Literature DB >> 3364056

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

B Franke1, D Missbach, K H Römer.   

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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Year:  1988        PMID: 3364056

Source DB:  PubMed          Journal:  Zentralbl Chir        ISSN: 0044-409X            Impact factor:   0.942


  2 in total

1.  Neonatal hydrometrocolpos associated with McKusick Kaufman syndrome.

Authors:  M Rohatgi; D K Gupta; M Luthra
Journal:  Indian J Pediatr       Date:  1989 May-Jun       Impact factor: 1.967

2.  Mckusick-kaufman syndrome presenting as acute intestinal obstruction.

Authors:  Ramesh B Hatti; Ashok V Badakali; R N Vanaki; Mahantesh S Samalad
Journal:  J Neonatal Surg       Date:  2013-01-01
  2 in total

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