Biological Clock Dysfunction in Optic Nerve Hypoplasia
关键词
抽象
描述
Objectives and Hypotheses. Our objectives are to define the scope and problems related to biological clock disorders in children with ONH and to develop effective treatments for this condition. Based on our observations, we hypothesize: (1) Daily rest-activity patterns and sleep will be abnormal in up to 50% of children with ONH. (2) It is possible to identify risk factors for abnormal circadian system function and sleep problems in ONH. (3) Nocturnal melatonin administration will improve abnormal sleep and activity patterns in children with ONH.
Design: These studies will involve collaborative efforts between Yale University and Dr. Mark Borchert of Childrens Hospital Los Angeles, who follows the largest population of children with ONH in the world. We will study children ages 2-10 years with documented ONH using standard criteria. Based on these criteria, we have more than 100 eligible patients.
To test our hypotheses, we will: (1) examine expressed rhythmicity in children with ONH. These studies will use actigraphy, sleep questionnaires, and assessment of melatonin secretory profiles. (2) We will correlate hypothalamic anatomical abnormalities and the degree endocrine dysfunction with sleep and expressed rhythmicity. (3) We will test if short-term administration of melatonin improves sleep-wake patterns in children with abnormally-expressed rhythmicity.
Potential Impact: Our preliminary data raise the possibility that children with ONH will have circadian system dysfunction resulting in abnormal rhythmicity and sleep. However, to date there have been no formal attempts to identify children with ONH who are at risk for such problems, nor have there been efforts aimed at developing potential treatments. The proposed prospective clinical study will represent an important attempt to identify a group of children with circadian system dysfunction.
At the completion of this study, we anticipate having determined risk factors for circadian system dysfunction in children with ONH. Insights gained from these studies should lead to the development of new approaches for treating circadian clock lesions in ONH, with the hope of improving the well-being of circadian system function in the boys and girls with this condition.
日期
| 最后验证: | 08/31/2020 |
| 首次提交: | 10/12/2008 |
| 提交的预估入学人数: | 01/18/2009 |
| 首次发布: | 01/20/2009 |
| 上次提交的更新: | 09/22/2020 |
| 最近更新发布: | 09/24/2020 |
| 实际学习开始日期: | 09/30/2008 |
| 预计主要完成日期: | 08/31/2011 |
| 预计完成日期: | 08/31/2011 |
状况或疾病
干预/治疗
Dietary Supplement: 1
Dietary Supplement: 2
相
手臂组
| 臂 | 干预/治疗 |
|---|---|
| Active Comparator: 1 Individuals with abnormal rhythmicity will be treated with melatonin to assess if sleep patterns are improved. | Dietary Supplement: 1 Oral administration before bed. We will test two doses (0.5 or 3.0 mg/m2. 6 week duration.) |
| Placebo Comparator: 2 | Dietary Supplement: 2 Oral administration before bed. 6 week duration. |
资格标准
| 有资格学习的年龄 | 2 Years 至 2 Years |
| 有资格学习的性别 | All |
| 接受健康志愿者 | 是 |
| 标准 | Inclusion Criteria: - Confirmed diagnosis of ONH (Diagnosis will be confirmed by ocular fundus photography.Disc-macula distance 0.35 or below. In eyes without ONH, the DD/DM ratio is greater than 0.3581.) - Ages 2-10 years - Ability to ambulate independently - Under care of endocrinologist if on pituitary hormone replacement therapy. Exclusion Criteria: - Non-ambulatory - Active malignancy - Cardio-respiratory disorder |
结果
主要结果指标
1. Assessment of rest-activity patterns [two years]
