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.

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.

OBJETIVO: Identificar os fatores de risco de descompensação distal pós-operatória (DDP) e definir estratégia cirúrgica de segurança em curvaturas de Lenke 1A, tratadas com parafusos pediculares. MÉTODO: Estudo radiológico retrospectivo de 63 pacientes com escoliose Lenke 1A, com acompanhamento mínimo de um ano. Os parâmetros avaliados foram idade, sexo, graus do ângulo de Cobb, sinal de Risser, relação da vértebra distal instrumentada (VDI) com a vértebra distal da curvatura (VD), com a vértebra estável (VE) e com a vértebra cuja distância da linha central vertical até o sacro (LVS) era superior a 10 mm de "distância vertebral" (DV). RESULTADOS: 8 casos (12,7%) desenvolveram DDP. O sinal de Risser foi 0 em 2 pacientes (25%) e 1 em 2 pacientes (25%). Relação VDI/VD: 4 pacientes (50%) mesmo nível (VD +0), 4 pacientes (50%) nível caudal (VD +1); relação VDI/VE: 5 pacientes (62,5%) 2 níveis cefálicos (VE -2), 3 pacientes (37,5%) 1 nível cefálico (VE -1); relação VDI/DV: 5 pacientes (62,5%) um nível cefálico DV (-1), 3 pacientes mesmo nível (DV +0). CONCLUSÕES: Risco de descompensação distal pós-operatória:VDI mesmo nível VD (VD +0), 2 níveis cefálicos VE (VE -2), 1 nível cefálico DV (DV -1). Estratégia cirúrgica nas curvaturas Lenke 1A: VDI: 1/2 níveis caudal até VD (VD +1/+2), um nível cefálico até VE (VE -1), mesmo nível DV (DV +0).

OBJECTIVE: To identify risk factors for postoperative distal decompensation (PDD) and safe surgical strategy in curvatures Lenke type 1A, treated with pedicle screws. METHOD: Retrospective radiographic study of 63 patients with scoliosis Lenke 1A, with follow-up of at least one year. The parameters evaluated were age, sex, degrees of Cobb's angle, Risser sign, relationship of distal instrumented vertebra (DIV) to the distal vertebra (DV) of the curvature, to the stable vertebra (SV) and to the vertebra which distance to the central vertical line to the sacrum (VLS) was greater than 10 mm of "vertebral distance" (VD). RESULTS: 8 cases (12.7%) developed DDP. The Risser sign was 0 in 2 patients (25%) and 1 in 2 patients (25%). DIV/DV relationship: 4 patients (50%) same level (DV +0), 4 patients (50%) caudal level (DV +1); DIV/SV ratio: 5 patients (62.5%) 2 cephalic levels (VD -2), 3 patients (37.5%) 1 cephalic level (SV -1); DIV/VD relationship: 5 patients (62.5%) 1 cephalic level (VD -1), 3 patients the same level (VD +0). CONCLUSIONS: Risk of postoperative distal decompensation: DIV same level DV (DV +0), 2 cephalic levels SV (SV -2), 1 cephalic level VD (VD -1). Surgical strategy in Lenke 1A curvatures: DIV: 1-2 levels caudal to DV (DV +1/+2), 1 cephalic level to SV (SV -1), the same level VD (VD +0).

OBJETIVO: Identificar los factores de riesgo de descompensación distal postoperatoria (D.D.P.) y definir una estrategia quirúrgica segura en curvas tipo Lenke 1A tratadas con tornillos pediculares. MÉTODO: Estudio radiológico retrospectivo de 63 pacientes con escoliosis Lenke 1A, con un seguimiento mínimo de un año. Se evaluó, edad, sexo, grados Cobb, signo de Risser, relación de la vértebra distal instrumentada (V.D.I.) con la vértebra distal de la curva (V.D.), vértebra estable (V.E.) y con la vértebra, cuya distancia a la línea central vertical al sacro (L.V.S.) era superior a 10 mm "distancia vertebral" (D.V.). RESULTADOS: 8 casos (12,7%) desarrollaron D.D.P. El signo de Risser fue 0 en 2 pacientes (25%) y I en 2 pacientes (25%). Relación de V.D.I. con V.D.: 4 pacientes (50%) mismo nivel (V.D. +0), 4 pacientes (50%) un nivel caudal (V.D. (+1); relación V.D.I. con V.E.: 5 pacientes (62,5%) 2 niveles cefálicos (V.E -2), 3 pacientes (37,5%) 1 nivel cefálico (V. E.-1); relación V.D.I. con D.V.: 5 pacientes (62,5%) un nivel cefálico D.V. (-1), 3 pacientes mismo nivel (D.V.+ 0). CONCLUSIONES: Riesgo de descompensación distal postoperatoria: V.D.I. mismo nivel V.D. (V.D. + 0), 2 niveles cefálicos V.E. (V.E.-2), 1 nivel cefálico D.V. (D.V. -1). Estrategia quirúrgica curvas Lenke 1A: V.D.I: 1/2 niveles caudales a V.D. (V.D. +1/+2), un nivel cefálico a V.E. (V.E -1), mismo nivel D.V. (D.V. +0).

RESUMEN La frecuencia de estenosis valvular aórtica en pacientes con falla renal es mayor que en la población general, lo que sugiere la presencia de mecanismos patogénicos acelerados o diferentes. En 135 pacientes con falla renal en tratamiento dialítico estudiamos las prevalencias de fibrosis y estenosis valvular aórtica y los factores presuntamente asociados con ella. Los datos clínicos y de laboratorio se obtuvieron de los registros de diálisis, en todos se hizo un ecocardiograma Doppler y se calculó el gradiente transvalvular aórtico y el índice de masa ventricular izquierda (IMVI). En 116 pacientes dosificamos colesterol total (CT), triglicéridos, HDL-colesterol, apolipoproteína A1 y B y lipoproteína (a); se calculó el LDL-colesterol, relación CT/HDL-colesterol, y el índice apo B/apo A1. La frecuencia de fibrosis (72%) y estenosis (6%) valvular aórtica fue más del doble que la conocida en la población general. El análisis multivariado mostró una asociación independiente de la patología valvular aórtica con la edad (p<0,0005), diabetes mellitus (p=0,013) y tiempo en diálisis (p=0,013). Concentraciones de CT mayores de 200 mg/dL se asociaron con patología valvular aórtica (p<0,004). La valvulopatía no mostró asociación con tabaquismo, hipertensión arterial o alteraciones fosfo-cálcicas. Comparadas con las normales, las válvulas con fibrosis tuvieron mayor valor de velocidad máxima (p<0,01), gradiente transvalvular (p=0,005) e IMVI (p=0,005). La alta frecuencia de enfermedad valvular aórtica en diálisis sugiere que los pacientes con falla renal presentan factores que favorecen su aparición; los cuatro factores independientes que identificamos: edad, diabetes mellitus, tiempo en diálisis e hipercolesterolemia se asocian también tradicionalmente con la aterosclerosis. Los aumentos de gradiente transvalvular y de IMVI en la fibrosis valvular aórtica señalan la presencia de alteraciones funcionales y morfológicas en etapas previas a la estenosis aórtica.

SUMMARY The prevalence of aortic valve stenosis in renal failure patients is higher than the observed in general population, suggesting accelerated or different pathogenic mechanisms. We studied the prevalence of aortic valve fibrosis and stenosis in 135 renal failure patients on maintenance dialysis, and the possible associated factors. Clinical and laboratory data were collected from dialysis registers; a cardiac echo-Doppler study was performed in all patients and the aortic transvalvular gradient and left ventricle mass index were calculated (LVMI). We measured total cholesterol (TC), triglycerides, HDL-cholesterol, apolipoprotein A1 and B and lipoprotein (a) in 116 patients; LDL-cholesterol, TC/HDL-cholesterol, and apo B/apo A1 ratio were calculated. The frequencies of aortic valve fibrosis (72%) and stenosis (6%) were more than double than in general population. An independent association of aortic valve disease with age (p<0,0005), diabetes mellitus (p=0,013), and time on dialysis (vintage) (p=0,013), was demonstrated by multivariate analysis. TC concentrations higher than 200 mg/dL were associated with aortic valve disease (p<0,004). There was no association with smoking, hypertension or phospho-calcium alterations. Compared to normal, fibrotic valves had higher values of maximal velocity (p<0,01), transvalvular gradient (p=0,005) and LVMI (p=0,005). The high prevalence of aortic valve disease in dialysis patients suggests that renal failure is a predisposing condition; the four independent factors that we recognized: age, diabetes mellitus, time on dialysis and hypercholesterolemia, are also traditionally associated with atherosclerosis. Higher transvalvular gradients and LVMI values suggest the presence of functional and morphologic alterations prior to aortic stenosis.