Preparation of ICD and overdenture in patients with Cushing's Syndrome: five-year follow-up / Preparo de PTI e overdenture em pacientes com Síndrome de Cushing: cinco anos de acompanhamento

The aim of this study is to report a clinical case of a patient with Cushing's syndrome, low self-esteem and need for oral rehabilitation. A 50-year-old female patient with hypercortisolism sought care at School of Dentistry of the Federal University of Minas Gerais. During anamnesis, the patient reported painful symptoms and mobility of the upper and lower teeth. On clinical examination, absence of many teeth were observed, periodontal disease and caries lesions. After discussion of the case, the planning involved the extraction of all teeth, and the preparation of Maxillary Immediate Complete Dentures (ICD's) and Immediate Mandibular Implant-retained Overdenture (IMIO). The treatment proposed allowed the restored aesthetics, phonetics and chewing(AU)

O objetivo desse estudo é relatar um caso clínico de uma paciente com síndrome de Cushing, baixa autoestima e necessidade de reabilitação oral. Paciente do sexo feminino, 50 anos de idade e com hipercortisolismo, procurou atendimento na Faculdade de Odontologia da Universidade Federal de Minas Gerais. Durante a anamnese, a paciente relatou sintomas dolorosos e mobilidade dos dentes superiores e inferiores. No exame clínico, foi observada a ausência de muitos dentes, doença periodontal e lesões cariosas. Após a discussão do caso, o planejamento envolveu a extração de todos os dentes, e o preparo de Prótese Total Imediata maxilar (PTI), e Overdenture Implantorretida Imediata mandibular. O tratamento proposto permitiu que restaurasse a estética, fonética e mastigação(AU)

Enfermedad de Cushing recurrente con hipercortisolismo catastrófico: reporte de caso y revisión de literatura / Catastrophic hypercortisolism and recurrence in Cushing´s disease: a case report and literature review / Doença de Cushing recorrente com hipercortisolismo catastrófico: relato de caso e revisão da literatura

Resumen: Presentamos un caso de una paciente femenina de 27 años, con síndrome de Cushing ACTH dependiente con hipercortisolismo severo, causado por un macroadenoma hipofisario recurrente y resistente pese a dos cirugías transesfenoidales, radioterapia y terapia médica. Dada la falla en las diferentes terapias se realiza una adrenalectomía bilateral como tratamiento definitivo. La paciente fallece en el posoperatorio por causa no clara. Si bien la adrenalectomía bilateral ha sido reportada como un tratamiento efectivo en pacientes con enfermedad de Cushing, se ha relacionado con una mortalidad significativa vinculada con la severidad del hipercortisolismo y las comorbilidades presentes. En este caso la adrenalectomía izquierda se tuvo que convertir a cielo abierto, asociada con mayor morbimortalidad.

Abstract: The study presents the case of a 27-year-old female patient with adrenocorticotropic hormone (ACTH) dependent Cushing's disease and severe hypercortisolism caused by recurrent pituitary macroadenoma that was resistant to treatment despite two transsphenoidal surgeries, radiotherapy and medical treatment. Upon failure of the different therapies a bilateral adrenalectomy was performed as the final treatment. The patient died in after surgery although the case of death was not clear. Despite bilateral adrenalectomy having been reports as an effective treatment in patients with Cushing's disease, it has been related to significant mortality rates in connection with the severity of hypercortisolism and existing comorbilities. In this case the left adrenalectomy ended up being an open surgery, which is associated to a higher mortality rate.

Resumo: Apresentamos o caso de uma paciente de 27 anos com síndrome de Cushing ACTH-dependente com hipercortisolismo grave causado por macroadenoma hipofisário, recorrente e resistente, apesar de haver sido submetida a duas cirurgias transesfenoidal, radioterapia e terapia medicamentosa. Diante do fracasso das diferentes terapias, foi realizada adrenalectomia bilateral como tratamento definitivo. A paciente faleceu no pós-operatório por causa não esclarecida. Embora a adrenalectomia bilateral tenha sido relatada como tratamento eficaz em pacientes com doença de Cushing, ela tem sido associada a mortalidade significativa relacionada à gravidade do hipercortisolismo e às comorbidades presentes. Neste caso, a adrenalectomia esquerda teve que ser convertida para cirurgia aberta, associada a maior morbimortalidade.

Cushing's syndrome due to ectopic adrenocorticotropin secretion by a parotid carcinoma

SUMMARY We report a rare case of Cushing's syndrome in a 37-year-old female who initially presented with localized acinic cell carcinoma of the parotid gland. In January 2014, she underwent a right parotidectomy with facial nerve preservation and adjuvant radiotherapy. In August 2018, she presented a histologically-proven local regional relapse. The patient was considered for salvage surgery with facial nerve sacrifice and remained with no evidence of disease. One year later the patient developed pulmonary dissemination and started to gain weight and developed facial plethora and acne on the face and upper trunk. In a physical examination, the patient presented moon face, buffalo hump, acne and stage 2 hypertension. Biochemical evaluation confirmed ACTH-dependent Cushing's syndrome. IHC for ACTH in the lung biopsy revealed strong positive staining for ACTH confirming a diagnosis of ectopic ACTH secretion by a metastatic parotid acinic cell carcinoma. Ketoconazole (600 mg/d) was started to treat the CS. In addition, as chemotherapy was initiated to treat the metastatic disease. After the fifth cycle of chemotherapy, ketoconazole was suspended and the patient remained in remission of CS for four months, when CS recurred. A unique feature of this case is related to the clinical CS relapse associated with disease progression, which needed prompt treatment with ketoconazole, resulting in a significant improvement in the patient's condition. Although rare, should be attentive for possible CS features in patients with high-grade salivary gland carcinomas, since the diagnosis of ectopic secretion of ACTH may significantly impact their management and outcomes.

Síndrome de Cushing secundario a administración tópica decorticoesteroides / Cushing Syndrome due to topical corticosteroid misuse

Los corticoesteroides tópicos son drogas muy comunes, frecuentemente utilizadas en patologías dermatológicas. Su mal uso puede causar efectos sistémicos, como el síndrome de Cushing y la supresión del eje hipotalámico ­ hipofisiario ­ adrenal. Presentamos un caso de un lactante menor de siete meses quien desarrolla un síndrome de Cushing secundario al uso de Clobetasol por una dermatitis en el área del pañal, por tiempo prolongado, sin prescripción médica. Al examen físico se evidencia obesidad a predominio central, con fascie de luna llena, hipertricosis en región frontal, telangiectasias aisladas en mejillas y cuello de búfalo. Los paraclínicos demuestran una hipercolesterolemia, hipertrigliceridemia, elevación de las transaminasas y cortisol sérico en la mañana disminuido. Se concluye que se debe informar a los padres de los efectos adversos sistémicos de los esteroides tópicos y se sugiere evitar en pacientes pediátrico(AU)

Topical corticosteroids are very common drugs used in the treatment of inflammatory skin diseases. Prolonged ormisuse of them may cause systemic adverse effects, including Cushing syndrome and hypothalamic-pituitary-adrenal axissuppression. We present a case of a seven months old maleinfant who developed iatrogenic Cushing syndrome after diaperdermatitis treatment through misuse of Clobetasol, withoutdoctor's prescription. We observe redness and a moon face, abuffalo hump, central obesity and hirsutism. Laboratory values revealed hypercholesterolemia, hypertriglyceridemia, elevationin liver enzymes and low early morning cortisol. To conclude,parents must be informed by physicians about the adverse effect of steroids and the should be avoided in very young infant(AU)

Cyclic ACTH-secreting thymic carcinoid: a case report and review of the literature

SUMMARY Cyclic Cushing's syndrome (CS) due to thymic carcinoid is a rare disorder. We report a case of cyclic CS due to ectopic adrenocorticotropic hormone (ACTH)-secreting atypical thymic carcinoid tumor and reviewed similar cases published in the literature. Our patient had hypercortisolemia lasting approximately one month, followed by normal cortisol secretion, with relapse one year later. Histopathology revealed an atypical ACTH-positive thymic carcinoid. Ectopic CS can be derived from atypical thymic carcinoids, which can be aggressive tumors with early relapse, suggesting that this type of tumor probably needs aggressive treatment.

A novel mutation in PRKAR1A gene in a patient with Carney complex presenting with pituitary macroadenoma, acromegaly, Cushing's syndrome and recurrent atrial myxoma

SUMMARY Carney complex (CNC) is a rare syndrome of multiple endocrine and non-endocrine tumors. In this paper we present a 23-year-old Iranian woman with CNC who harbored a novel mutation (c.642dupT) in PRKAR1A gene. This patient presented with pituitary macroadenoma, acromegaly, recurrent atrial myxoma, Cushing's syndrome secondary to primary pigmented nodular adrenocortical disease and pigmented schwanoma of the skin. PRKAR1A gene was PCR amplified using genomic DNA and analyzed for sequence variants which revealed the novel mutation resulting in substitution of amino acid cysteine instead of the naturally occurring valine in the peptide chain and a premature stop codon at position 18 (V215CfsX18). This change leads to development of tumors in different organs due to lack of tumor suppressive activity secondary to failure of synthesis of the related protein.

Manifestaciones clínicas predictivas del diagnóstico del hipercortisolismo endógeno / Clinical Manifestations Predicting Endogenous Hypercortisolism

Introducción: El diagnóstico clínico del hipercortisolismo endógeno puede ser complejo si las manifestaciones clínicas no son patognomónicas. Es importante conocer cuáles son las distintivas en nuestros pacientes, que permitan diagnosticar la enfermedad de forma precoz. Objetivo: Determinar sensibilidad, especificidad y valor predictivo positivo de las manifestaciones clínicas para diagnosticar el hipercortisolismo endógeno. Métodos: Estudio descriptivo transversal. Grupo I: 65 pacientes con hipercortisolismo endógeno y grupo II (comparación): 75 con sospecha clínica de hipercortisolismo endógeno a los que se les descartó la enfermedad. Se estudiaron pacientes con diagnóstico de hipercortisolismo endógeno (2004-2017), atendidos en el Instituto de Endocrinología. Se determinaron los valores de sensibilidad, especificidad y valor predictivo positivo para el diagnóstico de hipercortisolismo endógeno de cada síntoma y signo por separado, así como para las combinaciones de tres manifestaciones clínicas; además se realizó una regresión logística binaria para identificar las combinaciones de sintomatologías que mejor predicen la presencia de hipercortisolismo endógeno. Se consideró diferencia estadística significativa con p ≤ 0,05. Resultados: La mayoría de los síntomas presentó una sensibilidad inferior al 45 por ciento. La circunferencia de la cintura fue el único signo que mostró alta sensibilidad (76,9 por ciento), baja especificidad (28,6 por ciento) con valor predictivo positivo de 42,0 por ciento. Cuando se presentan dos o tres de estos signos: rubicundez, cara de "luna llena" e hirsutismo la posibilidad de tener hipercortisolismo endógeno es 75,4 por ciento, con valor predictivo positivo de 71 por ciento y, cuando no están presentes la probabilidad de no tenerlo es de 77,5 por ciento. Conclusiones: La combinación de sensibilidad, especificidad y valor predictivo positivo de los síntomas y signos de forma aislada no predice el diagnóstico de la enfermedad; sin embargo, la presencia de dos o tres de los síntomas y/o signos tiene una elevada sensibilidad y valor predictivo positivo para el diagnóstico del hipercortisolismo endógeno. Las combinaciones que mejor predicen la posibilidad de tener un hipercortisolismo endógeno son: piel fina, edema y acné; cara de "luna llena", hirsutismo y rubicundez, así como "giba de búfalo", hematomas y estrías(AU)

ABSTRACT Introduction: The clinical diagnosis of endogenous hypercortisolism can be complex if clinical manifestations are not pathognomonic. It is important to know what are distinctive in our patients, which allows to diagnose the disease early. Objective: To determine the sensitivity, specificity and positive predictive value of clinical manifestations for diagnosis. of endogenous hypercortisolism. Methods: Descriptive and cross-sectional study. Group I was made up of 65 patients with endogenous hypercortisolism; group II (comparison) was made up of 75 patients with clinical suspicion of endogenous hypercortisolism, finally ruled out. We studied patients diagnosed of endogenous hypercortisolism from 2004 to 2017, treated at the Endocrinology Institute. Sensitivity, specificity and positive predictive values were determined for the diagnosis of endogenous hypercortisolism for each symptom and sign separately, as well as for the combinations of three clinical manifestations; in addition, binary logistic regression was performed to identify the combinations of symptoms that best predict the presence of endogenous hypercortisolism. A statistically significant difference was considered with p ≤ 0.05. Results: Most of the symptoms presented a sensitivity lower than 45 percent. Waist circumference was the only sign that showed high sensitivity (76.9 percent), low specificity (28.6 percent), and positive predictive value of 42.0 percent. When two or three of these signs (redness, "full-moon" face, and hirsutism) are present, the possibility of having endogenous hypercortisolism is 75.4 percent, with positive predictive value of 71 percent; and, when they are not present, the probability of not having it is 77,5 percent. Conclusions: The combination of sensitivity, specificity and positive predictive value of the symptoms and signs in isolation does not predict the diagnosis of the disease; however, the presence of two or three of the symptoms and/or signs has a high sensitivity and positive predictive value for the diagnosis of endogenous hypercortisolism. The combinations that best predict the possibility of having an endogenous hypercortisolism are thin skin, edema and acne; "full-moon" face, hirsutism and redness; as well as "buffalo hump," bruises and stretch marks(AU)

Cateterismo de senos petrosos en el diagnóstico etiológico de síndrome de Cushing: primera experiencia en Uruguay / Petrosal sinus sampling for etiological diagnosis of Cushing's syndrome: first experience in Uruguay / Cateterismo dos seios petrosos no diagnóstico etiológico da síndrome de Cushing: primeira experiência no Uruguai

Resumen: Identificar la causa de un síndrome de Cushing dependiente de adrenocorticotropina (ACTH) es esencial para realizar un tratamiento correcto. La hipersecreción de ACTH es debida en su mayoría a un tumor hipofisario (enfermedad de Cushing) o, en un 10%-20%, a tumores con producción ectópica de esta hormona. Los test no invasivos tienen baja sensibilidad y especificidad para diferenciar estas dos etiologías. El patrón oro lo constituye el cateterismo bilateral de los senos petrosos inferiores (CSP). Mediante el CSP se demuestra la hipersecreción de ACTH a nivel hipofisario al documentar un gradiente de ACTH central a periférico en el drenaje del tumor. Se recomienda realizarlo en todo síndrome de Cushing ACTH dependiente, aunque suele reservarse para pacientes con diagnóstico de hipercortisolismo y hallazgos negativos o equívocos en la resonancia nuclear magnética (RNM) de la región selar. Presentamos el primer caso en Uruguay en que se utilizó el CSP como método diagnóstico, una mujer de 55 años que presentó un hipercortisolismo ACTH-dependiente con una imagen adenohipofisaria <6 mm. El gradiente petroso-periférico confirmó el diagnóstico de enfermedad de Cushing y no hubo complicaciones durante el procedimiento. Posteriormente se realizó la resección del adenoma mediante cirugía transesfenoidal, con buena evolución y confirmación inmunohistoquímica del tumor.

Summary: Identifying the cause of adrenocorticotropin (ACTH)-dependent Cushing's syndrome is key to define the appropriate treatment. Hypersecretion of the adrenocorticotropic hormone (ACTH) is mainly caused by a pituitary tumor (Cushing's syndrome) or, in 10% to 20% of cases, by tumors with ectopic production of this hormone. Differentiation between these two etiologies may not be easy due to the low sensitivity and specificity of non- invasive tests. Bilateral sampling of the lower petrosal sinus is the gold standard to differentiate between a pituitary and an ectopic origin, showing the pituitary ACHT hypersecretion and recording the central-to-peripheral ACTH gradient in the tumor's drainage. Despite it being highly recommended for all cases of ACTH-dependent Cushing's syndrome, it is reserved for patients with a diagnosis of hypercortisolism and negative or misleading findings in the MRI of the sellar region. The study presents the first case of petrosal sinus sampling for diagnostic purposes in Uruguay, in a 55-year-old woman with ACHT-dependent hypercortisolism showing an adenohypophysis image < 6 mm. The petrosal-peripheral gradient confirmed the diagnosis of Cushing's syndrome and no complications arose during the procedure. Afterwards a transsphenoidal surgery was performed for resection of the adenoma. Evolution was good and immunochemistry confirmed the tumor's etiology.

Resumo: Identificar a causa da síndrome de Cushing dependente de adrenocorticotropina (ACTH) é essencial para o tratamento adequado. A hipersecreção de ACTH se deve principalmente a um tumor hipofisário (doença de Cushing) ou, em 10%-20%, a tumores com produção ectópica desse hormônio. Os testes não invasivos apresentam baixa sensibilidade e especificidade para diferenciar essas duas etiologias. O padrão ouro é o cateterismo bilateral dos seios petrosos inferiores (CEP). O CSP demonstra hipersecreção de ACTH no nível da hipófise, documentando um gradiente de ACTH central a periférico na drenagem do tumor. É recomendado nos casos de síndrome de Cushing dependente de ACTH, embora seja geralmente reservado para pacientes com diagnóstico de hipercortisolismo e achados negativos ou duvidosos na ressonância magnética (RNM) da região selar. Apresentamos o primeiro caso no Uruguai em que o CSP foi usado como método diagnóstico, uma mulher de 55 anos que apresentava hipercortisolismo ACTH dependente com imagem da hipófise anterior <6 mm. O gradiente petroso-periférico confirmou o diagnóstico de doença de Cushing e não houve complicações durante o procedimento. A seguir, o adenoma foi ressecado por cirurgia transesfenoidal, com boa evolução e confirmação imunohistoquímica do tumor.

Coinfección por Mycobacterium abscessus y Mycobacterium tuberculosis en un paciente con síndrome de Cushing exógeno y otras comorbilidades / Mycobacterium abscessus and Mycobacterium tuberculosis coinfection in a patient with exogenous Cushing syndrome and other comorbilities

RESUMEN Mycobacterium abscessus es una micobacteria no tuberculosa de crecimiento rápido, que produce infección pulmonar, cutánea, diseminada y otras, sobre todo en pacientes con comorbilidades. El diagnóstico se basa en la identificación de la micobacteria por medios de cultivo o por pruebas moleculares. El tratamiento con macrólidos y amikacina continúa siendo el ideal, aunque depende de la localización y de la gravedad de la infección, sin embargo, se han identificado genes de resistencia en algunas subespecies que limitan la eficacia antibiótica. Presentamos el caso de un paciente con coinfección cutánea por Mycobacterium abscessus y Mycobacterium tuberculosis, quien presenta un síndrome de Cushing exógeno, factor predisponente para estas infecciones. Además, se identificaron hidatidosis y aspergilosis pulmonares. El tratamiento antituberculoso del paciente se ajustó para manejar ambas micobacterias, y su evolución fue favorable.

ABSTRACT Mycobacterium abscessus is a fast-growing non-tuberculous mycobacterium that causes lung, skin, disseminated and other infections, mainly in patients with comorbidities. The diagnosis is based on the identification of the mycobacterium by culture media or molecular tests. Treatment with macrolides and amikacin remains the optimal choice, although it depends on the location and severity of the infection; however, resistance genes have been identified in some subspecies that limit antibiotic efficacy. We present the case of a patient with cutaneous coinfection by Mycobacterium abscessus and Mycobacterium tuberculosis, who presented exogenous Cushing syndrome, a predisposing factor for these infections. In addition, hydatidosis and pulmonary aspergillosis were identified. The patient's anti-tuberculosis treatment was adjusted to manage both mycobacteria, resulting in a favorable evolution.

Ectopic Cushing syndrome in Colombia

ABSTRACT Objective The aim was to describe the clinical features of patients with ectopic Cushing syndrome (ECS) from Colombia and compare these findings with other series to provide the best management for these patients. Materials and methods Records of patients with ECS from 1986 to 2017 were retrospectively reviewed; patients with a diagnosis of adrenal or pituitary Cushing syndrome (CS) were excluded. Results Fourteen patients with ECS were analyzed in this study. The mean age was 54.4 (SD 17.1) years, and the female to male ratio was 1.33:1. Regarding the etiology of ECS, four patients had lung carcinoids (28.6%), three had small-cell lung carcinoma (21.4%), three had pancreatic neuroendocrine tumors (21.4%), one had medullary thyroid cancer (7.1%), one had non-metastatic pheochromocytoma (7.1%), one had metastatic thymoma (7.1%) and one patient had an occult source of ACTH (7.1%). The most common clinical features at presentation were moon-face, muscle weakness, diabetes mellitus and hypertension. Hyperpigmentation was present in 36% of patients, and 12 patients had hypokalemia with a mean value of 2.3 mEq/L (SD 0.71). The median basal cortisol, 24-hour urinary free cortisol (UFC) and ACTH were 30.5 ug/dL (IQR 21-59 ug/dL), 2,600 ug/24 h (IQR 253-6,487 ug/24 h) and 91 pg/mL (IQR 31.9-141.9), respectively. Thirteen patients (92.8%) had the site of the primary lesion identified. Six patients had undergone a surgical intervention to address the primary tumor. Resection was curative in 28.5% of patients. Death occurred in 57.1% of patients, and the median overall survival was 27 months. Intrathoracic tumors had the most aggressive behavior. Conclusions ECS is a rare disease; however, it is associated with high morbidity and mortality. A rapid intervention supported by an interdisciplinary group is required to improve overall survival and quality of life

Sampling of the petrosal sinuses. Indications, results and usefulness / Muestreo de senos petrosos. Indicaciones, resultados y utilidad

Abstract Objectives: to analyze, evaluate and describe the usefulness of petrosal sinus sampling for diagnosing central Cushing's syndrome. Materials and methods: the technical aspects and results of bilateral venous sampling of the petrosal sinuses at the Hospital Universitario San Vicente de Paul in Medellín, Colombia, from January 1, 2012 to December 31, 2018, were analyzed. Results: the average age was 43.3 years, with a range from 19 to 69 years. Laterality could be shown in 68.2% of cases, with a tendency to be located on the left in 53.3%. The central source of ACTH production could be shown in 95.4% of cases, with a basal average central/peripheral ratio of 21.7, and 70.8 after stimulation. All samples at 3, 5 and 10 minutes were confirmatory following stimulation. Conclusion: in our retrospective study, petrosal sinus catheterization provided laboratory confirmation of the central source of ACTH production in a high percentage of patients, with no immediate complications.

Resumen Objetivos: analizar, evaluar y describir la utilidad del muestreo de senos petrosos para diagnóstico del síndrome de Cushing de origen central. Material y métodos: se analizaron los aspectos técnicos y resultados del muestreo bilateral venoso de senos petrosos, desde el 1° de enero de 2012 a 31 de diciembre de 2018 en el Hospital Universitario San Vicente de Paúl en Medellín, Colombia. Resultados: el promedio de edad fue 43.3 años con un rango de edad desde los 19 hasta los 69 años. La lateralidad pudo ser demostrada en 68.2% de los casos con una tendencia a la localización en el lado izquierdo en 53.3%. El origen central de producción de ACTH logró ser demostrado en 95.4% de los casos, con una relación central/periferia basal promedio de 21.7 y postestimulación de 70.8. Todas las muestras a los 3, 5 y 10 minutos fueron confirmatorias tras la estimulación. Conclusión: en nuestro estudio retrospectivo el cateterismo de senos petrosos confirmó la fuente central de producción de ACTH por laboratorio en un alto porcentaje de pacientes sin ninguna complicación inmediata.

Síndrome de Cushing provocado por carcinoma suprarrenal gigante. Caso clínico

We report a previously healthy 34-year-old woman, presenting with a seven-month history of arterial hypertension, amenorrhea, weight gain, facial edema, acne, hirsutism and low back pain. A CT scan showed a right adrenal mass of 18 × 13 × 12.5 cm, and multiple vertebral and rib fractures. The hormonal study confirmed Cushing's Syndrome. Ketoconazole, spironolactone, cotrimoxazole, calcium / vitamin D were started. An adrenalectomy with a right nephrectomy were performed. The excised tumor measured 16 cm and weighed 1.55 kg. There was tumor embolism and a 4 mm soft tissue involvement (pT3NxMx). The right kidney was free of tumor. The patient was treated with chemotherapy (etoposide plus cisplatin). Study of vertebral fractures with magnetic resonance (MRI) showed crush fractures, without images of metastatic bone lesions. One year after surgery, a CT scan showed no signs of tumor recurrence. The patient was lost from follow-up thereafter.

Síndrome de Cushing exógeno de difícil diagnóstico / Exogenous Cushings syndrome with a difficult diagnosis

Resumen Introducción: el síndrome de Cushing (SC) es un grupo de signos y síntomas causados por la exposición crónica al exceso de glucocorticoides. El uso de fármacos con glucocorticoides es la causa más frecuente, pero algunos productos vendidos como suplementos nutricionales o medicamentos naturistas para el control de los dolores articulares pueden contenerlos de forma oculta, lo cual dificulta el enfoque diagnóstico Presentación del caso: paciente de 40 años con signos clínico típicos de síndrome de Cushing con resultados discordantes en las pruebas diagnósticas para identificar su origen. Finalmente se logra establecer que el paciente consumía de forma crónica un producto "naturista" conocido como artrin®, el cual fue analizado en el laboratorio de la institución con resultados positivos para cortisol. Conclusión: la exposición crónica a glucocorticoides exógenos, ya sea subrepticia, desconocida o prescrita, causa SC y debe distinguirse tempranamente de las formas endógenas para evitar la realización de pruebas diagnósticas y tratamientos inadecuados.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1500).

Abstract Introduction: Cushing's syndrome (CS) is a group of signs and symptoms caused by chronic exposure to excessive glucocorticoids. The use of medications containing glucocorticoids is the most common cause, but they may be hidden in some products sold as nutritional supplements or naturopathic medications, which makes the diagnostic approach more difficult. Case presentation: this was a 40-year-old patient with typical clinical signs of Cushing's syndrome and discordant results of diagnostic tests to identify its origin. It was ultimately determined that the patient had been chronically taking a "naturopathic" product known as artrin®, which was analyzed in the institution's lab and found to contain cortisol. Conclusion: chronic exposure to exogenous glucocorticoids, whether surreptitious, unknown or prescribed, causes CS and should be promptly distinguished from endogenous forms to avoid inappropriate diagnostic tests and treatments.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1500).

Síndrome de Cushing exógeno por interacción medicamentosa de ritonavir y fluticasona inhalada. Reporte de tres casos pediátricos / Exogenous Cushing syndrome due to drug interaction of ritonavir and inhaled fluticasone. Report of three pediatric cases

El incremento de la expectativa de vida con el advenimiento de la terapia antirretroviral de alta eficacia plantea desafíos en cuanto a la toxicidad e interacciones medicamentosas. El síndrome de Cushing exógeno por interacción entre ritonavir y fluticasona inhalada en niños con diagnóstico de infección por virus de la inmunodeficiencia humana y patología pulmonar crónica es infrecuente. Hasta el momento, hay 20 casos reportados. Se describen 3 casos pediátricos con diagnóstico de infección por virus de la inmunodeficiencia humana y patología pulmonar crónica que presentaron síndrome de Cushing exógeno con fluticasona inhalada en dosis habituales por la interacción medicamentosa entre esta y ritonavir. Los pacientes resolvieron el cuadro clínico luego de 2-4 meses de suspensión de la fluticasona y permanecieron asintomáticos en el seguimiento

The increase in life expectancy with the advent of highly effective antiretroviral therapy poses challenges in terms of toxicity and drug interactions. Exogenous Cushing syndrome by interaction between ritonavir and inhaled fluticasone in children diagnosed with human immunodeficiency virus infection and chronic pulmonary pathology is rare. So far, there are 20 cases reported. Three pediatric cases are reported, with a diagnosis of human immunodeficiency virus infection and chronic pulmonary pathology who presented exogenous Cushing syndrome with inhaled fluticasone at usual doses due to drug interaction between it and ritonavir. The patients resolved the clinical Síndrome de Cushing exógeno por interacción medicamentosa de ritonavir y fluticasona inhalada. Reporte de tres casos pediátricos Exogenous Cushing syndrome due to drug interaction of ritonavir and inhaled fluticasone. Report of three pediatric cases picture after 2-4 months of fluticasone suspension and remain asymptomatic in the follow-up.

Epidemiological, clinical, and laboratory aspects in a case series of canine hyperadrenocorticism: 115 cases (2010-2014) / Aspectos epidemiológicos, clínicos e laboratoriais em uma série de cães com hiperadrenocorticismo: 115 casos (2010-2014)

Diseases' clinical-epidemiological characterization assists in directing the diagnosis. The objective of this study was to describe epidemiological, clinical and laboratorial aspects of a case series of canine hyperadrenocorticism (HAC). One-hundred fifteen records of dogs diagnosed by the low dose dexamethasone suppression test and/or ACTH stimulation test were evaluated. Of the cases, 81.3% were HAC ACTH-dependent and 18.7% HAC ACTH-independent. Females were more affected, representing 69.3% of the cases. The mean age was 10.3±2.5 years and 64.9% were gonadectomized. Most of the patients were small size dogs, weighting less than 10kg (73.9%). The most frequent breeds were: Poodle (27%), Dachshund (17.4%), and Yorkshire Terrier (10.4%). The most frequent clinical manifestations were polyphagia (86%), polydipsia (82.6%), polyuria (80%), abdominal enlargement (82.6%), thin skin (79.1%), muscular weakness (78.3%) and panting (74.8%). However, eventually unusual HAC-associated signs would be present in some dogs in a more important way compared with the classic disease´s clinical signs. The CBC showed neutrophilia (66%), eosinopenia (58.3%) and lymphopenia (42.6%) as main hematological abnormalities. The most common findings in serum biochemistry were increased alkaline phosphatase activity (81.74%), increased ALT activity (62.6%), hypercholesterolemia (66%) and hypertriglyceridemia (54.7%). Urinalysis revealed hyposthenuria in 14.9% and isostenuria in 13.5%; besides proteinuria in 50% of the cases. Abdominal ultrasound showed bilateral adrenal hyperplasia (92.2%) with adrenal asymmetry in 20.8% of the cases, in addition to hepatomegaly (80.9%), biliary sludge (67.8%) and hepatic hyperechogenicity (47.8%). It was concluded that small size gonadectomized female dogs, mainly Poodles and Dachshunds, presented higher frequency in the population studied, and that the main changes observed in clinical and complementary tests were polyphagia, polyuria, polydipsia, abdominal enlargement, adrenal hyperplasia, increased phosphatase alkaline and hyperlipidemia. These results corroborated to a better disease characterization at Brazil. This work concluded that the population studied resembles the profile describe in European and North American epidemiologic studies, and that the HAC dog´s clinical picture looks similar worldwide.(AU)

A caracterização clínica-epidemiológica de doenças auxilia no direcionamento do diagnóstico. O objetivo deste trabalho foi descrever aspectos epidemiológicos, clínicos e laboratoriais de uma série de casos de hiperadrenocorticismo (HAC) canino. Foram avaliados 115 prontuários de cães diagnosticados pelo teste de supressão por baixa dose de dexametasona e/ou teste de estimulação com ACTH. Os casos de HAC ACTH-dependentes representaram 81,3% da população, e 18,7% foram ACTH-independentes. As fêmeas foram mais acometidas, representando 69,3% dos casos. A média de idade foi 10,3 ± 2,5 anos e 64,9% eram gonadectomizados. A maioria dos cães foi de porte pequeno, de até 10 kg (73,9%). As raças mais frequentes foram Poodle (27%), Dachshund (17,4%) e Yorkshire (10,4%). As manifestações clínicas mais relatadas foram polifagia (86%), polidipsia (82,6%), poliúria (80%), abdome pendular (82,6%), atrofia cutânea (79,1%), fraqueza muscular (78,3%) e dispneia (74,8%). Entretanto, eventualmente sinais clínicos pouco associados ao HAC se manifestaram de forma mais importante que os sinais clássicos da doença. O hemograma revelou neutrofilia (66%), eosinopenia (58,3%) e linfopenia (42,6%) como principais alterações hematológicas. Na bioquímica sérica foi observado aumento de fosfatase alcalina (81,74% dos casos), aumento da atividade da ALT (62,6%), hipercolesterolemia (66%) e hipertrigliceridemia (54,7%). A urinálise revelou hipostenúria em 14,9% e isostenúria em 13,5%; além de proteinúria em 50% dos casos. A ecografia abdominal evidenciou hiperplasia bilateral de adrenal (92,2%) com assimetria de adrenais em 20,8% dos casos, além de hepatomegalia (80,9%), lama biliar (67,8%) e hiperecogenicidade hepática (47,8%). Concluiu-se que fêmeas castradas de pequeno porte, principalmente das raças Poodles e Dachshunds, apresentaram maior frequência na população estudada e que as principais alterações observadas clínicas e nos exames complementares foram polifagia, poliúria, polidipsia, aumento abdominal, hiperplasia da adrenal, aumento de fosfatase alcalina e hiperlipidemia. Estes resultados corroboram para melhor caracterização da doença no Brasil. Este estudo concluiu que a população estudada se assemelha ao perfil populacional de cães com HAC descrito em estudos Europeus e Norte Americanos de forma que o perfil dos casos ao redor do globo parece similar.(AU)

Síndrome de Cushing iatrogénico secundario al mal uso de corticoïdes tópicos: Reporte de un caso pediátrico / Iatrogenic Cushing syndrome due to the misuse of topical corticosteroids: Pediatric case report

Los corticoides se utilizan en el tratamiento de muchas enfermedades dermatológicas, debido a sus efectos antiinflamatorios, inmunosupresores y antiproliferativos. Su uso indiscriminado puede ocasionar serios efectos adversos locales y sistémicos, fundamentalmente, en niños y frente a ciertos factores predisponentes. Si bien los corticoides tópicos son seguros y efectivos, se debe prestar principal atención a factores como la edad del paciente, el estado de la barrera cutánea, la localización, la potencia y el vehículo del corticoide antes de su prescripción, para disminuir la posibilidad de efectos adversos. Se presenta a una paciente de 5 meses de edad, la cual concurrió a la consulta por un hemangioma ulcerado en el glúteo izquierdo asociado a un síndrome de Cushing, debido a la aplicación local de un corticoide tópico durante más de 3 meses para tratar dicha lesión.

Corticosteroids are used in the treatment of many dermatological diseases due to their anti-inflammatory, immunosuppressive and antiproliferative effects. Its indiscriminate use can cause serious local and systemic adverse effects, mainly in children and in the presence of certain predisposing factors. While topical corticosteroids are safe and effective, it is important to pay attention to factors such as patient's age, skin barrier, clinical characteristic and localization of the lesion, potency and vehicle of the corticosteroid before its prescription to reduce the possibility of adverse effects. We present the case of a 5-month-old patient who presented an ulcerated hemangioma in the left buttock. Due to the local application of a topical corticosteroid on the hemangioma for more than 3 months, she developed Cushing's syndrome.

Calidad de vida en pacientes operados de hipercortisolismo endógeno / Quality of life in patients operated for endogenous hypercortisolism

RESUMEN Introducción: El hipercortisolismo endógeno provoca cambios en diferentes áreas de la vida en el sentido percibido de bienestar, lo que afecta la calidad de vida; esta debe tenerse en cuenta en la atención integral al paciente. Objetivos: Describir la calidad de vida percibida en los pacientes con hipercortisolismo endógeno e identificar si existe relación entre calidad de vida y algunas características clínicas y hormonales. Métodos: Estudio transversal, 60 pacientes: grupo I (con hipercortisolismo endógeno operados con menos de cinco años) y grupo II (personas con sospecha clínica de hipercortisolismo endógeno), edad entre 20 y 60 años. Se aplicaron cuestionarios de calidad de vida relacionados con el hipercortisolismo endógeno: CushingQoL y Tuebingen CD-25 y planilla de recolección de datos generales, clínicos y hormonales. Se determinó mediana (variables cuantitativas) y frecuencia en las cualitativas. La asociación del puntaje de los cuestionarios y variables cuantitativas, mediante el coeficiente de correlación de Spearman. Se compararon los valores promedio del puntaje entre categorías de las variables cualitativas (clínicas y hormonales categorizadas), mediante la prueba U-Mann Whitney; se consideró diferencia estadística significativa con p ≤ 0,05. Resultados: La mediana del puntaje de los dominios psicoemocional, sexualidad, área social, área corporal, y la suma de todos los dominios fueron mayores en el grupo I. La sexualidad (4,5), el área corporal (6,0) y la suma de todos los dominios (36,5) del I vs. 1,0, 4,0 y 28,0 respectivamente (grupo II), del Tuebingen CD-25 mostraron diferencias estadísticamente significativas, demostrando peor calidad de vida en el grupo I. Con el CushingQoL la mediana del puntaje del grupo I fue menor que la del II (54,1 vs. 74,0), con diferencias estadísticamente significativas (p = 0,00), lo que evidencia peor calidad de vida para el grupo I. Conclusiones: El hipercortisolismo endógeno incide negativamente en la calidad de vida percibida en el grupo de pacientes operados, especialmente en las áreas social, corporal, cognitiva, sexual, y de la conducta alimentaria(AU)

ABSTRACT Introduction: Endogenous hypercortisolism causes changes in different areas of life and the perceived sense of well-being, which affects the quality of life. This should be taken into account in the patient comprehensive care. Objectives: To describe the quality of life perceived in patients with endogenous hypercortisolism and to identify if there is a relationship between quality of life and some clinical and hormonal characteristics. Methods: Cross-sectional study, 60 patients: group I (with endogenous hypercortisolism operated less than five years) and group II (people with clinical suspicion of endogenous hypercortisolism), age between 20 and 60 years. Quality of life questionnaires related to endogenous hypercortisolism were applied: CushingQoL and Tuebingen CD-25 and general, clinical and hormonal data collection form. Median (quantitative variables) and frequency in the qualitative ones were determined. The association of the score of questionnaires and quantitative variables were obtained by means of the Spearman correlation coefficient. The average score values were compared between categories of qualitative variables (clinical and hormonal categorized), using the U-Mann Whitney test; significant statistical difference was considered with p ≤ 0.05. Results: The median score of the psycho-emotional domains, sexuality, social area, body area, and the sum of all domains were higher in group I. Sexuality (4.5), body area (6.0) and the sum of all domains (36.5) of group I vs. 1.0, 4.0 and 28.0 respectively (group II), of the Tuebingen CD-25 showed statistically significant differences, which is worse quality of life in group I. Using the CushingQoL, the median score of group I showed to be lower than in group II (54.1 vs. 74.0) there are statistically significant differences (p = 0.00), which shows worse quality of life in group I. Conclusions: Endogenous hypercortisolism negatively affects the quality of life perceived in the group of operated patients, especially in the social, bodily, cognitive, sexual areas, and eating behaviour(AU)