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- ItemAcesso aberto (Open Access)Comparative study of the growth and nutritional status of Brazilian and Nigerian school-aged children with sickle cell disease(Oxford Univ Press, 2017) Adegoke, Samuel A. [UNIFESP]; Figueiredo, Maria S. [UNIFESP]; Adekile, Adekunle D.; Braga, Josefina A. P. [UNIFESP]Background: Comparative studies of patients in different sociogeographic/ecological zones may unravel potential environmental and nutritional factors influencing disease phenotype. In sickle cell disease (SCD), differential access to comprehensive care may influence their growth and nutritional status. Methods: From June 2015 to February 2016, steady-state nutritional parameters of 109 Brazilian and 95 Nigerian children with SCD attending routine clinic visits at Universidade Federal de Sao Paulo, Brazil and Obafemi Awolowo University Teaching Hospital, Ile-Ife (Ilesa unit), respectively, were compared. Results: A relatively high proportion of the children in both centres (23.5%) were wasted [body-mass index (BMI)-for-age z-score<-2). BMI-for-age z-score, height-for-age z-score, upper arm fat area and fat percentage were lower in the Nigerian cohorts. More Nigerians, 29.5% (28/95) against 18.3% (20/109) were wasted, and had short stature, [12.6% (12/95) vs. 3.7% (4/109)] than Brazilians. A higher proportion of Brazilian patients were overweight or obese (9.2 vs. 4.3%), and taller for age (15.6 vs. 8.4%). None of the Nigerian patients had severe vitamin D deficiency, only 12.6% (12/95) had suboptimal vitamin D and 1.1% (1/95) had low serum zinc levels, unlike 79.8% (87/109) of the Brazilian patients with suboptimal vitamin D and 10.1% (11/109) with low zinc. Conclusion: Undernutrition is still prevalent among the two cohorts. Nigerian patients were thinner and had reduced linear growth for age. This observation justifies the continued need for specialized nutritional care for children with SCD. In addition to hydroxyurea therapy, research is needed to determine appropriate nutritional intervention and exercise regimens for these children.
- ItemAcesso aberto (Open Access)Determining the frequency of morphological characteristics in a sample of Brazilian children(Soc Brasil Pediatria, 2017) Perrone, Eduardo [UNIFESP]; Zanolla, Thais Arbocese [UNIFESP]; Fock, Rodrigo Ambrosio [UNIFESP]; Alvarez Perez, Ana Beatriz [UNIFESP]; Brunoni, DecioObjective: To establish the frequency of 82 morphological features in a sample of Brazilian children (between 3 and 13 years old), to understand the influence of age, gender, and ethnicity. Methods: This was a cross-sectional study that evaluated 239 children with typical development (between 3 and 13 years old) regarding the presence of 82 morphological characteristics. A previously described protocol, based on the London Dysmorphology Database, was applied to evaluate the sample. This protocol was culturally adapted to Brazilian Portuguese. Results: The frequency of 82 morphological characteristics was established in the sample; of 82 characteristics, 50% were considered morphological anomalies (frequency less than 4%). At least 25% of the sample presented more than one minor morphological anomaly. Age was shown to influence the frequency of the following morphological characteristics: widow's peak, prominent antihelix, prominent upper lip, irregular or crowded teeth, and clinodactyly, but had no influence on the frequency of minor morphological anomalies. Gender influenced dysplastic ears and attached earlobe, but had no influence on the frequency of minor morphological anomalies; ethnicity showed influence on camptodactyly and prominent antihelix. A statistically significant divergence was observed regarding 43 of the 73 morphological characteristics that could be compared with literature data (58.9%). Conclusions: The study determined the frequency of 82 morphological characteristics in 239 children with typical development. Age was the variable that showed more influence on the frequency of morphological characteristics, and comparison with literature data showed that the frequency depends on variables such as age and ethnicity. (c) 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda.
- ItemSomente MetadadadosHyperhomocysteinemia increases the risk of venous thrombosis independent of the C677T mutation of the methylenetetrahydrofolate reductase gene in selected Brazilian patients(Lippincott Williams & Wilkins, 2002-04-01) Morelli, V. M.; Lourenco, D. M.; D'Almeida, V; Franco, R. F.; Miranda, F.; Zago, M. A.; Noguti, MAE; Cruz, E.; Kerbauy, J.; Universidade Federal de São Paulo (UNIFESP); Universidade de São Paulo (USP)Fasting total homocysteine (tHcy) and the methylenetetrahydrofolate reductase (MTHFR) C677T mutation were evaluated in 91 patients with venous thromboembolism and without acquired thrombophilia, and in 91 age-matched and sex-matched controls. Hyperhomocysteinemia was detected in 11 patients (12.1%) and in two controls (2.2%), yielding an odds ratio (OR) for venous thrombosis of 6.1 [95% confidence interval (CI), 1.3-28.4]. After excluding 21 patients and four controls with other known genetic risk factors for venous thrombosis, the OR was not substantially changed (7.0; 95% Cl, 1.5-33.1). the prevalence of the MTHFR 677TT genotype was not significantly different in patients (9.9%) and in controls (5.5%), with an OR for venous thrombosis of 1.8 (95% CI, 0.6-5.8). Subjects with the MTHFR 677TT genotype showed higher levels of tHcy compared with the 677CC genotype in patients (P=0.010) and in controls (P=0.030). in conclusion, we found that fasting hyperhomocysteinemia is a risk factor for venous thrombosis in patients without known acquired thrombophilia and other genetic risk factors for venous thrombosis. Although tHcy levels are significantly higher in those homozygous for the MTHFR C677T mutation, this genotype does not increase the thrombotic risk in our study population. (C) 2002 Lippincott Williams Wilkins.
- ItemSomente MetadadadosIdentification of two novel HLA-B alleles in the Brazilian population: B*4509 and B*5212(Wiley-Blackwell, 2010-08-01) Veiga, T. R. [UNIFESP]; Rampim, G. F. [UNIFESP]; Gerbase-Lima, Maria [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
- ItemSomente MetadadadosInsulin resistance, beta-cell function, and glucose tolerance in Brazilian adolescents with obesity or risk factors for type 2 diabetes mellitus(Elsevier B.V., 2007-03-01) Querino da Silva, Regina Cintra; Miranda, Walkiria Lopes; Chacra, Antonio Roberto; Dib, Sergio Atala; Universidade Federal de São Paulo (UNIFESP)Objective: To evaluate insulin resistance (IR), beta-cell function, and glucose tolerance in 119 Brazilian adolescents with obesity or risk factors (RF) for type 2 diabetes mellitus (T2DM). Study Design: We analyzed weight (kg), height (m), body mass index (BMI; kg/m(2)), waist (W; cm), acanthosis nigricans (AN), systolic and diastolic blood pressure (SBP and DBP; mm Hg), fasting plasma glucose (FPG), and 2-h plasma glucose (2hPG) on oral glucose tolerance test (OGTT; 1.75 g of glucose/weight), lipid profile [total cholesterol (TC), fractions, and triglycerides (TGs)], fasting insulin (FI) and 2-h insulin on OGTT (2hI-RIA), HOMA-B (%; beta-cell function-HOMA program), HOMA-S (%; insulin sensitivity-HOMA program) and HOMA-IR [fasting plasma insulin (mU/ml) x fasting plasma glucose (mmol/L)/22.5]. Division according to number of RF-family history of T2DM (FHT2DM), obesity, hypertension, dyslipidemia, polycystic ovary syndrome (PCOS), and AN. G1: subjects with no or one RF; G2: subjects with two or more RFs. Statistical data were nonparametrical. Results: Fasting plasma glucose (G2: 81.6 +/- 10.2 vs. G1: 79.8 +/- 9.9 mg/dl) and 2hPG (88.1 +/- 18.0 vs. 87.0 +/- 19.9 mg/dl) were not different between G2 (n = 67) and G1 (n = 52), and all adolescents had normal glucose tolerance (NGT). Fasting insulin (13.0 +/- 7.9 vs. 7.6 +/- 3.9 mu IU/ml; P < .001) and 2hI (60.2 +/- 39.1 vs. 38.3 +/- 40.0 mu IU/ml; P < .001), HOMA-B (169.1 +/- 131.6% vs. 106.1 +/- 39.9%; P < .001), and HOMA-IR (2.62 +/- 1.7 vs. 1.52 +/- 0.8; P < .001) were higher in G2. HOMA-S (92.5 +/- 59.5% vs. 152.2 +/- 100.5%; P < .001) was also lower in this lattergroup. Conclusion: Brazilian adolescents with two or more RFs for the development of T2DM have higher IR and P-cell function and lower insulin sensitivity. However, adolescents with impaired glucose tolerance (IGT) or DM have not been found, differently from similar studies. Differences in ethnic background, environment, and lifestyle factors may account for this disparity. (c) 2007 Elsevier Inc. All rights reserved.
- ItemAcesso aberto (Open Access)Latent Autoimmune Diabetes of the Adult (LADA) in a Brazilian Indian(Associação Paulista de Medicina - APM, 2001-03-01) Vieira Filho, João Paulo Botelho [UNIFESP]; Moises, Regina Celia Mello Santiago [UNIFESP]; Sá, João Roberto De [UNIFESP]; Chacra, Antonio Roberto [UNIFESP]; Dib, Sergio Atala [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)CONTEXT: Latent autoimmune diabetes of the adult (LADA) as originally described represents perhaps as many as 10 -- 20% of adult-onset patients with diabetes. DESIGN: case report. CASE REPORT: A 38-year-old Brazilian Xavante-Jê Indian with Latent Autoimmune Diabetes of the Adult (LADA) is described, coming from the Sangradouro community in Poxoréu, Mato Grosso. The onset of diabetes after reaching 25 years of age, the evolution to insulin deficiency after a period of insulin-independence and the presence of auto-antibodies to glutamic acid decarboxylase (GAD) characteristic of LADA were present. This patient may represent the first case of LADA in a Brazilian with full Indian heritage. Further studies are necessary to verify the prevalence of this new type of diabetes in this population that does not have Caucasoid admixture and has a particular environmental background.