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[McKusick-Kaufman syndrome as a cause of acute abdomen in the neonatal period].

作者信息

Franke B, Missbach D, Römer K H

机构信息

Abteilung für Kinderchirurgie, Medizinischen Akademia Magdeburg.

出版信息

Zentralbl Chir. 1988;113(4):254-8.

PMID:3364056
Abstract

The McKusick-Kaufman-syndrome, also known by the name of hydrometrocolpos-hexadactyly syndrome, is an autosomal-recessive hereditary disease or spontaneous malformation. A hydrometrocolpos is formed in the process by a transversal vaginal septum and subsequently acts like an abdominal tumour to compress the iliac veins and to result in supravesical obstruction, eventually causing a condition of ileus and respiratory problems. Transvaginal drainage is used as primary therapy.

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