Fabíola Villac Adde

Nutritional follow-up of cystic fibrosis patients: the role of nutrition education

OBJECTIVE To evaluate the nutritional status of a group of cystic fibrosis patients and establish the role of nutrition education addressed to them in a comparative study before and after intervention. METHODS All cystic fibrosis patients in regular follow-up in the pulmonology clinic of Instituto da Crianca during 1996-99 were prospectively monitored for 3.5 years. Measurements of weight, height, mid upper arm circumference, skinfolds and calculations of weight/age, height/age, weight/height, mid upper arm circumference and triceps z scores, percentage of ideal weight for height, percentage of body fat, check of the use of enzymes with meals and of the use of nutritional supplements were performed at four points in time: initial (I), 7 (II), 13 (III) and 43 (IV) months after the first evaluation. Nutritional counseling was given both verbally and in writing (booklet) to all patients. RESULTS Seventy-four patients, 38F/36M, age range 6 months to 18.4 years were evaluated. At study entry the anthropometric data showed: percentage of ideal weight for height = 94+/-13, percentage of body fat = 15+/-7.1, z scores for weight/age = -1.13+/-1.3, z scores for height/age = -0.94+/-1.2, z scores for weight/height = -0.69+/-1.1, z scores for mid upper arm circumference = -1.35+/-1.3, triceps z scores = -0.74+/-0.9. Compliance with enzyme therapy and use of high-calorie supplements improved during the study period. There was a significant increase in weight/height and triceps z scores and percentage of body fat throughout the study period. After stratifying patients into three age groups the anthropometric improvement was only significant among children under 5 years of age CONCLUSIONS Mild malnutrition was present in this group of cystic fibrosis patients. The nutrition education led to an improvement in compliance with enzyme therapy, use of nutritional supplements and in nutritional status, mostly among the younger patients.

Comparison between classic Gibson and Cooke technique and sweat conductivity test in patients with and without cystic fibrosis

OBJECTIVE To compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with the sweat conductivity values obtained using Macroduct collection system in patients with and without cystic fibrosis (CF). The cost and time spent to carry out each test were also analyzed. METHODS The sweat test using both techniques was performed at the same time in patients with and without CF. Conductivity cutoff values to rule out or diagnose CF were < 75 and > or = 90 mmol/L, respectively, and for the classic test the chloride values were < 60 and > or = 60 mmol/L. RESULTS Fifty-two patients with CF (29 males and 23 females; aged from 1.5 to 18.2 years) underwent the sweat test using both techniques, showing median sweat chloride and conductivity values of 114 and 122 mmol/L, respectively. In all of them, conductivity was > or = 95 mmol/L, which provided the test with 100% sensitivity (95%CI 93.1-100). Fifty patients without CF (24 males and 26 females; aged from 0.5 to 12.5 years) had median sweat chloride and conductivity values of 15.5 and 30 mmol/L, respectively. In all cases, conductivity was < 70 mmol/L, which provided the test with 100% specificity (95%CI 92.9-100). Time spent to perform the tests was significantly shorter for the conductivity test, and its cost was also lower. CONCLUSIONS The conductivity test showed high sensitivity and specificity, and there was good correspondence between the tests. The time spent to carry out the conductivity test was shorter and the cost was lower in comparison with the classic test.

Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity.

INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV1), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 ± 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was −1.1 ± 1.106 and the mean FEV1 was 59 ± 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV1 (r  =  0.76), 6-minute walk test (r  =  0.71), chest radiography (r  =  0.71) and chest computed tomography (r  =  −0.78). When patients were divided according to FEV1, a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV1 <70% (r  =  0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations.

Sweat conductivity: An accurate diagnostic test for cystic fibrosis?

BACKGROUND Sweat chloride test is the gold standard test for cystic fibrosis (CF) diagnosis. Sweat conductivity is widely used although still considered a screening test. METHODS This was a prospective, cross-sectional, diagnostic research conducted at the laboratory of the Instituto da Criança of the Hospital das Clínicas, São Paulo, Brazil. Sweat chloride (quantitative pilocarpine iontophoresis) and sweat conductivity tests were simultaneously performed in patients referred for a sweat test between March 2007 and October 2008. Conductivity and chloride cut-off values used to rule out or diagnose CF were <75 and ≥90 mmol/L and <60 and ≥60 mmol/L, respectively. The ROC curve method was used to calculate the sensitivity, specificity, positive (PPV) and negative predictive value (NPV), as well as the respective 95% confidence intervals and to calculate the area under the curve for both tests. The kappa coefficient was used to evaluate agreement between the tests. RESULTS Both tests were performed in 738 children, and CF was ruled out in 714 subjects; the median sweat chloride and conductivity values were 11 and 25 mmol/L in these populations, respectively. Twenty-four patients who had received a diagnosis of CF presented median sweat chloride and conductivity values of 87 and 103 mmol/L, respectively. Conductivity values above 90 mmol/L had 83.3% sensitivity, 99.7% specificity, 90.9% PPV and 99.4% NPV to diagnose CF. The best conductivity cut-off value to exclude CF was <75 mmol/L. Good agreement was observed between the tests (kappa: 0.934). CONCLUSIONS The sweat conductivity test yielded a high degree of diagnostic accuracy and it showed good agreement with sweat chloride. We suggest that it should play a role as a diagnostic test for CF in the near future.

Follow-up on pediatric patients with bronchiolitis obliterans treated with corticosteroid pulse therapy.

BackgroundBronchiolitis obliterans (BO) is a rare but severe disease in children. Currently, there is no consensus on the treatment for BO with respect to the systemic use of corticosteroids. Here we report on the follow-up of children with a diagnosis of BO who were treated with corticosteroid pulse therapy.MethodsForty patients fulfilling the BO diagnosis criteria were treated with methylprednisolone pulse therapy in monthly cycles until clinical improvement. After the pulse therapy began, we analyzed the clinical and laboratory data at intervals. Statistical analyses were performed using non-parametric tests to compare repeated measures (Friedman, Wilcoxon) or paired nominal data (McNemar) (α = 5%).ResultsThe frequency of wheezing exacerbations and hospitalizations was reduced (p = 0.0042 and p < 0.0001, respectively) and oxygen saturation improved (p = 0.0002) in the pulse therapy-treated patients. Prolonged oral corticosteroid therapy was discontinued in 83% of these patients. The mean Z-score length for age improved from -1.08 to -0.63, and the mean Z-score weight for age improved from -0.91 to -0.59. The adverse effects during the infusion were temporary and none were serious.ConclusionsOur data suggest that pulse corticotherapy could be a safe alternative to prolonged systemic oral corticotherapy in children with BO, thus minimizing the adverse effects of the oral therapy. New prospective controlled studies are required to confirm this proposition.

Discinesia ciliar primária na infância

OBJECTIVES: To point out primary ciliary dyskinesia as a cause of chronic respiratory disease in children.METHODS: A 10 year literature review on Medline and by direct research about the subject.RESULTS AND CONCLUSIONS: Primary ciliary dyskinesia is a disorder characterized by an abnormal mucociliary clearance. It affects both the upper and lower respiratory tracts and usually the clinical manifestations start in the first years of life. It can progress to bronchiectasis. Kartageners syndrome is the typical genetic manifestation. The diagnosis may be based on an abnormal saccharin test, but its confirmation depends on abnormal ultrastructure of the cilia or abnormal ciliary function. Many ciliary defects are currently known. The treatment is supportive, with measures to enhance mucociliary clearance, such as chest physiotherapy, prevention of infections by immunizations and prompt antibiotic therapy in the acute respiratory infections.

Acute bronchodilator responsiveness to tiotropium in postinfectious bronchiolitis obliterans in children.

BACKGROUND Patients with postinfectious bronchiolitis obliterans (PIBO) usually have severe airflow obstruction and respond poorly to β-adrenergic drugs. However, the bronchodilator response to an anticholinergic agent such as tiotropium bromide is not known. We studied the acute bronchodilator response to tiotropium for up to 24 h in children with PIBO using spirometric and plethysmographic criteria. METHODS A randomized, double-blind, placebo-controlled, crossover, prospective study was performed in patients with stable PIBO, 6 to 16 years of age. Standard spirometry and plethysmography were performed before and at 30, 60, 120, and 180 min and 24 h after inhalation of 18 μg of tiotropium or a placebo. After 7 to 14 days, the drugs were inverted, and the procedures were repeated. The changes in lung function parameters at each time point were compared with the baseline by analysis of variance and Tukey posttest, and the differences in all time points assessments vs baseline in tiotropium vs placebo groups were compared using the Friedman test. RESULTS A total of 30 patients were enrolled in the study (23 boys, seven girls; aged 10.9 ± 2.8 years) with baseline lung function values (% predicted) of FVC, FEV1, FEV1/FVC, forced expiratory flow between 25% and 75% of FVC (FEF25%-75%), total lung capacity (TLC), residual volume (RV), RV/TLC, airway resistance (Raw), and specific airway conductance (sGaw) of 75 ± 15, 48 ± 14, 59 ± 11, 22 ± 11, 120 ± 19, 281 ± 101, 49 ± 13, 250 ± 65, and 23 ± 9, respectively. Statistically significant differences were observed after tiotropium inhalation in the following parameters compared with baseline: FVC at 60, 120, and 180 min and 24 h; FEV1 at 30, 60, 120, and 180 min; FEV1/FVC at 60, 120, and 180 min; FEF25%-75% at 60, 120, and 180 min; RV at 30, 60, 120, and 180 min; TLC at 30, 120, and 180 min; RV/TLC at 30, 60, 120, and 180 min; Raw at 30, 60, 120, and 180 min and 24 h; and sGaw at 30, 60, 120, and 180 min and 24 h. For the placebo group, no significant differences were observed in any lung function parameters at any time. The differences in the main functional measurements between the tiotropium and placebo groups were statistically significant. CONCLUSIONS Tiotropium acutely decreased airway obstruction and air trapping for up to 24 h in children with PIBO.

Dornase alfa improves the health‐related quality of life among Brazilian patients with cystic fibrosis—A one‐year prospective study

Health‐related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction.

Comparação entre o método clássico de Gibson e Cooke e o teste da condutividade no suor em pacientes com e sem fibrose cística

OBJECTIVE: To compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with the sweat conductivity values obtained using Macroduct® collection system in patients with and without cystic fibrosis (CF). The cost and time spent to carry out each test were also analyzed. METHODS: The sweat test using both techniques was performed at the same time in patients with and without CF. Conductivity cutoff values to rule out or diagnose CF were 90 mmol/L, respectively, and for the classic test the chloride values were 60 mmol/L. RESULTS: Fifty-two patients with CF (29 males and 23 females; aged from 1.5 to 18.2 years) underwent the sweat test using both techniques, showing median sweat chloride and conductivity values of 114 and 122 mmol/L, respectively. In all of them, conductivity was > 95 mmol/L, which provided the test with 100% sensitivity (95%CI 93.1-100). Fifty patients without CF (24 males and 26 females; aged from 0.5 to 12.5 years) had median sweat chloride and conductivity values of 15.5 and 30 mmol/L, respectively. In all cases, conductivity was < 70 mmol/L, which provided the test with 100% specificity (95%CI 92.9-100). Time spent to perform the tests was significantly shorter for the conductivity test, and its cost was also lower. CONCLUSIONS: The conductivity test showed high sensitivity and specificity, and there was good correspondence between the tests. The time spent to carry out the conductivity test was shorter and the cost was lower in comparison with the classic test.

Recommendations for Long-term Home Oxygen Therapy in Children and Adolescents☆

OBJECTIVE To advise pediatricians, neonatologists, pulmonologists, pediatric pulmonologists, and other professionals in the area on the main indications and characteristics of long-term home oxygen therapy in children and adolescents. DATA SOURCE A literature search was carried out in the MEDLINE/PubMed database (1990 to 2011). Additionally, references from selected studies were included. As consistent scientific evidence does not exist for many aspects, some of the recommendations were based on clinical experience. DATA SYNTHESIS Long-term home oxygen therapy has been a growing practice in pediatric patients and is indicated in bronchopulmonary dysplasia, cystic fibrosis, bronchiolitis obliterans, interstitial lung diseases, and pulmonary hypertension, among others. The benefits are: decrease in hospitalizations, optimization of physical growth and neurological development, improvement of exercise tolerance and quality of sleep, and prevention of pulmonary hypertension/cor pulmonale. The levels of oxygen saturation indicative for oxygen therapy differ from those established for adults with chronic obstructive pulmonary disease, and vary according to age and disease. Pulse oximetry is used to evaluate oxygen saturation; arterial blood gas is unnecessary. There are three available sources of oxygen: gas cylinders, liquid oxygen, and oxygen concentrators. The flows used are usually smaller, as are the number of hours/day needed when compared to the use in adults. Some diseases show improvement and oxygen therapy discontinuation is possible. CONCLUSIONS Long-term home oxygen therapy is increasingly common in pediatrics and has many indications. There are relevant particularities when compared to its use in adults, regarding indications, directions for use, and monitoring.