Long Term Nutritional Rehabilitation by Gastrostomy in Israeli Patients with Cystic Fibrosis: Clinical Outcome in Advanced Pulmonary Disease
Ori EfratiMeir Mei‐ZahavJoseph RivlinEitan KeremHannah BlauAsher BarakYoram BujanoverArie AugartenBrijit CochaviYaacov YahavDalit Modan‐Moses
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Objectives: Several studies have shown a linear correlation between nutritional status and pulmonary function in patients with cystic fibrosis. Our study aims were: 1) To evaluate the effect of nutritional supplementation via gastrostomy on nutritional, clinical, and pulmonary parameters, and 2) To identify predicting factors for success of long-term nutritional rehabilitation. Methods: Twenty-one Israeli patients, aged 8 months to 20 years, underwent gastrostomy insertion from 1992 to 2001. All patients were pancreatic insufficient, and all carried severe mutations (W1282X in 62% of the patients). Anthropometric and clinical data were obtained for each patient: 0−12 months before and 6−12 months and 18−24 months after gastrostomy placement. Standard deviation scores (SDS) for height, weight, and body mass index as well as percent of height-appropriate body weight were calculated. Results: The mean percent-of-predicted forced expiratory volume in 1 second (FEV1) decreased significantly during the first year of gastrostomy feeding (n = 16), from 44.2% ± 13.9 to 41% ± 13.3 (P = 0.05). However, during the second year of therapy (n = 10), a trend toward improvement was observed (from 39.4 ± 12.1 to 41.4 ± 16.1). Weight, and BMI z-scores as well as weight percent-of ideal body weight increased significantly. Height z-score for age decreased during the first year (from −1.9 ± 1.3 to −2.1 ± 1.4), However, a trend toward improvement was observed during the second year. A significant correlation was found between the change in weight z-score and height z-score during the first (r = 0.488, P = 0.016) and the second (r = 0.825, P < 0.001) years. There was no difference between compliers and noncompliers regarding height, weight, and BMI either before or after gastrostomy placement. A significant correlation between age at insertion of gastrostomy and improvement in height z-score (r = 0.52, P = 0.016) was observed. Cystic fibrosis related diabetes (n = 8) did not affect the response to supplemental feeding. Conclusions: We observed a trend toward improvement of pulmonary disease during the second year, and a significant improvement in weight, height, and BMI z-scores. Compliance, diabetes, and young age prior to tube insertion did not predict success of nutritional rehabilitation.Keywords:
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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.
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Pulmonary infections, possibly due to poor pulmonary functions, are common in individuals with muscular dystrophy. In order to evaluate the relative effects of seating devices on pulmonary function the forced vital capacity (FVC), forced expiratory volume in one second (FEV 1 ), and forced expiratory time (FET) in eight adults aged between 12 and 21 years with Duchenne Muscular Dystrophy was measured while seated in an adaptive and a nonadaptive seating device. The results showed a 28.0% increase in FVC, a 14.5% increase in FEV 1 , and a 17.3% increase in FET in the adaptive seating system compared to the nonadaptive system. This result emphasizes the need for proper seating as an important way of maximising pulmonary function and minimizing pulmonary complications in this population.
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Objective: To assess the effect on the pulmonary system among wood workers using the most important pulmonary function tests. M ethodology: Male wood workers of age ranging from 20-50 yrs from a wood industry in Mangalore were examined. Spirometric tests were carried out. Forced vital capacity and slow vital capacity tests were done on each subjects. Forced Vital Capacity (FVC) , Forced Expiratory Volume over the 1 st Second (FEV1) and FEV1/FVC – were observed and interpreted. The severity of obstructive and restrictive pulmonary airway was gauged using an electronic spirometer. Results: Almost half of the subjects demonstrated readings expressing mild to moderate obstructive and restrictive airway functions. A very small proportion showed severe obstructive airway function. Considerable decrease in spirometric parameters were found between group exposed to wood dust for less than 5 years and group exposed to wood dust for more than 10 years. Conclusion : The findings of the study suggests that wood dust adversely affects the pulmonary system and also recommends that measures should be taken to reduce the degree of exposure of respirable wood dust. Keywords : wood dust, pulmonary function test, Forced vital capacity, forced expiratory volume over first second.
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Pulmonary function tests were conducted in 212 male workers exposed to fur dust in a fur-processing factory, and in 148 unexposed male workers. The authors used the cumulative dose of dust exposure (mg-yr) as an exposure index to relate to pulmonary function injury, as measured by pulmonary function tests, in exposed workers. The results showed that fur workers had lower percentages of predicted pulmonary function, as measured by forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1.0), and maximal flow rate of expiration at 50% and 25% of forced vital capacity (V50 and V25, respectively), compared with controls (p < 0.01). As the cumulative dose of fur dust exposure increased, average levels of pulmonary function declined significantly (p < 0.01), and pulmonary function abnormalities (i.e., < 80% of predicted FVC and FEV1.0, or < 70% of predicted V50 and V25) increased significantly (p < 0.05). Multiple-regression results identified fur dust exposure as the leading risk factor associated with the decline in pulmonary function in the exposed group. The results demonstrated a dose-response relationship between fur dust exposure and respiratory system injury, as measured by pulmonary function tests in fur-processing workers. On the basis of this dose-response relationship and the use of lifetables, the authors proposed an exposure limit of 4 mg/m3 for fur dust.
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Tests of pulmonary function and radiographic imaging of the chest are the two key methods used in diagnostic evaluation of patients with pulmonary disease. Unlike blood pressure, acceptable normal values vary from person to person and from one demographic group to another. The first studies, in 1846, of spirometric assessment of forced vital capacity (FVC), the most basic pulmonary-function test, showed that normal values for vital capacity vary as a function of height and age.1 A few years later, it was shown that vital capacity was 6 to 12% lower in healthy black soldiers than in white or Native American . . .
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Objectives To evaluate factors affecting the preoperative pulmonary function in patients with scoliosis. Methods Preoperative pulmonary function texts (PFTs) were performed on 108 patients with spinal deformities, 17 males and 91 females, aged (15.2±5.36) (7-27 ), 70 being with idiopathic scoliosis (IS) and 38 with congenital scoliosis (CS). The parameters affecting the preoperative pulmonary function were analyzed. Results The Cobb angle of scoliosis was negatively correlated with vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in one second (FEV1), peak expiratory flow (PEF), forced expiratory flow between 25% to 75% of forced vital capacity ( FEF25-75 ), maxima mid-expiratory flow (MMEF), and maximal ventilatory volume (MVV), and not correlated with minute ventilation (MV) and ratio of forced expiratory volume in one second to forced vital capacity (FEVI/FVC%). The impairment of lung functions was more severe in the CS patients than in the IS patients. The degrees of impairment of lung functions in the groups of the Cobb angle ≥90° with the number of vertebrae involved ≥7 , with the age of onset < 10, and of cephalad location of the curve were more severe than those of the groups with different corresponding parameters. Conclusion The deterioration of pulmonary function mainly depends upon the age of onset, Cobb angle, location of the apex vertebra and the number of involved vertebrae.
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Scoliosis; Respiratory function tests
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Background
Systemic sclerosis (SSc) is a multisystem disease and pulmonary manifestations are common in SSc patients. Involvement of the lungs in SSc patients is detected with x-ray and functional pulmonary testing. The relationship of the development of the pulmonary abnormalities to duration of the disease and other organ involvement remains unclear.Objectives
The present study was designed to determine the pulmonary function indices in the SSc patients without symptoms of the lung involvement.Methods
Twenty-six women with definite diagnosis of SSc, aged 45.4+11.2 yrs were studied. Control values were obtained from 20 healthy age-matched women. Additionally, the SSc group was compared to the predicted spirometric data based on large population studies. The resting pulmonary function tests were performed with EDS-Sprint spirograph. The measurements were done between 8.00–9.00 AM.Results
Vital capacity and maximal voluntary ventilation were decreased in the SSc patients. Forced vital capacity and one-second forced expiratory volume were also decreased but the later was normal when corrected for reduction of the forced vital capacity. Forced expiratory rate at 50% and 75% of forced vital capacity was impaired in the patients. Forced expiratory flow rate at 25% of forced vital capacity did not differ from the controls. The rate of incidence of abnormal pulmonary functional test in the SSc patients was 30–70 per cent.Conclusion
Pulmonary involvement is common in SSc patients without overt manifestation of the lung disease. The lung function testing is a simple, cheap and safe method for evaluation of the respiratory system, and is recommended as a routine test for all patients with SSc.Vital capacity
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Objective To study the influences of respiratory training on pulmonary function and survival quality of sufferers of chronic obstructive pulmonary disease(COPD) in catabasis.Methods Forty-six patients with COPD in catabasis(pulmonary function is from level 2 to level 4) were given respiratory training for 6 months individually or in group;then the differences of pulmonary function and survival quality were observed before and after training.Results Thirty-six patients completed the experiment.The forced vital capacity(P0.05),forced expiratory volume in a second,forced expiratory volume in a second/ forced vital capacity and flow velocity of respiratory peak value were markedly improved after respiratory training(P0.01).The mean scores of 4 survival quality factors were all decreased remarkably(P0.01).Conclusion Respiratory training can improve pulmonary function of COPD sufferers during catabasis and upgrade their survival quality.
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