Journal of Disability and Oral Health

cover art

Cover Date:
March 2022
Electronic ISSN:
1754-2758
Vol:
23
Issue:
1

Hyperinsulinism/Hyperammonaemia Syndrome, considerations in dental management

Abstract
Hyperinsulinism/Hyperammonemia syndrome (HIHA) is a rare congenital metabolic disorder with implications in dental practice. Patient LH presented with dental pain and caries in three quadrants. Understanding of HIHA clinical presentation (seizures, hypoglycaemia, global developmental delay) can allow patient specific preventative advice and treatment.


Introduction
A recent encounter with a patient diagnosed with Hyperinsulinism/Hyperammonaemia syndrome (HIHA)
prompted an interest in the clinical background and implications for dental treatment. Reflecting the rarity of the
syndrome, a literature search performed by our local library team found no results specifically for HIHA syndrome and
dental or oral management despite its specific implications for dentistry. HIHA is a rare genetic disorder affecting
around 1 in 200,000 births and is the second most common form of congenital hyperinsulinism (Orphanet, 2015).

Article Price
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Authors

Articles from this issue

  • Title
  • Pg. Start
  • Pg. End

  1. Editorial
  2. 1
  3. 3

  1. Hyperinsulinism/Hyperammonaemia Syndrome, considerations in dental management
  2. 4
  3. 8

  1. Integrating domiciliary and hospital based special care dental services to provide patient-centred care under sedation and general anaesthesia
  2. 9
  3. 15

  1. Because everyone deserves a special smile
  2. 16
  3. 19

  1. Reported barriers to delivering preventative (oral health) advice and domiciliary dental care
  2. 20
  3. 26

  1. Challenges associated with providing specialist dental services for individuals with special health care needs in Australia
  2. 27
  3. 33

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