Maria Efigênia de Queiroz Leite

Percepção materna e autopercepção do estado nutricional de crianças e adolescentes de escolas privadas

OBJECTIVE: To describe and compare maternal perception and the self-perception of children/adolescents of their nutritional status, identifying factors associated with incorrect perceptions. METHODS: Cross-sectional study carried out in Salvador, BA, Brazil with 1,741 students aged 6 to 19 years, classified according to body mass index (BMI) percentiles as underweight (BMI p95). Students and their mothers answered questions on perception of weight, patterns of physical exercise and dieting. By means of multivariate analysis, factors associated with incorrect maternal and self- perceptions were analyzed. RESULTS: Self-perceptions were correct in 64.7% of cases and 75.3% of maternal perceptions were correct. The principal factor associated with incorrect self-perception was age between 6 and 9 years (OR = 1.59; 95%CI 1.15-2.20). Among girls, being overweight and practicing physical exercise were characteristics associated with better perception. For boys, the presence of overweight resulted in an increased risk of incorrect self-perception. Among mothers, having an overweight child (OR = 3.02; 95%CI 2.05-4.46) and a child aged from 6 to 9 years (OR = 1.88; 95%CI 1.28-2.76) were associated with incorrect perception. CONCLUSIONS: A failure to correctly perceive weight was very frequent among children and their mothers, especially when children were overweight. These factors could represent obstacles to correctly recognizing nutritional abnormalities.

Poor zinc and selenium status in phenylketonuric children and adolescents in Brazil

Because of the restricted intake of high-biologic-value protein, children with phenylketonuria (PKU) may have lower than normal plasma concentrations of copper, zinc, and selenium. The purpose of the present study was to investigate erythrocyte zinc levels and serum copper and selenium levels in children and adolescents with PKU by analyzing the relation between their diet and the laboratory profiles of these elements. The study was conducted in 32 children and adolescents with PKU, who were on a special diet. Dietary records and blood samples were collected from each subject. Erythrocyte zinc and serum selenium levels were below normal in 37.5% and 90.6% of the subjects, respectively. Plasma copper levels were normal. Metabolic formulas were the only source of 86.9% of the zinc, 65.6% of the copper, and 32.4% of the selenium. Despite this, there was no significant correlation between the zinc formula supply and erythrocyte zinc levels (rho = -0.143, P = .435) or the supply and serum levels for copper (rho = -0.117, P = .523) and selenium (rho = 0.113, P = .538). These results suggest that Brazilian patients with PKU present with low ingestion levels, low serum selenium levels, and low erythrocyte zinc levels.

Stool pattern changes in toddlers consuming a follow-on formula supplemented with polydextrose and galactooligosaccharides.

ABSTRACT Healthy 9- to 48-month-old children (n = 133) were randomized to receive a cows-milk–based follow-on formula (control) or the same formula with polydextrose and galactooligosaccharides (PDX/GOS) for 108 days. Pediatricians assessed diarrheal disease, stool pattern, acute respiratory infection, systemic antibiotic use, and growth. The 2 groups had similar weight-for-length/height z score and similar odds of having diarrheal disease, acute respiratory infection, and systemic antibiotic use; however, PDX/GOS had greater odds of increased defecation than control (P ⩽ 0.01). Addition of PDX and GOS to a follow-on formula was well tolerated and induced a pattern of more frequent and softer stools in toddlers.

Aspectos clínicos da fenilcetonúria em serviço de referência em triagem neonatal da Bahia

OBJETIVOS: descrever as caracteristicas clinicas dos pacientes com hiperfenilalaninemia acompanhados no Servico de Referencia em Triagem Neonatal (SRTN) do estado da Bahia. METODOS: estudo descritivo transversal, tendo como amostra todos os pacientes com diagnostico conhecido de Hiperfenilalaninemia residentes no estado da Bahia e acompanhados no SRTN ate setembro de 2005. Tal populacao e composta de 46 familias, num total de 51 pacientes. A analise dos dados foi descritiva, incluindo medidas de tendencia central e dispersao. RESULTADOS: houve discreto predominio do genero feminino (52,9%). A maioria dos pacientes (78,4%) teve seu diagnostico estabelecido atraves da triagem neonatal, tendo, portanto, tratamento precoce. Consanguinidade foi registrada em 32,6% das familias. A media de inicio do tratamento entre os pacientes diagnosticados pela triagem neonatal foi de 56,6 37,8 dias, enquanto que entre os pacientes com diagnostico tardio, foi de 7,1 anos. CONCLUSOES: o estudo descreve um grupo de pacientes representativo de uma patologia incluida no Programa Nacional de Triagem Neonatal (PNTN), sendo, portanto, de relevância para a saude publica. Entre os dados clinicos, chama a atencao a media de idade do inicio do tratamento, superior ao recomendado na literatura, alertando para a necessidade de um maior enfoque no diagnostico precoce.

Calcium and fat metabolic balance, and gastrointestinal tolerance in term infants fed milk-based formulas with and without palm olein and palm kernel oils: a randomized blinded crossover study

BackgroundEffects of palm olein (POL) on calcium and fat metabolic balance and gastrointestinal (GI) tolerance have been clinically evaluated but its use in combination with palm kernel oil (PKO), and canola oil has not been similarly assessed in infants.MethodsCalcium and fat balance and GI tolerance were evaluated in 33 healthy term infants (age = 68-159d) in a randomized, double-blinded, 14d crossover trial at a day care center in Salvador, Brazil; followed by a 4d hospital ward metabolic balance study in 17 of the male subjects. The study compared two commercially available milk-based powdered formulas in Brazil; one containing POL (44% of total fat), PKO (21.7%) and canola oil (18.5%) as predominant fats (PALM), and the other containing none (NoPALM). Occasional human milk (HM) supplementation was allowed at home.ResultsFormula and HM intakes, and growth were not different (p > 0.05). Calcium absorption (%) for infants fed NoPALM (58.8 ± 16.7%; means ± SD) was higher (p = 0.023) than those fed PALM (42.1 ± 19.2%), but was not significant (p = 0.104) when calcium intake was used as a covariate. Calcium intake was higher (p < 0.001) in NoPALM versus PALM fed infants. However, calcium retention (%) was higher in infants fed NoPALM compared to PALM with (p = 0.024) or without (p = 0.015) calcium intake as a covariate. Fat absorption (%) for NoPALM was greater than PALM fed infants (NoPALM = 96.9 ± 1.2 > PALM = 95.1 ± 1.5; p = 0.020 in Study Period I). Mean rank stool consistency was softer in infants fed NoPALM versus PALM (p < 0.001; metabolic period). Adverse events, spit-up/vomit, fussiness and gassiness were not different (p > 0.05). Formula acceptability was high and comparable for both formula feedings, regardless of HM supplementation.ConclusionsTerm infants fed PALM based formula (containing palm olein, palm kernel and canola oils) demonstrated lower calcium retention and fat absorption, and less softer stool consistency versus infants fed NoPALM based formula. Study suggested formula fat differences may affect GI function in infants.Clinical trial registrationClinical Trial.Gov # (http://www.clinicaltrials.gov): NCT00941564.

Zinc and other Micronutrients Supplementation through the use of Sprinkles: Impact on the Occurrence of Diarrhea and Respiratory Infections in Institutionalized Children

OBJECTIVE To evaluate the incidence of diarrheal disease (DD) and acute respiratory infection (ARI) in children undergoing supplementation of zinc and other micronutrients through the use of sprinkles, as well as their acceptance by these patients. METHOD This was a randomized double-blinded clinical trial of 143 healthy institutionalized children, aged 6 to 48 months. They were randomized into two groups and received daily zinc and micronutrients - test group (sprinkles), or micronutrients without zinc - control group. Children were supplemented for 90 days and followed regarding the outcomes of DD and ARI. RESULTS Of the randomized children, 52.45% belonged to the test and 47.55% to the control group. The incidence of DD in the test group was 14.7% and was 19.1% in the control group. The test group showed a lower risk of developing DD when compared to controls, but this finding was not statistically significant (RR=0.77 [0.37 to 1.6], p=0.5088). ARI had high incidence in both groups, 60% in the test group and 48.5% in the control group, with an increased risk of developing the disease in the test group, but with no statistical significance (RR=1.24 [0.91 to 1.68], p=0.1825). Regarding acceptance, the mean percentage of consumption, in days, of the entire content of the sachets containing sprinkles was 95.72% (SD=4.9) and 96.4% (SD=6.2) for the test and control groups, respectively. CONCLUSIONS Zinc supplementation through the use of sprinkles did not reduce the incidence of DD or ARI among the evaluated children. The sprinkles were well accepted by all study participants.

Aspectos clínicos e demográficos da fenilcetonúria no Estado da Bahia

OBJETIVO: Describir las caracteristicas clinicas y demograficas de los pacientes con diagnostico de Hiperfenilalaninemia (HPA) seguidos en el Servicio de Referencia en Seleccion Neonatal de Bahia. METODOS: Estudio transversal incluyendo a 99 familias (111 afectados) con fenotipo bioquimico de HPA, con recoleccion de datos en prontuarios y en base de datos laboratorial, incluso aspectos demograficos y clinicos. Se realizo el analisis descriptivo de los datos utilizando promedios (desviacion estandar) y medianas (percentiles 25-75). RESULTADOS: La incidencia de HPA en Bahia fue de un caso a cada 16.334 nacidos vivos (NV), con cobertura de 90,8%. Entre los pacientes seguidos, el 82% fueron diagnosticados por la seleccion neonatal, y en 11 familias habia mas de un caso. El fenotipo clasico de la fenilcetonuria fue diagnosticado en 63 (56,8%) pacientes. Entre los seleccionados, la mediana de edad en la primera consulta fue de 39,5 dias (fenilcetonuria clasica) y, de ellos, el 34% presentaba sintomas en ese momento, ninguno de ellos con retraso en el desarrollo neuropsicomotor. La consanguinidad fue descrita en 32,2% de los casos y hubo predominio de pacientes clasificados como blancos (63%). Los padres tenian baja escolaridad y bajos ingresos. Entre los 417 municipios de Bahia, el 14,6% presentaba al menos un caso, con concentracion en la region nordeste (9,9%) y capital de la provincia (14,4%). CONCLUSIONES: Los resultados evidenciaron edad tardia al inicio del tratamiento, lo que puede comprometer los resultados del programa. Se observo, ademas, la presencia de consanguinidad y recurrencia familiar, reforzando la importancia de investigacion familiar para diagnosticar individuos con deficiencia mental de etiologia no aclarada que pueden beneficiarse de tratamiento.Objective: To describe demographic and clinical characteristics of patients with hyperphenylalaninemia followed at the Neonatal Screening Reference Service of Bahia, Brazil. Method: Cross-sectional study including 99 families (111 affected individuals) with biochemical phenotype of hyperphenylalaninemia by chart review and laboratory database that include demographic and clinical features. Results: The incidence of hyperphenylalaninemia in Bahia was one case per 16,334 live births, covering 91% of them. Among patients followed, 82% were diagnosed by newborn screening and, in 11 families, there were more than one case. The classic phenotype of phenylketonuria was diagnosed in 63 (57%) patients. Among those screened, the median age at first consultation was 39.5 days. Among the patients, 34% had symptoms at the first medical consulta tion, none of them with delayed neurodevelopment. Consanguinity was reported in 32% of patients. Affected individuals were predominantly classified as white (63%). The parents had low education and low income. Among the 417 municipalities of Bahia, 15% had at least one case, with a concentration in the Northeast (10%) and in the capital of the State (14%). Conclusions: The results showed elevated age at the beginning of the treatment, which may compromise the program results. Presence of consanguinity and familial recurrence were also noted. Careful investigation of families searching for individuals with mental retardation of unknown etiology that would benefit from the treatment is important.

Breastfeeding and the anthropometric profile of children with sickle cell anemia receiving follow-up in a newborn screening reference service

OBJECTIVE: To study the breastfeeding history (BF) and the anthropometric status of children with Sickle Cell Disease (SCD). METHODS: A cross-sectional study of 357 children with SCD aged between 2 and 6 years, regularly followed at a Newborn Screening Reference Service (NSRS) between November 2007 and January 2009. The outcome was anthropometric status and the exposures were: BF pattern, type of hemoglobinopathy and childs age and gender. RESULTS: The mean (SD) age was 3.7 (1.1) years, 52.9% were boys and 53.5% had SCA (hemoglobin SS). The prevalence of exclusive breastfeeding (EBR) up to six months of age was 31.5%, the median EBR times (p25-p75) was 90.0 (24.0-180.0) days and the median weaning ages (p25-p75) was 360.0 (90.0-720.0) days respectively. Normal W/H children experienced EBR for a mean duration almost four times longer than malnourished children (p=0.01), and were weaned later (p<0.05). Height deficit was found in 5.0% of children, while all the children with severe short stature had had SCA (hemoglobin SS) and were older than 4 years of age. CONCLUSIONS: EBF time and weaning age were greater than that found in the literature, which is a possible effect of the multidisciplinary follow-up. Duration of EBF and later weaning were associated with improved anthropometric indicators.

Clinical and demographic aspects of phenylketonuria in Bahia State, Brazil

OBJETIVO: Describir las caracteristicas clinicas y demograficas de los pacientes con diagnostico de Hiperfenilalaninemia (HPA) seguidos en el Servicio de Referencia en Seleccion Neonatal de Bahia. METODOS: Estudio transversal incluyendo a 99 familias (111 afectados) con fenotipo bioquimico de HPA, con recoleccion de datos en prontuarios y en base de datos laboratorial, incluso aspectos demograficos y clinicos. Se realizo el analisis descriptivo de los datos utilizando promedios (desviacion estandar) y medianas (percentiles 25-75). RESULTADOS: La incidencia de HPA en Bahia fue de un caso a cada 16.334 nacidos vivos (NV), con cobertura de 90,8%. Entre los pacientes seguidos, el 82% fueron diagnosticados por la seleccion neonatal, y en 11 familias habia mas de un caso. El fenotipo clasico de la fenilcetonuria fue diagnosticado en 63 (56,8%) pacientes. Entre los seleccionados, la mediana de edad en la primera consulta fue de 39,5 dias (fenilcetonuria clasica) y, de ellos, el 34% presentaba sintomas en ese momento, ninguno de ellos con retraso en el desarrollo neuropsicomotor. La consanguinidad fue descrita en 32,2% de los casos y hubo predominio de pacientes clasificados como blancos (63%). Los padres tenian baja escolaridad y bajos ingresos. Entre los 417 municipios de Bahia, el 14,6% presentaba al menos un caso, con concentracion en la region nordeste (9,9%) y capital de la provincia (14,4%). CONCLUSIONES: Los resultados evidenciaron edad tardia al inicio del tratamiento, lo que puede comprometer los resultados del programa. Se observo, ademas, la presencia de consanguinidad y recurrencia familiar, reforzando la importancia de investigacion familiar para diagnosticar individuos con deficiencia mental de etiologia no aclarada que pueden beneficiarse de tratamiento.Objective: To describe demographic and clinical characteristics of patients with hyperphenylalaninemia followed at the Neonatal Screening Reference Service of Bahia, Brazil. Method: Cross-sectional study including 99 families (111 affected individuals) with biochemical phenotype of hyperphenylalaninemia by chart review and laboratory database that include demographic and clinical features. Results: The incidence of hyperphenylalaninemia in Bahia was one case per 16,334 live births, covering 91% of them. Among patients followed, 82% were diagnosed by newborn screening and, in 11 families, there were more than one case. The classic phenotype of phenylketonuria was diagnosed in 63 (57%) patients. Among those screened, the median age at first consultation was 39.5 days. Among the patients, 34% had symptoms at the first medical consulta tion, none of them with delayed neurodevelopment. Consanguinity was reported in 32% of patients. Affected individuals were predominantly classified as white (63%). The parents had low education and low income. Among the 417 municipalities of Bahia, 15% had at least one case, with a concentration in the Northeast (10%) and in the capital of the State (14%). Conclusions: The results showed elevated age at the beginning of the treatment, which may compromise the program results. Presence of consanguinity and familial recurrence were also noted. Careful investigation of families searching for individuals with mental retardation of unknown etiology that would benefit from the treatment is important.

Avaliação do consumo alimentar de pacientes com mucopolissacaridose

AIMS: To assess the dietary intake of children and adolescents with mucopolysaccharidosis. METHODS: Series of cases of mucopolysaccharidosis regularly followed in a referral center in Salvador, Bahia state, Brazil, in January-April 2012. Patients of both sexes aged between 2 and 18 years with food intake by mouth and without clinical complications were considered to inclusion. Structured anamnesis was applied contemplating socioeconomic, clinical, and dietary intake information. The food consumption data were obtained from 24-hour dietary recall and food record of three days. Possible dietary inadequacies were evaluated facing the recommendations of the Dietary Reference Intakes, 2005. Data were tabulated in 3.1 Epidata and analyzed by the statistical package R. RESULTS: Eight boys and two girls, with a median age of 10 years (interquartile range 6.4 years, minimum 3, maximum 16 years) were studied. The most common type of the disease was Mucopolysaccharidosis VI (60 %) and less frequent mucopolysaccharidosis I (10%). Six patients needed assistance to feed, nine had low calorie intake, and six had low intake of lipids. All patients had insufficient intake of fiber and adequate intake of carbohydrates and proteins. The intake of all micronutrients had impairments. CONCLUSIONS: There was a high frequency of inadequate dietary intake in children and adolescents with mucopolysaccharidosis. Some osteoarticular limitations caused by the disease lead to problems in food intake, and many patients need assistance in the act of eating. A specialized nutritional intervention may assist in quality of life and prognosis of these individuals.