Osteochondroplastic tracheobronchopathy: Four case reports. / Traqueobroncopatía osteocondroplástica: reporte de cuatro casos clínicos.

Introduction: Osteochondroplastic tracheobronchopathy is a rare benign chronic disease of unknown etiology. Bronchoscopy remains the gold standard for diagnosing osteochondroplastic tracheobronchopathy. Its typical findings are described as a cobblestone, rock garden, mountainscape, or stalactite cave appearance. The present work aims to show the main clinical features of this rare pathology. Clinical cases: The clinical data of four middle-aged patients, three men and one woman, were analyzed. The main clinical symptoms were chronic cough, dyspnea, and dysphonia. The patient's preliminary diagnosis was made by computed axial tomography of the chest, confirmed by bronchoscopy and histopathological examination. Treatment included medication for symptoms and, in one case, cryosurgery and argon plasma coagulation. Discussion: Diagnosing osteochondroplastic tracheobronchopathy was not easy, given its uncommon nature and non-specific symptoms often found in other pathologies. No case series articles on this pathology have been published in Peru. Therefore, we used the original articles published in other countries to reference our findings. Conclusion: Osteochondroplastic tracheopathy is a benign disease that typically affects adults. Men are more likely to be affected. Its clinical manifestations are non-specific and frequently of pharyngeal origin, and the cause is not yet defined. Chest computed axial tomography combined with bronchoscopy are the main diagnostic procedures. There is no standard treatment with consistent therapeutic effects.

Introducción: La traqueobroncopatía osteocondroplástica es una rara enfermedad crónica benigna de etiología desconocida. La broncoscopía sigue siendo el estándar de oro para el reconocimiento de traqueopatía osteocondroplástica. Sus hallazgos típicos se describen como un empedrado, un jardín de rocas, una apariencia de paisaje montañoso o de una cueva con estalactitas. El objetivo del presente trabajo es mostrar las principales características clínicas de una patología poco conocida. Casos clínicos: Se analizaron los datos clínicos de cuatro pacientes de mediana edad, tres fueron hombres y una mujer. Los principales síntomas clínicos fueron tos crónica, disnea, disfonía. Los pacientes tuvieron un diagnóstico preliminar mediante tomografía axial computarizada de tórax, confirmado por examen video broncoscópico e histopatológico. El tratamiento incluyó medicamentos para los síntomas y en un solo caso criocirugía y coagulación con argón plasma. Discusión: El diagnóstico de traqueobroncopatía osteocondroplástica no fue sencillo por ser una entidad rara, cuyos síntomas son inespecíficos y muy frecuentes en otras patologías. En Perú no se han publicado artículos de serie de casos sobre esta patología. Por lo tanto, tomamos como referencia artículos originales publicados en otros países para compararlos con nuestros hallazgos. Conclusión: La traqueopatía osteocondroplástica es una enfermedad benigna que predispone a los adultos, los hombres tienen más probabilidades de verse afectados. Sus manifestaciones clínicas son inespecíficas; frecuentemente de origen faríngeo y la causa no está aún definida. La tomografía axial computarizada de tórax combinada con video broncoscopía son los principales procedimientos para el diagnóstico. No existe un estándar de tratamiento con efectos terapéuticos consistentes.

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.

Traqueobroncopatía osteocondroplástica: reporte de cuatro casos clínicos / Osteochondroplastic tracheobronchopathy: Four case reports

Introducción La traqueobroncopatía osteocondroplástica es una rara enfermedad crónica benigna de etiología desconocida. La broncoscopía sigue siendo el estándar de oro para el reconocimiento de traqueopatía osteocondroplástica. Sus hallazgos típicos se describen como un empedrado, un jardín de rocas, una apariencia de paisaje montañoso o de una cueva con estalactitas. El objetivo del presente trabajo es mostrar las principales características clínicas de una patología poco conocida. Casos clínicos Se analizaron los datos clínicos de cuatro pacientes de mediana edad, tres fueron hombres y una mujer. Los principales síntomas clínicos fueron tos crónica, disnea, disfonía. Los pacientes tuvieron un diagnóstico preliminar mediante tomografía axial computarizada de tórax, confirmado por examen video broncoscópico e histopatológico. El tratamiento incluyó medicamentos para los síntomas y en un solo caso criocirugía y coagulación con argón plasma. Discusión El diagnóstico de traqueobroncopatía osteocondroplástica no fue sencillo por ser una entidad rara, cuyos síntomas son inespecíficos y muy frecuentes en otras patologías. En Perú no se han publicado artículos de serie de casos sobre esta patología. Por lo tanto, tomamos como referencia artículos originales publicados en otros países para compararlos con nuestros hallazgos. Conclusión La traqueopatía osteocondroplástica es una enfermedad benigna que predispone a los adultos, los hombres tienen más probabilidades de verse afectados. Sus manifestaciones clínicas son inespecíficas; frecuentemente de origen faríngeo y la causa no está aún definida. La tomografía axial computarizada de tórax combinada con video broncoscopía son los principales procedimientos para el diagnóstico. No existe un estándar de tratamiento con efectos terapéuticos consistentes.

Introduction Osteochondroplastic tracheobronchopathy is a rare benign chronic disease of unknown etiology. Bronchoscopy remains the gold standard for diagnosing osteochondroplastic tracheobronchopathy. Its typical findings are described as a cobblestone, rock garden, mountainscape, or stalactite cave appearance. The present work aims to show the main clinical features of this rare pathology. Clinical cases The clinical data of four middle-aged patients, three men and one woman, were analyzed. The main clinical symptoms were chronic cough, dyspnea, and dysphonia. The patient's preliminary diagnosis was made by computed axial tomography of the chest, confirmed by bronchoscopy and histopathological examination. Treatment included medication for symptoms and, in one case, cryosurgery and argon plasma coagulation. Discussion Diagnosing osteochondroplastic tracheobronchopathy was not easy, given its uncommon nature and non-specific symptoms often found in other pathologies. No case series articles on this pathology have been published in Peru. Therefore, we used the original articles published in other countries to reference our findings. Conclusion Osteochondroplastic tracheopathy is a benign disease that typically affects adults. Men are more likely to be affected. Its clinical manifestations are non-specific and frequently of pharyngeal origin, and the cause is not yet defined. Chest computed axial tomography combined with bronchoscopy are the main diagnostic procedures. There is no standard treatment with consistent therapeutic effects.

Drug-induced hypothyroidism in tuberculosis.

INTRODUCTION: Adverse reactions to tuberculosis treatment can impact patient adherence and prognosis. Hypothyroidism is a frequent adverse reaction caused using ethionamide, prothionamide, and para-aminosalicylic acid and is often underdiagnosed. AREAS COVERED: We searched Scielo, Scopus, and EMBASE databases, including 67 articles. Antitubercular drug-induced hypothyroidism has a prevalence of 17%. It occurs after 2 to 3 months of treatment and resolves within 4 to 6 weeks after discontinuation. It is postulated to result from the inhibition of thyroperoxidase function, blocking thyroid hormone synthesis. Symptoms are nonspecific, necessitating individualized thyroid-stimulating hormone measurement for detection. Specific guidelines for management are lacking, but initiation of treatment with levothyroxine, as is customary for primary hypothyroidism, is recommended. Discontinuation of antitubercular drugs is discouraged, as it may lead to unfavorable consequences. EXPERT OPINION: Antitubercular drug-induced hypothyroidism is more common than previously thought, affecting one in six MDR-TB patients. Despite diagnostic and treatment recommendations, implementation is hindered in low-income countries due to the lack of certified laboratories. New drugs for tuberculosis treatment may affect thyroid function, requiring vigilant monitoring for complications, including hypothyroidism.

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).

Ifosfamide-induced nephrotoxicity in oncological patients.

INTRODUCTION: Ifosfamide is an alkylating chemotherapeutic agent used in the treatment of various neoplasms. Its main adverse effects include renal damage. AREAS COVERED: A comprehensive review was conducted, including 100 articles from the Scielo, Scopus, and EMBASE databases. Ifosfamide-induced nephrotoxicity is attributed to its toxic metabolites, such as acrolein and chloroacetaldehyde, which cause mitochondrial damage and oxidative stress in renal tubular cells. Literature review found a 29-year average age with no gender predominance and a mortality of 13%. Currently, no fully effective strategy exists for preventing ifosfamide-induced nephrotoxicity; however, hydration, forced diuresis, and other interventions are employed to limit renal damage. Long-term renal function monitoring is essential for patients treated with ifosfamide. EXPERT OPINION: Ifosfamide remains essential in neoplasm treatment, but nephrotoxicity, often compounded by coadministered drugs, poses diagnostic challenges. Preventive strategies are lacking, necessitating further research. Identifying timely risk factors can mitigate renal damage, and a multidisciplinary approach manages established nephrotoxicity. Emerging therapies may reduce ifosfamide induced nephrotoxicity.

Ifosfamide is a type of chemotherapy used to treat different types of cancers. However, one of its main side effects is kidney damage. Researchers reviewed 100 articles from medical databases to understand how ifosfamide affects the kidneys. The kidney damage is caused by harmful substances produced when ifosfamide is broken down in the body. These substances can harm the cells in the kidneys. Studies have shown that 13% of the patients treated with ifosfamide can die. Currently, there is no perfect way to prevent kidney damage from ifosfamide, but doctors try to protect the kidneys by giving patients plenty of fluids and using other treatments, so it's important for patients who receive ifosfamide to have their kidney function checked regularly. Although ifosfamide is effective against cancer, its potential kidney side effects should be carefully considered by doctors when deciding on the best treatment for each patient.

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.

Cushing disease in pediatrics: an update.

Cushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood, the main features of CS are facial changes, rapid or exaggerated weight gain, hirsutism, virilization, and acne. Endogenous hypercortisolism should be established after exogenous CS has been ruled out based on 24-hour urinary free cortisol, midnight serum or salivary cortisol, and dexamethasone suppression test; after that, ACTH dependence should be established. The diagnosis should be confirmed by pathology. The goal of treatment is to normalize cortisol level and reverse the signs and symptoms. Treatment options include surgery, medication, radiotherapy, or combined therapy. CD represents a challenge for physicians owing to its multiple associated conditions involving growth and pubertal development; thus, it is important to achieve an early diagnosis and treatment in order to control hypercortisolism and improve the prognosis. Its rarity in pediatric patients has led physicians to have limited experience in its management. The objective of this narrative review is to summarize the current knowledge about the pathophysiology, diagnosis, and treatment of CD in the pediatric population.

Thyrotoxic Cardiomyopathy: State of the Art.

Thyroid hormones, mainly triiodothyronine, have genomic and non-genomic effects on cardiomyocytes related to the contractile function of the heart. Thyrotoxicosis, which is the set of signs and symptoms derived from the excess of circulating thyroid hormones, leads to increased cardiac output and decreased systemic vascular resistance, increasing the volume of circulating blood and causing systolic hypertension. In addition, the shortening of the refractory period of cardiomyocytes produces sinus tachycardia and atrial fibrillation. This leads to heart failure. Approximately 1% of patients with thyrotoxicosis develop thyrotoxic cardiomyopathy, a rare but potentially fatal form of dilated cardiomyopathy. Thyrotoxic cardiomyopathy represents a diagnosis of exclusion, and prompt identification is crucial as it is a reversible cause of heart failure, and heart function can be recovered after achieving a euthyroid state using antithyroid drugs. Radioactive iodine therapy and surgery are not the best initial therapeutic approach. Moreover, it is important to manage cardiovascular symptoms, for which beta blockers are the first-line therapeutic option.

Diabetic neuropathy: Past, present, and future.

Background: A sedentary lifestyle and an unhealthy diet have considerably increased the incidence of diabetes mellitus worldwide in recent decades, which has generated a high rate of associated chronic complications. Methods: A narrative review was performed in MEDLINE, EMBASES and SciELO databases, including 162 articles. Results: Diabetic neuropathy (DN) is the most common of these complications, mainly producing two types of involvement: sensorimotor neuropathy, whose most common form is symmetric distal polyneuropathy, and autonomic neuropathies, affecting the cardiovascular, gastrointestinal, and urogenital system. Although hyperglycemia is the main metabolic alteration involved in its genesis, the presents of obesity, dyslipidemia, arterial hypertension, and smoking, play an additional role in its appearance. In the pathophysiology, three main phenomena stand out: oxidative stress, the formation of advanced glycosylation end-products, and microvasculature damage. Diagnosis is clinical, and it is recommended to use a 10 g monofilament and a 128 Hz tuning fork as screening tools. Glycemic control and non-pharmacological interventions constitute the mainstay of DN treatment, although there are currently investigations in antioxidant therapies, in addition to pain management. Conclusions: Diabetes mellitus causes damage to peripheral nerves, being the most common form of this, distal symmetric polyneuropathy. Control of glycemia and comorbidities contribute to prevent, postpone, and reduce its severity. Pharmacological interventions are intended to relieve pain.

Lipschütz acute vulvar ulcer related to COVID-19 vaccination: First case report in South America.

Lipschütz ulcer is a non-sexually transmitted genital lesion of unknown etiology, which presents as a painful vulvar ulcer. Lipschütz ulcers have been described in most continents. This is the first case reported in Peru and South America. We present the case of a 33-year-old female patient with a Lipschütz ulcer after being vaccinated with the second dose of the AstraZeneca COVID-19 vaccine. She reported having had only one sexual partner in her lifetime. Laboratory results were negative for herpes simplex 2, Cytomegalovirus, Toxoplasma gondii, Epstein-Barr virus, and syphilis. The patient received symptomatic treatment. Ten days after the onset, the patient was significantly better during follow-up. This case report displays a potential adverse effect of the AstraZeneca COVID-19 vaccine as a Lipschütz ulcer triggered by the host humoral immune response. However, further research is needed to establish the causal relationship between these two.

La úlcera de Lipschütz es una lesión genital no transmitidas por vía sexual de etiología desconocida, se presenta como una úlcera vulvar muy dolorosa. Las úlceras de Lipschütz se han descrito en la mayoría de los continentes. Este es el primer caso que se reporta en Perú y Sudamérica. En este reporte de caso se presentauna paciente de 33 años con una úlcera de Lipschütz luego de haber sido vacunada con la segunda dosis de la vacuna AstraZeneca COVID-19. Refirió haber tenido una sola pareja sexual a lo largo de su vida. Los resultados de laboratorio fueron negativos para herpes simplex 2, Citomegalovirus, Toxoplasma gondii, virus de Epstein-Barr y sífilis. La paciente recibió tratamiento sintomático. Diez días después, durante el seguimiento, la paciente estaba significativamente mejor. Este reporte de caso expone un potencial efecto adverso de la vacuna AstraZeneca COVID-19, en forma de úlcera de Lipschütz, desencadenado por la respuesta inmune humoral del huésped. Sin embargo, es necesario realizar más investigación para establecer la relación causal entre ambos.

Lipschütz acute vulvar ulcer related to COVID-19 vaccination: First case report in South America / Úlcera vulvar aguda de Lipschütz relacionada con la vacunación por COVID-19: primer caso notificado en Sudamérica

Lipschütz ulcer is a non-sexually transmitted genital lesion of unknown etiology, which presents as a painful vulvar ulcer. Lipschütz ulcers have been described in most continents. This is the first case reported in Peru and South America. We present the case of a 33-year-old female patient with a Lipschütz ulcer after being vaccinated with the second dose of the AstraZeneca COVID-19 vaccine. She reported having had only one sexual partner in her lifetime. Laboratory results were negative for herpes simplex 2, Cytomegalovirus, Toxoplasma gondii, Epstein-Barr virus, and syphilis. The patient received symptomatic treatment. Ten days after the onset, the patient was significantly better during follow-up. This case report displays a potential adverse effect of the AstraZeneca COVID-19 vaccine as a Lipschütz ulcer triggered by the host humoral immune response. However, further research is needed to establish the causal relationship between these two.

La úlcera de Lipschütz es una lesión genital no transmitidas por vía sexual de etiología desconocida, se presenta como una úlcera vulvar muy dolorosa. Las úlceras de Lipschütz se han descrito en la mayoría de los continentes. Este es el primer caso que se reporta en Perú y Sudamérica. En este reporte de caso se presentauna paciente de 33 años con una úlcera de Lipschütz luego de haber sido vacunada con la segunda dosis de la vacuna AstraZeneca COVID-19. Refirió haber tenido una sola pareja sexual a lo largo de su vida. Los resultados de laboratorio fueron negativos para herpes simplex 2, Citomegalovirus, Toxoplasma gondii, virus de Epstein-Barr y sífilis. La paciente recibió tratamiento sintomático. Diez días después, durante el seguimiento, la paciente estaba significativamente mejor. Este reporte de caso expone un potencial efecto adverso de la vacuna AstraZeneca COVID-19, en forma de úlcera de Lipschütz, desencadenado por la respuesta inmune humoral del huésped. Sin embargo, es necesario realizar más investigación para establecer la relación causal entre ambos.

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.

[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.

Acromegalia por macroadenoma hipofisiario en paciente con síndrome de McCune-Albright. Reporte de caso y revisión de la literatura / Acromegaly due to pituitary macroadenoma in a patient with McCune-Albright syndrome. Case report and literature overview

Introducción: El síndrome de McCune-Albright (SMA) es una enfermedad rara caracterizada por la triada: manchas cutáneas de color café con leche, displasia fibrosa poliostótica y pubertad precoz. Puede afectar a diversos ejes hormonales, entre ellos el de la hormona de crecimiento (GH), pudiendo asociarse a acromegalia. Reporte de caso: describimos el caso de una mujer de 44 años, con pubertad precoz periférica, hemorragia uterina anormal, crecimiento de manos y pies, prognatismo, prominencia frontal, manchas café con leche y tumoraciones pétreas en cara y antebrazos. Resultados: Apoyados con exámenes laboratoriales y de imágenes, se llegaron a los diagnósticos de acromegalia, hipogonadismo hipogonadotropo y síndrome de McCune-Albright. La paciente fue sometida a tratamiento quirúrgico con persistencia de enfermedad clínica y laboratorial. Conclusión: El diagnóstico y tratamiento oportunos de la acromegalia y sus complicaciones brindará un mejor pronóstico a los pacientes con SMA.

Background: McCune-Albright syndrome (MAS) is a rare disease characterized by the triad: café-au-lait skin spots, polyostotic fibrous dysplasia, and precocious puberty. It can affect various hormonal axes, including growth hormone (GH), and may be associated with acromegaly. We describe the case Case report:of a 44-year-old woman with peripheral precocious puberty, abnormal uterinebleeding, growthof thehands and feet, prognathism, frontal prominence, café-au-lait spots, and stony tumorsonthefaceandforearms.Supportedby Results:laboratory and imaging tests, the diagnoses of acromegaly, hypogonadotropic hypogonadism and McCune-Albright syndrome were reached. The patient underwent surgical treatment with persistence of clinical and laboratory disease. Conclusion: Timely diagnosis and treatment of acromegaly and its complications will provide a better prognosis for patients with MAS

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.