Clinical Trial of Ketogenic Diet in the Treatment of Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-like Episodes(MELAS)
试验速览
- 阶段
- 不适用
- 状态
- 尚未招募
- 发起方
- 入组人数
- 100
- 试验地点
- 1
- 主要终点
- Change from Baseline Diagnostic Examination in 1 Year
研究概览
简要总结
The goal of this clinical trial is to evaluate the effectiveness of ketogenic diet in patients with MELAS syndrome. The main questions it aims to answer are:
Clarify the curative effects of ketogenic diet in the treatment of MELAS disease.
Prevent the aggravation of MELAS disease, and improve the quality of life of patients.
Provide reliable evidence-based medical basis for the clinical application of ketogenic diet in the treatment of MELAS syndrome patients.
The clinical data of the participants treated with ketogenic diet will be collected, including the completion of ketogenic diet and clinical data at the start of treatment and after 1 month, 3 months, 6 months and 12 months
详细描述
Ketogenic diet initiation and follow-up:
- Before starting ketogenic diet treatment, patients with abnormal indexes such as liver, kidney, lipid, humoral immunity, hematuria, routine biochemical, electrolyte, trace elements and bone metabolism should be excluded, energy and nutrients should be calculated according to their height and weight, and patients will be taught with ketogenic diet treatment education
- Patients were initiated ketogenic diet after a short period of fasting (fasting no more than 48 hours). After treatment was initiated, 13 of the calculated target energy was given on the first day, 23 of the target energy was given on the second day, and the target amount was reached on the third day. Nutritionists made appropriate adjustments to the target energy according to the level of blood keto measured by patients
- Weekly follow-up via telemedicine system or social media, outpatient follow-up at the initiation of ketogenic therapy and at 1 month, 3 months, 6 months, and 12 months respectively. Outpatient follow-up was done in a joint clinic with a dietitian and a neurologist to evaluate the effects and side effects of ketogenic therapy on a compliant diet and related clinical examinations
Clinical data collection:
- Demographic information and Medical history collection Gender, age, education level age of onset, genetic test, drug use, past history, personal history, family history
- Clinical symptom collection According to the Newcastle Mitochondrial Disease Adult Scale(NMDAS), including migraine headaches, seizures, stroke-like episodes and etc.
- Biochemical indicators Blood glucose, keto, lactate and pyruvate monitoring, dietary status, dietary ratio changes, energy changes, weight changes
- Cognitive function assessment Cognitive function was assessed using Fepsy software
- Diagnostic Examination Cranial MRI, scalp EEG
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients who meet the diagnostic criteria of MELAS and have received MELAS standard therapy but are not satisfied with the therapeutic effect, and voluntarily underwent ketogenic therapy
排除标准
- •Diseases with porphyria and disturbances in fatty acid transport and oxidation, severe electrolyte metabolism abnormalities, severe hemodynamic instability, acute respiratory infections, uncontrolled systemic infections, severe liver and renal failure, cholesterolemia (>300mgdl), abnormal coagulation, acute pancreatitis, eating disorders, ketogenic diet intolerance, significant weight loss, poor compliance
结局指标
主要结局
Change from Baseline Diagnostic Examination in 1 Year
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Cranial MRI（multimodal） Scalp EEG to evaluate posterior head α rhythm, discharge index, interictal epileptic discharges
Change from Baseline Clinical Symptom Score in 1 Year
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
According to the newcastle mitochondrial disease adult scale(NMDAS), including migraine headaches, seizures, stroke-like episodes and etc.
Hamilton Depression Scale(HAMD
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Change from Baseline Cognitive Function in 1 Year . Cognitive function was assessed using "Fepsy" software
Hamilton Anxiety Scale (HAMA)
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Change from Baseline Cognitive Function in 1 Year. Cognitive function was assessed using "Fepsy" software
Wechsler Intelligence Scale(WISC)
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Change from Baseline Cognitive Function in 1 Year . Cognitive function was assessed using "Fepsy" software
Mini-mental State Examination(MMSE)
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Change from Baseline Cognitive Function in 1 Year . Cognitive function was assessed using "Fepsy" software
Change from Baseline Biochemical indicators in 1 Year
时间窗: Baseline, 1 month, 3 months, 6 months and 12 months
Blood glucose
次要结局
- Demographic Information and Medical History Collection(Baseline)