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

University Hospital Erlangen28 enrolled

Overview

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

Enrollment

Conditions

X-Linked Hypohidrotic Ectodermal Dysplasia Exercise-induced Rise of Body Temperature

Interventions

Skin cooling devices

Eligibility

Sex: MALEMin age: 7 YearsMax age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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

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

Overview

Conditions

Interventions

Eligibility

Locations (1)