Very Large Sizes in Adolescents: Interest of the Tibial and Femoral EPIphysiodesis in Case of Request for Treatment.
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 20
- 试验地点
- 4
- 主要终点
- Sitting height (cm)
研究概览
简要总结
The very large sizes can be very disabling and at the origin of a difficult psychological experience. Some families are in demand for a growth-curbing treatment. Currently, only hormonal treatments are offered in France but they are at the origin of many side effects. Northern European countries applied a surgical technique, deviated from the one used for a long time in leg asymmetries, to sterilize the proximal tibial and distal femoral growth cartilage, with most of the growth taking place at this level. The goal of this project is to be able to offer this technique to families in demand.
详细描述
By "very large size" we mean a size greater than +3 SD (standard deviation) on the updated French reference curves. Most of the very large sizes are constitutional.
The first step in the management of the paediatrician endocrinologist with very large body height is to collect the auxological parameters (family height, not just parental height, birth height, weight, height, head circumference, body mass index, height, sitting height) and plot the corresponding adolescent growth curves. The clinical stage (interview, clinical examination) will attempt to rule out a diagnosis other than a very large constitutional size: a recent statural acceleration related to a hormonal cause with specific medical treatment (hyperthyroidism or hyper androgeny or early puberty or acromegaly or more rarely estrogen deficiency by mutation of the CYP19A1 aromatase gene or by mutation of the ERα receptor) and will seek a syndrome in which the very large size is a sign among others.
Among the main syndromes are Klinefelter's syndrome (prevalence 1 person/1,200 or 1/600 boy births), Marfan's syndrome (about 1/6,000 births, prevalence 1 to 5 persons/ 10000), triple X syndrome (1 to 5 persons/10,000).
Additional examinations will be guided by diagnostic hypotheses; the very large constitutional diagnosis remains a diagnosis of elimination.
The very large constitutional size is defined as family, i.e. related to the size of at least 1 of the 2 parents or close family members of the parents.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Other
- 盲法
- None
入排标准
- 年龄范围
- 8 Years 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Adolescent (e) consultant for very large size with a demand for growth inhibition
- •Very large idiopathic or secondary height without any other etiological treatment possible
- •Tanner score ≥ 3 for boys and ≥ 2 for girls
- •A0 ≤ 14 years for boys and ≤ 12.5 years for girls
- •A chronological age of 10 to 16 for boys and 8 to 14 for girls
- •Actual size from minimum 167.5 cm to 10 years old to at least 180 cm to 16 years old for boys and from minimum 160 cm to 8 years old to at least 174 cm to 14 years old for girls
- •Predicted height ≥ 198 cm for boys and ≥ ≥ 184 cm for girls (refer to chapter 6.6)
- •Radiological persistence of tibial and femoral tibial cartilage estimated residual growth ≥ 8 cm
- •Absence of psychological contraindication to a curbing treatment of growth
- •Agreement of the holders of the parental authority and the teenager for a surgery of epiphysiodesis type
- •Agreement of the holders of the parental authority and the teenager to take part in the study
- •Affiliated to a social security scheme
排除标准
- •Very large size related to a medically curable etiology (hypersecretion of growth hormone called acromegaly)
- •Refusal of the holders of the parental authority or the teenager for a surgery of the epiphysiodesis type
- •Refusal of the holders of the parental authority or the teenager to take part in the study
- •Medical or psychological contraindication to surgery.
结局指标
主要结局
Sitting height (cm)
时间窗: 1 month
Sitting height and span before and after surgery.
Number of orthopedic adverse events
时间窗: 1 month
Number of orthopedic adverse events following short-term and long-term clinical and radiological (EOS) surgery.
Number of surgery adverse events
时间窗: 1 month
Number of adverse events following surgery.
Final size (cm)
时间窗: 5 years
Final size standing compared to the predicted size.
Satisfaction of patient and parents
时间窗: 5 years
Adolescent and parental satisfaction with surgical management (verbatim analysis)
次要结局
未报告次要终点