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

NCT01135888 | Completed DMC

• 1 other identifier: ED09
• Study Type: observational
• Target: 28
• Locations: 1 country
• Sites: 1

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.

Conditions

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

Keywords

hypohidrotic ectodermal dysplasia; exertional hyperthermia; cooling device

Study Arms (4)

HED children

HED adolescents

Device: Skin cooling devices

Control children

Control adolescents

Device: Skin cooling devices

Interventions

Skin cooling devices DEVICE

Cooling vest and cooling bandana

Also known as: Cooline

Control adolescents; HED adolescents

Eligibility Criteria

• Age: 7 Years - 18 Years
• Sex: male
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

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

You may qualify if:

• for patients: hypohidrotic ectodermal dysplasia caused by EDA gene mutations
• regular fluid intake prior to the investigation
• written informed consent

You may not qualify if:

• 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

Sponsors & Collaborators

• University Hospital Erlangen: lead
• Pervormance GmbH: collaborator

Study Sites (1)

University Hospital Erlangen

Erlangen, Bavaria, 91054, Germany

Location

Related Publications (1)

• Hammersen JE, Neukam V, Nusken KD, Schneider H. Systematic evaluation of exertional hyperthermia in children and adolescents with hypohidrotic ectodermal dysplasia: an observational study. Pediatr Res. 2011 Sep;70(3):297-301. doi: 10.1203/PDR.0b013e318227503b.

  PMID: 21646941 DERIVED

Biospecimen

Retention: SAMPLES WITHOUT DNA

Serum

MeSH Terms

Conditions

Ectodermal Dysplasia 1, Anhidrotic

Condition Hierarchy (Ancestors)

Ectodermal Dysplasia; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin Abnormalities; Genetic Diseases, X-Linked; Genetic Diseases, Inborn; Skin Diseases, Genetic; Skin Diseases; Skin and Connective Tissue Diseases

Study Officials

• Holm Schneider, MD

  University Hospital Erlangen

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor of Pediatrics

Study Record Dates

• First Submitted: June 2, 2010
• First Posted: June 3, 2010
• Study Start: April 1, 2009
• Primary Completion: February 1, 2010
• Study Completion: February 1, 2010
• Last Updated: August 19, 2021

Record last verified: 2021-08

Locations

DE (1)