Kuwertz-Bröking E, Pohl J, Ernst R, von Lengerke H J, Schober O, Fründ S, Bulla M, Holzgreve W
Pädiatrische Nephrologie, Westfälische Wilhelms-Universität Münster.
Monatsschr Kinderheilkd. 1993 Jun;141(6):468-73.
Between 1984 and 1991, antenatal ultrasound scanning detected urinary tract malformations in 126 infants, who were investigated and treated postnatally in the childrens' hospital of the Westfälische Wilhelms-University Münster. 10 out of 126 children with urogenital changes, died in the first hours after birth, due to pulmonary hypoplasia (Potter's sequence), 1 further infant died later after cardiac operation, and another died of megacystic-megaureter-hypoperistaltic-syndrome. In the first months after birth 71 (61%) of 116 infants underwent urological surgery; 12/116 infants (10.3%) had severe bilateral kidney changes, some of them with severe deficiency of amniotic fluid before birth. 6/116 infants (5.2%) had chronic renal insufficiency, 2 of them will have to be dialyzed in early childhood and longterm, 14 patients (12%) are threatened by chronic renal failure. 14 patients (12%) developed severe arterial hypertension, all had to be treated with antihypertensive drugs, in 5 of them hypertension subsided after unilateral nephrectomy, another five had transient hypertension, but four require continued medical treatment. We describe the prenatal ultrasound findings, compared them with diagnosis after birth, illustrate diagnostics, plans of therapy, urological surgical interventions and nephrological consequences. Benefits and limitations of antenatal ultrasonography for the detection of urinary tract malformations and the treatment of those malformations before and after birth are discussed. In utero diagnosis of severe urinary tract abnormalities allows treatment of these infants immediately after birth, furthermore the prevention of severe infections, additional damage of renal tissue, and early diagnosis and treatment of arterial hypertension and metabolic imbalances caused by chronic renal insufficiency in early childhood.
1984年至1991年间,产前超声扫描在126名婴儿中检测出泌尿系统畸形,这些婴儿在明斯特威斯特法伦威廉大学儿童医院接受了产后检查和治疗。126名泌尿生殖系统有变化的儿童中,有10名在出生后的头几个小时因肺发育不全(波特序列)死亡,另有1名婴儿在心脏手术后死亡,还有1名死于巨膀胱-巨输尿管-蠕动减退综合征。在出生后的头几个月里,116名婴儿中有71名(61%)接受了泌尿外科手术;116名婴儿中有12名(10.3%)有严重的双侧肾脏改变,其中一些在出生前羊水严重不足。116名婴儿中有6名(5.2%)患有慢性肾功能不全,其中2名在幼儿期需要长期透析,14名患者(12%)面临慢性肾衰竭的威胁。14名患者(12%)出现严重的动脉高血压,所有人都必须接受降压药物治疗,其中5名患者在单侧肾切除术后高血压消退,另外5名患者有短暂性高血压,但有4名患者需要继续接受治疗。我们描述了产前超声检查结果,并将其与出生后的诊断进行了比较,阐述了诊断、治疗方案、泌尿外科手术干预及肾脏疾病后果。讨论了产前超声检查在检测泌尿系统畸形以及在出生前后治疗这些畸形方面的益处和局限性。对严重泌尿系统异常进行宫内诊断可使这些婴儿在出生后立即得到治疗,此外还可预防严重感染、肾组织的额外损伤,以及早期诊断和治疗幼儿期慢性肾功能不全引起的动脉高血压和代谢失衡。