We sincerely appreciate the comments made by Nasir et al. regarding our paper. The aim of our article was to focus the attention on the involvement of the urinary bladder in patient with NF1.
Our patient was referred to our institution for failure to thrive when he was 3 months old. He never showed dyspnea or cough. Although it is not reported in detail, he also underwent an extensive cardiological and pulmonary evaluation including physical examination, EKG and echocardiography. Repeated evaluations were necessary during the following 5 years, before performing a radical cystoprostatectomy. Currently he is under regular follow-up assessing all the systems. No signs of pulmonary hypertension as right atrial enlargement, right axis deviation, right ventricular hypertrophy, high-output cardiac failure were never detected.
NF1 can have various clinical manifestations in different times of appearance. We completely agree with Nasir et al. and recommend that all the patients with von Recklinghausen disease should be evaluated and monitored to exclude pulmonary hypertension as a major cause of morbidity and mortality.
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Published online: October 10, 2013
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© 2013 Journal of Pediatric Urology Company. Published by Elsevier Inc. All rights reserved.
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- Re: ‘Diffuse ganglioneuromatosis and plexiform neurofibroma of the urinary bladder: An uncommon cause of severe urological disease in an infant’. J Pediatr Urol. 2013 Apr; 9(3):e131–e133Journal of Pediatric UrologyVol. 10Issue 1
- PreviewWe read with interest the article by Salvitti et al. [1]. As stated by the authors, visceral involvement is typically insidious, progressive, and difficult to treat. It has been theorized that the plexiform neurofibroma involving the bladder is derived from the vesicoprostatic plexus in males and the urethrovaginal plexus in females [2]. Variable involvement of different segments of the plexus translates into a variable constellation of symptoms and signs that arise as neurofibromas enlarge, compressing and distorting local structures.
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