Biomedical subjects

A Linhart

Publications and source records attributed to A Linhart.

At least 19 recordsLinked to original sources

PubMed2006

Hyperhidrosis: a new and often early symptom in Fabry disease. International experience and data from the Fabry Outcome Survey.

Hypohidrosis is a classic feature of Fabry disease; in contrast, hyperhidrosis has only been rarely described. The aim of the study is to characterise the baseline descriptive data on hyperhidrosis (frequency, age at onset, sex ratio and outcome with and without enzyme replacement therapy) in hemizygous male and heterozygous female patients with Fabry disease. We describe case histories of five patients with Fabry disease and hyperhidrosis seen at three different centres. We have also analysed a cohort of 21 paediatric patients in the UK and a large European cohort of patients enrolled in the Fabry Outcome Survey (FOS). Five patients (three female, two male) with hyperhidrosis were originally identified, although each had additional symptoms related to Fabry disease. The age at onset of hyperhidrosis was less than 18 years in four cases. In the cohort of 21 paediatric patients (12 female, nine male), one female had hyperhidrosis; the age at onset of this symptom was 11 years. In the FOS cohort, 66 of 714 patients with Fabry disease had hyperhidrosis (44 of 369 females, 11.9%; 22 of 345 males, 6.4%). The female predominance was observed in seven of nine countries from which data were analysed. Hyperhidrosis is an increasingly recognised feature of the Fabry disease phenotype. It is more prevalent in females than in males and often appears in childhood or adolescence. The efficacy of enzyme replacement therapy on this recently recognised symptom should be assessed.

Adolescent↗

PubMed2006

[Contemporary prospects for the diagnostics and treatment of the chronic pulmonary hypertension].

Pulmonary hypertension is defined as the rise of mean pressure in the pulmonary artery over 25 mmHg at rest or over 30 mmHg during activity with accompanying increase of pulmonary vascular resistance over 3 WU (Wood's unit). According to the recent WHO classification from 2003 pulmonary hypertension can be categorized as pulmonary arterial hypertension, pulmonary venous hypertension, hypoxic pulmonary hypertension, chronic thromboembolic pulmonary hypertension and pulmonary hypertension from other causes. Because symptoms of the pulmonary hypertension are non-specific, the diagnosis is frequently late. Patients with higher risk of pulmonary hypertension require frequent echocardiographic examination. Treatment of the pulmonary hypertension is rather complex and economically demanding. It should be therefore centralized in specialized units. Decision on the pharmacotherapy is based on the acute pulmonary vasodilatation test. Only patients with the positive test (10% of patients) are indicated to the treatment with calcium channel blockers. In case of negative test, the treatment of choice in NYHA III stadium is bosentam per orally, in the NYHA IV stadium it is epoprostenol intravenously. In patients with chronic thromboembolic pulmonary hypertension, organized thrombotic material should be surgically removed together with the layer of the pulmonary artery (pulmonary endarterectomy) after preceding anticoagulation treatment lasting at least three months. Pulmonary hypertension center of the Cardiocenter of the General teaching hospital is the only unit in the Czech Republic which beside the complex therapy of the pulmonary arterial hypertension can employ also the surgical treatment of the chronic thromboembolic pulmonary hypertension.

Chronic Disease↗

PubMed2006

[Current approach to the diagnostics and therapy of acute coronary syndromes].

The aim of this review is to provide a comprehensive actual overview of the current approach to acute coronary syndromes diagnostics, therapeutics and secondary prevention. Authors stress early diagnosis, risk stratification, indication and timing of interventional therapy. It is not an intention to provide detailed description of all clinical studies implemented recently in the field of acute coronary syndromes.

Angina, Unstable↗

PubMed2006

[Pulmonary endarterectomy--the surgical treatment of chronic thromboembolic pulmonary hypertension].

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) in indicated cases can be successfully treated by the endarterectomy of pulmonary arteries (PEA). Symptomatically not treated CTEPH has highly unfavourable prognosis. Five years survival of patients with mean pulmonary pressure over 50 mmHg is only 10%. PEA was not available in the Czech Republic till 2004, when PEA program was initiated it the Cardiocenter of the General teaching hospital in Prague in collaboration with leading clinics in that field (Prof. Mayer, University of Mainz, BRD). METHODS AND RESULTS: Up-to-date surgical technique, which in various modifications has been used at majority of clinics, was elaborated by Jamieson and Daily at University of California in San Diego. It is based on reverse endarterectomy performed during complete circulatory arrest with brain protection by deep hypothermia. Till September 2005 twelve patients were operated with zero mortality. In one patient a suture of atrial septum defect was necessary to perform along. Average time of the circulatory arrest was 45 minutes; duration of the extracorporal circulation was 334 minutes. Average duration of the operation was 450 minutes. Duration of the mechanical ventilation was in average 45.5 hours. After one month already haemodynamic parameters (mPA, CI, PVR) significantly improved or normalized and the average length in the test of six minutes walking increased by 132 meters. CONCLUSION: PEA represents a treatment method for patients with CTEPH and surgically accessible pulmonary artery obstruction. Centralized care of those patients is a rational necessity enabling to get maximum experience with complicated diagnostics and treatment of those patients. Multidisciplinary collaboration is the essential condition for the success of the program.

Adult↗

PubMed2006

[Vascular involvement in thoracic outlet syndrome].

Vascular involvement in thoracic outlet syndrome is beside neurological symptoms one of the possible manifestations of this disease, typically affecting young, otherwise healthy individuals. The most frequent vascular manifestations of the thoracic outlet syndrome include symptoms related to the venous hypertension with possible outfall to the deep vein thrombosis. Arterial involvement is mostly revealed by the presence of ischemic symptoms. Several key points for the correct diagnosis should be stressed out. They include specific events in patient history, especially previous effort or trauma of the affected upper extremity, typically unilateral. Arterial manifestations are characterized by the absence of ischemic symptoms in other localisations. Deep vein thrombosis occurs in the absence of other distinct precipitating factor and/or hypercoagulable state. Some provocative manoeuvres may help in correct diagnosis assessment, especially in the connection with duplex ultrasound examination. Endovascular procedures both in venous and arterial thoracic outlet syndrome became rather a part of therapeutical process while their role in diagnosis decreasing. The treatment of thoracic outlet syndrome patients is currently evolving into an interdisciplinary approach integrating angiologists, interventional angiologists and vascular surgeons. This complex multidisciplinary approach improves long term results and quality of life of these patients.

Arm↗

PubMed2006

[Prevention of thrombembolic disease in internal medicine].

Although deep vein thrombosis is often considered to be associated with recent surgery, 50-70% of symptomatic thrombembolic events occur in nonsurgical patients. Hospitalization for acute medical illness is independently associated with about eightfold increase in relative risk for venous thrombosis. Thus correct evaluation of risks for thrombembolic disease in individual inpatient and appropriate prophylaxis offers the opportunity to improve the prognosis of acutely ill patient. The most efficient and easy way of pharmacologic prophylaxis is subcutaneous administration of low molecular weight heparin. Only in minority of patients mechanical methods of prevention are sufficient. The optimal duration of thromboprohylaxis in medical patients is unknown and we have to evaluate the changing conditions in each patient.

Hospitalization↗

PubMed2006

[Buerger's disease].

Buerger's disease (Thromboangiitis obliterans--TAO) is a nonatherosclerotic, segmental inflammatory disease that most frequently affects the small and medium-sized arteries and veins in the upper and lower extremities. There exists an extremely strong association between heavy tobacco use and TAO. The histopathology of the involved blood vessels varies according to the chronologic stage of the disease at which the tissue sample is obtained for examination. The histopathology is most likely to be diagnostic at the acute phase of the disease. Buerger's disease typically occurs in young male smokers, with the onset of symptoms before the age of 40 to 45 years. Several published series have shown an increasing prevalence of the disease in women. There is little information on the use of intra-arterial thrombolytic therapy, prostaglandin therapy, or angiogenesis. Discontinuation of tobacco use is the mainstay of treatment. Patients who successfully stop smoking almost never need amputation.

Humans↗

PubMed2006

[Endovascular treatment of acute vascular occlusions].

Acute vascular occlusion is a medical emergency and often a life threatening situation. It is caused by embolization into local arteries, by thrombosis or by occlusion of peripheral arterial bypass grafts. Percutaneous thrombectomy and local thrombolysis represent well established techniques for the treatment of acute limb ischemia and massive deep venous thrombosis especially for ileofemoral and ileocaval occlusion. The new techniques of thrombectomy allow very effective and minimally invasive therapy of acute vascular occlusions.

Acute Disease↗

PubMed2006

[Epidemiology of ischemic diseases of the lower extremities].

The most serious problem in angiology is peripheral arterial occlusive disease. The prevalence of this disease is 2 % in men under 50 years of age and 5 % in men over 70 years of age. Women reach the same numbers approximately 10 years later. The most serious consequence is that in many cases patients with PAOD have also coronary disease and ischemic brain disease. The most common cause of death in patients with PAOD is coronary artery disease (40-60 %); ischemic stroke develops as a cause of death in 10-20% of the patients. About 20-30% of the patients die due to noncardiac factors.

Arterial Occlusive Diseases↗

PubMed2005

[Takotsubo cardiomyopathy: case report and literature review].

The authors present an interesting case report of 69-year-old caucasian woman with Takotsubo cardiomyopathy. Takotsubo cardiomyopathy is a relatively recently described heart syndrome that probably develops due to the direct toxic effect of excessively released catecholamines on cardiac adrenoceptors during emotional or physical stress. The typical features include reversible left ventricular apical dyskinesis, chest pain with ST-T changes on ECG, minimal myocardial enzymatic release and the absence of coronary stenosis on coronary angiogram. Early coronary angiographic examination is highly recommended as the clinical picture of this syndrome mimics acute myocardial infarction. Betablockers are considered to be the treatment of choice.

Aged↗

PubMed2004

Hemodynamic response to laparoscopic cholecystectomy--impacts of increased afterload and ischemic dysfunction of the left ventricle.

The authors describe the results of intra-operative hemodynamic monitoring during laparoscopic cholecystectomy in patients with ischemic left ventricular dysfunction and with significant aortic stenosis. The results in the groups composed of 13 and 12 patients were compared with the findings in 10 young, non-obese, non-smokers without significant cardiovascular history and with normal findings during resting transthoracic echocardiography. Monitoring itself was conducted using transesophageal echocardiography 1) after the induction of anesthesia, 2) after the induction of capnoperitoneum, and 3) after setting the operative anti-Trendelenburg position. The measurements were performed at least in triplicate and the results were processed using ANOVA test. Significant differences were identified in the time course patterns of heart rate, mean arterial pressure, dual product (pressure-rate-product), and cardiac output. In terms of pathophysiology, we believe that the most important achievement was the identification of different time course patterns of individual parameters in the respective groups. The results in the group of patients with aortic stenosis were based particularly on the different time course of the mean arterial pressure, while the results in patients with ischemic disease were more dependent on the time course of the heart rate. Very interesting is a drop of peripheral vascular resistance after positioning of these patients which could be explained only partially by a beta-blocking or ACEI medication. In clinical terms, the most important finding was probably that no complications occurred in the entire group of 35 patients, of which 25 suffered from severe organic cardiopathies.

Aortic Valve Stenosis↗

PubMed2004

Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome Survey.

BACKGROUND: Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation of the substrate globotriaosylceramide in cells throughout the body leads to major organ failure and premature death. In response to the recent introduction of enzyme replacement therapy, the Fabry Outcome Survey (FOS) was established to pool data from European clinics on the natural history of this little-known disease and to monitor the long-term efficacy and safety of treatment. This paper presents the first analysis of the FOS database and provides essential baseline data against which the effects of enzyme replacement can be measured. DESIGN: Baseline data from a cohort of 366 patients from 11 European countries were analysed in terms of demography and clinical manifestations of Fabry disease. RESULTS: Misdiagnosis of Fabry disease is common, and the mean delay from onset of symptoms to correct diagnosis was 13.7 and 16.3 years in males and females, respectively. Although previously thought to have serious manifestations only in hemizygous men, the FOS database has confirmed that females heterozygous for Fabry disease are similarly affected. Furthermore, signs and symptoms of Fabry disease may be present from early childhood. CONCLUSIONS: With the advent of enzyme replacement therapy, it is important that general practitioners and physicians in a range of specialties recognize the signs and symptoms of Fabry disease so that effective treatment can be given. Baseline data from FOS demonstrate that enzyme replacement therapy should not be restricted to hemizygous men, but should be considered for both heterozygous females and children.

Age Factors↗

PubMed2004

Fabry disease: overall effects of agalsidase alfa treatment.

BACKGROUND: Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation of the substrate globotriaosylceramide in cells throughout the body leads to major organ failure and premature death. The Fabry Outcome Survey (FOS) is a European outcomes database which was established to collect data on the natural history of this little-known disease and to monitor the long-term efficacy and safety of enzyme replacement therapy (ERT) with agalsidase alfa. This paper presents the first analysis of the FOS database on the effects of ERT on renal function, heart size, pain and quality of life. DESIGN: The effects of 1 and 2 years of ERT with agalsidase alfa on renal function (assessed by estimated glomerular filtration rate), heart size (assessed by echocardiography), pain (assessed by the Brief Pain Inventory) and quality of life (assessed by the European Quality of Life Questionnaire EQ-5D) were analyzed in a cohort of 545 patients, 314 of whom were receiving treatment (188 for at least 12 months and 92 for at least 24 months; mean duration of treatment, 17 months; maximum duration, 56 months). RESULTS: Treatment with agalsidase alfa stabilized renal function in patients with a mild or moderate deterioration in renal function at baseline, reduced left ventricular size in patients who had an enlarged heart at baseline, and improved pain scores and quality of life. These improvements were similar in hemizygous men and heterozygous women with Fabry disease. CONCLUSIONS: Enzyme replacement therapy with agalsidase alfa leads to significant clinical benefits in patients with Fabry disease, and treatment is likely to alter the natural history of this disorder.

Adult↗

PubMed2004

Tobacco use amongst high school students in the Czech Republic.

OBJECTIVE: We were interested in the prevalence of smoking amongst teen-age students, its possible causes, and their understanding of its associated health risks. METHODS: We constructed a questionnaire that was responded to by a total of 419 students from 5 high schools in Prague, Czech Republic. Students were classified as non-smokers, mild (1-10 cigarettes daily), moderate (11-20 cigarettes daily), and severe smokers (>20 cigarettes daily). The survey also contained questions about passive smoking, motivation for smoking, the understanding of its associated health risks, alcohol consumption, and drugs. RESULTS: We found that amongst 16-20 years old high school students there are 37.5% smokers (38.0% men, and 37.0% women). The majority are mild smokers (82.3%), 15.8% moderate smokers and 1.9% heavy smokers. 29.0% of non-smokers reported passive smoking; i.e. that 65.7% of students are exposed to harmful effect of tobacco smoke. The average onset of smoking is at 14 years of age. The youngest smoker started smoking at the age of 5 years. Parents of 52.0% of students smoke (69.4% of smokers and 41.6% of non-smokers). Most of students know about the risk of lung cancer and cardiovascular diseases (86-99%). CONCLUSIONS: The prevalence of active and passive smoking among high school students is high. Parents smoking is significantly more frequent in teen-age smokers than in non-smokers. We consider the "teen-age" population together with their parents to be the key target for a successful antismoking campaign.

Adolescent↗

PubMed2004

[Use of stem cells for myocardial regeneration].

Remodelling of the left ventricle after myocardial infarction is a major cause of the heart failure development with possible death. Despite application of pharmacological, catheter and surgical interventions and the use of new mechanical devices, the myocardium lost during myocardial infarction cannot be regenerated. Implantation of bone-marrow stem cells into the heart might be a new method to restore myocardial viability. In animal experiments, attempts to replace fibrotic zone by transplanting stem cells have regularly succeeded in reconstituting myocardial structure--cardiomyocytes and capillary vessels. Repair of myocardium using bone-marrow derived multipotent stem cells was shown in experimental and in the first few clinical studies. Current status of our knowledge about the use of stem cells in the myocardial regeneration is described in this article, as well as the cautions, which are necessary during early period of this mode of treatment in humans.

Animals↗

PubMed2004

[Effect of primary coronary angioplasty on long-term prognosis in patients with diabetes mellitus].

OBJECTIVES: To investigate feasibility and safety of primary PCI in diabetic patients. BACKGROUND: Diabetic patients with acute myocardial infarction (AMI) have been shown to be at high risk for adverse clinical outcomes. Limited data is available on long term prognosis of diabetics treated with primary PCI. METHODS: Retrospective analysis of consecutive 67 diabetic patients and 211 non diabetic patients treated with primary PCI from 1/1995 to 12/1999, follow up for 38 +/- 12 months. RESULTS: The baseline characteristics were comparable in both groups. The mean age was 62 years in diabetic patients and 59 years in non diabetic patients. Hypertension (50% vs. 36%, p = 0.05), contraindications to thrombolytic treatment (13.4% vs. 5.7%, p = 0.037), cardiogenic shock (16.4% vs. 7.1%, p = 0.023), multivessel disease (34% vs. 23%, p = 0.07) and longer time delay to treatment (240 vs. 180 min., p = 0.05) were more often present in diabetic group. 47% of diabetic and 42% of nondiabetic patients received stents. The TIMI 2 or 3 flow rates were reached in 91% of diabetic patients and in 90% of nondiabetic patients, but TIMI 2 flow was found more often in diabetics (9% vs. 2.4%, p = 0.016). Higher rate of bleeding complications leading to significant change in the blood count (7.5% vs. 1.4%, p = 0.01) and higher 30 day mortality (11.9% vs. 5.2%, p = 0.05) was observed in diabetic group. However when the shock patients were excluded from the analysis, the 30 day mortality was different insignificantly in both groups (4.5% vs. 2.4%, p = 0.36). During follow up of 259 acute phase survivors 24 patients died. There was a trend to higher total long term mortality (22.3% vs. 13.2%, p = 0.07) and higher rate of nonfatal reinfarction (13.4% vs. 6.2%, p = 0.05) in diabetic group. CONCLUSIONS: Primary PCI is safe and effective treatment of diabetic patients presenting with AMI. The higher rate of slow flow in infarct related artery after PCI observed in diabetics can be one of reasons for higher 30 day mortality in this group. Mean ischemic time in diabetics is behind the 4 hour border, where the possible benefit from reperfusion decreases. The main reason for higher mortality in our diabetic group was the higher rate of cardiogenic shock. Higher risk of bleeding complications at puncture site in diabetic patients can be explained by the lower quality of vessel wall.

Angioplasty, Balloon, Coronary↗

PubMed2004

[Cardiac manifestation of Fabry's disease: current knowledge].

Fabry's disease is a rare lysosomal storage disease caused by the X-linked defect of the enzyme alpha-galactosidase A leading to the intracellular accumulation of glycosphingolipids in various organs and tissues. Cardiac involvement is frequent and, in individuals with some residual enzyme activity, may be the sole manifestation of the disease. Hemizygous men are generally more seriously affected than heterozygous women. The dominant cardiac manifestations include myocardial hypertrophy of the left ventricle, which, in some patients, mimics hypertrophic cardiomypathy. Left ventricular systolic function is usually preserved, on the other hand mild to moderate diastolic dysfunction is regularly detected. Valvular abnormalities are frequently noted. However, hemodynamically significant lesions are rare. Conduction system involvement leads initially to the shortening of atrioventricular conduction, in later stages, with a progression of the disease, antrioventricular blocks and various forms of supraventricular and ventricular arrhythmias appear. Myocardial ischemia in Fabry disease has in most cases a functional origin due to endothelial dysfunction of coronary arteries and also due to the increase oxygen demand of hypertrophied myocardium. The results of so far performed studies with enzyme replacement therapy are promising in preventing further deterioration and even improving function of affected organs.

Fabry Disease↗

PubMed2003

[Effect of delaying reperfusion therapy with PTCA on long term prognosis in patients with acute myocardial infarct].

BACKGROUND: The benefit of thrombolysis in patients with acute myocardial infarction (AMI) strongly depends on the time from onset of symptoms to the initiation of treatment. For AMI patients treated with PTCA this time seems to be important only to a certain time level. The aim of this study was to assess the influence of time to treatment of AMI with coronary angioplasty on short term and long term prognosis. METHODS: We followed 339 consecutive AMI patients treated with coronary angioplasty from 1995 to 1999 in a cardiac care unit. Patients were divided to five groups according to time to treatment. RESULTS: Time to treatment < 90 min. was achieved in 35 (10.5%); 91-210 min. in 105 (31%); 211-330 min. in 72 (21%); 331-690 min. in 74; > 691 min. in 53 (15.5%) patients. Ischemic time (time from symptom onset to reperfusion) in the groups was < 2 h.; 2-4 h.; 4-6 h; 6-12 h; > 12 h. respectively. The ejection fraction of left ventricle 3-5 days after AMI was 50%, 51%, 45%, 40%, 46% and the 30 day mortality was 5.7%, 2.9%, 11.1%, 10.8%, 11.3% in the groups respectively, showing no significant differences between the groups. However the higher rate of TIMI 3 flow was achieved in patients with time to treatment shorter than 3.5 h. compared to patients treated later (93.6% vs. 83.9%, p = 0.007). The lower 30 day mortality (3.6% vs. 11.1%, p = 0.012), lower 3 year mortality (8.6% vs. 19.1%, p = 0.003), lover frequency of heart failure during hospitalisation (11.4% vs. 28.1%, p < 0.001) as well as lower maximal level of released kreatinkinase (32 +/- 29 vs. 44 +/- 39 mukat/l, p = 0.005) was observed in patients treated within 3.5 h. from symptoms onset compared to patients treated later. CONCLUSION: The success rate of primary PTCA to achieve normal flow in infarct related artery is high, but decreases when treatment is started later than 3.5 h. from AMI onset. The short term and long term mortality as well as incidence of heart failure during acute phase is lowest when the intervention was started within 3.5 h. from symptoms onset. Initiation of intervention after 3.5 h. resulted in significant mortality increase, but further delay of treatment had minimal impact on patients prognosis. Great effort needs to be paid to start the primary PTCA within 3.5 h. from AMI onset in as many patients as possible. From our data we can indirectly conclude: patients without a chance for reperfusion with thrombolytic therapy within 4 h. from symptoms onset should be considered candidates for PTCA regardless the time of transportation. In patients with chance to reperfuse infarct related artery within 4 h. from symptoms onset with thrombolytic treatment (thrombolysis needs to be started before 2.5-3rd h.) while having low probability to start PTCA within 3.5 h., the thrombolysis should be given first and PTCA performed later if needed.

Angioplasty, Balloon, Coronary↗