内源性一氧化氮对大鼠慢性缺氧性肺动脉高压的调节作用

目的探讨一氧化氮对慢性缺氧性肺动脉高压的调节作用。方法采用Ｗｉｓｔａｒ大鼠１８只，随机将各配伍组大鼠分别分配到组Ⅰ（对照组）、组Ⅱ（缺氧组）及组Ⅲ［缺氧加一氧化氮合酶抑制剂Ｎω－硝基－Ｌ－精氨酸甲酯（Ｌ－ＮＡＭＥ组）］。研究Ｌ－ＮＡＭＥ对缺氧性肺动脉高压的影响。结果与对照组相比，缺氧加Ｌ－ＮＡＭＥ注射的大鼠肺动脉平均压上升百分数（５８％±１２％）明显高于单纯缺氧大鼠（３４％±１５％）（ｔ＝３．０１３，Ｐ＜０．０１）；缺氧加Ｌ－ＮＡＭＥ注射大鼠血浆一氧化氮下降百分数（３８％±４％）明显大于单纯缺氧大鼠（１２％±１２％）（ｔ＝２７；０．０５＞Ｐ＞０．０２）。结论大鼠慢性缺氧时一氧化氮释放减少，而一氧化氮释放减少可能参与缺氧性肺动脉高压的形成     

一氧化氮吸入治疗小儿急性呼吸衰竭

为观察一氧化氮（ＮＯ）治疗小儿急性呼吸衰竭的疗效，应用我院自行研制的ＮＯ吸入装置，对１５例急性呼吸窘迫综合征（ＡＲＤＳ）和急性呼吸衰竭（简称急性呼衰）患儿进行ＮＯ吸入治疗。结果：７例有效，ＮＯ吸入前后比较氧分压与吸入氧浓度比值上升４．１±２．３ｋＰａ（３０．５±１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）（ｔ＝４．５２，Ｐ＜０．０５），氧合指数降低９±３（ｔ＝４．６３，Ｐ＜０．０５）。对２例肺动脉导管压力监测显示，肺动脉压和肺血管阻力明显下降，体动脉压和心率无显著性变化。结论：ＮＯ吸入疗法对部分急性呼衰患儿有效，宜在急性低氧性呼衰、心功能未受严重损害时应用。     

小儿扩张型心肌病肺动脉压的多普勒超声估测及其临床意义

目的估测扩张型心肌病肺动脉压并探讨其临床意义。方法用多普勒超声检查２６例扩张型心肌病患儿及２６名配对健康儿。肺动脉压用测三尖瓣最大返流速度及肺动脉血流加速时间（ＡＴ）两种方法估测。结果２６例患儿中２１例有三尖瓣返流，肺动脉收缩压（ＰＡＳＰ）３．２～１０．４ｋＰａ（６．０±１．９ｋＰａ）（２４～７５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）。其中１８例（８６％）＞４ｋＰａ；对照组１４例有可测三尖瓣返流，ＰＡＳＰ均＜４ｋＰａ。患儿组ＡＴ缩短，ＡＴ与右室射血时间比值降低，也提示肺动脉压增高。ＰＡＳＰ与患儿左室短轴缩短分数、心功能低下持续时间及有无心内超声自发显影有关。随诊显示心功能好转者的ＰＡＳＰ较死亡或病情无改善者低，且后者肺动脉压继续增高。结论扩张型心肌病患儿多存在肺动脉高压，用多普勒超声估测的肺动脉压与患儿的病情及临床预后可能有一定的关系。     

小儿肺动脉高压的研究进展

小儿肺动脉高压的研究进展杜军保李树政当肺动脉收缩压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｓｙｓｔｏｌｉｃｐｒｅｓ－ｓｕｒｅ，ＰＡＳＰ）超过４．００ｋＰａ（３０ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）或肺动脉平均压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｍｅａｎ...     

一氧化氮对缺氧和急性肺损伤犬的血液动力学与气体交换的影响

应用化学发光法监测氮氧化物浓度，观察１０只缺氧和急性肺损伤犬在吸入不同浓度一氧化氮（ＮＯ）时血液动力学和气体交换功能的变化。结果显示，５～５０ＰＰＭＮＯ均可降低缺氧犬肺动脉压２５％±３％（Ｐ＜０．０１），降低肺血管阻力３７％±５％（Ｐ＜０．０１），并使急性肺损伤犬的动脉血氧分压／吸入氧浓度（ＰａＯ＿２／ＦｉＯ＿２）比值上升３３．４±２．３（Ｐ＜０．０５），肺内动静脉分流量与总血流量（Ｑ＿ｓ／Ｑ＿Ｔ）比值下降５％±２％（Ｐ＜０．０５）。提示，低浓度ＮＯ（５～２０ＰＰＭ）即可有效降低缺氧性和急性肺损伤犬肺动脉高压并改善其动脉氧合功能。     

一氧化氮合酶基因表达在新生鼠缺氧性脑损伤中的作用

新生儿缺氧性脑损伤可导致永久性神经功能损害。我们测定新生鼠缺氧性脑损伤模型服组织内诱生型一氧化氮合酶（ｉｎＯＳ）ｍＲＮＡ表达变化,以阐明它在发病中的作用。取新生ＳＤ鼠随机分组制作模型并行病理鉴定;采用ＲＴ－ＰＣＲ测定脑缺氧时脑组织细胞内ｉＮＯＳｍＲＡＮ表达的变化,深度度扫描获取数值。实验显示,缺氧２小时后损伤脑组织的ｉＮＯＳｍＲＮＡ表达显著升高（Ｐ〈０．００１）。结果表明,新生鼠缺氧后神经系统诱生     

先天性心脏病重度肺动脉高压性质的综合评价

目的探讨先天性心脏病（简称先心病）合并重度肺动脉高压患儿器质性肺动脉高压（简称肺高压）的诊断标准。方法３７例经手术治疗后早期肺动脉压力降至正常的患儿作为动力性肺高压组；７例经手术治疗后仍持续性肺动脉高压及６例临床诊断为器质性肺高压而未予手术的共１３例患儿作为器质性肺高压组，对比两组心导管检查血液动力学指标。结果两组肺血管阻力、肺小动脉楔压、肺循环血流量与体循环血流量之比（Ｑｐ／Ｑｓ）及降主动脉血氧饱和度（ＳａＯ２）差异均有显著意义，如按年龄大于２岁、肺动脉阻力＞７２ｋＰａ·ｓ－１·Ｌ－１（９Ｗｏｏｄ单位）、肺小动脉楔压≤１．６ｋＰａ（１２ｍｍＨｇ）、Ｑｐ／Ｑｓ＜２和动脉血氧饱和度＜０．９０作为临床诊断器质性肺高压的指标，则本组动力性肺高压组仅有５．４％的患儿符合上述指标３项或３项以上，而器质性肺高压组所有病例均符合上述指标３项或３项以上。结论先心病合并重度肺动脉高压存在上述５项指标中３项或３项以上，高度提示患儿存在器质性肺动脉高压     

低浓度一氧化氮及妥拉苏林对兔低氧性肺动脉高压的作用比较

目的　探讨吸入低浓度一氧化氮（ＮＯ） 及妥拉苏林气管内和右心室内给药对肺动脉高压的作用。方法　予急性低氧性肺动脉高压家兔模型吸入６×１０－ ６ ＮＯ及气管内滴入和右心室内注入０－５ ｍｇ／ｋｇ 妥拉苏林,通过左颈总动脉插管和右心室插管观察平均动脉压（ＭＡＰ） 、右心室收缩压（ＳＲＶＰ）及血气的变化。结果　低氧通气使家兔ＳＲＶＰ由（１４－０ ±２－２） ｍｍＨｇ（１ ｍｍＨｇ＝ ０－１３３ ｋＰａ）上升至（１７－４±２－０） ｍｍＨｇ（Ｐ＜０－０５）,吸入６×１０－６ ＮＯ 后降至基础水平（ Ｐ＜ ０－０５）;吸入前后ＭＡＰ差异无显著意义（Ｐ＞ ０－０５）。低氧通气同时吸入ＮＯ对动脉血氧分压无影响。妥拉苏林两种给药途径均使ＭＡＰ和ＳＲＶＰ下降（Ｐ＜０－０５） ;气管内给药还使ＳＲＶＰ／ＭＡＰ比值由０－２１±０－０６ 下降至０－２０±０－０６（ Ｐ＜０－０５）,该效应与吸入ＮＯ相比,差异有显著意义（ Ｐ＜ ０－０５） 。气管内给药使ＭＡＰ、ＳＲＶＰ下降的百分率均小于右心室内给药（Ｐ＜０－０５）。结论　吸入６ ×１０－６ ＮＯ能选择性扩张肺血管;妥拉苏林气管内给药具有一定的肺血管选择性,仍弱于ＮＯ的作用;但右心室内给药缺乏肺血管选择性。     

气管内气体吹入通气的实验与临床研究

目的确定气管内气体吹入通气（ＴＧＩ）对肺部气体交换的影响。方法先通过动物实验观察正常肺及损伤肺时ＴＧＩ对肺部气体交换的影响；后选择急性呼吸衰竭患儿，观察ＴＧＩ对５例不同性质肺部病变患儿的治疗效果。结果１４只兔在正常肺及损伤肺时，ＴＧＩ使动脉血二氧化碳分压（ＰａＣＯ２）分别从压力控制通气（ＰＣＶ）时的５．６±０．３ｋＰａ、７．７±０．２５ｋＰａ下降至３．９±０．１５ｋＰａ、５．６±０．４３ｋＰａ（Ｐ＜０．０１），而动脉血氧分压（ＰａＯ２）则无明显变化（Ｐ＞０．０５）。（２）５例急性呼吸衰竭患儿当保持吸气峰压（ＰＩＰ）不变时，可降低ＰａＣＯ２，或在保持ＰａＣＯ２稳定时可降低ＰＩＰ。结论ＴＧＩ作为一种机械通气方法，通过加速ＣＯ２的排出，可提高肺泡通气效率，但对肺部氧合无明显影响     

格林—巴利综合征患儿血清神经节苷脂抗体测定的临床意义

我们首次检测并发现３０例ＧＢＳ患儿血清ＧＳ－Ａｂ阳性率为２６．７％，其他神经疾病（ＯＤＮ）组阳性率为２．３％，两组间有显著性差异（ｘ＾２＝９．６８７，Ｐ＜０．００１）；ＧＢＳ患血清Ｅ－Ａｂ阳性率３６．７％，ＯＮＤ组阳性率９．３％，正常对照组全部阴性；１１例ＧＢＳ患儿血清Ｅ－Ａｂ阳性者中，有ＧＳ－Ａｂ阳性８例（７２．７％），Ｅ－Ａｂ平均滴度为１：２１．９，ＧＳ－Ａｂ平均滴度为１：１７．５。１１例ＧＢ     

High-altitude pulmonary edema with absent right pulmonary artery

High-altitude pulmonary edema potentially is fatal. Adults with unilateral absence of a right pulmonary artery are particularly susceptible to high-altitude pulmonary edema. The occurrence of high-altitude pulmonary edema was documented in a child with congenital absence of the right pulmonary artery. Improvement occurred only upon descent to low altitude. Physicians should be aware of this life-threatening condition in children ascending to high altitude, particularly in individuals with unilateral absence of a pulmonary artery.     

Common pulmonary vein atresia without anomalous pulmonary venous connection

Common pulmonary vein atresia without pulmonary venous connection is a rare form of congenital heart disease. No communication exists between the confluence of the pulmonary veins and the heart or a major systemic vessel. A case diagnosed antemortem is presented. Correct early diagnosis is imperative as surgery may be corrective.     

Liposteroid against refractory pulmonary haemorrhage in idiopathic pulmonary haemosiderosis

We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the usefulness of liposteroid in the management of refractory IPH.     

Congenital pulmonary arteriovenous fistula producing pulmonary arterial steal syndrome

Summary This report describes a congenital pulmonary arterial steal syndrome manifested as cyanosis and acidosis in a newborn. A fistulous connection between the right pulmonary artery and a large, anomalous right common pulmonary vein stole blood from the pulmonary arteries. The anomaly was suspected because of a pericardiac shadow on frontal and lateral chest films, substantiated by M-mode echocardiogram, confirmed at cardiac catheterization with angiocardiography, and analyzed at postmortem examination. Supported in part by a grant from The Rose M. Badgeley Charitable Trust     

Intralobar pulmonary sequestration occurring without alteration of pulmonary parenchyma

Typical changes of lung sequestration, including the scimitar sign, were seen on plain films in the left lower lobe. At aortography vasculature of a pulmonary sequestration was seen located paravertebrally in the left posterobasal segment. The arterial supply arose from a division of the thoracic aorta above the diaphragm; venous drainage was to the pulmonary veins. No pulmonary parenchymal changes were detected in the sequestration after resection, nor was there an extrapleural component of the sequestration.     

Pediatric pulmonary CT-scanning

Forty three lung scans, obtained in 29 anaesthetized children, were evaluated and compared with 85 scans performed in 52 sedated children. Confluent high absorptive areas in the lower parts of the lungs were found in 35 (81%) of the scans performed in children under general anaesthesia but such areas were not found in the scans performed under sedation. — For general anaesthesia, halothane-N₂O-O₂ was used in all but one patient. The radiological changes are presumably due to a fall in functional residual capacity with consequent airway closure. — It is important not to misinterpret these anaesthesia-induced pulmonary changes which may obscure or mimic true pathological lesions, such as parenchymal and pleural metastases.     

Visualization of pulmonary vein obstruction by pulmonary artery wedge injection

Summary One of the major postoperative complications of total anomalous pulmonary venous connection is obstruction of the pulmonary veins. This event may be apparent immediately after surgery when it is usually due to congenitally obstructed veins or later when it may be the result of failure of commensurate growth of the left atrium-common vein anastomosis or of scarring at the anastomotic site. The exact location of the obstruction becomes, then, of paramount importance in deciding if the patient can be helped by further surgery. At cardiac catheterization, pulmonary artery wedge injection was the only angiographic technique that visualized the site and degree of pulmonary venous obstruction in the case reported here.