RESUMO
OBJECTIVES: To characterize immunologic function and clinical characteristics in patients with chromosome 22q11.2 deletion syndrome and determine whether there was significant change over time. METHODS: This study characterized the laboratory and clinical features of the immunodeficiency in a cohort of 195 patients with chromosome 22q11.2 deletion syndrome and used cross-sectional and analysis of variance to compare the findings in different age groups with control patients. Changes over time were also characterized by a model effect method in a subset of patients who were studied serially. RESULTS: Diminished T cell counts in the peripheral blood are common in patients with chromosome 22q11.2 deletion syndrome. The pattern of changes seen with aging in normal control patients was also seen in patients with chromosome 22q11.2 deletion syndrome, although the decline in T cells was blunted. Autoimmune disease was seen in most age groups, although the types of disorders varied according to age. Infections were also common in older patients, though they were seldom life threatening. CONCLUSIONS: Slow declines in T cell populations are seen in chromosome 22q11.2 deletion syndrome. Clinical manifestations of immunodeficiency, such as recurrent infection and autoimmune disease, were common in this population but had little relationship to specific immunologic laboratory features.
Assuntos
Deleção Cromossômica , Cromossomos Humanos Par 22 , Síndrome de DiGeorge/imunologia , Formação de Anticorpos , Doenças Autoimunes/epidemiologia , Criança , Pré-Escolar , Modificador do Efeito Epidemiológico , Feminino , Humanos , Lactente , Recém-Nascido , Contagem de Linfócitos , Masculino , Subpopulações de Linfócitos T , Linfócitos TRESUMO
OBJECTIVE: To determine prospectively the relationship among growth, nutritional status, and pulmonary function over a 4-year period in a large cohort of children with cystic fibrosis (CF). STUDY DESIGN: CF Foundation National CF Patient Registry data collected from 1991 to 1995 for 968 children (507 male) aged 5 to 8 years with pancreatic insufficiency and forced expiratory volume in 1 second within 60% to 140% of predicted values (FEV(1)%) were analyzed longitudinally. Variables hypothesized to affect FEV(1)% included age, sex, z scores for height, weight, percent of height-appropriate body weight, and annual number of days hospitalized. RESULTS: The significant decline in FEV(1)% was curvilinear and dependent on baseline FEV(1)%; children with initial FEV(1)% > or = 90 declined 2.6 U/y more than those with initial FEV(1)% <90. Boys gained but girls declined in z scores for height. Girls decreased in z scores for weight at a greater rate than boys. The z scores for weight and percent of height-appropriate body weight were significantly associated with longitudinal changes in FEV(1)%, after adjustment was done for hospitalizations. CONCLUSIONS: Growth, nutritional status, and pulmonary function are not stable in prepubertal children with CF and pancreatic insufficiency. Important sex-related differences in growth occur before puberty. Growth and nutritional status are associated with changes in FEV(1)%, suggesting that nutritional intervention may slow the decline in pulmonary function in children with CF.
Assuntos
Fibrose Cística/fisiopatologia , Crescimento , Pulmão/fisiopatologia , Estado Nutricional , Estatura , Peso Corporal , Criança , Fibrose Cística/complicações , Progressão da Doença , Insuficiência Pancreática Exócrina/complicações , Feminino , Volume Expiratório Forçado , Hospitalização , Humanos , Estudos Longitudinais , Masculino , Estudos ProspectivosRESUMO
OBJECTIVE: Low bone mineral density (BMD) is a recognized complication of Crohn's disease (CD). The aim of this study was to identify the risk factors for low BMD in pediatric patients with CD. STUDY DESIGN: One hundred nineteen subjects with CD ranging in age from 5 to 25 years were enrolled. BMD of the lumbar spine was measured by dual-energy x-ray absorptiometry. Growth parameters were assessed by examination. Disease-specific variables and use of selected medications were determined by chart review. RESULTS: Powerful risk factors for low BMD z-score included hypoalbuminemia, exposure to nasogastric tube feeds, total parenteral nutrition, 6-mercaptopurine, and corticosteroids. Corticosteroid dosing at a level >7.5 mg/d, 5000 mg lifetime cumulative dose, or >12 months of lifetime exposure were significant risk factors for low BMD z-score. Weaker but significant associations with low BMD z-scores included measures of disease severity such as pediatric Crohn's disease activity index, hospital admissions, and length of hospital stay. Site and duration of disease were not predictive. CONCLUSIONS: The presence of several clinically available factors was predictive of poor bone mineral status in this sample of subjects with CD. Hypoalbuminemia, corticosteroid exposure, nasogastric tube feeds, total parenteral nutrition, and 6-mercaptopurine were the most powerful risk factors for low bone mineral status.
Assuntos
Densidade Óssea , Doença de Crohn/complicações , Absorciometria de Fóton , Adolescente , Corticosteroides/uso terapêutico , Adulto , Estatura , Peso Corporal , Criança , Pré-Escolar , Doença de Crohn/metabolismo , Nutrição Enteral , Feminino , Humanos , Vértebras Lombares/diagnóstico por imagem , Masculino , Mercaptopurina/uso terapêutico , Análise Multivariada , Nutrição Parenteral Total , Fatores de Risco , Albumina Sérica/análiseRESUMO
OBJECTIVE: To evaluate the growth of hypercholesterolemic children completing an innovative, physician-initiated, home-based nutrition education program or standard nutrition counseling that aims to lower dietary fat intake. STUDY DESIGN: From suburban pediatric practices, 261 3.9- to 9.9-year-old children with elevated cholesterol levels and 81 children with nonelevated cholesterol levels were identified. The children with hypercholesterolemia were randomly assigned to the home-based education program, standard nutrition counseling, or an at-risk control group. Height, weight, skinfold measures, and dietary intake were evaluated at baseline, 3, 6, and 12 months; changes in anthropometric measures among treatment groups were compared over time. RESULTS: The intervention groups demonstrated significant decreases in fat and saturated fat intake after the interventions; however, weight z-score, height z-weight-for-height-median, and sum of skinfolds did not vary among the treatment groups over the year. At baseline, height z-score, weight z-score, and weight-for-height-median were positively associated with caloric intake, whereas weight z-score, weight-for-height-median, and sum of skinfolds were positively associated with fat intake. When the groups were combined and the children were grouped by average fat intake quintiles, no association between fat intake and changes in weight z-score, height z-score, or weight-for-height-media was observed. Differences over time in sum of skinfolds among fat intake quintile groups (suggesting a negative association between fat intake and body fat) that approached statistical significance (p = 0.06) were observed. CONCLUSIONS: These results support the safety, with respect to growth, of physician-initiated dietary intervention and lower fat diets for children with hypercholesterolemia. In addition, low dietary fat intake was associated with lower body fat.
Assuntos
Dieta com Restrição de Gorduras , Crescimento , Hipercolesterolemia/dietoterapia , Estatura , Peso Corporal , Criança , Pré-Escolar , Gorduras na Dieta/administração & dosagem , Feminino , Educação em Saúde/métodos , Humanos , Hipercolesterolemia/fisiopatologia , Masculino , Fenômenos Fisiológicos da Nutrição , Valores de Referência , Dobras CutâneasRESUMO
OBJECTIVE: To describe the age-related changes in cardiovascular disease risk factors in young, hypercholesterolemic (HC) children. METHODS: Hypercholesterolemic (n = 227) and nonhypercholesterolemic (NHC) (n = 80) children between the ages of 4 and 10 years were identified. Height, weight, skin-fold and blood pressure measurements, and total cholesterol levels were measured. The HC group also had insulin levels evaluated. The groups were compared by analysis of variance. Simple Spearman correlations evaluated the associations between factors within each group. RESULTS: The HC and NHC groups had similar mean ages, heights, and weights, both contained 51% girls, and all were white subjects. Percent weight-for-height median, and biceps, triceps, suprailiac and subscapular skin-fold measurements were all larger for the HC group. A significant age interaction demonstrated that the HC group's larger suprailiac and sum of skin-fold measures were expressed in the 8.0- to 9.9-year-old children, but not the 4.0- to 5.9-year-olds. For both groups, systolic blood pressure was associated with the measures of adiposity. For the HC group, insulin levels were also associated with adiposity. CONCLUSIONS: These results suggest that: (1) children with HC have greater body fat, (2) the expression of the hypercholesterolemia precedes the expression of increased body fat, (3) body fat increases with age, and (4) altered insulin and blood pressure levels are expressed in association with the increased body fat in children with HC. Confirmation with longitudinal data is necessary.