Special Health Care Needs

Question 1

What are key aspects of Lesch-Nyhan Syndrome?

Answer 1

• Patient are unable to break down purines
• This causes uric acid to build up
• Patient have self mutilative behavior
• Removal of all teeth is common treatment due to patient biting self mutilative tendencies.

Question 2

Hurler and Hunter Syndrome are also known was what respectively?

Answer 2

MSP-1 (Hurler)
MSP-2 (Hunter)
MSP=Mucopolysaccharidosis
-It results in the build up of glycosaminoglycans due to deficiency of alpha-L iduronidase (Responsible for the degradation of mucopolysaccharides in lysosomes)

Question 3

What is one feature of Hurler and Hunter Syndrome that is more significant with regards to dentists?

Answer 3

Clawed hands.
-OH is difficult for these patients.
Patient also have widely spaced teeth and large tongues.