Diagnosis and treatment of pulmonary hypertension in the adolescent

This article discusses diagnostic and therapeutic options of adolescents with primary and secondary pulmonary hypertension. Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Previously, the diagnosis of pulmonary hypertension in children carried a poor prognosis. However, advances in the diagnosis and treatment of pulmonary hypertension over the last decade have markedly improved survival of many patients. A targeted approach includes treatment of the underlying disease, and therefore requires an extensive evaluation. Many of the treatment modalities are based on advanced understanding of basic pulmonary vascular biology.     

老年收缩期高血压的诊断及治疗

我国高血压患病人数已愈1．6亿，老年人高血压的发病率占高血压病人的60％～70％，而收缩期高血压又是老年高血压患者的一种主要血压特点。在WHO／ISH及中国高血压联盟指南的推动下，高血压的防治有了长足的进展，血压的控制模式也逐渐发生着变化，从对舒张压治疗的重视逐渐转变为对高血压患者收缩压控制的关注。     

肺动脉高压72例临床分析

目的 通过对肺动脉高压患者临床资料的分析,更好地了解肺动脉高压（PAH）,并探讨其治疗方法.方法 分析了72例PAH的临床表现,心动超声特点及心导管检查方法,采用了新的联合治疗方案.结果 经联合治疗方案6个月的治疗,6MWT增加了87.5 m,临床症状缓解,心功能改善.结论 对以往病情复杂,治疗棘手,预后不良的PAH采用新的药物联合治疗方案可获得明显的疗效,改善了患者的生存质量及预后.     

Applying critical pathways to neurosurgery patients at the University of Michigan Medical Center

Project Overview: In April 1990, The University of Michigan Hospitals began a major, multidisciplinary project to standardize care processes in order to increase efficiency and reduce costs while maintaining the quality of clinical care. A team of nurses began the project by developing critical pathways for two neurosurgery procedures--lumbar laminectomy and transphenoidal pituitary tumor resection. The pathways were reviewed by physicians and other staff from other disciplines and were implemented in January of 1991. Key Findings: Data from the first 14 months show a decrease in patients' average lengths of stay in both the intensive care unit (ICU) and routine care unit. Costs and variance data are being analyzed and further improvements to the pathways are being made. Eleven critical paths are now being used for neurosurgery patients. In retrospect, participants learned that physicians should be involved at the earliest stages of critical pathway development and in the process of implementation.     

Cognitive disorders appearing before the age of 65 in patients of the Alzheimer Centre of the VU Medical Centre: diagnoses and clinical characteristics

OBJECTIVE: To obtain a profile of the causes and clinical characteristics of cognitive disorders in patients referred to a memory clinic before the age of 65 years. DESIGN: Retrospective case-note study. METHOD: Data were collected from 127 subjects with objective cognitive disorders who visited the Alzheimer Centre of the VU Medical Centre in Amsterdam, the Netherlands, in the period from 1 January 2001 to 31 December 2003 with an onset of complaints before the age of 65. Besides the diagnoses, we investigated the clinical presentations, the occurrence of cardiovascular risk factors, the family history, and the presence of noncognitive neurological signs. RESULTS: The most common causes of cognitive decline under the age of 65 were Alzheimer's disease (46%) and frontotemporal dementia (23%). Vascular dementia was seen in 5% and dementia with Lewy bodies in 2%; 9% had mild cognitive impairment but no dementia. Hypertension and a positive family history for dementia were each present in 40% of the patients. Non-cognitive neurological abnormalities were found only in cases of non-Alzheimer dementia. During the period under investigation, the number of patients with objective cognitive disorders increased more than did the number without a cognitive disorder. CONCLUSION: Within the population of a memory clinic, Alzheimer's disease was the most frequent cause of cognitive decline under the age of 65, followed by frontotemporal dementia. The distribution differed from causes of dementia at an older age, where vascular dementia had the second place.     

Gynaecology: the experience of patients referred to NHS and private clinics

This cohort study compared the experience of patients seeking treatment for menorrhagia who were referred to National Health Service (NHS) or private clinics. Two-hundred and nine patients in 73 general practices in Berkshire, Buckinghamshire, Northamptonshire and Oxfordshire were recruited by their general practitioners and followed-up with questionnaires at nine months and 18 months after entry to the study. One hundred and fifty patients were referred to NHS clinics and 59 to private clinics; there were no significant differences between the two groups of patients in terms of symptom severity, reason for referral or treatment received. Patients who went to private clinics were more likely to report active participation in decisions about their care (p < 0.05 after adjustment for age and educational status), and were slightly more likely to be satisfied with the care they had received. The treatment decisions made in gynaecological clinics in the NHS and private sector were similar, but the decision-making styles appeared to be different. Private patients were more likely to participate in treatment decisions than NHS patients.     

先天性心脏病肺动脉高压患者血中雌激素含量变化与机制

目的 探讨先天性心脏病(CHD)患者出现肺动脉高压(PAH)后雌激素变化及其与肺动脉压力的相关性,为PAH激素治疗提供理论依据,同时探讨雌激素发生变化的机制.方法 以右心导管测量的肺动脉压力为标准,选取36例肺动脉压力正常和108例合并PAH的左向右分流型CHD患者,分别设为对照组和PAH组,采用化学发光免疫分析法测定两组患者血清雌二醇、孕酮、泌乳素和卵泡刺激素(FSH)含量.结果 PAH组雌二醇、孕酮、泌乳素、FSH含量均明显高于对照组(P<0.05).多元回归分析显示,三种性激素的含量变化与左向右分流水平无相关性,而与性别、年龄、月经周期等生理因素以及肺动脉压力明显相关,相关系数分别为0.607、0.531和0.518.FSH与泌乳素和雌二醇呈正相关,相关系数分别为0.917(P=0.012)和0.759(P=0.000).结论 在CHD患者中,雌二醇含量随患者肺动脉压力升高而升高,表明雌激素对肺动脉压力具有重要的调节作用,但引起雌激素升高的调节靶点位于垂体或下丘脑而非性腺.     

先天性心脏病肺动脉高压患者血中雌激素含量变化与机制

目的 探讨先天性心脏病(CHD)患者出现肺动脉高压(PAH)后雌激素变化及其与肺动脉压力的相关性,为PAH激素治疗提供理论依据,同时探讨雌激素发生变化的机制.方法 以右心导管测量的肺动脉压力为标准,选取36例肺动脉压力正常和108例合并PAH的左向右分流型CHD患者,分别设为对照组和PAH组,采用化学发光免疫分析法测定两组患者血清雌二醇、孕酮、泌乳素和卵泡刺激素(FSH)含量.结果 PAH组雌二醇、孕酮、泌乳素、FSH含量均明显高于对照组(P<0.05).多元回归分析显示,三种性激素的含量变化与左向右分流水平无相关性,而与性别、年龄、月经周期等生理因素以及肺动脉压力明显相关,相关系数分别为0.607、0.531和0.518.FSH与泌乳素和雌二醇呈正相关,相关系数分别为0.917(P=0.012)和0.759(P=0.000).结论 在CHD患者中,雌二醇含量随患者肺动脉压力升高而升高,表明雌激素对肺动脉压力具有重要的调节作用,但引起雌激素升高的调节靶点位于垂体或下丘脑而非性腺.     

先天性心脏病肺动脉高压患者血中雌激素含量变化与机制

目的探讨先天性心脏病（CHD）患者出现肺动脉高压（PAH）后雌激素变化及其与肺动脉压力的相关性,为PAH激素治疗提供理论依据,同时探讨雌激素发生变化的机制。方法以右心导管测量的肺动脉压力为标准,选取36例肺动脉压力正常和108例合并PAH的左向右分流型CHD患者,分别设为对照组和PAH组,采用化学发光免疫分析法测定两组患者血清雌二醇、孕酮、泌乳素和卵泡刺激素（PSH）含量。结果PAH组雌二醇、孕酮、泌乳素、FSH含量均明显高于对照组（P〈0．05）。多元回归分析显示,三种性激素的含量变化与左向右分流水平无相关性,而与性别、年龄、月经周期等生理因素以及肺动脉压力明显相关,相关系数分别为0．607、0．531和0．518。FSH与泌乳素和雌二醇呈正相关,相关系数分别为0．917（P=0．012）和0．759（P=0．000）。结论在CHD患者中,雌二醇含量随患者肺动脉压力升高而升高,表明雌激素对肺动脉压力具有重要的调节作用,但引起雌激素升高的调节靶点位于垂体或下丘脑而非性腺。     

Clinical deterioration after sildenafil cessation in patients with pulmonary hypertension

Sildenafil is a selective inhibitor of phosphodiesterase type 5 (PDE-5). Its chronic administration has been shown to improve exercise capacity, World Health Organization functional class, and haemodynamics in patients with symptomatic pulmonary arterial hypertension (PAH). There is however, no data describing the clinical consequences of sudden cessation of sildenafil treatment. In this series, 9 patients with NYHA Class II–IV PAH who were stable on 2 months of sildenafil monotherapy, had their sildenafil ceased to accommodate a 2-week washout period, required for enrollment in research involving an endothelin receptor antagonist. Six minute walk distance (SMWD) and clinical assessments were performed before cessation of sildenafil, and again 2 weeks later. Over the course of this 2-week washout period, 6 of the 9 patients reported increased breathlessness and fatigue, 1 of these was hospitalized with worsening right heart failure. The SMWD fell in 6 patients, with falls of greater than 100 m recorded in 4 patients. This was accompanied by a worsening of NYHA Class from 2.5 ± 0.2 to 3.1 ± 0.1 (mean ± SEM, p = 0.01). These data indicate that sudden cessation of sildenafil monotherapy, in patients with PAH, carries with it a significant and unpredictable risk of rapid clinical deterioration. We recommend that if sildenafil needs to be ceased, it would be more prudent to consider concurrent vasodilator therapy before the gradual cessation of sildenafil.     

A review of sitaxsentan sodium in patients with pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a life threatening, progressive condition which eventually leads to fatal right heart failure. Endothelin-1 (ET-1), a potent vasoconstrictor peptide, is increased in the pulmonary arteries of patients with pulmonary hypertension. Endothelin-1 acts through the stimulation of 2 subtypes of receptors (endothelin receptor subtypes A [ET(A)] and B [ET(B)]). In PAH patients, ETRAs block the deleterious vasoconstrictor effects of ET-1, and ETRA treatment in PAH patients has been shown to be safe and efficacious. Sitaxsentan is an orally active, highly ET(A) selective ETRA that, in clinical trials, has demonstrated improvements in exercise capacity, functional class and hemodynamics in PAH patients. Sitaxsentan has been shown to be safe, well tolerated, and associated with a lower incidence of liver toxicity than other approved ETRAs.     

病例管理模式在肺动脉高压肺部感染患者中的应用效果

目的探讨病例管理模式在住院肺动脉高压肺部感染患者干预中的应用效果,以减低感染率。方法调查2011年1月-2014年12月肺动脉高压肺部感染住院患者共394例,选取2011年1月-2013年5月的住院患者197例作为对照组,选取2013年6月-2014年12月住院患者197例患者作为观察组,对照组患者实施传统干预模式,观察组采取病例管理模式,两组患者干预20d后比较不良事件发生率、肺部感染率、平均住院时间及密尔顿抑郁量表(Hamilton RatingScale forDepres-sion.HAMD)评分。结果观察组患者不良事件的发生率为23.8%,低于对照组54.3%,差异有统计学意义(P<0.05);观察组患者的感染率为7.1%,较对照组患者的13.7%有明显下降,差异有统计学意义(P<0.05);观察组患者住院时间为(26.07±0.90)d,对照组患者为(32.74±0.80)d,两组比较差异有统计学意义(P<0.05);HAMD评分干预后较干预前低,差异有统计学意义(P<0.05)。结论实施病例管理模式能有力促进肺动脉高压伴肺部感染患者的治疗,有效预防、控制肺部感染。     

Diet needs of patients referred to home health

The purpose of this study was to document the dietary needs of patients referred to home health care. The charts of 812 patients taken for care by the Visiting Nurse Association of Chicago (VNA) were reviewed for the physician's diet order, number of restrictions, diagnoses, age, height, weight, and fee source. More than half of the patients had therapeutic diets ordered by their physicians. A quarter of the patients with therapeutic diets had two or more dietary modifications. An audit process was developed as a reliability check of the authors' determination of the appropriateness of the diet. Review of the charts by registered dietitians (R.D.s) showed that three-fourths of the patients appeared to need therapeutic diets. Forty-seven percent might have benefited from a different diet. More than half of the patients for whom the R.D.s recommended a special diet needed two or more modifications. This emphasizes the need for the R.D. to develop a system for monitoring the dietary requirements of all patients referred to home health care. It further underscores the need for collaboration between physician and dietitian. The registered dietitian who works in home health care agencies serving diverse age groups needs to be a generalist prepared to develop a plan for the total nutrition treatment of persons throughout the life cycle.     

Fetal surgery: for selected patients. The experiences of the Fetal Treatment Center in San Francisco

Foetal surgery, i.e. invasive treatment of the unborn child, has pros and cons. Foetal surgery is the therapy of choice in a selected group of patients. For obstructive uropathy, intrauterine urinary drainage is seldom indicated due to disappointing results. For congenital diaphragmatic hernia, the pressure in the respiratory tract is increased by tracheal occlusion therapy and so pulmonary hypoplasia is prevented. This probably has a beneficial effect for children with a poor prognosis. For congenital cystic adenomatoid malformation of the lung and foetal hydrops, resection of the lesion gives a survival rate of 70%. For sacrococcygeal teratoma and foetal hydrops, intrauterine resection of the tumour might save the life of the foetus. For high myelomeningocele, intrauterine covering of the defect has until now given no improvement in the neurological outcome. For twin transfusion syndrome, laser coagulation of the placental anastomoses is probably superior to serial amnioreduction. For women the mortality and morbidity associated with foetal surgery are low.     

血内皮祖细胞与一氧化氮在慢性阻塞性肺疾病并发肺动脉高压患者中的变化及意义

目的 观察慢性阻塞性肺疾病（COPD）并发肺动脉高压（PAH）病人外周血内皮祖细胞（EPCs）数量和功能及一氧化氮的变化,并探讨其在COPD并PAH发病中的作用.方法 选择本院住院的COPD病人60例,其中COPD并PAH（COPD＋PAH组）病人30例,COPD非PAH病人30例（COPD组）;正常对照20例（非COPD组）.用密度梯度离心法从外周血获取单个核细胞,将其接种在人纤维连接蛋白包被培养板,培养7d后对贴壁细胞进行细胞化学分析.通过集落形成试验、改良的Boyden小室和黏附能力测定实验计数EPC的数量、测定EPC的迁移和黏附能力.用硝酸还原酶法比色检测入选患者血浆一氧化氮（NO）水平.结果 和正常对照组[（24.9±4.1）克隆形成数]及COPD组[（21.9±3.9）克隆形成数]相比,COPD＋PAH组[（14.2±3.5）克隆形成数]外周血EPC数量明显减少,黏附和迁移能力显著降低（F≥9.15,q≥3.49,P〈0.05）,且EPC数量及黏附、迁移能力与肺动脉压力呈负相关（r=-0.79、-0.85、-0.89,P〈0.01）.COPD＋PAH组患者NO水平[（43.6±4.8）ng/ml]明显低于对照组[（67.17±4.9）ng/ml]（P〈0.01）,NO浓度与循环EPCs数量及迁移能力呈正相关（r=0.77、0.71,P〈0.01）,与循环EPCs黏附能力无关.结论 COPD病人PAH的发生可能与循环EPCs数量减少、迁移和黏附能力降低有关,这种变化可能与血浆NO水平减少有关.     

肺血容积效应合并肺挫裂伤的 CT 诊断分 析简

目的：探讨肺血容积效应合并肺挫裂伤的CT表现。方法：对46例胸部外伤仰卧位平扫见肺部背侧高密度影患者,再行侧卧位或俯卧位扫描,分析肺血容积效应合并肺挫裂伤的CT特征。结果：46例肺血容积效应患者中仰卧位发现12例合并肺挫裂伤,侧、俯卧扫描患者中发现26例合并肺挫裂伤,差异具有统计学意义（P＆lt;0.05）。结论：侧、俯卧位扫描在肺血容积效应合并肺挫裂伤中有诊断意义,避免误诊和漏诊。