Crisis adrenal neonatal secundaria a hipoplasia hipofisaria / Neonatal Adrenal Crisis Secondary to Pituitary Hypoplasia

Introducción: La insuficiencia adrenal hipotálamo hipofisaria usualmente se manifiesta secundaria a tumores y, cuando resulta congénita se asocia, con frecuencia, con otras deficiencias hormonales. La crisis adrenal suele presentarse en su debut y puede resultar potencialmente mortal. Objetivo: Examinar el caso de una paciente con insuficiencia adrenal central que debutó con una crisis adrenal congénita. Presentación del caso: Recién nacida a término, padres no consanguíneos, hospitalizada a los 9 días de vida por clínica de una semana con múltiples episodios eméticos y apnea. Ingresó con deshidratación severa, hipotensa y estuporosa. Además, se encontró acidosis metabólica severa, hipoglucemia persistente, hiponatremia e insuficiencia prerrenal. Ante la no mejoría de su estado hemodinámico, a pesar del uso de cristaloides y vasopresores, finalmente mejoró con la administración de dosis altas de hidrocortisona. El diagnóstico de deficiencia de cortisol de origen central se realizó con un test dinámico de insulina y la resonancia magnética nuclear hipofisaria. Conclusiones: La crisis adrenal se debe tener presente como diagnóstico diferencial en episodios agudos con inestabilidad hemodinámica persistente e hipoglucemia de difícil manejo. Adicionalmente, hay que considerar que existen otras causas menos comunes de insuficiencia adrenal en neonatos como la hipoplasia hipofisaria(AU)

Introduction: Hypothalamic-pituitary adrenal insufficiency usually manifests secondary to tumors and, when congenital, is often associated with other hormonal deficiencies. Adrenal crisis usually occurs at its onset and can be life threatening. Objective: To review the case of a patient with central adrenal insufficiency who had an onset with a congenital adrenal crisis. Case presentation: Term newborn, non-consanguineous parents, hospitalized at 9 days of life for a week-long clinical presentation with multiple emetic episodes and apnea. She was admitted with severe dehydration, hypotensive and stuporous. In addition, severe metabolic acidosis, persistent hypoglycemia, hyponatremia and prerenal failure were found. Given the lack of improvement of her hemodynamic status, despite the use of crystalloids and vasopressors, she finally improved with the administration of high doses of hydrocortisone. The diagnosis of cortisol deficiency of central origin was made with a dynamic insulin test and pituitary nuclear magnetic resonance imaging. Conclusions: Adrenal crisis should be kept in mind as a differential diagnosis in acute episodes with persistent hemodynamic instability and difficult-to-manage hypoglycemia. Additionally, other less common causes of adrenal insufficiency in neonates, such as pituitary hypoplasia, should be considered(AU)

Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia

ABSTRACT Primary adrenal insufficiency (PAI) is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Addison's disease (AD) and congenital adrenal hyperplasia (CAH) are the most frequent disorders in adults and children, respectively. Despite the diagnostic advances and the availability of glucocorticoid and mineralocorticoid replacements, adrenal crisis (AC) is still a potentially lethal condition contributing to the increased mortality, not only during the first year of life, but also throughout life. Failure in increasing glucocorticoid doses during acute stress, when greater amounts of glucocorticoids are required, can lead to AC and an increase morbimortality rate of PAI. Considering a mortality rate of 0.5 per 100 patient years, up to 1,500 deaths from AC are expected in Brazil in the coming decade, which represents an alarming situation. The major clinical features are hypotension and volume depletion. Nonspecific symptoms such as fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain are common. The main precipitating factors are gastrointestinal diseases, other infectious disease, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. Suspected AC requires immediate therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if necessary. AC is best prevented through patient education, precocious identification and by adjusting the glucocorticoid dosage in stressor situations. The emergency card, warning about acute glucocorticoid replacement, has high value in reducing the morbidity and mortality of AC.

Consideraciones en el manejo del adulto con Hiperplasia Suprarrenal congénita clásica por déficit de 21-Hidroxilasa / Management considerations for the adult with congenital adrenal hyperplasia due 21-hydroxylase deficiency

La Hiperplasia Suprarrenal Congénita (HSRC) corresponde a un grupo de defectos genéticos en la síntesis de cortisol. El 95% de ellas son debidas al déficit de 21-hidroxilasa por lo que nos referiremos solo a esta deficiencia. La hiperplasia suprarrenal congénita clásica (HSRC-C) debuta en recién nacidos o lactantes con insuficiencia suprarrenal primaria, diferentes grados de hiperandrogenismo clínico en mujeres y puede coexistir con hipotensión, hiperkalemia e hiponatremia si hay un déficit clínico de aldosterona. El objetivo de este artículo es actualizar el conocimiento y enfoques sugeridos para el manejo de la HSRC-C desde el inicio de sus controles en la etapa adulta. El diagnóstico diferencial en retrospectiva de la HSRC-C y la no clásica (HSRC-NC) a veces resulta difícil ya que esta enfermedad es un espectro fenotípico continuo. La insuficiencia suprarrenal y la dependencia a terapia corticoidal son los eventos principales para diferenciar estas dos patologías que tienen enfoques terapéuticos diferentes. El tratamiento de la HSRC-C en adultos abarca 2 objetivos primarios: la adecuada sustitución de la falla suprarrenal y el control de hiperandrogenismo mediante el uso de corticoides en sus dosis mínimas efectivas. En la mujer existen terapias complementarias para el control del hiperandrogenismo como anticonceptivos y otras que se encuentran en diferentes fases de investigación. Esto permite disminuir las dosis de corticoides en algunos casos. Es importante a la vez abordar tres objetivos secundarios: controlar el riesgo cardiometabólico propio de la enfermedad, evitar el sobre tratamiento corticoidal y manejar la infertilidad. La correcta monitorización del tratamiento en adultos tomando en cuenta los objetivos descritos permite una mejor calidad de vida en estos pacientes. Finalmente el consejo genético debe realizarse en todos los pacientes con HSRC que deseen fertilidad y en sus parejas. El estudio requiere de secuenciación del gen CYP21A2 y debe realizarse en un laboratorio de experiencia.

Congenital Adrenal Hyperplasia (CAH) are a group of genetic defects characterized by impaired cortisol synthesis. 95% of them are due to 21-hydroxylase deficiency. We will discuss only this enzyme's deficiency. Classic congenital adrenal hyperplasia (CAH-C) debuts in newborns or infants with primary adrenal insufficiency, some degree of clinical hyperandrogenism in newborn females, and can coexist with hypotension, hyperkalemia, and hyponatremia if there is a clinical aldosterone deficiency. The objective of this article is to update the knowledge and suggested approaches for the management of CAH-C from the beginning of its controls in the adult stage. The retrospective differential diagnosis of CAH-C and non-classical (CAH-NC) is sometimes difficult because this disease is a continuous phenotypic spectrum. Adrenal insufficiency and dependence on corticosteroid therapy are the main events to differentiate these two pathologies that have different therapeutic approaches. In adults, the treatment of CAH-C must include 2 primary objectives: adequate the replacement of adrenal failure and control of hyperandrogenism, through the use of corticosteroids in their minimum effective doses. In women there are complementary therapies for the control of hyperandrogenism, such as contraceptives and others that are in different phases of research. This makes it possible to reduce the doses of corticosteroids in some cases. It is important at the same time to address three secondary objectives: control the cardiometabolic risk of the disease secondary to corticosteroid treatment, avoid corticosteroid overtreatment and manage infertility. The correct monitoring of treatment in adults and taking in to account the objectives described, allows a better quality of life in these patients. Finally, genetic counseling must be carried out in all patients planning for children, with any type of CAH and in their partners. The study requires sequencing of the CYP21A2 gene and must be performed in a certified laboratory.

Surgical outcomes of the endoscopic endonasal transsphenoidal approach for large and giant pituitary adenomas: institutional experience with special attention to approach-related complications / Resultados cirúrgicos do acesso endonasal endoscópico transesfenoidal para adenomas hipofisários grandes e gigantes: experiência institucional com ênfase às complicações relacionadas ao acesso cirúrgico

ABSTRACT Objective In this study, we investigate our institutional experience of patients who underwent endoscopic endonasal transsphenoidal approach for treatment of large and giant pituitary adenomas emphasizing the surgical results and approach-related complications. Method The authors reviewed 28 consecutive patients who underwent surgery between March, 2010 and March, 2014. Results The mean preoperative tumor diameter was 4.6 cm. Gross-total resection was achieved in 14.3%, near-total in 10.7%, subtotal in 39.3%, and partial in 35.7%. Nine patients experienced improvement in visual acuity, while one patient worsened. The most common complications were transient diabetes insipidus (53%), new pituitary deficit (35.7%), endonasal adhesions (21.4%), and cerebrospinal fluid leak (17.8%). Surgical mortality was 7.1%. Conclusions Endoscopic endonasal transsphenoidal surgery is a valuable treatment option for large or giant pituitary adenomas, which results in high rates of surgical decompression of cerebrovascular structures.

RESUMO Objetivo Neste manuscrito investigamos a experiência institucional com o acesso endonasal endoscópico transesfenoidal no tratamento de adenomas hipofisários grandes e gigantes com ênfase às complicações relacionadas ao acesso cirúrgico. Método Foram incluídos neste estudo 28 pacientes consecutivos submetidos à cirurgia entre Março de 2010 e Março de 2014. Resultados O diâmetro médio pré-operatório dos tumores era 4,6 cm. Uma ressecção total foi obtida em 14,3%; quase total, em 10,7%; subtotal, em 39,3% e parcial, em 35,7%. Nove pacientes evoluíram com melhora na acuidade visual, enquanto um paciente apresentou piora da função visual. As complicações mais comuns foram diabetes insipidus transitório (53%), novo défice hipofisário (35,7%), sinéquias endonasais (21,4%) e fistula liquórica (17,8%). A mortalidade cirúrgica foi 7,1%. Conclusões A cirurgia por via endonasal endoscópica transesfenoidal é uma opção terapêutica extremamente útil para adenomas hipofisários grandes e gigantes, a resultar numa significativa descompressão das estruturas cerebrovasculares.

Neonatal cholestasis in congenital pituitary hormone deficiency and isolated hypocortisolism: characterization of liver dysfunction and follow-up / Colestase neonatal na deficiência congênita de hormônio hipofisário e hipocortisolismo isolado: caracterização da disfunção hepática e acompanhamento

INTRODUCTION: Neonatal cholestasis due to endocrine diseases is infrequent and poorly reco-gnized. Referral to the pediatric endocrinologist is delayed. OBJECTIVE: We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT). SUBJECTS AND METHODS: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. RESULTS: Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder. CONCLUSION: Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

INTRODUÇÃO: A colestase neonatal causada por doenças endócrinas é pouco frequente e reconhecida. Existe um atraso no encaminhamento dos pacientes a um endocrinologista pediátrico. OBJETIVO: Caracterizamos a colestase em recém-nascidos com deficiências congênitas de hormônio hipofisário (DCHH) e sua resolução após a terapia de reposição hormonal (TRH). SUJEITOS E MÉTODOS: Dezesseis pacientes (12 do sexo masculino) foram incluídos; sete com DCHH, e cinco com hipocortisolismo central isolado. RESULTADOS: O início da colestase ocorreu aos 18 dias de vida (variação 2-120). Dez e nove pacientes apresentaram elevação das transaminases e γGT, respectivamente. A consulta com um endocrinologista aconteceu aos 32 dias (variação 1-72). A remissão da colestase ocorreu em uma idade mediana de 65 dias, enquanto a remissão das enzimas hepáticas aconteceu aos 90 dias. Na coorte isolada, o hipocortisolismo foi uma desordem transitória. CONCLUSÃO: A colestase causada por deficiências hormonais foi completamente resolvida após a introdução da TRH. O hipocortisolismo pode ser uma causa transitória da colestase e precisa ser reavaliado após a remissão da colestase.

Disfunción suprarrenal en el paciente cirrótico, ¿síndrome hepatoadrenal o síndrome del “euadrenálico” enfermo? / Adrenal dysfunction in the cirrhotic patient, ¿hepatoadrenal syndrome or the non adrenal illness syndrome?

Adrenal insufficiency is a potentially lethal condition that is characterized by decreased production of glucocorticoids, mineralocorticoids and sex steroids. In normal conditions adrenocorticotropin and corticotrophin releasing hormone regulate cortisol production, but in critical illness other peptides and substances are produced that modulate the axis. In decompensated liver failure, there is a hemodynamic state similar to septic shock, even an increase in proinflammatory cytokines such as IL6 and TNF- alpha. In critically ill patients, a high proportion of adrenal insufficiency has been reported; therefore, some authors have argued that the same could happen in the cirrhotic patient, introducing the term hepatoadrenal syndrome. There are several reports of an increased frequency of adrenal insufficiency in the cirrhotic patient. However, these studies are heterogeneous in many aspects. There are differences in patient’s characteristics, in the criteria for the diagnosis of adrenal insufficiency, and in the laboratory tests used. Because over 90 percent of cortisol circulates bound to proteins, it is important to consider this issue in the diagnosis of adrenal insufficiency, especially in patients with hypoalbuminemia. Serum-free cortisol and salivary cortisol appear to be a good diagnostic method. Further studies need to be carried out in order to define the cut off points of these methods. These tests are not widely available.

La insuficiencia suprarrenal es una condición potencialmente letal, que se caracteriza por la disminución en la producción de glucocorticoides, mineralocorticoides y esteroides sexuales. Normalmente, la producción de cortisol es regulada por la adrenocorticotrofina y ésta por la hormona liberadora de corticotrofina, pero en estado de enfermedad, se producen otros péptidos y sustancias que modulan el eje. En la insuficiencia hepática descompensada, se produce un estado hemodinámico similar al shock séptico, incluso se produce un aumento de citoquinas proinflamatorias como IL6 y TNF- alfa. Como en el paciente crítico se ha reportado un gran porcentaje de insuficiencia adrenal, algunos autores han planteado que lo mismo podría ocurrir en el paciente cirrótico, introduciendo el término de síndrome hepatoadrenal. Varios autores han publicado que también existe un aumento en la frecuencia de insuficiencia adrenal en el paciente cirrótico. Sin embargo, en estos estudios la población analizada es heterogénea, se utilizan distintos criterios para definir insuficiencia adrenal y las pruebas de laboratorio utilizadas tienen limitaciones. Debido a que más del 90 por ciento del cortisol circula unido a proteínas, es importante considerar este factor para diagnóstico de la insuficiencia adrenal, especialmente en el paciente con hipoalbuminemia. En estos casos, el cortisol libre sérico parece ser junto al cortisol salival un buen método diagnóstico. Sin embargo, faltan estudios para definir los puntos de corte de estos exámenes y tienen la limitación que no están ampliamente disponibles.

Enfermedad de Addison en un niño con adrenoleucodistrofia ligada al cromosoma X / Primary adrenal failure associated to X-linked adrenoleukodystrophy: report of one case

Primary adrenal failure (PAF) can be congenital or acquired. X-linked adrenoleukodystrophy (ALD-X), produced by the mutation of the ABDC1 gene (Xq28), that leads to the plasma accumulation of very long chain fatty acids, is one of the congenital diseases associated to adrenal destruction. We report a 7 years old boy with fast progression of right strabismus and general symptoms as weariness, weakness and mucosal and skin pigmentation. A brain magnetic resonance image showed a leukoencephalopathy, characteristic of ALD-X. Low plasma cortisol, high ACTH levels and lack of response to ACTH test, confirmed the diagnosis of primary adrenal insufficiency. High plasma levels of C26:0 fatty acids, and C24/22, C26/22 ratios confirmed ALD-X.

Uso racional de corticoides en el paciente con shock séptico / Rational use of corticoids in a patient with septic shock

Introduction: Septic shock (SS) is a significant cause of mortality in NICUs. Objective: Review current knowledge on Hypothalamic-Pituitary-Adrenal Axis (HPA) and the scientific support for the use of gluco-corticoids in the use of this clinical picture. We know that the patient's ability to evolve into improvement or worsening depends upon the ability of the HPA axis to develop and sustain an adequate response to the stress provoked by SS. In some patients, due to many reasons, the prolongation of SS leads to a deficit of cortisol those results in functional acute adrenal insufficiency. Cortisol levels do not respond to ACTH stimulation test. There is no consensus among authors as to what is a normal concentration of cortisol during stress, or even if it is correlated with death among children with SS. The American College of Critical Care Medicine guidelines for SS in Pediatrics and Neonatology have made some recommendations for use of hydrocortisone.

Introducción: El shock séptico (SS) es una de las mayores causas de mortalidad en unidades de cuidados intensivos pediátricas. Objetivo: Revisar qué sabemos hasta ahora del papel que juega el eje hipotálamo-pituitaria-adrenal (HPA) en el SS y si hay evidencia científica que apoye el uso de glucocorticoides en el transcurso de este cuadro. Sabemos que de la habilidad del eje HPA para montar y sostener en el tiempo una adecuada respuesta al stress provocado por un SS dependerá si el paciente evoluciona a la mejoría o se agrava. En algunos pacientes debido a múltiples mecanismos, la prolongación del SS puede llevar a un déficit de cortisol, resultando en una insuficiencia adrenal aguda o funcional, la que se reflejaría en que los niveles de cortisol no responderían con un incremento significativo frente a una prueba de estimulo con ACTH. Entre los diversos reportes revisados, no existe consenso sobre cual sería la concentración "normal" de cortisol durante el stress y más aun no está claro si esto se correlacionaría con la mortalidad en el caso de los niños. Las guías de shock séptico en pediatría y neonatología del American College of Critical Care Medicine recomiendan en que tipo de pacientes con SS se debiera considerar tratamiento con hidrocortisona.

Prueba de cosintropina en tuberculosis activa grave / Cosyntropin test in severe active tuberculosis

BACKGROUND: Frequency of adrenal insufficiency in patients with tuberculosis varies from 0 to 58%; however, all published series excluded severely ill patients. Our objective was to investigate adrenal insufficiency with the low-dose cosyntropin test in patients with severe active tuberculosis. METHOD: From two large university affiliated hospitals, 18 patients with tuberculosis and criteria of sepsis or severe sepsis according to SCCM/ACCP criteria, defined by the present authors as severe active tuberculosis, participated in the study. A low-dose ACTH test with 10 mg of ACTH was performed. After ACTH test, all patients received a stress dose of hydrocortisone (240 mg/day) during their entire hospitalization along with four antituberculous drugs. Abnormal response was considered when elevation of serum cortisol was <7 microg/dl with respect to basal level, 60 min after ACTH administration. RESULTS: Adrenal insufficiency was found in seven patients (39%); no clinical or laboratory data were associated with the presence of abnormal adrenal response. Except in one patient with HIV infection, all the signs and symptoms improved after antituberculous and hydrocortisone treatment. The increment in serum cortisol value post-ACTH test was lower in patients with hypoalbuminemia. CONCLUSIONS: Adrenal insufficiency is frequent in severe active tuberculosis. The efficacy and security of supplemental steroid treatment in severe active tuberculosis should be established by a randomized clinical trial.

Função adrenal na sepse e choque séptico / Adrenal function in sepsis and septic shock

OBJETIVO: Rever os critérios diagnósticos e o tratamento de insuficiência adrenal, em pacientes da faixa etária pediátrica, com sepse grave e choque séptico. FONTES DOS DADOS: Os artigos foram selecionados através das bases de dados MEDLINE (1966-junho 2007), Embase (1994-2007) e Cochrane Library (2000-2007). As seguintes palavras-chave foram utilizadas: choque séptico, sepse, corticosteróides, insuficiência adrenal e crianças. SÍNTESE DOS DADOS: Não existe um critério bem estabelecido e aceito para definir insuficiência adrenal em pacientes criticamente enfermos. A incidência de insuficiência adrenal varia de acordo com o critério utilizado, podendo alcançar desde valores inferiores a 15 por cento até superiores a 61 por cento. O teste rápido de estímulo com hormônio adrenocorticotrófico (ACTH) é largamente utilizado como um teste simples para a identificação de não responsividade adrenocortical, mas existe muita discussão quanto à dose de corticotropina a ser utilizada. A dose de 250 µg é a dose padrão. Recentemente, baixas doses de corticotropina (1 µg) têm sido propostas, com a sugestão de que elas possam ter uma maior sensibilidade. Dúvidas ainda persistem quanto à eficácia da reposição com baixas doses de corticosteróides em crianças com choque refratário às catecolaminas. Mais estudos são necessários para determinar se o tratamento de tais pacientes alteraria morbidade e/ou mortalidade. CONCLUSÃO: Insuficiência adrenal é comum em crianças com sepse grave e choque séptico e pode contribuir para o desenvolvimento de choque refratário às catecolaminas. Contudo, dúvidas ainda persistem em relação à eficácia da terapêutica com baixas doses de corticosteróides.

OBJECTIVE:To review diagnostic criteria and treatment of adrenal insufficiency in pediatric patients with severe sepsis and septic shock. SOURCES: Articles were selected using MEDLINE (1966-June 2007), Embase (1994-2007) and Cochrane Library (2000-2007) databases. following key words were utilized: septic shock, sepsis, corticosteroids, adrenal insufficiency and children. SUMMARY OF THE FINDINGS: There are no well established and accepted criteria to define adrenal insufficiency in critically ill patients. Incidence of adrenal insufficiency varies according to different criteria, and it may range between low values of 15 percent and high values of 61 percent. The rapid corticotropin stimulation test is widely used as a method to identify adrenocortical hyporesponsiveness, but controversy exists as to the corticotropin dose to be used. The 250 µg dose is the standard dose. Low doses of corticotropin (1 µg) have recently been proposed, suggesting that they may have higher sensitivity. There are still doubts as to the efficacy of low doses of corticosteroids in children with catecholamine-refractory shock. Further studies are needed to determine whether the treatment of these patients would change morbidity and/or mortality. CONCLUSION: Adrenal insufficiency is common in children with severe sepsis and septic shock and may contribute to the development of catecholamine-refractory shock. However, doubts still persist regarding the efficacy of replacement therapy with low-doses steroids.

Insuficiência adrenal primária como primeira manifestação de câncer pulmonar metastático / Primary adrenal insufficiency as first manifestation of metastatic lung cancer

A insuficiência adrenal primária é, na maioria das vezes, causada por infecções e adrenalite auto-imune. Metástases adrenais são relativamente comuns em neoplasias de pulmão, mas usualmente são assintomáticas, mesmo quando bilaterais. Há poucos relatos de metástases adrenais levando à insuficiência adrenal. Descrevemos aqui um caso de insuficiência adrenal primária como primeira manifestação clínica de neoplasia pulmonar metastática. Paciente de 59 anos, feminina, branca, tabagista, queixava-se de dor em flanco direito associada a náuseas e emagrecimento. Exames laboratoriais confirmaram o diagnóstico de insuficiência adrenal primária. Iniciou tratamento com prednisona e fludrocortisona, com melhora progressiva dos sintomas. Na investigação da etiologia, tomografia computadorizada (TC) de abdômen mostrou aumento bilateral das adrenais. Foi submetida à biopsia de adrenal, com citopatológico positivo para células malignas. Linfonodo supraclavicular esquerdo foi biopsiado, com anátomo-patológico (AP) confirmando adenocarcinoma metastático, com imunohistoquímica sugerindo pulmão como sítio primário. Atentar para o diagnóstico de insuficiência adrenal nesse contexto é importante, porque os sintomas iniciais são inespecíficos, podendo ser atribuídos à neoplasia

Primary adrenal insufficiency is, in most cases, caused by infections and autoimmune adrenalitis. Adrenal metastasis are relatively common in lung cancer, but they are usually asymptomatic, even when bilateral. There are few reports of adrenal metastasis as a cause of adrenal insufficiency. We describe a case of primary adrenal insufficiency presenting as the first clinical manifestation of metastatic lung cancer. A 59 year-old, white, smoker woman, complaining of right flank pain associated with nausea and weight loss. Laboratory exams confirmed the diagnosis of primary adrenal insufficiency. It was started treatment with prednisone and fludrocortisones with progressive improvement. At the etiologic investigation, abdominal computadorized tomography (CT) showed bilateral increase of the adrenal glands. It was performed an adrenal biopsy and the cytologic study was positive for malignant cells. It was made another biopsy, of a supraclavicular lymph node, and the histopathologic study revealed a metastatic adenocarcinoma, immunohistochemistry study suggested lung as the primary site. Awareness of this diagnosis is important because initial symptoms of adrenal insufficiency are unspecific and may misguidedly be attributed to the neoplasm

Emergencias endocrinas en pediatría: diagnóstico y manejo / Diagnosis and management of endocrine emergencies in pediatrics

The study of endocrine emergencies in childhood is important due to their high mortality and residual morbidity, that can be reduced with an adequate diagnosis and/or therapy. In this article, we review hypoglycemia, adrenal crisis, hypocalcemia, hypercalcemia and thyroid storm in children, with focus on initial diagnostic approach and management.

Adrenal crisis due to bilateral adrenal hemorrhage in primary antiphospholipid syndrome.

The authors report a case of a 56-year-old Thai woman with a history of recurrent venous thrombosis, spontaneous abortion and Graves' disease who presented with bilateral flank pain, nausea, vomiting and low-grade fever followed by hypotension. Adrenal crisis from bilateral adrenal hemorrhage was diagnosed by a low serum cortisol level during hypotension and bilateral hyperdense oval masses in each of the adrenal glands in a computerized tomographic study. Her hemostatic and serologic profile was compatible with primary antiphospholipid syndrome. Rapid improvement was observed after the administration of intravenous hydrocortisone. She was discharged on long-term glucocorticoid replacement for her primary adrenal insufficiency as well as an anticoagulant for prevention of thrombosis. The antiphospholipid syndrome should be suspected in a patient presenting with adrenal crisis without a distinct etiology.

Insuficiência adrenal na criança com choque séptico / Adrenal insufficiency in children with septic shock

OBJETIVO: Revisar os critérios para o diagnóstico e o tratamento da insuficiência adrenal nos pacientes com choque séptico. FONTES DOS DADOS: Artigos publicados em revistas nacionais e internacionais, selecionados nas suas páginas eletrônicas e através do Medline, bem como referências citadas em artigos chaves. SíNTESES DOS DADOS: Nos trabalhos publicados na literatura, o achado de insuficiência adrenal em pacientes com choque séptico tem variado entre 17 por cento a 54 por cento. Os dados publicados até a presente data, na literatura consultada, revelam a inexistência de um consenso para o diagnóstico da insuficiência adrenal em pacientes com doenças críticas, particularmente naqueles com choque séptico. A presença de choque refratário a volume e resistente a catecolaminas pode ser aceito como sugestivo, enquanto que um cortisol basal inferior a 25 µg/dl é um critério diagnóstico indicativo de insuficiência adrenal. O teste de estimulação adrenal é um recurso útil na identificação dos pacientes com insuficiência adrenal relativa. Nossa opção de teste para estimulação adrenal em pediatria é a utilização de corticotropina em baixas doses (0,5 µg/ 1,73 m²). Um aumento inferior a 9 µg/dl no valor do cortisol pós-teste sugere a presença de insuficiência adrenal oculta (relativa). Nos pacientes com choque séptico apresentando insuficiência adrenal, suspeita ou confirmada, a utilização de hidrocortisona em dose de choque ou de estresse pode ser vital na sua evolução favorável. CONCLUSÕES: Os dados existentes na literatura, embora controversos, já nos permitem especular sobre quando iniciar o tratamento de reposição hormonal, sobre qual o nível sérico de cortisol aceito como adequado e em relação à escolha da dose de corticotropina, para a realização do teste de estimulação adrenal e diagnóstico de insuficiência adrenal oculta ou relativa nos pacientes com choque séptico.

Insuficiencia supra-renal en UTI: actualización bibliográfica / Supra-renal insufficiency in the ICU: update record

La insuficiencia supra-renal en los pacientes internados en UTI no es frecuente (1-30%). Sin embargo en aquellos que cursan con hipotensión que no responde a la expansión de volumen y a las drogas vasopresoras y con hemodinamia semejante a la del shock séptico es una posibilidad diagnóstica que debe ser confirmada o descartada, ya que se trata de una condición que pone en peligro la vida. Se describen, a manera recordatoria, la prueba de estimulación rápida con ACTH sintético y las modalidades terapeúticas.

A case of primary bilateral adrenal lymphoma with partial adrenal insufficiency

Unilateral or bilateral non-Hodgkin's lymphomas arising primarily in the adrenal glands are extremely rare. These lymphomas are usually present with large, bilateral adrenal masses with or without lymphadenopathy, and may be accompanied by adrenal insufficiency in some cases. A review of the literature indicates that patients with primary lymphoma of the adrenal glands usually do not have disease elsewhere, and if present, it is frequently extranodal. We report here an unusual case of primary bilateral adrenal lymphoma with partial adrenal insufficiency.

Trastornos endocrinos en el paciente VIH+/SIDA / Endocrine disturbances in the HIV+/AIDS patient

En el paciente VIH+/Sida se producen diversas alteraciones en el sistema endocrino, siendo los mecanismos involucrados: infección por el VIH, neoplasias, infecciones oportunistas y el síndrome consuntivo general. Las alteraciones en general son subclínicas y sólo evidentes en estadios avanzados de la infección por el VIH. En esta revisión además se alerta al clínico respecto a medicamentos usados en estos pacientes y que pueden condicionar alteraciones en la esfera endocrinológica