ABSTRACT
La enfermedad pulmonar avanzada (EPAV) es la principal causa de morbimortalidad en pacientes con Fibrosis Quística (FQ). Objetivo: describir características clínicas de pacientes con FQ con EPAV y mortalidad en el seguimiento. Método: Estudio descriptivo, retrospectivo de pacientes con FQ y EPAV: VEF1 4 años de vida. Un 75% era portador de infección crónica por Pseudomonas. Un 68% era dependiente de oxígeno y un 18% de ventilación mecánica no invasiva. El 70 % tuvo 2 o más hospitalizaciones el último año de seguimiento. De 27 pacientes derivados a trasplante, 7 se trasplantaron, 3 fallecieron en lista para trasplante, 9 presentaron alguna contraindicación: 4 de ellos por desnutrición y 5 por mala adherencia y escasa red de apoyo. En el seguimiento un 32% (n = 14) falleció, 93% de causa respiratoria. Conclusión: Un 39% de los pacientes tenían EPAV cuyo diagnóstico de FQ en promedio fue a los 11,2 años (SD ± 13 a). Las barreras de ingreso a lista para trasplante fueron: desnutrición, mala adherencia y falta de red de apoyo. Esta es una población con una elevada mortalidad.
Advanced cystic fibrosis lung disease (ACFLD) is the leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF). Objective: to describe clinical characteristics of patients with CF with ACFLD and mortality during follow-up. Method: Descriptive, retrospective study of patients with CF and ACFLD: FEVi < 40%, oxygen dependent, and/or referred to a lung transplantprogram. Clinical, microbiological, functional, genetic and mortality characteristics were collected. Results: Of 111 controlled patients, 39% met criteria for ACFLD. 52% were men and the mean age was 29,8 years- old. The average BMI was 19.9 kg/m2, 72% had pancreatic insufficiency and 87% had a genetic study, being the DF508 mutation the most frequent (67%). The average age of diagnosis was 11.2 years (SD ± 13 years), being in 54,5% over the age of 4 years. 75% had chronic Pseudomonas infection. 68% were oxygen dependent and 18% on noninvasive mechanical ventilation. In the last year of follow-up 70% had 2 or more hospitalizations. Of 27 patients who have been referred for transplantation, 7 underwent lung transplantation, 3 died waiting on the transplant list, 9 had contraindications: 4 due to malnutrition and 5 to poor adherence and poor support network. 32% (n = 14) of the ACFLD patients died, 93% due to respiratory causes. Conclusion: 39% of the patients had ACFLD. The average age for CF diagnosis was 11.2 years (SD ± 13 years) Barriers to entering the transplant list are: malnutrition, poor adherence, and lack of a support network. This is a population with a high mortality.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Young Adult , Cystic Fibrosis/physiopathology , Cystic Fibrosis/mortality , Comorbidity , Survival Analysis , Retrospective Studies , Analysis of Variance , Follow-Up Studies , Lung Transplantation , Cystic Fibrosis/surgery , Cystic Fibrosis/diagnosis , Cystic Fibrosis/microbiology , MalnutritionABSTRACT
La fibrosis quística (FQ) es una enfermedad hereditaria autosómica recesiva, causada por la mutación del gen que codifica la proteína CFTR (cystic fibrosis transmembrane conductance regulator), afecta varios órganos, pero la enfermedad pulmonar es la primera causa de morbimortalidad. El diagnóstico a través del screening neonatal (SNN) y los nuevos tratamientos moduladores del CFTR han aumentado el interés por pesquisar y monitorizar la función pulmonar antes del inicio de los síntomas para lograr un tratamiento adecuado y oportuno con una mejor calidad de vida. Existen numerosas formas de medir la función pulmonar según la edad, colaboración y recursos disponibles. En este artículo se resumen las pruebas clásicas y las más novedosas, como técnicas de imágenes, en la búsqueda de marcadores precoces de daño pulmonar, herramientas con los que cada centro de fibrosis quística debiera contar en la era de tratamientos moduladores del CFTR, que están cambiando el pronóstico de los pacientes con esta enfermedad.
Cystic fibrosis (CF) is an autosomal recessive inherited disease, caused by mutation of the gene encoding the CFTR protein (cystic fibrosis transmembrane conductance regulator), affects several organs, but lung disease is the first cause of morbidity and mortality. Diagnosis through neonatal screening (NNS) and new CFTR modulating treatments have increased interest in screening and monitoring lung function before the onset of symptoms to achieve adequate and timely treatment with a better quality of life. There are numerous ways to measure lung function based on age, collaboration, and available resources. This article summarizes the classic and the most innovative tests, which have emerged from imaging techniques in the search for early markers of lung damage, tools that each cystic fibrosis center should have in the era of CFTR modulating treatments, which are changing the prognosis of patients with this disease.
Subject(s)
Humans , Child, Preschool , Child , Adolescent , Respiratory Function Tests/methods , Cystic Fibrosis/physiopathology , Cystic Fibrosis/diagnostic imaging , Oscillometry , Plethysmography , Spirometry , Magnetic Resonance Imaging , Radiography, Thoracic , Tomography, X-Ray Computed , Respiratory System Agents/therapeutic use , Cystic Fibrosis Transmembrane Conductance Regulator , Cystic Fibrosis/drug therapyABSTRACT
Abstract Background: Cystic fibrosis (CF) is a potentially mortal disease characterized by a chronic pulmonary disease with persistent airway infection. Children with this disease are more susceptible to respiratory infections due to the limitation in mucociliary transport and anatomical disruption of the bronchial tree. SARS-CoV-2 causes COVID-19, a respiratory illness related to exacerbations of chronic pulmonary pathologies in children, such as CF and asthma. There are not enough case reports on pediatric patients with SARS-CoV-2 infection and CF, for which we share our experience. Case report: A 22-month-old male patient diagnosed with CF presented in the hospital with cough, fever, and increased respiratory work. The patient received supplemental oxygen and antibiotic and antiviral therapy. Positive results for type B influenza and RT-PCR (reverse transcription-polymerase chain reaction) for SARS-CoV-2 were obtained. Due to the persistence of respiratory difficulty, high-flow therapy was initiated, with a good response. After an episode of hypoxemia, bradycardia, and increased respiratory work secondary to accumulated secretions, orotracheal intubation and invasive mechanical ventilation were performed. The patient evolved with clinical and gasometric improvement. After 10 days of in-hospital antibiotic management with adequate clinical evolution, the patient was discharged to complete oral treatment and home isolation. Conclusions: We present a case of chronic respiratory disease and SARS-CoV-2 infection with severity criteria in a pediatric patient. The evolution was favorable with timely support management and antibiotic therapy in a third-level hospital.
Resumen Introducción: La fibrosis quística es una afección potencialmente mortal caracterizada por enfermedad pulmonar crónica con infección persistente de las vías aéreas. Los niños con esta enfermedad son más susceptibles a infecciones respiratorias debido a la limitación en el transporte mucociliar y la distorsión anatómica del árbol bronquial. El SARS-CoV-2 (coronavirus tipo 2 del síndrome agudo respiratorio grave) es el virus causante de la COVID-19, enfermedad respiratoria que puede estar relacionada con exacerbaciones de patologías pulmonares crónicas en niños, como la fibrosis quística y el asma. No hay suficientes reportes de casos de pacientes pediátricos con infección por SARS-CoV-2 y fibrosis quística, por lo cual se comparte la presente experiencia. Caso clínico: Paciente de sexo masculino de 22 meses de edad con diagnóstico de fibrosis quística que presentó tos, fiebre y aumento en el trabajo respiratorio. A su ingreso se inició manejo con oxígeno suplementario y tratamiento antibiótico y antiviral. Se obtuvo prueba positiva para influenza tipo B y para SARS-CoV-2 por RT-PCR (reacción en cadena de la polimerasa de transcriptasa inversa). Ante un episodio de hipoxemia, bradicardia y mayor trabajo respiratorio, requirió intubación orotraqueal y ventilación mecánica invasiva. El paciente evolucionó con mejoría clínica y gasométrica. Después de 10 días de manejo antibiótico intrahospitalario, con adecuada evolución clínica, egresó para completar tratamiento por vía oral y aislamiento en casa. Conclusiones: Se presenta el caso de un paciente pediátrico con enfermedad respiratoria crónica de base e infección por SARS-CoV-2 con criterios de gravedad. El paciente evolucionó favorablemente con el manejo de soporte oportuno y terapia de antibióticos en un hospital de tercer nivel.
Subject(s)
Humans , Infant , Male , Cystic Fibrosis/complications , COVID-19/physiopathology , Respiration, Artificial , Severity of Illness Index , Risk Factors , Cough/virology , Reverse Transcriptase Polymerase Chain Reaction , Cystic Fibrosis/physiopathology , Fever/virology , COVID-19/diagnosis , COVID-19/therapyABSTRACT
ABSTRACT Objective: To evaluate factors associated with the performance of children and adolescents with cystic fibrosis (CF) in the Modified Shuttle Test (MST) and compare it with healthy children and adolescents. Methods: This is a cross-sectional study, with children and adolescents divided into two groups: cystic fibrosis (CFG) and control (CG). Variables evaluated in the MST: walking distance, test level, heart rate variation (∆Hr), post-test mean arterial pressure (MAP Pt) and peripheral oxygen saturation variation (∆SPO2). Statistical analysis included Mann Whitney and Spearman coefficient tests, being significant p<0.05. Results: Sixty individuals aged 6-16 years old were evaluated. Anthropometric data was similar between groups. Differences between groups were shown for: baseline heart rate (BHr), peak heart rate (PHr), ∆Hr, recovery heart rate (RHr), post-test respiratory rate (PtBr), saturation variables, peripheral oxygen level (SpO2B) and level test. The ∆Hr and MAP Pt had a moderate positive correlation with distance and level test for both groups (respectively: r=0.6 / p<0.001; r=0.6 / p<0.001). In CFG, the level test had a significant association (r=0.4 - p=0.02) with %FEV1. Conclusions: Children with cystic fibrosis presented functional limitation in the Modified Shuttle Test, which was influenced by lung function.
RESUMO Objetivo: Avaliar os fatores que estão associados ao desempenho de crianças e adolescentes com fibrose cística (FC) no teste modificado de Shuttle (MST) e compará-los com os de crianças e adolescentes saudáveis. Métodos: Estudo de corte transversal com crianças e adolescentes divididos em dois grupos: grupo controle (GC) e grupo FC (GFC). As variáveis avaliadas no MST foram: distância caminhada, nível do teste, variação da frequência cardíaca (∆Fc), pressão arterial média pós-teste (PAMPt) e variação da saturação periférica de oxigênio (∆SpO2). Na análise dos dados, foram utilizados o Teste Mann-Whitney e o coeficiente de Spearman, sendo significante p<0,05. Resultados: Avaliaram-se 60 indivíduos (6-16 anos). Os grupos foram homogêneos em relação aos dados antropométricos. Foi observada diferença significante na frequência cardíaca basal (FcB), na frequência cardíaca de pico (FcP), na ∆Fc, na frequência cardíaca de recuperação (FcR), na frequência respiratória pós-teste (FRPt), na saturação periférica de oxigênio basal (SpO2B) e no nível do teste. A ∆Fc e a PAMPt tiveram correlação moderada positiva (respectivamente, r=0,6 / p<0,001; r=0,6 / p<0,001) com a distância caminhada e o nível do teste em ambos os grupos. No GFC o nível do teste teve associação (r=0,4 / p=0,02) com a porcentagem do predito do volume expiratório forçado do primeiro segundo (%VEF1). Conclusões: Crianças e adolescentes com FC apresentaram limitação funcional no teste modificado de Shuttle, influenciada pela função pulmonar.
Subject(s)
Humans , Male , Female , Child , Adolescent , Cystic Fibrosis/physiopathology , Exercise Test/methods , Cardiorespiratory Fitness , Physical Functional Performance , Oxygen Consumption , Blood Pressure , Case-Control Studies , Cross-Sectional Studies , Heart RateABSTRACT
Los avances en el campo de la fibrosis quística han aumentado la esperanza de vida de estos pacientes, por lo que cada vez es más prevalente la Diabetes Relacionada con la Fibrosis Quística (DRFQ) y sus complicaciones. La DRFQ se asocia a mayor morbimortalidad, deterioro de la función pulmonar y del estado nutricional. Por lo mismo, el manejo óptimo de esta patología depende de un diagnóstico precoz, tratamiento individualizado y vigilancia de las complicaciones diabéticas. El screening de DRFQ debe realizarse anualmente a partir de los 10 años, mediante una Prueba de Tolerancia a la Glucosa Oral (PTGO), lo cual permite el diagnóstico. El manejo de esta patología tiene por objetivo estabilizar y mejorar la función pulmonar y el estado nutricional y metabólico de los pacientes. Actualmente, la insulina es el tratamiento farmacológico de elección para controlar la hiperglicemia y el esquema de uso debe ser individualizado para cada persona. En caso de enfermedades agudas pueden existir mayores requerimientos de insulina. Además, se deben tener consideraciones especiales en cuanto a la dieta y la insuficiencia pancreática exocrina que presentan estos pacientes. Para la vigilancia de complicaciones microvasculares se debe realizar una monitorización anual a partir de los 5 años desde el diagnóstico de DRFQ. Debido a la complejidad de estos pacientes, para alcanzar el mejor cuidado posible se necesita un enfoque multidisciplinario con distintos profesionales de la salud coordinados, incluyendo en la toma de decisiones al paciente y su familia.
Advances made in the field of cystic fibrosis have increased the life expectancy of these patients, which is why Cystic Fibrosis-Related Diabetes (CFRD) and its complications are becoming more and more prevalent. CFRD is associated with increased morbidity and mortality, lower lung function and inadequate weight maintenance. Therefore, the optimal management of this pathology depends on an early diagnosis, individualized treatment and monitoring of diabetic complications. For CFRD, routine screening with an Oral Glucose Tolerance Test (OGTT) should be carried out yearly from the age of 10, which allows to diagnose it. The treatment goals in CFRD are to stabilize and improve lung function and obtain adequate weight gain. Currently, insulin is the pharmacological treatment of choice to control hyperglycemia and the insulin regimen must be personalized for each person. In acute illnesses, there may be higher insulin requirements. In addition, special considerations must be taken regarding diet and exocrine pancreatic insufficiency that these patients present. For the surveillance of microvascular complications, annual monitoring should be carried out 5 years after the diagnosis of CFRD. Due to the complexity of these patients, in order to achieve the best possible care, a multidisciplinary approach is needed with different coordinated health professionals, including the patients and their family in the decision-making process.
Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Diabetes Mellitus/etiology , Diabetes Mellitus/therapy , Patient Care Team , Mass Screening , Cystic Fibrosis/physiopathology , Nutrition Therapy , Diabetes Mellitus/diagnosis , Diabetes Mellitus/physiopathology , Insulins/therapeutic use , Glucose Tolerance Test , Hypoglycemic Agents/therapeutic useABSTRACT
ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.
RESUMO Objetivo: Verificar associação entre qualidade de vida (QV), capacidade funcional e estados clínico e nutricional em crianças e adolescentes com fibrose cística (FC). Métodos: Estudo transversal incluindo pacientes de oito a 18 anos de idade com FC. A QV, a capacidade funcional, o estado nutricional e o estado clínico foram avaliados por meio do Questionário de Fibrose Cística; do teste de caminhada dos 6 minutos (TC6M) e da força de preensão manual (FPM); dos percentis de estatura para a idade e do índice de massa corporal (IMC) para a idade; e da prova de função respiratória, respectivamente. Para a análise dos dados, utilizaram-se os testes de correlação de Pearson e de Spearman e a regressão logística. Resultados: Participaram do estudo 45 pacientes com 13,4±0,5 anos, sendo 60% do sexo feminino, 60% colonizados por Pseudomonas aeruginosa e 57,8% apresentando pelo menos uma mutação F508del. Ao avaliar a percepção da QV, o domínio peso alcançou os escores mais baixos e o digestório, os mais altos. Na prova de função pulmonar, o volume expiratório forçado do primeiro segundo médio foi 77,3±3,3%, e o TC6M e a FPM apresentaram valores na faixa de normalidade. Observou-se associação da QV com a capacidade funcional, o estado nutricional e o estado clínico dos pacientes com FC. Conclusões: Os participantes do estudo apresentaram boas condições clínicas e valores satisfatórios de capacidade funcional e QV. Os achados reforçam que a avaliação da QV pode ser importante para a prática clínica, no manejo do tratamento.
Subject(s)
Quality of Life/psychology , Cystic Fibrosis/physiopathology , Cystic Fibrosis/psychology , Logistic Models , Nutritional Status , Cross-Sectional Studies , Health Status Indicators , Hand Strength , Walk TestABSTRACT
Cystic Fibrosis is a multisystemic inherited disease that requires ongoing care by multidisciplinary teams. The objective of this study is to describe changes on nutrition and lung function in a cohort of patients in a Cystic Fibrosis (CF) Care Center at the Hospital Infantil Universitario San José in Bogotá (HIUSJ), between 2010 and 2013.Is a descriptive study in a cohort of CF patients during four years of follow-up. The quantitative variables were described using medians and interquartile ranges, and the qualitative variables with absolute frequencies and percentages. Descriptive statistics was used to summarize the findings. Of the 63 patients in the initial group, 47 (74.6%) completed the follow-up time. The age range was between 3 to 30 years. The median BMI increased as follows: 17.9 (RIQ: 12.5-25.6) in 2010, 18.6 (RIQ: 12.9-24.8) in 2011, 18.9 RIQ (13.6-26.5) in 2012 and 19.0 (RIQ: 13.5-25.8) in 2013, with lower values in men. The forced expiratory volume in the first second (FEV1) at admission was classified as severe (FEV1 <40%) in 7.1%, moderate (FEV1 40-69%) in 35.7%, mild (FEV1 70-79%) in 7.1% and as normal (FEV1> 80%) in 50%. It is concluded that during the 4 years of follow-up at the HIUSJ CF Center there is an improvement in BMI and a deterioration in lung function in the whole group. The importance of establishing more reference centers to improve clinical outcomes and of implement a National registry to follow up over time are highlighted.
La fibrosis quística es una enfermedad hereditaria, multisistémica, cuyo manejo continuo requiere de equipos multidisciplinarios de salud. El objetivo de este trabajo es describir la evolución nutricional y de la función pulmonar en una cohorte de pacientes en el centro de atención integral de la fibrosis quística (FQ), del Hospital Infantil Universitario San José de Bogotá (HIUSJB), entre 2010 y 2013. Estudio descriptivo, en una cohorte de pacientes, en seguimiento durante cuatro años. Las variables cuantitativas fueron descritas mediante medianas y rangos intercuartílicos y las cualitativas con frecuencias absolutas y porcentajes. De los 63 pacientes del grupo inicial, 47 (74.6%), completaron el tiempo de seguimiento. El rango de edad fue de 3 a 30 años. La mediana del IMC (índice de masa corporal) se incrementó así: 17.9 (RIQ:12.5-25.6) en el 2010, 18.6 (RIQ:12.9-24.8) en el 2011, 18.9 ( RIQ(13.6-26.5) en el 2012 y 19.0 (RIQ:13.5-25.8) en el 2013, con menores valores en los hombres. El volumen espiratorio forzado en el primer segundo (VEF1) al ingreso fue clasificado como severo (VEF1<40%) en el 7.1%, moderado (VEF1 40-69%) en el 35.7%, leve (VEF1 70-79%) en el 7.1% y como normal (VEF1>80%) en el 50%. Se concluye que durante los 4 años de seguimiento en el programa de FQ del HIUSJ, ocurre una mejoría del IMC en todo el grupo y un deterioro de la función pulmonar. Se resalta la importancia de constituir más centros de referencia para mejorar los desenlaces clínicos e implementar un registro Nacional para hacer seguimiento a través del tiempo.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Young Adult , Respiratory Function Tests , Nutritional Status , Delivery of Health Care, Integrated , Cystic Fibrosis/physiopathology , Pseudomonas aeruginosa/isolation & purification , Staphylococcus aureus/isolation & purification , Body Mass Index , Clinical Evolution , Forced Expiratory Volume , Epidemiology, Descriptive , Cohort Studies , Follow-Up Studies , Cystic Fibrosis/microbiology , Hospitalization/statistics & numerical dataABSTRACT
ABSTRACT Objective: To evaluate the association of body mass index (BMI) and albumin with pulmonary function in cystic fibrosis (CF) pediatric subjects. Methods: This is a cross-sectional study with clinically stable CF's subjects. Clinical (pulmonary function) and nutritional evaluation (body mass index and albumin) were performed. Univariate analysis was performed using simple linear correlations. Regression analysis was performed using an exit level of p<0.05. Results: Seventy-eight CF's subjects (mean age 12.8±3.8 years) with mean albumin 4.2±0.4 mg/dL, predicted forced expiratory volume in 1 second (FEV1%) 80.8±22.6 and BMI median percentile 51.2 (1.3-97.7). In the multiple regression models, albumin, age and BMI percentile were associated with pulmonary function. Subjects with lower than 25 BMI percentile had 12.2% lower FEV1%. An albumin increase of 0.1 mg was associated with 2.7% increase in predicted FEV1%, and one year increase in age was associated with reduction in 1.2% of predicted FEV1%. Conclusions: BMI percentile, albumin and age were independently associated with predicted FEV1% in a tertiary referral hospital.
RESUMO Objetivo: Avaliar a associação do Índice de Massa Corporal (IMC) e da albumina com a função pulmonar em pacientes pediátricos com fibrose cística (FC). Métodos: Estudo transversal com pacientes pediátricos com FC clinicamente estáveis. Foram realizadas avaliação clínica (função pulmonar) e nutricional (IMC e albumina). Análise univariada foi realizada usando correlação linear simples. Análise de regressão foi realizada usando o nível de significância de p<0,05. Resultados: Foram incluídos 78 pacientes com FC (média de idade 12,8±3,8 anos) com média de albumina de 4,2±0,4 mg/dL, volume expiratório forçado em um segundo (VEF1%) predito de 80,8±22,6 e mediana do percentual de IMC de 51,2 (1,3-97,7). No modelo de regressão múltipla, albumina, idade e percentual de IMC apresentaram associação com a função pulmonar. Indivíduos com IMC abaixo de 25% apresentaram VEF1% predito 12,2% menor. Um aumento de 0,1 mg de albumina teve associação com aumento de 2,7% no VEF1% predito, e um ano a mais de idade mostrou relação com a redução de 1,2% de VEF1% predito. Conclusão: O percentual de IMC, albumina e idade apresentaram associação independente com o VEF1% predito em um hospital terciário de referência.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Body Mass Index , Cystic Fibrosis/physiopathology , Albumins/metabolism , Lung/physiopathology , Biomarkers/metabolism , Linear Models , Forced Expiratory Volume , Cross-Sectional Studies , Cystic Fibrosis/metabolismABSTRACT
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.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Young Adult , Nutritional Status , Cystic Fibrosis/complications , Malnutrition/complications , Non-alcoholic Fatty Liver Disease/ethnology , Risk Management , Prevalence , Cross-Sectional Studies , Cystic Fibrosis/physiopathology , Malnutrition/diagnosis , Malnutrition/physiopathology , Non-alcoholic Fatty Liver Disease/diagnosis , Non-alcoholic Fatty Liver Disease/epidemiologyABSTRACT
Los canales de cloruros, de sodio, de bicarbonato y los de agua (aquaporinas) se coordinan para mantener la cubierta líquido superficial de las vías respiratorias, que es necesaria para el aclaramiento mucociliar. El mecanismo general para el transporte de electrolitos y agua depende principalmente de la expresión diferencial y distribución de los transportadores y bombas de iones. Los iones y el agua se mueven a través de las vía paracelular o transcelular. La ruta transcelular del transporte de electrolitos requiere un transporte activo (dependiente de ATP) o pasivo (siguiendo gradientes electroquímicos) de iones. La ruta paracelular es un proceso pasivo que está controlado, en última instancia, por los gradientes electroquímicos transepiteliales predominantes. La fibrosis quística es una enfermedad hereditaria que se produce por mutaciones en el gen que codifica la proteína reguladora de la conductibilidad transmembrana de la fibrosis quística (CFTR) que actúa como un canal de cloro y cumple funciones de hidratación del líquido periciliar y mantenimiento del pH luminal. La disfunción del canal de cloro en el epitelio respiratorio determina una alteración en las secreciones bronquiales, con aumento de su viscosidad y alteración de la depuración mucociliar y que asociado a procesos infecciosos puede conducir a daño pulmonar irreversible. La disfunción del CFTR, también se ha visto implicado en la patogénesis de la pancreatitis aguda, en la enfermedad pulmonar obstructiva crónica y la hiperreactividad en el asma. Existen fármacos que aprovechan los mecanismos fisiológicos en el transporte de iones, con un objetivo terapéutico.
The chloride channels, sodium and bicarbonate channels, and aquaporin water channels are coordinated to maintain the airway surface liquid that is necessary for mucociliary clearance. The general mechanism for the transport of electrolytes and fluids depends mainly on the differential expression and distribution of ion transporters and pumps. Ions and water move through the paracellular or transcellular pathways. The transcellular route of electrolyte transport requires an active transport (dependent on ATP) or passive (following electrochemical gradients) of ions. The paracellular pathway is a passive process that is ultimately controlled by the predominant transepithelial electrochemical gradients. Cystic fibrosis is a hereditary disease that is produced by mutations in the gene that encode cystic fibrosis transmembrane conductance regulatory protein (CFTR) that acts as a chloride channel and performs functions of hydration of periciliary fluid and maintenance of luminal pH. The dysfunction of the chlorine channel in the respiratory epithelium determines an alteration in the bronchial secretions, with an increase in its viscosity and alteration of the mucociliary clearance and that associated with infectious processes can lead to irreversible lung damage. CFTR dysfunction has also been implicated in the pathogenesis of acute pancreatitis, chronic obstructive pulmonary disease, and bronchial hyperreactivity in asthma. There are drugs that exploit physiological mechanisms in the transport of ions with a therapeutic objective.
Subject(s)
Humans , Biological Transport, Active/physiology , Mucociliary Clearance/physiology , Ion Transport/physiology , Chloride Channels/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator/metabolism , Cystic Fibrosis/metabolism , Chloride Channels/physiology , Cystic Fibrosis Transmembrane Conductance Regulator/physiology , Cystic Fibrosis/physiopathologyABSTRACT
Spirometry is better pulmonary function test for evaluating preschoolers with chronic lung disease and recurrent wheeze. It is useful, accessible and very good performance. For a correct interpretation it must be under the conditions specially controlled for this age group. In this review, product of the work done during the year 2018, by the Committee on pulmonary function in pediatric pulmonology Chilean society, will be showcased aspects for the realization and interpretation of spirometry in preschool children, with emphasis on the differences in the criteria typically described for older children and adults.
La espirometría es la prueba de función pulmonar más adecuada para evaluar a preescolares con enfermedades pulmonares crónicas y sibilancias recurrentes. Es útil, accesible y de buen rendimiento. Para una correcta interpretación debe realizarse bajo las condiciones especialmente normadas para este grupo etario. En esta revisión, producto del trabajo realizado durante el año 2018, por la comisión de función pulmonar de la sociedad Chilena de Neumología Pediátrica, se expondrán los aspectos actualizados para la realización e interpretación de la espirometría en preescolares, con énfasis en las diferencias de los criterios clásicamente descritos para niños mayores y adultos.
Subject(s)
Humans , Child, Preschool , Spirometry/methods , Respiratory Function Tests , Asthma/diagnosis , Asthma/physiopathology , Severity of Illness Index , Vital Capacity , Forced Expiratory Volume , Cystic Fibrosis/diagnosis , Cystic Fibrosis/physiopathology , Lung Diseases/diagnosis , Lung Diseases/physiopathologyABSTRACT
RESUMO O Modified Shuttle Walk Test (MSWT) é um teste de exercício potencialmente máximo que, associado à avaliação da força muscular respiratória (FMR), reflete a condição respiratória e a capacidade de exercício de escolares com fibrose cística (FC). O objetivo desta pesquisa foi investigar a relação entre FMR e distância percorrida (DP) no MSWT realizado por escolares com FC e comparar os dados obtidos com valores preditos na literatura. Trata-se de um estudo observacional transversal que incluiu escolares com FC. Realizou-se avaliação antropométrica, espirometria e FMR, utilizando as pressões inspiratória máxima (PImáx) e expiratória máxima (PEmáx) por meio da manovacuometria. Dois MSWT foram realizados, com intervalo de 30 minutos entre eles. Verificou-se a distribuição dos dados pelo teste de Shapiro-Wilk e aplicou-se teste t pareado para comparação entre valores das avaliações e predito, bem como para comparação entre gêneros. Aplicou-se teste de Pearson para correlação entre PImáx e PEmáx e DP no MSWT. Aceitou-se significância de 5%. Participaram 28 crianças (9,9±1,9 anos) destas, 57,14% apresentaram PImáx abaixo do predito (15 crianças) e 53,57% da PEmáx (16 crianças). A média da DP foi 730,4±266,1m, abaixo do predito na literatura. Não houve relação entre DP e FMR. Identificou-se correlação moderada entre valores de PImáx e PEmáx (r=0,58 e p=0,01). Não houve relação entre FMR e desempenho no MSWT nos escolares com FC estudados. A FMR, bem como o DP no MSWT, apresentou-se abaixo do predito na literatura.
RESUMEN El Modified Shuttle Walk Test (MSWT) es una prueba de ejercicio potencialmente máxima que, asociada a la evaluación de la fuerza muscular respiratoria (FMR), refleja la condición respiratoria y la capacidad de ejercicio de escolares con fibrosis quística (FQ). El objetivo de esta investigación fue investigar la relación entre FMR y distancia recorrida (DP) en el MSWT realizado por escolares con FQ y comparar los datos obtenidos con valores predichos en la literatura. Se trata de un estudio observacional transversal que incluyó a los escolares con FQ. Se realizó una evaluación antropométrica, espirometría y FMR, utilizando las presiones inspiratoria máxima (PImáx) y espiratoria máxima (PEmáx) por medio de la manovacuometría. Se realizaron dos MSWT, con un intervalo de 30 minutos entre ellos. Se verificó la distribución de los datos por la prueba de Shapiro-Wilk y se aplicó una prueba t pareada para la comparación entre los valores de las evaluaciones y el predicado, así como para la comparación entre los géneros. Se aplicó una prueba de Pearson para la correlación entre PImáx y PEmáx y DP en el MSWT. Se aceptó una significación del 5%. Participaron del estudio 28 niños (9,9±1,9 años); 57,14% presentaron PImáx por debajo del pronóstico (15 niños) y 53,57% por debajo del pronóstico para PEmáx (16 niños). El promedio de la DP fue 730,4±266,1m, por debajo del predicho en la literatura. No hubo relación entre DP y FMR. Se identificó una correlación moderada entre valores de PImáx y PEmáx (r=0,58 y p=0,01). No hubo relación entre FMR y desempeño en el MSWT en los escolares con FC. La FMR, así como la DP en el MSWT, se presentó abajo del predicho en la literatura.
ABSTRACT Modified Shuttle Walk Test (MSWT) is a potentially maximal exercise test that, together with the assessment of respiratory muscle strength (RMS), reflects the respiratory condition and exercise capacity of schoolchildren with cystic fibrosis (CF). This study aimed to investigate the relationship between the RMS and the performance in the MSWT by schoolchildren with CF and to compare the data obtained with the values predicted in the literature. This is a cross-sectional observational study that included schoolchildren with CF. Anthropometric evaluation, spirometry and RMS evaluation were performed, using the maximal inspiratory (MIP) and expiratory (MEP) pressures (Globalmed MVD300® manovacuometer) (ATS/ERS) (2002). Two MSWT were performed, with an interval of 30 minutes between them. The distribution of the data by the Shapiro-Wilk test was applied and paired t-test was used to compare the values of the evaluations with those predicted, as well as for comparison between genders. Pearson test was used for correlation between MIP and MEP and the performance in the MSWT. Significance of 5% was accepted. 28 children (9.9±1.9 years) participated; 57.14% showed MIP below the predicted (15 children) and 53.57% showed MEP below the predicted (16 children). The mean performance was 730.4±266.1m, which is lower than the values predicted in the literature. No relationship between performance and RMS was observed. A moderate correlation was observed between MIP and MEP values (r=0.58, p=0.01). No relationship between the RMS and the MSWT performance was observed in schoolchildren with CF of this study. The RMS and the performance in the MSWT were below the predicted in the literature.
Subject(s)
Humans , Male , Female , Child , Adolescent , Respiratory Muscles/physiopathology , Cystic Fibrosis/physiopathology , Walk Test/methods , Spirometry/methods , Anthropometry , Cross-Sectional Studies , Exercise Tolerance , Maximal Respiratory Pressures/methodsABSTRACT
Abstract Objectives: Volumetric capnography provides the standard CO2 elimination by the volume expired per respiratory cycle and is a measure to assess pulmonary involvement. Thus, the objective of this study was to evaluate the respiratory dynamics of healthy control subjects and those with cystic fibrosis in a submaximal exercise protocol for six minutes on the treadmill, using volumetric capnography parameters (slope 3 [Slp3], Slp3/tidal volume [Slp3/TV], and slope 2 [Slp2]). Methods: This was a cross-sectional study with 128 subjects (cystic fibrosis, 64 subjects; controls, 64 subjects]. Participants underwent volumetric capnography before, during, and after six minutes on the treadmill. Statistical analysis was performed using the Friedman, Mann-Whitney, and Kruskal-Wallis tests, considering age and sex. An alpha = 0.05 was considered. Results: Six minutes on the treadmill evaluation: in cystic fibrosis, volumetric capnography parameters were different before, during, and after six minutes on the treadmill; the same was observed for the controls, except for Slp2. Regarding age, an Slp3 difference was observed in cystic fibrosis patients regardless of age, at all moments, and in controls for age ≥ 12 years; a difference in Slp3/TV was observed in cystic fibrosis and controls, regardless of age; and an Slp2 difference in the cystic fibrosis, regardless of age. Regarding sex, Slp3 and Slp3/TV differences were observed in cystic fibrosis regardless of sex, and in controls in male participants; an Slp2 difference was observed in the cystic fibrosis and female participants. The analysis between groups (cystic fibrosis and controls) indicated that Slp3 and Slp3/TV has identified the CF, regardless of age and sex, while the Slp2 showed the CF considering age. Conclusions: Cystic fibrosis showed greater values of the parameters before, during, and after exercise, even when stratified by age and sex, which may indicate ventilation inhomogeneity in the peripheral pathways in the cystic fibrosis.
Resumo Objetivos: A capnografia volumétrica fornece o padrão de eliminação do CO2, pelo volume expirado por ciclo respiratório e avalia o comprometimento pulmonar. O objetivo do estudo foi avaliar a dinâmica respiratória de indivíduos controles saudáveis e em indivíduos com fibrose cística, em um protocolo de exercício submáximo por seis minutos em esteira, por parâmetros da capnografia volumétrica [slope 3(Slp3), Slp3/volume corrente (Slp3/TV) e slope 2(Slp2)]. Métodos: Estudo de corte transversal com 128 indivíduos [(fibrose cística) 64 indivíduos; (controles) 64 indivíduos]. Os participantes realizaram capnografia volumétrica antes, durante e após seis minutos em esteira. Análise estatística realizada pelos testes de Friedman, Mann-Whitney e Kruskal-Wallis, considerado a idade e o sexo. Alpha = 0,05. Resultados: Avaliação de seis minutos em esteira: na fibrose cística, os parâmetros da capnografia volumétrica foram diferentes antes, durante e após seis minutos em esteira, o mesmo ocorreu nos controles, exceto para o Slp2. Considerando a idade: (Slp3) diferença na FC, independentemente da idade, em todos os momentos e nos controles apenas para ≥ 12 anos; (Slp3/TV) diferença para fibrose cística e controles independentemente da idade; (Slp2) diferença apenas para o grupo fibrose cística, independentemente da idade. Considerando o sexo: (Slp3 e Slp3/TV) diferença para fibrose cística, independentemente do sexo, e controles apenas no sexo masculino; (Slp2) diferença para fibrose cística e sexo feminino. Análise entre grupos (fibrose cística versus controles): Slp3 e Slp3/TV identificou a fibrose cística, independentemente da idade e sexo, enquanto o Slp2 evidenciou a fibrose cística considerando a idade. Conclusão: A fibrose cística apresentou maiores valores dos parâmetros antes, durante e após exercício, inclusive quando se considerou idade e sexo, podendo indicar não homogeneidade da distribuição da ventilação nas vias periféricas.
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Young Adult , Tidal Volume/physiology , Forced Expiratory Volume/physiology , Pulmonary Ventilation/physiology , Capnography/methods , Cystic Fibrosis/physiopathology , Walk Test/methods , Spirometry , Case-Control Studies , Cross-Sectional StudiesABSTRACT
RESUMO Objetivo: Avaliar o perfil nutricional da população atendida em centro de referência em fibrose cística. Métodos: Estudo transversal incluindo pacientes com fibrose cística de um centro pediátrico de referência de São Paulo, em 2014. Todos os sujeitos que concordaram em participar do estudo foram incluídos. Foi aplicado um questionário sobre hábitos alimentares (recordatório de 24 horas) e características socioeconômicas. Dados antropométricos (comparados com referencial da Organização Mundial da Saúde de 2006 e 2007) e função pulmonar foram coletados do prontuário. Os integrantes da pesquisa foram estratificados em faixas etárias para análise estatística. Resultados: Dos 101 pacientes incluídos no estudo, 59,4% eram masculinos, sendo a maioria caucasiana (86,4%), com mediana de idade de 10 anos. A maioria dos pacientes (n=77, 75%) foi classificada como eutrófica, mas valores menores de escore Z de índice de massa corpórea (IMC) foram observados em escolares e adolescentes. A proporção de pacientes com peso abaixo do esperado foi de 10% (n=2) na faixa etária pré-escolar e de 35% (n=6) em escolares. O consumo alimentar mostrou-se adequado, e somente dois suplementos (triglicérides de cadeia média e suplemento em pó completo) tiveram utilização ligada à adequação da ingesta de macronutrientes. Características socioeconômicas não apresentaram associação com o estado nutricional ou com o consumo nutricional. Valores de função pulmonar não apontaram diferença significante em adolescentes nem em indivíduos com pior estado nutricional no grupo estudado. Conclusões: A maioria dos pacientes apresentou estado nutricional e consumo alimentar adequados, indicando bom manejo das ações nutricionais. Novos estudos com foco em pré-escolares devem ser realizados para avaliar se é possível reduzir o risco nutricional de pacientes com fibrose cística em idades posteriores.
ABSTRACT Objective: To evaluate the nutritional profile of the population assisted at a reference center for cystic fibrosis treatment. Methods: Cross-sectional study including patients with cystic fibrosis assisted at a pediatric reference center in São Paulo, Brazil, in 2014. All patients attending regular visits who agreed to participate in the study were included. A questionnaire on dietary habits (24-hour diet recall) and socioeconomic characteristics was applied. Anthropometric data (compared with the reference from the World Health Organization, 2006 and 2007) and pulmonary function data were collected from medical records. Patients were stratified into age groups for statistical analysis. Results: A total of 101 patients were included in the study (59.4% male, 86.4% Caucasian), with median age of 10 years old. Most patients (n=77; 75%) were classified as eutrophic, but lower values of body mass index (BMI) Z scores were observed in schoolchildren and adolescents. The proportion of underweight patients was 10% (n=2) among preschoolers and 35% (n=6) of the school age group. Dietary intake was adequate, and the use of only two supplements (medium chain triglycerides and complete powdered supplement) was associated with adequacy of macronutrient intake. The socioeconomic characteristics did not show any statistical association with the nutritional state or nutritional consumption. Lung function was not significantly different between neither adolescents nor individuals with worse nutritional status in this sample. Conclusions: Most of the patients presented adequate nutritional status and adequate consumption of calories and macronutrients, indicating appropriate nutritional management. New studies focusing on preschool children should be performed to assess if it is possible to reduce the nutritional risk of patients with cystic fibrosis at older ages.
Subject(s)
Humans , Male , Female , Child , Anthropometry/methods , Nutritional Status , Feeding Behavior , Respiratory Function Tests/methods , Respiratory Function Tests/statistics & numerical data , Brazil/epidemiology , Energy Intake , Body Mass Index , Food , Cross-Sectional Studies , Surveys and Questionnaires , Age Distribution , Cystic Fibrosis/diagnosis , Cystic Fibrosis/physiopathologyABSTRACT
Resumen: Introducción: La fibrosis quística (FQ) es una enfermedad multisistémica hereditaria y progresiva. Una mejor capacidad física puede retardar la progresión de la enfermedad, mejorando así el pronós tico y la supervivencia. El objetivo de esta investigación fue evaluar la capacidad física de los niños admitidos en el programa nacional de FQ de la Región Metropolitana, Chile. Pacientes y Método: Se utilizó un diseño de estudio transversal multicéntrico. Los criterios de inclusión fueron: niños de 6 a 12 años de edad, incluidos en el Programa Nacional de FQ, madurez sexual Tanner I, ausencia de exacerbaciones respiratorias en los últimos 30 días y ausencia de enfermedades musculoesqueléticas. La capacidad aeróbica máxima fue evaluada a través del consumo pico de oxígeno (VO2pico) y se determinó con un protocolo incremental en un cicloergómetro magnético conectado a un ergoespirómetro en el que paralelamente se analizaron los gases respiratorios: valores de consumo de oxí geno y producción de dióxido de carbono cada 30 segundos, umbral anaeróbico y carga máxima de trabajo. Además, se evaluaron los valores de capacidad vital forzada (CVF), volumen espiratorio al primer segundo (VEFj), relación VEFj/CVF y los flujos espiratorios forzados entre el 25 y 75% de la capacidad vital. Durante la prueba se registró: saturación arterial de oxígeno, frecuencia respiratoria, frecuencia cardíaca, presión arterial, volumen corriente y se consultó la percepción de fatiga de extre midades inferiores y disnea a través de la escala de Borg modificada. La duración aproximada del test fue alrededor de 10 minutos. Resultados: Se revisaron los registros clínicos de 43 niños, recogidos en seis centros de salud. Veintinueve niños cumplieron los criterios de inclusión siendo 23 reclutados. Dos niños no pudieron participar, reduciendo el grupo final de sujetos a 21 (13:8 varones:mujeres). La edad media fue de 8,8 ± 2 años; el peso fue de 30,5 ± 10,9 kg; la talla fue de 1,32 ± 0,11 m y el índice de masa corporal fue de 17,1 ± 3,5 (z-score 0,01 ± 1,34). Más de la mitad (61%) de los niños estaba eutrófico. El VO2pico obtenido fue de 43,7 ± 6,5 ml/min/kg (106,7 ± 19,8% de los valores teóricos de referencia). Sólo el 10% de los niños tenían valores inferiores a los valores teóricos esperados para población normal, ajustados por sexo y edad. No se encontraron correlaciones entre el VO2pico y las variables antropométricas y de función pulmonar. Conclusión: La mayoría de los niños evaluados (90%) tenían capacidad física similar a los valores teóricos de referencia para niños sanos ajustados por sexo y edad.
Abstract: Introduction: Cystic fibrosis (CF) is an inherited, progressive, multisystem disease. Better physical capacity may slow disease progression, thus improving prognosis and survival. The objective of this research was to evaluate the physical capacity of children admitted to the National CF Pro gram of the Metropolitan Region, Chile. Patients and Method: A multicenter, cross-sectional stu dy design was used. The inclusion criteria were children aged 6 to 12 years enrolled in the National CF Program; Tanner sexual maturity stage I, no respiratory exacerbations in the last 30 days, and no musculoskeletal pathologies. The maximum aerobic capacity was assessed through the peak oxygen uptake (VO2peak) and determined with an incremental protocol in a magnetic cycle ergometer connected to an ergo-spirometer with which, at the same time, respiratory gases, oxygen consumption and carbon dioxide production values every 30 seconds, anaerobic threshold, and maximum workload were analyzed. The values of forced vital capacity (FVC), forced expiratory volume in 1 second (FEVj), FEVj/FVC ratio, and forced expiratory flows between 25% and 75% of vital capacity were assessed through ergo-spirometry. At the beginning of the ergo-spirometry, arterial oxygen saturation, respiratory rate, heart rate, blood pressure, tidal volume and the per ception of lower extremity fatigue and dyspnea were recorded using the modified Borg scale. The test lasted approximately 10 minutes. Results: The clinical records of 43 children collected from six health centers were reviewed. Out of these, 29 children met inclusion criteria, and 23 were re cruited. Two children were unable to participate, reducing the final subject group to 21 (13 males, 8 females). The mean age was 8.8 ± 2 years; weight 30.5 ± 10.9 kg; height 1.32 ± 0.11 m; and body mass index 17.1 ± 3.5 (z-score 0.01 ± 1.34). More than half of the children (61%) had normal weight. The obtained VO2peak was 43.7 ± 6.5 ml/min/kg (106.7 ± 19.8% of the predictive values). Only 10% of the children had values lower than those predicted by sex and age. No correlations were found between VO2peak and anthropometric and pulmonary function variables. Conclu sion: Most of the evaluated children (90%) had physical capacity similar to healthy subjects by sex and age.
Subject(s)
Humans , Male , Female , Child , Physical Fitness/physiology , Cystic Fibrosis/physiopathology , Oxygen Consumption , Spirometry , Chile , Vital Capacity , Forced Expiratory Volume , Cross-Sectional Studies , Exercise TestABSTRACT
RESUMEN: El odontólogo como profesional integral del área de la salud, debe tener conocimiento acerca de distintas manifestaciones bioquímicas que pueden tener repercusión en la cavidad oral. El objetivo del trabajo fue determinar las manifestaciones bioquímicas y alteraciones en biomarcadores salivales en la cavidad oral producto de la fibrosis quística o del consumo crónico de medicamentos para el tratamiento de la FQ. Se seleccionó un total de cinco personas con fibrosis quística y cuatro personas sanas, pertenecientes a la ciudad de Concepción en la Octava Región de Chile. Se midió pH salival, capacidad buffer, concentración de proteínas totales, tasa de flujo salival estimulado y se determinó presencia de ciertas enzimas salivales en pacientes que padecen la enfermedad. Se pudo evidenciar que el pH salival en sujetos con fibrosis quística tiende a ser mayor a los valores de referencia, la tasa de flujo salival es mucho menor al igual que la capacidad buffer, la concentración de proteínas totales en saliva se encuentra igual a los valores de referencia y se determinó la presencia biomarcadores salivales a través de la técnica de electroforesis. La fibrosis quística afecta de muchas formas a las personas que la padecen, genera cambios a nivel de los biomarcadores salivales como también en la cavidad oral, por lo que el odontólogo debe estar capacitado para identificar estos cambios y poder tratar de la mejor manera a todo tipo de paciente.
ABSTRACT: The dentist as an integral health professional must have knowledge of various biochemical manifestations that may have repercussions on the oral cavity. The objective of the study was to determine the biochemical manifestations and salivary biomarker alterations in the oral cavity resulting from cystic fibrosis or chronic consumption of drugs for the treatment of CF. We selected a total of five people with cystic fibrosis and four healthy people, from the city of Concepcion in the eighth region of Chile. Salivary pH, buffer capacity, total protein concentration, stimulated salivary flow rate and the presence of certain salivary enzymes were measured in patients suffering from the disease. It was observed that the salivary pH in subjects with cystic fibrosis tends to be higher than the reference values, the salivary flow rate and buffer capacity are less than normal, the total protein concentration in saliva is equal to the reference values and the presence of salivary biomarkers was determined through the electrophoresis technique. Cystic fibrosis affects those who suffer the disease in many ways, it generates changes at the salivary biomarker level, as well as in the oral cavity. The dentist must therefore, be able to identify these changes in order to treat them in the best possible approach for all types of patients.
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Interleukin-8/metabolism , Matrix Metalloproteinase 9/metabolism , Cystic Fibrosis/physiopathology , Cystic Fibrosis/metabolism , Vascular Endothelial Growth Factor A/metabolism , Epidermal Growth Factor/metabolism , Chemokine CXCL10/metabolism , Saliva/chemistry , Biomarkers/metabolism , Proteins , Chile , Electrophoresis , Hydrogen-Ion Concentration , Informed ConsentABSTRACT
RESUMO Objetivo: Avaliar a associação entre estado nutricional, função pulmonar e morbidade em coorte de 36 meses de crianças e adolescentes com fibrose cística (FC). Métodos: Coorte prospectiva de 36 meses, com crianças e adolescentes com FC e idade entre 1 e 15 anos. No tempo inicial, o estado nutricional foi determinado a partir dos indicadores: peso-para-estatura e índice de massa corporal-para-idade, para crianças <2 anos e ≥2 anos, respectivamente, e classificado em: falência nutricional, risco nutricional e estado nutricional aceitável; também foi determinado por meio do percentil 50°, de acordo com a curva de crescimento da World Health Organization (WHO). A função pulmonar foi avaliada pelo volume expiratório forçado no primeiro segundo (VEF1). A morbidade foi determinada pela presença de infecção e hospitalização por exacerbação pulmonar. Foi calculado risco relativo (RR) e intervalo de confiança (IC95%), considerando significante p<0,05. Resultados: Foram avaliadas 38 crianças e adolescentes (mediana de idade 3,8 anos). Os pacientes classificados em falência nutricional no início do estudo mostraram um RR de 5,00 (IC95% 1,49; 16,76) para o comprometimento da função pulmonar após 36 meses. Aqueles classificados abaixo do percentil 50° apresentaram RR de 4,61 (IC95% 0,89; 23,81) para o desfecho. O estado nutricional não foi fator de risco para morbidade. Conclusões: O déficit nutricional esteve associado ao comprometimento da função pulmonar, mas não com a morbidade em crianças e adolescentes com FC.
ABSTRACT Objective: To evaluate the association between nutritional status, lung function and morbidity in a 36-month cohort in children and adolescents with cystic fibrosis. Methods: Prospective cohort of children and adolescents with cystic fibrosis aged 1-15 years. At the baseline, the nutritional status was determined by weight-for-height and body mass index-for-age for children <2 years and ≥2 years, respectively, and classified as: nutritional failure, nutritional risk and acceptable; and by the 50th percentile, according to the World Health Organization (WHO) growth charts. Lung function was assessed by forced expiratory volume in one second (FEV1). Morbidity was determined by the presence of infection and hospitalization by pulmonary exacerbation. Risk ratio and 95% confidence interval (95%CI) were calculated, being significant when p<0.05. Results: We evaluated 38 children and adolescents (median age 3.8 years). Patients that were classified as having nutritional failure at baseline had a RR of 5.00 (95%CI 1.49; 16.76) to present impaired lung function after 36 months. Those classified bellow the 50th percentile had a RR of 4.61 (95%CI 0.89; 23.81) to present the same outcome. Nutritional status was not a risk factor for morbidity in this cohort. Conclusions: Nutritional deficit was associated with impaired lung function, but not with morbidity in children and adolescents with cystic fibrosis.
Subject(s)
Humans , Male , Female , Infant, Newborn , Child, Preschool , Child , Adolescent , Nutritional Status , Cystic Fibrosis/complications , Cystic Fibrosis/physiopathology , Lung/physiopathology , Time Factors , Forced Expiratory Volume , Cohort StudiesABSTRACT
ABSTRACT BACKGROUND: Chronic lung infections, inflammation and depletion of nutritional status are considered to be prognostic indicators of morbidity in patients with cystic fibrosis. The aim of this study was to investigate the association between inflammatory markers and lung function, nutritional status and morbidity among children/adolescents with cystic fibrosis. DESIGN AND SETTINGS: Prospective three-year longitudinal study conducted in an outpatient clinic in southern Brazil. METHODS: Children/adolescents aged 1-15 years with cystic fibrosis were enrolled. Nutritional status was determined from weight-to-length and body mass index-to-age z-scores and was classified as acceptable, at risk or nutritional failure. Tumor necrosis factor-α, interleukin-1β, myeloperoxidase, C-reactive protein and C-reactive protein/albumin ratio were analyzed. Lung function was evaluated based on the forced expiratory volume in the first second and morbidity according to the number of hospitalizations for pulmonary exacerbation and infections by Pseudomonas aeruginosa. Lung function, nutritional status and morbidity were the outcomes. Odds ratios and 95% confidence intervals were to evaluate the effect of baseline inflammatory markers on the clinical outcomes after three years of follow-up and p-values < 0.05 were considered significant. RESULTS: We evaluated 38 children/adolescents with cystic fibrosis: 55% female; median age (with interquartile range), 3.75 years (2.71-7.00). Children/adolescents with high C-reactive protein/albumin ratio at baseline had odds of 18 (P = 0.018) of presenting forced expiratory volume in the first second ≤ 70% after three years. The other inflammatory markers were not associated with the outcomes. CONCLUSION: C-reactive protein/albumin ratio was associated with forced expiratory volume in the first second ≤ 70% after three years.
Subject(s)
Humans , Male , Female , Child , Adolescent , C-Reactive Protein/analysis , Serum Albumin/analysis , Tumor Necrosis Factor-alpha/blood , Peroxidase/blood , Inflammation Mediators/blood , Cystic Fibrosis/blood , Interleukin-1beta/blood , Respiratory Function Tests , Biomarkers/blood , Nutritional Status , Prospective Studies , Longitudinal Studies , Cystic Fibrosis/physiopathologyABSTRACT
OBJECTIVES: The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity. METHODS: This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05. RESULTS: A total of 31 patients (11 females; 16.4±2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender. CONCLUSIONS: The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.
Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Quality of Life/psychology , Cystic Fibrosis/psychology , Respiratory Function Tests , Spirometry , Severity of Illness Index , Body Mass Index , Forced Expiratory Volume/physiology , Cross-Sectional Studies , Surveys and Questionnaires , Cystic Fibrosis/physiopathologyABSTRACT
Abstract Objective We evaluated the association between vitamin D levels and nutritional status, pulmonary function and pulmonary exacerbations in children and adolescents with cystic fibrosis. Methods 25-hydroxyvitamin D (25(OH)D) levels of 37 children and adolescents were retrospectively evaluated. Pulmonary function, body mass index, height for age, and pulmonary exacerbations episodes were associated with vitamin D levels divided into two groups: sufficient (≥30ng/mL) and hypovitaminosis (<30ng/mL). Results Hypovitaminosis D (25(OH)D <30ng/mL) was observed in 54% of subjects. The mean level of 25(OH)D was 30.53±12.14ng/mL. Pulmonary function and nutritional status were not associated with vitamin D levels. Pulmonary exacerbations over a 2-year period (p=0.007) and the period from measurement up to the end of the follow-up period (p=0.002) were significantly associated with vitamin D levels. Conclusion Hypovitaminosis D was associated with higher rates of pulmonary exacerbations in this sample of children and adolescents with cystic fibrosis. Hypovitaminosis D should be further studied as a marker of disease severity in cystic fibrosis. Further prospective and randomized studies are necessary to investigate causality of this association.
RESUMO Objetivo Avaliar a associação entre níveis de vitamina D e estado nutricional, função pulmonar e exacerbações pulmonares em crianças e adolescentes com fibrose cística. Métodos Os níveis de 25-hidroxivitamina D (25(OH)D) de 37 crianças e adolescentes foram avaliados retrospectivamente. Função pulmonar, índice de massa corporal, altura para a idade, e episódios de exacerbações pulmonares foram associados aos níveis de vitamina D divididos em dois grupos: suficiente (≥30ng/mL) e hipovitaminose (<30ng/mL). Resultados Hipovitaminose D (25(OH)D <30ng/mL) foi observada em 54% dos pacientes. O nível médio de 25(OH)D foi de 30,53±12,14ng/mL. A função pulmonar e o estado nutricional não foram associados aos níveis de vitamina D. As exacerbações pulmonares durante um período de 2 anos (p = 0,007) e do período de medição até o final do período de seguimento (p=0,002) foram significativamente associadas aos níveis de vitamina D. Conclusão A hipovitaminose D esteve associada a maiores taxas de exacerbações pulmonares nesta amostra de crianças e adolescentes com fibrose cística. A hipovitaminose D deve ser mais estudada como marcador da gravidade da doença na fibrose cística. Outros estudos prospectivos e randomizados são necessários para investigar a relação causal desta associação.