Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We use the hyperbolic relationship between cytosolic [ADP] and the rate of phosphocreatine (PCr) resynthesis after exercise to estimate the apparent maximum rate of oxidative ATP synthesis (QMAX). We examine data from some human diseases in which mitochondrial oxidation may be impaired (due to reduced mitochondrial numbers, intrinsic mitochondrial defect or impaired vascular supply). Muscle responds to impaired oxidation by stimulating anaerobic ATP synthesis and/or by increasing [ADP], the stimulus to the mitochondrion. However, these responses interact: [ADP] depends on pH and [PCr], and lactic acid production tends to lower [ADP] (by lowering pH), while proton efflux has the opposite effect. We identify four patterns of results: (A) in mitochondrial myopathy, apparent QMAX is reduced and [ADP] is appropriately increased, because increased proton efflux reduces the pH change in exercise despite increased lactic acid production; (B) in some conditions (e.g., cyanotic congenital heart disease) apparent QMAX is reduced but there is no compensatory rise in [ADP], probably because anaerobic ATP synthesis during exercise is increased without increase in proton efflux; (C) in other conditions (e.g., myotonic dystrophy) [ADP] is increased during exercise but apparent QMAX is normal, suggesting either an increase in proton efflux and/or decrease in anaerobic ATP synthesis during exercise; (D) there are also conditions (e.g., respiratory failure) where, despite impaired oxygen supply, both apparent QMAX and end-exercise [ADP] are normal. We also discuss the metabolic conditions under which end-exercise [ADP] is increased by a mitochondrial defect.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Quantitative analysis by 31P magnetic resonance spectroscopy of abnormal mitochondrial oxidation in skeletal muscle during recovery from exercise. 826 62

Persistent or recurrent acute allograft rejection (AR) refractory to high-dose steroid therapy can adversely affect long-term outcomes of heart-lung (HLT), bilateral-lung (BLT), and single-lung (SLT) transplantations. The use of total lymphoid irradiation (TLI) for the management of refractory acute AR in six transplant recipients (two men, four women; mean age, 29.8 +/- 3.8 years) is detailed. There are two HLT (primary pulmonary hypertension [PPH], cystic fibrosis [CF]), 1 BLT (pulmonary hypertension postventricular septal defect repair), and 3 SLT (sarcoid, PPH, congenital heart disease with atrial septal defect) recipients. Refractory AR is defined as persistent rejection unresponsive to high-dose steroid therapy in all cases. The BLT and SLT recipients had at least two moderate and one mild AR events per patient. The HLT recipients had at least two moderate acute heart and one severe and one mild asynchronous acute lung rejection events per patient. A total of 800 cGy of total lymphoid irradiation (TLI) was administered over a 5-week period. Mild and transient leukopenia was the only observed side effect. The patient with PPH received TLI 313 days after HLT for recurrent AR at another institution and died of ARDS 4 weeks after completing TLI. The patient with CF received TLI 707 days after HLT and died 457 days after TLI of severe obliterative bronchiolitis (OB) with multiorgan failure. The patient with BLT received TLI 176 days after transplant and died 372 days after TLI of respiratory failure related to severe rejection. One patient with SLT received TLI 78 days after transplant and died 679 days after TLI of severe acute AR. The two remaining patients with SLTs have been free from acute AR for more than 4 years. The patient with sarcoidosis received TLI 37 days after SLT following a clinical rejection event and two severe acute AR events. He is alive with normal lung function 5 years later. The patient with PPH received TLI 108 days after SLT following three moderate acute AR events and is alive with stable OB 4 years later. These limited preliminary results suggest that TLI has merit for the treatment of intractable acute AR following HLT and lung transplantation.
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PMID:Total lymphoid irradiation for refractory acute rejection in heart-lung and lung allografts. 862 53

Extracorporeal membrane oxygenation (ECMO) was initially developed for respiratory failure. Its use, however, has evolved into an excellent method of preoperative and postoperative support in the treatment of infants and children with acquired and congenital heart disease. Along with ECMO, the left ventricular assist device (LVAD) and the intraaortic balloon pump (IABP) have also found a place in the management of paediatric patients with heart failure. This report documents 15 patients who were treated with one or a combination of these mechanical devices, either preoperatively or postoperatively. There is a 74% survival rate and the long-term outcome has been excellent in most cases. The use of heparin-coated devices and tight regulation of heparin has allowed the transfer of infants and children from standard cardiopulmonary bypass to assist devices in the operating room. Mechanical devices are an essential adjunct for the preoperative and postoperative treatment of infants and children with cardiac disease.
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PMID:Mechanical circulatory assistance in paediatric patients with cardiac failure. 863 45

Pregnancy increases the risk for respiratory failure from to numerous pulmonary diseases. Adult respiratory distress syndrome, aspiration, venous air embolism, asthma, thromboembolism, and heart disease are etiologies shared by non-pregnant women. However, their management is complicated by complex physiologic changes during pregnancy. Amniotic fluid embolism and tocolytic-induced pulmonary edema are unique to pregnancy and must be added to the list of causes of respiratory failure. Diagnostics and supportive care is difficult and must be directed with the mother and the fetus in mind. This dictates a thorough understanding of maternal physiology, and the safety of drug use during pregnancy.
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PMID:Acute respiratory failure in pregnancy. 863 95

Seventy one patients with active pulmonary tuberculosis who died during the past 5 years (1989 to 1993) were evaluated on their causes of death. Twenty two patients (31%) died directly of tuberculosis, and among them, 18 patients (81%) of 22 patients who died of tuberculosis) had very advanced tuberculosis. The majority of them (64%) were old age over 70 years and were bedridden due mostly to cerebrovascular injuries. The serum level of albumin was low in all 17 patients in whom it was measured. Establishment of diagnosis of tuberculosis was delayed over one month after the onset of symptoms in 59% of patients who died of severe disease. Sixty one percent (11/18) of patients died within the first month after the initiation of chemotherapy and about 90% (16/18) died within 3 months. Two patients died from massive hemoptysis and other patients died of either respiratory failure or tuberculosis meningitis. From these observations it was found that very advanced tuberculosis was the major cause of death in patients who died of tuberculosis and that the advanced disease was chiefly caused by the delay on the establishment of diagnosis, and it was most important to detect tuberculosis as early as possible, with regular check up of chest X-ray and frequent examination for AFB (acid-fast bacilli) for tuberculosis suspected patients. On the other hand, the majority of patients (49/71) died of complicating medical problem unrelated to tuberculosis. Seventeen patients died from malignancy (seven lung cancer, four lymphoma, two laryngeal cancer, etc). Ten deaths were the result of bacterial superinfection. Other patients died from respiratory failure due to COPD, arteiosclerotic heart disease, or cerebrovascular injuries, etc. Two patients of old age died of hepatic failure possibly caused by adverse reaction of TB chemotherapy. It was found that diseases unrelated to tuberculosis were the cause of death in approximately 70% of patients with active tuberculosis, and it should be emphasized to detect early and to treat these diseases, in particular malignancy. And it is also imperative that the chemotherapy for TB must be instituted very carefully with frequent monitoring of liver function in patients with old age.
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PMID:[Clinical evaluation on causes of death in patients with active pulmonary tuberculosis]. 868 6

Despite the increasing use of Doppler echocardiographic (DE) techniques to determine pulmonary arterial pressure in the neonate undergoing intensive care, there have been no studies comparing their repeatability in this population. Our objective was to compare the repeatability of four such techniques in neonates. The study was conducted in two regional neonatal units serving the North East of England. Group A (repeatability between observers): Two experienced observers performed detailed DE examinations, one directly after the other. Group B (within observer repeatability/temporal variability): One observer performed two examinations 1 hour apart. Group A comprised 15 preterm babies (26-36 weeks' gestation, 975-2915 g), most with mild respiratory failure; 4 healthy term babies; and 7 with congenital heart disease, in whom tricuspid regurgitation (TR) only was measured. Their ages were 18 hours to 12 days. Group B comprised 11 babies aged 12-64 hours with moderate to severe respiratory failure; 10 were preterm (26-36 weeks, 785-2800 g). We recorded four measurements: (1) Peak velocity of TR in m/s; (2) peak left-to-right ductal flow velocity (PDAmax in m/s); (3) TPV/RVET ratio; and (4) PEP/RVET ratio, where TPV = time to peak velocity at the pulmonary valve, PEP = right ventricular preejection period, and RVET = right ventricular ejection time. The Bland-Altman analysis was used to produce the coefficient of repeatability (CR: 95% confidence limits of repeatability), also expressed as a repeatability index (CR/mean value) and as a number of "confidence steps"-a measure of sensitivity of the technique to hemodynamic change (range of values within the population/CR). Between-observer and within-observer repeatabilities were similar. Within-observer CR and index (%) results were for TR +/- 0.26 m/s (9%); for PDAmax, +/- 0.48 m/s (39%); TPV/RVET 0.1:1.0 (34%), PEP/RVET 0.12:1.00 (36%). TR and PDAmax had the largest number of confidence steps in the expected range of values (TR 8.5; PDA max 6.5; TPV/RVET 3.2; PEP/RVET 3.2). The most repeatable technique was TR, but PDAmax would also be useful for a serial study owing to the potential for large change. Systolic time interval ratios were less repeatable and likely to be less sensitive indicators of hemodynamic change.
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PMID:Estimation of pulmonary arterial pressure in the newborn: study of the repeatability of four Doppler echocardiographic techniques. 878 Oct 85

Open lung biopsy has proven beneficial in the treatment of life-threatening pulmonary diseases. Its safety and efficacy in infants and children placed on extracorporeal membrane oxygenation (ECMO) for severe respiratory failure is not known. The authors reviewed eight cases (4 neonates, 3 infants, 1 child) who underwent open lung biopsy while on ECMO. The primary diagnoses were pneumonia (4), lymphoma (1), primary pulmonary hypertension (1), and complex congenital heart disease (2). The patients underwent biopsy after they had been on ECMO an average of 9.6 days (range, 1 to 14 days). Biopsy results confirmed the clinical diagnosis in five patients, two of whom had irreversible alveolar destruction resulting in ECMO withdrawal. Three patients had pathological diagnoses, which resulted in major therapy revisions (1 fungal infection and 2 noninfectious lesions that required steroid treatment). The overall average duration of ECMO treatment was 16.3 days (range, 10 to 24 days). Three patients were weaned successfully from ECMO, but only one infant survived to discharge. One nonlethal bleeding complication occurred after biopsy. Open lung biopsy is well tolerated during ECMO. It accurately determines pulmonary pathology and provides valuable prognostic information. Earlier biopsy for patients whose diagnoses are uncertain or who are not responding to ECMO may improve the mortality rate for this high-risk group.
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PMID:Open lung biopsy in pediatric patients on extracorporeal membrane oxygenation. 972 18

Aortopulmonary collaterals occur in a variety of congenital heart diseases, in chronic pulmonary infection and abscesses, in association with lung tumors, and after multiple pulmonary emboli. In patients with congenital cyanotic heart disease aortopulmonary collaterals mainly occur in conditions with reduced pulmonary blood flow. We investigated 12 preterm low-birth-weight infants, gestational age 29.3+/-3.3 weeks, with respiratory failure who suffered from moderate to severe chronic lung disease after a period of mechanical ventilation. All patients developed aortopulmonary collaterals after closure of a patent ductus arteriosus. Aortopulmonary collaterals could be displayed clearly by color Doppler echocardiography and originated mainly from the descending aorta or the aortic arch. Hypoxic and hypercapnic episodes favored the development of aortopulmonary collaterals, which disappeared after pulmonary hemodynamics and respiratory function had improved. In only one patient coiling of a large col lateral vessel had to be performed. Systemic-to-pulmonary collateral vessels potentially aggravate chronic lung disease by increasing collateral pulmonary blood flow and reducing lung compliance. We conclude that aortopulmonary collaterals occur in bronchopulmonary dysplasia and can cause major problems in ventilated premature infants. Echocardiographic evaluation is important to prevent aggravation of chronic lung disease of infants at risk.
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PMID:Echocardiographic evidence of aortopulmonary collaterals in premature infants after closure of ductus arteriosus. 992 77

After lung lobectomy or pneumonectomy, the mediastinal shift and diaphragmatic elevation are occurred. Because this phenomenon may affect the heart positional change, we studied the electrocardiographic QRS axis in the frontal plane (from leads I and III) and the postoperative arrhythmia. Seventy three patients who had no heart disease including arrhythmia before the surgery were recorded their electrocardiogram (ECG) before their surgery and after their discharge. When the postoperative ECG was recorded, they had no respiratory failure nor cancer recurrence, and their lungs were fully expanded in their thoracic cages. After right upper lobectomy (19 cases), the axis was twisted rightward slightly (2.1 degrees). Right middle lobectomy (2 cases, 9.5 degrees) and right upper and middle lobectomies (3 cases, 7.3 degrees) twisted the heart axes more rightwards. Right lower lobectomy (12 cases, -1.0 degree) and right middle and lower lobectomies (3 cases, -17.7 degrees) contorted their axes leftwards and right pneumonectomy (5 cases, 31.4 degrees) rightwards. The axes were turned rightwards after the left upper lobectomy (18 cases, 2.8 degrees) and the left lower lobectomy (7 cases, 3.9 degrees). Left pneumonectomy (4 cases, -4.0 degrees) twisted the axis leftwards. After the surgery, arrhythmias were recorded in 14 cases and, among these patients, 5 cases were required the oral anti-arrhythmic medication. Most of these cases changed their heart axes after the surgery and it is suggested that the axial deviation may contribute to their postoperative arrhythmia.
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PMID:[Lung resection affects the postoperative arrhythmia and electrocardiographic axis deviation]. 1045 63

Severe CAP is a life-threatening condition defined by the presence of respiratory failure or symptoms of severe sepsis or septic shock. It accounts for approximately 10% of hospitalized patients with CAP. The majority of patients with severe pneumonia have underlying comorbid illnesses, with COPD, alcoholism, chronic heart disease, and diabetes mellitus being the most frequent. S. pneumoniae, Legionella spp, GNEB (especially K. pneumoniae), H. influenzae, S. aureus/spp, Mycoplasma pneumoniae, respiratory viruses (especially influenza viruses), and P. aeruginosa represent the most important causative organisms of severe CAP. Rapid initiation of appropriate antimicrobial treatment is crucial for a favorable outcome. Initial antimicrobial treatment should be based on an epidemiological (empiric) approach. Microbial investigation may be helpful in the individual case but is probably more useful to define local antimicrobial policies based on local epidemiologic and susceptibility patterns. Mortality rates range from 21% to 54%. The most important prognostic factors include general health state of the patient, appropriateness of initial antimicrobial treatment, and the existence of bacteremia, as well as factors reflecting severe respiratory failure, severe sepsis, septic hypotension or shock, and the extent of infiltrates in chest radiograph. Initial antimicrobial treatment should consist of a second (or third) generation cephalosporin and erythromycin. Modifications of this basic regimen should be considered in the presence of distinct comorbid conditions and risk factors for distinct pathogens. Promising new approaches of nonantimicrobial treatment, including noninvasive ventilation, treatment of hypoxemia, and immunomodulation, are under investigation.
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PMID:Severe community-acquired pneumonia. 1051 5


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