Literature DB >> 7536394

Dubowitz syndrome: long-term follow-up of an original patient.

K E Hansen1, S J Kirkpatrick, R Laxova.   

Abstract

Dubowitz syndrome is an autosomal recessive disorder of growth retardation, characteristic face, mild mental retardation, and eczema originally described by Dubowitz [1965]. Little information is available on natural history and adulthood in this disorder. We report on a 30-year-old woman who was one of the first patients to be diagnosed with the condition [Grosse et al., 1971, Z Kinderheilkd 110:175-187]. Microcephaly, short stature, leg length discrepancy, hyperextensible joints, spina bifida occulta, and absence of anterior cruciate ligaments were present. Her facial appearance had been modified by several plastic surgery procedures. Eczema resolved with age, with occasional flareups. Asthma, headaches, and seizures were additional medical findings. Speech delays, an unusually soft, high-pitched voice, submucous cleft palate, and velopharyngeal insufficiency were noted in childhood. Mild mental retardation was present. At age 30 years she is living independently in her own apartment and working full-time in a nearby sheltered workshop.

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Year:  1995        PMID: 7536394     DOI: 10.1002/ajmg.1320550205

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  3 in total

1.  Anesthesia of a patient with Dubowitz syndrome -A case report-.

Authors:  Min Kee Lee; Yong Seock Lee
Journal:  Korean J Anesthesiol       Date:  2010-05-31

2.  Endocrinological features of a patient with 14q microdeletion and Dubowitz phenotype.

Authors:  Maria Elisa Amodeo; Elena Inzaghi; Annalisa Deodati; Stefano Cianfarani
Journal:  Mol Genet Genomic Med       Date:  2021-03-31       Impact factor: 2.183

3.  Dubowitz syndrome: a review and implications for cognitive, behavioral, and psychological features.

Authors:  Rebekah S Huber; Daniel Houlihan; Kevin Filter
Journal:  J Clin Med Res       Date:  2011-07-26
  3 in total

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