Prader-Willi氏症候群患者與下泌尿道排尿障礙
趙梓辰1、楊緒棣1、蔡立平2
1台北慈濟醫院 泌尿科，2兒科部
Prader-Willi Syndrome Patients and Low Urinary Tract Dysfunction
Tze-Chen Chao1, Stephen Shei-Dei Yang1, Li-Ping Tsai2
1Division of Urology and 2Department of Pediatrics, Taipei Tzu Chi General Hospital, Buddhist Tzu Chi Medical Foundation, Taipei, Taiwan
Purpose: To report the first non-invasive screening of lower urinary tract dysfunction (LUTD) in children, adolescents, and young adults with Prader-Willi Syndrome (PWS).
Materials and Methods: We recruited 27 PWS patients without lower urinary tract symptoms (LUTS) from a one-day holiday camps for PWS family. Of them 13 parents had complete dysfunctional voiding scoring system (DVSS) questionnaire. Uroflowmetry was performed in 26 patients. In addition, 13 patients underwent PVR measurement by transabdominal ultrasound. LUTD is defined as DVSS ≥7, peak flow rate (Qmax) < 15 m/s if age ≥7 years, and abnormal uroflow patterns.
Results: Mean and Median age of the patients were 17.9 ± 7.2 years and 16 years old. Male to female ratio was 13/14. Among the 13 patients, 5 (38%) had DVSS ≥7. Voided volume was <50ml in 1 patient, who was excluded from the following analysis. Bell pattern curves was observed in 18 (72%), and obstructive in 3 (12%), intermittent in 2 (8%), staccato in 1 (4%), tower in 1 (4%), and plateau in 0 patients. Five (19%) patients had a peak flow rate (Qmax) < 15ml/s. Of 12 patients had PVR, 7 had elevated PVR by age (> 6% of bladder volume). In all, 12 (44 %) had at least one abnormality.
Conclusions: About half of PWS patients in a holiday camp without LUTS may have LUTD. Non-invasive study of LUTD is recommended to all PWS patients.