支气管哮喘急性发作患者52例的院前急救分析

目的分析了支气管哮喘急性发作时进行院前急救的方法以及疗效。方法我急救中心于2009年1月到2011年5月呼吸科共接受支气管哮喘急性发作患者52例,对52例的临床治疗进行回顾性分析。结果患者在入院前进行急救处理,待病情明显转好后送医院进行治疗的47例,占90.38%。患者被紧急送到医院进行治疗患者有5例,占9.62%,其中1例在送往医院的过程中死亡。分析显示患者60岁以下病人病情的缓解能力（90.09%）显著高于60岁以上病人（70%）。结论支气管哮喘急性发作是一种常见的急救病种,该疾病病情发展极快,实施院前急救能明显缓解患者的病情的发展,能降低患者的死亡率,为患者得到更好的救治提供机会。     

A submandibular swelling: the salivary gland?

Four patients, three men aged 32, 51 and 74 years, respectively, and a woman aged 34 years, presented with a submandibular swelling. In all four patients, the swelling was caused by submandibular salivary-gland disease. The first patient appeared to have chronic sialoadenitis due to sialolithiasis, in the second patient the diagnosis was sialoadenosis due to anorexia-boulimia, the third patient had a pleomorphic adenoma and the fourth had a malignant neoplasm of the floor of the mouth causing obstruction of the submandibular gland. A submandibular swelling may have many different causes. It is sometimes difficult to differentiate between enlargement of the submandibular gland and an enlarged lymph node. Intra-oral inspection and palpation may give valuable information about the origin of some submandibular swellings. Using a combination of history, examination and, when necessary, fine-needle aspiration and r?ntgenological examination, the diagnosis of submandibular lumps is usually straightforward.     

Clinical manifestations, course and outcome of enzyme replacement therapy in Hungarian patients with Pompe's disease

Pompe's disease is an autosomal recessive disease caused by deficiency of acid-alpha-glucosidase. Aims and Methods: Authors analyzed the phenotype of 11 Hungarian patients with Pompe's disease and evaluated clinical parameters and response to enzyme replacement therapy during a long-term follow-up in 8 patients. Results: One patient with atypical infantile form presented with cardiomyopathy and a very slow progression of motor deficits; after 2 years of enzyme replacement therapy no disability was present at the age 6 years. Another patient was asymptomatic at the age of 2.5 years. The adult onset form was characterized by slight to prominent limb-girdle myopathy with an age of onset between 20 and 50 years. In 3 of such cases respiratory insufficiency was also present. Conclusions: Hungarian patients with Pompe's disease presented with a wide phenotypic variability ranging from atypical early childhood form with slowly progressive course to late-onset limb-girdle myopathy with variable courses. Enzyme replacement therapy resulted in significant improvement in motor and respiratory functions in most of the patients.     

A cost-utility analysis of the home parenteral nutrition program at Toronto General Hospital: 1970-1982

We performed an economic evaluation of a home parenteral nutrition (HPN) program by measuring the incremental costs and health outcomes for a cohort of 73 patients treated at our institution from November 1970 to July 1982. Over a 12-year time frame, we estimate that HPN resulted in a net savings in health care cost of $19,232 per patient and an increase in survival, adjusted for quality of life, of 3.3 years, compared with the alternative of treating these patients in hospital with intermittent nutritional support when needed. This result was sensitive to assumptions made about the cost of the alternative treatment strategy. When these assumptions were most unfavorable to the HPN program, we estimated that HPN resulted in incremental costs of $48,180 over 12 years, $14,600 per quality-adjusted life-year gained. We conclude that the cost-utility of HPN compares favorably with other health care programs, when HPN is used to treat patients with gut failure secondary to conditions such as Crohn's disease or acute volvulus. Since only one patient with active malignancy was enrolled in our HPN program, these results should not be extrapolated to patients with active malignancy.     

Interstitial lung disease in mixed connective tissue disease

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.     

Outcome of aggressive fibromatosis treated with radiation therapy

INTRODUCTION: The purpose of this study is to report the clinical course and outcome in 7 patients with aggressive fibromatosis. MATERIAL AND METHODS: Between the years 2000 and 2003, 7 patients who were treated with combined modalities were evaluated retrospectively. Patients' demographic information, including age and gender, tumour characteristics, surgical resection, and the use of radiotherapy were recorded and evaluated. RESULTS: The mean patient age was 34 years. The median time to follow-up was 15.5 months. Resection was performed with positive surgical margins in three cases. Three patients were evaluated as inoperable and one patient was treated with debulking surgery. All patients received radiation therapy with a median dose of 51 Gy. At follow-up, three patients had no evidence of disease, three patients were alive with disease, and one patient died 15 days after radiotherapy. CONCLUSION: Local control is the primary problem in aggressive fibromatosis. There is no appropriate treatment for aggressive fibromatosis and the type of treatment depends on tumour characteristics and location as well as patient characteristics.     

Hemifacial spasms caused by neurovascular compression

Three patients, one woman aged 52 years and two men aged 63 and 71 years, respectively, had involuntary movement on one side of their face due to hemifacial spasms. The first patient's spasms were misdiagnosed as a tic, the second patient had received injections of botulinum A toxin which gave no improvement and the third patient suffered from persistent symptoms after a first neurovascular decompression without intraoperative EMG monitoring. All three patients underwent microvascular decompression of the facial nerve with intraoperative EMG monitoring. All three patients were cured. Hemifacial spasm is a curable illness if appropriately diagnosed and treated. Oral medications have no effect on the disease. Local injection of botulinum A toxin is indicated when the spasm is mild or when surgery is contraindicated. The primary causative factor is vascular compression of the facial nerve at its exit zone. Therefore, decompressive surgery is the logical treatment, and the best results are obtained with intraoperative EMG monitoring.     

Three patients with pneumonia due to Legionella associated with a sauna, a cooling tower and a caravan in The Netherlands

In three male patients with lower respiratory disease, aged 51, 32 and 63 years, Legionnaires' disease was diagnosed by urinary antigen test and culture of the respiratory-tract fluid. In the second patient, the bronchoalveolar fluid also contained Streptococcus pneumoniae and Haemophilus influenzae. All three patients recovered after treatment with azithromycin in the first, cefotaxime, vancomycin and levofloxacin in the second, and erythromycin and ciprofloxacin in the third, respectively. Legionella pneumophila pneumonia is clinically not clearly distinct from other pneumonias and has a high mortality rate when not treated with the proper antibiotics. For that reason, adequate and swift diagnosis is of great importance. The urinary antigen test meets both of these criteria. Still, it is advisable to use culture and serology as well if Legionnaires' disease is suspected in a patient, since the urinary antigen test has limitations. In addition, patient isolates are ofepidemiological importance for public health. By comparing available patient isolates with Legionella strains from water sources, it is possible to identify sources of infection. In 2002, based on this principle, a project was started in The Netherlands aimed at identifying sources of infection, thereby preventing outbreaks of Legionnaires' disease by swift elimination of the source. Since the start of the project, 29 sources have been identified. In the cases described above these were a sauna, a cooling tower and a caravan, respectively. In suspected cases, respiratory-tract fluid must be collected to make possible such a source investigation.     

A retired shipyard worker with rapidly progressive pulmonary interstitial fibrosis

We present a case of progressive interstitial fibrosis in a retired shipyard worker who was exposed to asbestos during the postwar era of the late 1940s and 1950s, when asbestos exposures in the workplace were not regulated. Forty years later, at 63 years of age, the patient presented with restrictive lung disease. The patient was diagnosed with asbestos-related pleural disease and parenchymal asbestosis. He remained stable for the next 7 years, but then he began to manifest rapid clinical progression, which raised the possibility of an unusual variant of asbestosis, a concomitant interstitial process, or an unrelated disease. Lung biopsy was not undertaken because of the patient's low pulmonary reserve and limited treatment options. An empiric trial of oral steroids was initiated, but his pulmonary status continued to deteriorate and he died of pulmonary failure at 72 years of age. Many diseases result in pulmonary interstitial fibrosis. Ideally, open lung biopsy should be performed, but this procedure inevitably causes complications in many patients with end-stage restrictive lung disease. Furthermore, while the presence of asbestos bodies in tissue sections is a sensitive and specific marker of asbestos exposure, neither this finding nor any other charge is a marker indicative of asbestosis or the severity of asbestosis. With the enactment of the Asbestos Standard in the United States, asbestos exposures have been decreasing in this country. However, industries that produce asbestos products and wastes continue to expand in developing countries. Prevention of asbestos-related lung disease should be a global endeavor, and asbestos exposures should be regulated in both developed and developing countries.     

我国60岁以上女性眼病患者调查分析

目的:了解老年女性眼病患者发病情况和病因顺位。方法:各地防盲办公室将《眼病登记表》发至县、地市、省级医院和眼科专科医院,由眼科医生出诊时依照患者病历填写。结果:1997年1月1日～1998年12月31日60岁以上女性眼病患者共计74448例,其中白内障患者占64.32%,双盲与单盲两者共23983例,双眼低视力与单眼低视力两者共12469例,视力在各年龄组之间有显著性差异(P<0.001)。结论:60岁以上女性眼病患者中白内障占第1位,政府应正视老年性白内障高发病率的严重性,及早采取预防措施。     

Hope in action—facing cardiac death: A qualitative study of patients with life-threatening disease

Coping with existential challenges is important when struck by serious disease, but apart from cancer and palliative care little is known about how patients deal with such issues and maintain hope. To explore how patients with life-threatening heart disease experience hope when coping with mortality and other existential challenges, we conducted a qualitative study with semi-structured interviews. We made a purposive sample of 11 participants (26–88 years) who had experienced life-threatening disease: eight participants with serious heart disease, two with cancer, and one with severe chronic obstructive pulmonary disease. Analysis was by systematic text condensation. The findings showed that hope could enhance coping and diminish existential distress when patients were confronted with mortality and other existential challenges. Hope was observed as three types of dynamic work: to shift perception of mortality from overwhelming horror toward suppression or peaceful acceptance, to foster reconciliation instead of uncertainty when adapting to the new phase of life, and to establish go-ahead spirit instead of resignation as their identity. Meaning of life could, hence, be sustained in spite of serious threats to the persons'' future, everyday life, and self-conception. The work of hoping could be supported or disturbed by relationships with family, friends, and health care professionals. Hope can be regarded as an active, dynamic state of existential coping among patients with life-threatening disease. Physicians may support this coping and thereby provide personal growth and alleviation of existential distress by skillfully identifying, acknowledging, and participating in the work of hoping performed by the patient.     

冠心病患者康复护理研究进展

冠心病或缺血性心脏病,由于心脏动脉斑块的形成,导致心肌血流量减少,是最常见的心血管疾病,包括稳定型心绞痛、不稳定型心绞痛、心肌梗死和心源性猝死.常见的症状是胸痛或不适,可能蔓延到肩部、手臂、背部和颈部.近年来我国患有冠心病的人数较多,常发于年龄＞40岁的男性群体,并且受到社会压力、不健康生活习惯及遗传等因素影响,导致该...     

Bilateral cataract in childhood years: always an indication for screening on a metabolic disorder

In three young patients who presented with bilateral cataracts the cause proved to be an inherited metabolic disease. The first patient was a newborn aged 7 weeks, in whom galactokinase deficiency was diagnosed. The second patient was a boy aged 8 years with cerebrotendinous xanthomatosis. The third patient was a girl who was diagnosed with cataracts at the age of 3 months. At the age of 4 years the diagnosis 'rhizomelic chondrodysplasia punctata' was established. Screening for metabolic disorders in all children with bilateral cataracts is essential, as in some disorders progressive and severe symptoms can be avoided with timely initiation of treatment. In addition, diagnosis allows for family studies and genetic counselling to take place. This may result in prevention of disease by early therapeutic intervention and prenatal screening.     

Three patients with hereditary multiple exostoses and malignant degeneration of an osteochondroma located in the pelvis

In three male patients with hereditary multiple exostoses (HME), aged 50, 29 and 31 years, peripheral low-grade chondrosarcoma in the pelvic region led to swelling or pain. In the first patient, curative resection was not feasible because of the size and extension of the tumour. However, rapid tumour growth and unbearable pain necessitated a debulking procedure 16 months later. Histopathologic examination revealed a highly malignant dedifferentiated chondrosarcoma. The patient died two years after initial presentation as a result of local tumour growth. In the second patient, treatment consisted of wide resection of the tumour. Five years after the surgery the patient was free of disease. The third patient was initially treated by intralesional resection, followed by partial hemipelvectomy because of residual tumour. Thirteen months later, a local recurrence occurred that was treated by wide excision. Four years after the partial hemipelvectomy the patient was both pain-free and disease-free. Patients with HME are at increased risk for malignant degeneration of pelvic osteochondroma to chondrosarcoma. Periodic control of patients with pelvic osteochondromas is advised, preferably once every two years.     

Weight loss,joint pain and abdominal lymphadenopathy as signs of sarcoidosis,but also of Whipple's disease

Two patients, a woman aged 66 and a man aged 56 years, with an inflammatory syndrome, weight loss, joint pain and abdominal lymphadenopathy received long-term treatment with corticosteroids for alleged sarcoidosis. No long-term remission was induced and the patients were referred for a second opinion. Eventually the diagnosis of Whipple's disease was established 5 years after the appearance of the first symptoms in the case of the female patient and 4 years after in the case of the male patient. Both patients showed a marked clinical improvement after treatment with trimethoprim-sulfamethoxazole. An atypical presentation of alleged sarcoidosis should suggest the possibility of Whipple's disease, especially in the case of gastrointestinal symptoms and the failure to respond to corticosteroids, and warrants duodenal biopsy. The presence of granulomas with an elevated angiotensin-converting enzyme level is not pathognomonic for sarcoidosis. It is vitally important to distinguish the two disorders, as Whipple's disease is an infectious disorder that requires antibiotic therapy to prevent a fatal outcome.     

The first case of single pancreas transplantation in Hungary

Simultaneous pancreas kidney (SPK) transplantation is the only routinely used therapeutic option which can provide insulin independence, euglycemia and good renal replacement for type I diabetes mellitus patients with end stage renal disease. Several patients have some complications of diabetes without renal failure. For these patients pancreas transplantation alone is a therapeutic option. The first pancreas transplantation alone was performed 6 years after the launch of our pancreas transplant program. The patient was a 40-years-old man. Enteric drainage was used with portal venous drainage. Anti IL-2. R antibody, daclizumab was given as prolonged induction therapy. In spite of the technical and immunological difficulties there were neither technical failures nor acute rejection. 3 years after the transplantation the patient has a good quality of life without insulin therapy with excellent renal function. Conclusion: PTA transplant is a routinely used therapeutic option with good survival rate and good quality of life for type I diabetes mellitus patients without end stage renal disease.     

Clinical study of recurrence rate and survival time of patients with carcinoma of the breast treated by biopsy excision without any other therapy.

We report the recurrence rate and survival time in 31 patients with carcinoma of the breast who were treated by limited excision of the primary tumour and no other therapy. At three years the overall recurrence rate in the breast was 28%. One patient was found to have systemic disease and two died of other causes. The overall recurrence rate in the breast at five years was 33%. One patient died of disseminated disease at 6 years 4 months.     

Clinical depression, antidepressant use and risk of future cardiovascular disease

Many studies have shown that depression contributes to a higher risk of developing cardiovascular disease (CVD). Use of antidepressants and its association with CVD development has also been investigated previously but the results have been conflicting. Further, depression and use of antidepressants have been more widely studied in relation to coronary heart disease rather than stroke. A population-based cohort study consisting of 36,654 Swedish elderly twins was conducted with a follow-up of maximum 4 years. Information on exposures, outcomes and covariates were collected from the Swedish national patient registers, the Swedish prescribed drug registry and the Swedish twin registry. Depression and antidepressant use were both associated with CVD development. The risk was most pronounced among depressed patients who did not use antidepressants (HR 1. 48, CI 1.10–2.00). When assessing the two main CVD outcomes coronary heart disease and ischemic stroke separately, the predominant association was found for ischemic stroke while it was absent for coronary heart disease. The association between depression and stroke also remained significant when restricting to depression diagnoses occurring at least 10 years before baseline. The study supports that depression is a possible risk factor for development of CVD. Moreover, the hazard rate for CVD outcomes was highest among depressed patients who had not used antidepressants. The association with clinical depression is more marked in relation to stroke and disappears in relation to development of coronary heart disease.     

Relative cost effectiveness of the SPHERE intervention in selected patient subgroups with existing coronary heart disease

Heterogeneity exists within the patient population with coronary heart disease and the cost effectiveness of treatment may vary across subgroups within the overall population. This study compares the cost effectiveness of a secondary prevention intervention for a combined patient population relative to three selected subgroups: patients aged over 70?years; patients with a diagnosis other than angina only (that is, patients with a history of myocardial infarction, coronary artery bypass graft and/or percutaneous transluminal coronary angioplasty); and patients with diabetes. The results for the general population have been published elsewhere, but ongoing budget constraints require consideration of the appropriateness of targeting resources to patient subgroups. We adopt a probabilistic model to combine within trial and beyond trial impacts of treatment to estimate the lifetime health care costs and quality-adjusted life years of two primary care-based secondary prevention strategies: SPHERE Intervention—tailored practice and patient care plans and Control—standardised usual care. In all cases, the intervention was associated with mean cost savings and mean QALYs gains, when compared to the control, though statistical significance was never achieved. However, the probability of the intervention being cost effective was higher than 85% in all analyses across a range of potential cost-effectiveness threshold values. There is no compelling statistical evidence to support the targeting of specific subgroups across the general population. However, if affordability constraints are binding, the results do allow a tentative ranking of priorities based on the probabilistic subgroup analysis.     

Amyotrophic lateral sclerosis: mechanical ventilation--or not right?

In two patients (men aged 64 and 62 years, respectively) with amyotrophic lateral sclerosis (ALS) tracheostomy ventilation was initiated following (imminent) acute respiratory failure; in one patient this was done because advance directives were lacking, while in the other non-invasive ventilation (NIPPV) was no longer an option. A third ALS patient, a woman aged 36 years, already had chronic respiratory failure when she presented at the local centre for home mechanical ventilation. As a result, the placement of a percutaneous endoscopic gastrostomy (PEG) was impossible. Instead, she had to use a nasopharyngeal feeding tube, which diminished the effectiveness of her NIPPV. The fourth patient, a man aged 60 years, was referred in good time and hence had sufficient time to consider domiciliary ventilation. Following the placement of a PEG he started NIPPV electively. In patients with ALS, domiciliary ventilation should be discussed early in the course of the disease. Advance directives with regard to domiciliary ventilation are important, not only to avoid undesired (invasive) ventilation, but also with respect to the placement of a PEG and the appropriate use of oxygen and morphine.