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)

Two patients with hematogenous brain abscesses demonstrated by computerized tomographic (CT) scans were successfully treated without surgical intervention. The first patient had congenital cyanotic heart disease and multiple brain abscesses complicated by presumed rupture into the ventricles. By the time these clinical diagnoses were established, she had shown substantial improvement after antibiotic therapy alone. The second patient with bronchiectasis had a single abscess in the dominant hemisphere. Medical management was successful. Subsequent CT scans in both patients provided reassurance that clinical improvement was accompanied by anatomical healing. The use of noninvasive diagnostic techniques should encourage the formulation of new criteria for the management of cerebral abscess in carefully evaluated and closely observed patients.
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PMID:Use of computerized tomography in nonsurgical management of brain abscess. 42 May 98

Chronic cor pulmonale is more prevalent in northern India than in the south. It is equally common in men and in women and accounts for 20% of all admissions for heart disorder in Delhi. In a study of 766 patients (239 men and 527 women) carried out over a 15-year period there were some striking sex differences. Some 75% of men and 10% of women smoked. The women came from the poorest class and all of them cooked from an early age over smoky and primitive fireplaces in ill-ventilated huts, while only 7% of the men cooked their own food. Chronic bronchitis and bronchiectasis were the commonest associated lung disorders in both sexes. The women developed heart failure 10-15 years earlier and showed more severe congestive failure with larger hearts and greater derangement of pulmonary function. It is concluded that the cause of chronic cor pulmonale in women in Delhi was damage to the lungs from exposure to smoky cooking fuels from girlhood onwards, followed by repeated chest infections.
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PMID:Sex differences in chronic cor pulmonale in delhi. 99 Jan 63

Twenty patients with massive or recurrent hemoptysis underwent percutaneous transcatheter embolotherapy between 1979 and 1986 for the following diseases: cavitary aspergillosis (n = 4); cystic fibrosis (n = 4); tuberculosis (n = 3); bronchogenic carcinoma (n = 3); bronchiectasis (n = 3); small cell lung carcinoma 6 years after irradiation (n = 1); congenital heart disease, after Glenn and Blalock anastomoses (n = 1); and unknown interstitial disease (n = 1). Bronchial arteries were embolized in all but one patient. In nine patients (45%) nonbronchial systemic collateral arteries contributed significantly to areas of pathologic pulmonary tissue and frequently were the major arterial supply. These nonbronchial systemic collaterals included branches of the subclavian and axillary arteries (n = 7), intercostal arteries (n = 5), and phrenic arteries (n = 3) and accounted for 59.5% of the total number of arteries embolized. Recognition and occlusion of nonbronchial systemic collaterals providing blood to hypervascular pulmonary lesions is essential for successful percutaneous embolotherapy of hemoptysis.
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PMID:Nonbronchial systemic collateral arteries: significance in percutaneous embolotherapy for hemoptysis. 361 66

AA-protein was identified by SDS-acrylamide electrophoresis in amyloid fibrils fixed in formalin after isolation from fresh-frozen tissues obtained from patients with familial Mediterranean fever (FMF) amyloidosis and idiopathic AA-amyloidosis and, following deparaffination, rehydration and homogenization of embedded formalin-fixed tissues of old autopsy cases of the hereditary amyloidosis of FMF and amyloidosis acquired in association with tuberculosis, bronchiectasis, and rheumatoid arthritis. That AA-protein is unaltered by formalin was firmly established by agar gel diffusion using specific rabbit anti-AA serum. By contrast, AL proteins could not be demonstrated either in formalin-fixed amyloid fibrils derived from fresh-frozen tissues of a patient with presumably AL-amyloidosis dominated by cardiomegaly and one with AL-kappa amyloidosis or in blocks of cases of familial neuropathic amyloidosis, multiple myeloma, and idiopathic amyloidosis with cardiopathy. AA-protein is not denatured by formalin and retains its typical electrophoretic, chromatographic, and immunologic characteristics even 30 years after fixation and paraffin-embedding.
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PMID:Demonstration of AA-protein in formalin-fixed, paraffin-embedded tissues. 706 12

Heart-lung transplantation is a complex, expensive, and resource-intensive procedure. It is performed more often where there is coexistence of end-stage pulmonary disease with advanced cardiac disease that is not secondary to pulmonary hypertension (e.g., some forms of congenital heart disease, some situations with Eisenmenger's syndrome, and possibly the coexistence of end-stage heart and end-stage lung disease). Available data indicate that some disease states are associated with a higher probability of successful outcome after HLT than are others. Objectively validated and reliable patient selection criteria cannot be obtained from the current medical literature. Expert opinion provided to OHTA contained varying degrees of detail and uniformity with respect to patient selection (Tables 11 and 12). While most agree that "irreversible cardiopulmonary disease" is an indication for this procedure, there are no published data that have provided details as to how patients so designated were selected as candidates for HLT. For example, for chronic obstructive pulmonary disease (emphysema), neither expert opinion nor published studies of HLT provided data indicating the stage of the disease at which a decision to transplant is reasonable and objectively justifiable. A similar lack of detail for cystic fibrosis, bronchiectasis, pulmonary fibrosis, sarcoidosis, asbestosis, and other diseases was noted. Thus, there are insufficient data regarding transplant recipients to permit the development of objective criteria for a threshold for HLT in the various disease states. The circumstances are more problematic regarding suggested contraindications for HLT. While objective survival rates obtained from registry data indicated that survival bore little relationship to recipient age, various opinions provided to OHTA specified a maximum age for HLT candidates, ranging from under 45 years to 60 years. Adverse factors and contraindications, such as "other significant disease," "recurrent pulmonary emboli," "hepatic disease," "infection," and "insulin-dependent diabetes" were nonspecific and unsupported by published, objective evidence. More ambiguous contraindications included "psychiatric illness," "poor family support structure," "history of noncompliance with medical regimens," and lack of "emotional stability." While psychiatric, psychologic, or sociologic evaluations of potential HLT recipients undeniably have relevance to patient selection, use of such criteria should be supported at least by evidence of generally consistent application across the transplant community. Of note was the recommended requirement that the recipient possess "adequate financial resources," which implies that the benefits of HLT are not expected to be equitably provided to the public.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Institutional and patient criteria for heart-lung transplantation. 771 18

We assessed the long-term results of our experience with 109 patients with end-stage cardiopulmonary disease who underwent primary combined heart-lung transplantation at Stanford University Medical Center between March 1981 and January 1994. Average recipient age was 31 +/- 10 years (mean +/- standard deviation) median, 31 years; range, 1 month to 52 years. Recipient diagnoses included primary pulmonary hypertension (31%), Eisenmenger's syndrome (39%), complex congenital heart disease (8%), cystic fibrosis (14%), bronchiectasis (2%), and emphysema (3%). Immunosuppression was with cyclosporine and a tapering regimen of corticosteroids. In 1986 azathioprine was added, and since 1987 induction therapy with OKT3 has been employed. Actuarial survival rates at 1, 5, and 10 years were 68% +/- 4.6%, 43% +/- 5.4%, and 23% +/- 8.1%, respectively (mean +/- 1 standard error of the mean). Fourteen deaths occurred in the hospital for an operative mortality rate of 12.8% +/- 3.3%, and 61 deaths occurred overall. Causes of death included hemorrhage (five patients), infection (21), rejection (one), nonspecific pulmonary failure (four), graft coronary artery disease (six), and obliterative bronchiolitis (eight). Infection, rejection, and obliterative bronchiolitis were the major complications. Only 20% +/- 3.9% of patients were free from any infection 3 months after transplantation. Heart and lung rejection commonly occurred asynchronously; actuarial estimates of freedom from isolated lung rejection at 1 and 5 years were 47% +/- 5.2% and 40% +/- 5.6%, respectively. For simultaneous heart and lung rejection these estimates were 87% +/- 3.5% and 86% +/- 3.8%, and for isolated heart rejection 63% +/- 5.1% and 51% +/- 6.4%, respectively. Although graft coronary artery disease developed less frequently than in patients after isolated heart transplantation (90% +/- 4.6% of patients were free of graft coronary artery disease at 5 years), obliterative bronchiolitis remains a major long-term complication and cause of morbidity and mortality. Actuarial estimates of freedom from obliterative bronchiolitis at 1, 5, and 10 years were 71% +/- 5.1%, 51% +/- 6.1%, and 42% +/- 7.8%, respectively. These results show satisfactory early and medium-term outcome after combined heart-lung transplantation but also underscore that much progress is needed in controlling infection, rejection, and obliterative bronchiolitis, all of which remain as major impediments to long-term survival.
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PMID:Long-term results of combined heart-lung transplantation: the Stanford experience. 786 27

During the 1991 Gulf War, the Iraqi army set Kuwait oil wells on fire. Wells and some oil refineries were burned, resulting in Kuwait and the surrounding Gulf region being exposed to toxic gases. The oil fires reached their peak in February 1991. On March 7, the fires in some fields were still burning at peak strength. Sulfur dioxide, particulates, carbon monoxide, and nitrogen oxides were emitted into the atmosphere. All of these substances can cause adverse health effects, which vary according to concentration and duration of exposure. A survey conducted in Kuwait clinics and emergency rooms showed an increase in upper respiratory irritation consistent with environmental air sampling results, indicating occasional high levels of particulates. Patient visits related to gastrointestinal illness, heart disease, psychiatric illness, chronic bronchitis and emphysema, and bronchiectasis increased during the period following the burning of the oil wells. There was no documented evidence of an increase in visits for acute upper and lower respiratory infections or asthma. Public health workers must recognize the high priority of collecting long-term health data and developing public health systems to assess those data.
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PMID:Environmental surveys conducted in the Gulf region following the Gulf War to identify possible neurobehavioral consequences. 931 49

Clubbing was first described by Hippocrates more than 2.500 years ago. It may be seen alone or as part of an entity called hypertrophic osteoarthropathy which include periostitis, arthritis and sometimes thickening and edema of the skin around the affected joints. Pulmonary diseases such as cancer, abscess, empyema, bronchiectasis and cystic fibrosis are the major diseases known to be associate with hypertrophic osteoarthropathy. Digestive tract cancer, cyanogenic congenital heart disease are well known association. Many theories have attempted to explain the appearance of this sign but few have persisted. In this article, we review characteristics, relation with etiology and the basis of the pathophysiology of hypertrophic osteoarthropathy and particularly of clubbing.
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PMID:[Review on hypertrophic osteoarthropathy and digital clubbing]. 1280 75

In order to evaluate the clinical manifestations, management and outcome of childhood lung abscess, a retrospective chart review of 27 pediatric patients with International Classification of Diseases, Ninth Revision-Clinical Modification (ICD-9 CM) code of 503.1 (lung abscess) from August 1987 to August 2003 was conducted. Among the 27 patients (14 males and 13 females), 30% (8/27) were primary lung abscess and 70% (19/27) had underlying chronic diseases (secondary lung abscess). The predisposing factors of the primary group (n = 8) included 6 cases of respiratory tract infection, 1 with choking during swimming, and 1 with laceration wound. The underlying diseases in the secondary group (n = 19) included 10 cases of hematologic disorder (52%), 3 of congenital heart disease, 2 of central nervous system anomalies, and 1 each of hyperimmunoglobulin E syndrome, chronic lung disease, liver cirrhosis with fistula formation, and Swyer-James syndrome. Eleven patients (41%) underwent diagnostic tapping, including echo-guided aspiration (10 cases) and computed tomography-guided percutaneous needle aspiration (1 case). Positive yield rate from aspiration of lung abscess was 63.6% (7/11). Surgical intervention was performed in 8 (42%) of the secondary group and in 1 patient from the primary group. The pathogens were identified in 11 patients (41%): 5 with oral flora, 2 with Staphylococcus aureus plus other pathogens, 1 with S. aureus alone, 1 with Pseudomonas aeruginosa plus Proteus mirabilis, 1 with P. aeruginosa alone, and 1 with Aspergillus. The average duration of parenteral antibiotic use was 40 days. Five cases (18.5%) died due to poor control of the underlying diseases, and 4 of the patients (15%) had sequelae (2 with bronchiectasis and 2 with lung fibrosis). Seventy percent of lung abscess occurred in children with underlying medical conditions. Early percutaneous aspiration has an important role in identification of pathogens. Oral anaerobes and S. aureus are the core pathogens in primary lung abscess and gram-negative pathogens should also be considered in secondary lung abscess.
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PMID:Clinical management and outcome of childhood lung abscess: a 16-year experience. 1598 68

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder reflecting abnormalities in the structure and function of motile cilia and flagella, causing impairment of mucociliary clearance, left-right body asymmetry, and sperm motility. Clinical manifestations include respiratory distress in term neonates, recurrent otosinopulmonary infections, bronchiectasis, situs inversus and/or heterotaxy, and male infertility. Genetic discoveries are emerging from family-based linkage studies and from testing candidate genes. Mutations in 2 genes, DNAI1 and DNAH5, frequently cause PCD as an autosomal recessive disorder. A clinical genetic test has been recently established for DNAI1 and DNAH5, which involves sequencing 9 exons that harbor the most common mutations. This approach will identify at least one mutation in these 2 genes in approximately 25% of PCD patients. If biallelic mutations are identified, the test is diagnostic. If only one mutation is identified, the full gene may be sequenced to search for a trans-allelic mutation. As more disease-causing gene mutations are identified, broader genetic screening panels will further identify patients with PCD. Ongoing investigations are beginning to identify genetic mutations in novel clinical phenotypes for PCD, such as congenital heart disease and male infertility, and new associations are being established between 'ciliary' genetic mutations and clinical phenotypes.
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PMID:Genetic causes of bronchiectasis: primary ciliary dyskinesia. 1753 28


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