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)

We report a male patient, chromosomal complement 44 XY with Turner's phenotype, who has multiple skeletal, genitourinary and mild cardiac abnormalities, without hypogonadism. This patient developed a diffuse infiltrative pulmonary disease which result in pulmonary fibrosis, respiratory insufficiency and cardiac failure. He has also mixed cryoglobulinemia (Type III) with antigammaglobulin antibodies. The relationship among these problems and his phenotype is discussed. Apparently there is only a coincidental association.
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PMID:[Classical interstitial pneumonitis and mixed cryoglobulinemia in a male with Turner phenotype. Report of a case and review of the literature]. 43 55

Patients with idiopathic pulmonary fibrosis (IPF) inevitably experience declines in functional status that are most frequently due to progressive pulmonary fibrosis. However, the cause of the clinical deterioration is often uncertain, and disease progression is difficult to distinguish from disease-associated complications or adverse effects of therapy. In studies of the clinical course of IPF, mortality is most frequently due to respiratory failure (38.7%); other causes of death include heart failure (14.4%), bronchogenic carcinoma (10.4%), ischemic heart disease (9.5%), infection (6.5%), and pulmonary embolism (3.4%). Other, usually nonfatal, disease-associated complications include pneumothorax, corticosteroid-induced metabolic side effects and myopathy, and therapy-related immunosuppression. In evaluating clinical deterioration in patients with IPF, disease-associated complications and adverse effects of therapy should be distinguished from progressive pulmonary fibrosis. The cause of clinical deterioration will alter the therapeutic intervention required and will influence patient prognosis and duration of survival. This article examines the causes of clinical deterioration in patients with IPF and the diagnostic procedures for assessing disease-associated complications and staging IPF progression.
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PMID:Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. 218 1

Few data are available concerning pulmonary function in patients with severe chronic congestive heart failure. Of 315 patients evaluated for potential cardiac transplantation at UCLA, 132 underwent pulmonary function tests. The latter patients had severe heart failure with a mean left ventricular ejection fraction of 19 percent and mean cardiac index of 2.1 L/min/m2. Diffusion impairment either alone or combined with restrictive and/or obstructive ventilatory defects occurred in 67 percent of the patients evaluated. Diffusion impairment occurred as the sole abnormality in 31 percent of the patients and in combination with a restrictive ventilatory defect in 21 percent. A reduction in diffusing capacity has not been previously described as a frequent finding in patients with chronic congestive heart failure. In contrast to other studies involving patients with acute heart failure, obstructive ventilatory defects were uncommon. None of the lung function abnormalities was associated with smoking status, prior drug use, chest roentgenographic changes, hemodynamic findings, or clinical features, including duration of congestive heart failure. The mechanism for the diffusion impairment is unclear but could be due to chronic passive congestion with pulmonary fibrosis and/or recurrent pulmonary emboli. Recognition of diffusion impairment as a common finding in patients with severe chronic congestive heart failure who are candidates for heart transplantation is important for proper interpretation of possible post-transplant changes in diffusing capacity due to other causes.
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PMID:Ventilatory and diffusion abnormalities in potential heart transplant recipients. 220 36

Type III procollagen peptide (PCP) is a byproduct of type III collagen synthesis and a potential marker of collagen secretion. In chronic diffuse interstitial lung diseases, elevated PCP concentrations have been found in serum as well as in bronchoalveolar lavage fluid. It has been proposed that PCP is a marker of early, active stages of fibrosis. As severe fibrosis is a frequent complication in adult respiratory distress syndrome (ARDS), we investigated PCP in patients with ARDS and compared the results with those from patients requiring mechanical ventilation because of heart failure and after neurosurgical and surgical interventions, and those from spontaneously breathing patients, including healthy volunteers and patients with pneumonia, liver cirrhosis, and renal failure. PCP concentrations in patients with ARDS were extremely elevated compared with those in control subjects (p less than 0.001) and correlated positively with FiO2 (r = 0.71, p less than 0.01). These results support the pathophysiologic concept of early fibrogenesis in ARDS. As preventing pulmonary fibrosis in ARDS is essential in improving survival rate, we believe PCP can be a valuable diagnostic tool in ARDS.
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PMID:Determination of serum concentrations of type III procollagen peptide in mechanically ventilated patients. Pronounced augmented concentrations in the adult respiratory distress syndrome. 224 Aug 30

The peri-operative anaesthetic management of 11 patients with pulmonary fibrosis undergoing single-lung transplantation is presented. Intra-operative problems, the early postoperative phase of recovery and intensive care, and other incidents in which general anaesthesia was required for the management of complications, are featured. Results, both short- and long-term, are mentioned. Major intra-operative events that cause concern appear to be related to the severity of the presenting illness and the development of respiratory failure. Others have reported the development of intra-operative cardiac failure. All cases were successfully managed operatively using conventional one-lung anaesthesia, although resort to partial cardiopulmonary bypass may have been indicated in some. The indications and attitudes to utilising cardiopulmonary bypass in the evolution of techniques for facilitating single-lung transplantation are reviewed.
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PMID:Isolated lung transplantation for pulmonary fibrosis. 225 97

The posterior wall of the intermediate bronchial trunk (PWIB) is visible in most cases on profile thoracic radiographs. A retrospective study showed that it is thickened (over 3 mm) in cases of cardiac failure, right pneumonitis, adenopathy, right bronchopulmonary neoplasias and pulmonary fibrosis, either acute or chronic. The latter etiology had not yet been described in the literature. In our experience, assessment of the thickness of the intermediate bronchus provides a reliable criterium of response to treatment. The pathological mechanisms of intermediate bronchus thickening due to an increase of bronchial wall thickness or of peribronchial interstitial tissue are discussed.
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PMID:[Measurement of the posterior wall of the intermediate bronchus. Diagnostic value]. 232 10

Between February 1988 and December 1989, 15 combined heart-lung, 2 double lung and 5 single lung transplants were performed at our institution for end stage lung disease. The indication for heart-lung transplantation was primary lung disease with associated secondary heart failure in 11 cases, diffuse pulmonary disease with extensive adenopathy of the hilum in 2 cases and profuse and antibiotic-resistant tracheobronchial infection due to Pseudomonas in 2 cases. A double lung transplant was performed in 2 patients with hypertensive emphysema. The indication for a single lung transplantation was emphysema in 2 cases and pulmonary fibrosis in 3 cases; in this last indication, transplantation should be performed on the right side with a slight lengthening of the main bronchus to avoid the side-effects of mediastinal shift. There were 2 early deaths, 7 secondary deaths (from the 2nd to the 5th month) due to viral or bacterial infectious complications, and 1 late death in the 7th month (infection due to a syncitial virus). All 12 surviving patients have an excellent functional result; the size of the tracheal or bronchial anastomosis ranges from 85% to 100% of normal. From this experience, we conclude that specificity and severity of lung hazards are mainly related to bronchial infection, dependence on steroids and pleural adhesions. Moreover, posttransplant pulmonary oedema, mucociliary dysfunction and the differential diagnosis between rejection and infection require careful endobronchial suction and periodical sampling.
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PMID:Lung and heart-lung transplantation for end-stage lung disease. The Bordeaux Lung and Heart-Lung Transplant Group. 236 Oct 20

Since January 1988, the Bordeaux group has performed 15 transplantations for lung disease: 9 heart-lung transplants, 1 heart + left lung, 1 double lung, 2 right lungs and 2 left lungs. The transplantations were performed for pulmonary emphysema (10 cases), pulmonary artery hypertension (2 cases), cystic fibrosis (1 case), pulmonary fibrosis (2 cases). Cardiopulmonary transplantation was not always performed because of associated heart failure but sometimes because of large intrahilar adenopathy or intractable bronchial infection. Pulmonary transplantation is recommended on the right side in cases of pulmonary fibrosis. One patient died postoperatively (ischaemia of the transplant). Four others died during the 2nd and 3rd months from poorly defined but probably infectious pulmonary syndromes. The tracheobronchial patency of the 10 survivors was 80% or 100% of the predicted value. The respiratory functional result was excellent in the short and intermediate term. Specific difficulties essentially consisted of pleural symphyses, hilar adenopathy, bronchial infection, steroid dependence of certain subjects, the difficulty of identifying the cause and treating lung opacities during the 2nd and 3rd months.
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PMID:[Lung and heart-lung transplantation in respiratory tract diseases. Evaluation and development of the indications based on our first 15 cases]. 258 94

Establishing the diagnosis of drug-induced pneumonitis is always difficult and requires that the following criteria be met: administration of the drug on a long-term basis; knowledge that the drug is able to induce pulmonary disorders; occurrence during therapy of interstitial pneumonitis with clinical, radiological and functional characteristics of this type of lung disease; exclusion of all other causes of interstitial pneumonitis (cardiac failure, infections, collagen vascular diseases, malignancies); bronchoalveolar lavage specimen, revealing lymphocytosis with an inverted CD4/CD8 lymphocyte ratio, isolated or associated with neutrophil and/or eosinophil alveolitis; finally, full recovery within several weeks or months after drug withdrawal unless irreversible pulmonary fibrosis has occurred. Certain specific characteristics correspond to the therapeutic class of the drug, i.e. antimicrobial, cardiovascular, antiinflammatory, neurological, metabolic, antiallergy or some other drugs.
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PMID:[Drug-induced pneumopathies (excluding cytostatic drugs)]. 261 Apr 52

Amiodarone has remarkable efficacy, but it also has a high incidence of severe side effects. Nevertheless, it is extensively used. The findings of an amiodarone treatment follow-up period of one to 72 months (mean 19 +/- 17) in 95 patients with recurrent life threatening arrhythmias resistant to other antiarrhythmic drugs are described. The mean loading dose of 800 mg/day for one week was followed by an average dose of 600 mg/day for two weeks. The long term daily dose was 400 mg in 80 patients and 200 mg in the remaining 15 patients. Amiodarone was particularly effective in suppressing complex ventricular arrhythmias. It also decreased premature ventricular beats by 83% and atrial premature beats by 41%. Supraventricular tachycardias were completely controlled and the ventricular response to atrial fibrillation was slowed. Twelve of the 95 patients (12.6%) died while taking amiodarone, two of sudden death and 10 of heart failure. Side effects were recorded in 77 (81%) patients. They were generally dose related and subsided with a decrease in dose or discontinuation of the medication. There was a correlation between the concentration of rT3 and the significance of the side effects. Thirty-nine patients stopped taking amiodarone, however, only 14 of these stopped because of toxicity: five developed pulmonary fibrosis; three had neurological toxicity; two had bradyarrhythmias; two developed hepatic dysfunction; one had hypothyroidism; and one patient suffered an aggravation of a pre-existent heart failure. It was concluded that amiodarone is a very effective treatment for supraventricular and ventricular arrhythmias. However, it does have numerous and severe side effects.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long term efficacy and toxicity of amiodarone in the treatment of refractory cardiac arrhythmias. 270 79


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