Bilateral adrenal hemorrhage caused by anticoagulant therapy after joint replacement surgery: One case report and literature review / 中华内分泌代谢杂志

Bilateral adrenal hemorrhage is a rare cause of primary adrenal insufficiency, and bilateral adrenal hemorrhage due to anticoagulant use is even rarer. We describe the case of a 62-year-old woman receiving post total knee arthroplasty anticoagulant therapy who presented fever, vomitting, stomachache, and severe fatigue on the 8th day. It was until 4 months later that the patient was finally diagnosed with adrenal insufficiency resuting from bilateral adrenal hemorrhage, her symptoms were relieved by glucocorticoid replacement therapy. In order to promote the awareness, diagnosis, and mangement of post-surgery anticoagulants induced bilateral adrenal hemorrhage, the clinical characteristics of the reported cases were summarized and analyzed.

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.

Approach to the primary adrenal insufficiency caused by anti-phospholipid syndrome / 中华内分泌代谢杂志

The causes of primary adrenal insufficiency(PAI) are varying, however, anti-phospholipid syndrome (APS) is a relatively rare one. PAI lacks unique clinical manifestations, so the confirmation of PAI was easily to be neglected by physicians. We report a case with abdominal pain as the first complaint, followed by multiple infections, thrombotic events, and aggravating fatigue. Through a series of the laboratory examination, medical imaging, and pathology examination, this patient was diagnosed as PAI caused by APS. Combining this report with literature review, we aim to raise awareness of the disease and avoid misdiagnosis and missed diagnosis.

Analysis of clinical manifestations and gene mutations of 13 child patients with rare causes of primary adrenal insufficiency / 中华内分泌代谢杂志

Objective To analyze the clinical manifestations and gene mutations of rare causes of primary adrenal insufficiency (PAI) in childhood.Methods The clinical features,laboratory tests and gene mutation of 13 patients with PAI in our hospital from September 2010 to August 2017 were analyzed retrospectively.Patients with congenital adrenal hyperplasia,X-linked adrenoleukodystrophy with neurological onset or a clear family history,and autoimmune adrenal insufficiency were excluded.Results The median age of 13 cases (12 males,1 female) was 3 years and 10 months.Medical history or clinical manifestations on the first visit included hyperpigmentation,electrolyte imbalance/salt-wasting crisis,gastrointestinal symptoms,and fatigue,etc.All developments of external genitalia were normal.All cases presented with decreased serum cortisol and increased ACTH levels.Some of the cases showed decreased aldosterone level and plasma renin activity,while 17α-hydroxyprogesterone,testosterone,and androstenedione were in the normal range.Part of cases revealed delayed bone age and adrenal atrophy.Three gene mutations were detected in 13 patients,including NR0B 1 gene (9/13),ABCD 1 gene (3/13),and CYP 11A 1 gene (1/13).NR0B1,and ABCD1 gene mutations were pathogenic mutations,consistent with clinical characteristics.CYP11A1 gene mutation was heterozygote,which cannot fully explain the clinical features.Conclusion PAI in childhood presents common clinical manifestations of adrenal insufficiency,e.g.hyperpigmentation and electrolyte imbalance/sah-wasting crisis,but without specificity.Gene mutational analysis is necessary for precise diagnosis and prognosis estimation.NR0B1 and ABCD1 gene mutations were common in childhood with rare causes of PAI.

Hypoglycemia due to 3b-Hydroxysteroid Dehydrogenase type II Deficiency in a Newborn.

Background: 3β-hydroxysteroid dehydrogenase type II deficiency results in decreased production of all three groups of adrenal steroids. Recurrent hypoglycemia as a presenting feature of this disorder has not been reported earlier. Case characteristics: A genotypically and phenotypically normal female newborn delivered by in-vitro fertilization presenting with recurrent hypoglycemia. Primary adrenal insufficiency with insignificant mineralocorticoid deficiency and slightly elevated levels of 17-hydro-xyprogesterone, dehydroepiandrosterone sulphate and testosterone. Outcome: Successfully managed only with corticosteroid replacement. Message: Congenital adrenal hyperplasia can rarely cause recurrent hypoglycemia in newborns.

Addison Disease: Early Detection and Treatment Principles.

Primary adrenal insufficiency, or Addison disease, has many causes, the most common of which is autoimmune adrenalitis. Autoimmune adrenalitis results from destruction of the adrenal cortex, which leads to deficiencies in glucocorticoids, mineralocorticoids, and adrenal androgens. In the United States and Western Europe, the estimated prevalence of Addison disease is one in 20,000 persons; therefore, a high clinical suspicion is needed to avoid misdiagnosing a life-threatening adrenal crisis (i.e., shock, hypotension, and volume depletion). The clinical manifestations before an adrenal crisis are subtle and can include hyperpigmentation, fatigue, anorexia, orthostasis, nausea, muscle and joint pain, and salt craving. Cortisol levels decrease and adrenocorticotropic hormone levels increase. When clinically suspected, patients should undergo a cosyntropin stimulation test to confirm the diagnosis. Treatment of primary adrenal insufficiency requires replacement of mineralocorticoids and glucocorticoids. During times of stress (e.g., illness, invasive surgical procedures), stress-dose glucocorticoids are required because destruction of the adrenal glands prevents an adequate physiologic response. Management of primary adrenal insufficiency or autoimmune adrenalitis requires vigilance for concomitant autoimmune diseases; up to 50% of patients develop another autoimmune disorder during their lifetime.

Enfermedad de Addison en una gemela de 19 meses / Addison´s disease in a 19 months old twin girl

La enfermedad de Addison, o insuficiencia adrenal primaria, es una entidad en la cual la corteza adrenal secreta cantidades insuficientes de glucocorticoides, mineralocorticoides y andrógenos debido a un daño adrenal. Se presenta el caso de una niña de 19 meses con una historia de fatiga crónica, pérdida de peso, vómitos, hiporexia e hiperpigmentación en piel labios y encías. Estos signos y síntomas tenían dos meses de evolución y empeoraron hasta que el diagnóstico correcto fue hecho y el tratamiento adecuado fue iniciado. Es una de las pacientes más jóvenes diagnosticada con enfermedad de Addison en Costa Rica, así como una edad de presentación temprana comparada con el promedio para esta patología. Se compara la paciente con su hermana gemela sana, de manera que los signos de la enfermedad son aún más evidentes y se presenta una revisión de la enfermedad de Addison como diagnóstico que debe estar presente en el diferencial de todo médico general

A Case of Primary Adrenal Insufficiency Presenting as the Initial Clinical Manifestation of Primary Antiphospholipid Antibody Syndrome / 대한류마티스학회지

Primary adrenal insufficiency is one of the rare clinical manifestations of antiphospholipid antibody syndrome. The pathogenesis is mainly ascribed to the hemorrhagic infarction secondary to thrombosis. We describe a 19-year-old man who developed symptoms of adrenal insufficiency and then was diagnosed of primary antiphospholipid antibody syndrome, which is the first reported case in adult patients in Korea. The possibility of adrenal failure should be considered in the management of antiphospholipid antibody syndrome and also, when primary adrenal insufficiency is accompanied by no definite etiology, the antiphospholipid antibody syndrome should be considered as a possible etiology.

Insuficiência adrenal primária como primeira manifestação de câncer pulmonar metastático / Primary adrenal insufficiency as first manifestation of metastatic lung cancer

A insuficiência adrenal primária é, na maioria das vezes, causada por infecções e adrenalite auto-imune. Metástases adrenais são relativamente comuns em neoplasias de pulmão, mas usualmente são assintomáticas, mesmo quando bilaterais. Há poucos relatos de metástases adrenais levando à insuficiência adrenal. Descrevemos aqui um caso de insuficiência adrenal primária como primeira manifestação clínica de neoplasia pulmonar metastática. Paciente de 59 anos, feminina, branca, tabagista, queixava-se de dor em flanco direito associada a náuseas e emagrecimento. Exames laboratoriais confirmaram o diagnóstico de insuficiência adrenal primária. Iniciou tratamento com prednisona e fludrocortisona, com melhora progressiva dos sintomas. Na investigação da etiologia, tomografia computadorizada (TC) de abdômen mostrou aumento bilateral das adrenais. Foi submetida à biopsia de adrenal, com citopatológico positivo para células malignas. Linfonodo supraclavicular esquerdo foi biopsiado, com anátomo-patológico (AP) confirmando adenocarcinoma metastático, com imunohistoquímica sugerindo pulmão como sítio primário. Atentar para o diagnóstico de insuficiência adrenal nesse contexto é importante, porque os sintomas iniciais são inespecíficos, podendo ser atribuídos à neoplasia

Primary adrenal insufficiency is, in most cases, caused by infections and autoimmune adrenalitis. Adrenal metastasis are relatively common in lung cancer, but they are usually asymptomatic, even when bilateral. There are few reports of adrenal metastasis as a cause of adrenal insufficiency. We describe a case of primary adrenal insufficiency presenting as the first clinical manifestation of metastatic lung cancer. A 59 year-old, white, smoker woman, complaining of right flank pain associated with nausea and weight loss. Laboratory exams confirmed the diagnosis of primary adrenal insufficiency. It was started treatment with prednisone and fludrocortisones with progressive improvement. At the etiologic investigation, abdominal computadorized tomography (CT) showed bilateral increase of the adrenal glands. It was performed an adrenal biopsy and the cytologic study was positive for malignant cells. It was made another biopsy, of a supraclavicular lymph node, and the histopathologic study revealed a metastatic adenocarcinoma, immunohistochemistry study suggested lung as the primary site. Awareness of this diagnosis is important because initial symptoms of adrenal insufficiency are unspecific and may misguidedly be attributed to the neoplasm

A case of successful pregnancy in Addison's disease / 대한산부인과학회지

Primary adrenal insufficiency is a rare disease which can lead to fatal outcomes when complicated in pregnancy. The vague symptoms such as nausea, fatigue and abdominal pain may interfere with its diagnosis. But once the diagnosis is made after a simple ACTH stimulation test, a successful pregnancy and delivery can be accomplished by replacement of steroids. It is to be noted that proper management in crises such as labor, delivery, operation and postpartum is very important. We report a case of successful delivery of a woman with primary adrenal insufficiency with a brief review of literature.

A Case of Polygrandular Automune type II syndrome associated empty sella / 대한내분비학회지

The polyglandular autoimmune(PGA) syndrome designate as the dysfimction of endocrine and nonendocrine systems involving two or more organs on the basis of an autoimmune mechanism. There are 3 types of PGA syndrome and their etiology or pathogenesis is still not complete by understood. Type I PGA is present in the patients who have at least two of the triad of Addison's disease, hypopacathyroidism, and chronic mucocutaneous candidiasis. Type II PGA is present in the those who have Addisons disease with autoimmune thyroid disease and/or insulin dependent diabetes mellitus, but who do not have hypoparathyroidism or candidiasis. Type III PGA is present in the one who have autoimmune thyroid disease, without Addisons disease, but with another autoimmune disease report a case of autoimmune polyglandular syndrome type II in a seventy-three years old female patient who had primary adrenal insufficiency, primary hypothyroidism, and empty sella, pulmonary tuberculosis.