These results show that the cytotoxic effects of CT cause severe damage to the germinal epithelium with subtle effects on Sertoli cells. To assess Sertoli cell function in men with primary testicular damage after treatment with CT in childhood and adolescence, the inhibin B level needs to be interpreted in the context of the circulating FSH, especially when normal FSH levels are observed.

BackgroundThe prevalence of diabetes mellitus is higher in individuals with Down syndrome (DS) than in the general population; it may be due to the high prevalence of obesity presented by many of them. The aim of this study was to evaluate the insulin resistance (IR) using the HOMA (Homeostasis Model Assessment) method, in DS adolescents, describing it according to the sex, body mass index (BMI) and pubertal development.Methods15 adolescents with DS (8 males and 7 females) were studied, aged 10 to 18 years, without history of disease or use of medication that could change the suggested laboratory evaluation. On physical examination, the pubertal signs, acanthosis nigricans (AN), weight and height were evaluated. Fasting plasma glucose and insulin were analysed by the colorimetric method and RIA-kit LINCO, respectively. IR was calculated using the HOMA method. The patients were grouped into obese, overweight and normal, according to their BMI percentiles. The EPIINFO 2004 software was used to calculate the BMI, its percentile and Z score.ResultsFive patients were adults (Tanner V or presence of menarche), 9 pubertal (Tanner II – IV) and 1 prepubertal (Tanner I). No one had AN. Two were obese, 4 overweight and 9 normal. Considering the total number of patients, HOMA was 1.7 ± 1.0, insulin 9.3 ± 4.8 μU/ml and glucose 74.4 ± 14.8 mg/dl. The HOMA values were 2.0 ± 1.0 in females and 1.5 ± 1.0 in males. Considering the nutritional classification, the values of HOMA and insulin were: HOMA: 3.3 ± 0.6, 2.0 ± 1.1 and 1.3 ± 0.6, and insulin: 18.15 ± 1.6 μU/ml, 10.3 ± 3.5 μU/ml and 6.8 ± 2.8 μU/ml, in the obese, overweight and normal groups respectively. Considering puberty, the values of HOMA and insulin were: HOMA: 2.5 ± 1.3, 1.4 ± 0.6 and 0.8 ± 0.0, and insulin: 13.0 ± 5.8 μU/ml, 7.8 ± 2.9 μU/ml and 4.0 ± 0.0 μU/ml, in the adult, pubertal and prepubertal groups respectively.ConclusionThe obese and overweight, female and adult patients showed the highest values of HOMA and insulin.

Aims: Isolated premature pubarche (PP) is commonly caused by premature adrenarche (PA), and links between PA, children born small for gestational age (SGA) and insulin resistance have already been made in some populations. Subjects and Methods: We assessed anthropometric data, pubertal landmarks and metabolic profile at diagnosis and during the study in 52 girls with the diagnosis of isolated PP from a Brazilian cohort. Results: The prevalence of obesity (25%), dyslipidemia (63.5%) and born SGA children (21.2%) was greater among PP girls than in the reference population (4, 46.8 and 10%, respectively). There was no increase in the prevalence of insulin resistance and no correlation with birth weight, onset of PP or other pubertal signs. The Z score of heights at PP diagnosis was greater than the Z score of mid-parental height, but achieved final height (n = 16 girls, p = 0.002) was similar to normal population standards and to the predicted final height based on bone age at PP diagnosis (p = 0.08). Thelarche and menarche occurred at normal age, just earlier than expected. Conclusion: The prevalence of children born SGA, obese and dyslipidemic, but not of hyperinsulinemic children, was high in our cohort of PP girls. Puberty started earlier than usual but within the normal range.

Septo-optic dysplasia (SOD), also referred to as de Morsier syndrome, is a rare congenital condition, characterized by two of the classic triad features: midline brain abnormalities, optic nerve hypoplasia (ONH) and pituitary endocrine dysfunction. We report 5 children with SOD, originally referred to be evaluated due to short stature, who also presented bilateral optic nerve hypoplasia, nystagmus and development delay. In 4 of the patients, we identified neuroimaging abnormalities of the hypothalamo-pituitary axis such as anterior pituitary hypoplasia (3/5), ectopic posterior pituitary (4/5), thin or absent stalk (3/5) and empty sella (1/5). We also encountered diverse pituitary deficiencies: growth hormone (3/5), adrenocorticotropic hormone (3/5), thyroid-stimulating hormone (2/5) and antidiuretic hormone (1/5). Only one child presented intact pituitary function and anatomy. Although rare, SOD is an important cause of congenital hypopituitarism and it should be considered in children with optic nerve hypoplasia or midline brain abnormalities for early diagnosis and treatment. Key words: septo-optic dysplasia, optic nerve/abnormalities, hypopituitarism. Displasia septo-óptica resumoA displasia septo-óptica (DSO, síndrome de Morsier) é uma condição congênita rara definida por dois critérios da tríade: defeitos de linha média, hipoplasia de nervo óptico e insuficiência hipotálamo-hipofisária. Descrevemos 5 casos de DSO, encaminhados por baixa estatura, com hipoplasia dos nervos ópticos, nistagmo e atraso global do desenvolvimento. Destes, 4 possuem alteração na ressonância magnética de sela túrcica e insuficiência hipotálamo-hipofisária, tendo sido observada adeno-hipófise hipoplásica (3/5), neuro-hipófise ectópica (4/5), haste afilada/ausente (3/5) e sela vazia (1/5), além de déficit dos eixos somatotrófico (3/5), adrenocorticotrófico (3/5), tireotrófico (2/5) e do hormônio antidiurético (1/5). Apenas um paciente não apresenta alteração anatômica ou funcional do eixo hipotálamo-hipofisário. Embora rara, a DSO é causa importante de hipopituitarismo congênito, devendo ser considerada em crianças com hipoplasia de nervo óptico ou defeito de linha média para seu diagnóstico e tratamento precoces, evitando seqüelas graves. Palavras-chave: displasia septo-óptica, nervo óptico/anormalidades., hipopituitarismo.

IntroduçãoO murici (Byrsonima ssp., Malpighiaceae) é um fruto do Cerrado consumido principalmente in natura, sendo encontrado de dezembro a março, nas regiões serranas do Sudeste, nos cerrados de Mato Grosso e Goiás e no litoral do Norte e Nordeste do Brasil. Quando maduro, apresenta-se amarelado, com diâmetro de 1,5 a 2 cm e um forte odor semelhante a queijo rançoso (REZENDE;FRAGA, 2003;ALVES;FRANCO, 2003). Sua comercialização se restringe às feiras livres e mercados locais. A polpa é carnosa e macia, podendo ser consumida in natura ou sob a forma de sucos, geléias, sorvetes e licores ( ALVES; FRANCO, 2003).Há poucos estudos sobre as técnicas agronômicas adequadas para o cultivo e propagação desta frutífera (USP, 2007), assim como o seu potencial de utilização na alimentação humana, o que evidencia a importância de pesquisas sobre o valor nutricional do murici, bem como de produtos derivados deste fruto de sabor e aroma exóticos.A desidratação dos frutos para a obtenção do murici-passa contribui para a preservação do produto, concentra o conteúdo de macronutrientes e possibilita sua adição em formulações, ressaltando a qualidade sensorial e nutricional de alimentos. AbstractResearch on the nutritional value of the Cerrado (vast tropical savanna ecoregion of Brazil) fruits and their derivative products contribute to the use of regional products in human nourishment. This work aimed at evaluating the chemical, physical, and microbiological composition of murici in natura and murici dried fruits. The fruits were processed in three repetitions (MP1, MP2, MP3) using the osmotic dehydration followed by drying in a forced air circulation oven. The centesimal composition, water activity, pH, and soluble solids of murici dried and murici in natura were analyzed. The data were analyzed by the variation coefficient, the variance analysis, and the Tukey test (p < 0,05). The dehydrated fruits lost about 63 to 68% of water concentrating their macronutrients, their energy, and their soluble solids when compared to the murici in natura. Water activity varied from 0,78 to 0,85 characterizing the dried murici as a dry product with intermediate moisture. The pH remained below 4,5 for both dehydrated and in natura fruits. The dried murici fruit presented satisfactory consumption microbiological conditions. It can be concluded that the nutritional value of the dried murici fruit indicates a fiber-rich food, and its production can contribute to the value of regional food. Keywords: dried murici; dehydration; chemical evaluation; nutritional value. ResumoEstudos sobre o valor nutricional dos frutos do Cerrado e de seus produtos contribuem para utilização dos alimentos regionais na alimentação humana. Este trabalho teve por objetivo avaliar a composição química, física e microbiológica dos frutos de murici-passa e in natura. O processamento do Murici-Passa (MP) foi realizado em três repetições (MP1, MP2 e MP3), utilizando-se a desidratação osmótica seguida de secagem em estufa com circulação forçada de ar. Analisou-se a composição cen...