Brief Title
Short Term Effects and Risks of Physical Exercise in Subjects With Hypohidrotic Ectodermal Dysplasia
Official Title
Short Term Effects and Risks of Physical Exercise in Subjects With Hypohidrotic Ectodermal Dysplasia (Christ-Siemens-Touraine Syndrome)
Brief Summary
Because of their lack of sweat glands individuals with hypohidrotic ectodermal dysplasia (HED) are at particular risk of life-threatening hyperthermia during exercise in a warm environment. In this study, the effects of physical exercise are investigated in boys and male adolescents with X-chromosomally inherited HED as well as age-matched controls, who undergo standardized exertion on a bicycle ergometer at ambient temperatures of 25°C and 30°C. Body core temperature during and after ergometry, heart rate, performance, and serum lactate as a marker of metabolic stress are measured. Subjects with HED are expected to show an endangering rise of body temperature in connection with physical exercise. To clarify, whether novel cooling devices may reduce the likelihood of overheating, the effects of such devices are evaluated at 30°C.
Study Type
Observational
Condition
X-Linked Hypohidrotic Ectodermal Dysplasia
Intervention
Skin cooling devices
Study Arms / Comparison Groups
HED children
Description:
Publications
* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.
Recruitment Information
Recruitment Status
Device
Estimated Enrollment
28
Start Date
April 2009
Completion Date
February 2010
Primary Completion Date
February 2010
Eligibility Criteria
Inclusion Criteria: - for patients: hypohidrotic ectodermal dysplasia caused by EDA gene mutations - regular fluid intake prior to the investigation - written informed consent Exclusion Criteria: - acute febrile illness - acute or chronic heart disease - arterial hypertension - gastrointestinal disorders - implantable electronic devices - MRI investigation scheduled for the 5 days subsequent to the study - clinical signs or diagnostic findings of dehydration
Gender
Male
Ages
7 Years - 18 Years
Accepts Healthy Volunteers
Accepts Healthy Volunteers
Contacts
Holm Schneider, MD, ,
Location Countries
Germany
Location Countries
Germany
Administrative Informations
NCT ID
NCT01135888
Organization ID
ED09
Responsible Party
Principal Investigator
Study Sponsor
University Hospital Erlangen
Collaborators
Pervormance GmbH
Study Sponsor
Holm Schneider, MD, Principal Investigator, University Hospital Erlangen
Verification Date
August 2021