From liver to hormones: The endocrine consequences of cirrhosis.

Hepatocrinology explores the intricate relationship between liver function and the endocrine system. Chronic liver diseases such as liver cirrhosis can cause endocrine disorders due to toxin accumulation and protein synthesis disruption. Despite its importance, assessing endocrine issues in cirrhotic patients is frequently neglected. This article provides a comprehensive review of the epidemiology, pathophysiology, diagnosis, and treatment of endocrine disturbances in liver cirrhosis. The review was conducted using the PubMed/Medline, EMBASE, and Scielo databases, encompassing 172 articles. Liver cirrhosis is associated with endocrine disturbances, including diabetes, hypoglycemia, sarcopenia, thyroid dysfunction, hypogonadotropic hypogonadism, bone disease, adrenal insufficiency, growth hormone dysfunction, and secondary hyperaldosteronism. The optimal tools for diagnosing diabetes and detecting hypoglycemia are the oral glucose tolerance test and continuous glucose monitoring system, respectively. Sarcopenia can be assessed through imaging and functional tests, while other endocrine disorders are evaluated using hormonal assays and imaging studies. Treatment options include metformin, glucagon-like peptide-1 analogs, sodium-glucose co-transporter-2 inhibitors, and insulin, which are effective and safe for diabetes control. Established standards are followed for managing hypoglycemia, and hormone replacement therapy is often necessary for other endocrine dysfunctions. Liver transplantation can address some of these problems.

A comprehensive review of genetic causes of obesity.

BACKGROUND: Obesity is a multifactorial chronic disease with a high, increasing worldwide prevalence. Genetic causes account for 7% of the cases in children with extreme obesity. DATA SOURCES: This narrative review was conducted by searching for papers published in the PubMed/MEDLINE, Embase and SciELO databases and included 161 articles. The search used the following search terms: "obesity", "obesity and genetics", "leptin", "Prader-Willi syndrome", and "melanocortins". The types of studies included were systematic reviews, clinical trials, prospective cohort studies, cross-sectional and prospective studies, narrative reviews, and case reports. RESULTS: The leptin-melanocortin pathway is primarily responsible for the regulation of appetite and body weight. However, several important aspects of the pathophysiology of obesity remain unknown. Genetic causes of obesity can be grouped into syndromic, monogenic, and polygenic causes and should be assessed in children with extreme obesity before the age of 5 years, hyperphagia, or a family history of extreme obesity. A microarray study, an analysis of the melanocortin type 4 receptor gene mutations and leptin levels should be performed for this purpose. There are three therapeutic levels: lifestyle modifications, pharmacological treatment, and bariatric surgery. CONCLUSIONS: Genetic study technologies are in constant development; however, we are still far from having a personalized approach to genetic causes of obesity. A significant proportion of the affected individuals are associated with genetic causes; however, there are still barriers to its approach, as it continues to be underdiagnosed. Video Abstract (MP4 1041807 KB).

Urea como opción terapéutica en síndrome de antidiuresis inadecuada

El síndrome de antiduresis inadecuada (SADI) se produce por una alteración en el eje hipotálamo-neurohipófisis, por una falla en la respuesta regulatoria osmótica o por factores no osmóticos, con complicaciones en relación directa al grado de hiponatremia e impacto en la calidad de vida y mortalidad del paciente. El tratamiento consiste en la normalización de la natremia, y la búsqueda de la etiología. Presentamos el caso de un paciente adulto con el diagnóstico de SADI idiopático y trastorno neurocognitivo asociado, con respuesta favorable al tratamiento con urea.

Syndrome of Inappropriate Antidiuresis (SIAD), is produced by an alteration in the hypothalamus-neurohypophysis axis due to a failure in the osmotic regulatory response or non-osmotic factors, with complications directly related to the degree of hyponatremia and impact on quality of life and patient mortality. Management consists of normalization of natremia, and the search for the underlying etiology. We present the case of an adult patient diagnosed with idiopathic SIAD and associated neurocognitive disorder, with a favorable response to treatment with urea.

Imatinib use in the management of a patient with Doege-Potter syndrome.

Summary: Doege-Potter syndromeis a paraneoplastic syndrome characterized by nonislet cell tumor hypoglycemia due to a solitary fibrous tumor, which produces insulin-like growth factor II. In this report, we present the case of a 67-year-old male with recurrent and refractory hypoglycemia due to DPS successfully treated with imatinib. He initially presented with neuroglycopenic symptoms and dyspnea secondary to a giant tumor in the left hemithorax, which was totally resected. During follow-up, 7 years later, he presented with thoracoabdominal tumor recurrence associated with severe hypoglycemia and underwent subtotal tumor resection, with a subsequent improvement of symptoms. The following year, he had a recurrence of his intra-abdominal tumor, which was unresectable, associated with severe hypoglycemia refractory to dextrose infusion and corticosteroids, thus receiving imatinib with a favorable response. The clinical presentation, diagnostic approach, progression of the disease, and response to treatment with imatinib in the management of a patient with large, recurrent, and unresectable mesenchymal tumors with insulin-like growth factor-2 secretion causing hypoglycemiahighlight the importance of this case report. Learning points: Doege-Potter syndrome (DPS) is a rare cause of tumoral hypoglycemia of non-pancreatic origin. Some malignant or benignant neoplasms have ectopic secretion of insulin-like growth factor-2. Total surgical removal is the principal treatment in patients with DPS. Tyrosine kinase inhibitors management in DPS may contribute to improved tumor control in patients with unresectable tumors and severe hypoglycemia.

Thyroid dysfunction and female infertility. A comprehensive review.

INTRODUCTION: Female infertility is defined as the inability to achieve pregnancy following one year of consistent, unprotected sexual intercourse. Among the various endocrine factors contributing to this complex issue, thyroid dysfunction assumes a pivotal and noteworthy role. METHODS: A narrative review, encompassing 134 articles up to 2023, was conducted utilizing the PubMed/Medline, EMBASE, and Scielo databases. The primary focus of this review was to investigate the effects of thyroid dysfunction on female infertility. RESULTS: Thyroid disorders exert a significant influence on folliculogenesis, fertilization, and implantation processes. Thyroid autoimmunity, although associated with diminished ovarian reserve, does not typically necessitate levothyroxine therapy. On the other hand, both subclinical and overt hypothyroidism often require levothyroxine treatment to enhance fertility and optimize obstetric outcomes. Hyperthyroidism warrants prompt intervention due to its heightened risk of miscarriage. Furthermore, thyroid dysfunction exerts notable effects on assisted reproductive technologies, underscoring the importance of achieving euthyroidism prior to ovarian stimulation. CONCLUSION: Women presenting with thyroid dysfunction must undergo meticulous and individualized assessments since fertility outcomes, whether achieved through natural conception or assisted reproductive technologies, can be significantly influenced by thyroid-related factors.

Refractory Hypothyroidism: Unraveling the Complexities of Diagnosis and Management.

INTRODUCTION: Refractory hypothyroidism (RH) represents a challenge in the diagnosis and treatment within the field of thyroidology. It is defined as the inability to achieve disease control despite using levothyroxine (LT4) doses of 1.9 µg/kg/d or higher. METHODS: A comprehensive review, encompassing 103 articles, was conducted using the Scielo, Scopus, and EMBASE databases, providing an approach to evaluation and diagnosis of this condition. RESULTS: LT4 disintegrates and dissolves within an acidic gastric environment before being absorbed in the jejunum and ileum. It then extensively binds to serum transporter proteins and undergoes deiodination to yield tri-iodothyronine, the biologically active hormone. There are various nonpathological causes of RH, such as noncompliance with treatment, changes in the brand of LT4, food and drug interferences, as well as pregnancy. Pathological causes include lactose intolerance, Helicobacter pylori infection, giardiasis, among others. The diagnosis of RH involves conducting a thorough medical history and requesting relevant laboratory tests to rule out causes of treatment resistance. The LT4 absorption test allows for the identification of cases of malabsorption. The treatment of RH involves identifying and addressing the underlying causes of noncompliance or malabsorption. In cases of pseudomalabsorption, supervised and weekly administration of LT4 may be considered. DISCUSSION: Early recognition of RH and correction of its underlying cause are of utmost importance, as this avoids the use of excessive doses of LT4 and prevents cardiovascular and bone complications associated with this condition.

Endocrine factors associated with infertility in women: an updated review.

INTRODUCTION: Infertility is defined as the inability to conceive after unprotected sexual intercourse for at least 12 consecutive months. Our objective is to present an updated narrative review on the endocrine causes of infertility in women. AREAS COVERED: A comprehensive review was conducted using Scielo, Scopus, and EMBASE databases, comprising 245 articles. The pathophysiology of infertility in women was described, including endocrinopathies such as hypothalamic amenorrhea, hyperprolactinemia, polycystic ovary syndrome, primary ovarian insufficiency, obesity, thyroid dysfunction, and adrenal disorders. The diagnostic approach was outlined, emphasizing the necessity of hormonal studies and ovarian response assessments. Additionally, the treatment plan was presented, commencing with non-pharmacological interventions, encompassing the adoption of a Mediterranean diet, vitamin supplementation, moderate exercise, and maintaining a healthy weight. Subsequently, pharmacological treatment was discussed, focusing on the management of associated endocrine disorders and ovulatory dysfunction. EXPERT OPINION: This comprehensive review highlights the impact of endocrine disorders on fertility in women, providing diagnostic and therapeutic algorithms. Despite remaining knowledge gaps that hinder more effective treatments, ongoing research and advancements show promise for improved fertility success rates within the next five years. Enhanced comprehension of the pathophysiology behind endocrine causes and the progress in genetic research will facilitate the delivery of personalized treatments, thus enhancing fertility rates.

Coexistence of Thyrolipomatosis and Tongue Squamous Cell Carcinoma: A Case Report.

Thyrolipomatosis, a diffuse non-neoplastic infiltration of fatty tissue in the thyroid gland, is an extremely rare condition with only about 30 cases reported worldwide. A few of these cases report the concurrency of thyrolipomatosis and malignant neoplasms in the thyroid or colon, but never with tongue cancer. A 44-year-old female patient with an infiltrative tongue mass suggestive of carcinoma presented for an outpatient consultation. Cervical imaging revealed multiple lymphadenopathies and a multinodular goitre with diffuse fatty infiltration, suggestive of thyrolipomatosis. Surgical intervention included partial resection of the tongue and thyroid (left hemiglossectomy and right hemithyroidectomy, respectively) and lymphadenectomy. The thyroid specimen showed diffuse fat metaplasia of the stromal thyroid tissue, confirming incidental thyrolipomatosis. During post-operative follow-up, the patient presented with recurrence of squamous cell carcinoma as indicated by new right-sided thyroid nodules, left-sided lymphadenopathies with confirmatory biopsy, and a growing neck mass that became infected. The patient developed septic shock and later died. Thyrolipomatosis causes thyroid swelling and can be clinically detected as goitres or as an incidental finding. Diagnosis is suggested by cervical imaging (ultrasonography, computed tomography or magnetic resonance), but confirmation is histological after thyroidectomy. Although thyrolipomatosis is benign, it could develop concurrently with neoplastic diseases, especially on embryologically related tissues (e.g. thyroid and tongue). This case report is the first in the literature describing the coexistence between thyrolipomatosis and tongue cancer in an adult Peruvian patient.

Complicaciones cardiovasculares asociadas a la acromegalia. Estado del arte / Cardiovascular complications associated with acromegaly. A state-of-the-art

Resumen La acromegalia es una enfermedad rara, causada principalmente por un tumor hipofisiario secretor de hormona de crecimiento (GH), se caracteriza por tener progresión lenta y asociarse a un compromiso multisistémico, siendo el aparato cardiovascular uno de los más comprometidos, llegando, incluso, desde hace más de 10 años a representar la principal causa de muerte. Las complicaciones cardiovasculares se desarrollan como resultado de las concentraciones sanguíneas elevadas de la GH y del factor de crecimiento similar a la insulina tipo 1 (IGF-1), los cuales ejercen efectos directos e indirectos sobre el endotelio, grandes vasos, riñón y los cardiomiocitos; produciendo hipertensión arterial, enfermedad valvular, arritmia cardiaca y una cardiopatía propia de la acromegalia denominada miocardiopatía acromegálica. Luego de la revisión bibliográfica actualizada relacionada con la fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento del compromiso cardiovascular, encontramos que desde el punto de vista cardiovascular los pacientes con acromegalia pueden variar desde un estado asintomático hasta presentar disfunción cardiaca severa, siendo los marcadores bioquímicos y exámenes de imagen herramientas diagnósticas que permiten evaluar el grado de afección cardiovascular para poder brindar un tratamiento individualizado. La normalización de los valores de la GH y del IGF-1 mejora los parámetros cardiovasculares y, por tanto, su pronóstico.

Abstract Acromegaly is a rare disease, mainly caused by a pituitary tumor secreting growth hormone. It is characterized by slow progression and is associated with a multisystemic involvement, being the cardiovascular system, one of the most involved, even reaching, more than 10 years ago, to represent the main cause of death. Cardiovascular complications develop as a result of elevated blood concentrations of growth hormone and insulin-like growth factor 1, which exert direct and indirect effects on the endothelium, large vessels, kidney and cardiomyocytes; causing arterial hypertension, valve disease, cardiac arrhythmia and a specific heart disease called acromegalic cardiomyopathy. After the literature overview related to the pathophysiology, clinical manifestations, diagnosis and treatment of cardiovascular involvement, we found that from a cardiovascular point of view, patients with acromegaly can range from an asymptomatic state to severe cardiac dysfunction, being the biochemical markers and imaging studies diagnostic tools that allow assessment the degree of cardiovascular disease in order to provide individualized treatment. The normalization of growth hormone and insulin-like growth factor 1 levels improves cardiovascular parameters, and therefore its prognosis.

Diagnosis and management of small bowel neuroendocrine tumors: A state-of-the-art.

This review provides an update on the epidemiology, pathophysiology, symptoms, diagnosis and treatment of neuroendocrine neoplasms (NENs) of the small bowel (SB). These NENs are defined as a group of neoplasms deriving from neuroendocrine cells. NENs are currently the most common primary tumors of the SB, mainly involving the ileum, making the SB the most frequently affected part of the gastrointestinal tract. SB NENs by definition are located between the ligament of Treitz and the ileocecal valve. They are characterized by small size and induce an extensive fibrotic reaction in the small intestine including the mesentery, resulting in narrowing or twisting of the intestine. Clinical manifestations of bowel functionality are related to the precise location of the primary tumor. The majority of them are non-functional NENs and generally asymptomatic; in an advanced stage, NENs present symptoms of mass effect by non-specific abdominal pain or carcinoid syndrome which appears in patients with liver metastasis (around 10%). The main manifestations of the carcinoid syndrome are facial flushing (94%), diarrhea (78%), abdominal cramps (50%), heart valve disease (50%), telangiectasia (25%), wheezing (15%) and edema (19%). Diagnosis is made by imaging or biochemical tests, and the order of request will depend on the initial diagnostic hypothesis, while confirmation will always be histological. All patients with a localized SB NEN with or without near metastasis in the mesentery are recommended for curative resection. Locoregional and distant spread may be susceptible to several therapeutic strategies, such as chemotherapy, somatostatin analogs and palliative resection.

Glucemia de ingreso asociada a mortalidad y estancia hospitalaria en un servicio multidisciplinario de un hospital nacional peruano

Objetivo: Evaluar la asociación entre glucemia de ingreso y desenlaces adversos en pacientes hospitalizados con COVID-19 en un hospital nacional peruano. Métodos: Estudio observacional tipo cohorte retrospectiva. Se revisaron historias clínicas electrónicas de pacientes hospitalizados por COVID-19 en un servicio de especialidades. Los pacientes se clasificaron según niveles de glucemia al ingreso: ≤ y >140mg/dL; el desenlace primario fue mortalidad y el secundario un compuesto que incluyó mortalidad, shock séptico, ventilación mecánica o traslado a UCI. Se evaluó la estancia hospitalaria y se realizó un subanálisis de regresión logística multivariada en pacientes diabéticos. Resultados: Se evaluaron 169 pacientes, media de edad 61 años, 64.5% varones. 71% presentaban alguna comorbilidad, siendo las más frecuentes: hipertensión arterial (34%), obesidad (30%), diabetes (26%). El 70% presentó gravedad tomográfica. La mediana de glucemia de ingreso fue 126.5mg/dL (RIC: 109-157mg/dL), uno de cada 3 presentó glucemia >140mg/dL. La tasa de mortalidad fue 9700 muertes por cada 100 000 personas-semana, con frecuencia de 21.3%. No se encontró diferencia significativa entre hiperglucemia y normoglucemia, tanto en mortalidad como desenlace compuesto. Los pacientes con hiperglucemia de ingreso presentaron mayor estancia hospitalaria que los normoglucémicos (19 días vs 13 días, p180mg/dL presentó OR de 6.42 (IC95%: 1.07-38.6), ajustado a edad y a gravedad clínica de ingreso. Conclusiones: La hiperglucemia al ingreso se asoció a mayor estancia hospitalaria, y los pacientes diabéticos con hiperglucemia >180mg/dL presentaron un riesgo 6 veces mayor de presentar desenlace adverso.

Objective: Evaluate the association between glycemia on admission and adverse outcomes in hospitalized patients with COVID19 in a Peruvian national hospital. Methods: Retrospective, observational cohort study. We collected data from electronic medical records of COVID19 patients in a medical specialties service. Patients were classified according to blood glucose levels on admission: ≤ and >140mg/dL. Primary outcome was mortality, and the secondary a composite that included mortality, septic shock, mechanical ventilation, or transfer to ICU. We also evaluated hospital stay and a multivariate logistic regression sub analysis was performed in diabetic patients. Results: 169 patients were evaluated. The mean age was 61 years, 64.5% were male. 71% had at least one comorbidity, the most frequent: arterial hypertension (34%), obesity (30%) and diabetes (26%). 70% presented tomographic gravity. Median blood glucose at admission was 126.5mg/dL (IQR: 109-157mg/dL), one of every 3 had blood glucose levels >140mg/dL. Mortality rate was 9700 deaths per 100000 person-weeks, with a frequency of 21.3%. No significant difference was found between hyperglycemia and normoglycemia, mortality and in composite outcome. Patients with hyperglycemia on admission had longer hospital stay than normoglycemic patients (19 vs 13 days, p180mg/dL presented OR of 6.42 (95% CI: 1.07-38.6) for composite outcome, adjusted for age and clinical severity at admission. Conclusions: Hyperglycemia at admission was associated with a longer hospital stay and diabetic patients with hyperglycemia >180mg/dL had a 6-fold increased risk of presenting an adverse outcome.

[Clinical-epidemiological profile of patients with thyrotoxic periodic paralysis in two peruvian hospitals]. / Perfil clínico-epidemiológico de pacientes con parálisis periódica tirotóxica en dos hospitales peruanos.

Background: Thyrotoxic Periodic Paralysis (PPT) is an uncommon complication of hyperthyroidism, it is the most frequent cause of acute flaccid paralysis in adults. Material and methods: A retrospective observational study was carried out in 2 reference hospitals of the social security in Lima-Peru, which included 22 patients diagnosed with PPT during the period 2014-2021. Results: the average age at diagnosis was 35.77 ± 9.6 years, all of mixed race, in 82% of the patients the diagnosis of hyperthyroidism was established from this entity, the etiology in 95% was autoimmune (Graves-Basedow) except for one whose etiology was toxic multinodular goiter. The triggering event reported in 54% of patients was the intake of copious food high in carbohydrates, followed by exercise (27%), the most frequent presentation time was during the morning (41% of the cases), the main weakness pattern compromised lower limbs (45% paraplegia, 18% paraparesis), only 36% were diagnosed with PPT in their first episode of motor weakness. Conclusions: We consider that this condition should be suspected in any young male patient of any ethnicity with acute muscle weakness, associated with low serum potassium levels and symptoms of thyrotoxicosis, although its absence should not rule out the diagnosis. The precipitating factor should be identified as much as possible and initial therapy with propanolol with or without intravenous or oral potassium replacement should be established, with adequate subsequent monitoring to minimize the risk of rebound hyperkalemia.

Introducción: la paralisis periodica tirotóxica (PPT) es una complicación poco común del hipertiroidismo, corresponde la causa más frecuente de parálisis aguda flácida del adulto. Material y métodos: se realizó un estudio observacional retrospectivo en 2 hospitales referenciales del seguro social en Lima-Perú, que incluyo a 22 pacientes (21 hombres, 1 mujer) diagnosticados con PPT durante el periodo 2014-2021. Resultados: la edad promedio al diagnóstico fue de 35.77 ± 9.6 años, todos de raza mestiza, en 82% de los pacientes el diagnostico de hipertiroidismo fue establecido a partir de esta entidad, la etiología en el 95% fue autoinmune (enfermedad de Graves-Basedow) excepto uno cuya etiología fue bocio multinodular tóxico. El evento desencadenante reportado en 54% de pacientes fue la ingesta de comida copiosa alta en carbohidratos, seguido del ejercicio (27%), el horario más frecuente de presentación fue durante la mañana ( 41% de los casos), el patrón de debilidad principal comprometió miembros inferiores(45% paraplejia, 18% paraparesia), solo 36% fue diagnosticado de PPT en su primer episodio de debilidad motora. Conclusiones: consideramos que se debe sospechar esta condición en cualquier paciente varón joven de cualquier etnia con debilidad muscular aguda, asociado a niveles séricos bajos de potasio y síntomas de tirotoxicosis, aunque su ausencia no debe descartar el diagnostico. Se debe identificar en lo posible el factor precipitante e instaurar terapia inicial con propanolol con o sin reemplazo de potasio endovenoso u oral, con monitoreo posterior adecuado que minimice el riesgo de hiperpotasemia de rebote.

Pleuropericardial compromise associated with Graves' disease - a diagnostic challenge.

Not required for Clinical Vignette.

[Cardiovascular complications associated with acromegaly. A state-of-the-art]. / Complicaciones cardiovasculares asociadas a la acromegalia. Estado del arte.

Acromegaly is a rare disease, mainly caused by a pituitary tumor secreting growth hormone. It is characterized by slow progression and is associated with a multisystemic involvement, being the cardiovascular system, one of the most involved, even reaching, more than 10 years ago, to represent the main cause of death. Cardiovascular complications develop as a result of elevated blood concentrations of growth hormone and insulin-like growth factor 1, which exert direct and indirect effects on the endothelium, large vessels, kidney and cardiomyocytes; causing arterial hypertension, valve disease, cardiac arrhythmia and a specific heart disease called acromegalic cardiomyopathy. After the literature overview related to the pathophysiology, clinical manifestations, diagnosis and treatment of cardiovascular involvement, we found that from a cardiovascular point of view, patients with acromegaly can range from an asymptomatic state to severe cardiac dysfunction, being the biochemical markers and imaging studies diagnostic tools that allow assessment the degree of cardiovascular disease in order to provide individualized treatment. The normalization of growth hormone and insulin-like growth factor 1 levels improves cardiovascular parameters, and therefore its prognosis.

La acromegalia es una enfermedad rara, causada principalmente por un tumor hipofisiario secretor de hormona de crecimiento (GH), se caracteriza por tener progresión lenta y asociarse a un compromiso multisistémico, siendo el aparato cardiovascular uno de los más comprometidos, llegando, incluso, desde hace más de 10 años a representar la principal causa de muerte. Las complicaciones cardiovasculares se desarrollan como resultado de las concentraciones sanguíneas elevadas de la GH y del factor de crecimiento similar a la insulina tipo 1 (IGF-1), los cuales ejercen efectos directos e indirectos sobre el endotelio, grandes vasos, riñón y los cardiomiocitos; produciendo hipertensión arterial, enfermedad valvular, arritmia cardiaca y una cardiopatía propia de la acromegalia denominada miocardiopatía acromegálica. Luego de la revisión bibliográfica actualizada relacionada con la fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento del compromiso cardiovascular, encontramos que desde el punto de vista cardiovascular los pacientes con acromegalia pueden variar desde un estado asintomático hasta presentar disfunción cardiaca severa, siendo los marcadores bioquímicos y exámenes de imagen herramientas diagnósticas que permiten evaluar el grado de afección cardiovascular para poder brindar un tratamiento individualizado. La normalización de los valores de la GH y del IGF-1 mejora los parámetros cardiovasculares y, por tanto, su pronóstico.

Carcinoma de paratiroides en un paciente con hiperparatiroidismo terciario / Parathyroid carcinoma in a patient with tertiary hyperparathyroidism

Introducción: La mayoría de los pacientes con enfermedad renal crónica terminal en hemodiálisis desarrollan hiperparatiroidismo secundario. Algunas veces este tipo de hiperparatiroidismo secundario suele ser severo y refractario al tratamiento médico, inclusive algunos desarrollarán hiperparatiroidismo terciario, el tratamiento de ambas situaciones suele ser la paratiroidectomía subtotal o total. Reporte de Caso: Presentamos el caso de un paciente de 29 años, con enfermedad renal terminal en hemodiálisis durante 13 años, quien desarrolló hiperparatiroidismo terciario por lo que fue sometido a paratiroidectomía subtotal con autotrasplante de media glándula; el estudio patológico informó la presencia de un carcinoma de paratiroides en una de las cuatro glándulas paratiroides extirpadas. Conclusión: El carcinoma de paratiroides es una neoplasia rara que causa hiperparatiroidismo primario en menos de 1% de todos los casos, y se ha reportado en algunos pacientes operados por hiperparatiroidismo terciario.

Background: Most patients with terminal chronic kidney disease on hemodialysis develop secondary hyperparathyroidism. Sometimes this type of secondary hyperparathyroidism becomes severe and refractory to medical treatment, some will even develop tertiary hyperparathyroidism, the treatment of both situations is usually subtotal or total parathyroidectomy. Case Report: We present the case of a 29-year-old patient with end-stage kidney disease on hemodialysis for 13 years, who developed tertiary hyperparathyroidism, for which he underwent subtotal parathyroidectomy with half gland autotransplantation; the pathological study reported the presence of a parathyroid carcinoma in one of the four excised parathyroid glands. Conclusion: Parathyroid carcinoma is a rare neoplasm that causes primary hyperparathyroidism in less than 1% of all cases, and has been reported in some patients operated on for the development of tertiary hyperparathyroidism.

Assessment of hormonal status in male infertility. An update.

BACKGROUND AND AIMS: The prevalence and incidence of infertility are increasing worldwide; they are associated with a significant economic and social impact. Infertility is defined as the inability to achieve pregnancy after 12 months or more of regular unprotected sex. In recent times, the male factor has gained importance and currently it contributes to approximately 50% of infertility cases. Multiple etiologies are stated, such as metabolic, anatomical, genetic or even idiopathic causes; however, the main cause is semen abnormalities. The aim of this manuscript is to provide a complete review of hormonal assessment of male infertility, as well as to review the physiology and pathophysiology related to the male gonadal axis. METHODS: This study is a narrative abstract carried out on basis of systematic bibliographic review, using articles indexed in PubMed/Medline, Scopus, Embase and Scielo, which were published during the last 20 years. RESULTS: The cornerstone of the evaluation of the hormonal status is semen analysis. Clinicians must rule out hypogonadism in those patients who present oligospermia and azoospermia, by determining levels of testosterone and gonadotropins, which provide the functionality status of the hypothalamic-pituitary-testicular axis. Evaluation of the adrenal, thyroid, and lactotroph axis are indicated in those patients with central hypogonadism. CONCLUSIONS: Despite advances in the diagnosis of male infertility, some causes are not fully understood, therefore, it is crucial to perform a timely hormonal evaluation of the male factor in infertile couples, in order to provide adequate treatment and improve fertility rates.

Tratamiento del hiperaldosteronismo primario durante el embarazo: reporte de un caso / Management of primary hyperaldosteronism during pregnancy: Case report

Resumen ANTECEDENTES: El hiperaldosteronismo primario es la principal causa de hipertensión arterial de origen endocrino en la población general; su manifestación durante el embarazo es poco frecuente, casi siempre provocado por un adenoma adrenal productor de aldosterona. De 1962 a la fecha se han descrito alrededor de 50 casos. Objetivo: Reportar un caso de hiperaldosteronismo durante el embarazo y revisar la bibliografía relacionada. CASO CLÍNICO: Paciente de 39 años, con antecedente médico de hipertensión arterial, en curso del segundo trimestre del quinto embarazo, que acudió a consulta por hipertensión no controlada e hipocalemia. El tratamiento incluyó un antagonista del receptor de aldosterona, que permitió el control de la tensión arterial y la finalización del embarazo. Posteriormente se identificó un nódulo adrenal, cuya resección resultó en normalización de las concentraciones de aldosterona, la actividad de renina plasmática, calemia y remisión de la hipertensión hasta la actualidad. CONCLUSIÓN: Las recomendaciones para el tratamiento de pacientes con hiperaldosteronismo durante el embarazo se basan en los casos publicados y los datos de toxicidad generados de estudios en animales. De ahí la importancia de este caso, que aporta información importante y puede considerarse en situaciones similares.

Abstract BACKGROUND: Primary hyperaldosteronism is the main cause of arterial hypertension of endocrine origin in the general population; its presentation during pregnancy is infrequent; having described about 50 cases since 1962, the most common cause is the presence of an adrenal adenoma. OBJECTIVE: To report the first case of hyperaldosteronism treated during pregnancy in Peru and reviews the literature. CLINICAL CASE: A 39-year-old patient with a history of arterial hypertension and poor obstetric history, who was referred to our center in the second trimester of the 5th pregnancy due to uncontrolled hypertension and symptomatic hypokalemia. Management included an aldosterone receptor antagonist, allowing the control of blood pressure and the culmination of the pregnancy with the delivery of a healthy girl. Subsequently, the presence of an adrenal nodule was confirmed, the resection of which resulted in normalization of aldosterone concentration, plasma renin activity, kalemia, and remission of hypertension to date. CONCLUSION: Recommendations on the management of hyperaldosteronism during pregnancy are based only on published cases and drug toxicity data were generated in animal studies. Hence the importance of this report, which provides information that can be considered in similar situations.

Liothyronine use in primary hypothyroidism - current concepts.

Hypothyroidism is an endocrine disorder whose management raises many challenges in clinical practice. Its standard treatment is levothyroxine (LT4). The goal of the treatment is to normalize signs and symptoms, as well as to achieve thyroid-stimulating hormone (TSH) concentrations within the reference range, on an individual basis. It is known that 5-10% of hypothyroid patients remain symptomatic, despite achieving the target TSH levels, which, in turn, affects their quality of life. After ruling out other causes of non-thyroid origin for this persistence, it is suggested that these patients could benefit from the use of liothyronine (LT3), added to LT4, especially if polymorphism of the deiodinase 2 (D2) genes is documented. There exist a variety of LT3 preparations, whose concentrations vary from 5 to 50 ug, with the recommended LT4/LT3 ratio of 13:1-20:1. The goals of combination therapy should be to achieve a physiological ratio of free triiodothyronine/free thyroxine (FT3/FT4) and non-suppression of TSH. Because there is currently no guide that makes evidence-based recommendations on the use of LT3 in primary hypothyroidism, more clinical studies are needed to be able to identify hypothyroid patients who may benefit from the use of LT3, by identifying new biomarkers.

Síndrome poliuria - polidipsia de causa autoinmune en el posparto de una mujer de 27 años

La hipofisitis autoinmune es un cuadro caracterizado por la infiltración linfocítica de la hipófisis que produce deficiencia de una o más hormonas, tanto de la adenohipófisis como de la neurohipófisis. Para el diagnóstico, es necesario un alto índice de sospecha, más aún considerando la relación temporal con el embarazoo el parto. Las características clínicas e imagenológicas sugieren el diagnóstico. A pesar que el diagnóstico definitivo es por biopsia, esta no se suele realizar por los potenciales efectos adversos del procedimiento. Presentamos un caso que describe la forma de manifestación de la enfermedad y las características imagenológicas típicas en la resonancia magnética nuclear.

Autoimmune hypophysitis is a condition characterized by lymphocytic infiltration of the pituitary resulting in deficiency of one or more hormones of both the adenohypophysis and the neurohypophysis. For diagnosis, a high index of suspicion is necessary, even more so considering the temporal relationship with pregnancy or childbirth. Clinical and imaging features are suggestive of the diagnosis. Although the definitive diagnosis is by biopsy, this is not usually performed because of the potential adverse effects of the procedure. We present a case describing the form of manifestation of the disease and the typical imaging features on magnetic resonance imaging.

[Bilateral adrenal incidentaloma in a patient with acute appendicitis]. / Incidentaloma adrenal bilateral en un paciente con apendicitis aguda.

BACKGROUND: Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals . CLINICAL CASE: We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade. CONCLUSIONS: In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.

INTRODUCCIÓN: los incidentalomas adrenales son tumores que suelen ser detectados en estudios de imágenes indicados sin sospecha de enfermedad adrenal. El feocromocitoma es un tumor neuroendocrino que puede presentarse esporádicamente o asociado a síndromes genéticos, habiendo sido descrito en 0.1-5% de pacientes con neurofibromatosis tipo 1, que es una enfermedad progresiva multisistémica de herencia autosómica dominante que afecta a 1/2600-3000 individuos. CASO CLÍNICO: se reporta el caso de un paciente de 50 años quien ingresó al departamento de Emergencia por apendicitis aguda y cuya tomografía mostró la presencia de masas adrenales bilaterales. Luego de la intervención quirúrgica fue hospitalizado para estudio. El paciente negó cefalea, diaforesis, hipertensión arterial, palpitaciones y pérdida de peso; en el examen físico se evidenció presencia de múltiples lentígines, manchas café con leche > 15 mm, lesiones nodulares fijas y pedunculadas, compatibles con neurofibromas focales, sin signos sugestivos de hipo o hipercortisolismo. En la analítica se identificaron metanefrinas totales y ácido vanilil-mandélico en orina encontrándose en valores muy elevados, siendo sometido a adrenalectomía laparoscópica en 2 tiempos, luego de una adecuada preparación que incluyó bloqueo alfa y beta adrenérgicos. CONCLUSIONES: en los pacientes con masas adrenales es necesario reconocer la importancia de una evaluación clínica integral para guiar un adecuado estudio diagnóstico, así como la realización de un óptimo estudio preoperatorio que incluye las pruebas hormonales para descartar funcionalidad.