Pediatric noninvasive ventilation and sleep unit, AP-HP, Hôpital Necker Enfants-Malades, Paris, France.
Pediatric noninvasive ventilation and sleep unit, AP-HP, Hôpital Necker Enfants-Malades, Paris, France; Paris Descartes University, Paris, France.
J Crit Care. 2017 Dec;42:317-323. doi: 10.1016/j.jcrc.2016.04.003. Epub 2016 Apr 14.
The aim of the study was to analyze the diagnostic and therapeutic value of a polygraphy (PG) in infants hospitalized for unexplained respiratory failure or life-threatening events in the PICU.
The PG of 13 infants (4 girls), mean age 6.8±7.7months, were analyzed.
Eight infants were admitted for unexplained respiratory failure and 5 for life-threatening events. PG showed features suggestive of respiratory muscle weakness in 5 infants whose final diagnoses were nemaline rod myopathy (n=2), congenital myasthenia (n=2), and diaphragmatic dysfunction (n=1). Four of these patients were successfully treated with noninvasive ventilation (NIV). PG was suggestive of brainstem dysfunction in 4 infants; 2 were treated successfully with NIV and another with caffeine. PG showed obstructive sleep apnea in 3 infants; 2 were treated successfully with NIV and one patient was lost during follow up. A typical pattern of congenital central hypoventilation syndrome was observed in the last patient who was treated successfully with invasive ventilation. One patient with diaphragmatic dysfunction and one with brain stem dysfunction died.
PG may assist the diagnosis and guide the management of unexplained respiratory failure or life-threatening events in infants hospitalized in the PICU.
本研究旨在分析婴儿在 PICU 因不明原因呼吸衰竭或危及生命事件住院时进行多项生理记录仪检查(PG)的诊断和治疗价值。
对 13 名婴儿(4 名女性)的 PG 进行了分析,平均年龄为 6.8±7.7 个月。
8 名婴儿因不明原因呼吸衰竭入院,5 名婴儿因危及生命的事件入院。PG 显示 5 名婴儿有呼吸肌无力的特征,这些婴儿的最终诊断为杆状体肌病(n=2)、先天性肌无力(n=2)和膈肌功能障碍(n=1)。其中 4 名患者经无创通气(NIV)治疗成功。PG 提示 4 名婴儿有脑干功能障碍;2 名患者经 NIV 治疗成功,另 1 名患者在随访期间失访。第 3 名婴儿 PG 显示阻塞性睡眠呼吸暂停,2 名患者经 NIV 治疗成功,1 名患者在随访期间失访。最后一名患者患有先天性中枢性通气不足综合征,经有创通气治疗成功。1 名膈肌功能障碍患者和 1 名脑干功能障碍患者死亡。
PG 可辅助诊断并指导婴儿在 PICU 因不明原因呼吸衰竭或危及生命事件住院时的治疗。