Dubov Tom, Toledano-Alhadef Hagit, Chernin Gil, Constantini Shlomi, Cleper Roxana, Ben-Shachar Shay
Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
The Gilbert Israeli Neurofibromatosis Center, Tel-Aviv Medical Center, Tel-Aviv, Israel.
Pediatr Nephrol. 2016 Jan;31(1):131-6. doi: 10.1007/s00467-015-3191-6. Epub 2015 Aug 28.
Neurofibromatosis type 1 (NF1) is a common neurocutaneous disease characterized by café-au-lait spots, axillary and inguinal freckling, neurofibromas, and optic gliomas. Increased rates of hypertension (HTN) were reported among NF1 patients, however, the prevalence of HTN and pre-HTN in pediatric NF1 patients has not been clarified.
Blood pressure (BP) measurements, weight, and renal ultrasound were assessed in 224 NF1 pediatric patients followed in a specialized NF1 clinic.
The cohort's mean age was 9.1 ± 4.1 years. Overweight and obesity were found in 12.9 and 10.3 % of them, respectively. BP was measured averagely 2.9 times per patient on different occasions. Blood pressure was in the pre-HTN and HTN ranges in 14.9 and 16.9 % of measurements, respectively. BP >95th was detected in 20.5 % at the first measurement. Of 114 children with at least three BP measurements, 18.4 % had two values in the HTN range and 6.14 % had at least three. Overweight was not associated with HTN among children with NF1. Urinary tract ultrasonographic abnormalities were detected in 6.8 % (11/161) of cases.
The prevalence of increased BP in pediatric NF1 is much higher than in the general pediatric population. BP has to be regularly assessed and managed in this high-risk population.
1型神经纤维瘤病（NF1）是一种常见的神经皮肤疾病，其特征为牛奶咖啡斑、腋窝和腹股沟雀斑、神经纤维瘤以及视神经胶质瘤。有报道称NF1患者中高血压（HTN）发病率升高，然而，小儿NF1患者中HTN和高血压前期的患病率尚未明确。
对在一家专门的NF1诊所就诊的224例小儿NF1患者进行血压（BP）测量、体重测量及肾脏超声检查。
该队列的平均年龄为9.1±4.1岁。分别有12.9%和10.3%的患者超重和肥胖。每位患者平均在不同时间测量BP 2.9次。分别有14.9%和16.9%的测量值处于高血压前期和HTN范围。首次测量时，20.5%的患者BP>第95百分位数。在114例至少进行了三次BP测量的儿童中，18.4%有两个值处于HTN范围，6.14%有至少三个值处于该范围。NF1患儿中超重与HTN无关。6.8%（11/161）的病例检测到泌尿系统超声异常。
小儿NF1患者中BP升高的患病率远高于普通小儿人群。必须对这一高危人群定期进行BP评估和管理。
