertensive patients (16%); 5 had a mean SBP >95th percentile, 3 mean SBP-DBP >95th percentile, and 3 a mean DBP >95th percentile. Laboratory and other investigations to exclude secondary hypertension were normal. Cardiac abnormalities were found in 13 of the 69 patients (18.8%) with NF1. There were no significant clinical and cardiac differences between the normotensive and hypertensive group. Our data emphasize the importance of periodic ABPM in NF1 patients to diagnose hypertension early and avoid target organ damage and increased mortality.
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ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-003-1397-5