Short Term Effects and Risks of Physical Exercise in Subjects With Hypohidrotic Ectodermal Dysplasia

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X-linked Hypohidrotic Ectodermal Dysplasia
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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