Analysis of genitourinary anomalies in patients with VACTERL (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistula, Renal anomalies, Limb abnormalities) association
Analysis of genitourinary anomalies in patients with VACTERL (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistula, Renal anomalies, Limb abnormalities) association
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DOI：
10.1111/j.1741-4520.2010.00303.x
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发表时间：
2011-06-01
影响因子：
1.3
通讯作者：
Pineda-Alvarez, Daniel E.
中科院分区：
文献类型：
--
作者：
Solomon, Benjamin D.;Raam, Manu S.;Pineda-Alvarez, Daniel E.
本研究的目的是描述 VACTERL 协会中泌尿生殖 (GU) 异常的一种新模式，其中涉及影响椎骨、肛门、心脏、气管和食道、肾脏和四肢的先天性异常。我们收集了 105 名诊断为 VACTERL 关联的患者的临床数据，并分析了符合更严格纳入标准的 89 名患者的子集。 21% 的患者有 GU 异常，女性的情况更为严重（但不是更频繁）。在没有影响肾脏、下椎骨或下胃肠道系统的畸形的患者中发现了异常。即使患者没有空间相似的畸形，也应该高度怀疑 GU 异常的存在。
The goal of this study was to describe a novel pattern of genitourinary (GU) anomalies in VACTERL association, which involves congenital anomalies affecting the vertebrae, anus, heart, trachea and esophagus, kidneys, and limbs. We collected clinical data on 105 patients diagnosed with VACTERL association and analyzed a subset of 89 patients who met more stringent inclusion criteria. Twenty-one percent of patients have GU anomalies, which are more severe (but not more frequent) in females. Anomalies were noted in patients without malformations affecting the renal, lower vertebral, or lower gastrointestinal systems. There should be a high index of suspicion for the presence of GU anomalies even in patients who do not have spatially similar malformations.