Malignant Hyperthermia in Children: An Analysis of the North American Malignant Hyperthermia Registry

Malignant Hyperthermia in Children: An Analysis of the North American Malignant Hyperthermia Registry
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DOI:
10.1213/ane.0b013e3182a8fad0
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发表时间:
2014-02-01
影响因子:
5.7
通讯作者:
Litman, Ronald S.
Litman, Ronald S.
中科院分区:
医学2区
文献类型:
--
作者:
Nelson, Priscilla;Litman, Ronald S.

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背景:儿童恶性高热(MH)的临床特征尚未阐明。在这项研究中,我们使用北美恶性高热登记,以确定不同年龄组的儿童急性MH的临床特征的差异。我们假设在临床表现、临床过程和结果方面存在差异,这些差异与年龄相关。第二个目的是确定与儿科MH.METHODS:我们进行了一项回顾性研究,北美恶性高热登记,以确定儿科受试者(最多,包括18岁)的MH临床分级评分为35分或以上,表明非常可能或几乎肯定的MH。根据年龄比较3个队列的术前患者特征、围麻醉期因素和结局数据:0 - 24个月、25个月-12岁和13 - 18岁。结果:我们分析了264份记录:最年轻年龄组35份,中年组163份,老年组66份。根据家族史或体格检查,没有任何迹象表明存在任何诱发MH的危险因素。窦性心动过速、高碳酸血症和体温快速升高是急性MH最常见的体征(分别观察到73.1%、68.6%和48.5%),并且在最年长的年龄组中更常见。最高温度和钾峰值较高,在最老的年龄组。咬肌痉挛在中年人群中更常见。最年轻的年龄组更有可能出现皮肤斑点,并且出现肌肉僵硬的可能性约为一半。最年轻的年龄组也表现出显着较高的峰值乳酸水平和较低的峰值肌酸激酶值。各年龄组的治疗相似。有10例MH相关死亡,6例在中年组,4例在老年组。14.4%的受试者在初始治疗后症状复发,各年龄组之间无差异。其中两名受试者,1名在中年组和1名在最老的年龄组,死亡后复发events.CONCLUSIONS:有急性MH的临床特征不同年龄组儿童之间的差异。年龄较大的受试者表现出更高的体温和更高的钾水平,而最年轻的受试者有更高水平的代谢性酸中毒。在发展MH之前,每个年龄组的大多数儿童表型正常。
BACKGROUND: Clinical characteristics of malignant hyperthermia (MH) in pediatric patients have not been elucidated. In this study, we used the North American Malignant Hyperthermia Registry to determine differences in clinical characteristics of acute MH across pediatric age groups. We hypothesized that there are differences in clinical presentation, clinical course, and outcomes, which correlate with age. A secondary aim was to determine the types of preexisting medical conditions associated with pediatric MH.METHODS: We performed a retrospective review of the North American Malignant Hyperthermia Registry to identify pediatric subjects (up to and including 18 years) with an MH clinical grading score at or above 35 indicating very likely or almost certain MH. Preoperative patient characteristics, perianesthetic factors, and outcome data were compared for 3 cohorts based on age: 0 to 24 months, 25 months to 12 years, and 13 to 18 years. We used statistical analysis to determine differences among the groups.RESULTS: We analyzed 264 records: 35 in the youngest age group, 163 in the middle age group, and 66 in the oldest group. There was no indication of any predisposing risk factors for MH based on family history or physical examination. Sinus tachycardia, hypercarbia, and rapid temperature increase were the most common signs of acute MH (observed in 73.1%, 68.6%, and 48.5%, respectively) and were more common in the oldest age cohort. Higher maximum temperatures and higher peak potassium values were seen in the oldest age cohort. Masseter spasm was more common in the middle age cohort. The youngest age cohort was more likely to develop skin mottling and was approximately half as likely to develop muscle rigidity. The youngest age group also demonstrated significantly higher peak lactic acid levels and lower peak creatine kinase values. Treatments were similar across age cohorts. There were 10 MH-associated deaths, 6 in the middle age group and 4 in the oldest age group. Recrudescence of symptoms after initial treatment occurred in 14.4% of subjects, with no difference across age cohorts. Two of these subjects, 1 in the middle age group and 1 in the oldest age group, died after the recrudescence event.CONCLUSIONS: There are differences in clinical characteristics of acute MH among different age cohorts in childhood. Older subjects demonstrated higher body temperatures and higher potassium levels, and the youngest subjects had greater levels of metabolic acidosis. Most children in each age group were phenotypically normal before developing MH.