Near-Adult Heights and Adult Height Predictions Using Automated and Conventional Greulich–Pyle Bone Age Determinations in Children with Chronic Endocrine Diseases

Objectives To validate adult height predictions (BX) using automated and Greulich–Pyle bone age determinations in children with chronic endocrine diseases. Methods Heights and near-adult heights were measured in 82 patients (48 females) with chronic endocrinopathies at the age of 10.45±2.12 y and at time of transition to adult care (17.98±3.02 y). Further, bone age (BA) was assessed using the conventional Greulich–Pyle (GP) method by three experts, and by BoneXpert™. PAH were calculated using conventional BP tables and BoneXpert™. Results The conventional and the automated BA determinations revealed a mean diference of 0.25±0.72 y (p=0.0027). The automated PAH by BoneXpert™ were 156.26 ± 0.86 cm (SDS ? 2.01 ± 1.07) in females and 171.75 ± 1.6 cm (SDS ? 1.29 ± 1.06) in males, compared to 153.95 ± 1.12 cm (SDS ? 2.56 ± 1.5) in females and 169.31 ± 1.6 cm (SDS?1.66±1.56) in males by conventional BP, respectively and in comparison to near-adult heights 156.38±5.84 cm (SDS?1.91±1.15) in females and 168.94±8.18 cm (SDS?1.72±1.22) in males, respectively. Conclusion BA ratings and adult height predictions by BoneXpert™ in children with chronic endocrinopathies abolish rater-dependent variability and enhance reproducibility of estimates thereby refning care in growth disorders. Conventional methods may outperform automated analyses in specifc cases.

Endocrine Disorders in a Specialized Hospital in Guyana

Background:Endocrine diseases are characterized by hormonal alterations (excess or defect). Due to the low prevalence (less than five case 5 / 10 000 inhabitants), a large number of them, qualify to be classified as rare diseases such as those of organs like: hypophysis, adrenal glands, gonads as well as some congenital thyroid diseases. Others like Diabetes are considering almost epidemic.Objective:To define the types of diseases observed in the only Endocrinology Clinic in Guyana. Methods:The diagnoses of all patients who attended the endocrinology clinic of the Georgetown Public Hospital Corporation from June 1, 2016 to May 31, 2017, were analyzed.Results:During the one year of this study, approximately 639 patients attended the endocrinology clinic. Of this, 178 patients had thyroid-related diseases with 80 of these having thyrotoxicosis, 49 having hypothyroidism followed by 110 patients with diabetes mellitus. Pituitary tumors were also diagnosed with 2 of acromegaly and 6 of prolactinomas. Cases of hypoadrenalism (n = 5), hypogonadism (n = 4), and pheochromocytoma (n = 6) were not rare; gonadal disease were also found in 17 patients. Thyroid disease wasthe most frequent diagnosis followed by diabetes mellitus. New emerging endocrine disorders such as hyperlipidemia (n = 1) were rare. Some persons attending the clinic were also noted to be overweight /obese however this was not the primary reason for joining the clinic. Traditional diseases such as Sheehan Syndrome have become rare due to improvements in Obstetric care.

Paying attention to the underlying inherited metabolic disorders and endocrine diseases for the cases of critical illness and sudden death / 中国实用儿科杂志

Sudden death syndrome is the leading cause of child death in high income countries. It affects neonates to adults. The seemly healthy person suddenly died during a daily activity,sleep or exercise. Underlying genetic disorders are main causes of sudden cardiac death or brain death. Sudden unexplained death syndrome was first noted in 1977 in the United States of America. In some countries such as the United States of America,England,Thailand and Japan,the etiological studies were performed in the cases died suddenly. Those studies showed that heart attack and encephalopathy due to varied genetic disorders are the two major causes. Sudden cardiac death accounts for more than half of the cases. Sudden death or sudden death-like syndrome,would be the first manifestation of underlying inherited metabolic disorders and endocrine disorders,such as primary carnitine deficiency,long QT syndrome,arrhythmia,hypomagnesemia,hypokalemia,hyperkalemia,hypocalcemia,hypoglycemia,mitochondrial diseases,etc. Inherited metabolic disorders and endocrine disorders include thousands of diseases,such as amino acids,organic acids,glucose,fatty acids and electrolytes metabolic disturbance. Some patients presented as acute critical illness and sudden death. Some disorders could be detected by newborn screening or selective screening using biochemical,electrophysiological,imaging,pathological or genetic techniques. The mortality and disability could be reduced by effective intervention of diet and medicine.

Steroid hormone profiling by LC-MS:the change in methodology powering precision medicine / 中华检验医学杂志

Steroid hormone profiling reflects the functions of endocrine system, offering precise information for the diagnostics and treatment of endocrine diseases. Liquid chromatography-mass spectrometry technique is highly specific and sensitive,and is superior to traditional immunoassay and has become important in the clinical detection of steroid hormones. It should apply this technique and promote its growth according to the clinical requirements and guidelines.

Neoplasmas da tireoide em cães: 26 casos / Thyroid neoplasms in dogs: 26 cases

Foram revisados os protocolos de biópsias e necropsias do Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV/UFSM) entre 2004 e 2014, e todos os casos de tumores que acometeram a glândula tireoide foram selecionados. A partir disso, foram anotados sexo, idade e raça dos cães afetados. Também foi avaliado o significado clínico dos tumores, presença de aumento de volume cervical, lobos afetados, tamanho dos tumores, presença e localização de metástases e padrão histológico dos neoplasmas. Nos 11 anos que compreenderam este estudo, foram diagnosticados 26 tumores de glândula tireoide (quatro eram provenientes de exames de biópsias e 22 de necropsias), 53,9% dos cães eram fêmeas e 46,1% eram machos; 65,4% eram cães com raça definida. Desses, Boxers foram os mais afetados (15,4% dos casos). Dos 23 protocolos em que a idade foi informada, 60,9% dos cães eram idosos e 39,1% eram adultos. A avaliação dos protocolos permitiu constatar que 38,5% dos animais haviam apresentado aumento de volume cervical no momento do exame clínico. Dos cães necropsiados 13,6% morreram ou foram submetidos à eutanásia em decorrência de complicações causadas por metástases dos tumores de tireoide. Dessa forma, em 86,4% dos cães, os neoplasmas foram considerados achados incidentais de necropsia. Nos quatro casos provenientes de material de biópsia, o desfecho não foi conhecido. Em nenhum cão os tumores causaram síndrome clínica devido à hipo ou hiperfunção da tireoide. Em 24 protocolos havia informação sobre os lobos afetados e 75% dos cães tiveram acometimento unilateral da glândula. Os diagnósticos histológicos dos tumores permitiram constatar que 16 (61,5%) neoplasmas eram carcinomas de células foliculares (nesses casos, o padrão folicular-compacto foi o mais frequente [43,75%]), seis (23,1%) eram adenomas de células foliculares, dois (7,7%) eram carcinossarcomas e dois (7,7%) eram tumores de células C (adenoma e carcinoma).(AU)

Biopsy and necropsy reports of the Laboratório de Patologia Veterinária at the Universidade Federal de Santa Maria (LPV/UFSM) were reviewed, including data from 2004 to 2014; all cases of the thyroid tumors were selected. From this point on, it was recorded the gender, age and breed of affected dogs. Additionally, it was evaluated the clinical significance of these tumors, presence of cervical swelling, affected lobes, tumors size, presence and location of metastasis and histological pattern of neoplasms. Twenty six thyroid tumors were diagnosed in the study period (four came from biopsies while the other 22 were from necropsies). Of the affected dogs, 53.8% were females and 46.2% were males; most of the animals (65.4%) were purebred. Of these, Boxers were the most affected (15.4%). In 23 protocols the age was informed; 60.9% of the dogs were elderly and 39.1% were adults. The protocols evaluation allowed establishing that 38.5% of the animals had cervical swelling, at the time of clinical examination. Out of the dogs necropsied, only 13.6% died or were euthanized due to complications caused by metastases of thyroid tumors. Thus, in 86.4% of the dogs, the neoplasms were considered as incidental necropsy findings. In the four cases from biopsy samples, the outcomes were not known. There were no tumors causing clinical syndrome in these dogs, due to thyroid hypo- or hyperfunction. In 24 protocols it was found information about the affected lobes, indicating that 75% of the dogs had unilateral glandular involvement. The histological diagnosis showed that 16 (61.5%) neoplasms were follicular cell carcinomas (in these cases, the follicular-compact pattern was the most frequent [43.8%]); 6 (23.1%) were follicular cells adenoma; two (7.7%) were carcinosarcomas and two (7.7%) were C cells tumors (adenoma and carcinoma).(AU)

Gammagrafía de receptores de somatostatina con -DOTATATE PET/CT: experiencia inicial en Cuba / Somatostatin receptor scintigraphy with -DOTATATE PET/CT: initial experience in Cuba

En 1870 Rudolf Heidenhain descubrió las células neuroendocrinas, las cuales dan origen a los Tumores Neuroendocrinos (TNE) que son una forma rara de cáncer, la mayoría de los cuales expresan receptores de somatostatina. El fundamento de la Gammagrafía de Receptores como imagen metabólico-molecular se fundamenta en el empleo de -DOTA-péptidos con enlazamiento específico a los receptores de somatostatina. La presente publicación tiene el propósito de dar a conocer nuestras primeras experiencias en el estudio de diferentes tipos histológicos de TNE por medio de la Gammagrafía de Receptores de Somatostatina (GRS) empleando el -DOTATATE PET/CT realizados en el Centro PET/CT e incluyendo Imagen Molecular del Departamento de Medicina Nuclear del Instituto Nacional de Oncología y Radiobiología (Inor)

In 1870, Rudolf Heidenhain discovered neuroendocrine cells, which can lead to the development of the Neuroendocrine Tumors (NER), a rare form of cancer, most of which express somatostatin receptors. The basis of Receptor Scintigraphy as a metabolic-molecular image is based on the use of -DOTA-peptides with specific binding to somatostatin receptors.The purpose of this publication is to present our first experiences in the study of different histological types of TNE by means of Somatostatin Receptor Scintigraphy (GRS) using -DOTATATE performed at the PET / CT Center and Molecular Image of the Department of Nuclear Medicine of the National Institute of Oncology and Radiobiology (Inor)

Uso de baixa dose de ACTH sintético no teste de estimulação da função adrenal para o diagnóstico e controle do hiperadrenocorticismo canino: avaliação da eficácia diagnóstica / Use of low-dose of synthetic ACTH in the adrenal stimulation test for diagnosis and monitoring of canine hyperadrenocorticism: evaluation of diagnostic efficacy

O teste de estimulação com ACTH é o teste de escolha para realizar o controle dos valores de cortisol endógeno em pacientes sob tratamento para o hiperadrenocorticismo canino, podendo ser utilizado também para diagnosticar a doença. Os protocolos atuais utilizam doses que variam entre 5ug/kg a 250ug/animal por administração intravenosa ou intramuscular. Não se constatam estudos com doses menores que as de 5ug/kg em pacientes portadores de hiperadrenocorticismo. No presente estudo, foi testada a dose de 1ug/kg/IV; comparada à dose consagrada de 5ug/kg/IV, em grupos de animais suspeitos de HAC (HAC Diag), animais portadores de HAC e em tratamento (HAC Control) e animais sadios (Sadios). Na dose de 1ug/kg/IV, os valores basais de cortisol dos Sadios foram iguais à média 2,40ug/dL(+/-1,57ug/dL), dos HAC control foi de média 1,53ug/dL(+/-0,93,ug/dL) e dos HAC diag foi média 3,37ug/dL(+/-1,57ug/dL). Os valores pós-ACTH na dose de 1ug/kg foram de média 11,43ug/dL(+/-2,46ug/dL) para animais sadios, 2,67ug/dL(+/-1,39ug/dL) para o grupo HAC Control e média 16,56ug/dL(+/-7,62ug/dL) para o grupo HAC Diag. Os valores basais de cortisol na dose de 5ug/kg foram 0,89ug/dL(+/-0,23ug/dL) para o grupo HAC Control; média 3,08ug/dL(+/-1,99 ug/dL) para o grupo HAC Diag. Os valores pós-ACTH na dose de 5ug/kg foram de média 3,71ug/dL(+/-1,57ug/dL), para o grupo HAC control e média 22,52ug/dL (+/-8,75ug/dL) para o grupo HAC diag. Analisando-se os resultados obtidos, constatou-se que as doses 1 e 5ug/kg de ACTH sintético não diferem entre si, promovendo o mesmo tipo de variação nos valores de cortisol (ANOVA; p=0,225). Também, que a dose de 1ug/kg de ACTH foi igualmente eficaz na elevação dos níveis de cortisol nos três grupo testados (Sadios, HAC Control e HAC Diag; ANOVA, p<0,05). E, pelo teste de Dunn observamos que o grupo HAC control apresenta Δ-cortisol (delta =diferença entre cortisol após estimulo e o cortisol basal) significativamente menor que o dos grupos diagnóstico (p<0,05) e animais sadios (p<0,05). Concluiu-se que a dose de 1ug/kg de ACTH sintético pode ser utilizada com eficácia para a realização do teste de estimulação com ACTH.(AU)

ACTH stimulation is the gold standard test to monitor levels of endogen control of patients under treatment for canine hyperadrenocorticism and it may also be used to diagnose the disease. Current protocols use doses ranging from 5ug/kg to 250ug per animal by intravenous or intramuscular administration. There are no studies with doses inferior to 5ug/kg in adrenopatic patients. In the present investigation, the dose of 1ug/kg/IV was tested; compared with the recommended dose of d 5ug/kg/IV in groups of dogs under clinical suspicion of HAC (HAC Diag), animals under treatment for HAC (HAC Control) and healthy animals (Healthy). Under the dose of 1ug/kg/IV, average results for baseline cortisol values were equal to 2.40ug/dL(+/- 1.57ug/dL) for healthy, 1.53g/dL(+/-0.93ug/dL) for HAC Control and 3.37ug/dL(+/-1.57ug/dL) for HAC Diag. Post-ACTH values in the dose of 1ug/kg were average 11.43ug/dL(+/-2.46ug/dL) for healthy animals, 2.67ug/dL (+/-1.39ug/dL) for HAC Control and 16.56ug/dL(+/-7.62ug/dL) for HAC Diag group. Basal cortisol values at a dose of 5ug/kg were 0.89ug/dL (+/-0.23ug/dL) Control group for HAC; average 3.08ug/dL (+/-1.99ug/dL) for HAC Diag group. Baseline cortisol under the dose of 5ug/kg were average 3.71ug/dL(+/-1.57ug/dL) for HAC Control and 22.52g/dL(+/-8.75ug/dL) for HAC diag. Based on the present results, it was found that both doses of 1 and 5ug/kg of synthetic ACTH do not differ, providing the same kind of change in cortisol values (ANOVA, p=0.225). Also, the dose of 1ug/kg of ACTH was equally effective in raising levels of cortisol in the three groups tested (Healthy, HAC and HAC Control Diag; ANOVA, p<0.05). Through the Dunn test it was observed that HAC Control presented HAC-Δ cortisol (delta = difference between cortisol after stimulation and basal cortisol) significantly lower than HAC Diag (p<0.05) and healthy animals (p<0.05). Therefore the dose of 1ug/kg of synthetic ACTH can be effectively used to perform the ACTH stimulation test effectively.(AU)

¿Paradojas en la atención a la sexualidad de personas con enfermedades endocrinas? / Paradox in the care of sexuality of people with endocrine diseases?

El impacto sexual provocado por las endocrinopatías se ha documentado en la literatura científica, pero se le brinda escasa atención desde los espacios de salud. Se propone reflexionar sobre algunos aspectos relacionados con esta realidad a partir del análisis de los procesos de atención de salud, de la investigación y de las políticas sociales, utilizando como perspectiva transversal la determinación y el carácter sociocultural e histórico de la sexualidad, la salud, la ciencia y la tecnología. El artículo se organiza en cuatro subacápites interrelacionados: 1) los campos del saber sobre la sexualidad de personas con endocrinopatías; 2) algunos determinantes de la invisibilización del tema en los espacios asistenciales (tales como la hegemonía de las especialidades biomédicas, las representaciones sociales respecto a las categorías cuerpo, enfermedad y sexualidad, la perspectiva médico-paciente, y la insuficiente capacitación de los proveedores de salud sobre estos temas); 3) el proceso de investigación y algunos de sus paradigmas fundamentales; y 4) la situación de las políticas y programas relacionados con la sexualidad de personas con endocrinopatías. Se concluye apostando por principios como la multi-inter-trans-disciplina, la participación social, la necesidad de integrar diferentes saberes y de flexibilizar los paradigmas científicos, y la apropiación social de los productos resultantes del proceso científico en cualquiera de sus expresiones, como bases para visualizar y prestar atención a temas de salud como la sexualidad de personas con endocrinopatías(AU)

Sexual impact caused by endocrinopathies has been documented in the scientific literature, but little attention is paid in the health circles. The article was intended to make reflections on some aspects of these realities, on the basis of the analysis of processes of health care, research and social policies, using determination and sociocultural and historical character of sexuality, health, science and technology as cross perspective. The article was organized in four interrelated subsections: 1) fields of knowledge about sexuality of people with endocrinopathies; 2) some determinants of the invisibility of the topic in assistance sites (such as hegemony of biomedical specialties, social representations in respect to the categories body, disease and sexuality; the physician-patient perspective and the poor training of health providers on these topics); 3) the research process and some of its fundamental paradigms; and 4) the situation of the policies and programs related to sexuality of people with endocrinopathies. The article was concluded by committing to the principles of multi-inter-transdiscipline, social involvement, need of integrating different know-how and of making scientific paradigms more flexible, and the social appropriation of resulting products of the scientific process in any expression, as foundations to visualize and to pay attention to health topics like sexuality of people with endocrinopathies(AU)

Alterações Ósseas e do Sistema Estomatognático associadas a Doenças Endócrino Metabólicas / Cambios en los huesos y del sistema estomatognático asociadas con las enfermedades endocrino-metabólicas / Diseases Bone Changes and changes in the Stomatognathic System Associated with Metabolic Endocrine Diseases

Introdução: a prevalência de doenças endócrino-metabólicas tem aumentado nos últimos anos com a melhoria da expectativa de vida da população. Diabete Melittus, hipotireoidismo, hipertireoidismo, hipoparatireoidismo e hiperparatireoidismo podem levar as alterações no sistema estomatognático, contudo, a literatura ainda é limitada nesta área. Objetivo: Abordar as características e o processo fisiopatológico de doenças endócrino-metabólicas, com destaque para as alterações ósseas e do sistema estomatognático. Metodologia: Os dados foram coletados através da seleção de artigos sobre alterações ósseas e do sistema estomatognático associadas ao hipotireoidismo, hipertireoidismo, hipoparatireoidismo, hiperparatireoidismo e diabete mellitus, publicados nos bancos de dados, Bireme, MEDLINE e Scielo. Resultados: Foram coletados 73 artigos publicados na literatura entre os anos 1995 e 2012, dos quais 40 foram selecionados por se enquadrar no tema proposto. A literatura consultada demonstrou que alterações dos níveis séricos dos hormônios tireoidianos e do paratormônio, assim como diabete mellitus, influenciam no metabolismo ósseo. Atrasos na esfoliação e erupção dentária, no crescimento e desenvolvimento ósseo são observados no hipotireoidismo, enquanto o hipertireoidismo apresenta densidade óssea mineral diminuída, osteoporose dos ossos maxilares, esfoliação acelerada dos dentes decíduos e erupção prematura dos permanentes. No hipoparatireoidismo, observa-se osteoesclerose, dentição hipoplásica e reduzida remodelação óssea. Intensa atividade osteoclástica, ausência de lâmina dura e tumor marrom podem estar presentes no hiperparatireoidismo. Diabete mellitus pode ocasionar oesteoporose, doença periodontal e hipoplasia de esmalte. Embora conhecidas, a magnitude e as causas destas alterações no sistema estomatognático ainda são pouco elucidadas. Conclusões: É imprescindível que o cirurgião dentista tenha conhecimento sobre as manifestações locais ou sistêmicas associadas a estas desordens endócrino-metabólicas que podem acometer os pacientes, para que possa reconhecê-las e contribuir com o seu diagnóstico e tratamento precoces(AU)

Introducción: La prevalencia de las enfermedades endocrino-metabólicas se ha incrementado en los últimos años por el incremento de la esperanza de vida de la población. La diabetes mellitus, hipotiroidismo, hipertiroidismo, hipoparatiroidismo e hiperparatiroidismo pueden presentar alteraciones en el sistema estomatognático, sin embargo, los estudios encontrados en la literatura son todavía limitados en esta área. Objetivo: abordar las características y procesos fisiopatológicos de estas enfermedades y los cambios especialmente en los huesos y del sistema estomatognático. Métodos: los datos fueron recolectados a través de la selección de artículos sobre las alteraciones óseas del sistema estomatognático en pacientes afectados con el hipotiroidismo, el hipertiroidismo, el hipoparatiroidismo, el hiperparatiroidismo y la diabetes mellitus, publicada en las bases de datos, BIREME, SciELO y MEDLINE. Resultados: se recolectaron 73 artículos publicados entre los años 1995 y 2012, de los cuales 40 fueron seleccionados por la relación con el tema de interés para nuestro propósito. La literatura ha demostrado que los cambios en los niveles séricos de la hormona tiroidea y paratiroidea, y la diabetes mellitus afectan el metabolismo óseo. En el hipotiroidismo se observan retrasos en la exfoliación y erupción de los dientes, en el crecimiento y desarrollo óseo. El hipertiroidismo, hay disminución de la densidad mineral ósea, osteoporosis de los huesos de la mandíbula, exfoliación acelerada de los dientes temporales y erupción prematura de los permanentes. En el hipoparatiroidismo, se observan osteosclerosis, dientes hipoplásicos, y remodelación ósea reducida. Puede estar presente intensa actividad de los osteoclastos, ausencia de la lámina dura y tumor pardo en el hiperparatiroidismo. La diabete mellitus puede causar oesteoporosis, enfermedad periodontal e hipoplasia del esmalte. Aunque conocidas, la magnitud y las causas de estos cambios en el sistema estomatognático aún no están claros.Conclusiones: es esencial que el dentista tenga conocimiento de las manifestaciones locales o sistémicas asociadas con estos trastornos endocrinos y metabólicos para que pueda reconocerlos y ayudar en el diagnóstico precoz y el tratamiento(AU)

Introduction: the prevalence of endocrine-metabolic diseases has increased in recent years due to improvement of the life expectancy of the population. Diabetes mellitus, hypothyroidism, hyperthyroidism, hypoparathyroidism and hyperparathyroidism may present alterations in the stomatognathic system; however, some studies found in literature are still limited in this area. Objective: to address the characteristics and pathophysiological process of these diseases, especially bone changes and in the stomatognathic system. Methods: data were collected through the selection of articles about bone alterations of the stomatognathic system associated with hypothyroidism, hyperthyroidism, hypoparathyroidism, hyperparathyroidism, and diabetes, published in databases, BIREME, MEDLINE and SciELO. Results: seventy three articles published between 1995 y 2012 were revised, and 40 were selected on the basis of their association with the topic of interest. It has shown that changes in serum levels of thyroid and parathyroid hormones, and diabetes mellitus have an impact on bone metabolism. Delays in tooth eruption and exfoliation, bone growth and development are observed in hypothyroidism, whereas decreased bone mineral density, jawbones osteoporosis, accelerated exfoliation of deciduous teeth and premature eruption of permanent teeth were observed in hyperthyroidism. In hypoparathyroidism, there were osteosclerosis, hypoplastic teeth, and reduced bone remodeling. Intense osteoclastic activity, brown tumors and the absence of lamina dura in hyperparathyroidism may be observed. Diabetes mellitus can cause osteoporosis, periodontal disease and enamel hypoplasia. Although they are known, the magnitude and causes of these changes in the stomatognathic system are still unclear. Conclusions: it is essential that dentist can recognize the local or systemic manifestations associated with these endocrine and metabolic disorders and help to early diagnosis and treatment(AU)

Astrocitoma pilocítico da região selar/suprasselar determinando manifestações endócrinas / Pilocytic astrocytoma of sellar/suprasellar region determining endocrine manifestations

Pilocytic astrocytoma (PA) is a grade I glial neoplasm arising mainly in the cerebellum of children. Herein, the authors report a case of PA in a 21 year-old male patient, who presented headache, vomiting and delayed pubertal development. Serum level of cortisol and testosterone corresponded to 32.8 ug/dl and 0.19 ng/ml, respectively. The computed tomography/magnetic resonance (CT/RM) imaging showed an expansive process compromising suprasellar/hypothalamic region and determining hydrocephalus. The patient underwent resection of the process. Histological evaluation revealed a glial neoplasm constituted by loose glial tissue, small microcysts, areas of dense piloid tissue and Rosenthal fibers. The neoplastic cells were immunoreactive for glial fibrillary acidic protein (GFAP) and negative for chromogranin and synaptophysin. The diagnosis of PA was then established.

O astrocitoma pilocítico (AP) é uma neoplasia glial grau I encontrada principalmente no cerebelo de crianças. Os autores relatam um caso de AP em paciente masculino, 21 anos de idade, que apresenta cefaleia, vômitos e retardo do desenvolvimento puberal. Os níveis séricos de cortisol e testesterona corresponderam a 32,8 ug/dl e 0,19 ng/ml. A tomografia computadorizada/ressonância magnética (TC/RM) identificaram um processo expansivo que comprometia a região suprasselar/hipotalâmica e determinava hidrocefalia.O paciente foi submetido à ressecção do processo. À microscopia, foi identificada uma neoplasia glial constituída por tecido glial frouxo, pequenos microcistos, áreas de tecido piloide denso e fibras de Rosenthal. As células neoplásicas foram imunopositivas para glial fibrillary acidic protein (GFAP) e negativas para cromogranina e sinaptofisina. O diagnóstico de AP foi, então, estabelecido.

Progress in research of endocrine abnormalities in Turner syndrome / 临床儿科杂志

Turner syndrome (TS) is the result of complete or partial deletion of an X chromosome. It is often associated with endocrine dysfunction such as thyroid dysfunction, growth and developmental disorders, glucose and lipid metabolism disor-ders, and so on. Long-term follow-up of children with TS should regularly monitor thyroid autoantibodies, thyroid function, blood glucose and blood lipids for early detection and intervention of the complications, in order to further improve the life quality of children with TS.

131I-MIBG y tumores neuroendocrinos / 131I-MIBG and neuroendocrine tumours

Los tumores neuroendocrinos constituyen un amplio grupo de neoplasias que tienen su origen en diversos tejidos estrechamente ligados por su origen embriológico común a la cresta neural. Estos tumores tienen la capacidad de sintetizar péptidos neurotransmisores y hormonas, además de almacenar catecolaminas. Algunos expresan receptores de somatostatina en sus membranas, lo cual ha permitido que la medicina nuclear pueda participar en el diagnóstico, tratamiento y seguimiento de estas. Dichos tumores por tener su origen en diferentes y variados tipos de tejidos presentan una gran diversidad de signos y síntomas que son diferentes para cada uno de ellos. Estos signos y síntomas dependen fundamentalmente de sus características bioquímicas, dadas por las sustancias que segregan; por su localización, por tanto, del sitio de aparición del tumor, la infiltración local y las posibles metástasis a distancia provocadas por él. El diagnóstico de los tumores neuroendocrinos se realiza por medio de imágenes de medicina nuclear, las cuales se obtienen por diferentes técnicas y radiofármacos como el ácido-dimercapto-succínico (99mTc-DMSA(V)), la metoxi-iso-butil-isonitrilo (99mTc-MIBI), la meta-iodo-bencil-guanidina marcada con 131l o 123l (131l -MIBG o 123l-MIBG), el octreotido marcado con 111l n, la tomografía de emisión de positrones, empleando análogos de somatostatina marcados con 68Ga, así como anticuerpos monoclonales anti antígeno carcinoembrionario. Para el tratamiento de estos tumores en medicina nuclear se emplean fundamentalmente los análogos de somatostatina marcados con 177Lu o con 90Y. El presente trabajo tiene como objetivo mostrar nuestra experiencia en la utilidad de la 131l -MIBG en el diagnóstico y tratamiento de los tumores neuroendocrinos.

Neuroendocrine tumours are neoplasms that arise from various tissues closely linked to the neural crest by their common embryological origin. These tumours have the ability to synthesize neurotransmitter peptides and hormones, as well as to store catecholamines. Some of these tumours express somatostatin receptors at their membranes, what have allowed nuclear medicine to be involved in their diagnosis, treatment and monitoring. Since they arise from different and varied types of tissues, these tumours have a wide range of signs and symptoms different for every one of them. These signs and symptoms mainly depend on their biochemical characteristics, given by the substances they secrete, as well as by their location, and consequently, they also depend on the place where the tumour appears, its local infiltration, and potential long-distance metastasis resulting from the tumour). Neuroendocrine tumours are diagnosed by means of nuclear medicine images, which are obtained by using different techniques and radiopharmaceuticals such as 99mTc dimercaptosuccinic acid (DMSA(V)), 99Tc-methoxy-isobutyl-isonitrile (MIBI), metaiodobenzylguanidine (MIBG) labelled with 131l or 123l (131l -MIBG or 123l-MIBG), 111 pipe n-labelled octreotide, positron emission tomography, using 68Ga-labelled somatostatin analogues and carcinoembryonic antigen monoclonal antibodies. Nuclear medicine uses mainly somatostatin analogues labelled with 90Y or 177 Lu for the treatment of these tumours. This paper is aimed at showing our experience in the use of 131-MIBG for the diagnosis and treatment of neuroendocrine tumours.

Grado de conocimiento de la Diabetes Mellitus en la población de barrio "Quijarro" en la provincia Cercado del Departamento de Cochabamba / Degree of Knowledge of Diabetes Mellitus in the Town of District "Quijarro" in the Cercado Province in the Cochabamba Department

La diabetes Mellitus (DM) es una enfermedad crónica, comprende un grupo de trastornos metabólicos frecuentes, además comparten el fenotipo de hiperglucemia. Hay varios tipos de DM con interacción genética y ambiental. Generalmente se encuentra afectada la población con un desequilibrio en el consumo de glucosa, carbohidratos, etc. Se realizó un estudio sobre el conocimiento de Diabetes Mellitus en el Barrio de "Quijarro" en la provincia Cercado de la ciudad de Cochabamba, durante los meses de Agosto a Diciembre del 2010 para evaluar los conocimientos de la población sobre la Diabetes y su prevención. El estudio es prospectivo, transversal y descriptivo. Se recolectó la información a través de Test, con preguntas directas acerca de los cuidados y los conocimientos que se tenía sobre Diabetes Mellitus. El resultado obtenido fue que el 25% de los entrevistados practica una vida saludable, y que solo el 13% tiene los conocimientos suficientes sobre la enfermedad de la diabetes. Por lo tanto, al observar los mínimos porcentajes, la mayoría de los habitantes se encuentran en riesgo de padecer Diabetes debido a la inadecuada información sobre esta.

Diabetes Mellitus (DM) is a chronic disease, it comprises a group of common metabolic disorders, they also share the phenotype of hyperglycemia. There are several types of DM with genetic and environmental interaction. Generally the population is affected with an imbalance in glucose uptake and carbohydrate, etc. Objectives of a study was conducted on knowledge of Diabetes Mellitus was conducted in the "Quijarro" neighborhood in the Cercado province in the city of Cochabamba, during the months of August to December 2010 to assess people's knowledge about diabetes and its prevention. The study is a prospective and descriptive transversal study. We collected information through test, with direct questions about the care and knowledge they possess. The result was that 25% practice a healthy life, and that only 13% have sufficient knowledge about the disease of diabetes. Therefore, by observing the mínimum percentages, the majority of people are at risk for diabetes due to inadequate information about it.

Avaliação dos principais efeitos endócrinos tardios em crianças e adolescentes sobreviventes ao tratamento de neoplasias malignas / Evaluation of the main late endocrine effects in children and adolescents after treatment of malignancies

OBJETIVO: Relatar os efeitos endócrinos tardios em crianças e adolescentes após tratamento oncológico e associá-los à doença de base e ao tratamento. SUJEITOS E MÉTODOS: Foram realizadas avaliações clínicas e laboratoriais visando à detecção de distúrbios endócrinos em 320 pacientes após terapia oncológica, seguidos por seis anos. RESULTADOS: Em 94 pacientes, detectaram-se: 32 pacientes apresentaram baixa estatura (nove em tratamento com hormônio de crescimento), 14 tiveram puberdade precoce (10 em uso de análogo de GnRH) e 19 revelaram ser portadores de distúrbios de tireoide (12 com hipotireoidismo, seis com nódulos de tireoide e um com tireoidite linfocitária crônica). Obesidade foi encontrada em 18 deles. Seis com diabetes insípido e cinco com puberdade atrasada, três com pan-hipopituitarismo. Houve associação entre a radioterapia e a presença de endocrinopatias. CONCLUSÃO: Noventa e quatro de 320 (30 por cento) dos pacientes fora de terapia apresentaram alteração endócrina, o que enfatiza a importância do seguimento precoce e regular, possibilitando-lhes, com tratamento, melhor qualidade de vida.

OBJECTIVE: To report the main endocrine effects after cancer treatment in children and adolescents and associate them to the disease and its treatment. SUBJECTS AND METHODS: Clinical and lab evaluation for endocrinopathy was performed in 320 patients after cancer therapy have been followed for six years. RESULTS: The most prevalent endocrine late effects in patients were: 32 patients had short stature, nine of them were under growth hormone therapy. Precocious puberty was found in 14 patients, 10 of them received GnRH analog. Thyroid diseases were present in 19 patients (12 with hypothyroidism; six with thyroid nodules/cysts; one with chronic lymphocytic thyroidytis). Obesity was found in 18 patients. Six presented insipidus diabetes, five delayed puberty and three panhypopituitarism. Radiation was associated with the appearance of the aforementioned endocrinopathies. CONCLUSION: Ninety four of 320 (30 percent) patients presented endocrine late effects which emphasize the importance for these patients to be regularly followed-up in order to precociously diagnose endocrine late effects and provide them a better quality of life.

Molecular Genetic Diagnosis of Genetic Endocrine Diseases

Many endocrine disorders have a genetic component. The genetic component is the major etiologic factor in monogenic disorders, while multiple genes in conjunction with environmental and lifestyle factors contribute to the pathogenesis in complex disorders. The development of the molecular basis of inherited endocrine diseases has undergone a dramatic evolution during the last two decades. The application of molecular technology allowed us to increase our understanding of endocrine diseases, and to impact on the practice of pediatric endocrinology related to diagnosis and genetic counseling. Identification of the mutation in the particular disease by genetic testing leads to precise diagnosis in the equivocal cases and prenatal diagnosis. However, clinicians should be cautious about determining therapeutic decisions solely on the basis of molecular studies, especially in the area of prenatal diagnosis and termination of pregnancy. This review describes an introduction to molecular basis of various inherited endocrine diseases and diagnosis by genetic testing.

Manifestaciones psiquiátricas en enfermedades endocrinas / Psychiatric manifestations in endocrine diseases

Se estudiaron 36 pacientes que asistieron a la consulta externa de Endocrinología del Instituto Superior de Medicina Militar "Dr. Luis Díaz Soto", después de haber sido tratados por especialistas en Psiquiatría o Psicología sin resultados favorables para su salud. El interrogatorio, examen físico e investigaciones de laboratorio señalaron que estos pacientes sufrían enfermedades endocrinometabólicas con predominio de afecciones tiroideas, presentes en 20 enfermos (86 %). Las manifestaciones psiquiátricas más frecuentes fueron: ansiedad, depresión, nerviosismo, astenia e irritabilidad. Todos los pacientes se controlaron cuando se les trató su enfermedad endocrina de base. El 92 % de ellos se diagnosticaron correctamente después de llevar tratamiento psiquiátrico por más de 1 año, con la consiguiente demora en la recuperación de la salud, gastos innecesarios de medicamentos y pérdidas laborales. Se evidenció la necesidad de profundizar en el diagnóstico durante la fase inicial de la enfermedad para ofrecer una adecuada atención médica.

A study was carried out in 36 patients assisting to the outpatients Endocrinology department at "Dr. Luis Díaz Soto" Military Medicine Superior Institute, after being treated by Psychiatry or Psychology specialists, without favorable outcomes for their health. The interrogatory, physical examination, and laboratory research, indicated that these patients suffered from endocrine-metabolic diseases, mainly thyroid affections, occurring in 20 patients (86 %). The more frequent psychiatric manifestations were: anxiety, depression, nervousness, asthenia, and irritability. All patients controlled themselvs when their basic endocrine disease was treated. Ninety two per cent of them were correctly diagnosed after having a psychiatric treatment for more than a year, with the consequent delay in health recuperation, innecessary expenses in drugs, and working losses. The need of going deeper into the diagnosis during the initial phase of the disease, to offer an adequate medical care, was evident.