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61.
Arterialized blood gases were analyzed in 143 patients with Duchenne muscular dystrophy (DMD) to assess the relationship between forced vital capacity (FVC) and hypercapnia. The majority of patients studied had PaCO2 values in the low or normal range. Only six older patients had hypercapnia (PaCO2 greater than or equal to 45 mm Hg), and all these patients had FVC values less than or equal to 40% predicted. We conclude that hypercapnic respiratory failure occurs as a late preterminal event in DMD.  相似文献   
62.

Objective

Progressive weakness of respiratory muscles remains one of the leading causes of death among patients with Duchenne muscular dystrophy (DMD). Currently, there are few pulmonary function data among Chinese DMD patients. This study was carried out to evaluate the sniff nasal inspiratory pressure (SNIP) change among a group of Chinese DMD patients, and compare it with the SNIP value of patients with neuromuscular disorders in other countries.

Methods

SNIP data were collected in three research groups that consists of 581 subjects: 125 DMD boys who have taken steroid (Age 5.0–13.3, DMD-steroid group), 145 DMD steroid-naive boys (Age 5.0–13.9, DMD-nonsteroid group), and 311 healthy controls (Age 5.0–14.0, Control group).

Results

The SNIP for DMD-nonsteroid group, DMD-steroid group and Control group were: 56.5 (±14.3)?cm H2O,66.4 (±15.5)?cm H2O and 78.9 (±21.5) respectively. The SNIP in the DMD-nonsteroid group became significantly different from that of the healthy controls since age 7.0–8.9. The significant difference of SNIP between DMD-steroid group and DMD-nonsteroid group at age 7.0–10.9. The peak value of SNIP in the DMD-nonsteroid group appeared at age 8.7, and decreased dramatically thereafter, while in DMD-steroid group and the Control group peaked at 10.2?years and 12.2?years respectively. There was a bit difference between SNIP in this group and that in previous researches which may be due to geographical distribution and ethnic backgrounds.

Conclusion

This study strengthens the previous findings that SNIP can be used to evaluate respiratory dysfunction during the early stage of young patients with neuromuscular disorders, and demonstrates that steroid is effective in slowing the decrease of SNIP in this group of Chinese DMD boys.  相似文献   
63.
A worker exposed intermittently to hexamethylene diisocyanate (HDI) developed episodes of dyspnea, wheezing, and fever on working days. Complete lung function tests performed when the subject was asymptomatic were normal except for increased airway responsiveness to histamine, which significantly improved after a 3 wk period off work. At that time, specific inhalation challenges with HDI were carried out. After being exposed for 5 min, the subject developed general malaise, cough, fever, and leukocytosis, together with a mixed restrictive and obstructive breathing defect. We demonstrated a subsequent increase in airway hyperexcitability, which lasted for 2 mo. The subject was also challenged with diphenylmethane diisocyanate (MDI) for 15 min. A late obstructive reaction was documented. Increased levels of specific IgG antibodies against HDI-human serum albumin (HSA) and MDI-HSA were demonstrated.  相似文献   
64.
Intestinal lymphoid hyperplasia and recurrent pulmonary infections by pyogenic bacteria are well-recognized accompaniments of common variable (late onset) hypogammaglobulinemia. A 35-yr-old woman with this illness had progressive pulmonary insufficiency caused by nodular lymphoid interstitial pneumonitis, rather than by infectious lung damage, and intestinal lymphoid nodular hyperplasia. B cells were abundant in the intestinal nodules but absent in the pulmonary nodules by immunoperoxidase staining. Pulmonary lymphocytes isolated in single-cell suspension from the biopsy were 0.5% B cells and 82% T cells. Prednisone therapy improved pulmonary function and decreased the intestinal lymphoid nodules. Lymphocytic interstitial pneumonitis should be considered in patients with hypogammaglobulinemia and restrictive lung disease.  相似文献   
65.
Eight atopic adolescent subjects without diagnosis of clinical asthma but with signs of hyperactive airways were studied. The subjects were exposed for 30 min at rest followed by 10 min during moderate exercise on a treadmill to the following: (1) filtered air, (2) 1 mg/m3 NaCl droplet aerosol, (3) 1 ppm SO2 and NaCl droplet aerosol, or (4) 1 ppm SO2. All exposures were at 75% relative humidity and 22 °C. Exposures to either SO2 mode produced statistically significant changes in pulmonary function, whereas sham exposures to air on NaCl did not. These results are similar to those seen earlier in a group of extrinsic asthmatic adolescent subjects and are three to 22 times greater than changes we saw in a group of normal adolescent subjects. The changes seen after inhalation of SO2 were not statistically different from those seen after inhalation of SO2 and NaCl droplet aerosol. Our results indicate that inhalation of 1 ppm SO2 by a group of atopic adolescents can produce exercise-induced bronchospasm at a level of exercise that has no effect by itself.  相似文献   
66.

Objectives

To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance (PVR).

Methods

Among our cohort of patients with Fontan circulation, 8 children, 8 adolescents, and 8 adults had PVR ≥2 WU*m2. These patients were treated with ERAs (minors with bosentan, adults with macitentan) and reevaluated after 6 months. Pre- and posttreatment hemodynamic variables were assessed by cardiac catheterization. Functional capacity was evaluated by cardiopulmonary exercise testing (CPET). Our primary endpoint was to obtain a reduction of PVR; the secondary endpoint was to obtain an improvement of functional capacity.

Results

Under treatment, New York Heart Association class improved for adolescents and adults. PVR decreased (P = .01) in all groups: in children from the median value 2.3 (interquartile range 2.0-3.1) to 1.9 (1.4-2.3) WU*m2, in adolescents from 2.3 (2.1-2.4) to 1.7 (1.4-1.8) WU*m2, and in adults from 2.8 (2.0-4.7) to 2.1 (1.8-2.8)WU*m2. In 71% of patients, PVR fell to less than 2 WU*m2. Cardiac index increased in adolescents from 2.6 (2.4-3.3) to 3.6 (3.4-4.3) L/min/m2, P = .04, and in adults from 2.1 (2.0-2.3) to 2.8 (2.3-4.7) L/min/m2, P = .03. CPET showed that only adolescents displayed a significant functional improvement. Anaerobic threshold improved from 17 (13-19) to 18 (13-20) mL/kg/min, P = .03; oxygen consumption and VO2 max increased from 1.3 (1.0-1.6) to 1.7 (1.1-1.9) L/min, P = .02 and from 25 (21-28) to 28 (26-31) L/min, P = .02, respectively. Oxygen pulse increased from 7.9 (5.7-10.4) to 11.2 (8.2-13.0) L/beat, P = .01.

Conclusions

This is the first study that assesses by cardiac catheterization and CPET the effects of ERA in patients with Fontan circulation with increased PVR. These results suggest that ERAs might provide most pronounced hemodynamic and functional improvement in adults and adolescents.  相似文献   
67.
目的探讨第3秒用力呼气容积下降对轻度肺损伤及早期治疗的意义。方法对150例于我院门诊就诊的患者(咳嗽咳痰>8周)行肺功能检查,根据肺功能结果将其分为3组,每组50例,A组FEV1/FVC、FEV3/FVC均正常,B组仅FEV3/FVC下降,观察各组肺功能指标的变化。并对B组患者进行常规治疗及指导,1年后对B组患者行肺功能复查,并对结果进行分析。结果治疗前,B组较A组有较高的TLC、RV、RV/TLC,而DLCO相对偏低。B组与C组比较,C组有更高的TLC、RV/TLC改变,而DLCO的降低更明显,两组比较有显著差异(P<0.01)。经过1年后再次复查肺功能,A组未见明显下降,B组较C组肺功能下降更加明显。结论第3秒用力呼气容积可作为气道轻度损伤检查的敏感指标,对早期肺损伤患者的治疗具有一定指导意义。  相似文献   
68.
目的:分析健康人1s用力呼气容积与用力肺活量比值的变异情况,并据此评价2001年GOLD和2002年中华呼吸学会制定的COPD诊治指南中将FEV。/FVC〈70%作为COPD诊断分级先决条件的普适性和可行性。方法:测定260例健康人的用力肺活量和最大呼气流量——容积曲线,并进行统计学处理,同时依年龄分为≤35、36—55及≥56岁3组进行统计学处理及比较分析。结果:260例健康人FEV1/FVC均值为83.1%,随着年龄的增加趋于下降,3个年龄组之间两相比较差异有显著性意义(P〈O.05),其它指标如FVC%预计值、FEV1/FVC、PEF%预计值在〈55岁下降不明显,〉56岁下降幅度差异有显著性意义(P〈0.05),FEV。%预计值则于健康人的各年龄阶段都未见显著下降。结论:FEV1/FVC的变异方向难以把握,尤其是COPD患者,其不似FEV1%预计值那样与COPD的病变程度具有良好的趋同性和平行性,作为COPD诊断分级的先决条件缺乏普适性且不能覆盖所有的COPD患者,临床实践中缺乏良好的可操作性,因此FEV1/FVC〈70%作为COPD诊断分级的先决条件缺乏充分的理论依据,建议COPD诊治指南加以修订。  相似文献   
69.
10名慢性阻塞性肺疾病(COPD)患者口服两个不同剂量的茶碱,分别达到两个稳态,测定谷浓度值,同时测定最大肺活量(FVC),一秒钟用力呼气量(FEV1)。提高茶碱稳态浓度前后的FVC值无显著差异,揭示在常规剂量下茶碱对COPD病人通气功能的改善是有限的。引用公式:FVC=Css×m+i,预测病人FVC值与实测值差异无显著性(P>0.05)。不同稳态浓度的FVC值可用此公式预测。  相似文献   
70.

Background

Patients with advanced non-small cell lung cancer (NSCLC) continue to have a poor prognosis. The majority of patients are not indicated for surgery for a radical cure, and systemic chemotherapy is the mainstay of treatment. However, long-term survival is rare due to the resistance to therapy. On the other hand, surgery is performed only under certain conditions for colon cancer and esophageal cancer. Few reports are available about salvage thoracic surgery in patients with primary lung cancer. The purpose of this study was to show the outcomes of salvage surgery for lung cancer, and we discuss possible future treatment strategies based on our findings.

Methods

Three hundred and fifty-two patients with primary lung cancer underwent surgical resection, and we evaluated those who underwent salvage operations. We also examined the relationships between the performance of a salvage operation and the clinicopathological characteristics of the patients. The clinical outcomes of salvage surgery for lung cancer were assessed.

Results

Salvage thoracic operations were performed in eight (2.3%) of the 352 patients. The surgical procedures were lobectomy in four patients, segmentectomy in two, and pneumonectomy and wedge resection were each performed in one patient. There was no postoperative mortality. All patients were alive at the time of the analysis. The mean follow-up period for the salvage operation cases was 14.0 months. No significant correlation was identified between the incidence of salvage surgery and the age, gender, histology, postoperative stay or hospital stay. The incidence of advanced stage disease was higher in the salvage cases than in the overall cases.

Conclusions

Salvage thoracic surgery was possible, and moderately improved the prognosis, without prolongation of the postoperative stay or hospital stay. A salvage operation might be considered a reasonable and proper treatment for carefully selected patients.  相似文献   
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