Primary adipsia and severe hypernatremia in a Pit Bull dog / Adipsia primária e hipernatremia severa em um cão Pit Bull

Primary adipsia is a rare condition in which there is failure in the activation threshold of the hypothalamic osmoreceptors, leading to osmolality imbalance. Here, we reported the case of a Pit Bull dog with an altered level of consciousness (started after weaning) and adipsia. There was an increase in plasma osmolality (444 mOsm/kg), sodium (223.7 mg/dL), and chlorine (173 mg/dL) levels. Based on the suspicion of primary adipsia, water was administered via a nasogastric tube, with clinical improvement. The owner was instructed to supply water with food. Eight months after discharge, the dog returned with parvovirus and died. In the anatomopathological examination, no structural changes were observed in the central nervous system. To our knowledge, this is the first report of hypernatremia due to adipsia in a Pit Bull dog, showing that this is a differential diagnosis that should be considered in this breed.

Adipsia primária é uma rara condição em que há falha no limiar de ativação dos osmoreceptores hipotalâmicos, levando ao desequilíbrio da osmolalidade. Este artigo tem como objetivo relatar o caso de um cão da raça Pit Bull apresentando alteração no nível de consciência (iniciado após o desmame) e adipsia. Foi verificado aumento da osmolalidade plasmática (444 mOsm/kg), sódio (223,7 mg/dL) e cloro (173 mg/dL). Baseado na suspeita de adipsia primária, iniciou-se administração de água via sonda nasogástrica, com melhora clínica. O tutor foi orientado a fornecer água junto a alimentação. Oito meses após alta, o paciente retornou com parvovirose e veio a óbito. No exame anatomopatológico, não foram observadas alterações estruturais no sistema nervoso central. Este é o primeiro relato de hipernatremia por adipsia em um cão Pit Bull, mostrando que este é um diagnóstico diferencial que deve ser considerado nesta raça.

Síndrome hipotalámico como presentación inusual de recaída en el sistema nervioso central de leucemia linfoblástica aguda en niños: reporte de 4 casos / Hypothalamic syndrome as an unusual presentation of CNS relapse of acute lymphoblastic leukemia in children: report of 4 cases

La leucemia linfoblástica aguda (LLA) es la patología oncológica más frecuente en pediatría, y corresponde al 23% de las neoplasias en menores de 15 años. Alrededor del 20% de los pacientes con LLA presentan recaídas, en la mayoría de los casos, en la médula ósea. Las recaídas extramedulares son inusuales y las dos localizaciones más frecuentes son el sistema nervioso central (SNC) y los testículos. Cuando las recaídas ocurren en el SNC, suelen manifestarse con un síndrome meníngeo. El síndrome hipotalámico se define como la presencia de hiperfagia, obesidad y/o cambios en el estado de ánimo, y es una forma de presentación clínica inusual de las recaídas en el SNC y debe alertar al pediatra para mantener un alto índice de sospecha.Se describen cuatro casos que se presentaron con síndrome hipotalámico al momento de desarrollar una recaída de LLA en el SNC

Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, corresponding to 23% of cancer in children younger than 15 years old. About 20% of ALL cases will relapse, commonly in the bone marrow. Extramedullar relapses are unusual, and the two most frequent locations are CNS and testicles. ALL relapses, when diagnosed in the CNS, frequently present with clinical features of a meningeal syndrome. The hypothalamic syndrome, consisting of hyperphagia, obesity and / or behavior disturbances, corresponds to an unusual presentation of relapses in this location and should alert pediatricians to suspect it.We describe 4 ALL cases of hypothalamic syndrome at the time of CNS relapse

Síndrome diencefálico en un niño con desnutrición crónica e hiperactividad / Diencephalic syndrome in a child with chronic malnutrition and hyperactivity

El síndrome diencefálico es una causa infrecuente de desnutrición. Se produce por la disfunción del hipotálamo y está asociado a tumores del encéfalo. Los pacientes presentan una grave y progresiva pérdida de peso, aunque el apetito y la ingesta calórica son, por lo general, adecuados. Característicamente, los síntomas neurológicos son tardíos, lo que retrasa la sospecha diagnóstica. Se presenta a un paciente de 2 años y 6 meses de edad con desnutrición crónica grado II, derivado con diagnóstico presuntivo de enfermedad celíaca con mala adherencia y fracaso del tratamiento. Durante la internación, se arribó al diagnóstico de síndrome diencefálico secundario a un astrocitoma pilocítico grado I.

Diencephalic syndrome is an infrequent cause of malnutrition. It is produced by a malfunctioning hypothalamus, and it is related to encephalic tumors. Patients present a serious and progressive weight loss although the appetite and calorie intake are, usually, adequate. Neurological symptoms typically have a late appearance, delaying diagnostic suspicion. We present a patient aged 2 years and a half with grade II chronic malnutrition, referred with presumptive diagnosis of celiac disease, with poor adherence and treatment failure. During hospitalization, diagnosis of diencephalic syndrome secondary to grade I pilocytic astrocytoma was reached.

Propentofylline decreases hypothalamic astrogliosis induced by hypercaloric diet in the rat / A propentofilina diminui a astrogliose hipotalâmica induzida pela dieta hipercalórica no rato

ABSTRACT Obesity is associated with a chronic and low-grade inflammatory response in the hypothalamus, where astrogliosis occurs with the upregulation of the astrocyte structural protein GFAP. As propentofylline (PPF) has inhibitory effects on astrocyte and microglial activation during inflammation, this study aimed to investigate if this xanthine derivative could decrease the astrocyte reaction induced by a hypercaloric diet (HD). Male Wistar rats were divided into four groups: NDS - rats receiving a normocaloric diet (ND) and daily saline solution; NDP - rats receiving ND and daily PPF (12.5 mg/kg/day, intraperitoneal route); HDS - rats receiving HD and saline solution, HDP - rats receiving HD and PPF. On the 21st day, rats were anesthetized, and perfused, and brains were collected for GFAP immunohistochemical study in the hypothalamus. Results showed that HD induced increased weight gain and hypothalamic astrogliosis. Propentofylline decreased the expression of GFAP in the HDP group, although it did not affect the weight gain induced by this diet.

RESUMO A obesidade está associada com uma resposta inflamatória crônica e de baixo grau no hipotálamo, onde ocorre astrogliose com a superexpressão da proteína astrocitária GFAP. Como a propentofilina (PPF) possui efeitos inibitórios sobre a ativação astrocitária e microglial durante a inflamação, este estudo visou a investigar se esta xantina podia diminuir a reação astrocitária induzida pela dieta hipercalórica (HD). Ratos Wistar machos foram divididos em 4 grupos: NDS- ratos recebendo dieta normocalórica (ND) e solução salina diária; NDP- ratos recebendo ND e PPF diária (12.5 mg/kg/dia, via intraperitoneal); HDS- ratos recebendo HD e solução salina, HDP- ratos recebendo HD e PPF. No 21° dia, os ratos foram perfundidos e os encéfalos, coletados para estudo imuno-histoquímico para a GFAP no hipotálamo. Os resultados mostram que a HD induziu aumento do ganho de peso e astrogliose no hipotálamo. A PPF diminuiu a expressão de GFAP no grupo HD, embora não tenha afetado o ganho de peso induzido por esta dieta.

Duodenal-jejunal bypass normalizes pancreatic islet proliferation rate and function but not hepatic steatosis in hypothalamic obese rats

Modifications in life-style and/or pharmacotherapies contribute to weight loss and ameliorate the metabolic profile of diet-induced obese humans and rodents. Since these strategies fail to treat hypothalamic obesity, we have assessed the possible mechanisms by which duodenal-jejunal bypass (DJB) surgery regulates hepatic lipid metabolism and the morphophysiology of pancreatic islets, in hypothalamic obese (HyO) rats. During the first 5 days of life, male Wistar rats received subcutaneous injections of monosodium glutamate (4 g/kg body weight, HyO group), or saline (CTL). At 90 days of age, HyO rats were randomly subjected to DJB (HyO DJB group) or sham surgery (HyO Sham group). HyO Sham rats were morbidly obese, insulin resistant, hypertriglyceridemic and displayed higher serum concentrations of non-esterified fatty acids (NEFA) and hepatic triglyceride (TG). These effects were associated with higher expressions of the lipogenic genes and fatty acid synthase (FASN) protein content in the liver. Furthermore, hepatic genes involved in β-oxidation and TG export were down-regulated in HyO rats. In addition, these rats exhibited hyperinsulinemia, β-cell hypersecretion, a higher percentage of islets and β-cell area/pancreas section, and enhanced nuclear content of Ki67 protein in islet-cells. At 2 months after DJB surgery, serum concentrations of TG and NEFA, but not hepatic TG accumulation and gene and protein expressions, were normalized in HyO rats. Insulin release and Ki67 positive cells were also normalized in HyO DJB islets. In conclusion, DJB decreased islet-cell proliferation, normalized insulinemia, and ameliorated insulin sensitivity and plasma lipid profile, independently of changes in hepatic metabolism.

Hamartomas hipotalámicos: distintas formas de debut. Casos clínicos / Hypothalamyc hamartomas: different ways of clinical debut. Cases report

Los hamartomas hipotalámicos son malformaciones no neoplásicas de sustancia gris compuestas por neuronas hiperplásicas. Suelen ser lesiones pequenas localizadas en la base del cerebro, en el piso del tercer ventrículo y, generalmente, asintomáticas. Sin embargo, pueden ocurrir con alteraciones conductuales-cognitivas, crisis epilépticas y/o signos de pubertad precoz central en función de la localización en la que se encuentren. Se presentan dos pacientes de 2 años 8 meses y 7 años, con presencia de hamartomas hipotalámicos diagnosticados tras el estudio de pubertad precoz central. La paciente de menor edad presenta, además, crisis gelásticas, típicamente asociadas a hamartomas hipotalámicos. Tras los hallazgos clínicos y radiológicos, se trataron con análogos de gonadotropinas, y se observó una regresión de los signos puberales y una no progresión del tamano de los hamartomas.

Hypothalamic hamartomas are benign tumors of gray substance composed by hyperplasic neurons. They are usually asymptomatic small masses with extensions into the third ventricular cavity. In some instances they can cause cognitive behavioral alterations, seizures and/or central precocious puberty depending on the location. Here we present two cases of central precocious puberty due to hypothalamic hamartomas at 2(8/12) and 7 years of age. The younger patient also presents gelastic seizures, typically associated with hypothalamic hamartomas. After the clinical and radiological findings, they started treatment with GnRH analogues and a regression of the puberty signs without progression in the hamartomas size was observed.

Síndrome de deprivación glucocorticoidea / Glucocorticoid deprivation syndrome

El síndrome de deprivación glucocorticoidea se manifiesta en pacientes con hipercortisolismo endógeno después de la cirugía, y en individuos que han recibido tratamientos con dosis elevadas de glucocorticoides por más de 2 semanas. El eje hipotálamo-hipófiso-adrenocortical regula la secreción diaria de cortisol y presenta un ritmo circadiano. El ritmo puede perderse por estrés, enfermedad, o por la administración farmacológica de glucocorticoides. La retirada de corticoides puede causar insuficiencia adrenal secundaria, síndrome de retirada o deprivación de corticoides, y reactivación de la enfermedad de base para la cual fueron indicados. La insuficiencia adrenal secundaria es la complicación más temida, y constituye la principal causa de crisis adrenal e insuficiencia adrenal secundaria en la actualidad. El síndrome de retirada o deprivación de corticoides es autolimitado, y puede ser tratado fácilmente con el incremento temporal de la dosis de corticoide, seguido por una retirada lenta de este. Es necesario conocer las ventajas y limitaciones que trae aparejado el tratamiento con glucocorticoides, así como su descontinuación gradual. Debe evaluarse adecuadamente el estado del eje hipotálamo-hipófiso-adrenocortical al término del tratamiento prolongado, o con dosis suprafisiológicas de glucocorticoides(AU)

Glucocorticoid deprivation syndrome occurs in patients with endogenous hypercortisolism after surgery and in individuals receiving treatments at high doses of glucocorticoids for more than 2 weeks. The hypothalamus-hypophysis-adrenocortical axis regulates the daily cortisol secretion and presents a Circadian rhythm that may be affected by stress, disease or glucocorticoid administration The corticoid withdrawal may cause secondary adrenal insufficiency, corticoid deprivation or withdrawal syndrome and reactivation of the underlying disease for which they were prescribed. The secondary adrenal insufficiency is currently the most serious complication and the main reason for adrenal crisis and secondary adrenal insufficiency. The corticoid deprivation or withdrawal syndrome is self-limited and may be easily treated with temporary increase of the corticoid dose, followed by slow withdrawal of this agent. It is then necessary to find out the advantages and limitations of the glucocorticosteroid treatment and its gradual cessation. The state of the hypothalamus-hypophysis-adrenocortical axis should be adequately evaluated at the end of a long treatment, or with the use of supraphysiological doses of glucocorticoids(AU)

Alteraciones en el gasto energético en reposo y parámetros respiratorios en pacientes con síndrome de Prader-Willi / Abnormalities in energy expenditure and respiratory parameters in patients with Prader Willy syndrome

Introducción: El síndrome de Prader Willi (SPW) es un trastorno genético complejo relacionado con insuficiencia hipotalámica. Una de las manifestaciones clínicas es la obesidad asociada a un desequilibrio entre la ingesta y el gasto energético, debido a la hiperfagia y a la baja tasa metabólica que presentan estos pacientes. Distintos procedimientos e intervenciones han sido utilizadas para tratar de limitar el consumo de alimentos, pero no hay resultados contundentes al respecto. Objetivo: Comparar el gasto energético en reposo (GER) de pacientes con SPW con pacientes obesos sin el Síndrome. Sujetos y métodos: Se realizó un estudio comparativo observacional, transversal y descriptivo en pacientes con SPW y pacientes obesos sin SPW pareados por edad, sexo e IMC. Se midió por medio de calorimetría indirecta el GER de los pacientes, así como por antropometría, IMC, pliegue cutáneo de tríceps y bíceps. Se utilizó una prueba T de Student para muestras independientes que comprobara la homogeneidad de los pacientes y se realizó una serie de análisis de ANCOVA sobre cada uno de los parámetros de la calorimetría indirecta. Resultados: Se analizó 20 pacientes mestizo-mexicanos entre 2 y 26 anos: 10 pacientes con SPW y 10 controles obesos. Se identificó una diferencia estadísticamente significativa en el GER, resultando 17.67% menor en los sujetos con SPW; así también en el volumen de oxígeno (mil/min) y volumen TIDAL (p < 0.05). Conclusión: Pacientes con SPW per se producen modificaciones en el GER a lo largo de la vida en comparación con los pacientes con obesidad.

Introduction: Prader Willi Syndrome (PWS) is a complex genetic disorder with many manifestations relating to hypothalamic insufficiency. One such feature is obesity associated with an imbalance between caloric intake and expenditure due to hyperphagia and slow metabolic rate. Different procedures, as the use of appetite suppressants, surgical interventions and change in behavioral techniques have been used to try to limit food intake, but there is not convincing results about it. Aim: To compare the resting energy expenditure (REE) in PWS patients with obese patients without the syndrome matched by age and BMI (Body Mass Index). Subjects and methods: We conducted a comparative, observational and descriptive study in PWS and obese patients, matched for age, sex and BMI. RER was measured by indirect calorimerty and by anthropometry (BMI, skin-fold triceps and bíceps). A t-Student test for independent samples was used to demonstrate homogeneity between subjects and a series of ANCOVA analysis was performed on each of the parameters displayed by the indirect calorimetry. Results: Twenty mestizo-mexican patients, 10 with PWS and 10 with obesity were analyzed matched by age, BMI and gender. We found a statistically significant difference in the REE, the volume of oxygen (ml/min) and tidal volume (p <0.05). Conclusions: Prader Willy Syndrome per se produces changes in the REE in comparison with patients with only obesity.

Disfunção hipotalâmica na obesidade / Hypothalamic dysfunction in obesity

A obesidade, definida como o acúmulo excessivo ou anormal de gordura que pode causar dano à saúde do indivíduo, é considerada atualmente um dos principais problemas de saúde pública. Resulta de um desequilíbrio entre a ingestão alimentar e o gasto corporal de energia. O controle do balanço energético de animais e seres humanos é realizado pelo sistema nervoso central (SNC) por meio de conexões neuroendócrinas, em que hormônios periféricos circulantes, como a leptina e a insulina, sinalizam neurônios especializados do hipotálamo sobre os estoques de gordura do organismo e induzem respostas apropriadas para a manutenção da estabilidade desses estoques. A maioria dos casos de obesidade se associa a um quadro de resistência central à ação da leptina e da insulina. Em animais de experimentação, a dieta hiperlipídica é capaz de induzir um processo inflamatório no hipotálamo, que interfere com as vias intracelulares de sinalização por esses hormônios, resultando em hiperfagia, diminuição do gasto de energia e, por fim, obesidade. Evidências recentes obtidas por intermédio de estudos de neuroimagem e avaliação de marcadores inflamatórios no líquido cefalorraquidiano de indivíduos obesos sugerem que alterações semelhantes podem estar presentes também em seres humanos. Nesta revisão, apresentamos sumariamente os mecanismos envolvidos com a perda do controle homeostático do balanço energético em modelos animais de obesidade e as evidências atuais de disfunção hipotalâmica em humanos obesos.

Obesity, defined as abnormal or excessive fat accumulation that may impair life quality, is one of the major public health problems worldwide. It results from an imbalance between food intake and energy expenditure. The control of energy balance in animals and humans is performed by the central nervous system (CNS) by means of neuroendocrine connections, in which circulating peripheral hormones, such as leptin and insulin, provide signals to specialized neurons of the hypothalamus reflecting body fat stores, and induce appropriate responses to maintain the stability of these stores. The majority of obesity cases are associated with central resistance to both leptin and insulin actions. In experimental animals, high-fat diets can induce an inflammatory process in the hypothalamus, which impairs leptin and insulin intracellular signaling pathways, and results in hyperphagia, decreased energy expenditure and, ultimately, obesity. Recent evidence obtained from neuroimaging studies and assessment of inflammatory markers in the cerebrospinal fluid of obese subjects suggests that similar alterations may be also present in humans. In this review, we briefly present the mechanisms involved with the loss of homeostatic control of energy balance in animal models of obesity, and the current evidence of hypothalamic dysfunction in obese humans.

Repercusiones endocrinólogicas en el apnea obstructiva del sueño / Endocrine impact of Obstructive sleep apnea

Las alteraciones en la oxigenación que ocurren en el Sindrome Obstructivo del Apnea de sueño comprometen el funcionamiento tisular de todos los órganos del ser humano; con relación a la disfunción endocrinológica, los aspectos concernientes a esta no son bien conocidos. La intención de este trabajo fue revisar la información existente al respecto, a través de una búsqueda en las referencias MEDLINE entre los años 1995 y 2009.

The obstructive sleep apnea cause and intermmitent decreased oxigenation in all the organs, compromising thefunction of all of them; in relation with the endocrine dysfunction they are not well known. This review consists ina search of references in Medline, between 1995 and 2009.

Amenorrea hipotalámica: consideraciones etiológicas y clínicas a raíz de una paciente / Hypothalamic amenorrhea: report of one case

Hypothalamic amenorrhea is secondary to the dysfunction of the hypothalamic pacemaker that regulates the pulsatile secretion of gonadotropin releasing hormone (GnRH). We report a 21 years old female with an isolated and persistent hypogonadotropic hypogonadism probably of congenital origin. The patient had a good olfactory function, did not have anatomical alterations and two GnRH stimulation tests showed a pre puberal pattern. Hormone replacement therapy allowed her to complete her puberal development.

The brain is the conductor: diet-induced inflammation overlapping physiological control of body mass and metabolism: [review] / O cérebro é o maestro: inflamação induzida por dieta interfere no controle fisiológico da adiposidade corporal e do metabolismo: [revisão]

Obesity is currently a worldwide pandemic. It affects more than 300 million humans and it will probably increase over the next 20 years. The consumption of calorie-rich foods is responsible for most of the obesity cases, but not all humans exposed to high-calorie diets develop the disease. This fact has prompted researchers to investigate the mechanisms linking the consumption of high-calorie diets to the generation of an imbalance between energy intake and expenditure. According to recent studies, the exposure to fat-rich diets induces an inflammatory response in the hypothalamic areas involved in the control of feeding and thermogenesis. The inflammatory process damages the neuronal circuitries that maintain the homeostatic control of the body's energy stores, therefore favoring body mass gain. This review will focus on the main advances obtained in this field.

Obesidade é hoje um grave problema de saúde pública no mundo. Mais de 300 milhões de pessoas são obesas e esse número deve crescer substancialmente nos próximos 20 anos. As dietas ricas em calorias são a principal causa de obesidade, porém, nem todos os indivíduos expostos a dietas altamente calóricas se tornam obesos. Tal fato estimulou pesquisadores a investigarem os mecanismos que ligam o consumo de dietas ricas em calorias ao desenvolvimento de um balanço inadequado entre consumo e gasto energético. De acordo com estudos recentes, o consumo de dietas ricas em gorduras induz a ativação de uma resposta inflamatória nas áreas do hipotálamo envolvidas com o controle da fome e da termogênese. Tal processo inflamatório lesa os circuitos neuronais que mantêm o controle homeostático das reservas corporais de energia, favorecendo assim o ganho de massa adiposa. Esta revisão irá focar os principais avanços obtidos nesta área.

Etiopatogenia de la amenorrea hipotalámica funcional: Interacción de las respuestas hormonales del sistema nervioso central y neuropéptidos periféricos / Ethiopatogeniae of functional hypothalamic amenorrhea: Interactions of central nervous system and peripherical neuropeptides responses

La amenorrea hipotalámica funcional (AHF) es una patología compleja provocada generalmente por el estrés psicológico, o por alteraciones psicógenas como la depresión en otras entidades como la amenorrea nerviosa y bulimia o en la excesiva actividad física (atletas competitivas, bailarinas clásicas) entre las causas más frecuentes. Habitualmente, estos factores se presentan asociados a dietas cuali y cuantitativamente inadecuadas, originando alteraciones en el balance energético (consumo /gasto calórico) y modificaciones en la composición corporal. En general, estas alteraciones se presentan combinadas e inducen reacciones hormonales tendientes a defender la homeostasis metabólica general. Los protagonistas de estos procesos adaptativos son un "circuito" constituido en el sistema nervioso central que se interrelaciona con otro "circuito periférico hormonal". Las manifestaciones del primero incluyen principalmente una atenuación funcional de los ejes hipotalamo-hipófiso-somatotrófico, prolactínico y tiroideo, amplificación de la secreción nocturna de la melatonina e hiperactividad adrenal. A su vez, las interrelaciones, a nivel hipotalámico, del eje CRH-ACTH-sistemas-opiodeo-dopaminérgico y la consiguiente inhibición de la secreción de Gn RH, constituye un mecanismo fisiopatológico crucial para el desarrollo de la disfunción gonadal. Por otro lado, el grado de alteración de la actividad pusátil del Gn RH está condicionado por la etiopatogenia, intensidad nosológica y el tiempo de evolución. La variabilidad y el tipo de irregularidades del ciclo menstrual, que frecuentemente son observados en las atletas competitivas, constituyen un reflejo representativo de ello. Por otro lado, el hipoestrogenismo e hipoandrogenismo de estas mujeres repercuten negativamente en el metabolismo general y osteocálcico, en particular. La osteopenia con su riesgo de fracturas, está omnipresente en este síndrome. No menos importante, el hipoestrogenismo es también uno de los factores de riesgo cardiovascular. No obstante, la administración de anticonceptivos que combinan estrógenos más progesterona, pueden aumentar significativamente los niveles de la proteína C reactiva, un reconocido y seguro marcador de riesgo cardiovascular. Ello induce a tomar precauciones en su utilización en estas amenorricas desnutridas. Asimismo, la administración de anticonceptivos no ha demostrado brindar un beneficio sustancial en el tratamiento de la osteosporosis de estas pacientes. El "circuito periférico", funcionalmente interrelacionado con el central, está principalmente compuesto por la leptina, adiponectina, ghrelin, insulina e IGF-1, péptidos provenientes del compartimiento graso ("adipocitokinas") tracto gastrointestinal superior, páncreas e hígado, respectivamente. Estos péptidos no sólo están involucrados en los mecanismos centrales del apetito y saciedad, sino que también participan en las respuestas de adaptación homeostática, tendientes a revertir los desvíos del metabolismo intermedio y fosfocálcico, que en grado variable afectan a estas pacientes. Conclusiones: la AHF relacionada en general con el estrés psicofísico prolongado y la mala nutrición, es un síndrome complejo, cuyas respuestas hormonales centrales y periféricas de adaptación, resultan primordiales en estas mujeres que padecen un cierto grado de compromiso nutritivometabólico general. Enfocando globalmente este abigarrado síndrome, estimamos que la AHF es, obviamente, un epifenómeno menos vital.

Functional Hypothalamic Amenorrhea (FHA) is a complex pathology produced by psicological stress, as observed in amenorrhea nervosa and bulimia or in intensive physical activity (athletas, competitions, classic dancing). Frequently, these factors are associated to quali-quantitative inadequated diets and therefore they induce alterations in the energetic balance (caloric intake, caloric expend and modification in body composition). These factors are usually combinated and they induce hormonal reactions trend to sustain the general metabolic homeostasis. The protagonists of this adaptative process are a "central nervous system circuit" interrelated with "periferical hormonal circuit". The responses to the first, mainly includes functional atenuation of Hypothalamic- Hipophyseal Somatotropic, Prolactin and Thyroid axes, amplification of nocturnal melatonine secretion and hyper adrenal activity. The relationships, to hypothalamic level, of CRH-ACTH, opioid, and dopaminergic systems, and the subsequent inhibition of Gn-RH secretion, constitute a physiopathological mechanism for the development of gonadal disfunction. On the other hand, the degree of alteration in the Gn-RH pulsatility is conditioned by the ethipahogenic, nosologic intensity and time of evolution. Variability and type of irregularity of menstrual cycle observed in competitive athletes, is a demonstrative example. On the other hand, hypoestrogenism and hypoandrogenism in these women affect negatively the general metabolism and, particularly, the osteocalcic system. Osteopenia with its fracture risk is omnipresence in this syndrome. As important as that hypoestrogenism is also one of cardiovascular factor risk. However the administration of contraceptives which combines estrogens plus progesterone, may significantly increase the C-reactive protein level, a known and safety marker of cardiovascular risk. Therefore it induces to take precautions in its administration in these unnourished amenorrheic women. Also, the administration of contraceptives has not showed a substantial benefit in osteoporosis treatment of this patients. A "periferic circuit", functionally interrelated with the "central circuit", is mainly composed by leptin, adiponectine, ghrelin, insulin and IGF1 peptides, coming from fat compartiment (adipokines), gastro-intestinal tract, pancreas and liver, respectively. These peptides, are not only involved in the central mechanisms of appetite and saciety, but they also participate in homeostatic adaptative responses, with the aim of to balance the alteration of middle and phosphocalcic metabolism which affect these patients in a variable form. CONCLUSIONS: FHA, generally related with psychic/physic stress and malnutrition, is a complex syndrome in which the central and peripheral adaptative hormonal responses are mainly important in these women which are affected of a kind of nutritive and general metabolic compromise. Analazing this complex process we considerate that the hypothalamic amenorrhea is obviously a less vital phenomenon.

Hamartoma hipotalámico asociado a dos quistes aracnoideos: a propósito de un caso / Hypothalamic hamartoma associated with arachnoid cyst: report of a case

Hypothalamic hamartomas are heterotopias of the nervous tissue which are similar to the grey matter of the hypothalamus. They may cause precocious puberty and/or gelastic seizures. We present the case of a patient who began her pubertal development at the age of 9 months. Cranial magnetic resonance imaging showed a hypothalamic hamartoma associated with middle cranial fossa and pineal region arachnoid cysts, a very rare association.

Los hamartomas hipotalámicos son heterotopias de tejido nervioso que se asemejan a la sustancia gris del hipotálamo. Pueden causar pubertad precoz y/o crisis gelásticas. Presentamos el caso de una paciente que a los 9 meses de edad comenzó desarrollo puberal y que en la resonancia magnética craneal se objetivó un hamartoma hipotalámico asociado a dos quistes aracnoideos, uno en fosa craneal media y otro en región pineal, asociación que es muy infrecuente.

La distrofia adiposo genital o síndrome de Frohlich: su contribución al establecimiento de la neuroendocrinología / The adiposogenital distrophy or Frohlich syndrome and the beginning of the concept of neuroendocrinology

Hace casi un siglo Harvey Cushing llamó la atención médica mundial con motivo de un informe clínico consistente en el caso de obesidad e infantilismo sexual ocasionado por un tumor en la base del cráneo al cual se le dio el nombre de distrofia adiposo-genital o síndrome de Frohlich por haber sido quien lo describió por primera vez. El estudio de este síndrome permitió establecer la conexión entre la hipófisis y el hipotálamo que eran estructuras con funciones poco conocidas hasta ese momento. Así se fue gestando la neuroendocrinología clínica.

A century has passed since Harvey Cushing presented a syndrome characterized by obesity and sexual infantilism, caused by a tumour located in the base of the brain, and named it as Adiposogenital dystrophy or Frohlich's syndrome, on the name of its discoverer. Further study of this syndrome contributed to establish the hormonal connection between the brain and the anterior pituitary, and then the concept of neuroendocrinology.

Anoftalmía bilateral por displasia septo-óptica: síndrome de De Morsier / Anophthalmia bilateral caused by septo-optic dysplasia: the De Morsier syndrome

Introducción: La displasia septo-óptica es una enfermedad congénita caracterizada por hipoplasia de uno o ambos nervios ópticos que se acompaña de malformaciones cerebrales de la línea media y disfunción hipotálamo-hipofisiaria. Objetivo: Mostrar un caso ilustrativo del estudio de pacientes con anoftalmía y retraso psicomotor, con énfasis en los diagnósticos diferenciales. Caso clínico: Lactante femenina de 18 meses de edad con anoftalmía bilateral, asociada con ausencia en el desarrollo de los nervios ópticos e hipoplasia del septum pellucidum corroborada por tomografía craneal, el cariotipo normal descartó la posibilidad de cromosomopatía. Conclusión: Caso clínico esporádico de displasia septo-óptica: síndrome de De Morsier extremo sin manifestaciones neurológicas o endocrinológicas.

Anorexia nervosa: repercussões endócrinas de interesse para o ginecologista / The endocrine consequences of anorexia nervosa of interest to the gynecologist

Os autores fazem revisão acerca das repercussões da anorexia nervosa (AN) nos sistemas genital e reprodutivo feminino, do ponto de vista endócrino. Analisam, ainda, as alterações ocorridas no eixo hipotála-mo-hipófise-gônadas que trazem como consequência a amenorréia e atualizam teorias sobre o seu mecanismo...