CONTINUED ON PAGES 20&21 It brought me into contact with one of the world's leading experts, Professor Angus Clarke from Cardiff's Institute for Medical Genetics, who's spent decades working on the most common form of the disease, X-Linked
Hypohidrotic ectodermal dysplasia.
[2] There are 2 major types of this condition depending on the number and functionality of the sweat glands: (1) X-linked anhidrotic or
hypohidrotic, (ChristSiemens-Touraine syndrome) and (2) Hidrotic, (Clouston's syndrome).
Based on these investigations, our patients with ED, particularly the
hypohidrotic type, presented a high risk of enamel caries and susceptibility to opportunistic oral infections, which may be likely triggered by reduction of salivary flow and/or possible immunological disorders.
Patients with
hypohidrotic ectodermal dysplasia, also known as Christ-Siemens-Touraine syndrome, can present with EVHC.
It is classified into two major types based on number and functionality of sweat glands: (1) Anhidrotic or
hypohidrotic: Sweat glands are significantly reduced or absent and called as Christ-Siemens-Touraine syndrome.
Mutations in this gene cause X-linked
hypohidrotic ectodermal dysplasia (HED), which is characterised by sparse hair, fewer and smaller teeth, and a lack of sweat glands [42].
The diseases include phenylketonuria, sickle cell anemia, dystrophic epidermolysis bullosa, X-linked
hypohidrotic ectodermal dysplasia, and Friedreich's ataxia-just a few of the more than 1000 genetic disorders that are well-described and many more that are not.
Mutations in the human homologue of mouse dl cause autosomal recessive and dominant
hypohidrotic ectodermal dysplasia.
In several cases of dental implant placement in young boys with x-linked
hypohidrotic ED--treated from 1.5 to 6 years of age--treatment outcome has been reported to be successful [20,23,21].
According to The Macomb Daily, Binion has
hypohidrotic ectodermal dysplasia, which is characterized by a reduced ability to sweat, sparse hair growth, missing teeth and facial deformities.
Binion has a rare disorder called "
hypohidrotic ectodermal dysplasia" which is typified by a reduced ability to sweat, sparse hair growth, missing teeth and facial deformities.