Subcutaneous adipose tissue plays a beneficial effect on subclinical atherosclerosis in young survivors of acute lymphocytic leukemia

Adriana Aparecida Siviero-Miachon,1,2 Angela Maria Spinola-Castro,1,2 Maria Lucia de Martino Lee,2 Carlos Manoel de Castro Monteiro,3 Antonio Carlos de Camargo Carvalho,4 Antonio Ramos Calixto,5 Bruno Geloneze,5 Gil Guerra-Junior6 1Division of Pediatric Endocrinology, Department of Pediatrics, Federal University of Sao Paulo (UNIFESP/EPM), 2Pediatric Oncology Institute – IOP/GRAACC, Federal University of Sao Paulo (UNIFESP/EPM), 3Private Office, Castro Monteiro, Sao Paulo, 4Division of Cardiology, Federal University of Sao Paulo (UNIFESP/EPM), 5Laboratory of Investigation on Metabolism and Diabetes (LIMED), Faculty of Medical Sciences, State University of Campinas (UNICAMP), 6Division of Pediatric Endocrinology, Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP), Brazil Purpose: The aim of this study was to evaluate the relationship between body composition, metabolic profile, adipokines, and carotid intima-media thickness (cIMT) in young survivors of childhood acute lymphocytic leukemia (ALL). Patients and methods: This cross-sectional study compared 55 ALL survivors, of chronological age between 15 years and 24 years, assigned into two groups according to the exposure to cranial radiation therapy (CRT; 25 irradiated and 30 nonirradiated) with 24 leukemia-free controls, and assessed body fat mass (dual-energy X-ray absorptiometry), computed tomography scan-derived abdominal adipose tissue, lipid profile, blood pressure (BP), adipokines, and cIMT by a multiple regression analysis. Results: Treatment with CRT had an effect on all of the variables derived from the computed tomography scan: visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) (P<0.050). In a multiple linear regression model, cIMT positively correlated with exposure to CRT (P=0.029), diastolic BP (P=0.016), and leptin-to-adiponectin ratio (P=0.048), while negatively related to SAT (P=0.007). Conclusion: In young survivors of childhood ALL, CRT modified the distribution of fat and played a critical role in determining cIMT. Leptin-to-adiponectin ratio, a biomarker of abdominal obesity and metabolic syndrome, and diastolic BP also influenced cIMT, a marker of subclinical atherosclerosis. Nonetheless, adiposity-associated vascular disease might be attenuated by SAT. Changes in body fat must be evaluated in this group of patients in the early course of survivorship in order to avoid premature cardiovascular disease associated with atherosclerosis. Yet, further research as regards the possible protective effect of SAT on vascular disease is warranted. Keywords: precursor cell lymphoblastic leukemia–lymphoma/radiotherapy, abdominal fat, metabolic syndrome X, adipokines, endothelium, atherosclerosi

Turner syndrome and metabolic derangements: Another example of fetal programming

Background and aim: Turner syndrome (TS) patients have an increased risk of weight gain and metabolic syndrome. To date, it is unknown what factors are involved in this metabolic process, even though it is recognized that TS patients are frequently born small-for-gestational age. The aim of this study was to evaluate the correlation between lipid and glucose profiles with being overweight and birth weight and length in TS patients. Study design: This was a cross-sectional study. Subjects and outcome measures: Serum glucose, insulin (HOMA-IR), total cholesterol, and triglycerides were measured in 64 patients with TS. Data regarding birth weight and length and current body mass index (BMI) were also evaluated. Results: Total cholesterol showed a significant negative correlation with birth weight and a positive correlation with BMI; triglycerides showed significant negative correlation with birth weight and length and a positive correlation with BMI; and HOMA-IR showed a significant negative correlation with birth weight and length. Low birth weight and a high BMI were predictive for 28% of total cholesterol and triglycerides; and low birth weight for 22% of HOMA-IR. Conclusions: Lipid profile was correlated with a high current BMI and low birth weight and length in TS patients and glucose profile only with low birth weight. Thus far, growth retardation may play a role in metabolic derangements in this group of patients, being considered another example of fetal programming. (C) 2011 Elsevier Ireland Ltd. All rights reserved.8829910

Growth hormone effect on body composition in Turner syndrome

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)This study analyzes the body composition of young adult women with Turner syndrome (TS) either treated or not treated with recombinant human growth hormone (rhGH) and compares them with a group of healthy women. Fifty-two non-treated TS patients (23.0 +/- A 5.8 years), 30 treated with rhGH (21.5 +/- A 1.5 years), and 133 healthy young adult women (22.9 +/- A 3.2 years) were evaluated regarding height (H) and weight, body mass index (BMI), brachial perimeter and tricipital cutaneous fold (fat and lean areas at the arm), sitting height (SRH = sitting height/H x 100), leg length (leg/H), waist and hip circumferences (waist/hip), and bioimpedance (percentages of water, lean mass, and fat mass). Age at start of rhGH therapy varied from 7.8 to 15.1 years (10.0 +/- A 1.3 years), duration of treatment from 2.8 to 8.2 years (3.7 +/- A 1.5 years), and the mean dose was 0.42 mg/kg/w (from 0.32 to 0.50 mg/kg/w). Body composition (except height) did not differ between TS groups, but there were differences when compared to the control group: weight and sitting height were lower in TS patients; and BMI, SHR, and leg/H were higher. There was an association between all groups with regards to BMI, waist, SHR, and leg/H, but not in percentage of fat mass. SHR was positively correlated with BMI, waist, hip, and percentage of fat mass. This sample of TS patients (with and without rhGH therapy) did not differ in BMI or body composition. However, there were differences between patients with TS patients and normal healthy women. Regardless of rhGH therapy, TS patients should be monitored, particularly for sitting height, SHR, leg length, leg/H, and waist/hip.403486491Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES

Effects of growth hormone on body proportions in Turner syndrome compared with non-treated patients and normal women

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Background: The majority of anthropometric assessments in Turner syndrome (TS) patients has focused on height. AIM: To analyze body proportions in young adult TS patients either treated or not treated with rhGH, and to compare them with a group of age-matched healthy women. Subjects and methods: Standing height, sitting height, weight, foot and leg lengths, arm span, head circumference, biliac and bi-acromial diameters were measured in 52 non-treated TS patients, 30 treated with rhGH and 133 healthy women. Results: Age at the start of rhGH therapy varied from 7.8 to 15.1 yr (10.0 +/- 1.3 yr), the duration of treatment from 2.8 to 8.2 yr (3.7 +/- 1.5 yr) and the mean recombinant human GH (rhGH) dose was 0.42 mg/kg/week (from 0.32 to 0.50 mg/kg/week). Non-treated patients did not show any difference in anthropometric variables when compared with the treated ones, except for hand length (p=0.02) and height (p=0.05), which were increased in the treated group. All anthropometric variables, except head circumference, were different when comparing TS patients (either treated or not) with age-matched healthy women. Conclusion: Brazilian TS patients either treated or not with rhGH showed almost no differences in terms of their body proportions. This result is probably due to the late age at the start of treatment, and/or the short period of rhGH administration. Hand length was different between the groups, showing the importance of including the extremities in body proportion assessment during rhGH treatment of TS patients. (J. Endocrinol. Invest. 33: 691-695, 2010) (C) 2010, Editrice Kurtis3310691695Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES

Fatores associados a atraso no diagnóstico da síndrome de Turner Investigación de factores asociados a retraso en el diagnóstico del síndrome de Turner Variables associated with diagnostic delay in Turner syndrome

OBJETIVO: Investigar as possíveis razões do atraso no diagnóstico da síndrome de Turner (ST), ou seja, aquele realizado após a idade em que se pode estabelecer o atraso puberal. MÉTODOS: Estudo transversal com obtenção de dados dos prontuários de 29 pacientes com ST diagnosticadas com mais de dois anos, entre 2004 e 2007. Foram comparados antecedentes pessoais e familiares e dados de exame físico das pacientes diagnosticadas com menos de 13 anos (limite a partir do qual se pode caracterizar atraso puberal em meninas) com os daquelas diagnosticadas após os 13 anos por meio dos testes t de Student e exato de Fisher. RESULTADOS: Não houve diferenças significativas quanto à estatura materna e da própria paciente, história de afecções associadas (consideradas individualmente), escolaridade dos pais, recorrência familiar de baixa estatura, presença de cada sinal dismórfico isoladamente e total de sinais observados. Os dois grupos diferiram quanto à presença de ao menos uma afecção sugestiva dessa síndrome (associada ao diagnóstico mais precoce) e ao número de irmãos (maior no diagnóstico tardio e associado à menor escolaridade materna). CONCLUSÕES: O diagnóstico precoce relacionou-se mais à presença de alguma das afecções associadas à ST (possivelmente determinando-se encaminhamento a serviços de maior complexidade) do que a sinais dismórficos. Há indicações de que déficit de crescimento menos evidente, dificuldade dos médicos em reconhecer anomalias sugestivas dessa síndrome e determinantes socioeconômicos contribuam para o atraso no diagnóstico. É necessário enfatizar na formação pediátrica o reconhecimento do espectro clínico dessa síndrome e ampliar os serviços públicos de genética.<br>OBJETIVO: Investigar las posibles razones del retraso en el diagnóstico del síndrome de Turner (ST), es decir, aquél realizado después de la edad en que se puede establecer retraso puberal. MÉTODOS: Estudio transversal con obtención de datos de los prontuarios de 29 pacientes diagnosticadas con ST mayores de 2 años de edad entre 2004 y 2007. Se compararon antecedentes personales y familiares y datos de examen físico de las pacientes diagnosticadas a una edad menor de 13 años (límite a partir del cual se puede caracterizar retraso puberal en niñas) con los de aquellas diagnosticadas después de los 13 años mediante las pruebas t y chi-cuadrado. RESULTADOS: No hubo diferencias significativas respecto a la estatura materna y de la paciente misma, historial de afecciones asociadas (consideradas individualmente), escolaridad de los padres; recurrencia familiar de baja estatura, presencia de cada señal dismórfica aislada y total de señales observadas. Los dos grupos difirieron respecto a la presencia de al menos una afección sugestiva de ese síndrome (asociada al diagnóstico más temprano) y al número de hermanos (mayor en el diagnóstico tardío y asociado a la menor escolaridad materna). CONCLUSIONES: El diagnóstico temprano se relacionó más a la presencia de alguna de las afecciones asociadas al ST (posiblemente determinando encaminamiento a servicios de mayor complejidad) que a señales dismórficas. Hay indicaciones de que déficit de crecimiento menos evidente, dificultades de los médicos en reconocer anomalías sugestivas de ese síndrome y factores socioeconómicos contribuyan para el retraso en el diagnóstico. Es necesario enfatizar en la formación pediátrica el reconocimiento del espectro clínico de ese síndrome y ampliar los servicios públicos de genética.<br>OBJECTIVE: To investigate the possible reasons for diagnostic delay in Turner syndrome (TS), i.e., a diagnosis made after the age when pubertal delay may be established. METHODS: Cross-sectional study with data obtained from the records of 29 TS patients aged more than two years who were diagnosed between 2004 and 2007. Data on personal and family history and physical examination from patients diagnosed before 13 years old (age limit from which pubertal delay may be characterized in girls) were compared to those of girls diagnosed after 13 years by Fisher exact test and Student's t-test. RESULTS: No significant differences were noted regarding mothers' and patients' stature, personal history of TS-associated diseases (considered individually), parental schooling, familial recurrence of short stature, presence of each dysmorphic feature considered separately, and total number of dysmorphic features. The two groups differed regarding the presence of at least one TS-associated disease (which was associated to early diagnosis) and number of siblings (which was higher among patients with delayed diagnosis and associated with lower maternal schooling). CONCLUSIONS: Early diagnosis was more associated with the presence of a TS-associated disease (which may have required referral to secondary or tertiary health care services) than with the presence of dysmorphic signs. The results indicate that less evident growth deficit, physicians' inability to recognize abnormalities associated with TS and socioeconomic aspects may contribute to diagnostic delay. Pediatric training should emphasize recognition of the clinical spectrum of TS and public genetic services should be expanded