Adrenalitis tuberculosa: a propósito de una actualización / Tuberculous adrenalitis: in regard to a recent update

Introducción: la insuficiencia suprarrenal primaria (IA) descrita por Thomas Addison en 1855 atribuía como principal causa a la infección por tuberculosis (TBC) diseminada, pero con el paso del tiempo ha disminuido en los países desarrollados. En aquellos en vías de desarrollo se mantiene alta esta etiología infecciosa, en especial en pacientes con VIH. Objetivo: realizar una revisión narrativa de la literatura reciente sobre la adrenalitis por TBC, incluyendo el enfoque, manejo y seguimiento en los casos de insuficiencia suprarrenal primaria (IA). Materiales y métodos: búsqueda y análisis de los artículos disponibles en los últimos 5 años bajo los descriptores en ciencias de la salud (DeCS) enfermedad de Addison, tuberculosis, insuficiencia suprarrenal primaria y adrenalitis en español en las bases de Google scholar y LILACS, y en inglés en PubMed y ClinicalKey. Conclusiones: la insuficiencia adrenal o adrenalitis por TBC ha descendido como causa de IA primaria, pero en el contexto de reemergencia de infección por VIH, continúa siendo una causa importante de IA en países en desarrollo. En estos casos además de la suplencia con corticosteroides el tratamiento de la causa específica es de importancia para impactar en la respuesta clínica, la supervivencia y la calidad de vida.

Introduction: primary adrenal insufficiency (AI) was described by Thomas Addison in 1855, and the vast majority of cases were attributable to disseminated tuberculosis (TB), but TB has decreased in developed countries over time. Conversely, in developing countries, this infectious etiology remains high, especially in patients with HIV infection. Objective: to perform a narrative review of the recent literature on tuberculous adrenalitis, including the approach, management and follow-up in cases of primary adrenal insufficiency (AI). Materials and methods: search and analysis of articles available over the past 5 years, using Health Sciences Descriptors (DeCS), Addison ́s disease, tuberculosis, primary adrenal insufficiency and adrenalitis, in Spanish in the Google scholar and LILACS databases, and in English in the PubMed and ClinicalKey databases. Conclusions: adrenal insufficiency or adrenalitis due to TB has decreased as a cause of primary AI, but in the context of the resurgence of HIV infection, TB remains an important cause of AI in developing countries. In these cases, in addition to corticosteroid replacement therapy, treatment of the specific cause is important to impact clinical response, survival and quality of life.

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

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

Enfermedad de addison e insuficiencia adrenal aguda: presentación de un caso y revisión de la literatura / Addison disease and acute adrenal insufficiency: case report and literature review

La enfermedad de Addison es una patología endocrinológica ocasionada por la disminución en la secreción de hormonas esteroideas por parte de la corteza adrenal; debida a múltiples etiologías (más comúnmente la tuberculosis en países en vías de desarrollo); con una evolución lenta, insidiosa y progresiva. Pudiendo llegar a una insuficiencia adrenal aguda, misma que puede llegar a ser de extrema gravedad; y en caso de no ser diagnosticada y tratada adecuadamente puede llevar a la muerte. El tratamiento primordial lo constituye la terapia hormonal sustitutiva con fármacos corticoesteroideos.

Addison's disease is an endocrinological pathology caused by the decrease in the secretion of steroid hormones by the adrenal cortex, due to multiple etiologies (most commonly tuberculosis in developing countries); with a slow, insidious and progressive evolution. Being able to reach an acute adrenal insufficiency, which can become extremely serious, and if it is not diagnosed and treated properly, it can lead to death. The primary treatment is hormone replacement therapy with corticosteroid drugs

Infecção de trato urinário inferior por Citrobacter sp em cadela com hipoadrenocorticismo / Citrobacter sp lower urinary tract infection in a bitch with hypoadrenocorticism

Citrobacter sp. é um microrganismo frequentemente encontrado em vagina de cadelas, mas corresponde a menos de 3% do total de microrganismos isolados em urocultura. O hipoadrenocorticismo (HA) é uma doença endócrina incomum e que leva a poliúria e hipostenúria. O objetivo deste trabalho é relatar o caso de uma fêmea da espécie canina, da raça Teckel, 11 anos, atendida com queixa de urina de odor alterado há vários dias, além de vômito, diarreia e inapetência há três dias, e poliúria e polidipsia há 2 meses, com histórico de tratamento anterior com mitotano para hiperadrenocorticismo. O diagnóstico foi de ITU por Citrobactersp., além de HA iatrogênico. Como destaques nos exames, relação sódio:potássio de 22,6 foi observada, sugestiva de HA, que foi confirmado por teste de estimulação com ACTH, além de urina inicialmente hipostenúrica (densidade 1,006), o que pode ser atribuído ao washout medular renal devido à hiponatremia causada pelo hipoadrenocorticismo. O tratamento foi feito com amoxicilina associada a ácido clavulânico por 20 dias, além da terapia específica para HA com prednisona e fludrocortisona, que foi continuado. A paciente recuperou-se bem, a densidade urinária aumentou após início do tratamento para HA e não houve recidiva da ITU em período de acompanhamento de 8 meses. Acredita-se que a baixa densidade urinária causada pelo HA tenha sido fator essencial para a ocorrência da ITU por Citrobacter.

Citrobacter sp. is a normal constituent of bitches' vagina, but it is related to less than 3% of total isolated microorganism in uroculture. Hypoadrenocorticism (HA) is an endocrine disease uncommonly diagnosed that leads to polyuria and hypostenuria. The aim of this work is report the case of a Teckel bitch, 11 years old, attended with complaint of altered urinary smell for several days, besides vomiting, diarrhea and inappetence for three days, and polyuria and polydispsia for two months, with previous mitotane treatment for hyperadrenocorticism. The diagnosis was Citrobacter UTI, besides iatrogenic HA. The most important exam results include sodium:potassium ratio of 22.6, suggestive of HA, that was confirmed by ACTH stimulation test. Besides, hypostenuria (urinary specific gravity ­ USG - of 1.006) was noted, attributed to renal medular washout due to hyponatremia seen in HA. Therapy comprised amoxicillin associated with clavulanic acid for 20 days, in addition to specific therapy for AH with prednisone and fludrocortisone. The patient recovery well, USG increased after the beginning of the treatment for HA and there was no UTI reinfection for a 8 month period. It is believed that low USG due to HA was essential for the occurrence of Citrobacter UTI.

Novel ABCD1 Gene Mutation in a Korean Patient with X-Linked Adrenoleukodystrophy Presenting with Addison's Disease

X-linked adrenoleukodystrophy (X-ALD) occurs due to mutations in the ABCD1 gene that encodes the peroxisomal membrane protein peroxisomal transporter ATP-binding cassette sub-family D member 1 (ABCD1). Degradation of very long-chain fatty acids in peroxisomes is impaired owing to ABCD dysfunction, subsequently leading to adrenomyeloneuropathy, cerebral adrenoleukodystrophy, and adrenal insufficiency. X-ALD frequently induces idiopathic Addison's disease in young male patients. Here, we confirmed the diagnosis of X-ALD in a young male patient with primary adrenal insufficiency, and identified a novel ABCD1 gene mutation (p.Trp664*, c.1991 G>A).

Autoimmune Polyglandular Syndrome type 2

SUMMARY Autoimmune polyglandular syndrome type 2 (APS 2) is defined by the presence of Addison's disease (AD) associated with autoimmune thyroid disease and/or Type 1 diabetes mellitus (T1DM). It is a rare disease, affecting about 1.4-2 cases/100,000 inhabitants. Its less frequent clinical presentation is the combination of AD, Graves' disease, and T1DM. We present the case of a 42-year-old woman with a history of total thyroidectomy due to Graves' disease, type 2 diabetes mellitus, and hypertension, who sought the ED due to asthenia, dizziness, nausea, and vomiting. She reported having stopped antihypertensive therapy due to hypotension and presented a glycemic record with frequent hypoglycemia. On physical examination, she had cutaneous hyperpigmentation. She had no leukocytosis, anemia, hypoglycemia, hyponatremia or hyperkalemia, and a negative PCR. Serum cortisol <0.5 ug/dl (4,3-22,4), urine free cortisol 9 ug/24h (28-214), ACTH 1384 pg/mL (4,7-48,8), aldosterone and renin in erect position of 0 pg/ml (41-323) and 430.7 uUI/ml (4.4-46.1) respectively. Quantiferon TB was negative; computerized axial tomography of the adrenals showed no infiltrations, hemorrhage, or masses. The 21-hydroxylase antibody assay was positive. B12 vitamin was normal, anti-GAD antibodies were positive, anti-insulin, anti-IA2, and anti-transglutaminase antibodies were all negative. The patient started insulin therapy and treatment for AD with prednisolone and fludrocortisone with good clinical response. This case aims to alert to the need for high clinical suspicion in the diagnosis of AD. Since this is a rare autoimmune disease, it is important to screen for other autoimmune diseases in order to exclude APS.

RESUMO A síndrome poliglandular autoimune tipo 2 (SPGA2) é definida pela presença de doença de Addison (DA) associada à doença tiroideia autoimune e/ou diabetes mellitus tipo 1 (DMT1). Trata-se de uma doença rara, afetando cerca de 1,4-2 casos/100.000 habitantes. A apresentação clínica menos frequente é a combinação de DA, doença de Graves e DMT1. Apresenta-se mulher de 42 anos, com antecedentes de tiroidectomia total por doença de Graves, diabetes mellitus tipo 2 e hipertensão, que recorre ao SU por quadro arrastado de astenia, emagrecimento, tonturas, náuseas e vômitos. Referia ter suspendido terapêutica anti-hipertensora por hipotensão e apresentava registro glicêmico com hipoglicemias frequentes. Ao exame físico, salientava hiperpigmentação cutânea. Analiticamente sem leucocitose, anemia, hipoglicemia, hiponatremia ou hipercaliemia, PCR negativa. Cortisol sérico matinal <0,5 ug/dl (4,3-22,4), cortisol livre na urina 9 ug/24h (28-214), ACTH 1.384 pg/mL (4,7-48,8), aldosterona e renina em posição ereta de 0 pg/mL (41-323) e 430,7 uUI/mL (4,4-46,1), respectivamente. Realizado estudo complementar para averiguar causa de insuficiência suprarrenal primária. Quantiferon TB negativo, tomografia axial computadorizada das suprarrenais sem infiltrações, hemorragia ou massas. Anticorpos anti-21-hidroxilase positivos. Foi aprofundada a investigação com vitamina B12 normal, anti-GAD positivo, anti-insulina, anti-IA2, antitransglutaminase, negativos. Nesse contexto, a doente iniciou insulinoterapia e tratamento dirigido para a DA com prednisolona e fludrocortisona, com boa resposta clínica. Este caso tem como objetivo alertar para a necessidade de elevada suspeição clínica no diagnóstico de DA. Sendo esta uma doença autoimune rara, é importante rastrear outras doenças autoimunes no sentido de excluir SPGA.

Doença de Addison e esclerose múltipla: relato de caso / Addison's disease and multiple sclerosis: case report

A doença de Addison é uma endocrinopatia rara, de etiologia autoimune. É caracterizada por défice na secreção de glicocorticoides e mineralocorticoides. A esclerose múltipla consiste em patologia neurológica, de origem autoimune, que resulta na desmielinização da bainha de mielina. O objetivo deste relato foi demonstrar a associação rara entre essas duas patologias e suas possíveis relações imunológicas. A paciente analisada é do sexo feminino, 41 anos, portadora de esclerose múltipla, que posteriormente foi diagnosticada com insuficiência adrenal primária. (AU)

Addison's disease is a rare endocrinopathy of autoimmune etiology. It is characterized by a secretion's deficit of glucocorticoids and mineralocorticoids. Multiple sclerosis is a neurological pathology of autoimmune origin, which results in demyelination of the myelin sheath. The purpose of this report is to demonstrate the uncommon association between these two pathologies and their possible immunological relationships. The analyzed patient is a woman, 41 years old, with multiple sclerosis, who was later diagnosed with primary adrenal insufficiency. (AU)

Autoimmune Diseases and Gastric Cancer Risk: A Systematic Review and Meta-Analysis / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Autoimmunity is an alternative etiology of gastric inflammation, the initiating event in the gastric carcinogenic cascade. This mechanism may be an increasingly important cause of gastric cancer with the waning prevalence of its primary etiologic factor, chronic Helicobacter pylori infection. MATERIALS AND METHODS: PubMed and EMBASE were searched up to September 2018. Autoimmunity and 96 specific manifestations were considered for associations with gastric cancer risk. Random effects analysis was used to calculate pooled relative risk estimates (RR) and 95% confidence intervals (CI). RESULTS: We found a total of 52 observational studies representing 30 different autoimmune diseases. Overall, the presence of an autoimmune condition was associated with a gastric cancer pooled RR of 1.37 (95% CI, 1.24 to 1.52). Among the 24 autoimmune conditions with two or more independent reports, nine were significantly associated with increased gastric cancer risk: dermatomyositis (RR, 3.69; 95% CI, 1.74 to 7.79), pernicious anemia (RR, 2.84; 95% CI, 2.30 to 3.50), Addison disease (RR, 2.11; 95% CI, 1.26 to 3.53), dermatitis herpetiformis (RR, 1.74; 95% CI, 1.02 to 2.97; n=3), IgG4-related disease (RR, 1.69; 95% CI, 1.00 to 2.87), primary biliary cirrhosis (RR, 1.64; 95% CI, 1.13 to 2.37), diabetes mellitus type 1 (RR, 1.41; 95% CI, 1.20 to 1.67), systemic lupus erythematosus (RR, 1.37; 95% CI, 1.01 to 1.84), and Graves disease (RR, 1.27; 95% CI, 1.06 to 1.52). CONCLUSION: Our analysis documents the wide range of autoimmune diseases associated with gastric cancer. These associations may reflect unreported links between these conditions and autoimmune gastritis. Further studies are warranted to investigate potential causal mechanisms.

Vitiligo in a Patient with Systemic Lupus Erythematosus: A Case Report / 대한피부과학회지

Vitiligo is a multifactorial disorder. Neural, biochemical, and autoimmune mechanisms have been hypothetically suggested as etiopathological contributors to this condition. Autoimmunity focuses primarily on genetic factors and the association between vitiligo and other autoimmune disorders including autoimmune thyroid disease, rheumatoid arthritis, psoriasis, type 1 diabetes, pernicious anemia, and Addison's disease. We describe a 35-year-old man with systemic lupus erythematosus who developed concurrent vitiligo and discoid lupus erythematosus suggesting the possible autoimmune association between these 2 different diseases.

Multifocal oral melanoacanthoma associated with Addison's disease and hyperthyroidism: a case report

SUMMARY Oral melanoacanthoma is a mucocutaneous, pigmented, rare, benign, and probably reactive lesion. This paper reports for the first time in the literature a case of multifocal oral melanoacanthoma in a patient diagnosed with Addison's disease and concomitant Graves' disease with hyperthyroidism. The patient presented with oral pigmented lesions, which were hypothesized to be mucosal pigmentation associated with Addison's disease. Due to their unusual clinical pattern, these oral lesions were biopsied and diagnosed as oral melanoacanthoma on histopathology and immunohistochemistry for HMB-45. At the moment of this report, the patient was being treated for her systemic conditions, but the lesions had not regressed. Reactive hyperpigmentation of the skin and mucous membranes may be found in Addison's disease and hyperthyroidism. This case reinforces the hypothesis of a reactive nature for oral melanoacanthoma and highlights the need for investigation of endocrine disorders in patients with multifocal oral melanoacanthoma.

Characteristics of Korean Patients with Primary Adrenal Insufficiency: A Registry-Based Nationwide Survey in Korea

BACKGROUND: Primary adrenal insufficiency (PAI) is a rare, potentially life-threatening condition. There are few Korean studies on PAI, and most have had small sample sizes. We aimed to examine the etiology, clinical characteristics, treatment, and mortality of PAI in Korean patients. METHODS: A nationwide, multicenter, registry-based survey was conducted to identify adults diagnosed with or treated for PAI at 30 secondary or tertiary care institutions in Korea between 2000 and 2014. RESULTS: A total of 269 patients with PAI were identified. The prevalence of PAI was 4.17 per million. The estimated incidence was 0.45 per million per year. The mean age at diagnosis was 49.0 years, and PAI was more prevalent in men. Adrenal tuberculosis was the most common cause of PAI in patients diagnosed before 2000; for those diagnosed thereafter, adrenal metastasis and tuberculosis were comparable leading causes. The etiology of PAI was not identified in 34.9% of cases. Of the patients receiving glucocorticoid replacement therapy, prednisolone was more frequently administered than hydrocortisone (69.4% vs. 26.5%, respectively), and only 27.1% of all patients received fludrocortisone. We observed an increased prevalence of metabolic disease and osteoporosis during the follow-up period (median, 60.2 months). The observed overall mortality and disease-specific mortality rates were 11.9% and 3.1%, respectively. CONCLUSION: The prevalence of PAI is significantly lower in Koreans than in reports from Western countries. The high frequency undetermined etiology in patients with PAI suggests the need to reveal accurate etiology of PAI in Korea.

Adrenal Tuberculosis Mimicking a Malignant Tumor with Primary Adrenal Insufficiency

Adrenal tuberculosis is a relatively infrequent cause of primary adrenocortical insufficiency in developed countries. Isolated adrenal tuberculosis, especially with enlargement of adrenal glands, can cause diagnostic problems and requires differentiation from primary or secondary neoplasms. A 40-year-old woman presented to Soonchunhyang University Gumi Hospital with altered consciousness. She showed biochemical evidence of adrenal insufficiency without signs of tuberculosis. Abdominal computed tomography (CT) scan showed a calcified mass 3.3×2.3 cm in size and peripheral enhancement of the left adrenal gland. A laparoscopic adrenalectomy was performed for a diagnosis of left adrenal malignant tumor. The diagnosis of adrenal tuberculosis was confirmed by a final histological examination.

Conduct protocol in emergency: Acute adrenal insufficiency / Protocolo de condutas em emergência: insuficiência adrenal aguda

Summary Introduction: Acute adrenal insufficiency or addisonian crisis is a rare comorbidity in emergency; however, if not properly diagnosed and treated, it may progress unfavorably. Objective: To alert all health professionals about the diagnosis and correct treatment of this complication. Method: We performed an extensive search of the medical literature using specific search tools, retrieving 20 articles on the topic. Results: Addisonian crisis is a difficult diagnosis due to the unspecificity of its signs and symptoms. Nevertheless, it can be suspected in patients who enter the emergency room with complaints of abdominal pain, hypotension unresponsive to volume or vasopressor agents, clouding, and torpor. This situation may be associated with symptoms suggestive of chronic adrenal insufficiency such as hyperpigmentation, salt craving, and association with autoimmune diseases such as vitiligo and Hashimoto's thyroiditis. Hemodynamically stable patients may undergo more accurate diagnostic methods to confirm or rule out addisonian crisis. Delay to perform diagnostic tests should be avoided, in any circumstances, and unstable patients should be immediately medicated with intravenous glucocorticoid, even before confirmatory tests. Conclusion: Acute adrenal insufficiency is a severe disease that is difficult to diagnose. It should be part of the differential diagnosis in cases of hypotensive patient who is unresponsive to vasoactive agents. Therefore, whenever this complication is considered, health professionals should aim specifically at this pathology.

Resumo Introdução: a insuficiência adrenal aguda ou crise addisoniana é uma comorbidade rara na emergência; porém, se não diagnosticada e tratada de forma correta, pode evoluir de maneira desfavorável. Objetivo: alertar a todos os profissionais da saúde sobre o diagnóstico e tratamento corretos dessa complicação. Método: foi realizada uma ampla pesquisa na literatura médica, por meio de ferramentas específicas, sendo selecionados 20 artigos sobre o tema. Resultados: a crise addisoniana é de difícil diagnóstico pela inespecificidade de seus sinais e sintomas. No entanto, pode ser suspeitada em pacientes que chegam à emergência com queixa de dor abdominal, hipotensão não responsiva a volume ou a agentes vasopressores, obnubilação e torpor. Esse quadro pode vir associado a sintomas sugestivos de insuficiência adrenal crônica, como hiperpigmentação e avidez por sal, bem como a doenças autoimunes, como vitiligo e tireoidite de Hashimoto. Pacientes estáveis hemodinamicamente podem passar por métodos diagnósticos mais apurados para se confirmar ou descartar a crise addisoniana. Os exames diagnósticos não podem retardar, em hipótese alguma, o tratamento de pacientes instáveis, que deve ser iniciado imediatamente com glicocorticoide endovenoso, inclusive antes das provas confirmatórias. Conclusão: a insuficiência adrenal aguda é uma patologia grave e de difícil diagnóstico, que deve fazer parte do diagnóstico diferencial do médico ao atender um paciente hipotenso sem reposta à infusão de drogas vasoativas. Logo, na suspeita dessa complicação, o profissional não deve tardar a agir especificamente nessa patologia.

Primary hypoadrenocorticism in a dog / Hipoadrenocorticismo primário em um cão / Hipoadrenocorticismo primario en un perro

This report describes the clinical and laboratorial findings as well as the therapeutic protocol performed in a three-year-old mongrel female intact dog, referred to the Veterinary Hospital of FAMEZ/UFMS. The animal had a previous history of recurrent gastrointestinal signs (such as lethargy, vomiting, loss of appetite, melena and abdominal pain), acute crisis episodes, bradycardia, hypotension, hypothermia and increase of capillary refill time, recognized as addisonian crisis due to primary hypoadrenocorticism. Laboratorial findings included anemia, eosinophilia, neutrophilia, lymphocytosis, sodium-potassium ratio of 14,02 mEq/L and prerenal azotemia. Based on that, it was confirmed the diagnosis of primary hypoadrenocorcitism. Thus, it was recommended supplementation therapy with mineralocorticoid (aldosterone) and glucocorticoid (cortisol) corresponding respectively, fludrocortisone acetate of 0.2 mg per kg of BW, by mouth, once daily and prednisone 0.2 mg per kg of BW, by mouth, twice daily until further recommendations. The prognostic was excellent, since the animal significantly improved body condition, andclinical signs disappeared after therapy which lead the sodium-potassium ratio to 35.11 mEq/L. Thus, the clinician must always suspect of primary hypoadrenocorticism in dogs with intermittent nonspecific signs that get better with support therapy. Presumably, hypoarenocorticism must be under diagnosed in veterinary medicine, reinforcing the need to require specific exams in patients that show this wax and wane feature of clinical signs.

O presente relato descreve os achados clínicos, laboratoriais e conduta terapêutica de um animal da espécie canina, fêmea, com três anos de idade, inteiro, sem raça definida, diagnosticado com hipoadrenocorticismo primário atendido no Hospital Veterinário da FAMEZ/UFMS. O animal apresentou histórico de recidivas de sinais gastrintestinais (letargia, vômitos, perda de apetite, melena e dor abdominal), crise adrenal aguda, bradicardia, hipotensão, hipotermia e aumento do tempo de preenchimento capilar. As alterações laboratoriais compreenderam linfocitose, anemia, eosinofilia, neutrofilia, densidade urinária < 1.030, relação sódio: potássio 14,02 mEq/L e azotemia pré-renal. Baseado nos achados clínicos-laboratoriais confirmou-se o hipoadrenocorticismo primário. Em seguida, foi instituído terapia de suplementação de mineralocorticoide (aldosterona) e glicocorticoide (cortisol), correspondendo respectivamente ao acetato de fludrocortisona na dose de 0,2 mg/kg por via oral uma vez ao dia e prednisona 0,2 mg/kg por via oral duas vezes por dia até novas recomendações. O prognóstico foi excelente para este caso, já que houve melhora significativa do animal, com o desaparecimento dos sinais clínicos e com nova relação sódio: potássio de 35,11 mEq/L. Assim, deve-se sempre suspeitar de hipoadrenocorticismo primário canino em pacientes com o curso de aparecimento e desaparecimento com sinais inespecíficos que melhorem com terapia de suporte. Presume-se que o hipoadrenocorticismo primário em cães seja subdiagnosticado na medicina veterinária, por isso a importância dos clínicos em suspeitar e solicitar exames específicos em pacientes que apresentam esse curso da doença.

El informe describe los hallazgos clínicos, de laboratorio y manejo terapéutico de un perro, hembra, con tres años de edad, entera, mestizo, con diagnóstico de hipoadrenocorticismo primario atendido en el Hospital Veterinario de la FAMEZ/UFMS. El animal tuvo un historial de signos gastrointestinales recurrentes (letargia, vómitos, pérdida de apetito, melena y dolor abdominal), crisis renal aguda, bradicardia, hipotensión, hipotermia y un aumento del tiempo de llenado capilar. Las alteraciones de laboratorio presentaron linfocitosis, anemia, eosinofilia, neutrofilia, densidad de la orina < 1,030, relación sodio: potasio 14,02 mEq/L y azotemia prerrenal. Con base en los hallazgos clínicos y de laboratorio, se confirmó el hipoadrenocorticismo primario. A continuación, se introdujo terapia con administración de mineralocorticoide (aldosterona) y glucocorticoide (cortisol), que correspondieron respectivamente al acetato de fludrocortisona a una dosis de 0,2mg/kg por vía oral una vez al día y prednisona 0,2 mg/kg por vía oral dos veces al día hasta nuevas recomendaciones. El pronóstico fue excelente para este caso, ya que hubo mejora significativa del animal, desapareciendo los signos clínicos y con una nueva relación sodio: potasio de 35,11 mEq/L. Por lo tanto, siempre se debe sospechar del hipoadrenocorticismo primario canino en pacientes con el curso de aparecimiento y desaparecimiento con signos inespecíficos que mejoran con terapia de soporte. Es posible que el hipoadrenocorticismo primario en perros sea diagnosticado en la medicina veterinaria, así la importancia de los clínicos en sospechar y solicitar exámenes específicos en pacientes que presentan ese curso de la enfermedad.

Addison Disease Due to Adrenal Tuberculosis Presenting as an Abscess in a Nearby Psoas Muscle with a Duodenal Fistula / 대한내과학회지

An 80-year-old male with nausea and poor oral intake was referred for evaluation of hyponatremia. Primary adrenal insufficiency was diagnosed by a rapid adrenocorticotropic hormone (ACTH) stimulation test. The cause of the adrenal insufficiency was revealed to be adrenal tuberculosis presenting as a bilateral adrenal mass on computed tomography imaging. During the first few months of treatment, the size of the tuberculous mass increased and spread to an adjacent area, and further adrenal hormone replacement was needed. In addition, there was a newly developed tuberculous abscess in a nearby psoas muscle with a duodenal fistula. Thus, we report a case of a long-term clinical course of Addison's disease with changes in hormone replacement as a result of active adrenal tuberculosis, together with a review of the literature.

Acute adrenal insufficiency as the primary manifestation of extrapulmonary tuberculosis: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Acute adrenal insufficiency (AI) is a life-threatening condition. While Addison’s disease (AD) is rare, in developing countries, tuberculosis (TB) still remains as the primary cause in 7 to 20% of cases. Urinary TB is also the third most common form of extrapulmonary disease. We report a case of 37-year-old male who presented with weakness, anorexia, weight loss, dysuria, flank pain and low grade fever. Examination revealed hypotension, hyperpigmentation, hyponatremia, hypoglycemia and low serum cortisol. He was diagnosed to have adrenal crisis due to Addison’s disease and extrapulmonary TB manifesting as urinary tract infection (UTI). He was treated with corticosteroids and anti-TB medications. Urologic reconstructive surgery was subsequently planned.

Primary Adrenal Insufficiency in a Newborn With Adrenal Hypoplasia Congenita Caused by a Mutation of the DAX1 Gene

Adrenal hypoplasia congenita (AHC) is a rare inherited disorder of the adrenal gland caused by deletion or mutation of the dosage-sensitive sex-reversal AHC critical region on the X chromosome, gene 1 (DAX1) gene. The DAX1 gene is expressed in the adrenal cortex, the pituitary gland, the hypothalamus, the testis, and the ovary. Most affected infants present with failure to thrive, salt wasting, and hypoglycemic seizure in early life. Immediate mineralocorticoid and glucocorticoid replacement is essential. Most boys with AHC present with hypogonadotropic hypogonadism, resulting in failure to enter puberty and the need for testosterone treatment. However, a recent study revealed that the onset of puberty in boys with AHC can be variable, ranging from arrested or absent to precocious. We describe a case involving a newborn who presented with primary adrenal insufficiency due to a mutation of the DAX1 gene and was finally diagnosed with AHC.

Enfermedades autoinmunitarias asociadas a diabetes mellitus tipo 1A / Autoimmune diseases in type 1A diabetes mellitus

Type 1A diabetes (DM1A) is an autoimmune disease that comprises 10% of patients with diabetes mellitus. Its frequency is gradually increasing in countries like Mexico. Patients with DM1A commonly have hypothyroidism, Addison disease, celiac disease and less common diseases such as polyglandular syndrome. These diseases are related to susceptibility genes such as HLA, CTLA-4 and PTPN22, which induce central and peripheral immunologic tolerance. This review article emphasizes the importance of searching other autoimmune diseases in patients with DM1A, to improve their prognosis and quality of life.

A Case of Addison's Disease Accompanied by Painless Thyroiditis and Hyperkalemic Periodic Paralysis / 대한내과학회지

In cases of hyperkalemia with preserved renal function, the differential diagnoses that should be considered are drug-related disorders, primary tubular disease, and hormonal diseases including primary adrenal insufficiency. Addison's disease represents a rare disorder characterized by primary adrenal failure, general weakness, poor appetite, nausea, dizziness, and hyperpigmentation. It may also cause fatal adrenal crisis, involving hypotension, loss of consciousness, hyperkalemia, or hyperkalemic periodic paralysis under stressful conditions. We describe herein the case of a 54-year-old Korean male who developed Addison's disease, due to adrenal tuberculosis, in addition to painless thyroiditis, which led to hyperkalemic periodic paralysis.

A Case of Adrenal Tuberculosis Combined with Tuberculous Peritonitis-Induced Adrenal Crisis / 대한내과학회지

An adrenal crisis is a serious medical emergency. Most such crises develop in patients with Addison's disease when they are stressed. However, the clinical features of an impending adrenal crisis are nonspecific, delaying diagnosis and management. Adrenal tuberculosis is very rare among the extrapulmonary forms of tuberculosis. We herein report a case of an adrenal crisis triggered by bilateral adrenal tuberculosis. A 35-year-old man was admitted to our hospital because of drowsiness, fever, and hypotension. Abdominal computed tomography revealed bilateral adrenal enlargement, and computed tomography-guided adrenal gland biopsy allowed us to diagnose adrenal tuberculosis.