在整个儿科年龄段的普拉德-威利综合征患者的低通风
Catherine Chen1,2, Iulia Ioan3, Marine Thieux2,4,5
1Service de pneumologie pédiatrique, Hôpital Femme Mère Enfant, Hospices, Civils de Lyon, Bron, France.
Pediatric pulmonology
|January 5, 2024
概括
气囊低通风影响了35%的患有普拉德-威利综合征 (PWS) 的儿童,通常没有症状. 建议定期查,特别是在年龄和生长激素 (GH) 治疗时,以监测PWS患者的呼吸系统健康.
科学领域:
- 儿科肺病学 儿科肺病学
- 遗传学 是一个遗传学.
- 睡眠医学 睡眠医学
背景情况:
- 普拉德-威利综合征 (PWS) 是一种复杂的遗传疾病,对呼吸道并发症的数据有限.
- 缺少PWS患者的标准化呼吸系统随访,特别是在气管低通风方面.
研究的目的:
- 为了确定PWS儿童中膜低通风的患病率.
- 在这个人群中识别与膜低通风相关的潜在风险因素.
主要方法:
- 对患有PWS的儿童进行回顾性多睡眠图 (PSG) 研究 (2007-2021年).
- 纳入标准:使用皮肤 CO2 (PtcCO2) 或潮 CO2 (ETCO2) 的测量进行 PSG.
- 膜低通风被定义为pCO2 ≥50 mmHg 在总睡眠时间的≥2%或连续5分钟.
主要成果:
- 35% (20/57) 患有PWS的儿童表现出大气管低通风.
- 中位数pCO2max为49mmHg; 25%的低呼吸儿童没有症状.
- 较高的平均年龄和生长激素 (GH) 治疗与低通风相关.
结论:
- 气膜低通风是儿科PWS的一个重要问题,可能随着年龄和GH治疗而增加.
- 建议对所有PWS患者进行定期的氧度测量查,无论性别,BMI或呼吸暂停指数如何.
相关概念视频
Alterations in Respiration II
869
There are numerous types of normal and abnormal respiration. Based on ventilatory movements, breathing patterns are classified as regular, deep, or shallow. Examples include Biot's breathing, Cheyne-Stokes respiration, Kussmaul's breathing, hyperventilation, and hypoventilation. Each pattern is clinically significant and aids in evaluating patients.
In Biot's breathing, the respiratory rate and depth are irregular, alternating between periods of deep gasping and apnea. Common causes...
In Biot's breathing, the respiratory rate and depth are irregular, alternating between periods of deep gasping and apnea. Common causes...
869
Hyperpnea and Hyperventilation
1.1K
Hyperventilation refers to a higher-than-normal rate and depth of breathing, often associated with anxiety attacks. This excessive breathing surpasses the body's need to expel CO2, leading to a condition known as hypocapnia - an unusually low level of carbon dioxide in the blood. Hypocapnia can constrict cerebral blood vessels, reducing blood flow to the brain, which may result in dizziness or fainting. Early signs include tingling and muscle spasms in the hands and face, caused by falling...
1.1K
Pulmonary Cycle: Exhalation
1.6K
In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.6K
Acute Respiratory Failure-II
238
Type I Respiratory Failure, or hypoxemic respiratory failure, occurs when the partial pressure of oxygen (PaO2) in arterial blood falls below 60 mmHg while breathing room air without a corresponding increase in arterial carbon dioxide levels (PaCO2). This condition highlights a significant impairment in the lungs' capacity to oxygenate the blood.
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
238
Acute Respiratory Failure-III
187
Hypercapnic respiratory failure, also known as Type 2 or ventilatory respiratory failure, is a severe condition characterized by the body's inability to effectively remove carbon dioxide (CO2) from the bloodstream. It leads to an arterial CO2 pressure (PaCO2) exceeding 45 mmHg and a blood pH above 7.35. This situation indicates that the body's ventilatory demand, or the ventilation needed to maintain normal PaCO2 levels, surpasses its supply or the maximum gas flow achievable without...
187
Acute Respiratory Failure-IV
159
Respiratory failure can manifest suddenly or gradually, characterized by a rapid decline in PaO2 and a rapid rise in PaCO2. This situation indicates a severe respiratory problem that may quickly become a life-threatening emergency. One of the early signs of hypoxemic Acute Respiratory Failure (ARF) is a change in mental status due to the brain's sensitivity to oxygen levels and changes in acid-base balance. Symptoms such as restlessness, confusion, and agitation suggest inadequate oxygen...
159


