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“波特综合征”的超声检查结果(作者译)

[Ultrasonographic findings in "Potter's syndrome" (author's transl)].

作者信息

Schmidt W, Kubli F, Schroeder T

出版信息

Geburtshilfe Frauenheilkd. 1981 May;41(5):374-81. doi: 10.1055/s-2008-1036813.

Abstract

The foetal Potter's syndrome represents a relatively frequent malformation syndrome, the incidence being 0.3% of all live births. The phenotypical changes seen in these newborn occur in complete bilateral renal agenesia = the original Potter's syndrome, but they are also found in various congenital cystic diseases of the kidneys and/or the other urinary organs. In additional to the original Potter's syndrome, a patho-anatomical classification of cystic deformations of the kidneys or of the efferent urinary organs was introduced by Osathanondh and Potter (1976) (Potter Type I-Type IV). These severe foetal malformations can be diagnosed at least in part via timely ultrasonic examination during the first half of the pregnancy period. In such cases, an oligoamnion or sometimes an anhydramnion is regularly observed as principal sign. This diagnosis is probably confirmed if the foetal urinary bladder is not demonstrable following application of furosemide (Campbell). Occasionally the cystic changes of the urinary organs can be proven direct by means of sonography. Ten examples of cases are described presenting the typical ultrasonographic findings in the so-called extended "Potter's syndrome" (oligoamnion or anhydramnion, malformations of the kidneys and/or the efferent urinary organs and phenotypical changes). Due to their infaust prognosis these foetal diseases result in the indication for termination of pregnancy.

摘要

胎儿波特综合征是一种相对常见的畸形综合征,发病率占所有活产婴儿的0.3%。这些新生儿出现的表型变化见于完全性双侧肾缺如(即原始的波特综合征),但也见于各种先天性肾脏和/或其他泌尿器官的囊性疾病。除了原始的波特综合征外,Osathanondh和Potter(1976年)还引入了肾脏或传出泌尿器官囊性畸形的病理解剖分类(波特I型至IV型)。这些严重的胎儿畸形至少可以部分通过孕期前半期及时的超声检查来诊断。在这种情况下,羊水过少或有时羊水过少是常见的主要体征。如果应用速尿后未显示胎儿膀胱(坎贝尔法),则可能证实该诊断。偶尔,泌尿器官的囊性变化可通过超声检查直接证实。本文描述了10例病例,呈现了所谓扩展型“波特综合征(羊水过少或羊水过少、肾脏和/或传出泌尿器官畸形以及表型变化)的典型超声检查结果。由于其预后不良,这些胎儿疾病导致了终止妊娠的指征。

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