The State of Sexual Development in Children With Inherited Epidermolysis Bullosa
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 50
- 试验地点
- 1
- 主要终点
- 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
研究概览
简要总结
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.
详细描述
Determination of sexual development in children with various types of inherited epidermolysis bullosa using clinical, laboratory and instrumental methods for diagnosing the condition.
Phenotyping of patients with inherited epidermolysis bullosa, taking into account their physical and sexual development, indicators of self-attitude and socialization.
Development of algorithms for individual care for patients with inherited epidermolysis bullosa, including an assessment of the psychological state at different age stages (primary school and adolescence), the volume and form of psychological and pedagogical assistance in complex rehabilitation.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Prospective
入排标准
- 年龄范围
- 8 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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.
排除标准
- •Hypergonadotropic hypogonadism;
- •previous treatment with sex steroids or gonadotropins;
- •the presence of endocrine diseases (hypothyroidism, hypercorticism, hyperprolactinemia, diabetes mellitus);
- •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).
结局指标
主要结局
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
时间窗: 1 day
Determination of serum levels of sex hormones in patients (luteinizing hormone, follicle-stimulating hormone, estradiol, total testosterone).
Assessment of the state of sexual development of children with various types of inherited epidermolysis bullosa using the Tanner scale
时间窗: 1 day
The endocrinologist's assessment of pubertal development in boys and girls using the Tanner Scale
Determination of additional hormonal indicators
时间窗: 1 day
Determination of blood levels of adrenocorticotropic hormone, cortisol, dehydroepiandrosterone-sulfate, 17-hydroxyprogesterone, prolactin, insulin, thyrotropic hormone, triiodothyronine and thyroxine.
Functional development and morphometric parameters of the genital glands in children with various types of inherited epidermolysis bullosa by ultrasound diagnostics
时间窗: 1 day
Ultrasound examination of pelvic organs in girls and scrotal organs in boys to determine morphometric indices.
The state of bone age in children with various types of inherited epidermolysis bullosa using X-ray examination of the hands
时间窗: 1 day
Determination of bone and chronological age correspondence by radiography of the left wrist joint.
Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the personality drawing technique of K. Machover
时间窗: 1 day
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
Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the Pearce-Harris scale
时间窗: 1 day
Determination of the relationship between self-esteem and gender development in children with different types of hereditary epidermolysis bullosa using the Peirce-Harris scale
Determination of the psychological state of children with various types of inherited epidermolysis bullosa using the T. Aachenbach questionnaire
时间窗: 1 day
Determination of the relationship between self-esteem and gender development in children with different types of hereditary epidermolysis bullosa using the T. Aachenbach questionnaire
Determination of the severity of the course of the disease in children with various types of inherited epidermolysis bullosa using EBDASI
时间窗: 1 day
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)
Determination of the level of intellectual development in children with various types of inherited epidermolysis bullosa using D. Wexler's test
时间窗: 1 day
Determination of the level of intellectual development and its components (verbal and non-verbal intelligence) in children with various types of inherited epidermolysis bullosa
次要结局
未报告次要终点