Características clínicas, nutricionais e perfil do consumo alimentar de pacientes pediátricos com osteogenesis imperfecta

Abstract

RESULTADOS: Participaram do estudo 63 indivíduos (42,9% OI tipo I; 17,5 OI tipo III; 39,7 OI tipo IV). As características clínicas dos indivíduos estavam de acordo com a variabilidade fenotípica da doença. Todos os indivíduos com OI tipo III possuiam baixa estatura grave. Em relação estado nutricional, a maioria dos indivíduos foram classificados como eutróficos, entretanto somando sobrepeso e obesidade foi observado 37,0%, 44,6% e 32,0% para OI tipo I, III e IV, respectivamente. Os resultados das avaliações das dobras cutâneas mostraram-se concordantes à classificação do estado nutricional dos indivíduos, pois pacientes com dobras cutâneas classificadas acima do percentil 85 apresentaram estado nutricional de sobrepeso e obesidade. A gordura corporal calculada através do DEXA apresentou forte correlação (r=0, 803) com a gordura corporal calculada pelo somatório das dobras cutâneas. Em relação ao consumo alimentar a média do percentual de adequação de calorias apresentou diferença significativa entre os dois métodos (OMS ou Kcal/cm) (p=0, 002). Consumo de energia acima de 110% foi observado em 45,6% e 40,4% dos indivíduos para ambos os métodos. A OI tipo III apresentou uma média do percentual de adequação de calorias mais elevado que a OI tipo I e IV em ambos os métodos. Para a classificação da adequação do consumo alimentar de macronutrientes, 12,7% dos indivíduos apresentaram consumo abaixo do ponto de corte mínimo estabelecido para carboidrato, enquanto que 23,8% e 30,8% dos indivíduos apresentaram consumo alimentar acima do ponto de corte máximo para proteína e lipídio, respectivamente. Observamos uma associação entre o diagnóstico nutricional e os pontos de corte de consumo alimentar estabelecidos. A classificação consumo alimentar de cálcio abaixo do ponto de corte mínimo foi observado em 76,2% dos indivíduos, sendo 79,5% a média do percentual de adequação do consumo de cálcio, estando abaixo do ponto de corte mínimo. A média do consumo de cálcio ingerido foi de 770mg/dia. Foi observada uma correlação inversa (r= -0 527) entre a idade e a adequação no consumo de cálcio. CONCLUSÃO: Este estudo demonstra que a OI apresentam uma variabilidade clínica grande. A baixa estatura é uma característica marcante na OI, principalmente, em indivíduos com tipo III. Os indivíduos, em sua maioria, foram classificados como eutróficos, porém foi observada incidência de sobrepeso e obesidade nos pacientes. As dobras cutâneas mostraram- se concordantes com o diagnóstico nutricional dos indivíduos. O percentual de gordura corporal calculada pelo somatório das dobras cutâneas apresentou forte correlação com a percentual de gordura corporal calculado pelo DEXA. Em relação, ao consumo alimentar, indivíduos classificados com OI tipo III, apresentaram maior consumo de energia, do que os indivíduos com OI tipos I e IV. Para o consumo de macronutrientes, embora a maioria dos indivíduos apresentarem consumo adequado, alguns indivíduos apresentaram baixo consumo de carboidrato e alto consumo de proteína e lipídio. O baixo consumo de cálcio apresentou- se 76,2% da população estando abaixo do ponto de corte mínimo. Foi observada também uma correlação inversa entre idade e adequação no consumo de cálcio. Este estudo manifesta a necessidade de uma intervenção nutricional direcionada a estes pacientes uma vez que a adequação do estado nutricional e do consumo alimentar são fatores importantes para a saúde óssea.

BACKGROUND: Osteogenesis Imperfecta (OI) is an inherited disease that results in decreased bone mass and fragility leading to an increased susceptibility to fractures. OBJECTIVE: The aim of this study was to evaluate clinical, anthropometric, nutritional status and describe the profile of food intake in pediatric patients with OI. METHODS: We conducted a cross-sectional study of pediatric patients form 0-19 years of age of both gender attending the OI outpatient clinic of Hospital de Clínicas de Porto Alegre. All subjects underwent clinical evaluation, anthropometric measurements and nutritional assessment. Percentage of body fat was calculated using the sum of skinfolds (triceps and subscapular) and measured by Dual Energy X-Ray Absoptiometry (DEXA). Both measurements were correlated. Food intake was calculated using the food diary for three days and for calculation of calories two methods were used: reference table by age by WHO and the formula Kcal / cm. The values used to ensure adequate intake of macronutrients (carbohydrate, protein and lipid) were according to FAO/ WHO and the food intake of micronutrients (calcium) according to DRI, considering the Adequate Intake (AI) for age. It was established as suitable for food intake of calories and nutrients intake between the cutoffs of 90 to 110%. For data analysis SPSS V.18 was used. The tests for statistical analysis were One Way ANOVA, t-student, Kappa, Pearson correlation tests. We considered significant values p <0.05. RESULTS: The study enrolled 63 subjects (42.9% OI type I, 17.5% OI type III, 39.7% OI type IV). The clinical characteristics of individuals were in agreement with the phenotypic variability of the disease. All individuals with OI type III had been classified with severe short stature. The nutritional status of most individuals were classified as normal, however overweight or obesity were observed respectively in 37.0%, 44.6% and 32.0% for OI type I, III and IV, respectively. The results of evaluations of skinfolds were shown to be consistent with the classification of nutritional status of individuals, because patients with skinfolds above the 85th centile showed nutritional status of overweight and obesity. Body fat estimated by DEXA showed a strong correlation (r = 0.803) with body fat calculated from the sum of skinfolds. Regarding the profile of food consumption the average proportion of adequate calories showed significant difference between the two methods (WHO or Kcal/cm) (p = 0.002). Food consumption in excess of 110% was observed in 45.6% and 40.4% of subjects for both methods. The OI type III showed an average proportion of adequate calories higher than OI type I and IV in both methods. To classify the adequacy of dietary intake of macronutrients 12.7% of subjects had intake below the threshold cutoff for carbohydrate, whereas 23.8 and 30.8% of subjects had food intake above the cutoff limit for protein and lipid. We observed an association between nutritional status and the cutoff of food consumption set. Classification dietary intake of calcium below the minimum cutoff point was observed in 76.2% of subjects and the average intake of calcium was 770mg/dia. We observed an inverse correlation (r = -0.527) between age and calcium intake. CONCLUSION: This study demonstrates that the OI have a great clinical variability. Short stature is a hallmark in OI, especially in individuals with type III. Individuals, in most cases, were classified as normal, but it was found that the incidence of overweight and obesity in patients. The skinfolds were shown to be consistent with the diagnosis of nutritional subjects. skinfolds showed a strong correlation with body fat percentage calculated by DEXA. In relation to the food intake, individuals classified as OI type III, had higher energy consumption than individuals with OI type I and IV. For the consumption of macronutrients, although most people develop adequate intake, some individuals had low carbohydrate intake and high intake of protein and lipid. The low intake of calcium was 76.2% of the population being below the minimum cutoff. There was also an inverse correlation between age and fitness for consumption of calcium. This study shows the need for a nutritional intervention targeted to these patients since their nutritional status and dietary intake are important factors for bone health.