Krzemień Grażyna, Szmigielska Agnieszka, Wawer Zofia, Roszkowska-Blaim Maria
Katedra i Klinika Pediatrii i Nefrologii WUM, Marszałkowska 24, 00-576 Warszawa,
Med Wieku Rozwoj. 2013 Oct-Dec;17(4):301-5.
The incidence of posterior urethral valves is estimated to be from 3:1000 to 8:1000 and this is one of the most common causes of obstruction of urinary tract in boys. About 13-17% of children with posterior urethral valves develop end stage renal failure. We present a 6-month-old boy with late diagnosis of posterior urtehral valves. Antenatal ultrasound investigation of the urinary tract was normal. A small degree of oligohydramnios was found during delivery. At the age of six months the boy was admitted to hospital because of urinary tract infection, hypertension (130/90 mmHg) and acute kidney injury (urea - 46 mg/dL, creatinine - 1.1 mg/dL, GFR - 35.5 mL/min/1.73 m2 ). Bilateral hydronephrosis and megaureters, low-capacity bladder with hypertrophied wall were seen on ultrasound examination. Voiding cystourethrograhy revealed vesicoureteral refluxes (III/V), hypertrophy of the bladder wall with numerous diverticula and dilated posterior urethra. During urethroscopy urethral valves were resected. Increased intravesical pressure (leak point up to 305 cm H2 O) was found on urodynamic test. Renal scintigraphy (99mTc-EC) revealed decreased intake of isotope in the left kidney (5%), and the right kidney intake was 95% ERPF. The patient was qualified for left-sided nephrectomy, which was postponed because of high leak point and high risk of worsening of vesicoureteral reflux to right kidney after nephrectomy. Anticholinergic and α-blocker treatment was started. At the age of 11 months left-side nephrectomy was performed because of recurrent urinary tract infections. After 3.5-year follow-up blood pressure, physical development, kidney function tests, and urinalysis are normal. Additionally to this investigation the significance of early diagnosis including prenatal (PUV) for further development as well as further therapeutic procedure is discussed.
后尿道瓣膜症的发病率估计为千分之三至千分之八,这是男孩尿路梗阻最常见的原因之一。约13 - 17%患有后尿道瓣膜症的儿童会发展为终末期肾衰竭。我们报告一名6个月大的男孩,后尿道瓣膜症诊断较晚。产前泌尿系统超声检查正常。分娩时发现有轻度羊水过少。6个月大时,该男孩因尿路感染、高血压(130/90 mmHg)和急性肾损伤(尿素 - 46 mg/dL,肌酐 - 1.1 mg/dL,肾小球滤过率 - 35.5 mL/min/1.73 m²)入院。超声检查可见双侧肾积水和巨输尿管、膀胱容量小且壁肥厚。排尿性膀胱尿道造影显示膀胱输尿管反流(III/V级)、膀胱壁肥厚伴多发憩室以及后尿道扩张。尿道镜检查时切除了尿道瓣膜。尿动力学检查发现膀胱内压升高(漏点高达305 cm H₂O)。肾闪烁扫描(⁹⁹ᵐTc - EC)显示左肾同位素摄取减少(5%),右肾摄取为有效肾血浆流量的95%。该患者符合左侧肾切除术条件,但因漏点高以及肾切除术后膀胱输尿管反流至右肾恶化风险高而推迟手术。开始使用抗胆碱能药物和α受体阻滞剂治疗。11个月大时,因反复尿路感染进行了左侧肾切除术。经过3.5年的随访,血压、身体发育、肾功能检查和尿液分析均正常。此外,还讨论了包括产前(后尿道瓣膜症)早期诊断对后续发展以及进一步治疗程序的意义。