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Biomedical subjects

M Corey

Publications and source records attributed to M Corey.

At least 109 records · Page 6Linked to original sources

Association of respiratory viral infections with pulmonary deterioration in patients with cystic fibrosis.

In a two-year prospective study, we examined the effect of respiratory viral infections on pulmonary function in 49 patients with cystic fibrosis (mean age, 13.7 years). Nineteen normal siblings (mean age, 14) served as controls. Subjects were assessed quarterly and at the time of any respiratory illness. Each assessment included nasal washes for viral isolation and blood drawing for respiratory viral serologic studies. Pulmonary-function tests were performed at least twice yearly. Respiratory illnesses were reported significantly more often in the patients (3.7 per year) than in the normal siblings (1.7 per year), although the frequency of proved viral infections (1.67 per year) was identical. In the patients with cystic fibrosis significant correlations (P less than 0.0001) were found between the annual incidence of viral infections and every measure of disease progression in the two-year period, including the rate of decline of the Shwachman score (r = 0.71), the percentage of ideal weight for height (r = 0.80), the forced vital capacity (r = 0.85), the forced expiratory volume in the first second (r = 0.84), the forced midexpiratory flow rate (r = 0.68), and the frequency (r = 0.53) and duration (r = 0.84) of hospitalizations for respiratory exacerbations. We conclude that frequency of viral respiratory infections is closely associated with pulmonary deterioration in patients with cystic fibrosis.

Adolescent↗

Pseudomonas cepacia infection in cystic fibrosis: an emerging problem.

The prevalence of Pseudomonas cepacia infection increased from 10% in 1971 to 18% by 1981 in a population of approximately 500 patients with cystic fibrosis. Carriage of P. aeruginosa has remained unchanged at 70% to 80% over the same period. Patients infected with P. cepacia have greater impairment of pulmonary function than those with P. aeruginosa. A syndrome characterized by high fever, severe progressive respiratory failure, leukocytosis, and elevated erythrocyte sedimentation rate has occurred in eight patients over the past 3 years, with a 62% fatality rate. Because P. cepacia strains are uniformly resistant to ticarcillin, piperacillin, and aminoglycosides, and because ceftazidime is ineffective despite in vitro activity, treatment of these infections is very difficult. Prevention of acquisition and effective treatment of P. cepacia in patients with cystic fibrosis are now major clinical problems in our clinic.

Adolescent↗

Pancreatic function testing in cystic fibrosis.

None of the currently available clinical techniques for measuring pancreatic function in patients with CF can be considered "ideal". The "direct" pancreatic stimulation test provides the most information and remains the only truly accurate test of exocrine function, particularly in those individuals with pancreatic sufficiency. The invasive, complex nature of the pancreatic stimulation tests precludes its use as a routine method of assessing pancreatic function in all patients and limits its value for monitoring function in those individuals who are expected to deteriorate with time. Many of the indirect tests of pancreatic function are of value for identifying CF patients with complete pancreatic insufficiency who may require therapy with pancreatic enzyme supplements and fat soluble vitamins. However, none of the currently available "indirect" tests of exocrine function are sensitive enough to evaluate or monitor CF patients with pancreatic sufficiency. Blood tests, capable of measuring pancreatic enzymes in the circulation, such as immunoreactive trypsinogen, may be of value for monitoring pancreatic function in patients with CF, but further longitudinal studies are necessary to fully evaluate this approach. More sophisticated "indirect" tests of exocrine pancreatic function are vitally needed to facilitate adequate assessment of this important digestive organ in patients with cystic fibrosis.

Cystic Fibrosis↗

Improved prognosis in CF patients with normal fat absorption.

CF patients with normal fat absorption, as a group, have lower mean sweat chloride levels, maintain better pulmonary function and weight for their age, and appear to survive longer than CF patients with steatorrhea. The prognostic advantage for CF males in general is not seen in the pulmonary function data for patients with normal fat absorption, but may be reflected in the smaller number of females in this group. Males in both groups are clearly better at maintaining good weight than are females. Whether this means that nutritional intervention can improve pulmonary course or that other factors (genetic, endocrinological, environmental) dictate nutritional and pulmonary state, as well as sex differences, remains to be shown. Non-steatorrheic patients are far less likely than steatorrheic patients to have Pseudomonas infecting their lungs. This is a significant prognostic advantage since the progressive lung disease and eventual mortality of most CF patients can be charted by their acquisition of P. aeruginosa and the increasing frequency of exacerbation and attempts to eradicate this organism. CF has been called a lethal genetic disease because affected homozygotes did not generally survive to procreate. However, increasing numbers of young women with CF are surviving to an age where pregnancy and child-rearing are options for them. The majority of patients who reach this stage with sufficiently good health to embark on a pregnancy are patients with normal fat absorption. They also appear to be more likely to remain well throughout pregnancy and as young mothers. There is no doubt that CF patients with normal fat absorption have a better prognosis than those with typical CF malabsorption.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Prognostic value of sweat-chloride levels in cystic fibrosis: a negative report.

Sweat-chloride levels at the time of diagnosis have been reviewed from 708 patients with cystic fibrosis. Sixty-one patients, shown to be non-steatorrheic, were found to have significantly lower levels than 647 patients with severe pancreatic insufficiency. Although patients who retain pancreatic function are known to have less severe pulmonary involvement and a better overall prognosis (5), we were unable to show any correlation of sweat-chloride levels at diagnosis with pulmonary function, current age or Shwachman clinical score which might be of prognostic value.

Chlorides↗

Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption.

The clinical presentation, courses, and sweat chloride values of 72 CF patients with normal fat absorption are described. In general, these patients had milder clinical symptoms and a lower mean sweat chloride value than their counterparts with steatorrhoea. Pulmonary function tests, including FEV1, FVC, FEF25%-75%, PaO2, and RV/TLC%, were significantly better in patients with normal fat absorption compared with both male and female patients who had steatorrhoea. The maintenance of better pulmonary function, coupled with the low mortality, suggests that patients without steatorrhoea have a better prognosis. This difference remains unexplained, but may be contributed to by nutritional, genetic, or pancreatic factors.

Adolescent↗

Antibiotic prophylaxis in cystic fibrosis: inhaled cephaloridine as an adjunct to oral cloxacillin.

The effect of prophylactic antibiotics on bacterial colonization of the respiratory tract and on general progression of cystic fibrosis was studied in a two-year prospective study of 47 mildly to moderately affected patients. One group of patients received inhaled cephaloridine and the other received no inhaled antibiotic; both groups received cloxacillin orally. Carriage of Haemophilus influenzae was greater in the group not receiving inhaled antibiotic (55% vs 20%). Rates of carriage of Staphylococcus aureus (23%). Pseudomonas aeruginosa (greater than 90%). Pseudomonas cepacia (45%), and other organisms were similar in both groups. There were no significant differences between the two groups in incidence of respiratory tract infections or hospital admissions, clinical scores, radiologic scores, or rate of change of pulmonary function. Although continuous antistaphylococcal antibiotic prophylaxis may be successful in suppressing colonization with S. aureus, it may also contribute to the high rates of carriage of Ps. aeruginosa and Ps. cepacia observed in patients with cystic fibrosis.

Administration, Oral↗

Pregnancy in cystic fibrosis: a better prognosis in patients with pancreatic function?

Pregnancy in patients with cystic fibrosis is likely to become a more common occurrence as a result of improving survival rates in females with this condition. Eleven pregnancies in seven patients with cystic fibrosis are presented. Despite previous reports on the hazards of pregnancy in women with cystic fibrosis, only one pregnancy required a therapeutic abortion to preserve the health of the mother. Nine of the pregnancies occurred in five mothers who had no pancreatic insufficiency, and no significant deterioration in pulmonary function occurred during or after pregnancy. These patients who do not require pancreatic enzyme supplementation appear to represent a special subgroup within cystic fibrosis in whom not only pulmonary function but also the overall prognosis is better, and in whom pregnancy can be tolerated without undue hazard.

Adult↗

Immunoassay of serum conjugates of cholic acid in cystic fibrosis.

Pre- and post-prandial serum conjugates of cholic acid (SCCA) were measured by radioimmunoassay (RIA) in 83 patients with cystic fibrosis (CF), 14 of whom did not have steatorrhoea, and in 25 controls. Of the CF patients with steatorrhoea, 38% had fasting SCCA levels greater than 3 standard deviations above mean fasting control values, whereas no CF patient without steatorrhoea had elevated fasting SCCA levels. Steatorrhoeic patients with palpable livers had higher pre- and post-prandial SCCA levels. Post-prandial SCCA levels failed to discriminate between control and CF groups however. Other serum tests of liver function, including the aspartate amino transferase, alkaline phosphatase, albumin, gamma globulin, and albumin : globulin ratio, failed to correlate with the SCCA. Changes in serum protein constituents correlated strongly with pulmonary dysfunction. The results suggest that elevation of fasting SCCA levels in CF patients is a more sensitive indicator of liver dysfunction than other tests and is a better discriminator than post-prandial SCCA levels between normal and abnormal liver function. The test is recommended for early detection of liver dysfunction in CF patients.

Adolescent↗

Response to aerosol salbutamol, SCH 1000, and placebo in cystic fibrosis.

The responses of 20 patients with cystic fribrosis to a B2 agonist, salbutamol, to an anticholinergic agent, SCH 1000, and to a placebo containing difluorodichloroethane and soya lecithin delivered by metered aerosol were compared. Flow rates decreased significantly after placebo (p < 0.05). FEV1 increased significantly after salbutamol (p < 0.05), but the degree of these changes was small. There was a small but significant increase in FVC but no change in flow rates after SCH 1000. Specific conductance increased significantly (p < 0.01) after both salbutamol and SCH 1000. Thoracic gas volume remained unchanged with both drugs and placebo. Four of 20 patients had a clinically significant increase in flow rates with SCH 1000 and three with salbutamol. The consistent increases in sGaw coupled with minimal changes in flow rates, suggest that the physiological effects of both agents is to increase the compressibility of the airway. The results after placebo demonstrate the increased airway reactivity to irritants in cystic fibrosis. In view of this, attention should be paid to the possible irritant effects of inhaled medications.

Adolescent↗

Pulmonary function and bronchial reactivity in children after croup.

Ninety-six children who were admitted into hospital with croup 8.5 yr previously were studied to assess lung function, atopic disease, and bronchial reactivity. Although absolute values in pulmonary function showed only small differences, children who have had croup are members of a population that is significantly different from normal children. Thirty-five per cent had heightened bronchial reactivity, as determined by methacholine challenge. There was no increased incidence of atopic disease in the children studied. One can speculate that these children who have had croup may be a population susceptible to developing chronic obstructive airway disease as adults.

Asthma↗

Bronchial hyperreactivity in cystic fibrosis and asthma.

We studied 113 patients with CF and compared their responsiveness to inhaled methacholine to that of 50 asthmatic children and 26 normal children. Positive responses to MCh occurred in 51% of the patients with CF and 98% of those with asthma. There was a significant correlation between a positive response to MCh in CF and poor pulmonary function. There was no relationship between allergic rhinitis or positive allergen skin tests and a positive MCh response. Dose-response curves and time course to MCh challenge differed in CF and asthma. Bronchial hyperreactivity is thus common in CF but is different from that in asthma.

Adolescent↗

Five- to seven-year course of pulmonary function in cystic fibrosis.

The pulmonary function records of 132 patients with cystic fibrosis followed for 5 to 7 years were reviewed. Changes in forced vital capacity, 1-sec forced expiratory volume, mean forced expiratory flow during the middle half of the forced vital capacity, and 1-sec forced expiratory volume as a percentage of forced vital capacity were examined. There was considerable variation in the rates of change, but the general pattern was consistent with a theory of exponential decline, mean forced expiratory flow during the middle half of the forced vital capacity showing the earliest and most dramatic changes. The pulmonary function of 33 patients (25 per cent) remained stable or improved throughout follow-up, possibly reflecting mild forms of lung disease or the efficacy of therapy. Twenty of these patients (15 per cent) maintained completely normal pulmonary function. The rate of decline in pulmonary function values, with progress of the disease, was steeper in the female patients.

Adolescent↗