Long-term inhaled bronchodilator therapy in cystic fibrosis.
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Administration, Inhalation↗
Biomedical subjects
D M Orenstein
Publications and source records attributed to D M Orenstein.
Oxygen supplementation during exercise in cystic fibrosis.
Fourteen female and 22 male patients with cystic fibrosis (CF), 8 to 29 yr of age, performed two progressive exercise tests to exhaustion on a cycle ergometer, breathing normoxic air (21% O2) for one test, and hyperoxic air (30% O2) for the other test. The order of gas administration was randomized. Minute ventilation (VE), oxygen uptake (VO2), end-tidal CO2 tension (PETCO2), work rate, oxyhemoglobin saturation (SAO2), and heart rate (HR) were measured throughout the tests. The SaO2 of 11 patients at peak exercise was 90% or less ("Low Sat" group). The SaO2 of 23 patients remained above 90% throughout the exercise ("High Sat" group). Hyperoxic air minimized desaturation during exercise in the Low Sat group to 2 +/- 2% compared to a decrease of 10 +/- 5% with normoxic air. The decrease in saturation was not significant for the High Sat group (1 +/- 1% for both 21% and 30% O2). Peak work rate and VO2 did not differ significantly between normoxic and hyperoxic conditions. However, VE and HR at peak exercise tended to be lower, and PETCO2 was higher during peak exercise with 30% O2 than 21% O2 for both groups. During submaximal exercise, O2 desaturation was diminished and HR was significantly lower with supplemental O2, specifically in the Low Sat group. VE was significantly lower for both groups during submaximal exercise with hyperoxic air. The results suggest that O2 supplementation minimizes O2 desaturation and enables patients with CF to exercise with reduced ventilatory and cardiovascular work.
Adolescent↗
Quality of well-being before and after antibiotic treatment of pulmonary exacerbation in patients with cystic fibrosis.
General quality of life has only recently been measured with an objective tool in patients with cystic fibrosis (CF), and there have been no reported attempts to document changes in patients' overall well-being over time, as patients deteriorate or respond to intervention. We applied the Quality of Well-Being scale (QWB) in 28 patients with CF before and after a two-week course of oral ciprofloxacin used to treat pulmonary exacerbations. There were significant correlations between changes in QWB and various pulmonary function test results; QWB vs FEV1: r = 0.4, p less than 0.03; QWB vs FVC: r = 0.5, p less than 0.01; and QWB vs SaO2: r = 0.4, p less than 0.05. Thus, the QWB can track changes in general well-being in CF patients over a brief time and detect changes associated with pulmonary exacerbation and its treatment.
Attitude to Health↗
The ocular surface in cystic fibrosis.
Seventeen patients with cystic fibrosis and 17 age-, race-, and sex-matched controls were examined under standardized conditions. Testing included slit-lamp biomicroscopy, fluorescein staining, rose bengal staining, Schirmer's basic tear test, tear film break-up time, tear pH, tear lysozyme, tear protein, lid and conjunctival cultures, and conjunctival impression cytology. Cystic fibrosis patients showed a statistically significant increase in the incidence of fluorescein staining and clinical blepharitis, as well as significantly decreased Schirmer testing and tear lysozyme. Ocular surface abnormalities in these patients may be attributable to aqueous and lipid tear film deficiencies. Cystic fibrosis patients showed normal conjunctival epithelial cell morphology, grew no pathogenic organisms, and had a decreased incidence of conjunctival bacterial colonization.
Adolescent↗
Depression and anxiety in pediatric inflammatory bowel disease and cystic fibrosis.
The lifetime and current prevalence of depression and anxiety disorders was determined in 41 children with Crohn's disease, 12 children with ulcerative colitis, and 52 children with cystic fibrosis, using the Kiddie-Schedule for Affective Disorders and Schizophrenia interview. The lifetime prevalence of depression was 29% in Crohn's disease, 21% in ulcerative colitis, and 11.5% in cystic fibrosis. The difference in the prevalence of depression between Crohn's disease and cystic fibrosis was significant (p less than 0.05). The lifetime and current prevalence of dysthymia was significantly greater in ulcerative colitis than Crohn's disease (p less than 0.01) or cystic fibrosis (p less than 0.01). The lifetime prevalence of atypical depression was significantly greater in Crohn's disease than cystic fibrosis (22% versus 5.8%, p less than 0.05) and was also greater in ulcerative colitis than cystic fibrosis (21% versus 5.8%, p = 0.1). There was no difference between the groups in the current prevalence of major depression or atypical depression, or in the lifetime or current prevalence of anxiety disorders.
Adolescent↗
The quality of well-being in cystic fibrosis.
Traditional outcome measures in CF include PFTs, exercise tests, and several scoring systems that depend on pulmonary status and are largely subjective. The Quality of Well-being scale (QWB) is a widely used tool for measuring quality of life by three subscales: mobility, physical activity, and social activity, with points assigned within each subscale. The QWB has been shown to be valid in patients with COPD. We administered the QWB scale to 44 patients with CF, aged 7 to 36 years, and examined the relationship between QWB and PFTs, and in 15 patients the QWB vs exercise performance (peak VO2) on a progressive cycle ergometer test. QWB was significantly correlated with each variable examined: QWB vs FEV1, r = 0.5518 (p less than .0001); QWB vs FEF25-75%, r = 0.4793 (p less than .001); QWB vs PEFR, r = 0.4018 (p less than .01); QWB vs peak VO2, r = 0.5778 (p less than .01). The QWB scale is an objective measure that is significantly correlated with measures of performance and pulmonary function in CF. The relationship is not one of identity; further, the QWB is broad based and takes into account general well-being, not just pulmonary health, adding an important dimension to the care of patients with CF.
Activities of Daily Living↗
Gross hematuria following sclerotherapy of esophageal varices in patients with cystic fibrosis.
We report two patients with portal hypertension secondary to cystic fibrosis who developed transient gross hematuria following injection sclerotherapy of esophageal varices. Both patients developed this complication within 6 h of sclerosing sessions during which sodium morrhuate was used. Each cleared her hematuria within 4 days without developing oliguric renal insufficiency. Subsequent sclerotherapy was associated with no untoward effects. These are the only two pediatric patients among 40 whose varices we have sclerosed who have developed gross hematuria at the time of sclerotherapy, and they are our only two patients undergoing sclerotherapy who have had cystic fibrosis. The factors rendering them vulnerable to renal or urinary tract insult in the absence of other systemic complications are unclear. However, the therapeutic endoscopist should be cognizant of this potential adverse reaction when performing sclerotherapy.
Adolescent↗
Use of bronchoalveolar lavage in immunocompromised children with pneumonia.
Because pneumonia in the immunocompromised host presents a diagnostic dilemma to the pediatrician, we prospectively studied the use of bronchoalveolar lavage (BAL) with flexible fiberoptic bronchoscopy in 14 immunocompromised children with pneumonia over a 5-month period. The children received immunosuppressive agents because of organ transplants, cancer, or systemic lupus erythematosus. A diagnosis was made by BAL in 10 (71%) of the 14 children. Pathogenic organisms included Pneumocystis carinii in six, cytomegalovirus (CMV) in two, Aspergillus fumigatus in one, and a mixed infection of Candida albicans and CMV in one. There were no complications. Because BAL by flexible fiber-optic bronchoscopy is a rapid and safe procedure and a diagnosis can be made in a majority of cases, we recommend its use in immunocompromised children with pneumonia before resorting to lung biopsy.
Adolescent↗
Gastroesophageal reflux and respiratory disease in children.
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Bronchial Spasm↗
Exercise tolerance and exercise conditioning in children with chronic lung disease.
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Asthma↗
Munchausen syndrome by proxy simulating cystic fibrosis.
The case of a child with apparent cystic fibrosis whose many signs, symptoms, and laboratory results were convincingly portrayed by his mother is presented. The mother falsified the history, and cunningly altered sweat tests and stool fat analyses, and stole sputum from patients with cystic fibrosis to make her child appear to have cystic fibrosis. This case of Munchausen syndrome by proxy highlights the extent to which the diagnosis of cystic fibrosis rests on reliable history and laboratory data and emphasizes the extremes to which perpetrators of this form of child abuse may go to make their case. Medical personnel must educate civil authorities about this syndrome and the dangers it represents to the child.
Body Weight↗
Exercise conditioning in children with asthma.
A controlled study of 20 children with asthma who participated in a 4-month running program (30 min/day, 3 days/wk), using inhalation of a beta-2 stimulant prior to each running session to prevent exercise-induced asthma, showed significant increases in work tolerance (120.5 +/- 45.0 W before, 131.5 +/- 43.5 W after, P = 0.002) and cardiopulmonary fitness (peak oxygen consumption 37.6 +/- 8.6 ml/kg/min before, 43.1 +/- 10.5 ml/kg/min after, P less than 0.001). Asthma severity judged by daily asthma diary scores and twice daily peak flows did not change. Thirteen control patients with asthma did not exercise and demonstrated no change in work tolerance, fitness, or severity of asthma. Thus, children with asthma can safely engage in a running program and can increase work tolerance and fitness without worsening their asthma.
Adolescent↗
Heat acclimation in cystic fibrosis.
Cystic fibrosis (CF) patients may be at risk for heat illness because of their high sweat chloride and sodium concentrations ([Cl-], [Na+]), but it is not known if they can heat acclimate. We studied 10 CF patients and 10 normal controls on 8 consecutive days of cycle exercise in the heat (37 degrees C dry bulb, 24-29 degrees C wet bulb). Both groups acclimated. CF peak rectal temperature (Tre) was 38.2 +/- 0.3 degrees C on day 1 and 37.8 +/- 0.4 degrees C on day 8 (P less than .005), and peak heart rates (HR) were 151 +/- 24 beats/min on day 1 and 136 +/- 22 beats/min on day 8 (P less than 0.025). Control temperature (T) and HR were similar. Controls decreased sweat [Cl-] from 37.2 +/- 14.6 meq/l on day 1 and to 24.9 +/- 10.6 meq/l on day 8 (P less than 0.005). CF sweat [Cl-] was significantly higher and did not change with acclimation (day 1, 71.1 +/- 20.9 meq/l; day 8, 72.6 +/- 21.6 meq/l, NS). Before and after acclimation, exercise-heat sessions resulted in significant decreases in serum [Cl-] in CF patients (104.5 +/- 4.6 to 101.3 +/- 4.4 meq/l on day 1, P less than 0.05; 103.5 +/- 5.1 to 99.7 +/- 4.2 meq/l on day 8, P less than 0.025) but not in controls. Serum [Cl-] was significantly lower in CF than control subjects at every measurement. Both groups had significant renal Na+ conservation after exercise on both days.(ABSTRACT TRUNCATED AT 250 WORDS)
Acclimatization↗
Oxygen saturation during exercise in cystic fibrosis.
Cystic fibrosis (CF) is often associated with low hemoglobin oxygen saturation and with limited exercise tolerance, yet published reports do not agree on the effect of exercise on oxygenation in CF. We studied oxygen saturation (SaO2) by ear oximetry in 91 patients with CF during progressive exercise to exhaustion. Only 13 of 91 patients changed SaO2 by 5% or more; of these, 4 patients increased SaO2 by 5% or more, whereas 9 decreased by 5% or more. Small changes in SaO2 did not relate to resting pulmonary function, but large decreases in SaO2 were much more likely to be found in patients with forced expiratory volume in one second (FEV1) less than 50% of VC than in those with better pulmonary function (desaturation of 5% or greater was found in only 1 of 62 patients with FEV1 greater than 50% of VC, but in 8 of 29 patients with FEV1 less than or equal to 50% VC). However, even in severely affected patients, modest increases or no change in saturation were more common than large decreases, and 17 of 29 patients with FEV1 less than 50% VC ended exercise with SaO2 greater than 90%, including 3 patients with initial SaO2 below 90%. No single resting pulmonary function test or combination of tests could predict oxygen changes with exercise. Most patients with CF tolerate even maximal exercise without significant desaturation, but patients with FEV1 less than 50% of VC should have supervised exercise testing with ear oximetry before undertaking an exercise program.
Adolescent↗
The infant seat as treatment for gastroesophageal reflux.
Positioning in the infant seat ("chalasia chair") for treatment of infants with gastroesophageal reflux is presumed to have a beneficial effect. We undertook a controlled, prospective study of such positioning to evaluate this purported benefit. Nine infants with documented gastroesophageal reflux participated in 18 paired two-hour postprandial trials in an infant seat and in the horizontal prone position. Distal esophageal pH monitoring demonstrated longer exposure to gastroesophageal reflux while infants were in the seat than when they were prone (28.2 +/- 6.4 per cent vs. 12.8 +/- 3.7 per cent of total time with pH less than 4.0, P = 0.023), a difference due largely to more episodes (16.0 +/- 2.4 vs. 10.1 +/- 2.3 per two-hour postprandial period, P = 0.002). We conclude that the infant seat, rather than being therapeutic in gastroesophageal reflux in children under six months of age, is actually detrimental, when compared with simply placing an infant prone.
Clinical Trials as Topic↗
Magnesium deficiency in cystic fibrosis.
We have described the first case of symptomatic magnesium deficiency in a patient with cystic fibrosis and have discussed its causes. Such deficiency probably occurs with significant frequency in cystic fibrosis and should be considered in such patients so that important morbidity can be prevented.
Adult↗