Chen Jing, Mao Jianhua, Ye Lezhen, Zong Dongmei, Qiao Xiaohui
Department of Nephrology, Ningbo Women and Children's Hospital, Ningbo, China.
Department of Nephrology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Transl Pediatr. 2020 Dec;9(6):863-866. doi: 10.21037/tp-20-311.
Williams-Beuren syndrome (WBS) is an autosomal dominant disorder caused by a gene deletion on chromosome 7q11.23. Patients with WBS usually show a group of features such as developmental delay, cardiovascular anomalies, mental retardation, and characteristic facial appearance. It occurs in 1:7,500 live births and affects males and females equally. Recent studies showed that lower urinary tract symptoms were also frequent in WBS patients. However, there is extremely rare study report non-monosymptomatic nocturnal enuresis as the main manifestation of Williams syndrome in children. We reported a child with non-monosymptomatic nocturnal enuresis and multiple bladder diverticula as the main implications of Williams syndrome. A 7.6-year-old girl was admitted to our hospital due to frequent micturition, urgency, and nocturnal enuresis for 4 years, and B ultrasound of urinary system revealed multiple bladder diverticula. The patient was found to have 7q11.23 deletion that involves the elastin gene for WBS. Multiple bladder diverticula in WBS patients can lead to many lower urinary tract symptoms. The treatment for the lower urinary tract symptoms in WBS patients with multiple bladder diverticula is lacking. Lower urinary tract symptoms should be considered as a significant indicator of the clinical diagnosis of WBS and have a significant negative impact on patient's quality of life.
威廉姆斯-贝伦综合征(WBS)是一种常染色体显性疾病,由7号染色体q11.23区域的基因缺失引起。WBS患者通常表现出一系列特征,如发育迟缓、心血管异常、智力障碍和特殊面容。其发病率为1:7500活产儿,男女发病率相同。最近的研究表明,WBS患者下尿路症状也很常见。然而,极少有研究报告非单症状性夜间遗尿症作为儿童威廉姆斯综合征的主要表现。我们报告了一名以非单症状性夜间遗尿症和多发膀胱憩室为主要表现的威廉姆斯综合征患儿。一名7.6岁女孩因尿频、尿急和夜间遗尿4年入院,泌尿系统B超显示多发膀胱憩室。该患者被发现存在涉及WBS弹性蛋白基因的7q11.23缺失。WBS患者的多发膀胱憩室可导致许多下尿路症状。目前缺乏针对伴有多发膀胱憩室的WBS患者下尿路症状的治疗方法。下尿路症状应被视为WBS临床诊断的重要指标,且对患者生活质量有显著负面影响。