Abstract
Menkes syndrome is x-linked recessive disorder which originates because of a problem with the congenital absorption of the copper in the epithelial cells of the small intestine. Various clinical findings are manifested due to the decline of some copper-dependent enzyme activity. However, little is known about the specific characteristics of the oral traits in Menkes syndrome children owing to the lack of sufficient case reports. The findings of a three-year-old boy affected Menkes syndrome were as follows.
1. Low body height and weight.
2. Mental and physical developmental retardation and delay of bone age.
3. Osteoarhritis of the spine and funnel shaped breast.
4. Kinky hair and abnormal pigmentation in skin and hair.
5. Micrognathia of mandible and crowding in anterior lower dental arch.
6. Hypsistaphylia and deep overbite.
7. Rampant caries owing to a continuous over-intake of sport-drink and poor oral hygiene.
