This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Kabuki syndrome

Authoring team

  • Kabuki syndrome is also known as Kabuki make-up syndrome, as sufferers mimic the facial makeup of actors in the Japanese Kabuki theatre
  • Kabuki syndrome is a congenital condition characterised by multiple anomalies and usually associated with mild to moderate learning disabilities
  • key features seen in Kabuki syndrome are its peculiar facies
    • a review has stated that Kabuki syndrome:
      • characterized by a dysmorphic face, postnatal growth retardation, skeletal abnormalities, mental retardation, and unusual dermatoglyphic patterns
        • facial features most commonly identified in patients with Kabuki syndrome include long palpebral fissures, eversion of the lower eyelids, large ears, highly arched eyebrows, and a flat nasal tip
      • in 2003, approximately more than 350 cases had been reported from all over the world
        • besides these five cardinal manifestations, joint laxity (74%), dental abnormalities (68%), and susceptibility to infections including recurrent otitis media (63%) were well recognized as other frequent features
        • a variety of visceral anomalies such as cardiovascular anomalies (42%), renal and/or urinary tract anomalies (28%), biliary atresia, diaphragmatic hernia, and anorectal anomaly were also reported
        • some patients were said to have normal intelligence (16%) and normal heights, suggesting that they may have reproductive fitness to have their children
        • have been 13 chromosomal abnormalities associated with Kabuki syndrome - however, no common abnormalities or breakpoints that possibly contribute to positional cloning of the putative Kabuki syndrome gene(s) are known
  • Kabuki syndrome may be accompanied by a weakened immune system and increased susceptibility to infection (2)
  • cleft palate is a feature that is sometimes observed in patients with Kabuki syndrome (3)

Kabuki Syndrome support group

Reference:

  1. Matsumoto N et al. Kabuki make-up syndrome: a review. American Journal of Medical Genetics 2003; 117C: 57?65
  2. Wessels MW, Brooks AS, Hoogeboom J, Niermeijer MF, Willems PJ. Kabuki syndrome: a review study of three hundred patients. Clinical Dysmorphology, 2002; 11: 95?102.
  3. Iida T et al. Cleft palate in Kabuki syndrome: a report of six cases. Cleft Palate Craniofac J. 2006 Nov;43(6):756-61

Create an account to add page annotations

Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

Connect

Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.