Dolor y edema de miembros inferiores por dermopatía tiroidea: presentación inusual de la enfermedad de Graves en una paciente adolescente / Lower limb pain and edema due to thyroid dermopathy: an unusual presentation of Graves disease in an adolescent patient

La enfermedad de Graves es un proceso inmunomediado en el que autoanticuerpos se dirigen contra el receptor de tirotrofina. Por su acción estimulante sobre la glándula tiroides, se genera crecimiento glandular difuso y aumento de la hormonogénesis. Se caracteriza por el comienzo subagudo de síntomas constitucionales, neuromusculares, cardiovasculares, gastrointestinales y oculares, seguidos en algunos casos de la aparición de manifestaciones cutáneas como la dermopatía tiroidea o mixedema. En pediatría la enfermedad de Graves es infrecuente (aunque es la causa más frecuente de hipertiroidismo), pero la cronología de aparición de los síntomas está bien descrita; es rara la aparición de dermopatía en ausencia de otros síntomas de hipertiroidismo y sin afectación ocular. Se presenta el caso de una paciente de 15 años con dermopatía tiroidea por enfermedad de Graves sin oftalmopatía ni otros síntomas de hipertiroidismo clínico asociados.

Graves disease is an immune-mediated process characterized by the presence of autoantibodies to thyrotropin receptors. Its stimulating action on the thyroid gland causes diffuse glandular growth and increased hormone production. Graves disease is characterized by a subacute onset of non-specific, neuromuscular, cardiovascular, gastrointestinal, and eye symptoms, sometimes followed by skin manifestations, such as thyroid dermopathy or myxedema. In pediatrics, Graves disease is rare (although it is the most frequent cause of hyperthyroidism). However, the chronology of symptom onset has been well described; the development of dermopathy in the absence of other symptoms of hyperthyroidism and without eye involvement is rare. Here we describe the case of a 15-year-old female patient with thyroid dermopathy due to Graves disease without eye disease or other associated clinical symptoms of hyperthyroidism.

Graves' disease, myxedema, and papillary thyroid carcinoma / Doença de Graves, mixedema e carcinoma papilífero de tireoide

Graves' disease (GD) is the leading cause of hyperthyroidism and diffuse toxic goiter in iodine-sufficient geographi-cal areas. GD is associated with classical manifestations such as ophthalmopathy and thyroid dermopathy, in addi-tion to diffuse goiter, which may be the site of carcinomas, as a complication. Case report: A 52-year-old woman presented with goiter and symptoms compatible with hyperthyroidism, such as heat intolerance, weight loss, fati-gue, increased sweat, tachycardia, fine tremors, increased intestinal transit, anxiety, emotional lability, insomnia, exophthalmos, and pretibial myxedema. A complementary investigation confirmed the diagnosis of hyperthyroidism (high free T4 and total T3 levels and low thyroid-stimulating hormone - TSH levels). Ultrasound images showed dif-fuse enlargement of the thyroid lobes by approximately 10 times and the presence of three thyroid nodules, one of which was larger than 2 cm with heterogeneous echogenicity and vascularization throughout the nodule; ultrasoun-d-guided fine needle aspiration revealed cytology compatible with Bethesda IV; scintigraphy revealed a low uptake area (cold nodule) amid a diffuse high-uptake goiter. A thyroidectomy was performed, and the anatomical specimen diagnosis revealed papillary thyroid carcinoma in the right lobe, with adjacent parenchyma compatible with GD. Histopathological examination of the skin showed the presence of myxedema compatible with Graves' dermopathy. The patient evolved with the normalization of TSH levels and a reduction of cutaneous manifestations. Conclusion:GD abnormalities may not be restricted to the classic clinical manifestations, and a careful investigation may reveal the coexistence of carcinomas. (AU)

A doença de Graves (DG) é a principal causa de hipertireoidismo e bócio difuso tóxico em áreas geográficas com iodo suficiente. DG está associada a manifestações clínicas clássicas como oftalmopatia e dermopatia da tireoide, além do bócio difuso, que pode ser sítio de carcinomas, como uma complicação. Relato de caso: Mulher de 52 anos apresentou bócio e sintomas compatíveis com hipertireoidismo como intolerância ao calor, emagrecimento, fadiga, sudorese aumentada, taquicardia, tremores finos, trânsito intestinal aumentado, ansiedade, labilidade emocional, insônia, exoftalmia e mixedema pré-tibial. A investigação complementar confirmou o diagnóstico de hipertireoidis-mo (níveis elevados de T4 livre e T3 total; níveis baixos de hormônio estimulante da tireoide - TSH). As imagens ultrassonográficas mostraram aumento difuso dos lobos tireoidianos em aproximadamente 10 vezes e a presença de três nódulos tireoidianos, um dos quais, maior que 2 cm, com ecogenicidade e vascularização heterogêneas em todo o nódulo, cuja punção aspirativa por agulha fina guiada por ultrassom revelou citologia compatível com Bethesda IV; e a cintilografia evidenciou uma área de baixa captação (nódulo frio) em meio a um bócio difuso de alta captação. Foi realizada tireoidectomia e o diagnóstico da peça anatômica revelou carcinoma papilífero de tir-eoide em lobo direito, com parênquima adjacente compatível com DG. O exame histopatológico da pele mostrou a presença de mixedema compatível com dermopatia de Graves. A paciente evoluiu com normalização dos níveis de TSH e redução das manifestações cutâneas. Conclusão: As anormalidades da DG podem não estar restritas às manifestações clínicas clássicas, e uma investigação criteriosa pode revelar a coexistência de carcinomas, (AU)

Urgencias endocrinológicas: coma mixedematoso / Endocrine emergencies: myxedema coma

Introducción: en el presente reporte de caso se presenta una urgencia endocrinológica, el coma mixedematoso. Se considera una descompensación del hipotiroidismo y su mortalidad es alta alcanzando hasta 52% en algunas series de casos. Puede desencadenarse por múltiples factores y su presentación clínica es muy variada ya que comprende la afección de diversos sistemas. Presentación del caso: se presenta el caso de una paciente femenina de 42 años de edad que consultó al servicio de urgencias del Hospital de San José de Bogotá con sospecha inicial de una de falla cardiaca descompensada, sin embargo, cursó con pobre mejoría ante el manejo instaurado, por lo cual se realizaron estudios de extensión en donde se encontró una función tiroidea deprimida. Discusión y conclusiones: en este momento se consideró que la paciente padecía un coma mixedematoso, se instauró manejo con dosis altas de levotiroxina oral con lo cual evolucionó de manera favorable y se logró dar de alta a los pocos días.

Introduction: herein we present an endocrine emergency, myxedema coma, which is a form of decompensated hypothyroidism, whose mortality may be as high as 52% as evidenced in some case series. It may be triggered by multiplefactors and has varied clinical presentations for it affects diverse organ systems. Case presentation:we present the case of a 42-year-old female who presented to the emergency department of Hospital de San José in Bogotá with clinical suspicion of decompensated heart failure upon admission, however, her response was poor to the treatment she received. Extension studies were performed which showed low thyroid function. Discussion and conclusions: at this point the patient was diagnosed with myxedema coma, and was started on high doses of oral levothyroxine, with which she evolved favorably and was discharged a few days later.

Myxedema coma as a cause associated with multiple etiologies of heart failure

ABSTRACT Introduction: Myxedema coma is the rarest and most extreme manifestation of hypothyroidism in adults. It is more frequent in older women and is associated with various clinical manifestations including diseases with systemic involvement, such as heart failure, pulmonary thromboembolism, and atrial fibrillation in unstable patients. Case presentation: A 73-year-old female patient with a history of heart failure, hypertension and overweight, presented functional deterioration, dyspnea at rest, tachypnea, tachycardia, desaturation, generalized edema, distal cyanosis, and jugular venous pressure. Laboratory tests showed mild anemia, normal blood glucose and electrolytes, azotemia with impaired renal function, TSH: 100 μUI/ml, free T4: 0.21 ng/dL, atrial fibrillation with accelerated ventricular rhythm and high lactate level in blood. Based on the findings, heart failure associated with de novo myxedema coma was diagnosed, as well as pulmonary thromboembolism associated with signs of right systolic dysfunction. The patient's condition gradually improved with hormone replacement therapy, heart failure treatment, and anticoagulation. Conclusion: Myxedema coma is a rare and extreme form of hypothyroidism. It is associated with high mortality and morbidity rates because it has severe systemic manifestations. Timely diagnosis and early implementation of supportive and specific treatment, especially with aggressive thyroid hormone replacement therapy and intensive care unit monitoring, help improve the prognosis of patients.

RESUMEN Introducción. El coma mixedematoso representa la máxima y más rara expresión del hipotiroidismo en los adultos. Esta complicación es más frecuente en mujeres ancianas y tiene diversas formas clínicas asociadas como enfermedades con compromiso sistémico, entre ellas la falla cardíaca, el tromboembolismo pulmonar y la fibrilación auricular en pacientes inestables. Presentación del caso. Paciente femenina de 73 años con antecedentes de insuficiencia cardíaca, hipertensión arterial y sobrepeso, quien presentó deterioro funcional, disnea en reposo, taquipnea, taquicardia, desaturación, edemas generalizados, cianosis distal e ingurgitación yugular. Los exámenes paraclínicos mostraron anemia leve, glucemia y electrolitos normales, azoados con deterioro de la función renal, TSH: 100 μUI/mL, T4 libre: 0.21 ng/dL, ritmo de fibrilación auricular con respuesta ventricular rápida e hiperlactatemia. A partir de los hallazgos se diagnosticó falla cardíaca asociada a coma mixedematoso de novo, que requirió soporte inotrópico y vasopresor, y tromboembolismo pulmonar asociado a signos de disfunción sistólica derecha. Finalmente, la paciente tuvo evolución lenta hacia la mejoría con la suplencia hormonal, optimización de falla cardíaca y anticoagulación. Conclusión. El coma mixedematoso es una forma extrema e infrecuente de hipotiroidismo que se asocia con altos niveles de morbimortalidad dado que implica manifestaciones sistêmicas graves. El diagnóstico oportuno y la implementación temprana de un tratamiento de soporte y específico, sobre todo con suplencia agresiva de hormonas tiroideas y vigilancia en unidad de cuidados intensivos, ayudan a mejorar el pronóstico de los pacientes con esta complicación.

Convulsiones, derrame pericárdico y rabdomiólisis en debut de coma mixedematoso / Myxedema coma presenting with seizures, pericardial effusion, and rhabdomyolysis

RESUMEN El coma mixedematoso es la complicación más grave de un hipotiroidismo. Ocurre, por lo general, en mujeres ancianas con hipotiroidismo conocido sin un adecuado manejo y en presencia de un evento desencadenante. El diagnóstico es difícil y debe realizarse en forma oportuna para disminuir el riesgo de muerte. El coma es una de las presentaciones neurológicas de esta urgencia endocrinológica y no es necesario su presencia para el diagnóstico. En este reporte de caso se presentan varías manifestaciones inusuales en un paciente masculino con hipotiroidismo profundo que, al diagnóstico, debutó con coma mixedematoso con predictores de mal pronóstico durante la hospitalización, pero debido al abordaje temprano y el manejo integral, se dio una resolución satisfactoria a esta urgencia endocrinológica infrecuente.

SUMMARY Myxedema coma is the most serious complication of hypothyroidism. It usually occurs in the context of elderly women, with known hypothyroidism without proper management and in the presence of a triggering event. The diagnosis is challenging and must be made in a timely manner to prevent the development of adverse outcomes. Coma is one of the neurological manifestations of the entity, not being necessary for its diagnosis. This case report presents a constellation of unusual manifestations of a male patient with myxedema coma at the debut of severe hypothyroidism with predictors of poor prognosis during hospitalization, but due to the early approach and comprehensive management, this uncommon endocrinological emergency was satisfactorily resolved.

Coma mixedematoso e trombo de veia cava superior. Relato de caso e revisão da literatura / Myxedema coma and superior vena cava thrombus: case report and literature review

O coma mixedematoso é uma emergência endocrinológica rara e consiste na máxima expressão do hipotireoidismo, com alta mortalidade por suas complicações hemodinâmicas e ventilatórias, podendo ser agravadas por distúrbios da coagulação. Relatamos o caso de uma paciente diagnosticada com coma mixedematoso e trombo de veia cava superior. Buscamos salientar os distúrbios de coagulação frequentes no hipotireoidismo grave, que contribuem para o aumento da mortalidade deste grupo de pacientes. O diagnóstico e o tratamento precoce do coma mixedematoso, aliados à instituição imediata da terapia para o fenômeno trombótico encontrado, permitiram a evolução favorável do quadro. O relato, juntamente da bibliografia pesquisada, orientou o raciocínio sobre a relação dos distúrbios de coagulação, que ocorrem no hipotireoidismo descompensado. Apesar de poucos relatos, estes distúrbios podem ser frequentes e devem ser pesquisados, pois contribuem com o aumento da mortalidade.(AU)

Myxedema coma is a rare endocrinological emergency, consisting of the highest expression of hypothyroidism with high mortality due to hemodynamic and ventilatory complications, which may be aggravated by coagulation disorders. We report the case of a patient diagnosed with myxedema coma and superior vena cava thrombus. We sought to emphasize the frequent coagulation disorders in severe hypothyroidism, which contribute to increased mortality in this group of patients. The diagnosis and early treatment of myxedema coma, together with the immediate institution of therapy for the thrombotic phenomenon found, allowed the favorable evolution of the condition. The report, together with the literature, has guided the rationale for the influence of coagulation disorders that occur in decompensated hypothyroidism. Despite the few number of reports, these disorders can be frequent and should be investigated because they contribute to the increase in mortality.(AU)

Hipotiroidismo congénito y sus manifestaciones bucales / Congenital hypothyrodism and its oral manifestations

El hipotiroidismo es el más común de los trastornos de la tiroides, puede ser congénito si la glándula tiroides no se desarrolla correctamente (hipotiroidismo congénito). La predominancia femenina es una característica. Entre las características odontológicas del hipotiroidismo se observan labios gruesos, lengua de gran tamaño, que debido a su posición suele producir mordida abierta anterior y dientes anteriores en abanico, destaca que la dentición temporal y permanente presentan un retardo eruptivo característico y, aunque los dientes son de tamaño normal, suelen estar apiñados por el tamaño pequeño de los maxilares. Se presentan dos casos clínicos de pacientes de sexo femenino que acuden a la clínica de Especialidad en Odontopediatría de la Universidad Autónoma del Estado de México con diagnóstico de hipotiroidismo congénito.

Hypothyroidism is one of the most common thyroid disorders. Hypothyroidism can be congenital in cases when the thyroid gland does not develop normally. Female predominance is a characteristic of congenital hypothyroidism. Dental characteristics of hypothyroidism are thick lips, a large-sized tongue which, due to its position, can elicit anterior open bite as well as fanned-out anterior teeth. In these cases, delayed eruption of primary and permanent dentitions can be observed, and teeth, even though normal-sized, are crowded due to the small-sized jaws. This study presents clinical cases of female patients diagnosed with congenital hypothyroidism who sought treatment at the Dental Pediatrics Unit of the Autonomous University of the State of Mexico.

Mixedema de la vía aérea superior como causa de Sahos transitorio: reporte de caso / Upper airway myxedema as a cause of transient osas. Case report

El Síndrome de Apnea-Hipopnea del Sueño (SAHOS) y el hipotiroidismo tienen varios signos y síntomas en común; esta similitud crea un solapamiento en sus presentaciones clínicas, que pudieran llevar a un sinnúmero de casos de hipotiroidismo sin diagnosticar durante el SAHOS. La incidencia de los estados de hipotiroidismo en pacientes con síndrome de apnea del sueño aún no se conoce, por lo que es razonable evaluar la función tiroidea en todos los pacientes. Estudios previos han demostrado una asociación entre el hipotiroidismo y la apnea obstructiva del sueño; sin embargo, los trastornos endocrinos asociados a la obstrucción de la vía aérea superior en el adulto continúan siendo una de las causas más raras de obstrucción, después del trauma, infección, reacciones alérgicas y malignidad. Se presenta el caso clínico de una paciente de 41 años de edad, con hipotiroidismo manifiesto y mixedema supraglótico, que desarrolló síndrome transitorio severo de apnea-hipopnea del sueño durante un mes, el cual mejoró después del tratamiento médico con reemplazo de hormona tiroidea...

The Obstructive Sleep Apnea Syndrome (OSAS) and hypothyroidism have several signs and symptoms in common, this similarity creates an overlap in their clinical presentations that could lead to a number of cases of hypothyroidism undiagnosed for OSAS. Incidence of hypothyroidism in patients with Obstructive Sleep Apnea Syndrome is not yet known, so it is reasonable to evaluate thyroid function in all patients. Previous studies have shown an association between hypothyroidism and obstructive sleep apnea, however, endocrine disorders associated with obstruction of the upper airway in adults remains one of the rarest causes of obstruction, after trauma, infection, allergic reactions and malignancy. Its presented a clinical case of a female 41 years old patient with overt hypothyroidism and supraglottic myxedema, who developed severe Transient Obstructive Sleep Apnea Syndrome for a month, which improved after medical treatment with thyroid hormone replacement...

Coma mixedematoso / Myxedema coma

El coma mixedematoso es la forma más severa y profunda del hipotiroidismo. Se presenta con mayor frecuencia en mujeres y ancianos. Entre los factores precipitantes se encuentran: la sepsis, la exposición al frío, los eventos agudos graves, el uso de anestésicos, sedantes o narcóticos, así como la descontinuación del tratamiento sustitutivo con hormonas tiroideas, entre otros. El diagnóstico clínico se realiza por la presencia de síntomas y signos característicos de un hipotiroidismo severo, con hipotermia y alteraciones de la conciencia. Apoyan este diagnóstico los hallazgos de laboratorio: hiponatremia, hipoxemia, hipercapnia, alteraciones hemoquímicas y el aumento de la tirotropina por la disminución de las hormonas tiroideas en el caso de la enfermedad primaria. El tratamiento se debe realizar en una unidad de cuidados intensivos, con monitorización, medidas de soporte respiratorio y cardiovascular, calentamiento corporal interno, hidratación, corrección de la hipotensión y de los trastornos electrolíticos. Se administrarán, además, glucocorticoides, antibióticos de amplio espectro y hormonas tiroideas. La evolución depende de la demora en el inicio del tratamiento, la edad, las comorbilidades, la hipotermia persistente y las complicaciones asociadas(AU)

Myxedema coma is the most severe and deepest form of hypothyroidism. It occurs more often in the women and the elderly. Among the unleashing factors found are sepsis, exposure to cold, acute severe events, use of anesthetic drugs, sedatives or narcotics as well as the interruption of the replacement treatment with thyroid hormones, among others. The clinical diagnosis is based on the presence of symptoms and signs that are characteristic of severe hypothyroidism, with hypothermia and altered consciousness. This diagnosis is also supported by the lab findings: hyponatremia, hypoxemia, hypercapnia, hemochemical alterations and the rise of thyrotropin due to the decrease of thyroid hormones in the case of the primary disease. The patient should be treated in an intensive care unit, by using monitoring, respiratory and cardiovascular support, internal body heating, hydration, correction of hypotension and control of electrolytic disorders. Additionally, glycocorticoids, broad-spectrum antibiotics and thyroid hormones should be administered. The progression of the disease depends on the delay in starting the treatment, the age, the comorbidities, the persistent hypothermia and the associated complications(AU)

Cardiomiopatía mixedematosa. Reporte de un caso / Myocardiopathie myxedematosa. Report of case

Introducción: El hipotiroidismo o enfermedad de Gull (1873) es la afección endocrina más frecuente en nuestros tiempos en los países de todo el mundo y en cualquier raza. La incidencia anual de nuevos casos en la población general es de 1-2 por ciento. Se ha estimado que alcanza 6-7 por ciento de la población femenina de edad superior a 60 años. Presentación: Se presenta el caso de una mujer negra de 32 años con hipotiroidismo postiroidectomia. Abandona el tratamiento e ingresa con insuficiencia cardiaca, derrame pericárdico y síntomas de hipotiroidismo con mixedema. Discusión: En esta cardiomiopatía se integran dilatación con hipocinesia, infiltración con restricción, trastornos de la conducción y el ritmo en algunos y en muchos de los casos aparece también afectación del pericardio con derrame asociada. Conclusión: Evolucionó satisfactoriamente al restituir tratamiento y otras medidas. En algunos casos el daño es irreversible.

Introduction: Hypothyroidism or Gull's disease (1873) is the most frequent endocrine affection in all races worldwide.The annual incidence of new cases is 1-2 percent of general population. It has been estimated that this rate reaches 6-7 percent of the female population over 60 years. Presentation: It's a case report of a 32 years old black woman with hypothyroidism post-thyroidectomy. The patient gives up treatment and enters the hospital with cardiac insufficiency, pericardium effusion and symptoms of hypothyroidism with myxedema. Discussion: Symptoms such as dilatation with hypokynesia, infiltration with restrictions, pericardium disorders with associated effusión and dysrhythmias in some cases are usually imtegrated to this cardiomyopathy. Conclusion: The patient had a satisfactory evolution after reenter the hospital with the former measures and treatment. In some cases damage is irreversible.

Coma mixedematoso / Myxedematous coma

É relatado o caso de um paciente de 76 anos, masculino, em coma mixedematoso, que evoluiu para óbito após 27 dia de tratamento intensivo. Discutem-se os métodos diagnóstico e a conduta terapêutica no coma do hipotireoidismo.

This case report of myxedema coma in a male adult patient (76 years old), who died after 27 days in the Intensive Care Unit (ICU). The diagnosis and treatment of myxedema coma is discussed.