Importance of ethical and psychological features in the intersex management

The treatment of intersex disorders has passed through different historical periods. The most important was the age of surgery, during which the dominant concept was that at birth the brain would be adaptable, psychosexually neutral and gender identity the result of educational and social processes. Thus, the preferred and systematic treatment was towards female assignment because from the surgical point of view it was technically more feasible the construction of a vagina than a penis with future normal function. During the 90 s various other aspects began to be considered, mainly ethical questions. It became evident the importance of prenatal exposition to androgens, the various environmental and cultural factors related to sexuality and more recently the recognition of the brain as an endocrine organ. It is now quite clear the existence of a wide spectrum of sexual identities as well as the different ways individuals conduct their social sexual behavior. Surgical procedures are still discussed, especially with the purpose to determine the best moment to be performed and in the context of some ethical aspects involved. Today the moment is more complex. The work is done with families by multidisciplinary teams in order to offer a more adequate psychosocial orientation to intersex patients, but all the steps on how to achieve those aims are not known yet.A história das condutas na intersexualidade passou por diferentes períodos ao longo do tempo, sendo o mais importante a era cirúrgica. A condição de ser homem ou mulher era considerada como não sendo inata, mas apreendida e sujeita a influências culturais e ambientais. Assim, a conduta preferencial e sistemática era pela criação no sexo feminino, posto que do ponto de vista cirúrgico, seria mais fácil construir uma vagina do que um pênis com funcionalidade sexual futura. Na década de 90, vários outros aspectos começaram a ser considerados, como as questões éticas, ficando evidente a importância da exposição fetal aos andrógenos, os fatores ambientais, culturais e, mais recentemente, do cérebro como órgão endócrino. Passou-se a tentar entender a existência de um amplo espectro entre as identidades masculina e feminina, assim como em relação à forma como o indivíduo conduz seu comportamento sexual na sociedade. Ainda discute-se o aspecto cirúrgico, no sentido de determinar qual o momento ideal para realizar os procedimentos necessários à adequação de acordo com o gênero, e quais os principais aspectos éticos envolvidos. Atualmente, vive-se em um momento mais complexo, onde, trabalhando em equipes multidisciplinares com as famílias, busca-se oferecer uma orientação psicossocial adequada para os pacientes com intersexualidade orgânica, mas ainda não se sabe, com segurança, como atingir esse objetivo.Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Setor de Endocrinologia Pediátrica e do AdolescenteUNIFESP, Depto. de Pediatria Setor de Endocrinologia Pediátrica e do AdolescenteSciEL

Interventions for preventing obesity in children

Universidade Federal de São Paulo, Escola Paulista Med, Dept Pediat, EPM Unifesp, São Paulo, BrazilAssoc Paulista Med, Dept Endocrinol, São Paulo, BrazilUniversidade Federal de São Paulo, Escola Paulista Med, Dept Pediat, EPM Unifesp, São Paulo, BrazilWeb of Scienc

Pediatric Endocrinology 2011

Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de PediatriaIrmandade da Santa Casa de Misericórdia de São Paulo Unidade de Endocrinologia Pediátrica Departamento de Pediatria e PuericulturaUniversidade Estadual de Campinas Unidade de Endocrinologia Pediátrica, Faculdade de Ciências Médica Departamento de PediatriaUNIFESP, EPM, Depto. de PediatriaSciEL

Adiposidade em pacientes tratados por câncer na infância: entendendo a fisiopatologia da obesidade

As childhood cancer treatment has become more effective, survival rates have improved, and a number of complications have been described while many of these patients reach adulthood. Obesity is a well-recognized late effect, and its metabolic effects may lead to cardiovascular disease. Currently, studies concerning overweight have focused on acute lymphocytic leukemia and brain tumors, since they are at risk for hypothalamic-pituitary axis damage secondary to cancer therapies (cranial irradiation, chemotherapy, and brain surgery) or to primary tumor location. Obesity and cancer have metabolic syndrome features in common. Thus, it remains controversial if overweight is a cause or consequence of cancer, and to date additional mechanisms involving adipose tissue and hypothalamic derangements have been considered, comprising premature adiposity rebound, hyperinsulinemia, leptin regulation, and the role of peroxisome proliferator-activated receptor γ. Overall, further research is still necessary to better understand the relationship between adipogenesis and hypothalamic control deregulation following cancer therapy.Os avanços do tratamento contra o câncer infantil têm resultado no aumento da sobrevida e das complicações, à medida que os pacientes atingem a maioridade. A obesidade é um evento reconhecido, e seus efeitos metabólicos levam à doença cardiovascular. Atualmente, o estudo da obesidade tem enfocado a leucemia linfocítica aguda e os tumores cerebrais, já que ambos têm risco para lesões hipotalâmicas, secundárias às terapias (irradiação cranial, quimioterapia, e cirurgia) ou à localização do tumor. Obesidade e câncer têm em comum fatores para síndrome metabólica. Entretanto, a relação de causa e efeito entre obesidade e câncer permanece controversa, sendo que são considerados outros mecanismos envolvendo o tecido adiposo e lesões hipotalâmicas, como o rebote precoce de adiposidade, hiperinsulinemia, regulação da leptina, e o papel do receptor ativado por proliferadores de peroxissoma γ. Concluindo, mais estudos são necessários para entender a relação entre adipogênese e descontrole hipotalâmico em sobreviventes de câncer.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Universidade Federal de São Paulo (UNIFESP) Divisão de Endocrinologia Pediátrica Departamento de PediatriaUniversidade Estadual de Campinas Faculdade de Ciências Médicas Divisão de Endocrinologia PediátricaUNIFESP, Divisão de Endocrinologia Pediátrica Depto. de PediatriaSciEL

Adiposidade em pacientes tratados por câncer na infância: entendendo a fisiopatologia da obesidade

As childhood cancer treatment has become more effective, survival rates have improved, and a number of complications have been described while many of these patients reach adulthood. Obesity is a well-recognized late effect, and its metabolic effects may lead to cardiovascular disease. Currently, studies concerning overweight have focused on acute lymphocytic leukemia and brain tumors, since they are at risk for hypothalamic-pituitary axis damage secondary to cancer therapies (cranial irradiation, chemotherapy, and brain surgery) or to primary tumor location. Obesity and cancer have metabolic syndrome features in common. Thus, it remains controversial if overweight is a cause or consequence of cancer, and to date additional mechanisms involving adipose tissue and hypothalamic derangements have been considered, comprising premature adiposity rebound, hyperinsulinemia, leptin regulation, and the role of peroxisome proliferator-activated receptor γ. Overall, further research is still necessary to better understand the relationship between adipogenesis and hypothalamic control deregulation following cancer therapy.Os avanços do tratamento contra o câncer infantil têm resultado no aumento da sobrevida e das complicações, à medida que os pacientes atingem a maioridade. A obesidade é um evento reconhecido, e seus efeitos metabólicos levam à doença cardiovascular. Atualmente, o estudo da obesidade tem enfocado a leucemia linfocítica aguda e os tumores cerebrais, já que ambos têm risco para lesões hipotalâmicas, secundárias às terapias (irradiação cranial, quimioterapia, e cirurgia) ou à localização do tumor. Obesidade e câncer têm em comum fatores para síndrome metabólica. Entretanto, a relação de causa e efeito entre obesidade e câncer permanece controversa, sendo que são considerados outros mecanismos envolvendo o tecido adiposo e lesões hipotalâmicas, como o rebote precoce de adiposidade, hiperinsulinemia, regulação da leptina, e o papel do receptor ativado por proliferadores de peroxissoma γ. Concluindo, mais estudos são necessários para entender a relação entre adipogênese e descontrole hipotalâmico em sobreviventes de câncer.190200Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP

Detection of metabolic syndrome features among childhood cancer survivors: A target to prevent disease

Along with the growing epidemic of obesity, the risk of atherosclerosis, cardiovascular disease morbidity, and mortality are increasing markedly. Several risk factors for cardiovascular disease, such as visceral obesity, glucose intolerance, arterial hypertension, and dyslipidemia commonly cluster together as a condition currently known as metabolic syndrome. Thus far, insulin resistance, and endothelial dysfunction are the primary events of the metabolic syndrome. Several groups have recommended clinical criteria for the diagnosis of metabolic syndrome in adults. Nonetheless, in what concerns children and adolescents, there are no unified definitions, and modified adult criteria have been suggested by many authors, despite major problems. Some pediatric disease states are at risk for premature cardiovascular disease, with clinical coronary events occurring very early in adult life. Survivors of specific pediatric cancer groups, particularly acute lymphocytic leukemia, central nervous system tumors, sarcomas, lymphomas, testicular cancer, and following bone marrow transplantation, may develop metabolic syndrome traits due to: hormonal deficiencies (growth hormone deficiency, thyroid dysfunction, and gonadal failure), drug or radiotherapy damage, endothelial impairment, physical inactivity, adipose tissue dysfunction, and/or drug-induced magnesium deficiency. In conclusion, some primary and secondary prevention remarks are proposed in order to reduce premature cardiovascular disease risk in this particular group of patients

Pediatric Endocrinology 2011

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The use of growth hormone to treat endocrine-metabolic disturbances in acquired immunodeficiency syndrome (Aids) patients

Acquired Immunodeficiency Syndrome (Aids) was initially related to HIV-associated wasting syndrome, and its metabolic disturbances to altered body composition. After Highly Active Antiretroviral Therapy (HAART) was started, malnutrition has declined and HIV-associated lipodystrophy syndrome has emerged as an important metabolic disorder. Aids is also characterized by hormonal disturbances, principally in growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The use of recombinant human GH (hrGH) was formerly indicated to treat wasting syndrome, in order to increase lean body mass. Even though the use of hrGH in lipodystrophy syndrome has been considered, the decrease in insulin sensitivity is a limitation for its use, which has not been officially approved yet. Diversity in therapeutic regimen is another limitation to its use in Aids patients. The present study has reviewed the main HIV-related endocrine-metabolic disorders as well as the use of hrGH in such conditions.As primeiras descrições da síndrome da imunodeficiência adquirida (Aids) associavam-se à síndrome de emaciamento, e os distúrbios metabólicos às alterações na composição corporal. Após a introdução da terapia anti-retroviral altamente ativa (HAART), houve declínio na desnutrição, e surge a lipodistrofia como importante distúrbio metabólico. A Aids também se caracteriza por distúrbios hormonais, principalmente no eixo hormônio de crescimento/fator de crescimento insulina-like tipo 1 (GH/IGF-1). O uso do GH recombinante humano (hrGH) foi inicialmente indicado na síndrome de emaciamento, a fim de aumentar a massa muscular. Embora também não existam dúvidas quanto aos efeitos do hrGH na lipodistrofia, a diminuição na sensibilidade à insulina limita o seu uso, o qual ainda não está oficialmente aprovado. A diversidade nos esquemas de tratamento é outro limitante do uso dessa medicação em pacientes com Aids. Esta revisão apresenta os principais distúrbios endócrino-metabólicos associados à Aids e ao uso do hrGH nessas condições.Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Serviço de Endocrinologia PediátricaUniversidade Estadual de Campinas Faculdade de Ciências Médicas Departamento de PediatriaUnicamp FCM Departamento de PediatriaUNIFESP, Depto. de Pediatria Serviço de Endocrinologia PediátricaSciEL

Short stature in chronic kidney disease: physiopathology and treatment with growth hormone

Growth failure is frequent and a clinically important issue in children with chronic kidney disease (CKD). Many factors contribute to impaired growth in these children, including abnormalities in the growth hormone (GH) - insulin-like growth factor 1 (IGF-1) axis, malnutrition, acidosis, renal bone disease and glucocorticoid associated treatment. The management of growth failure in children with CKD is complicated by the presence of other-disease related complications requiring medical intervention. Despite evidence of GH efficacy and safety in this population, this therapy is still underutilized. This review shows the impact, the causes and the treatment of growth failure in children with CKD.O atraso no crescimento é freqüente e grave em crianças com doença renal crônica (DRC). Vários fatores contribuem para o comprometimento do crescimento nestas crianças, incluindo as alterações no eixo hormônio de crescimento (GH) - insulin-like growth factor 1 (IGF-1), desnutrição, acidose, doença renal óssea e uso de corticóides. Em crianças com DRC, o tratamento do atraso no crescimento é difícil em virtude da presença de doenças associadas que necessitem de adequado tratamento médico. Apesar de as evidências a respeito da segurança e de a eficácia do GH nesta população, este tratamento ainda é pouco utilizado. Esta revisão mostra o impacto, as causas e o tratamento do atraso no crescimento em crianças com DRC.Universidade Estadual de Campinas Faculdade de Ciências Médicas Clínica MédicaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Programa de Pós-graduação do Curso de PediatriaUNIFESP-EPM Departamento de PediatriaUnicamp FCM Departamento de PediatriaUNIFESP, EPM, Programa de Pós-graduação do Curso de PediatriaUNIFESP, EPM Depto. de PediatriaSciEL

Cranial radiotherapy predisposes to abdominal adiposity in survivors of childhood acute lymphocytic leukemia

Background: Advances in treatment of acute lymphocytic leukemia increased the likelihood of developing late treatment-associated effects, such as abdominal adiposity, increasing the risk of cardiovascular disease in this population. Cranial radiotherapy is one of the factors that might be involved in this process. the aim of this study was to determine the effect of cranial radiotherapy on adiposity indexes in survivors of acute lymphocytic leukemia.Methods: A comparative cross-sectional study of 56 acute lymphocytic leukemia survivors, chronological age between 15 and 24 years, assigned into two groups according to the exposure to cranial radiotherapy (25 irradiated and 31 non-irradiated), assessed according to body fat (dual energy X-ray absorptiometry), computed tomography scan-derived abdominal adipose tissue, lipid profile, and insulin resistance.Results: Cranial radiotherapy increased body fat and abdominal adipose tissue and altered lipid panel. Yet, lipids showed no clinical relevance so far. There were significantly more obese patients among those who received cranial radiotherapy (52% irradiated versus 22.6% non-irradiated), based on dual energy X-ray absorptiometry body fat measurements. Nonetheless, no association was observed between cranial radiotherapy and body mass index, waist circumference, waist-to-height ratio or insulin resistance.Conclusions: Adolescent and young adult survivors of childhood acute lymphocytic leukemia showed an increase in body fat and an alteration of fat distribution, which were related to cranial radiotherapy. Fat compartment modifications possibly indicate a disease of adipose tissue, and cranial radiotherapy imports in this process.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Universidade Federal de São Paulo, Div Pediat Endocrinol, Dept Pediat, UNIFESP EPM, São Paulo, BrazilIOP GRAACC, Pediat Oncol Inst, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Prevent & Social Med, Div Biostat, UNIFESP EPM, São Paulo, BrazilUniv Estadual Campinas, Lab Invest Metab & Diabet LIMED, Fac Med Sci, UNICAMP, Campinas, SP, BrazilUniversidade Federal de São Paulo, Dept Diagnost Imaging, UNIFESP EPM, São Paulo, BrazilUniv Estadual Campinas, Dept Pediat, Div Pediat Endocrinol, Fac Med Sci,UNICAMP, Campinas, SP, BrazilUniversidade Federal de São Paulo, Div Pediat Endocrinol, Dept Pediat, UNIFESP EPM, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Prevent & Social Med, Div Biostat, UNIFESP EPM, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Diagnost Imaging, UNIFESP EPM, São Paulo, BrazilFAPESP: 06/06162-9Web of Scienc