Epidermolysis Bullosa Clinical Trial
Official title:
The State of Sexual Development in Children With Inherited Epidermolysis Bullosa
Verified date | July 2021 |
Source | National Medical Research Center for Children's Health, Russian Federation |
Contact | n/a |
Is FDA regulated | No |
Health authority | |
Study type | Observational |
The aim of the study is to determine the state of sexual development in patients with inherited epidermolysis bullosa; the study is planned to include boys and girls aged 8 to 18 years with a diagnosis of epidermolysis bullosa simplex, junctional epidermolysis bullosa, Kindler syndrome.
Status | Active, not recruiting |
Enrollment | 50 |
Est. completion date | June 1, 2023 |
Est. primary completion date | December 1, 2021 |
Accepts healthy volunteers | No |
Gender | All |
Age group | 8 Years to 18 Years |
Eligibility | Inclusion Criteria: 1. Male and female patients from 8 to 18 years of age with an established diagnosis of epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa, Kindler syndrome. Exclusion Criteria: 1. Hypergonadotropic hypogonadism; 2. previous treatment with sex steroids or gonadotropins; 3. the presence of endocrine diseases (hypothyroidism, hypercorticism, hyperprolactinemia, diabetes mellitus); 4. the presence of chronic somatic diseases that are not complications of inherited epidermolysis bullosa (bronchopulmonary diseases, cardiovascular diseases, diseases of the urinary system, diseases of the gastrointestinal tract). |
Country | Name | City | State |
---|---|---|---|
Russian Federation | National Medical Research Center for Children's Health | Moscow |
Lead Sponsor | Collaborator |
---|---|
National Medical Research Center for Children's Health, Russian Federation |
Russian Federation,
Type | Measure | Description | Time frame | Safety issue |
---|---|---|---|---|
Primary | Assessment of the state of sexual development of children with various types of inherited epidermolysis bullosa using the Tanner scale | The endocrinologist's assessment of pubertal development in boys and girls using the Tanner Scale | 1 day | |
Primary | Determination of the state of functional activity of the sex glands in children with various types of inherited epidermplysis bullosa by the method of biochemical study of the level of sex hormones | Determination of serum levels of sex hormones in patients (luteinizing hormone, follicle-stimulating hormone, estradiol, total testosterone). | 1 day | |
Primary | Determination of additional hormonal indicators | Determination of blood levels of adrenocorticotropic hormone, cortisol, dehydroepiandrosterone-sulfate, 17-hydroxyprogesterone, prolactin, insulin, thyrotropic hormone, triiodothyronine and thyroxine. | 1 day | |
Primary | Functional development and morphometric parameters of the genital glands in children with various types of inherited epidermolysis bullosa by ultrasound diagnostics | Ultrasound examination of pelvic organs in girls and scrotal organs in boys to determine morphometric indices. | 1 day | |
Primary | The state of bone age in children with various types of inherited epidermolysis bullosa using X-ray examination of the hands | Determination of bone and chronological age correspondence by radiography of the left wrist joint. | 1 day | |
Primary | Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the Pearce-Harris scale | Determination of the relationship between self-esteem and gender development in children with different types of hereditary epidermolysis bullosa using the Peirce-Harris scale | 1 day | |
Primary | Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the personality drawing technique of K. Machover | Determination of the relationship between self-esteem and gender development in children with different types of hereditary epidermolysis bullosa using the personality drawing technique of K. Machover | 1 day | |
Primary | Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the T. Aachenbach questionnaire | Determination of the relationship between self-esteem and gender development in children with different types of hereditary epidermolysis bullosa using the T. Aachenbach questionnaire | 1 day | |
Primary | Determination of the level of intellectual development in children with various types of inherited epidermolysis bullosa using D. Wexler's test | Determination of the level of intellectual development and its components (verbal and non-verbal intelligence) in children with various types of inherited epidermolysis bullosa | 1 day | |
Primary | Determination of the severity of the course of the disease in children with various types of inherited epidermolysis bullosa using EBDASI | Determination of the severity of the course of the disease in children with various types of inherited epidermolysis bullosa using Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) | 1 day |
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