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Management of squamous cell cancer of the oesophagus in a patient with a polyglandular endocrinopathy (APECED) and achalasia.

INTRODUCTION: We report a case of a 37-year-old man, with a background of a rare polyglandular autoimmune syndrome and achalasia, who developed an oesophageal tumour. Both autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) or type I polyglandular syndrome and achalasia confer increased risk of development of oesophageal squamous cell carcinoma. METHODS: Despite having had multiple endoscopic examinations and dilatations in the recent past, this patient presented with dysphagia, and on endoscopy, he was found to have a mid-oesophageal tumour. A multidisciplinary team appro

Pernicious anemia in a patient with Type 1 diabetes mellitus and alopecia areata universalis.

A 27-year-old male, who had developed diabetes mellitus type 1 (DMT1) since the age of eighteen and alopecia areata universalis nine months later, attended the outpatient clinics complaining of general fatigue and shortness of breath. A Schilling test was indicative of pernicious anemia. Antigastric parietal cell (AGPA) and anti-intrinsic factor antibodies were positive, confirming diagnosis of pernicious anemia. Thyroid and Addison's disease were excluded. Gastroscopy revealed atrophic gastritis without any evidence of carcinoid tumors. The aim of this case, which, to our knowledge, is the fi

Iatrogenic hyperadrenocorticism in a cat following a short therapeutic course of methylprednisolone acetate.

Iatrogenic hyperadrenocorticism (or iatrogenic Cushing's syndrome) is an adrenal disorder that may result from long-term administration of glucocorticoids for therapeutic purposes, most often given to treat allergic or immune-mediated disorders. Prolonged treatment with synthetic glucocorticoids can suppress hypothalamic corticotrophin releasing hormone and plasma adrenocorticotrophic hormone (ACTH), thus causing a functional inactivity of the adrenal cortex. The result is a clinical syndrome of hyperadrenocorticism but with basal and ACTH-stimulated plasma cortisol concentrations that are con

Cushing's syndrome: historical aspects.

A brief overview of the most important steps leading to our present knowledge of hypercorticism is given. The adrenals were described in 1563 and the pituitary had been known since Antiquity. Until Addison's description of adrenal insufficiency in 1855 virtually nothing was known of their function. Cushing in 1912 described his famous patient with hypercorticism but assumed it to be a polyglandular disorder. For almost 40 years the etiology was disputed, though Bauer early had postulated that hypercorticism ultimately reflected adrenal hyperfunction, either primarily or secondarily. Though Kra

Unusual manifestations of type 1 autoimmune polyendocrinopathy.

We present a family with five members affected by Type 1 autoimmune polyendocrinopathy. All patients had chronic mucocutaneous candidiasis and dental abnormalities. Four patients had ocular abnormalities, four had hypoparathyroidism, and three had Addison's disease. The family was unusual in that all four affected females had premature ovarian failure. The ocular abnormalities included two patients with subcapsular lens opacities, one patient with asymptomatic corneal opacities, and one patient with severe bilateral iridocyclitis with cataract formation. One patient had pernicious anaemia and

[Spontaneous rectal hematoma].

Five cases of spontaneous rectus sheath haematomas observed during one year are reported. The female to male ratio was four to one. The age range was 26-75 years. Precipitating factors were cough in three patients, physical exercise in one patient, and none in one patient. In three cases there were possible predisposing factors: anticoagulant therapy, Addison's disease with steroid treatment and abdominal scar. The localisations were three lower right quadrant, one lower left and one upper right. In three cases correct diagnoses were made based on clinical findings, confirmed by ultrasonograph

Altered interaction between triiodothyronine and its nuclear receptors in absence of cortisol: a proposed mechanism for increased thyrotropin secretion in corticosteroid deficiency states.

Thyroid hormones occasionally appear less effective when administered alone to patients with panhypopituitarism, and manifestations suggestive of hypothyroidism have been reported in patients suffering from untreated Addison's disease. In the latter condition, thyrotropin secretion is increased: this occurs already after as little as 2 days of temporary withdrawal of therapy with substitution doses of corticosteroids while circulating levels of thyroid hormones remain within normal limits. Therefore, a possible role of cortisol in interaction between triiodothyronine and its nuclear receptors

Effects of angiotensin II on arginine-vasopressin in physiological and pathological situations in man.

Studies were designed to determine whether angiotensin II has a direct stimulatory effect on arginine-vasopressin in man and to determine the role, if any, played by angiotensin II in the control of vasopressin release in physiological and pathological conditions. Acute infusion of angiotensin II in normal volunteers produced small but definite increases in plasma levels of arginine-vasopressin (5-4+/-0-3(S.E.M.) to 6-4+/-0-2 pg/ml) only when plasma angiotensin II levels were supraphysiological. Concurrent measurements of plasma arginine-vasopressin and angiotensin II were made during acute ch

Lack of ACTH lowering effect of sodium valproate in patients with ACTH hypersecretion.

The effect of an oral dose of 200 or 400 mg sodium valproate (DPA) on ACTH and cortisol secretion was assessed in 11 patients with Cushing's disease (3 bilaterally adrenalectomized), 3 patients with Nelson's syndrome and 6 patients with Addison's disease. In none of the patients examined DPA induced changes in ACTH (and cortisol) levels appreciably different from the fluctuations recorded after placebo administration. The effect of a long term administration of sodium valproate (600-1000 mg/day) was evaluated in 2 patients with active Cushing's disease and in 1 patient with Nelson's syndrome (

Computed tomography in adrenal disease.

Ninety-eight patients with suspected adrenal disease were examined by computed tomography (CT). In 73 patients adrenal disease was confirmed on biochemical and other grounds (Cushing's syndrome (38), primary aldosteronism (8), phaeochromocytoma (12), androgen excess (7), Addison's disease (1) and non-functioning adrenal masses (7)). The CT appearances of hyperplasia and benign and malignant adrenal tumours are described in detail. Ninety-six per cent of the adrenal glands were identified, and all but one of the 39 adrenal mass lesions were correctly identified and localised by CT. The failure

A Case of Autoimmune Polyglandular Syndrome Type 2 in Childhood: Unraveling a Rare and Complex Endocrine Puzzle.

Autoimmune polyendocrine syndrome type 2 (APS-2) is a rare autoimmune disorder characterized by the coexistence of multiple endocrine gland dysfunctions, most commonly type 1 diabetes mellitus (T1DM), autoimmune thyroid disease, and Addison's disease. It typically manifests in middle-aged women, with pediatric cases being exceedingly uncommon. We present the case of a nine-year-old boy with a known history of T1DM diagnosed at the age of six years, who presented to the emergency department with severe hypoglycemia and was subsequently found to have severe hypothyroidism. Clinical features incl

Autoimmune polyendocrine syndromes associated with autoimmune rheumatic diseases.

Autoimmune polyendocrine syndromes (APSs), also called autoimmune polyglandular syndromes, are a group of autoimmune diseases characterized by the co-occurrence of dysfunctions of several (at least two) endocrine glands. They develop under the influence of environmental factors in genetically predisposed people. Autoimmune polyendocrine syndromes may accompany autoimmune rheumatic diseases and worsen their course - APS-2 and APS-3 are the most common. The APS-2 includes the coexistence of, e.g. Hashimoto's disease, celiac disease and rheumatoid arthritis (RA). In APS-3, rheumatic diseases such

Generalized vitiligo and associated autoimmune diseases in Japanese patients and their families.

BACKGROUND: Generalized vitiligo is an acquired disorder in which depigmented macules result from the autoimmune loss of melanocytes from the involved regions of skin. Generalized vitiligo is frequently associated with other autoimmune diseases, particularly autoimmune thyroid diseases (Hashimoto's thyroiditis and Graves' disease), rheumatoid arthritis, adult-onset type 1 diabetes mellitus, psoriasis, pernicious anemia, systemic lupus erythematosus, and Addison's disease. METHODS: One hundred and thirty-three Japanese patients with generalized vitiligo were enrolled in this study to investiga

Заболеваемая беременностью гипертензия и связь с будущими аутоиммунными заболеваниями.

ЦЕЛЬ: изучение связей между гипертоническими расстройствами беременности и последующим развитием аутоиммунных заболеваний. МЕТОДЫ: В этом ретроспективном когортном исследовании использовался TriNetX - федеративная сеть данных реального мира. Используя данные Глобальной сети сотрудничества, мы собрали электронные медицинские записи 102 организаций здравоохранения, 131 million записи пациентов из 2006 в адрес 2020. Исследование позволило оценить риск аутоиммунных заболеваний у женщин пожилого возраста. 16-45 Годы. Two Были сопоставлены следующие категории: обусловленная беременностью когорта гип

Совпадение в аннотации оригинала
OBJECTIVE: To explore the associations between hypertensive disorders of pregnancy and the subsequent development of autoimmune diseases. M … bly higher (hazard ratio 1.87, 95% CI, 1.60-2.18), along with elevated risks of multiple sclerosis, Addison disease, antiphospholipid syndrome, inflammatory bowel disease, mixed connective tissue disease, and rheumatoid arthritis. Subgroup analysis revealed that women of advanced maternal age with pregnancy-induced hypertension had a similar risk of developing au …

Towards an Earlier and Timely Diagnosis of Type 1 Diabetes: Is it Time to Change Criteria to Define Disease Onset?

Type 1 diabetes (T1D) is the immune-mediated form of diabetes requiring insulin treatment and affecting both children and adults. The incidence of T1D is increasing dramatically and has doubled in the past 2 decades. In the recent years, significant knowledge on the disease natural history has been gained and, nowadays, diabetes-related autoantibodies make T1D a predictable disease. Despite this great advance in the field of T1D, we still use diagnostic criteria defined by the American Diabetes Association (ADA) in 1997. In other autoimmune endocrine disorders (e.g., Hashimoto's thyroiditis an

SEVERE HYPERCALCEMIA SECONDARY TO PARAFFIN OIL INJECTIONS IN A BODYBUILDER WITH SIGNIFICANT FINDINGS ON SCINTIGRAPHY.

OBJECTIVE: Non-parathyroid hormone (PTH) mediated hypercalcemia in young patients is rare. It encompasses a broad differential including malignancy, granulomatous diseases, Addison disease, and toxicity of vitamin A and D. We present an unusual case of non-PTH mediated hyper-calcemia in a previously healthy bodybuilder, secondary to multifocal granulomatous disease from paraffin oil injections. METHODS: The patient was evaluated with laboratory tests including serum calcium, 25-hydroxyvitamin D, 1,25-hydroxyvitamin D, parathyroid hormone, and parathyroid hormone-related peptide. Imaging studi…

Hypophosphatasia with Coexisting Endocrinopathies: A Diagnostic Dilemma.

Adult-onset hypophosphatasia presents a diagnostic challenge due to confounding clinical and analytical factors. Although rare, the consequence of missed diagnosis is significant, as it can potentiate skeletal mineralization defects. Despite the recent development of diagnostic criteria, integration into routine clinical practice remains limited, partly due to the variable course of disease progression. Effective management often requires a multidisciplinary team, including rheumatologists, orthopedic surgeons, endocrinologists, medical geneticists, dentists, physical and occupational therapis