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

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.

Completed

Looking for future studies?

Notify Me

Key information

Conditions

X-Linked Hypohidrotic Ectodermal Dysplasia Abnormalities, Multiple Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Ectodermal Dysplasia Ectodermal Dysplasia 1, Anhidrotic Exercise-induced Rise of Body Temperature Genetic Diseases, Inborn Genetic Diseases, X-Linked Skin Abnormalities Skin Diseases Skin Diseases, Genetic Skin and Connective Tissue Diseases

Age range

7 year–18 year

Sex eligibility

Male

Study type

Observational

Primary location

University Hospital Erlangen

Erlangen, Bavaria, 91054, Germany

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Skin cooling devices

Device

Cooling vest and cooling bandana

Other names: Cooline

Sponsors and collaborators

Lead sponsor

University Hospital Erlangen

Other

Collaborators

Pervormance GmbH

Registry information

Official study title

Short Term Effects and Risks of Physical Exercise in Subjects With Hypohidrotic Ectodermal Dysplasia (Christ-Siemens-Touraine Syndrome)

Important dates

Study start: 2009
Primary completion: 2010
Study completion: 2010
First posted: Jun 3, 2010
Registry last updated: Aug 19, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed