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09-SEPTEMBER-2008 02:07:44 - aldosteronism Redirected from Conn syndrome Primary hyperaldosteronism Classification and external resources Aldosterone ICD-10 E26.0 ICD-9 255.1 DiseasesDB 3073 MedlinePlus 000330 eMedicine med/432 MeSH D006929 Primary aldosteronism, also known as primary hyperaldosteronism and Conn syndrome, is characterized by the overproduction of the mineralocorticoid hormone aldosterone by the adrenal glands.1 Aldosterone causes increase in sodium and water retention and potassium excretion in the kidneys, leading to arterial hypertension high blood pressure. Also increase production of mineralocorticoid from the adrenal gland is evident. It is a rare but recognised cause of hypertension. Contents 1 Signs, symptoms and findings 2 Diagnosis 3 Causes 4 Therapy 5 Eponym 6 References Signs, symptoms and findings Primary hyperaldosteronism, aside from high blood pressure, may manifest with muscle cramps due to hyperexcitability of neurons, muscle weakness due to hypoexcitability of skeletal muscles, headaches due to the low potassium, and metabolic alkalosis due to increased secretion of H+ ions by the kidney. The high pH of the blood makes calcium less available to the tissues and causes symptoms of hypocalcemia low calcium levels. It can be mimicked by liquorice ingestion glycyrrhizin and Liddle syndrome. Diagnosis Measuring aldosterone alone is not considered adequate to diagnose primary hyperaldosteronism. Rather, both renin and aldosterone are measured, and the ratio is diagnostic.23 Usually, renin levels are suppressed, leading to a very low renin-aldosterone ratio 0.0005. This test is confounded by antihypertensive drugs, which have to be stopped up to 6 weeks. If plasma levels of renin and aldosterone suggest hyperaldosteronism, CT scanning can confirm the presence of an adrenal adenoma. If the clinical presentation primarily involves hypertension and elevated levels of catecholamines, CT or MRI scanning can confirm a tumor on the adrenal medulla, typically a pheochromocytoma. Causes The syndrome is due to: aldosterone-secreting adrenal adenoma benign tumor, 50-60% hyperplasia of the adrenal gland 40-50% rare forms Therapy The treatment for hyperaldosteronism depends on the underlying cause. In patients with a single benign tumor adenoma, surgical removal adrenalectomy is curative. This is usually performed laparoscopically, through several very small incisions. For patients with hyperplasia of both glands, successful treatment is often achieved with spironolactone or eplerenone, drugs that block the effect of aldosterone. In males, one common side effect of the lower-cost spironolactone drug therapy sometimes seen is gynecomastia. Fortunately, gynecomastia usually does not occur with the slightly more costly eplerenone drug therapy. In the absence of proper treatment, individuals with hyperaldosteronism often suffer from poorly controlled high blood pressure, which may be associated with increased rates of stroke, heart disease, and kidney failure. With appropriate treatment, the prognosis is excellent.4 Eponym It is named after Dr Jerome W. Conn 1907-1994, the American endocrinologist who first described the condition at the University of Michigan in 1955.1 References ^ a b Conn JW, Louis LH 1955. Primary aldosteronism: a new clinical entity. Trans. Assoc. Am. Physicians 68: 215-31; discussion, 231-3. PMID 13299331. ^ Tiu S, Choi C, Shek C, Ng Y, Chan F, Ng C, Kong A 2005. The use of aldosterone-renin ratio as a diagnostic test for primary hyperaldosteronism and its test characteristics under different conditions of blood sampling. J Clin Endocrinol Metab 90 1: 72-8. doi:10.1210/jc.2004-1149. PMID 15483077. ^ United Bristol Healthcare NHS Trust, the major teaching trust in South West England ^ Columbia Adrenal Center, Hyperaldosteronism Conn's Syndrome v d e Endocrine pathology: endocrine diseases E00-35, 240-259 Pancreas/ glucose metabolism Diabetes mellitus types: type 1, type 2, MODY, complications: coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor Rabson-Mendenhall syndrome - Insulin resistance Hypothalamic/ pituitary axes Pituitary Hyperpituitarism Acromegaly, Hyperprolactinaemia, SIADH Hypopituitarism Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus Adiposogenital dystrophy - Empty sella syndrome - Pituitary apoplexy - ACTH deficiency Thyroid Hypothyroidism Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema Hyperthyroidism Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii Thyroiditis De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis Euthyroid sick syndrome - Thyroid hormone resistance - Thyroid nodule Parathyroid Hypoparathyroidism Pseudohypoparathyroidism - Hyperparathyroidism Primary, Secondary, Tertiary Adrenal Adrenocortical hyperfunction: Cushing's syndrome Nelson's syndrome, Pseudo-Cushing's syndrome - Hyperaldosteronism Conn syndrome, Bartter syndrome CAH Lipoid, 3β, 11β, 17α, 21α Adrenal insufficiency Addison's disease, Waterhouse-Friderichsen syndrome - Hypoaldosteronism Gonads ovarian Polycystic ovary syndrome, Premature ovarian failure testicular 5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency general Hypogonadism, Delayed puberty, Precocious puberty Other Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature Laron syndrome, Psychogenic dwarfism - Multiple endocrine neoplasia 1, 2 - Progeria - Woodhouse-Sakati syndrome - thymus Abscess of thymus, Thymus hyperplasia see also congenital, neoplasia Retrieved from http://en..org/wiki/Primary_aldosteronism Categories: Endocrinology Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Français Nederlands 日本語 Polski Português СрпÑ?ки / Srpski Svenska This page was last modified on 27 August 2008, at 21
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