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双侧孤立性胎儿高回声肾的产前诊断。能否预测长期预后?

Prenatal diagnosis of bilateral isolated fetal hyperechogenic kidneys. Is it possible to predict long term outcome?

作者信息

Tsatsaris V, Gagnadoux M F, Aubry M C, Gubler M C, Dumez Y, Dommergues M

机构信息

Department of Obstetrics, Maternité Port-Royal Hôpital Cochin, AP-HP, Paris, France.

出版信息

BJOG. 2002 Dec;109(12):1388-93. doi: 10.1046/j.1471-0528.2002.02055.x.

DOI:10.1046/j.1471-0528.2002.02055.x
PMID:12504976
Abstract

OBJECTIVE

To study perinatal and long term outcome following prenatal diagnosis of hyperechogenic kidneys.

DESIGN

Prospective observational cohort study.

SETTING

The Maternité Port-Royal Hôpital Cochin and at the Departments of Obstetrics and Paediatric Nephrology, Necker Enfants Malades in Paris, France.

POPULATION

Forty-three fetuses with isolated bilateral hyperechogenic kidneys.

METHODS

All patients referred with isolated bilateral hyperechogenic fetal kidneys were followed up prospectively up to 34-132 months. The following prenatal items were analysed: fetal kidney size, amniotic fluid volume, gestational age at diagnosis, family history and renal ultrasound in parents. Postmortem examination was carried out in cases with perinatal death. Postnatal follow up of survivors included postnatal ultrasound, blood pressure, serum creatinine, proteinuria, need for restricted diet, weight and height and renal biopsy when available.

MAIN OUTCOME MEASURES

Aetiology of hyperechogenicity, perinatal mortality and renal function in survivors.

RESULTS

The aetiology could be established by family history, postmortem or postnatal data, but not by prenatal ultrasound. There were 20 autosomal recessive, 8 autosomal dominant polycystic kidney diseases, 9 other renal disorders and 6 symptom-free survivors without aetiological diagnosis. There were 19 terminations of pregnancy, 5 neonatal deaths and 19 survivors, of whom 14 had normal renal function three had mild and two had end stage renal failure. None of those with severe oligohydramnios and fetal kidneys > 4 SD survived (n = 14, 10 terminations and 4 neonatal deaths), whereas of the 17 with normal amniotic fluid volume and kidneys < 4 SD, 14 survived, of whom 9 were symptom-free.

CONCLUSION

Aetiology could not be established prenatally in the absence of familial data. Kidney size and amniotic fluid volume were the best prenatal predictors of outcome.

摘要

目的

研究产前诊断为肾回声增强后的围产期及长期结局。

设计

前瞻性观察队列研究。

地点

法国巴黎科钦皇家妇产医院以及内克尔儿童医院的产科和儿科肾脏病科。

研究对象

43例孤立性双侧肾回声增强的胎儿。

方法

对所有转诊来的孤立性双侧胎儿肾回声增强患者进行前瞻性随访,随访时间长达34 - 132个月。分析以下产前项目:胎儿肾脏大小、羊水量、诊断时的孕周、家族史以及父母的肾脏超声检查结果。围产期死亡病例进行尸检。存活者的产后随访包括产后超声检查、血压、血清肌酐、蛋白尿、是否需要限制饮食、体重和身高,如有条件还包括肾活检。

主要观察指标

回声增强的病因、围产期死亡率以及存活者的肾功能。

结果

病因可通过家族史、尸检或产后数据确定,但不能通过产前超声确定。其中20例为常染色体隐性遗传,8例为常染色体显性多囊肾病,9例为其他肾脏疾病,6例无症状存活者病因未明确。19例终止妊娠,5例新生儿死亡,19例存活,其中14例肾功能正常,3例轻度异常,2例终末期肾衰竭。羊水过少且胎儿肾脏大于4个标准差的患者无一存活(共14例,10例终止妊娠,4例新生儿死亡),而羊水正常且肾脏小于4个标准差的17例患者中,14例存活,其中9例无症状。

结论

在没有家族数据的情况下,产前无法确定病因。肾脏大小和羊水量是结局的最佳产前预测指标。

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