Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 51-year-old man and a 39-year-old woman with the hypereosinophilic syndrome developed a restrictive cardiomyopathy in the course of cardiac involvement. Echocardiography demonstrated typical thrombotic obliteration at the apex of both ventricles. Doppler ultrasound showed changes of impaired filling in both ventricles. Glucocorticoid treatment (prednisone, 85 mg daily) had to be drastically reduced (to 10 mg daily) in case 1, because of the development of a Cushing syndrome. The man died 18 months later of advanced heart failure. In the woman the eosinophil count fell after prednisone administration (1 mg/kg daily) and there was no recurrence. But heart failure due to impaired diastolic filling has persisted so that orthotopic cardiac transplantation has been planned.
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PMID:[Cardiac involvement in the hypereosinophilia syndrome. the importance of echocardiography in the follow-up]. 139 38

We describe a female newborn infant with McCune-Albright syndrome. In addition to the cutaneous pigmentation, she had apparent manifestations of hyperthyroidism and Cushing syndrome since birth. X-ray examinations showed many scattered lucencies in multiple bones. Endocrinological findings were as follows: serum T 4 276 nmol/l; free T 4 125 pmol/l; TSH less than 1 mU/l; serum cortisol greater than 2210 nmol/l; plasma ACTH less than 10 pg/ml; urinary free cortisol 865 nmol/day; estradiol 0.36 nmol/l. Regardless of treatment with antithyroid drugs and an inhibitor of 3 beta-hydroxysteroid dehydrogenase, the patient died of cardiac failure at the age of 4 months. Autopsy findings included a follicle cyst in the right ovary and multinodular hyperplasia in the thyroid and both adrenals. To our knowledge such a severe neonatal form of McCune-Albright syndrome has not been described in the literature.
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PMID:A case of neonatal McCune-Albright syndrome with Cushing syndrome and hyperthyroidism. 175 13

We describe a case of Cushing's syndrome complicating pregnancy presented with acute heart failure, hypertension and glucose intolerance. A left adrenal adenoma was removed at 24 weeks of gestation. The pregnancy was ended with an emergency lower-segment Caesarean section at 31 weeks of gestation because of severe pre-eclampsia and HELLP syndrome. The case is reported not only because of its rarity but also to induce the discussion of surgical treatment during pregnancy.
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PMID:Cushing's syndrome in pregnancy secondary to adrenal adenoma. A case report and literature review. 956 49

An extremely ill patient, with Cushing's syndrome caused by an ACTH-secreting pituitary macroadenoma, experienced complications of end-stage cardiomyopathy, profound psychosis, and multiple metabolic disturbances. Initially treated unsuccessfully by a combination of conventional surgical, medical, and radiotherapeutic approaches, he responded dramatically to high-dose long-term mifepristone therapy (up to 25 mg/kg x d). Treatment efficacy was confirmed by the normalization of all biochemical glucocorticoid-sensitive measurements, as well as by the significant reversal of the patient's heart failure, the resolution of his psychotic depression, and the eventual unusual return of his adrenal axis to normal. His 18-month-long mifepristone treatment course was notable for development of severe hypokalemia that was attributed to excessive cortisol activation of the mineralocorticoid receptor, which responded to spironolactone administration. This case illustrates the efficacy of high-dose long-term treatment with mifepristone in refractory Cushing's syndrome. The case also demonstrates the potential need for concomitant mineralocorticoid receptor blockade in mifepristone-treated Cushing's disease, because cortisol levels may rise markedly, reflecting corticotroph disinhibition, to cause manifestations of mineralocorticoid excess.
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PMID:Successful long-term treatment of refractory Cushing's disease with high-dose mifepristone (RU 486). 1150 80

Patients with Cushing's syndrome can rarely present with systolic heart failure as the mainstay feature. In these patients, heart failure is usually secondary to left ventricular hypertrophy (LVH) and not to dilated cardiopathy. We herewith report a patient with Cushing's syndrome, who presented with systolic ventricular failure secondary to dilated myocardiopathy and signs of proximal myopathy as predominant features. All symptoms regressed after successful treatment.
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PMID:Dilated cardiomyopathy as a presenting feature of Cushing's syndrome. 1271 23

Cushing's syndrome secondary to ectopic adrenocorticotropic hormone (ACTH) secretion is rarely observed in breast carcinoma and only four cases have been previously published. We report here the case of a 50-year-old woman who presented with a history of diffuse bone pain associated with multiple hepatic, pulmonary, and bone metastases. A core needle biopsy specimen revealed an invasive ductal carcinoma in the right breast. The patient subsequently developed an ACTH-dependent paraneoplastic Cushing's syndrome and she died of arrhythmia and heart failure, despite treatment. At autopsy, immunohistochemical staining showed chromogranin A and ACTH positivity in the breast tumor and a lung metastasis. The mRNA expression of the pro-opiomelanocortin (POMC) gene was detected in tumoral cells by reverse transcriptase polymerase chain reaction (RT-PCR). This is the first case of Cushing's syndrome secondary to ectopic ACTH secretion where the presence of ACTH by immunohistochemistry and the expression of the POMC gene by RT-PCR have both been demonstrated in a breast carcinoma with metastases. The clinical history and the pathologic findings are presented with the methods and results of the molecular analysis. This case illustrates an example of ectopic ACTH syndrome in a breast carcinoma with neuroendocrine (NE) differentiation. This NE phenotype is directly related to the synthesis of ACTH by the tumoral cells. It should be kept in mind that an ectopic ACTH syndrome may be produced not only by small cell carcinoma or endocrine tumors but also by breast cancer. No relationship has been established between NE features and prognostic factors or patient outcome for this peculiar type of breast carcinoma. The demonstration of mRNA POMC in breast carcinoma with NE features suggests a depression and/or an activation of the POMC gene linked to the NE differentiation.
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PMID:Pro-opiomelanocortin expression in a metastatic breast carcinoma with ectopic ACTH secretion. 1523 95

Edema develops as one of symptoms and signs in several endocrine disorders, and sometimes can be important clue in detecting the basal endocrine disorder. In patients with long-standing hypothyroidism, characteristic edematous skin changes develop and be called myxedema. In hyperthyroid patients with Graves' disease, peripheral edema sometimes develop with or without heart failure. Severe eyelid puffiness composing Graves' ophthalmopathy and 'circumscribing myxedema', mostly in the pretibial regions, are also highly disease-specific disorders. In Cushing's syndrome, both adrenal and ACTH-dependent, peripheral edema is sometimes important sign leading suspicion of this syndrome. In diabetic patients, attention should be paid to edema constantly especially with nephropathy and hypertension. In diabetic nephropathy stage 3B, aggravation of renal function is often progressive. Recently the range of therapeutic options of glycemic controls has been extended with introduction of thiazolidinediones (TZDs). Weight gain and peripheral edema are recognized side effects of these drugs, particularly when used in combination with insulin. The potential risk of worsened heart failure should be taken into consideration when TZDs are used in patients with diabetes and heart diseases.
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PMID:[Edema in endocrine and metabolic diseases]. 1567 23

Hormonal regulation is not possible without the cardiovascular system, and thus the heart plays a special role not only in the action and synthesis, but also in the distribution of hormones. Severe endocrine disorders with cardiac involvement are often threatening for the patient. The impact of aberrant thyroid function, the sympathetic-adrenal symptoms of which predominantly affect the heart, is well known. Diabetes mellitus and the associated metabolic syndrome are major causes of cardiovascular disease and determine its morbidity and lethality rates. Acromegaly causes a complex cardiomyopathy that may result in cardiac failure refractive to conventional treatment. The excessive production of adrenal hormones in Cushing's syndrome, hyperaldosteronism and pheochromocytoma primarily harms the heart by causing severe hypertension. The same holds true for long-standing hyperparathyroidism. Recent prospective studies did not confirm the protective effect of hormone replacement therapy on cardiovascular disease.
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PMID:[Endocrine disorders and the heart]. 1733 54

Cushing's Syndrome (CS) may sometimes lead to dilated cardiomyopathy, even though this condition can be partially or completely reversed after treatment. In this article we report the case of a 28-yr-old woman with CS secondary to adrenal adenoma who exhibited congestive heart failure as an initial symptom. Two weeks before being admitted to our hospital, the patient started complaining of shortness of breath, orthopnea, paroxysmal nocturnal dyspnea and generalized edema. A physical examination did not reveal signs of hypercortisolism. Chest auscultation revealed bilateral diffused crepitation; blood pressure was 180/120 mmHg with heart rate of 90 beats/min. A chest X-ray showed a cardiac shade enlargement due to congestive heart failure. Transthoracic echocardiography demonstrated a dilated left ventricle and an impaired left ventricular systolic function. The patient's urinary cortisol excretion was elevated and circadian rhythm of cortisol was absent. ACTH level was low. In addition, plasma cortisol failed to decrease after administration of dexamethasone. An abdominal magnetic resonance imaging scan showed a 7-cm right adrenal mass. The patient was administered oxygen, spironolactone, ACE-inhibitor and the signs and symptoms of heart failure gradually improved. A laparoscopic right adrenalectomy was performed and pathological examination of the gland showed a benign adrenocortical adenoma. After the adrenalectomy the patient was started on hydrocortisone therapy and 5 months later the wall thickness of the left ventricle was within normal range and the patient's blood pressure was 130/80 mmHg. In conclusion we report the case of heart failure as the main clinical symptom in CS secondary to adrenal adenoma.
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PMID:Cushing's syndrome patient who exhibited congestive heart failure. 1764 30

Endocrine tumors could be defined by their ability to produce structural proteins or hormones common to nervous and endocrine cells. They might induce physiological transforms or outcome adverse events which should be well known in order to prevent or treat them early. The goal of this review was to describe these changes, to describe preoperative assessment, and to discuss intraoperative monitoring and drugs choice based on the literature from the last 30 years. As an example, it should be noticed that: (1) preoperative blood pressure control is essential to prepare phaeochromocytoma for surgery. It should be followed during anaesthesia by intensive fluid load, reversible anaesthetic drugs and rational cardiovascular medications use (as for example remifentanil, sevoflurane, calcium channel blockers and esmolol), and after surgery by narrow clinical and biological monitoring; (2) after medullar thyroid cancer, main adverse events include cervical compressive haematoma and recurrent laryngeal nerve injury as for any thyroid surgery; (3) during pituitary surgery, air embolism might be expected, whereas water dysregulation (diabetes insipidus), corticotroph insufficiency, cerebrospinal fluid (CSF) leak might occur postoperatively. In acromegaly, difficult endotracheal intubation is possible whereas severe Cushing's syndrome may be complicated with hypertensive cardiac failure, infections, thrombosis, delayed cicatrisation; (4) somatostatine analogs are a keystone in carcinoid tumors preoperative and anaesthetic management.
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PMID:[Anaesthesia for endocrine tumor removal]. 1946 26


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