Biomedical subjects

M Corey

Publications and source records attributed to M Corey.

At least 91 records · Page 5Linked to original sources

PubMed1988

A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto.

Two cystic fibrosis (CF) clinic populations of similar size and age distribution were compared with respect to growth, pulmonary function, and survival. Height and weight measurements were made on 499 patients in Boston (mean +/- SD age 15.9 +/- 9.6, range 1 month to 45 years) and on 534 patients in Toronto (mean +/- SD age 15.2 +/- 8.3, range 1 month to 43 years). Males constituted 57% in the Boston group, 58% in Toronto. Pulmonary function test results were recorded for 64% of the Boston patients and 77% of the Toronto patients. Survival curves for the period 1972-1981 generated by the CF Patient Registry were compared. Patients in Boston tended to be shorter than patients in Toronto. This pattern was seen in both sexes in the 10-20 year age groups. Toronto males also weighed more than Boston males. Mean forced expiratory volume in one second (FEV1) was not different in Boston and Toronto CF patients whether expressed as a percent of predicted or in litres by age groups. Median age of survival in Boston was 21 years, in Toronto 30, the two curves showing a marked separation from age 10. Although progressive pulmonary disease is the major cause of mortality in cystic fibrosis, the differences in growth and survival in these two patient groups, with very similar age-specific pulmonary function, suggest further examination of nutritional guidance and intervention in CF, especially regarding the traditional restriction of dietary fat.

Adolescent↗

PubMed1988

Changes in body composition during refeeding of patients with anorexia nervosa.

Changes in body composition were studied in 13 girls with anorexia nervosa before and during 2 months of refeeding. Fat body mass and fat-free body mass were derived from skin-fold measurements. Total body potassium was measured by whole body counter, and intracellular water was calculated from it. Extracellular water was measured as the bromide space after oral bromide administration. A gradual increase was noted in weight, fat body mass, fat-free body mass, and total body potassium during refeeding. Extracellular water was expanded on admission and increased in all patients in the first weeks of treatment; later it fell to normal. Most of the changes in fat-free body mass over the first weeks of refeeding could be accounted for by an expansion in extracellular water. Particular care must therefore be taken with fluid balance during the first few weeks of refeeding.

Adipose Tissue↗

PubMed1988

Role of conventional physiotherapy in cystic fibrosis.

Because of the time and the emotional cost involved in performing daily conventional chest physiotherapy in patients with cystic fibrosis, a 3-year prospective study was undertaken to compare the long-term effects of postural drainage accompanied by percussion and the forced expiratory technique with the effects of the forced expiratory technique alone. Patients who performed the forced expiratory technique alone had mean annual rates of decline that were significantly different from zero for forced expiratory volume in 1 second (p less than 0.001), forced expiratory flow between 25% and 75% of vital capacity (p less than 0.001), and Shwachman clinical score (p less than 0.004). In the group performing conventional physiotherapy with percussion and postural drainage, only the mean annual rate of decline for forced expiratory flow between 25% and 75% of vital capacity was significantly different from zero (p less than 0.03), and it was significantly different from the mean rate of decline associated with the forces expiratory technique alone (p less than 0.04). We conclude that conventional chest physiotherapy should remain a standard component of therapy in cystic fibrosis.

Adolescent↗

PubMed1988

The association of cystic fibrosis, gastroesophageal reflux, and reduced pulmonary function.

Between 1971 and 1984, 57 patients with cystic fibrosis (CF) and clinically suspected gastroesophageal reflux (GER) underwent a barium-meal examination and routine pulmonary function testing. Reflux was demonstrated in 18 patients; in six of these it was complicated by hiatus hernia, esophagitis, or stricture formation. Compared with 412 CF patients without known GER, the 18 patients with demonstrable reflux had significantly reduced forced expiratory volume and forced vital capacity. GER should be looked for carefully on any barium-meal study in patients with CF; these patients have an increased incidence of reflux, with its implications for lung function, and are not good candidates for surgical intervention.

Adolescent↗

PubMed1988

Allergic bronchopulmonary aspergillosis in cystic fibrosis: a secretory immune response to a colonizing organism.

One hundred and seventeen patients with cystic fibrosis (CF) were evaluated for criteria suggestive of allergic bronchopulmonary aspergillosis (ABPA) and atopy. We found positive skin tests to Aspergillus to be more common in patients with CF than comparison groups with asthma or non-specific nasal symptoms. This increased prick skin test reactivity to Aspergillus was especially noticeable in a group of CF patients having only one or two skin tests positive, preponderantly Aspergillus. Twelve of the CF patients (10%) had further immunologic criteria suggestive of ABPA. These patients had significantly worse lung function than the CF patients without such criteria.

Adolescent↗

PubMed1987

Pseudomonas species contamination of cystic fibrosis patients' home inhalation equipment.

A prevalence study was undertaken to determine whether aerosol equipment used at home by patients with cystic fibrosis (CF) could provide a reservoir for Pseudomonas aeruginosa or Pseudomonas cepacia. Home maintenance of this equipment was also evaluated for its relationship to contamination. In nine of 36 patients, Pseudomonas species were isolated from one or more pieces of home equipment. Only patients colonized with P. aeruginosa had contaminated equipment. P. aeruginosa was recovered from equipment used by five patients; no P. cepacia was recovered. Aerosolization masks were the most commonly contaminated pieces of equipment (20%), followed by nebulizers (17%), medication syringes (10%), connective tubing (6%), and saline solution (4%). Nebulizers and syringes were significantly more likely to be contaminated if they had been in use for 1 month or longer; nebulizers and masks were more likely to be contaminated if they were cleaned or were rinsed only with tap water after use. We conclude that equipment may serve as a reservoir to reintroduce or perpetuate colonization of some patients with CF, but that contamination of equipment with P. aeruginosa is not common.

Child↗

PubMed1987

Energy expenditure of patients with cystic fibrosis.

Resting energy expenditure was measured by open-circuit indirect calorimetry in 71 patients, aged 8.9 to 35.5 years, with cystic fibrosis who had no recent history of acute lung infection. Pulmonary function and nutritional status were studied simultaneously. In most patients, resting energy expenditure was above normal (range 95% to 153% of predicted values for age, sex, and weight as derived from the Harris Benedict equations), and was negatively correlated with pulmonary function (P less than 0.01) and nutritional status (P less than 0.01) when expressed as a percentage of body fat. Pulmonary status was positively correlated with nutritional status (P less than 0.01). We conclude that resting energy expenditure in patients with cystic fibrosis exceeds normal values and that the increase correlates with a deterioration in lung function and nutritional status.

Adolescent↗

PubMed1987

Randomized trial of ceftazidime versus placebo in the management of acute respiratory exacerbations in patients with cystic fibrosis.

A randomized trial of ceftazidime versus placebo was conducted in patients with cystic fibrosis hospitalized for acute respiratory exacerbations. Patients 12 years of age or older were included if they had mild to moderately severe illness according to the following criteria: erythrocyte sedimentation rate less than or equal to 50 mm/hr and less than three other abnormalities (leukocyte count greater than or equal to 15,000/microliter, pulse greater than or equal to 100 beats/min, respirations greater than or equal to 30/min, or temperature greater than or equal to 38.5 degrees C). In all 16 episodes treated with ceftazidime, the patients were rated improved in comparison with 10 of 12 patients treated with placebo. Three placebo-treated patients dropped out of the study within 3 to 5 days because they wanted antibiotic therapy. None of the 15 placebo-treated patients showed clinical deterioration. There were no significant differences in rate of improvement of symptom score, weight gain, or pulmonary function between the two treatment groups. There was no difference in the course during the 6 to 24 months after the study period. Intravenous antibiotics are not essential in the management of all acute respiratory exacerbations of mild to moderate severity in patients with cystic fibrosis.

Adolescent↗

PubMed1987

False positivity of Legionella serology in patients with cystic fibrosis.

Respiratory deterioration accounts for the morbidity and mortality observed in patients with cystic fibrosis. The role of Legionella in this deterioration was determined in a 2-year prospective study of 49 patients with cystic fibrosis and 19 sibling controls. Sera were obtained from participants on enrollment and at quarterly intervals. Legionella antibodies were measured in parallel using an indirect fluorescent assay. No seroconversions were observed. Eleven of 49 patients with cystic fibrosis (22%) were seropositive compared to none of 19 siblings (P less than 0.05). Six of the 11 patients demonstrated high titers (greater than or equal to 1:512) that persisted throughout the study. Absorption with pools of various Pseudomonas species reduced the antibody titers such that only 3 remained positive after absorption. Legionella was not found to be an important cause of clinical deterioration during this study. The results of the absorption studies suggest that high titers to Legionella in this population are due to cross-reacting antibodies.

Antibodies, Bacterial↗

PubMed1987

Coagulase-negative staphylococci as true pathogens in newborn infants: a cohort study.

We examined the pathogenicity of coagulase-negative staphylococci (CONS) in newborn infants by comparing presenting nonspecific signs of infection in infants with and without CONS bacteremia. During a 6-month period 799 blood cultures were obtained in a tertiary care nursery; 81 (10.1%) grew CONS and 25 (3.0%) grew other bacteria. A comparison group of 121 infants was selected randomly from ill patients whose blood cultures were negative. In addition 70 well infants were matched to CONS-positive cases. Abnormal clinical signs, complete blood cell counts, C-reactive protein, alpha-1-acid glycoprotein and prealbumin were determined at the time of culture. Signs that discriminated best between infants with and without CONS bacteremia were identified by logistic regression analysis. Infants with CONS bacteremia did not differ from infants with sepsis caused by recognized pathogens, except for lethargy, which was significantly more common in unequivocal infection. Infants with presumed infection but negative blood cultures, and noninfected control patients had abnormal signs significantly less often than CONS-positive infants. C-reactive protein, hyperthermia, increased oxygen requirements and lethargy were the most useful signs in identifying neonatal bloodstream infection. This cohort study provides objective evidence for the pathogenicity of CONS in newborn infants.

Acute-Phase Proteins↗

PubMed1986

The musculoskeletal manifestations of cystic fibrosis.

Twenty-seven patients with CF and joint complaints were reviewed. Twelve patients had arthritis, 11 had HPO, and four had symptoms of mechanical neck and back pain. We have confirmed the association of CF with an episodic form of arthritis. For the first time, we have reported that a subgroup of these patients later develop persistent synovitis with progressive asymmetric, erosive disease. The etiology of CF arthritis is unclear, but may relate to chronic infection and immune complex mechanisms. CF arthritis can be contrasted with CF induced HPO, which is associated with worse lung disease, a male predominance, and an older mean age of onset of symptoms. Mechanical neck and back pain may relate to an associated scoliosis or kyphosis in CF.

Adolescent↗

PubMed1986

Prognostic factors associated with patient survival during nutritional rehabilitation in malnourished children and adolescents with cystic fibrosis.

Nineteen children and adolescents with cystic fibrosis and malnutrition were given intensive nutritional support in an effort to reverse malnutrition. Standard techniques of enteral and parenteral feeding were used. As 10 of 19 patients died shortly after nutritional intervention began, we retrospectively analyzed patient data in order to discover whether or not any patient characteristics were associated with survival. Using a multivariate analysis, a linear discriminant function was derived employing average heart rate, the presence or absence of Pseudomonas cepacia in the sputum culture, PaCO2, and the patient's age at the time of intervention. This function correctly classified these 19 patients. A further 10 nonselected CF patients who also received nutritional support were similarly assessed using the function, and again, survival was accurately predicted. Therefore, this function can be used to predict the likelihood of patient survival during the provision of advanced nutritional support. It may be helpful in deciding whether or not advanced nutritional support is warranted in certain CF patients.

Adolescent↗

PubMed1986

Effects of nutritional status on exercise performance in advanced cystic fibrosis.

Initial evaluation of 22 patients with cystic fibrosis (CF) on entry into a trial of home oxygen therapy was used to elucidate the possible effects of poor nutritional status on exercise performance in CF. The patients had advanced lung disease (mean FEV1, 36 percent predicted) and all had a stable resting PaO2 less than or equal to 65 mm Hg. Nutritional status was determined by calculating weight as a percentage of ideal for height (Wt/Ht) for each subject. Exercise testing consisted of a progressive exercise test on a cycle ergometer to measure maximum work capacity (Wmax), and a steady state test at 50 percent of baseline Wmax. During the steady state test, cardiac output (Q) and stroke volume (SV) were computed by the indirect Fick (CO2) method. Wmax, SV, Q and lung function results are expressed as percent predicted. Mean (+/- SD) Wmax was 58 +/- 15 percent predicted. Wmax correlated with both FEV1 and Wt/Ht, but FEV1 and Wt/Ht were not related. During steady state exercise, 12 of 22 patients had a SV less than 80 percent predicted. SV correlated with Wt/Ht, but not with lung function. Thirteen of the 22 patients had a Wt/Ht less than or equal to 90 percent and were considered malnourished. When compared with the well-nourished patients (Wt/Ht greater than 90%), these malnourished subjects had significantly lower mean values for Wmax%, SV% and Q% predicted, but not for lung function parameters. We conclude that: in patients with CF and advanced lung disease, nutritional status plays a significant role in determining exercise capacity; lower exercise tolerance of malnourished patients is an independent effect, as nutritional status and lung function were not related; and malnourished patients with CF have an altered cardiac performance on exercise testing which is due to a reduced SV rather than an impaired heart rate response.

Adolescent↗

PubMed1986

Psychologic adjustment of the family with a member who has cystic fibrosis.

Seventy-eight percent of the 384 patients 2 years of age and older who were enrolled in the Cystic Fibrosis Clinic at the Hospital for Sick Children participated in this study of family adjustment to cystic fibrosis. Residence with at least one parent at the time of the survey was a condition for inclusion in the study. The effect of the disease on the family was assessed using three questionnaires: an inventory of family problems as perceived by the parents and two standardized measures of family interaction. Measures of behavior and self-concept in each patient and sibling, physical disease parameters for each patient, and demographic characteristics of the families were examined and analyzed in relation to the responses to the questionnaires on family interaction. Families of patients with cystic fibrosis were found to function very well, with normal mean scores on both standardized profiles. Children with cystic fibrosis showed more behavior problems than expected from the test norms, and healthy siblings of children with cystic fibrosis had behavior problem scores midway between those with the disease and norm scores. Measures of self-concept were remarkably similar for patients and siblings and showed levels at or above those for children and young adults in the general population. Parents psychosocial scores were influenced by the patients' physical parameters, but the severity of the disease generally was unrelated to psychosocial scores of patients and siblings. A notable exception was the correlation of emotional disturbance with amount of hospitalization in patients older than 12 years of age.

Adaptation, Psychological↗

PubMed1986

Serum immunoreactive pancreatic lipase and cationic trypsinogen for the assessment of exocrine pancreatic function in older patients with cystic fibrosis.

Indirect and qualitative tests of pancreatic function are commonly used to screen patients with cystic fibrosis for pancreatic insufficiency. In an attempt to develop a more quantitative assessment, we compared the usefulness of measuring serum pancreatic lipase using a newly developed enzyme-linked immunosorbent immunoassay with that of cationic trypsinogen using a radioimmunoassay in the assessment of exocrine pancreatic function in patients with cystic fibrosis. Previously, we have shown neither lipase nor trypsinogen to be of use in assessing pancreatic function prior to 5 years of age because the majority of patients with cystic fibrosis in early infancy have elevated serum levels regardless of pancreatic function. Therefore, we studied 77 patients with cystic fibrosis older than 5 years of age, 41 with steatorrhea and 36 without steatorrhea. In addition, 28 of 77 patients consented to undergo a quantitative pancreatic stimulation test. There was a significant difference between the steatorrheic and nonsteatorrheic patients with the steatorrheic group having lower lipase and trypsinogen values than the nonsteatorrheic group (P less than .001). Sensitivities and specificities in detecting steatorrhea were 95% and 86%, respectively, for lipase and 93% and 92%, respectively, for trypsinogen. No correlations were found between the serum levels of lipase and trypsinogen and their respective duodenal concentrations because of abnormally high serum levels of both enzymes found in some nonsteatorrheic patients. We conclude from this study that both serum lipase and trypsinogen levels accurately detect steatorrhea in patients with cystic fibrosis who are older than 5 years but are imprecise indicators of specific pancreatic exocrine function above the level needed for normal fat absorption.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1985

Cystic fibrosis in Ontario.

The incidence of cystic fibrosis in Ontario, Canada has been determined from clinical data, from the cystic fibrosis database of the Hospital for Sick Children, Toronto, and from population statistics in the Province of Ontario. The survey included 420 confirmed cases of cystic fibrosis born during the period 1966-1980. The mean incidence during this period was one in 2,927. In the last 5-year period, a decline was noted in incidence that may have reflected in part the effectiveness of early diagnosis and genetic counseling in affected families. During the period of the survey, over 60% of cases were diagnosed within the first year of life, 74% by age 2 years, and 90% by age 5 years. Clinical diagnosis in the first year of life was more common in males (65%) than in females (54%), a consistent finding during the period of the survey. The incidence of meconium ileus was 15.7% of ascertained cases of cystic fibrosis, with similar incidences in males (16.4%) and females (14.4%). Although survival has not been the subject of this survey, mortality in the neonatal period was significantly higher in males than in females with cystic fibrosis.

Age Factors↗

PubMed1985

Family adaptation and psychosocial adjustment to cystic fibrosis in the preschool child.

The parents of 80% (41 of 51) of preschoolers with cystic fibrosis (CF) diagnosed at least 1 year prior to the study and attending the Hospital for Sick Children (HSC) CF Clinic completed the Problem Inventory (PINV), Preschool Behavior Questionnaire (PBQ) and Family Assessment Measure (FAM). The mean age of the CF children was 3.7 years. Parents of a control group of 31 healthy daycare children with a mean age of 3.6 years completed the same questionnaires. Parents of healthy preschoolers reported more child-related problems for 2-5 year olds than did parents of CF children (P less than 0.001) suggesting that parents who have confronted the CF diagnosis go on to minimize the normal stresses of the developmental period. Considerable agreement was seen between PINV scores for mothers and fathers in each group, revealing that parents in a given family perceive similarly the impact their child has upon them. The mean PBQ for CF preschoolers was not significantly different from that of the control group, although there was some tendency toward hostile aggressive behavior in the CF group. Surprisingly, total FAM scores of all samples showed no significant differences with the exception of a better total FAM score for fathers of CF children when compared to control fathers revealing that the CF family is not, during the early years of relative health stability, adversely affected. Two subscales were significantly elevated, social desirability (for CF mothers and fathers) and denial (for CF mothers only), describing an important response style which may enhance mastery of long-term stress.(ABSTRACT TRUNCATED AT 250 WORDS)

Adaptation, Psychological↗

PubMed1985

Age-related alterations in immunoreactive pancreatic lipase and cationic trypsinogen in young children with cystic fibrosis.

Serum immunoreactive pancreatic lipase and cationic trypsinogen are elevated in young infants with cystic fibrosis (CF) and may be useful neonatal screening tests for CF. We compared lipase measured by a recently developed ELISA immunoassay with trypsinogen measured by radioimmunoassay in 70 children (ages 0.1 to 9.9 years) with CF who had various degrees of pancreatic dysfunction and in 79 similarly aged children without CF (controls). In the control children, lipase activity increased with advancing age, whereas trypsinogen showed no age-related trend. Lipase and trypsinogen were significantly elevated in the infants with CF who were younger than 1 year, irrespective of pancreatic function (trypsinogen, P less than 0.001; lipase, P less than 0.05). Sensitivities in detecting CF were 76% and 90% for lipase and trypsinogen, respectively. After the first year of life, lipase and trypsinogen values declined toward normal, the rate of decline of lipase being greater than that of trypsinogen; 67% of lipase values were within or below the normal range by 3 years, whereas 67% of trypsinogen values continued to be elevated. We conclude that trypsinogen is an excellent screening test for CF in young infants regardless of pancreatic function, and that the addition of a serum pancreatic lipase determination does not improve the accuracy of trypsinogen as a screening test for cystic fibrosis.

Age Factors↗