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OBJECTIVES: To evaluate the feasibility of measuring habitual physical activity (HPA) in children with cystic fibrosis (CF) and to assess the relation between HPA and the rate of decline in FEV1 over a period of 2 years. STUDY DESIGN: At regular clinic visits, 109 patients (7 to 17 years; 56 girls) performed pulmonary function testing and completed the Habitual Activity Estimation Scale (HAES). Patients were divided into quartiles, based on activity levels derived from the HAES. RESULTS: Girls in the two lowest activity quartiles had a more rapid rate of decline FEV1 (-3.40% and -3.05% predicted, respectively) than girls in the two highest activity quartiles (-0.93% and +1.17% predicted, respectively) (P = .02). In boys, the rate of decline of FEV1 was similar in all activity quartiles (-1.95% predicted). Patients reported significantly more activity in summer compared with spring, winter, and fall (P < .0001), and boys reported greater activity than girls (6.5 +/- 2.9 vs 5.4 +/- 2.5 h/d, P < .05). CONCLUSIONS: The annual rate of change of FEV1 was related to activity quartile in girls but not in boys. This research suggests that an inactive lifestyle may partially explain the poorer survival of female patients with CF. The HAES is a feasible tool for routine follow-up of HPA in our CF clinic.

Assuntos

Assuntos

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OBJECTIVE: To evaluate the long-term pulmonary sequelae of survivors of bronchopulmonary dysplasia (BPD) of sufficient severity to have required supplemental oxygen for at least 1 month after term. STUDY DESIGN: Fifteen patients with a mean age of 1.1 years were matched to preterm infants of similar gestational age and age at time of study. Pulmonary function testing included spirometry, plethysmographic lung volumes, carbon monoxide diffusion capacity, and in 9 of 15 subjects with BPD, measurement of lung static elastic recoil pressures. RESULTS: The subjects with BPD had a mean expiratory volume in 1 second (FEV1) of 64% +/- 21% predicted (4 had an FEV1 < 50% predicted) compared with 85% +/- 11% (P < .01) for the preterm children in the control group. Subjects with BPD had a significant degree of gas trapping with a residual volume to total lung capacity ratio of 37% +/- 13% compared with 25% +/- 4% for the control group (P < .01). An inverse relationship was seen between the FEV1 and the time on supplemental oxygen (r = -0.84, P < .0001), with 3 of the 4 children whose FEV1 was < 50% requiring oxygen for more than 900 days. Those with the greatest degree of airflow limitation and gas trapping had the greatest abnormalities in both shape and position of the pressure volume curves of the lung. CONCLUSION: Severe BPD may result in moderate to severe long-term abnormalities in pulmonary function tests.

Assuntos

Assuntos

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Pulmonary function tests in adults with sickle cell disease have shown a restrictive pattern that has been attributed to the sequelae of acute chest syndrome (ACS). We compared pulmonary function test results in 37 children with sickle cell anemia (20 with SS hemoglobin (HbSS), 14 with SC hemoglobin, and 3 with S beta hemoglobin) with those in 22 control subjects matched for sex, race, and height and compared pulmonary function in patients with and without a history of ACS. Of the 10 patients with a history of ACS, all but one had HbSS. Pulmonary function tests measured forced vital capacity (FVC), the diffusion capacity of carbon monoxide, and the plethysmographic determination of lung volumes. The FVC and forced expiratory volume in 1 second (FEV1), expressed as the percentage of the predicted value, were significantly less for those with HbSS with or without a history of ACS than for control subjects (p < 0.05), but the FEV1/FVC ratio, an index of airway obstruction, was normal in all groups. Total lung capacity was also significantly lower in patients with HbSS with or without a history of ACS than in control subjects (p < 0.05), but the ratio of residual volume to total lung capacity, another index of airway obstruction, was normal. We conclude that children with sickle cell disease, particularly those with HbSS, may have abnormally small lungs that function normally relative to their size; clustering of ACS episodes is not specifically associated with the observed abnormality.

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The effect of nocturnal oxygen therapy on mortality and morbidity rates and on progression of disease was tested in a double-blind, randomized trial of 28 subjects with advanced cystic fibrosis. Patients were selected on the basis that their awake arterial oxygen tension was less than 65 mm Hg when they were clinically stable. Oxygen was prescribed in 1 L/min increments to obtain an awake arterial oxygen tension of greater than or equal to 70 mm Hg. Subjects received humidified oxygen or room air from modified concentrators. They were enrolled over a 3-year period and followed for an average (+/- SD) of 26 +/- 9 months. The average number of hours per night of concentrator use was 5.3 +/- 3.2 hours in the air group and 7.0 +/- 1.9 hours in the oxygen group. Over the follow-up period there were four deaths in each group, and oxygen therapy had no significant effect on the frequency of hospitalizations. Progression of disease was ascertained from nutritional status, pulmonary function, blood gas values, exercise ability, and right ventricular ejection fraction response to exercise (as measured by equilibrium-gated radionuclide angiocardiography), and psychologic status was measured by standardized tests of mood, self-esteem, and cognitive function; group comparisons for the first year revealed no significant differences; however, school or work attendance was maintained in the oxygen group but deteriorated in the air group. Clinical signs of cor pulmonale were documented during follow-up in 10 patients in toto, and all lived at least 9 months from the onset of these signs. The lack of association between the onset of these signs and imminent death, or the usefulness of measurements of the maximal oxygen uptake during progressive exercise and the right ventricular ejection fraction response to exercise as prognostic indicators, suggest that death may not be the result of cor pulmonale. We conclude that nocturnal oxygen treatment in patients with cystic fibrosis did not appear to affect mortality rates, the frequency of hospitalizations, or the progression of disease; oxygen use should be instituted only after the development of symptoms related to hypoxemia.

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Acute illness in early childhood generates chronic anxiety in parents, which may manifest itself in part by inappropriate use of health care. To minimize this and the development of other psychosocial sequelae associated with neonatal illness, a family support system (FSS) was developed and implemented in a neonatal intensive care unit. The effectiveness of the FSS was assessed by the evaluation of emergency room and inpatient hospital service utilization in 80 patients born before, and 90 patients born after the institution of the program. At the outset, the groups had similar medical and social characteristics. There was no difference between the two groups in the utilization of emergency services in the first year after discharge. However, during the second year the control group used the emergency room twice as often as the study group did (P less than 0.025). During the first 2 years, half of the control group was readmitted, compared with less than a third of the study group (P less than 0.005). Overall, after discharge from the neonatal intensive care unit the control group spent an average of 9 days per patient in hospital, compared with a mean of 3 days per patient in the study group (P less than 0.025). It appears, therefore, that the FSS may be an effective way to reduce some of the psychosocial sequelae of illness in newborn infants requiring intensive care.

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Ten children with cystic fibrosis, aged 3.5 to 12 years, whose weights were lower than 90% of the expected weight for height, received high-calorie elemental enteral alimentation for four weeks. Clinical, anthropometric, and biochemical evaluations as well as blood gas analyses and chest radiograph scoring were performed in all. Pulmonary function tests were performed in the five older children, and progressive exercise tests in three. These evaluations were done before, immediately after, and two months after termination of therapy. Nutritional therapy resulted in an increase of caloric intake and in dramatic weight gain, which persisted only for a short time and was mainly related to adipose tissue accretion. No functional improvement accompanied the amelioration in nutritional status. This short-term nutritional therapy in malnourished children with cystic fibrosis was effective in increasing relative weight and energy stores, but there was no evidence of any long-term functional benefit.

Assuntos

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Both the immediate and long-term effects of chest physical therapy administered by a parent were evaluated in eight children with cystic fibrosis. Spirometric and plethysmographic evaluations were performed pre-CPT and at 5 and 30 minutes post-CPT. The pre-CPT measurements after a three-week period with no CPT were compared with the values while receiving CPT on a regular twice daily basis. There was a significant decrease after three weeks without CPT for FVC (P less than 0.025), FEV1 (P less than 0.005), FEF25-75 (P less than 0.005), and Vmax60TLC (P less than 0.025). When the patients had been receiving CPT on a regular basis, the only immediate effect was an increase in PEFR after 30 minutes post-CPT (P less than 0.05). After three weeks without CPT, there were increases at 30 minutes post-CPI for FVC (P less than 0.005) and Vmax60TLC (P less than 0.05). These findings indicate that although there may be little immediate functional improvement when CPT is received on a regular basis, a three-week period without CPT leads to a worsening of the functional status, which is reversed with renewal of regular CPT.

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Five children born prematurely, eight to ten years ago, who survived the Wilson-Mikity syndrome were compared with six apparently normal prematurely born children and eight normal children born at term, by means of flow volume curves obtained while breathing air and while breathing a mixture of 80% helium and 20% oxygen. The flow rates in air of the group who survived the Wilson-Mikity syndrome were significantly lower than those of the prematurely born normal children (P less than 0.01), and than those of the eight normal children (P less than 0.001). The volume of isoflow while breathing air compared to that while breathing HeO2 was highly variable, but not significantly different in the three groups. Three of the five survivors of the Wilson-Mikity syndrome had a marked concavity in the shape of their flow volume curves. It is concluded that these abnormalities are due to focal increases of compliance or resistances or both of terminal lung units and result in adjacent areas of the lung emptying at different rates. The relationship between physiologic and anatomic abnormalities seen at autopsy in those who die to the disease suggests that they are due to the same pathologic process. Whether or not these abnormalities will disappear with future lung growth remains to be seen.

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Seven children born prematurely who survived the respiratory distress syndrome, seven children born prematurely who had no neonatal lung disease, and seven normal children born at term were studied by comparison of flow volume curves obtained while breathing air to those obtained while breathing 80% helium and 20% oxygen. Expiratory flow rates in air both groups of prematurely born children were lower than flow rates of the children born at term, and the volumes of iso-flow were higher in the survivors of RDS than those of the children born at term. The differences in flow rates in air suggest an increase in large airway resistance in both groups of prematurely born children. It is speculated that this may be secondary to growth retardation related to prematurity. The elevated Viso V in the RDS group suggests an increase in small airway resistance secondary to the disease or to its therapy.

Assuntos