[pubertal Growth And Final Height In 40 Patients With Type 1 Diabetes Mellitus].

Type 1 diabetes mellitus (DM1), the most important chronic endocrine-metabolic disease in children and adolescents, may lead to delayed growth and puberty. In this study we analyzed the influence of DM1 on growth spurt and puberty of patients whose onset of the disease was before or at the beginning of this phase. Data from 40 patients, 25 females, who had attained final height were retrospectively obtained, including duration of disease, patient's height and weight SDS at each consultation, parental target height, yearly growth velocities (GV), peak of growth spurt, duration of puberty, magnitude of growth spurt and glycated hemoglobin (HbA1C) levels. 37 patients had an adequate final height to parental target height, and only 3 were below the lower limit. There was no significant association among the variables and the appropriate final height to the target height, except for GV on growth spurt, when it was lower than or equal to 6 cm/year. The age of onset of DM1 and the age of peak of growth spurt was similar to previously data reported; regarding the age of the onset of puberty, there was no delay. However, the magnitude of the growth spurt and the peak of GV were lower. According to HbA1C levels, all patients exhibited a bad chronic control of DM1. In this group of inadequately controlled patients, the final height was lower than expected when compared to the height at onset of DM1, probably due to a slow GV during puberty, which however had no influence on the final height to parental target height.49396-40

Growth in patients with the salt-wasting form of congenital adrenal hyperplasia during the first two years of life

OBJECTIVES: to assess the growth and nutritional recovery of patients with the classical salt-wasting form of congenital adrenal hyperplasia in the first two years of life. METHODS: z scores for weight and height were calculated for 21 patients at birth, on the occasion of the first medical consultation and at one and two years of age. The concentrations of 17-hydroxyprogesterone, androstenedione and the doses of hydrocortisone prescribed at the first medical concentrations up to the age of two years were determined (at one and two years of age respectively). RESULTS: the mean age for the first medical consultation was 36.7 days. The z score for weight at birth was -0.23±1.4; on the occasion of the first consulta tion -2.31±1.3; at the age of one year -1.43±1.6 and at the age of two years -0.77± 1.3. The z score for height at birth was -0.69±2.3; on the occasion of the first consultation -1.87±1.7; at one year of age 1.68±1.1 and at two years -1.07±1.0. The difference between the scores at two years of age and on the occasion of the first medical consultation was 1.54±1.7 for weight and 0.80±1.6 for height. The mean dosage of hydrocortisone prescribed was 21.3 and 19.9 mg/m2/day for periods 1 and 2 and the concentrations (ng/dL) of 17-hydroxyprogesterone and androstenedione were 9.1 and 0.14 for period 1 and 4.4 and 0.27 for period 2. CONCLUSIONS: nutritional recovery was observed to occur on treatment and, at two years of age, weight and height are normal, although below the average for the population at large.OBJETIVOS: avaliar crescimento e recuperação nutricional de pacientes com hiperplasia congênita supra-renal, forma clássica perdedora de sal, nos dois primeiros anos de vida. MÉTODOS: analisamos escores z de peso e comprimento de 21 pacientes ao nascimento, primeira consulta, com um e dois anos de idade. Determinamos concentrações de 17-hidroxiprogesterona, androstenediona e doses de hidrocortisona prescritas da primeira consulta até um e dois anos de idade (períodos 1 e 2, respectivamente). RESULTADOS: a média de idade na primeira consulta foi 36,7 dias. Escore z do peso ao nascimento foi -0,23±1,4; na primeira consulta -2,31±1,3; com um ano -1,43±1,6 e dois anos -0,77± 1,3. Escore z do comprimento ao nascimento foi -0,69±2,3; na primeira consulta -1,87±1,7; com um ano -1,68±1,1 e dois anos -1,07±1,0. A diferença entre os escores aos dois anos e na primeira consulta foi 1,54±1,7 para o peso e 0,80±1,6 para o comprimento. Média de hidrocortisona prescrita foi 21,3 e 19,9 mg/m2/dia nos períodos 1 e 2 e concentrações (ng/dL) de 17-hidroxiprogesterona e androstenediona foram 9,1 e 0,14 no período 1 e 4,4 e 0,27 no 2, respectivamente. CONCLUSÕES: foram observados recuperação nutricional com o tratamento e, aos dois anos, peso e comprimento normais, embora inferiores aos da população.41542

Normalization of height and excess body fat in children with salt-wasting 21-hydroxylase deficiency

OBJECTIVE: To evaluate growth and body composition of patients with the salt wasting form of classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and to compare them with healthy children. METHODS: Twenty-one prepubertal patients (eight boys and 13 girls) between 2.1 and 10.2 years and 67 prepubertal healthy controls (36 boys and 31 girls) between 1.2 and 11.7 years were included. Weight, height, upper-arm circumference, skinfolds, body composition determined by bioimpedance, and bone age were measured. The following data were obtained from the medical records: parents' height, serum levels of 17-hydroxyprogesterone and &#916;4-androstenedione, prescribed hydrocortisone doses, weight and length at birth, in the beginning of the treatment, and at 2 years. RESULTS: Patients had lower weight and length z scores at the first appointment compared with the same data at birth, showing recovery after the beginning of the treatment without advanced bone age. Mean height z score was higher in controls (0.28±0.86) than in patients (-0.61±0.99, p < 0.001); this difference disappeared when the patients' height was adjusted to their bone age (0.33±1.68, p = 0.912). Patients had higher body mass index (p < 0.001), fat mass (p < 0.001), and fat mass index (p < 0.001) than controls. There was no difference in the skinfolds between the two groups (p = 0.157). CONCLUSIONS: Patients had growth recovery with mean height similar to the general population; however, they had higher body fat, which seems to be visceral, since there was no difference between the skinfolds of both groups.OBJETIVO: Avaliar crescimento e composição corporal de portadores da forma clássica perdedora de sal da hiperplasia adrenal congênita por deficiência da 21-hidroxilase, comparando-os com crianças saudáveis. MÉTODOS: Foram incluídos 21 pacientes (oito meninos e 13 meninas), entre 2,1 e 10,2 anos, e 67 controles pré-púberes (36 meninos e 31 meninas), entre 1,2 e 11,7 anos. Avaliou-se peso, estatura, perímetro braquial, dobras cutâneas, composição corporal por bioimpedância e idade óssea. Foram obtidas dos prontuários dos pacientes as seguintes informações: estatura dos pais, valores de 17-OH progesterona e &#916;4-androstenediona, dose de hidrocortisona prescrita, dados de peso e estatura ao nascimento, no início do tratamento e aos 2 anos de idade. RESULTADOS: Os pacientes apresentaram menor escore z de peso e de altura na primeira consulta em relação à situação de nascimento, com posterior recuperação após o início do tratamento, sem apresentar avanço da idade óssea. A média do escore z da altura dos controles (0,28±0,86) foi maior que a dos casos (-0,61±0,99, p < 0,001). Essa diferença desaparece quando se ajusta a altura dos pacientes para a idade óssea (0,33±1,68, p = 0,912). Os pacientes apresentaram maiores índices de massa corporal (p < 0,001), massa gorda (p < 0,001) e índice de massa gorda (p < 0,001) do que os controles. Não houve diferença entre as dobras cutâneas dos 2 grupos (p = 0,157). CONCLUSÕES: Os pacientes apresentaram recuperação do crescimento com média de estatura semelhante à da população geral, porém com maior adiposidade corporal, que parece ser visceral, já que não houve diferença entre as dobras cutâneas.26326

[evidences For Subclinic Chronic Autoimmune Thyroid Disease In Girls With Turner Syndrome].

Patients with Turner syndrome (TS) frequently exhibit transient, recurrent and asymptomatic variations of TSH and/or thyroid hormones (TH). This work was carried out to evaluate thyroid function and structure in patients with TS who had had such variations in hormone concentrations. Our sample comprised 24 patients, 17 less than 20-years old. Evaluation included serum levels of TSH, free T4, total T3, TPO and Tg autoantibodies, thyroid ultrasound (US) and scintigraphy with 99mTc-pertechnetate. Thirteen patients had abnormal TSH and/or TH levels; 23 exhibited US features compatible with chronic thyroid disorder, particularly thyromegaly (established according to volume expected for stature) and heterogeneous echogenicity. Uptake was normal in 21 cases and tracer distribution was homogeneous in 22. The finding of abnormal hormone concentrations was independent of age, length of time since the first similar finding, thyroid autoantibodies, number of abnormalities at US and abnormal scintigraphic findings. Patients aged more than 20 years had higher frequency of thyroid antibodies and heterogeneous echogenicity, and thyroid volume was significantly correlated to length of time since detection of the first hormone variation, indicating progressive thyroid disease. These results suggest that subclinical thyroid dysfunction in TS is due to chronic autoimmune thyroid disease.51401-

Search for new particles in events with energetic jets and large missing transverse momentum in proton-proton collisions at root s=13 TeV

A search is presented for new particles produced at the LHC in proton-proton collisions at root s = 13 TeV, using events with energetic jets and large missing transverse momentum. The analysis is based on a data sample corresponding to an integrated luminosity of 101 fb(-1), collected in 2017-2018 with the CMS detector. Machine learning techniques are used to define separate categories for events with narrow jets from initial-state radiation and events with large-radius jets consistent with a hadronic decay of a W or Z boson. A statistical combination is made with an earlier search based on a data sample of 36 fb(-1), collected in 2016. No significant excess of events is observed with respect to the standard model background expectation determined from control samples in data. The results are interpreted in terms of limits on the branching fraction of an invisible decay of the Higgs boson, as well as constraints on simplified models of dark matter, on first-generation scalar leptoquarks decaying to quarks and neutrinos, and on models with large extra dimensions. Several of the new limits, specifically for spin-1 dark matter mediators, pseudoscalar mediators, colored mediators, and leptoquarks, are the most restrictive to date.Peer reviewe

Probing effective field theory operators in the associated production of top quarks with a Z boson in multilepton final states at root s=13 TeV

Peer reviewe

[growth And Body Composition In Children With Type 1 Diabetes Mellitus].

To evaluate the growth and body composition of pre-pubertal diabetic children, and to check for influence of the age of diabetes onset and length, sex, insulin requirement and glycosylated hemoglobin. 59 diabetic children (39 M; 29 F), age 1.2-11.5 years, and 67 controls (36 M; 31 F), age 1.2-11.7 years were included. Weight, height, body mass index (BMI), arm circumference, skin folds, fat mass and muscle areas were evaluated and transformed into standard deviation scores (SDS). Among the diabetic children the mean height SDS was -0.13 (+/- 0.97) while in the control group it was 0.28 (+/- 0.86) (p= 0.013). The difference between the first and the current height SDS showed that the height SDS decreased significantly (p< 0.001) and multiple regression analysis indicated correlation with the duration of the disease. The mean arm fat SDS also revealed difference (p< 0.001). The means for weight, BMI, addition of 3 skinfolds and muscle mass did not demonstrate difference between the groups. The diabetic children showed reduction of height SDS during the period studied and they were significantly shorter than the controls, even though their statures were within the population standards. The arm fat area also showed an increase in relation with the controls.50490-

Síndrome de Turner: um diagnóstico pediátrico frequentemente realizado por não pediatras

OBJETIVO: Analisar as características clínicas de pacientes com suspeita de síndrome de Turner (ST) em um serviço de referência. MÉTODOS: Análise retrospectiva de 425 pacientes. Foram colhidos dados de idade, estatura e estádio puberal no momento do diagnóstico, bem como da especialidade do médico que encaminhou a paciente. Comparação das pacientes com e sem ST e daquelas com ST de acordo com a especialidade e análise de correlação entre estatura e idade ao diagnóstico. RESULTADOS: O diagnóstico de ST foi feito, em 36,9% dos casos, com média de idade de 12,0 anos e escore z da estatura = -3,09; havia atraso puberal em 71,4% das 63 pacientes maiores de 13 anos. Comparadas às demais, as meninas com ST apresentavam maior deficit na estatura e maior frequência de atraso puberal. Pacientes com ST encaminhadas por pediatras eram significativamente mais jovens (9,3 anos versus 15,4 anos), porém com estatura e frequência de atraso puberal semelhantes às daquelas encaminhadas por não pediatras. Houve correlação linear negativa significativa entre idade ao diagnóstico e estatura no total de pacientes com ST, mas não entre as encaminhadas por não pediatras. CONCLUSÕES: A média de idade ao diagnóstico da ST ainda é superior àquela dos países desenvolvidos, e a presença de sinais puberais espontâneos e/ou de deficit de crescimento menos acentuado em algumas pacientes pode contribuir para o atraso na suspeita clínica. É necessária divulgação entre pediatras e não pediatras dos conhecimentos necessários ao diagnóstico precoce da ST