Yates K, Festa M, Gillis J, Waters K, North K
Department of Paediatric Intensive Care, The Children's Hospital at Westmead, Sydney, Australia.
Arch Dis Child. 2004 Feb;89(2):170-5. doi: 10.1136/adc.2002.019562.
To determine the outcome of children with neuromuscular disease (NMD) following admission to a tertiary referral paediatric intensive care (PICU).
All children with chronic NMD whose first PICU admission was between July 1986 and June 2001 were followed up from their first PICU admission to time of study. The outcomes recorded were death in or outside of PICU, duration of PICU admission, artificial ventilation during admission and following discharge from PICU, and readmission to PICU.
Over 15 years, 28 children were admitted on 69 occasions. Sixteen (57%) children had more than one admission. The median duration of PICU admission was 4 days (range 0.5-42). Twenty three per cent of unplanned admissions resulted in the commencement of respiratory support that was continued after discharge from the PICU. Severity of functional impairment was not associated with longer duration of stay or higher PRISM scores. Ten children (36%) died, with four (14%) deaths in the PICU. A higher proportion of children with severe limitation of function were among children that died compared to survivors.
Most children with NMD admitted to the PICU recover and are discharged without the need for prolonged invasive ventilation. However, in this group of children, the use of non-invasive home based ventilation is common and they are likely to require further PICU admission.
确定患有神经肌肉疾病（NMD）的儿童入住三级转诊儿科重症监护病房（PICU）后的治疗结果。
对1986年7月至2001年6月首次入住PICU的所有慢性NMD患儿，从其首次入住PICU开始进行随访直至研究时。记录的结果包括在PICU内或PICU外死亡、PICU住院时间、住院期间及从PICU出院后的人工通气情况，以及再次入住PICU的情况。
在15年期间，28名儿童共69次入住PICU。16名（57%）儿童不止一次入住。PICU住院时间的中位数为4天（范围0.5 - 42天）。23%的非计划入住导致开始呼吸支持，且在从PICU出院后仍持续。功能损害的严重程度与住院时间延长或更高的PRISM评分无关。10名儿童（36%）死亡，其中4名（14%）在PICU死亡。与存活者相比，功能严重受限的儿童在死亡儿童中所占比例更高。
大多数入住PICU的NMD患儿康复并出院，无需长时间进行有创通气。然而，在这组儿童中，使用基于家庭的无创通气很常见，且他们可能需要再次入住PICU。
