Trial record 5 of 28 for:    "hypohidrotic ectodermal dysplasia" OR "Ectodermal Dysplasia" OR "Anhidrotic Ectodermal Dysplasias" OR "Christ-Siemens-Touraine Syndrome"

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

This study has been completed.
Sponsor:
Collaborator:
Pervormance GmbH
Information provided by:
University Hospital Erlangen
ClinicalTrials.gov Identifier:
NCT01135888
First received: June 2, 2010
Last updated: July 25, 2011
Last verified: July 2011
  Purpose

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.


Condition Intervention
X-Linked Hypohidrotic Ectodermal Dysplasia
Exercise-induced Rise of Body Temperature
Device: Skin cooling devices

Study Type: Observational
Study Design: Observational Model: Case Control
Time Perspective: Prospective
Official Title: Short Term Effects and Risks of Physical Exercise in Subjects With Hypohidrotic Ectodermal Dysplasia (Christ-Siemens-Touraine Syndrome)

Resource links provided by NLM:


Further study details as provided by University Hospital Erlangen:

Biospecimen Retention:   Samples Without DNA

Serum


Enrollment: 28
Study Start Date: April 2009
Study Completion Date: February 2010
Primary Completion Date: February 2010 (Final data collection date for primary outcome measure)
Groups/Cohorts Assigned Interventions
HED children
HED adolescents Device: Skin cooling devices
Cooling vest and cooling bandana
Other Name: Cooline
Control children
Control adolescents Device: Skin cooling devices
Cooling vest and cooling bandana
Other Name: Cooline

  Eligibility

Ages Eligible for Study:   7 Years to 18 Years
Genders Eligible for Study:   Male
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population

subjects with X-chromosomally inherited hypohidrotic ectodermal dysplasia (HED) and age-matched controls

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
  Contacts and Locations
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Please refer to this study by its ClinicalTrials.gov identifier: NCT01135888

Locations
Germany
University Hospital Erlangen
Erlangen, Bavaria, Germany, 91054
Sponsors and Collaborators
University Hospital Erlangen
Pervormance GmbH
Investigators
Principal Investigator: Holm Schneider, MD University Hospital Erlangen
  More Information

Additional Information:
No publications provided by University Hospital Erlangen

Additional publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
Responsible Party: Holm Schneider, MD / Professor of Pediatrics, Head of the Division of Molecular Pediatrics, University Hospital Erlangen
ClinicalTrials.gov Identifier: NCT01135888     History of Changes
Other Study ID Numbers: ED09
Study First Received: June 2, 2010
Last Updated: July 25, 2011
Health Authority: Germany: Institutional Ethics Committee

Keywords provided by University Hospital Erlangen:
hypohidrotic ectodermal dysplasia
exertional hyperthermia
cooling device

Additional relevant MeSH terms:
Ectodermal Dysplasia
Ectodermal Dysplasia 1, Anhidrotic
Abnormalities, Multiple
Congenital Abnormalities
Skin Abnormalities
Skin Diseases, Genetic
Genetic Diseases, Inborn
Skin Diseases
Genetic Diseases, X-Linked

ClinicalTrials.gov processed this record on July 28, 2014