Journal of Diagnostics Concepts & Practice ›› 2022, Vol. 21 ›› Issue (06): 677-683.doi: 10.16150/j.1671-2870.2022.06.003
• Interpretation of guideline • Previous Articles Next Articles
Received:2022-11-11
Online:2022-12-25
Published:2023-04-23
CLC Number:
| 影响安全或功能的标志 | 严重 | 标准 |
|---|---|---|
| 生命安全情况 | 重症监护、气道支持、威胁安全的自主神经功能障碍 | 没有左边情况 |
| 行动能力 | 约束在床、可能受伤的运动障碍 | 没有约束在床 |
| 护理级别 | 全天候安全支持、一对一照护 | 不需要全天候的护理 |
| 精神症状 | 自杀想法、危险的冲动控制问题（受伤风险）、自伤行为 | 精神症状不会立刻影响生命安全 |
| 自理能力 | 不能自理、需要完全协助（如厕、穿衣和喂食） | 能够自理或部分自理 |
| 沟通能力 | 不能表达清楚 | 能沟通或表达需求 |
| 警觉性 | 对当前环境无反应 | 能大致注意当前环境的事件 |
| 癫痫发作 | 频繁需要抢救药物终止癫痫发作 | 癫痫发作不需要抢救药物干预 |
| 儿童版本的改良Rankin量表评分 | 4~5分 | 0~3分 |
| 评分分数 | 描述 | 注释 |
|---|---|---|
| 0 | 无症状 | 无 |
| 1 | 不影响儿童日常活动和玩耍及学习习惯的非致残性症状 | 玩耍、学习习惯包括上学或幼儿园 |
| 2 | 轻度症状，导致儿童日常活动和玩耍、学习习惯受到一些限制，但不影响与年龄相符的基本功能 | 基本功能为饮水、进食、穿衣、脱衣、梳头、洗涤、沐浴 症状：可能包括轻度的身体、认知和/或相关症状 |
| 3 | 中度症状，严重干扰日常活动和玩耍、学习习惯，或不能完全独立地实现与年龄相符基本功能 | 基本功能和症状同上 |
| 4 | 中重度的症状，明显不能独立地实现与年龄相符基本功能，但患者不需要持续的关注 | 基本功能和症状同上 |
| 5 | 严重残疾，完全依赖，需要持续的关注 | 约束在床，可能有意识受损、躁动、自主神经障碍和严重的运动障碍 |
| 6 | 死亡 | 无 |
| 免疫治疗 | 使用类型 | 使用剂量/方式 |
|---|---|---|
| 静注甲强龙 | 一线免疫治疗 | 每天20~30 mg/kg（最大1 g/d），3~5 d |
| 延长的一线免疫治疗 | 每天20~30 mg/kg（最大1 g/d），1~3 d，每月1次 | |
| 口服强的松 | 一线免疫治疗：静注皮质激素的替代方案 一线免疫治疗：静注皮质激素后减量 延长的一线免疫治疗 | 每天2 mg/kg（最大 60 mg/d），1 周，然后逐渐减量 |
| 口服地塞米松 | 一线免疫治疗：静注皮质激素的替代方案 | 每天20 mg/m2（分成2或3次, 最大12 mg 1 d 3次），3 d |
| 一线免疫治疗：静注皮质激素后减量 延长的一线免疫治疗 | 每天20 mg/m2（分成2或3次, 最大12 mg 1 d 3次），3 d，每3~4周 | |
| 治疗性血浆置换 | 一线免疫治疗 | 1个疗程通常是在7~10 d，进行5~7次单倍或双倍血浆体积置换 |
| 静注免疫球蛋白 | 一线免疫治疗 | 2 g/kg，分2~5 d给药 |
| 延长一线免疫治疗 | 1~2 g/kg，分1~2 d给药，每月1次 | |
| 静注利妥昔单抗 | 二线免疫治疗 | 以下剂量可接受：500~1 000 mg（体重<40 kg，500 mg；体重>40 kg，1 000 mg）2次，间隔2周给药，或375~750 mg/m2（最大1 g），2次间隔 2 周给药，或375 mg/m2（最大1 g）每周1次，连续4 周 |
| 维持免疫抑制（>6个月） | 当 CD19细胞出现再增殖（或第一个疗程后，约6个月），重复给利妥昔单抗（与上述剂量相同或根据当地建议减少剂量） | |
| 静注环磷酰胺 | 二线免疫治疗 | 500~1 000 mg/m2（最大 1 500 mg），每月1次冲击治疗，最长6个月 |
| 口服吗替麦考酚酯 | 维持免疫抑制（>6个月） | 每天600 mg /m2（最大1 g/次），每日2次 |
| 静注托珠单抗 | 升级二线免疫治疗 | 每月给予1次，12 mg/kg（<30 kg）或8 mg/kg（≥30 kg）（最大800 mg），超过6个月或更长时间（所需免疫抑制持续时间） |
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