Reppucci Diana, Medin Debra, Al-Saleh Suhail, Smith Mary Jane, Barter Jill, Amin Reshma
Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada M5G IX8; University of Toronto, Toronto, ON, Canada.
Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada M5G IX8.
Can Respir J. 2016;2016:9712579. doi: 10.1155/2016/9712579. Epub 2016 Mar 29.
We present a case of a 15-month-old boy with Cornelia de Lange Syndrome (NIPBL gene mutation). On a PSG, central sleep apnea (central apnea-hypopnea index of 19/hour) and nocturnal hypoventilation (transcutaneous CO2 > 50 mmHg for 53% of the night) were found. A positive pressure initiation study was aborted because the patient developed a serious adverse reaction. The differential diagnosis included a skin fragility condition versus an allergic contact dermatitis to the interface; this could be from the povidone-iodine solution used to clean the NiPPV interface or from the plastic of the interface itself. A skin biopsy was performed which was normal. The reaction was likely secondary to an allergic contact dermatitis from the povidone-iodine solution used to clean the NiPPV interface. The patient is currently tolerating NiPPV.
我们报告一例15个月大患有科妮莉亚·德朗热综合征（NIPBL基因突变）的男孩。在多导睡眠图监测中，发现有中枢性睡眠呼吸暂停（中枢性呼吸暂停低通气指数为19次/小时）和夜间通气不足（经皮二氧化碳分压>50 mmHg持续占夜间时长的53%）。由于患者出现严重不良反应，正压通气起始研究中止。鉴别诊断包括皮肤脆性状况与对接口处的过敏性接触性皮炎；这可能源于用于清洁无创正压通气（NiPPV）接口的聚维酮碘溶液，或源于接口本身的塑料材质。进行了皮肤活检，结果正常。该反应可能继发于用于清洁NiPPV接口的聚维酮碘溶液引起的过敏性接触性皮炎。患者目前耐受NiPPV。
