当前位置: X-MOL 学术Sleep › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
1236 A Case of Systemic Amyloidosis and Sleep-Related Hypoventilation
Sleep ( IF 5.6 ) Pub Date :  , DOI: 10.1093/sleep/zsaa056.1230
Lauren M Castner 1 , Mark D Garwood 1
Affiliation  

Abstract
Introduction
Amyloidoses are a group of systemic diseases characterized by misfolded protein fragment deposition within the organs, including the heart, kidney, liver, gastrointestinal tract, nervous system, pulmonary system, and soft tissues1. Obstructive and central sleep apnea are known to occur frequently in those with cardiac amyloidosis. This case discusses a patient with systemic amyloidosis and chronic hypercarbic, hypoxic respiratory failure.
Report of Case
A 66 year old female with a history of systemic amyloidosis, non-ischemic cardiomyopathy, hypertension, and obstructive sleep apnea was admitted for acute on chronic heart failure. Despite intravenous diuresis, she remained hypoxemic, requiring 1 liter per minute of oxygen.She was found to have bilaterally reduced diaphragmatic excursion and a restrictive ventilatory defect on spirometry. She had a preceding history of chronic carbon dioxide retention with elevated CO2 levels for greater than a year (52-74 mmHg).Sleep medicine was consulted to assist in evaluation of the patient’s obstructive sleep apnea and hypoxic, hypercarbic respiratory failure. Baseline polysomnogram revealed sleep related hypoventilation with transcutaneous CO2 (TCO2) ranging between 77-86 mmHg without clear obstructive sleep apnea. A bilevel positive airway pressure (BPAP) titration was then performed (TCO2 54-69 mmHg) and while the patient’s obstructive sleep apnea was well treated, sleep-related hypoventilation and central apneas persisted. Average volume assured pressure support (AVAPS) was initiated for management of sleep related hypoventilation. In follow up, the patient is feeling well, off oxygen, with daytime TCO2 38 mmHg.
Conclusion
This case demonstrates a rare complication of systemic amyloidosis in the setting of respiratory failure attributed to amyloid infiltration of the diaphragm. In the few previously reported cases of neuromuscular respiratory failure in systemic amyloidosis there is rapid progression and high mortality3, which highlights the importance of assessing for sleep disordered breathing and additional causes of respiratory failure in a patient with a complex systemic disease.


中文翻译:

1236例系统性淀粉样变性病和与睡眠有关的换气不足

摘要
介绍
淀粉样蛋白是一组全身性疾病,其特征是蛋白质片段在器官(包括心脏,肾脏,肝脏,胃肠道,神经系统,肺系统和软组织)中的错误折叠沉积。已知患有淀粉样淀粉样变性的患者经常发生阻塞性和中枢性睡眠呼吸暂停。该病例讨论了患有系统性淀粉样变性病和慢性高碳,低氧性呼吸衰竭的患者。
案件报告
一位66岁的女性,患有系统性淀粉样变性病,非缺血性心肌病,高血压和阻塞性睡眠呼吸暂停,因慢性心力衰竭而入院。尽管有静脉利尿作用,但她仍处于低氧状态,每分钟需要1升氧气,发现她的双侧diaphragm肌偏移减少,肺活量测定仪出现通气受限。她先前有慢性二氧化碳滞留病史,且二氧化碳水平升高超过一年(52-74 mmHg)。曾咨询过睡眠药物以帮助评估患者的阻塞性睡眠呼吸暂停和低氧,高碳酸血症性呼吸衰竭。基线多导睡眠图显示与睡眠有关的通气不足,经皮CO2(TCO2)介于77-86 mmHg之间,而没有明显的阻塞性睡眠呼吸暂停。然后进行双水平气道正压(BPAP)滴定(TCO2 54-69 mmHg),尽管患者的阻塞性睡眠呼吸暂停得到了很好的治疗,但与呼吸有关的通气不足和中枢性呼吸暂停持续存在。启动平均体积保证压力支持(AVAPS)来管理与睡眠有关的通气不足。在随访中,白天的TCO2为38 mmHg,患者没有氧气,感觉良好。
结论
这种情况表明,由于the肌淀粉样蛋白浸润导致的呼吸衰竭的情况下,系统性淀粉样变性病的罕见并发症。在先前报道的少数几例系统性淀粉样变性神经肌肉呼吸衰竭病例中,病情进展迅速,死亡率高3,这突显了评估患有复杂系统疾病的患者的睡眠呼吸障碍和其他呼吸衰竭原因的重要性。
更新日期:2020-05-27
down
wechat
bug