[McKusick-Kaufman syndrome as a cause of acute abdomen in the neonatal period].
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.
Franke
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B
Abteilung für Kinderchirurgie, Medizinischen Akademia Magdeburg.
Missbach
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D
Römer
K H
KH
D000006:Abdomen, Acute / Q000209:etiology*; D000015:Abnormalities, Multiple / Q000235:genetics; D002582:Cervix Mucus / Q000378:metabolism*; D005260:Female; D005385:Fingers / Q000002:abnormalities; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities; D013577:Syndrome; D014521:Urethra / Q000002:abnormalities*; D014599:Uterus / Q000002:abnormalities*; D014621:Vagina / Q000002:abnormalities*
Das McKusick-Kaufman-Syndrom als Ursaché eines akuten Abdomens im Neugeborenenalter.