2nd Urology Department, Sisli Etfal Training and Research Hospital, Istanbul, Turkey.
Urology. 2011 Dec;78(6):1397-401. doi: 10.1016/j.urology.2011.08.018. Epub 2011 Oct 11.
To report the long-term follow-up results of patients with Hinman-Allen syndrome (HAS) at our institution.
The data from 22 children with HAS were retrospectively analyzed. The patients were followed up every 3-6 months with serial physical examinations, voiding charts, urine culture, postvoid residual urine volume determination, serum creatinine measurement, and urinary imaging. The follow-up time was calculated from the day of the first visit to the day of the latest dimercaptosuccinic acid scan. Urotherapy, pharmacotherapy, clean intermittent catheterization, biofeedback therapy, and surgery were performed sequentially and/or combined, depending on the disease course. Renal deterioration was defined as any presence of a new scar or cortical thinning compared with the findings from the first dimercaptosuccinic acid scan. Upper urinary tract deterioration was defined as the persistence or progression of hydronephrosis on ultrasonography.
The mean age at referral was 9.18 ± 3.36 years (range 2-14), and the mean follow-up period was 80.90 ± 19.57 months (range 54-144). Conservative therapy resulted in improvement of the bladder function in 14 patients; however, 8 patients required surgery owing to failure of this approach. Asymptomatic bacteriuria developed in one half of the children (n = 11, 50%), and in 6 (22.7%), ≥1 febrile urinary tract infection developed. None of the patients had upper urinary tract deterioration; however, renal deterioration developed in 3 patients (13.6%). The mean creatinine levels had remained stable at the end of the follow-up.
Close follow-up at a single institution and proactive treatment resulted in successful stabilization of HAS in most of our children with HAS.
报告本机构内 Hinman-Allen 综合征（HAS）患者的长期随访结果。
回顾性分析 22 例 HAS 患儿的资料。患者每隔 3-6 个月接受一次随访，包括体格检查、排尿图、尿液培养、残余尿量测定、血清肌酐测量和尿液影像学检查。随访时间从首次就诊当天计算至最新的二巯丁二酸扫描当天。根据疾病进程，依次或联合采用尿动力学治疗、药物治疗、间歇性清洁导尿、生物反馈治疗和手术治疗。肾损害定义为与首次二巯丁二酸扫描结果相比，新出现瘢痕或皮质变薄。上尿路恶化定义为超声检查显示肾积水持续或进展。
转诊时的平均年龄为 9.18 ± 3.36 岁（范围 2-14 岁），平均随访时间为 80.90 ± 19.57 个月（范围 54-144 个月）。14 例患者经保守治疗后膀胱功能改善，但 8 例患者因保守治疗失败而需要手术。一半（n = 11，50%）的患儿出现无症状菌尿，6 例（22.7%）患儿发生≥1 次发热性尿路感染。无一例患儿出现上尿路恶化，但 3 例（13.6%）患儿出现肾损害。随访结束时，平均肌酐水平保持稳定。
在单一家机构进行密切随访并积极治疗，使我们大多数 HAS 患儿的病情得到成功稳定。
