Primary Pigmented Nodular Adrenocortical Disease
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Primary Pigmented Nodular Adrenocortical Disease
Primary pigmented nodular adrenocortical disease (PPNAD) was first coined in 1984 by Carney et al. it often occurs in association with Carney complex (CNC). CNC is a rare syndrome that involves the formation of abnormal tumours that cause endocrine hyperactivity. PPNAD arises due to the enlargement of the cortex of the adrenal glands, resulting in Cushing's syndrome that is independent of the pituitary hormone Adrenocorticotropic hormone, ACTH. Signs and symptoms PPNAD is a rare cause of high cortisol levels in the blood and often manifests as ACTH-independent Cushing's syndrome. The effects of PPNAD can often be cyclical so the symptoms of Cushing's syndrome will not always be as severe, which may complicate diagnosis. The classic symptoms of Cushing's syndrome include rapid central weight gain, a puffy red face and a buffalo hump at the back of the neck due to fat deposits. Skin changes in Cushing's syndrome include thinning and bruising easily, developing striae and hyperpig ...
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Carney Complex
Carney complex and its subsets LAMB syndrome and NAME syndrome are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity. It is distinct from Carney's triad. Approximately 7% of all cardiac myxomas are associated with Carney complex. Presentation The spotty skin pigmentation and lentigines occur most commonly on the face, especially on the lips, eyelids, conjunctiva and oral mucosa. Cardiac myxomas may lead to embolic strokes and heart failure and may present with fever, joint pain, shortness of breath, diastolic rumble and tumor plop. Myxomas may also occur outside the heart, usually in the skin and breast. Endocrine tumors may manifest as disorders such as Cushing syndrome. The most common endocrine gland manifestation is an ACTH-independent Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). The LAMB acronym refers to lentigines, atrial myxomas, and blu ...
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Myxoma
A myxoma (New Latin from Greek 'muxa' for mucus) is a myxoid tumor of primitive connective tissue. It is most commonly found in the heart (and is the most common primary tumor of the heart in adults) but can also occur in other locations. Types Table below: 1.SMA, smooth muscle actin. 2.MSA, muscle-specific actin. 3.EMA, epithelial membrane antigen. Symptoms and signs Symptoms associated with cardiac myxomas are typically due to the effect of the mass of the tumor obstructing the normal flow of blood within the chambers of the heart. Because pedunculated myxomas are somewhat mobile, symptoms may only occur when the patient is in a particular position. Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6) by the myxoma. High levels of IL-6 may be associated with a higher risk of embolism of the myxoma. Symptoms of a cardiac myxoma include: * Dyspnea on exertion * Paroxysmal nocturnal dyspnea * Fever * Weight loss (see cachexia) * Lightheadedn ...
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Endocrine Diseases
Endocrine diseases are disorders of the endocrine system. The branch of medicine associated with endocrine disorders is known as endocrinology. Types of disease Broadly speaking, endocrine disorders may be subdivided into three groups: # Endocrine gland hypofunction/hyposecretion (leading to hormone deficiency) # Endocrine gland hyperfunction/hypersecretion (leading to hormone excess) # Tumours (benign or malignant) of endocrine glands Endocrine disorders are often quite complex, involving a mixed picture of hyposecretion and hypersecretion because of the feedback mechanisms involved in the endocrine system. For example, most forms of hyperthyroidism are associated with an excess of thyroid hormone and a low level of thyroid stimulating hormone. List of diseases Glucose homeostasis disorders * Diabetes ** Type 1 Diabetes ** Type 2 Diabetes ** Gestational Diabetes ** Mature Onset Diabetes of the Young * Hypoglycemia ** Idiopathic hypoglycemia ** Insulinoma * Glucagonoma Thyro ...
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Adrenal Gland
The adrenal glands (also known as suprarenal glands) are endocrine glands that produce a variety of hormones including adrenaline and the steroids aldosterone and cortisol. They are found above the kidneys. Each gland has an outer cortex which produces steroid hormones and an inner medulla. The adrenal cortex itself is divided into three main zones: the zona glomerulosa, the zona fasciculata and the zona reticularis. The adrenal cortex produces three main types of steroid hormones: mineralocorticoids, glucocorticoids, and androgens. Mineralocorticoids (such as aldosterone) produced in the zona glomerulosa help in the regulation of blood pressure and electrolyte balance. The glucocorticoids cortisol and cortisone are synthesized in the zona fasciculata; their functions include the regulation of metabolism and immune system suppression. The innermost layer of the cortex, the zona reticularis, produces androgens that are converted to fully functional sex hormones in the gonads ...
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Adrenal Gland Disorders
Adrenal gland disorders (or diseases) are conditions that interfere with the normal functioning of the adrenal glands. Adrenal disorders may cause hyperfunction or hypofunction, and may be congenital or acquired. The adrenal gland produces hormones that affects growth, development and stress, and also helps to regulate kidney function. There are two parts of the adrenal glands, the adrenal cortex and the adrenal medulla. The adrenal cortex produces mineralocorticoids, which regulate salt and water balance within the body, glucocorticoids (including cortisol) which have a wide number of roles within the body, and androgens, hormones with testosterone-like function.Adrenal Glands
Johns Hopkins Medicine Health Library.
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Adrenalectomy
Adrenalectomy (Latin root Ad "near/at" + renal "related to the kidneys" + Greek '' ‑ectomy'' “out-cutting”; sometimes written as ADX for the procedure or resulting state) is the surgical removal of one (unilateral) or both (bilateral) adrenal glands. It is usually done to remove tumors of the adrenal glands that are producing excess hormones or is large in size (more than 2 inches or 4 to 5 centimeters). Adrenalectomy can also be done to remove a cancerous tumor of the adrenal glands, or cancer that has spread from another location, such as the kidney or lung. Adrenalectomy is not performed on those who have severe coagulopathy or whose heart and lungs are too weak to undergo surgery. The procedure can be performed using an open incision (laparotomy) or minimally invasive laparoscopic or robot-assisted techniques. Minimally invasive techniques are increasingly the gold standard of care due to shorter length of stay in the hospital, lower blood loss, and similar complication ...
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Scintigraphy
Scintigraphy (from Latin ''scintilla'', "spark"), also known as a gamma scan, is a diagnostic test in nuclear medicine, where radioisotopes attached to drugs that travel to a specific organ or tissue (radiopharmaceuticals) are taken internally and the emitted gamma radiation is captured by external detectors (gamma cameras) to form two-dimensional images in a similar process to the capture of x-ray images. In contrast, SPECT and ''positron emission tomography'' (PET) form 3-dimensional images and are therefore classified as separate techniques from scintigraphy, although they also use gamma cameras to detect internal radiation. Scintigraphy is unlike a diagnostic X-ray where external radiation is passed through the body to form an image. Process Scintillography is an imaging method of nuclear events provoked by collisions or charged current interactions among nuclear particles or ionizing radiation and atoms which result in a brief, localised pulse of electromagnetic radiation, ...
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CT Scan
A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or radiology technologists. CT scanners use a rotating X-ray tube and a row of detectors placed in a gantry (medical), gantry to measure X-ray Attenuation#Radiography, attenuations by different tissues inside the body. The multiple X-ray measurements taken from different angles are then processed on a computer using tomographic reconstruction algorithms to produce Tomography, tomographic (cross-sectional) images (virtual "slices") of a body. CT scans can be used in patients with metallic implants or pacemakers, for whom magnetic resonance imaging (MRI) is Contraindication, contraindicated. Since its development in the 1970s, CT scanning has proven to be a versatile imaging technique. While CT is most prominently used in medical diagnosis, ...
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Dexamethasone Suppression Test
The dexamethasone suppression test (DST) is used to assess adrenal gland function by measuring how cortisol levels change in response to oral doses or an injection of dexamethasone. It is typically used to diagnose Cushing's syndrome. The DST was historically used for diagnosing depression, but by 1988 it was considered to be "at best, severely limited in its clinical ability" for this purpose. Physiology Dexamethasone is an exogenous steroid that provides negative feedback to the pituitary gland to suppress the secretion of adrenocorticotropic hormone (ACTH). Specifically, dexamethasone binds to glucocorticoid receptors in the anterior pituitary gland, which lie outside the blood–brain barrier, resulting in regulatory modulation. Test Procedures There are several types of DST procedures: # Overnight DST - An oral dose of dexamethasone is given between 11pm and midnight, and the cortisol level is measured at 8 - 9am the next morning # Two-day DST - This involves giving an ...
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Expert Review Of Cardiovascular Therapy
''Expert Review of Cardiovascular Therapy'' is a monthly peer-reviewed medical journal covering all aspects of cardiology. It was established in 2003 and is published by Informa. The journal is abstracted and indexed in Index Medicus/MEDLINE/PubMed, Embase/Excerpta Medica, EMCare, Chemical Abstracts, Scopus Scopus is Elsevier's abstract and citation database launched in 2004. Scopus covers nearly 36,377 titles (22,794 active titles and 13,583 inactive titles) from approximately 11,678 publishers, of which 34,346 are peer-reviewed journals in top-l ..., and CINAHL. External links * Cardiology journals English-language journals Expert Review journals Monthly journals Academic journals established in 2003 {{Cardiology-journal-stub ...
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Carney Complex
Carney complex and its subsets LAMB syndrome and NAME syndrome are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity. It is distinct from Carney's triad. Approximately 7% of all cardiac myxomas are associated with Carney complex. Presentation The spotty skin pigmentation and lentigines occur most commonly on the face, especially on the lips, eyelids, conjunctiva and oral mucosa. Cardiac myxomas may lead to embolic strokes and heart failure and may present with fever, joint pain, shortness of breath, diastolic rumble and tumor plop. Myxomas may also occur outside the heart, usually in the skin and breast. Endocrine tumors may manifest as disorders such as Cushing syndrome. The most common endocrine gland manifestation is an ACTH-independent Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). The LAMB acronym refers to lentigines, atrial myxomas, and blu ...
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Cushing's Syndrome
Cushing's syndrome is a collection of signs and symptoms due to prolonged exposure to glucocorticoids such as cortisol. Signs and symptoms may include high blood pressure, abdominal obesity but with thin arms and legs, reddish stretch marks, a round red face, a fat lump between the shoulders, weak muscles, weak bones, acne, and fragile skin that heals poorly. Women may have more hair and irregular menstruation. Occasionally there may be changes in mood, headaches, and a chronic feeling of tiredness. Cushing's syndrome is caused by either excessive cortisol-like medication, such as prednisone, or a tumor that either produces or results in the production of excessive cortisol by the adrenal glands. Cases due to a pituitary adenoma are known as Cushing's disease, which is the second most common cause of Cushing's syndrome after medication. A number of other tumors, often referred to as ectopic due to their placement outside the pituitary, may also cause Cushing's. Some of ...
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