Laaban J P, D'Orbcastel O R
Poumon Coeur. 1980 Sep-Oct;36(5):335-43.
After a brief recall of etiology and anatomo-pathology, the authors tried to sum up the knowledge concerning the physio-pathology of kyphoscolioses. First the respiratory repercussion of the disease was studied with its classical spirometric data, the abnormalities of gas exchanges (alveolar hypoventilation, decreased diffusion capacity, inhomogeneous distribution of the ratio ventilation/perfusion which plays a large part in these abnormalities), altered ventilatory mechanics and the response to CO2. Then they studied the anatomical and functional repercussion on the lesser circulation which is at the origin of the chronic pulmonary heart. When present, correlations with the clinic are underlined. In a second part, the clinical evolution of the respiratory insufficiency in kyphoscolioses is approached. The brutality of respiratory decompensations and the very poor prognosis linked to the beginning of a chronic pulmonary heart are underlined. Finally, the therapeutical side of the disease is approached. The classical medical and surgical means are recalled and the paper ends on the changing of the prognosis, which became possible because of the techniques of ventilation at home, currently in expansion.
在简要回顾病因学和解剖病理学之后,作者试图总结有关脊柱侧凸生理病理学的知识。首先,通过其经典的肺功能测定数据、气体交换异常(肺泡通气不足、弥散能力下降、通气/灌注比分布不均,这在这些异常中起很大作用)、通气力学改变和对二氧化碳的反应来研究该疾病的呼吸影响。然后,他们研究了对小循环的解剖和功能影响,而小循环是慢性肺源性心脏病的起源。当存在相关性时,会强调与临床的联系。在第二部分中,探讨了脊柱侧凸呼吸功能不全的临床演变。强调了呼吸失代偿的突然性以及与慢性肺源性心脏病开始相关的极差预后。最后,探讨了该疾病的治疗方面。回顾了经典的医学和外科手段,文章最后论述了由于目前正在推广的家庭通气技术,预后得以改变。