Bronchodilator response assessment through impulse oscillometry system and spirometry in children and adolescents with cystic fibrosis.

OBJECTIVE: To investigate the effect of bronchodilator on the respiratory mechanics and pulmonary function of children and adolescents with cystic fibrosis. METHODS: Cross-sectional study on clinically stable children and adolescents with cystic fibrosis aged from six to 15 years. Participants underwent impulse oscillometry and spirometry evaluations before and 15 minutes after bronchodilator inhalation. The Kolmogorov-Smirnov test was applied to verify the sample distribution, and the Student's t-test and Wilcoxon test were used to compare the data before and after bronchodilator inhalation. RESULTS: The study included 54 individuals with a mean age of 9.7±2.8 years. The analysis showed a statistically significant improvement in impulse oscillometry and spirometry parameters after bronchodilator inhalation. However, according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) recommendations (2020 and 2021), this improvement was not sufficient to classify it as a bronchodilator response. CONCLUSIONS: The use of bronchodilator medication improved respiratory mechanics and pulmonary function parameters of children and adolescents with cystic fibrosis; however, most patients did not show bronchodilator response according to ATS/ERS recommendations.

Teste de elastase pancreática fecal para pacientes com dúvida diagnóstica de insuficiência pancreática exócrina em pacientes com Fibrose Cística / Fecal pancreatic elastase test for patients with diagnostic doubt of pancreatic insufficiency exocrine in patients with Cystic Fibrosis

INTRODUÇÃO: A FC é uma doença genética, com incidência nacional de aproximadamente 1:7.576 nascidos vivos. A FC é uma doença complexa e multissistêmica, de característica progressiva e potencialmente letal, que afeta o epitélio do sistema respiratório, gastrintestinal, hepático e genitourinário e que ocorre mais frequentemente em populações descendentes de caucasianos. Estima-se que ao final do primeiro ano de vida, cerca de 85% dos pacientes tenham Insuficiência Pancreática Exócrina (IPE). De 10 a 15% dos pacientes, os quais possuem suficiência pancreática, podem desenvolver insuficiência em qualquer fase da vida. Atualmente, os testes fornecidos pelo Sistema Único de Saúde (SUS) são dois, o qualitativo (Sudam III) e o quantitativo de gordura nas fezes. O método quantitativo (método de Van de Kamer) é considerado o padrão-ouro na detecção da esteatorreia. Entretanto, este método exige dieta com ingestão controlada de gordura e coleta de fezes por três dias consecutivos, além de não diferenciar a razão da esteatorreia. Nos pacientes com FC, a esteatorreia é majoritariamente causada pela IPE, embora pacientes com IPE com quadros mais leves nem sempre apresentem esteatorreia. Neste contexto, o teste de elastase pancreática fecal (EL-1) apresenta-se como uma alternativa ao diagnóstico com o método quantitativo de Van de Kamer, principalmente pelo fato das alteraç

Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis.

Lung clearance index (LCI) is a biomarker of ventilation inhomogeneity. Data are scarce on its usefulness in daily practice for monitoring the effects of treatments in older children and adults with CF. In this French observational study of lumacaftor-ivacaftor, 63 of 845 patients (7.5%) had available LCI performed at baseline and at six (M6; n=34) or 12 months (M12; n=46) after lumacaftor-ivacaftor initiation. At inclusion, median [IQR] age was 16 years [13-17], ppFEV1 was 72.8 [59.6-80.7], and LCI was 12.3 [10.3-15.0]. At both M6 and M12, no statistically significant LCI increases of 0.13 units or 1.34% (95% CI: -4.85-7.53) and 0.6 units or 6.66% (95% CI: -0.03-13.5) were observed. Discordant results between LCI and ppFEV1 were observed in one-third of the patients. In daily practice, LCI monitoring in adolescents and young adults with moderate lung disease gives results that are more heterogenous than those reported in children with milder disease.

Association between insurance variability and early lung function in children with cystic fibrosis.

BACKGROUND: Lower socioeconomic status (SES) has consistently been associated with poorer outcomes in individuals with cystic fibrosis (CF). Previous studies have compared outcomes for children with and without private insurance coverage, however the potential role of changes in insurance status on early health outcomes in children with CF remains unknown. OBJECTIVES: To describe the variability in insurance status in early childhood and to evaluate whether insurance variability was associated with poorer outcomes at age 6. METHODS: Retrospective observational study using the Cystic Fibrosis Foundation Patient Registry. Insurance status was defined as: always private (including Tricare), exclusively public, or intermittent private insurance (private insurance and exclusively public insurance in separate years) during the first 6 years of life. Outcomes at age 6 included body mass index (BMI) and FEV1 percent predicted (maxFEV1pp). RESULTS: From a 2000-2011 birth cohort (n = 8,109), 42.3% always had private insurance, 30.0% had exclusively public insurance, and 27.6% had intermittent private insurance. BMI percentiles did not differ between groups; however, children with intermittent private insurance and exclusively public insurance had a 3.3% and 6.6% lower maxFEV1pp at age 6, respectively, compared to those with always private insurance. CONCLUSIONS: A substantial proportion of young children in a modern CF cohort have public or intermittent private insurance coverage. While public insurance has been associated with poorer health outcomes in CF, variability in health insurance coverage may also be associated with an intermediate risk of disparities in lung function as early as age 6.

The remaining barriers to normalcy in CF: Advances in assessment of CF lung disease.

Despite early diagnosis of cystic fibrosis (CF) through newborn screening, a substantial proportion of infants and young children with CF still demonstrate physiologic and structural evidence of lung disease progression, such as obstructive airway disease and bronchiectasis. The growing availability of highly effective CF transmembrane conductance regulatory modulator therapy to the vast majority of people with CF has led to the potential to alter the natural history of CF lung disease, but to assess the full impact of these therapies on CF lung disease and to help guide treatment, sensitive measures of early and mild disease are needed. Chest imaging using computed tomography or magnetic resonance imaging is one approach, but technologic barriers and/or concern about exposure to ionizing radiation may limit its use. However, advances in physiologic measurement techniques and exhaled breath analysis offer another option for assessment of CF lung disease.

New strategies of physical activity assessment in cystic fibrosis: a pilot study.

BACKGROUND: Regular physical activity (PA) is a valued part of cystic fibrosis (CF) care. Although the accelerometer, SenseWear Armband (SWA), accurately measures habitual PA in CF, it is mostly used for research purposes. For the first time, we analyzed different methods of measuring PA in daily life by the use of smartphones and other electronic devices such as smartwatch and Fitbit. METHODS: Twenty-four stable adults with CF (mean age 37.5 ± 11.5SD yrs.; FEV1 58 ± 19% predicted, BMI 22.9 ± 3.2) were studied. Daily PA was monitored for seven consecutive days. All patients wore the accelerometer SWA and at the same time they monitored PA with the electronic device they used routinely. They were allocated into one of four arms according to their device: Smartwatch, Fitbit, Android smartphones and iOS smartphones. PA related measurements included: duration of PA, energy expenditure, number of steps. RESULTS: There was a good agreement between SWA and Fitbit for number of steps (p = 0.605) and energy expenditure (p = 0.143). iOS smartphones were similar to SWA in monitoring the number of steps (p = 0.911). Significant differences were found between SWA and both Smartwatch and Android smartphones. CONCLUSIONS: Fitbit and iOS smartphones seem to be a valuable approach to monitor daily PA. They provide a good performance to measure step number compared to SWA.

A longitudinal assessment of non-invasive biomarkers to diagnose and predict cystic fibrosis-associated liver disease.

BACKGROUND & AIMS: A practical, inexpensive, and non-invasive biomarker of liver fibrosis is needed as a reliable screening test for cystic fibrosis-associated liver disease (CFLD). Studies have shown the utility of AST to Platelet Ratio Index (APRI), fibrosis index based on 4 factors (FIB-4), and gamma-glutamyl transferase (GGT) as good biomarkers for identifying CFLD. The goal of the study was to evaluate the effectiveness of APRI, FIB-4, AST/ALT ratio, platelet count, GGT, and GGT platelet ratio (GPR) in predicting CFLD development. METHODS: Data was collected from CF Foundation Patient Registry for patients aged 3-21 years at Johns Hopkins from January 1, 2002 to December 31, 2014. Collected data included demographic characteristics, presence of splenomegaly, hepatomegaly, ascites, and variceal bleeding, AST, ALT, GGT, platelet count, and FEV1. The sensitivity and specificity of each biomarker were analyzed and reported by the area under receiver operating characteristic (AUROC) curve. RESULTS: By the end of the study, 144 "healthy" CF, 12 CFLD, 19 CF-associated pulmonary disease (CFPD), and 4 CFLD with CFPD cases were identified. APRI scores were higher in CFLD, 0.85 versus 0.28 in "healthy" CF and 0.23 in CFPD groups (p<0.001). GPR had the highest AUROC curve at 0.91. CONCLUSIONS: GPR, GGT, APRI score, and platelet count were potentially useful biomarkers while FIB-4 did not predict CFLD development. Cost-effectiveness studies are needed to analyze the utility of these biomarkers in clinical practice.

Cystic fibrosis 2019: Year in review.

The evidence base for modulator therapies in cystic fibrosis (CF) has continued to expand, and it is likely that up to 90% of people with CF could benefit. Worldwide there are however marked inequalities of access to basic CF care and modulator therapies. For infants and young children there is now an evidence base for inhaled hypertonic saline. There is increasing evidence that structural lung disease in CF is not due purely to infection and that mucus retention and inflammation are also key, and further evidence of the value of azithromycin in those chronically infected with Pseudomonas aeruginosa. Finally, exercise is good for you, but airway clearance is better for mucus clearance.

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography.

BACKGROUND: Chest computed tomography (CT) in children with cystic fibrosis (CF) is sensitive in detecting early airways disease. The pressure-controlled CT-protocol combines a total lung capacity scan (TLC PC-CT) with a near functional residual capacity scan (FRC PC-CT) under general anesthesia, while another CT-protocol is acquired during free breathing (FB-CT) near functional residual capacity. The aim of this study was to evaluate the sensitivity in detecting airways disease of both protocols in two cohorts. METHODS: Routine PC-CTs (Princess Margaret Children's Hospital) and FB-CTs (Erasmus MC-Sophia Children's Hospital) were retrospectively collected from CF children aged 2 to 6 years. Total airways disease (%disease), bronchiectasis (%Bx), and low attenuation regions (%LAR) were scored on CTs using the Perth-Rotterdam annotated grid morphometric analysis-CF method. The Wilcoxon signed-rank test was used for differences between TLC and FRC PC-CTs and the Wilcoxon rank-sum test for differences between FRC PC-CTs and FB-CTs. RESULTS: Fifty patients with PC-CTs (21 male, aged 2.5-5.5 years) and 42 patients with FB-CTs (26 male, aged 2.3-6.8 years) were included. %Disease was higher on TLC PC-CTs compared with FRC PC-CTs (median 4.51 vs 2.49; P < .001). %Disease and %Bx were not significantly different between TLC PC-CTs and FB-CTs (median 4.51% vs 3.75%; P = .143 and 0.52% vs 0.57%; P = .849). %Disease, %Bx, and %LAR were not significantly different between FRC PC-CTs and FB-CTs (median 2.49% vs 3.75%; P = .055, 0.54% vs 0.57%; P = .797, and 2.49% vs 1.53%; P = .448). CONCLUSIONS: Our data suggest that FRC PC-CTs are less sensitive than TLC PC-CTs and that FB-CTs have similar sensitivity to PC-CTs in detecting lung disease. FB-CTs seem to be a viable alternative for PC-CTs to track CF lung disease in young patients with CF.

Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study.

BACKGROUND: Assessment tools for early cystic fibrosis (CF) lung disease are limited. Detecting early pulmonary disease is crucial to increasing life expectancy by starting interventions to slow the progression of the pulmonary disease with the many treatment options available. OBJECTIVE: To compare the utility of lung T1-mapping MRI with ultrashort echo time (UTE) MRI in children with cystic fibrosis in detecting early stage lung disease and monitoring pulmonary exacerbations. MATERIALS AND METHODS: We performed a prospective study in 16 children between September 2017 and January 2018. In Phase 1, we compared five CF patients with normal spirometry (mean 11.2 years) to five age- and gender-matched healthy volunteers. In Phase 2, we longitudinally evaluated six CF patients (median 11 years) in acute pulmonary exacerbation. All children had non-contrast lung T1-mapping and UTE MRI and spirometry testing. We compared the mean normalized T1 value and percentage lung volume without T1 value in CF patients and healthy subjects in Phase 1 and during treatment in Phase 2. We also performed cystic fibrosis MRI scoring. We evaluated differences in continuous variables using standard statistical tests. RESULTS: In Phase 1, mean normalized T1 values of the lung were significantly lower in CF patients in comparison to healthy controls (P=0.02) except in the right lower lobe (P=0.29). The percentage lung volume without T1 value was also significantly higher in CF patients (P=0.006). UTE MRI showed no significant differences between CF patients and healthy volunteers (P=0.11). In Phase 2, excluding one outlier case who developed systemic disease in the course of treatment, the whole-lung T1 value increased (P=0.001) and perfusion scoring improved (P=0.02) following therapy. We observed no other significant changes in the MRI scoring. CONCLUSION: Lung T1-mapping MRI can detect early regional pulmonary CF disease in children and might be helpful in the assessment of acute pulmonary exacerbations.

Utility and validity of dynamic chest radiography in cystic fibrosis (dynamic CF): an observational, non-controlled, non-randomised, single-centre, prospective study.

INTRODUCTION: Dynamic chest radiography (DCR) uses novel, low-dose radiographic technology to capture images of the thoracic cavity while in motion. Pulmonary function testing is important in cystic fibrosis (CF). The tolerability, rapid acquisition and lower radiation and cost compared with CT imaging may make DCR a useful adjunct to current standards of care. METHODS AND ANALYSIS: This is an observational, non-controlled, non-randomised, single-centre, prospective study. This study is conducted at the Liverpool Heart and Chest Hospital (LHCH) adult CF unit. Participants are adults with CF. This study reviews DCR taken during routine CF Annual Review (n=150), validates DCR-derived lung volumes against whole body plethysmography (n=20) and examines DCR at the start and end of pulmonary exacerbations of CF (n=20). The primary objectives of this study are to examine if DCR provides lung function information that correlates with PFT, and lung volumes that correlate whole body plethysmography. ETHICS AND DISSEMINATION: This study has received the following approvals: HRA REC (11 December 2019) and LHCH R&I (11 October 2019). Results are made available to people with CF, the funders and other researchers. Processed, anonymised data are available from the research team on request. TRIAL REGISTRATION NUMBER: ISRCTN 64994816.

Six-Minute Walk Test: Clinical Role, Technique, Coding, and Reimbursement.

The 6-min walk test (6MWT) is a commonly used test for the objective assessment of functional exercise capacity for the management of patients with moderate-to-severe pulmonary disease. Unlike pulmonary function testing, the 6MWT captures the often coexisting extrapulmonary manifestations of chronic respiratory disease, including cardiovascular disease, frailty, sarcopenia, and cancer. In contrast with cardiopulmonary exercise stress testing, this test does not require complex equipment or technical expertise. In this low complexity, safe test, the patient is asked to walk as far as possible along a 30-m minimally trafficked corridor for a period of 6 min with the primary outcome measure being the 6-min walk distance (6MWD) measured in meters. There has been interest in other derived indexes, such as distance-desaturation product (the product of nadir oxygen saturation and walk distance), which in small studies has been predictive of morbidity and mortality in certain chronic respiratory conditions. Special attention to methodology is required to produce reliable and reproducible results. Factors that can affect walk distance include track layout (continuous vs straight), track length, oxygen amount and portability, learning effect, and verbal encouragement. The absolute 6MWD and change in 6MWD are predictive of morbidity and mortality in patients with COPD, pulmonary arterial hypertension, and idiopathic pulmonary fibrosis and patients awaiting lung transplant, highlighting its use in management decisions and clinical trials. As of January 2018, Current Procedural Terminology code 94620 (simple pulmonary stress test) has been deleted and replaced by two new codes, 94617 and 94618. Code 94617 includes exercise test for bronchospasm including pre- and postspirometry, ECG recordings, and pulse oximetry. Code 94618, pulmonary stress testing (eg, 6MWT), includes the measurement of heart rate, oximetry, and oxygen titration when performed. If 94620 is billed after January 2018 it will not be reimbursed.

A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: Results of a randomised controlled trial.

BACKGROUND: Respiratory exacerbations impair lung function and health-related quality of life in people with CF, with delayed identification of exacerbations often resulting in worse outcomes. We developed a smartphone application (app) for adults with CF to report symptoms to the CF team, and investigated its impact on antibiotic use and other outcomes. METHODS: Participants were randomised to intervention (use of the app weekly or sooner if symptoms had worsened) or control (usual care). The app comprised questions relating to symptoms suggestive of an exacerbation. If worsening symptoms were reported, the participant was contacted by the nurse practitioner. The primary outcome measure was the number of courses and days of intravenous (IV) antibiotics. RESULTS: Sixty participants (29 female, aged [mean ±â€¯SD] 31 ±â€¯9 years, FEV1 60 ±â€¯18% predicted) were recruited, with 29 (48%) allocated to the intervention group. Over the 12-month follow-up, there was no clear effect of the app on the number of courses of IV antibiotics (incidence rate ratio [IRR] 1; 95% confidence interval [CI] 0.6 to 1.7), however number of courses of oral antibiotics increased (IRR 1.5; 95% CI 1.0 to 2.2). The median [IQR] time to detection of exacerbation requiring oral or IV antibiotics was shorter in the intervention group compared with the control group (70 [123] vs. 141 [140] days; p = .02). No between-group differences were observed in other outcomes. CONCLUSION: The use of an app reduced time to detect respiratory exacerbations that required antibiotics, however did not demonstrate a clear effect on the number of courses of IV antibiotics.

Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination.

BACKGROUND: The combination of lumacaftor and ivacaftor (LUM/IVA) is a recently approved CFTR modulator treatment for homozygous F508del CF patients. Our study aimed at evaluating the change in the rate of lung function decline after one-year treatment with LUM/IVA. METHODS: The study evaluated patients homozygous for F508del, 12 to 23 years old. All had been treated for one year with LUM/IVA. The collected data included the percent predicted values of FEV1 (ppFEV1) and FVC (ppFVC), and the FEV1/FVC ratio (FEV1/FVC), that corresponded to 12, 24, and 36 months prior to, and 12 months after the initiation of LUM/IVA; also, the 3 highest values of the ppFEV1 (and the corresponding ppFVC, and FEV1/FVC) for the periods 0-12 months, 12-24 months, and 24-36 months prior to as well as the 12-month period after the initiation of LUM/IVA. The baseline lung function was estimated before the commencement of the drug. Data were analyzed longitudinally with generalized estimating equations models and continuous linear splines. A single knot was used that corresponded to the time point of LUM/IVA initiation. RESULTS: Fifty-two patients were analyzed. The multivariate longitudinal analysis of spirometric indices with linear splines demonstrated a significant change in the slopes of ppFEV1 and ppFVC decline, reflecting a significant improvement after the initiation of LUM/IVA treatment. CONCLUSIONS: In this real-world study, lung function improved over a relatively short time period of only one year, after the commencement of LUM/IVA.

New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV1: preliminary results.

OBJECTIVES: Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients. METHODS: The CF intra-hospital standard chest 1.5 T MRI protocol comprises of very short echo-time sequences in submaximal in- and expiration for functional information. Quantitative measurements (Vol/SI at in- and expiration, relative differences (Vol_delta/SI_delta), and cumulative histograms for normalized SI values across the expiratory lung volume) were assessed for correlation to pulmonary function: lung clearance index (LCI) and forced expiratory volume in 1 s (FEV1). RESULTS: In 49 patients (26 male, mean age 17 ± 7 years) significant correlation of Vol_delta and SI_delta (R = 0.86; p < 0.0001) during respiration was observed. Individual cumulated histograms enabled severity disease differentiation (mild, severe) to be visualized (defined by functional parameter: LCI > 10). The expiratory volume at a relative SI of 100% correlated significantly to LCI (R = 0.676 and 0.627; p < 0.0001) and FEV1 (R = - 0.847 and - 0.807; p < 0.0001). Clustering patients according to Vol_SI_100 showed that an amount of ≤ 4% was related to normal, while an amount of > 4% was associated with pathological pulmonary function values. CONCLUSION: Functional pulmonary MRI provides a radiation-free severity assessment tool and can contribute to early detection of lung impairment in CF. Lung volume with SI below 100% of the inspiratory volume represents overinflated tissue; an amount of 4% of the expiratory lung volume was a relevant turning point. KEY POINTS: • Signal intensity and lung volumes are used as potential metric parameters for lung impairment. • Quantification of trapped air impacts on therapy management. • Functional pulmonary MRI can contribute to early detection of lung impairment.

Addressing the burden of illness in adults with cystic fibrosis with screening and triage: An early intervention model of palliative care.

BACKGROUND: Novel models that improve generalist-level palliative care for cystic fibrosis (CF) are needed to address the burden of this illness. A screening-and-triage model has the potential to identify clinical problems requiring immediate follow-up by CF professionals. This study describes such a model and its immediate impact on care delivery for CF patients during a two-year period. METHODS: Eligible adults completed monthly online screening for sources of distress. If results revealed one or more "indicators of concern" on two consecutive screenings, this triggered an attempted triage by a social worker. Completed triages led to prompt follow-up by CF professionals for clinical problems, if indicated. Process data were summarized and generalized linear mixed models were used to evaluate baseline patient characteristics (symptom distress, quality of life, and sociodemographics) associated with the need for prompt follow-up. RESULTS: A total of 1,015 monthly surveys were completed by 74 patients; 634 (66 patients) had >1 indicators of concern; and 164 surveys (46 patients) had >1 indicators for two consecutive surveys (e.g., global distress, pain, dyspnea, and psychological symptoms). The 164 attempted triages yielded 84 completed triages (51.2%), of which 39 (46.4%) required prompt follow-up. In multivariable analyses, older patients and those with higher symptom distress at baseline were more likely to require prompt follow-up (p < .05). CONCLUSIONS: Web-based screening that assesses varied domains of distress or burden can identify a subset of CF patients whose clinical problems may benefit from immediate medical or psychological attention. Additional investigations should improve screening efficiency.

Prevalence of unmet palliative care needs in adults with cystic fibrosis.

BACKGROUND: Physical and emotional burdens impair quality of life (QoL) in many adults with cystic fibrosis (CF). Palliative care (PC) improves QoL in other serious illnesses, yet the full array of palliative needs amenable to PC are unknown in CF. METHODS: We surveyed 164 adults with CF using the Supportive Care Needs Survey 34 (SCNS-34) to assess unmet PC needs across five domains, the Edmonton Symptom Assessment System (ESAS) to assess symptom burden, and the Cystic Fibrosis Questionnaire-Revised (CFQ-R) to assess CF-specific QoL. We assessed associations between SCNS-34 domain scores and respondent characteristics, including symptom burden and FEV1. RESULTS: Median age was 29 years; 56% of respondents were male. Median FEV1 was 57% predicted. 78% of respondents reported ≥1 unmet PC need; physical and daily living (72%) and psychological (66%) needs were most prevalent. Symptom burden was correlated with all SCNS-34 domains scores, and strongly correlated with the physical (r = 0.79) and psychological (r = 0.72) domain scores. FEV1 was moderately inversely correlated with the physical domain score (r = -0.41). Forty-four of the 45 inverse correlations between SCNS-34 domain scores and CFQ-R domain scores were significant. Patient-reported depressive and anxiety symptoms were significantly associated with higher scores in five and four SCNS-34 domains, respectively. CONCLUSIONS: Adults with CF have substantial unmet PC needs. Patient-reported symptom burden is more strongly associated with reporting unmet PC needs than FEV1. Routine screening of unmet PC needs, using tools such as the SCNS-34, may enable CF care teams to optimize the provision of primary and specialist PC.

Prevalence of hepatic steatosis among children and adolescents with cystic fibrosis and its association with nutritional status / Prevalência de esteatose hepática em crianças e adolescentes com fibrose cística e associação com o estado nutricional

ABSTRACT Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student's t test, Mann-Whitney test and chi-square test with significance level of 5%. Results: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043. Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.

RESUMO Objetivo: Determinar a prevalência de esteatose hepática (EH) em crianças e adolescentes com fibrose cística (FC) e associá-la com o estado nutricional. Métodos: Estudo transversal com crianças e adolescentes com diagnóstico de FC. Foram aferidos o peso e a altura para o cálculo do índice de massa corpórea (IMC) e classificação do estado nutricional. A circunferência do braço (CB), a dobra cutânea tricipital (DCT) e a circunferência muscular do braço (CMB) foram empregadas para avaliação da composição corporal. A ultrassonografia abdominal foi realizada para o diagnóstico de EH. Os testes estatísticos empregados foram o teste t de Student, o teste de Mann-Whitney e o teste do qui-quadrado, com nível de significância de 5%. Resultados: Dos 50 pacientes avaliados, 18 (36%) apresentaram EH (Grupo A) e 32 (64%) não (Grupo B). Para as médias de idade (Grupo A: 13,3±5,0 anos; e Grupo B: 11,7±5,0 anos), IMC (Grupo A: 18,0±4,1; e Grupo B: 15,7±3,8) e DCT (Grupo A: 8,4±3,5 mm; e Grupo B: 7,0±2,5 mm), não houve diferença significativa entre os grupos. A média da CB e da CMB diferiram significativamente entre os grupos, sendo mais elevada no grupo com EH, com valores p respectivos de 0,047 e 0,043. Conclusões: É alta a frequência de EH em pacientes com FC e ela não está relacionada com a desnutrição, segundo os parâmetros de IMC, DCT e CMB. Os valores de CB e CMB indicaram maior reserva de massa muscular nos pacientes com EH.