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首页> 外文期刊>Pediatrics: Official Publication of the American Academy of Pediatrics >Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.
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Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.

机译：特纳综合征中胎儿淋巴水肿与先天性心血管缺陷之间的关联。

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OBJECTIVES: Turner syndrome (TS) is associated with congenital cardiovascular defects (CCVDs), most commonly bicuspid aortic valve (BAV) and aortic coarctation (COARC), congenital renal anomalies, and fetal lymphedema. It has been theorized that compressive or obstructive effects of fetal lymphedema may actually cause cardiovascular and renal dysmorphogenesis in TS. The objective of this study was to determine whether there is a specific association between a history of fetal lymphedema and CCVDs in monosomy X, or TS, independent of karyotype or general severity of the phenotype. METHODS: This was a prospective study of 134 girls and women who have TS (mean age: 30 years) and were clinically evaluated for evidence of fetal lymphedema, classified as central (signified by the presence of neck webbing) or peripheral (current or perinatal, or dysplastic fingernails). The presence of BAV and/or COARC was detected by magnetic resonance imaging combined with echocardiography, and renal anomalies were determined by ultrasound. RESULTS: There is a strong association between developmental central lymphedema, signified by neck webbing, and the presence of BAV (chi2 = 10) and COARC (chi2 = 8). The association between webbed neck and CCVDs was independent of karyotype. There was, in contrast, no significant association between renal anomalies and webbed neck or CCVDs. CONCLUSIONS: The strong, statistically significant association between neck webbing and the presence of BAV and COARC in TS suggests a pathogenetic connection between fetal lymphatic obstruction and defective aortic development. The presence of neck webbing in TS should alert the clinician to the possibility of congenital cardiovascular defects.
机译：目的：特纳综合征（TS）与先天性心血管缺陷（CCVD），最常见的二尖瓣主动脉瓣（BAV）和主动脉缩窄（COARC），先天性肾异常和胎儿淋巴水肿有关。从理论上讲，胎儿淋巴水肿的压缩或阻塞作用实际上可能导致TS发生心血管和肾脏畸形。这项研究的目的是确定胎儿淋巴水肿史与单核X或TS中的CCVD之间是否存在特异性关联，而与核型或表型的一般严重性无关。方法：这是一项对134名患有TS（平均年龄：30岁）并经过临床评估的胎儿淋巴水肿证据的女孩和妇女的前瞻性研究，分类为中枢性（通过出现颈带出现）或周围性（当前或围产期）或指甲发育不良）。通过磁共振成像结合超声心动图检测BAV和/或COARC的存在，并通过超声确定肾脏异常。结果：发育中枢淋巴水肿（以颈部织带表示）与BAV（chi2 = 10）和COARC（chi2 = 8）的存在密切相关。蹼状颈与CCVD之间的关联与核型无关。相反，肾异常与蹼状颈或CCVD之间无明显关联。结论：颈部织带与TS中BAV和COARC的存在之间在统计学上的显着相关性提示胎儿淋巴阻塞与主动脉发育不良之间存在致病性联系。 TS中存在颈部织带应提醒临床医生先天性心血管缺陷的可能性。

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