IRCCS Santa Maria Nascente, Fondazione Don Carlo Gnocchi, Milan.
IRCCS Santa Maria Nascente, Fondazione Don Carlo Gnocchi; Department of Psychology, Università Cattolica del Sacro Cuore, Milan.
Monaldi Arch Chest Dis. 2021 Aug 3;91(4). doi: 10.4081/monaldi.2021.1828.
The standard treatment for patients with neuromuscular respiratory failure is non-invasive ventilation (NIV) as non-invasive ventilation support-setting (NVS). NVS is administered through a nasal or face mask and/or mouthpiece with the potential to cause nasal ulcers, discomfort, and/or aesthetic issues, resulting in poor compliance. We reported the observation of a 45-year-old woman with limb-girdle muscular dystrophy (LGMD), secondary to Dysferlin deficiency, who was on NVS since 2017 for nocturnal hypoventilation. In 2018, despite nocturnal ventilation, due to weight gain and daytime hypoventilation, a nasal mask was introduced. We initiated daytime intermittent abdominal pressure ventilation (IAPV) to mitigate cosmetic problems, improving in pO2 and decreasing in pCO2 versus baseline (52>84 mmHg, 46>33 mmHg respectively) at 6 (85 mmHg, 42 mmHg) and 18 months (93 mmHg, 38 mmHg), respectively. IAPV was effective, safe, and well-tolerated in our patients who did not tolerate standard daytime NVS with the known interface.
对于神经肌肉性呼吸衰竭患者，标准治疗方法是非侵入性通气（NIV）作为非侵入性通气支持设置（NVS）。NVS 通过鼻罩或面罩和/或接口来实施，可能会导致鼻溃疡、不适和/或美容问题，从而导致顺应性差。我们报告了一例 45 岁女性的观察结果，她患有肢带型肌营养不良症（LGMD），继发于 Dysferlin 缺乏症，自 2017 年以来因夜间通气不足而接受 NVS 治疗。2018 年，尽管进行了夜间通气，但由于体重增加和白天通气不足，引入了鼻罩。我们开始日间间歇性腹部压力通气（IAPV）以减轻美容问题，与基线相比，pO2 升高，pCO2 降低（分别为 52>84mmHg，46>33mmHg），分别在 6（85mmHg，42mmHg）和 18 个月（93mmHg，38mmHg）时。IAPV 在我们的患者中是有效、安全且耐受良好的，他们不能耐受标准的日间 NVS 及其已知接口。
