Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary function and cardiopulmonary complications were studied in a group of 40 patients with cystic fibrosis who reached the age of 25 years. Mean values for vital capacity (VC), functional residual capacity, residual volume (RV), the ratio of RV over total lung capacity (RV/TLC), conductance, and the ratio of the forced expiratory volume in one second over VC were abnormal. There was a variable pattern of progression from patient to patient. The men differed from the women only in that they had a significantly larger TLC and inspiratory capacity than the women. The resultant preservation of VC may have an advantage for survival in those patients in whom it is observed. Pseudomonas aeruginosa was encountered with increasing frequency with age. Massive hemoptysis did not result in early death. The occurrence of rightsided heart failure secondary to cor pulmonale, with or without respiratory failure, was a poor prognostic sign.
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PMID:Pulmonary function and morbidity in 40 adult patients with cystic fibrosis. 10 32

Thirty-one patients with cystic fibrosis of varying severity were examined by echocardiography. Right ventricular dimension (RVD) was above upper normal limit in 14 patients and right ventricular dimension index (RVD index) was higher than the upper normal limit in 11 patients. Furthermore, there was a significant relationship between increasing RVD index and 1) decreasing forced vital capacity (FVC) both actual test results and average 6 months values; and 2) decreasing peak-expiratory flow rate (PEFR) both actual test results and average 6 months values. This observation suggests a persistent heart involvement. Five patients had either heart failure and/or electrocardiographic evidence of right ventricular abnormality. These patients had increased RVD index and one patient with the highest RVD index died 8 weeks after the examination. The present study has shown the usefulness of echocardiographic measurement of right ventricular dimension and of septal motion in assessing cor pulmonale, before development of electrocardiographic abnormalities and right heart failure.
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PMID:Assessment of cor pulmonale in cystic fibrosis by echocardiography. 14 4

Survival studies were done on 36 children with cystic fibrosis and heart failure. Thirty percent did not survive the first four weeks, and the median survival for the group was between two and three months. By the end of the first year from the onset of failure, 74 percent had died and at 30 months, 87 percent had died.
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PMID:Cystic fibrosis complicated by heart failure. 113 33

Growing experience in terms of immunosuppression, recipient and donor selection as well as organ preservation has established thoracic organ transplantation as a therapeutic option for many children with end-stage cardiopulmonary diseases. While dilated cardiomyopathy and isolated myocardial failure represent the main indications for cardiac transplantation, replacement of the lungs or heart and lungs is necessitated in cystic fibrosis, primary and secondary pulmonary hypertension as well as some types of complex congenital heart defects involving the pulmonary arteries. We have performed a total of 20 heart, 4 heart-lung, 2 single lung and 1 double lung transplantation in the paediatric group up to 17 years of age. While with respect to the limited experience worldwide, early mortality after lung and heart-lung transplantation is still high (50%), long-term results in isolated cardiac transplantation using triple drug immunosuppression are excellent (79% survival after 6 years) without major impairment of renal function, arterial blood pressure, growth development and physical rehabilitation as well as social reintegration. Freedom from graft atherosclerosis of the allografted heart is documented over a 5 year follow up, while no data are available on the incidence of obliterative bronchiolitis after lung transplantation in the paediatric group. Despite only limited evidence of long-term dysfunction, diagnosis and prevention of chronic rejection should be given utmost attention to allow for a normal life span in this younger age group.
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PMID:Thoracic organ transplantation in the paediatric age group. The Hannover experience. 134 7

Cyclosporine therapy after heart and lung transplantation implies a number of specific aspects such as: kidney deficiency associated to heart failure, which delays its use as for cardiac transplantation; intestinal absorption disturbances that could be linked to the cystic fibrosis disease for lung transplantation. For both types of transplantation, local efficiency could indicate an interesting, but still unexplored therapeutic effect. Finally, it seems that the immunosuppressive effect could be linked to important pharmacological effect on calcium and could explain the specific aspects of rejection of patients under cyclosporine.
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PMID:[Cyclosporine. Obscure sides of cardiac and pulmonary transplantations]. 149 96

Since December 1985, we have performed 38 transplantations: cardiac (CT) n: 31, cardiopulmonary (CPT) n: 1, or bipulmonary (BPT) n: 6. There were 31 male and 7 female patients, aged 7 to 62, mean 46. In the cardiac group, the cardiomyopathy was primitive in 13, ischemic in 16, valvular in 2. Five patients had undergone one or more previous operations. Three patients had a biventricular assist device (1,6 and 7 days before transplant) for acute cardiac failure. The indication of CPT or BPT was pulmonary artery hypertension (1), silicosis (1), cystic fibrosis (4). There were 4 post-operative deaths in the CT group (12.9%); failure of graft, low cardiac output, pulmonary artery hypertension by multiple pulmonary thrombosis, and 2 deaths in the CPT and BPT groups (28%). The mean post-operative hospital stay was one month. All patients with CT were treated by an initial maintenance bitherapy protocol (cyclosporine, steroids) and observed by myocardial biopsies and echocardiograms. In 40 per cent of the patients, Azathioprine was subsequently added. The patients had 2.1 rejection episode/patient/year, either spontaneously reversed of treated medically. There were two late deaths (2 and 7 months) by refractory rejection. 78 per cent of the patients were alive one year after transplant. All survivors have recovered a normal life, some of them with full-time work.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Heart and heart-lung transplantation. 3 years' experience in Timone CHU (Marseilles 1985-1988)]. 210 56

Cardiomyopathy (CMP) was found in 26 children with cystic fibrosis (CF), 24 of them died, the majority of them during the first 3 years of life. Only 4 of them were older than 10 years. 2 children are living. CMP must be suspected in young children with CF and early heart failure. When CMP is the cause of sudden death, CF has to be suspected. The combination of changes in skeletal and cardiac muscles in CF is reported here for the first time.
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PMID:Cardiomyopathy and changes of skeletal muscles in cystic fibrosis. 213 Jun 94

Proper documentation of new antimicrobial drugs for governmental registration authorities includes extensive pharmacokinetic studies. Pharmacokinetics represents the bridge between the in vitro and in vivo phases of drug development. Both healthy human volunteers and patients must be studied, the former during the initial stages of the pharmacokinetic studies. The documentation should give information on the following: absorption from the gastrointestinal tract, bioavailability, pharmacokinetic model, impact of increasing doses (oral and intravenous), metabolism, routes and degree of elimination, interaction with food and other drugs, impact of the steady state, and serum protein binding. Basic pharmacokinetic parameters used are the serum half-life, clearance, distribution volume and dose dependence. The bioavailability of oral doses must be determined using the same dose sizes and subjects. Data on extravascular penetration should also be included in complete documentation. Key diseases in which the pharmacokinetics should be studied are reduced renal and liver function, heart failure, pregnancy, cystic fibrosis and intestinal diseases. The consequences of low age (e.g. newborns) and old age also require some attention.
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PMID:Requirements for the documentation of pharmacokinetic properties of antimicrobial agents. 222 81

In a 2-year-old boy with untreated cystic fibrosis, an acute deterioration of his chronic respiratory insufficiency developed due to bilateral pneumonia. This condition caused acute right-sided heart failure and nontransmural myocardial infarction of the inferior wall. In concordance with this diagnosis, a marked increase of CPK-MB levels combined with transient severe ischemia on the ECG and the absence of myocardial injury at echocardiography was seen. At 3 years follow-up, he was in good clinical condition.
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PMID:Nontransmural myocardial infarction as a complication of untreated cystic fibrosis. 232 73

While fifty years ago 20 p. 100 of cystic fibrosis patients only reached the age of one year, more than 50 p. 100 of the patients now live more than twenty years. The clinical manifestations of cystic fibrosis are more diverse in adults than in children, so that the diagnosis might concern several specialties. In actual fact, only 3 to 7 p. 100 of cystic fibroses are diagnosed after thirteen to sixteen years, and in half the cases the symptoms had been present before the age of one year. In adults, the respiratory manifestations of cystic fibrosis are predominant, whereas the gastrointestinal manifestations tend to be blurred. Radiography of the chest shows interstitial lesions (opacities, cystic images, disorders of ventilation), principally located in the right side and the apex. The most common functional defect is an obstructive syndrome corresponding to a gradual involvement of the peripheral airways. A number of complications may develop, including recurrent Pseudomonas infection of the lung, pneumothorax, heart failure, malnutrition, liver cirrhosis, episodes of intestinal occlusion, etc. The longer life span of these patients raises the problems of diabetes with its vascular complications, infertility or pregnancy, social and professional insertion, and so forth. The prognosis of cystic fibrosis in adults depends on the date the diagnosis was made, on the therapeutic follow-up and on the creation of specialized centres. The control of Pseudomonas infections and the development of lung transplantation are the main advances to be expected.
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PMID:[Cystic fibrosis in adults]. 236 14


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