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Biomedical subjects

S B Fiel

Publications and source records attributed to S B Fiel.

At least 37 records · Page 2Linked to original sources
PubMed1993

Cystic fibrosis.

Cystic fibrosis, an autosomal recessive disorder, is the most common genetic disease of Caucasians. One in 25 Caucasians are carriers of the gene. The gene is found far less commonly in other races. There are over 230 different alleles of the gene, located on the 7th chromosome. The gene encodes for a membrane protein that functions as an ion channel. The survival of cystic fibrosis patients has been gradually increasing, with a mean survival in 1990 of 28 years. If the current trend of improved survival continues, it is estimated that half of cystic fibrosis patients will be over 18 years old by 1996. Disease is found in many organs including the lungs, sinuses, pancreas, gastrointestinal tract, hepatobiliary system, sweat glands and reproductive tract. The majority of patients die of pulmonary disease. The airways become chronically colonized with bacteria that cannot be eradicated, leading to bronchitis, bronchiectasis, and finally, pulmonary fibrosis with respiratory failure. The pulmonary disease may be complicated by massive hemoptysis and pneumothorax. Patient survival rates have increased because of antibiotic therapy and improved nutrition with pancreatic enzyme replacements. New treatments for the pulmonary disease are under clinical trial and include antiproteases, amiloride, a sodium channel blocker, and DNase. The insertion of the normal cystic fibrosis allele into an animal model using a modified adenovirus with effective transcription suggests that gene therapy may be possible in the future, but safety and technical problems have to be addressed.

Cystic Fibrosis↗
PubMed1992

Fertility and pregnancy in patients with cystic fibrosis.

Issues related to fertility and pregnancy, once moot, are now extremely relevant to the care of a growing number of CF patients entering adulthood. With rare exception, men are infertile, due to the almost universal presence of malformations of the reproductive tract causing obstructive azoospermia. Emphasis in the care of these patients should be directed toward confirmation of infertility and counseling to allay anxieties. In contrast, a significant albeit unknown proportion of women are fertile and a steadily increasing number of these women are conceiving. The accumulated clinical experience has demonstrated that pregnancy is well-tolerated by patients with mild disease while associated with increased maternal and fetal complications in those with severe disease. In light of current uncertainties in accurately predicting outcome in all but the most clear-cut cases, the physician must exercise clinical judgment in providing a realistic assessment of the medical risks involved and of the advisability of pregnancy. This assessment should be based on a thorough and objective evaluation of the pulmonary, cardiac, and nutritional status of the patient. Future efforts, assisted by data collected for the national CF patient registry, should be directed toward better defining the long-term impact of pregnancy on the natural history of CF and more precisely defining the pregravid parameters useful in predicting outcome for both mother and child.

Counseling↗
PubMed1990

Life beyond pediatrics. Transition of chronically ill adolescents from pediatric to adult health care systems.

Transition from pediatric to adult health care is fraught with difficulties. On the one hand, the adult care system is not properly prepared to receive patients who are survivors of the so-called childhood disorders. On the other hand, patients and families have difficulty leaving the protective environment created by pediatric caregivers, who in turn may have mixed feelings about letting the patients go. Normalization of development and social adaptation for chronically ill adolescents, however, should include a change in the environment in which health care is received. This article examines some of the issues surrounding transition and transfer from pediatric to adult health care systems and propose some avenues toward implementation of such programs.

Adolescent↗
PubMed1988

Oral vs repository corticosteroid therapy in acute asthma.

Acutely ill asthmatic patients treated in the usual fashion in an emergency room setting and discharged within six hours were studied to determine whether therapy with a single injection of a repository corticosteroid (methylprednisolone sodium acetate) could be as effective as a tapering course of oral corticosteroids in decreasing asthma symptomatology and relapse within seven days. Seventeen patients (18 episodes of asthma) formed the study population. Eight episodes occurred in patients who received depot methylprednisolone (group 1) and ten episodes in patients who received oral corticosteroid treatment (group 2). All patients in both groups improved following emergency room treatment. Relapse occurred in two of ten patients in group 2 and none in group 1. Symptoms attributable to asthma recurred in significantly more patients in group 2 than in group 1 (9 vs 0, p less than .01). Side effects from therapy with corticosteroids were rare. This study indicates that intramuscular repository corticosteroids are at least as effective as oral corticosteroids in the management of the acute asthmatic outpatient, with a distinct advantage with regard to patient compliance.

Acute Disease↗
PubMed1988

Asbestos-related pleural disease and asbestosis: a comparison of CT and chest radiography.

High-resolution CT (HRCT) has the ability to demonstrate both asbestos-related pleural disease and parenchymal abnormalities consistent with asbestosis. The role of CT in the diagnosis of asbestosis can be defined by comparing it with radiography. We evaluated 60 men who had a history of occupational exposure to asbestos and whose outside chest radiographs were considered abnormal. Chest radiographs (inside films) and HRCT were performed in all patients at our institution and were interpreted independently by experienced radiologists. Outside film results were compiled from the submitted reports. The final conclusion regarding the interpretation of the radiologic examinations was determined by consensus when disagreements existed. Positive predictive values (the likelihood that a positive report is correct) for pleural disease were: outside films 56%, inside films 79%, HRCT 100%. The positive predictive values for parenchymal disease were: outside films 51%, inside films 83%, HRCT 100%. The addition of HRCT to chest radiography is most useful in eliminating false-positive diagnoses of asbestos-related pleural disease caused by subpleural fat and false-positive diagnoses of parenchymal asbestosis in patients with extensive plaques or emphysema obscuring lung detail. The interpretation of chest radiographs in patients exposed to asbestos is often extremely difficult and subjective, and we recommend that positive findings (except calcified plaques) be confirmed with HRCT.

Asbestos↗
PubMed1987

Magnetic resonance imaging in young adults with cystic fibrosis.

It is difficult to distinguish between atelectasis, mucoid impaction, and peribronchial inflammation on chest roentgenograms (CXR) in patients with cystic fibrosis (CF). Differentiation between hilar adenopathy and prominent pulmonary vessels is also sometimes difficult. We studied 16 young adults with CF using both magnetic resonance imaging (MRI) and CXR to evaluate the usefulness of MRI in this clinical context. The same patients were studied with abdominal ultrasound and MRI for evaluation of the pancreas, gallbladder, liver, and spleen. The MRI was superior to CXR in detecting hilar and mediastinal adenopathy and in differentiating nodes from prominent vessels. It was useful in the evaluation of bronchiectasis. The CXR was superior for assessing infiltrates, hyperinflation, sternal bowing, volume loss, and hilar retraction. The MRI was only slightly better than sonography in depicting fatty infiltration of the pancreas. The modalities were equally effective in detecting hepatosplenomegaly and signs of portal hypertension. Gallbladder evaluation was far superior with sonography.

Adolescent↗
PubMed1987

MRI of tracheobronchomegaly.

Tracheobronchomegaly, a disorder of uncertain etiology, is manifested by the enlargement of the trachea and mainstem bronchi. While the diagnosis may be suggested on chest radiographs, bronchography in the past has been the definitive diagnostic modality. Recently, however, computed tomography has been used to confirm the diagnosis of TBM. We have now demonstrated TBM using MRI, and, in this patient, have shown it to be as good as CT.

Humans↗
PubMed1984

Compliance with an experimental drug regimen for treatment of asthma: its magnitude, importance, and correlates.

This paper reports on data from a double-blind, randomized controlled study of out-patient use of corticosteroids following an acute asthma attack. Issues related to compliance are examined, including: (1) the extent of non-compliance; (2) impact of non-compliance on interpreting the drug trial results; and (3) correlates of non-compliance. Of the 102 cases enrolled in the study, 25.5% were excluded from analysis because they were lost to follow-up (10.8%) or non-compliers (14.7%). Based on data for compliers, the drugs were found to reduce relapse rates and asthma symptomatology; when non-compliers were included in the analysis, the steroid drug appeared ineffective for reducing relapses and less effective for improving overall illness status. Examination of 24 potential correlates of compliance yielded a few significant associations, and only the "usual habit of compliance" correlation suggests an avenue for future action. The implications of the study findings for design and interpretation of clinical trials, as well as for improved management of chronic diseases, are discussed.

Adolescent↗