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先天性大叶性肺气肿婴儿期肺叶切除术后的肺生长和气道功能

Lung growth and airway function after lobectomy in infancy for congenital lobar emphysema.

作者信息

McBride J T, Wohl M E, Strieder D J, Jackson A C, Morton J R, Zwerdling R G, Griscom N T, Treves S, Williams A J, Schuster S

出版信息

J Clin Invest. 1980 Nov;66(5):962-70. doi: 10.1172/JCI109965.

Abstract

To characterize the outcome of lobectomy in infancy and the low expiratory flows which persist after lobectomy for congenital lobar emphysema, 15 subjects with this history were studied at age 8-30 yr. Total lung capacity was normal in all, but higher values (P < 0.05) were observed in nine subjects with upper lobectomy than in five subjects with right middle lobectomy. Ratio of residual volume to total lung capacity was correlated (P < 0.05) with the amount of lung missing as estimated from normal relative weights of the respective lobes. Xe(133) radiospirometry in eight subjects showed that the operated and unoperated sides had nearly equal volumes at total lung capacity, but that the operated side was larger than the unoperated side at residual volume. Perfusion was equally distributed between the two sides. Similar findings were detected radiographically in four other subjects. Forced expiratory volume in 1 s and maximal midexpiratory flow rate averaged 72 and 45% of predicted, respectively. Low values of specific airway conductance and normal density dependence of maximal flows in 12 subjects suggested that obstruction was not limited to peripheral airways. Pathologic observations at the time of surgery and morphometry of the resected lobes were not correlated with any test of pulmonary function. These data show that lung volume can be completely recovered after lobectomy for congenital lobar emphysema in infancy. The volume increase occurs on the operated side, and probably represents tissue growth rather than simple distension. The response to resection is influenced by the particular lobe resected and may be associated with decreased lung recoil near residual volume. Low expiratory flows in these subjects could be explained by several mechanisms, among which a disproportion between airway and parenchymal growth in infancy (dysanaptic growth) is most compatible with our data.

摘要

为了描述婴儿期肺叶切除术的结果以及先天性肺叶气肿肺叶切除术后持续存在的低呼气流量,对15例有此病史的患者在8至30岁时进行了研究。所有患者的肺总量均正常,但9例上叶切除患者的值高于5例右中叶切除患者(P<0.05)。残气量与肺总量的比值与根据各肺叶正常相对重量估算的缺失肺量相关(P<0.05)。8例患者的Xe(133)放射性肺通气测定显示,在肺总量时,手术侧和未手术侧的容积几乎相等,但在残气量时,手术侧大于未手术侧。两侧的灌注分布均匀。在另外4例患者中,影像学检查也发现了类似的结果。第1秒用力呼气量和最大呼气中期流速分别平均为预测值的72%和45%。12例患者的比气道传导率较低,最大流速的密度依赖性正常,提示阻塞不仅限于外周气道。手术时的病理观察和切除肺叶的形态测量与任何肺功能测试均无相关性。这些数据表明,婴儿期先天性肺叶气肿肺叶切除术后肺容积可完全恢复。容积增加发生在手术侧,可能代表组织生长而非单纯的扩张。对切除的反应受切除的特定肺叶影响,可能与残气量附近肺弹性回缩力降低有关。这些患者的低呼气流量可由多种机制解释,其中婴儿期气道与实质生长不均衡(发育异常生长)与我们的数据最为相符。

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