Mon . 20 Jul 2020
TR | RU | UK | KK | BE |

Addison's disease

addison's disease in dogs, addison's disease symptoms
Addison’s disease, also known as primary adrenal insufficiency and hypocortisolism, is a long-term endocrine disorder in which the adrenal glands do not produce enough steroid hormones1 Symptoms generally come on slowly and may include abdominal pain, weakness, and weight loss Darkening of the skin in certain areas may also occur Under certain circumstances, an adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness An adrenal crisis can be triggered by stress, such as from an injury, surgery, or infection1

Addison's disease arises from problems with the adrenal gland such that not enough of the steroid hormone cortisol and possibly aldosterone are produced,1 most often due to damage by the body's own immune system in the developed world and tuberculosis in the developing world4 Other causes include certain medications, sepsis, and bleeding into both adrenal glands14 Secondary adrenal insufficiency is caused by not enough adrenocorticotropic hormone ACTH produced by the pituitary gland or CRH produced by the hypothalamus Despite this distinction, adrenal crises can happen in all forms of adrenal insufficiency Addison's disease is generally diagnosed by blood tests, urine tests, and medical imaging1

Treatment involves replacing the absent hormones1 This involves taking a corticosteroid such as hydrocortisone and fludrocortisone12 These medications are usually taken by mouth1 Lifelong, continuous steroid replacement therapy is required, with regular follow-up treatment and monitoring for other health problems5 A high-salt diet may also be useful in some people If symptoms worsen, an injection of corticosteroid is recommended and people should carry a dose with them Often, large amounts of intravenous fluids with the sugar dextrose are also required Without treatment, an adrenal crisis can result in death1

Addison’s disease affects about 09 to 14 per 10,000 people in the developed world13 It occurs most frequently in middle-aged females1 Secondary adrenal insufficiency is more common3 Long-term outcomes with treatment are typically good6 It is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 18557 The adjective "addisonian" is used to describe features of the condition, as well as people with Addison’s disease8

Contents

  • 1 Signs and symptoms
    • 11 Addisonian crisis
  • 2 Causes
    • 21 Adrenal destruction
    • 22 Adrenal dysgenesis
    • 23 Impaired steroidogenesis
  • 3 Diagnosis
    • 31 Suggestive features
    • 32 Testing
  • 4 Treatment
    • 41 Maintenance
    • 42 Crisis
  • 5 Epidemiology
  • 6 Prognosis
  • 7 History
    • 71 Discovery
  • 8 Other animals
  • 9 References
  • 10 External links

Signs and symptomsedit

The symptoms of Addison's disease develop gradually and may become established before they are recognized They can be nonspecific and are potentially attributable to other medical conditions

The signs and symptoms include fatigue; lightheadedness upon standing or difficulty standing, muscle weakness, fever, weight loss, anxiety, nausea, vomiting, diarrhea, headache, sweating, changes in mood or personality, and joint and muscle pains Some patients have cravings for salt or salty foods due to the loss of sodium through their urine8 Hyperpigmentation of the skin may be seen, particularly when the patient lives in a sunny area, as well as darkening of the palmar crease, sites of friction, recent scars, the vermilion border of the lips, and genital skin9 These skin changes are not encountered in secondary and tertiary hypoadrenalism10

On physical examination, these clinical signs may be noticed:8

  • Low blood pressure with or without orthostatic hypotension blood pressure that decreases with standing
  • Darkening hyperpigmentation of the skin, including areas not exposed to the sun Characteristic sites of darkening are skin creases eg, of the hands, nipple, and the inside of the cheek buccal mucosa; also, old scars may darken This occurs because melanocyte-stimulating hormone MSH and ACTH share the same precursor molecule, pro-opiomelanocortin POMC After production in the anterior pituitary gland, POMC gets cleaved into gamma-MSH, ACTH, and beta-lipotropin The subunit ACTH undergoes further cleavage to produce alpha-MSH, the most important MSH for skin pigmentation In secondary and tertiary forms of adrenal insufficiency, skin darkening does not occur, as ACTH is not overproduced

Addison's disease is associated with the development of other autoimmune diseases, such as type I diabetes, thyroid disease Hashimoto's thyroiditis, celiac disease, or vitiligo1112 Addison’s disease may be the only manifestation of undiagnosed celiac disease11 Both diseases share the same genetic risk factors HLA-DQ2 and HLA-DQ8 haplotypes13

The presence of Addison's in addition to mucocutaneous candidiasis, hypoparathyroidism, or both, is called autoimmune polyendocrine syndrome type 114 The presence of Addison's in addition to autoimmune thyroid disease, type 1 diabetes, or both, is called autoimmune polyendocrine syndrome type 215

Addisonian crisisedit

Main article: Adrenal crisis

An "Addisonian crisis" or "adrenal crisis" is a constellation of symptoms that indicates severe adrenal insufficiency This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function such as adrenal hemorrhage, or an intercurrent problem eg, infection, trauma in someone known to have Addison's disease It is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment

Characteristic symptoms are:16

  • Sudden penetrating pain in the legs, lower back, or abdomen
  • Severe vomiting and diarrhea, resulting in dehydration
  • Low blood pressure
  • Syncope loss of consciousness and ability to stand
  • Hypoglycemia reduced level of blood glucose
  • Confusion, psychosis, slurred speech
  • Severe lethargy
  • Hyponatremia low sodium level in the blood
  • Hyperkalemia elevated potassium level in the blood
  • Hypercalcemia elevated calcium level in the blood
  • Convulsions
  • Fever

Causesedit

The negative feedback loop for glucocorticoids

Causes of adrenal insufficiency can be categorized by the mechanism through which they cause the adrenal glands to produce insufficient cortisol These are adrenal dysgenesis the gland has not formed adequately during development, impaired steroidogenesis the gland is present but is biochemically unable to produce cortisol or adrenal destruction disease processes leading to glandular damage8

Adrenal destructionedit

Autoimmune adrenalitis is the most common cause of Addison's disease in the industrialized world Autoimmune destruction of the adrenal cortex is caused by an immune reaction against the enzyme 21-hydroxylase a phenomenon first described in 199217 This may be isolated or in the context of autoimmune polyendocrine syndrome APS type 1 or 2, in which other hormone-producing organs, such as the thyroid and pancreas, may also be affected18

Adrenal destruction is also a feature of adrenoleukodystrophy, and when the adrenal glands are involved in metastasis seeding of cancer cells from elsewhere in the body, especially lung, hemorrhage eg, in Waterhouse-Friderichsen syndrome or antiphospholipid syndrome, particular infections tuberculosis, histoplasmosis, coccidioidomycosis, or the deposition of abnormal protein in amyloidosis19

Adrenal dysgenesisedit

All causes in this category are genetic, and generally very rare These include mutations to the SF1 transcription factor, congenital adrenal hypoplasia due to DAX-1 gene mutations and mutations to the ACTH receptor gene or related genes, such as in the Triple A or Allgrove syndrome DAX-1 mutations may cluster in a syndrome with glycerol kinase deficiency with a number of other symptoms when DAX-1 is deleted together with a number of other genes8

Impaired steroidogenesisedit

To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia

Of the synthesis problems, congenital adrenal hyperplasia is the most common in various forms: 21-hydroxylase, 17α-hydroxylase, 11β-hydroxylase and 3β-hydroxysteroid dehydrogenase, lipoid CAH due to deficiency of StAR and mitochondrial DNA mutations8 Some medications interfere with steroid synthesis enzymes eg, ketoconazole, while others accelerate the normal breakdown of hormones by the liver eg, rifampicin, phenytoin8

Diagnosisedit

Suggestive featuresedit

Routine laboratory investigations may show:8

  • Hypercalcemia
  • Hypoglycemia, low blood sugar worse in children due to loss of glucocorticoid's glucogenic effects
  • Hyponatremia low blood sodium levels, due to loss of production of the hormone aldosterone, to the kidney's inability to excrete free water in the absence of sufficient cortisol, and also the effect of corticotropin-releasing hormone to stimulate secretion of ADH
  • Hyperkalemia raised blood potassium levels, due to loss of production of the hormone aldosterone
  • Eosinophilia and lymphocytosis increased number of eosinophils or lymphocytes, two types of white blood cells
  • Metabolic acidosis increased blood acidity, also is due to loss of the hormone aldosterone because sodium reabsorption in the distal tubule is linked with acid/hydrogen ion H+ secretion Absent or insufficient levels of aldosterone stimulation of the renal distal tubule leads to sodium wasting in the urine and H+ retention in the serum

Testingedit

Cortisol Aldosterone

In suspected cases of Addison's disease, demonstration of low adrenal hormone levels even after appropriate stimulation called the ACTH stimulation test or synacthen test with synthetic pituitary ACTH hormone tetracosactide is needed for the diagnosis Two tests are performed, the short and the long test It should be noted that dexamethasone does not cross-react with the assay and can be administered concomitantly during testing

The short test compares blood cortisol levels before and after 250 micrograms of tetracosactide intramuscular or intravenous is given If, one hour later, plasma cortisol exceeds 170 nmol/l and has risen by at least 330 nmol/l to at least 690 nmol/l, adrenal failure is excluded If the short test is abnormal, the long test is used to differentiate between primary adrenal insufficiency and secondary adrenocortical insufficiency

The long test uses 1 mg tetracosactide intramuscular Blood is taken 1, 4, 8, and 24 hr later Normal plasma cortisol level should reach 1000 nmol/l by 4 hr In primary Addison's disease, the cortisol level is reduced at all stages, whereas in secondary corticoadrenal insufficiency, a delayed but normal response is seen

Other tests may be performed to distinguish between various causes of hypoadrenalism, including renin and adrenocorticotropic hormone levels, as well as medical imaging - usually in the form of ultrasound, computed tomography or magnetic resonance imaging

Adrenoleukodystrophy, and the milder form, adrenomyeloneuropathy, cause adrenal insufficiency combined with neurological symptoms These diseases are estimated to be the cause of adrenal insufficiency in about 35% of male patients with idiopathic Addison’s disease, and should be considered in the differential diagnosis of any male with adrenal insufficiency Diagnosis is made by a blood test to detect very long chain fatty acids20

Treatmentedit

Corticosteroids to replace cortisols not secreted by the adrenal glands

Maintenanceedit

Treatment for Addison's disease involves replacing the missing cortisol, sometimes in the form of hydrocortisone tablets, or prednisone tablets in a dosing regimen that mimics the physiological concentrations of cortisol Alternatively, one-quarter as much prednisolone may be used for equal glucocorticoid effect as hydrocortisone Treatment is usually lifelong In addition, many patients require fludrocortisone as replacement for the missing aldosterone

People with Addison's are often advised to carry information on them eg, in the form of a MedicAlert bracelet or information card for the attention of emergency medical services personnel who might need to attend to their needs2122 It is also recommended that a needle, syringe, and injectable form of cortisol be carried for emergencies22 People with Addison's disease are advised to increase their medication during periods of illness or when undergoing surgery or dental treatment22 Immediate medical attention is needed when severe infections, vomiting, or diarrhea occur, as these conditions can precipitate an Addisonian crisis A patient who is vomiting may require injections of hydrocortisone instead23

Crisisedit

Standard therapy involves intravenous injections of glucocorticoids and large volumes of intravenous saline solution with dextrose glucose This treatment usually brings rapid improvement If intravenous access is not immediately available, intramuscular injection of glucocorticoids can be used When the patient can take fluids and medications by mouth, the amount of glucocorticoids is decreased until a maintenance dose is reached If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate24

Epidemiologyedit

The frequency rate of Addison's disease in the human population is sometimes estimated at roughly one in 100,00025 Some put the number closer to 40–144 cases per million population 1/25,000–1/7,000126 Addison's can affect persons of any age, sex, or ethnicity, but it typically presents in adults between 30 and 50 years of age27 Research has shown no significant predispositions based on ethnicity26

Prognosisedit

Outcomes are typically good when treated Most can expect to live relatively normal lives Someone with the disease should be observant of symptoms of an "Addison's crisis" while the body is strained, as in rigorous exercise or being sick, the latter often needing emergency treatment with intravenous injections to treat the crisis28

Individuals with Addison's disease have more than a doubled mortality rate29 Furthermore, individuals with Addison's disease and diabetes mellitus have an almost 4 time increase in mortality compared to individuals with only diabetes30

Historyedit

Discoveryedit

Addison’s disease is named after Thomas Addison, the British physician who first described the condition in On the Constitutional and Local Effects of Disease of the Suprarenal Capsules 185531 All of Addison's six original patients had tuberculosis of the adrenal glands32 While Addison's six patients in 1855 all had adrenal tuberculosis, the term "Addison's disease" does not imply an underlying disease process

The condition was initially considered a form of anemia associated with the adrenal glands Because little was known at the time about the adrenal glands then called "Supra-Renal Capsules", Addison’s monograph describing the condition was an isolated insight As the adrenal function became better known, Addison’s monograph became known as an important medical contribution and a classic example of careful medical observation33

Other animalsedit

Main article: Hypoadrenocorticism in dogs

The condition has been diagnosed in all breeds of dogs In general, it is underdiagnosed, and one must clinically suspect it as an underlying disorder for many presenting complaints Females are overrepresented, and the disease often appears in middle age 4–7 yr, although any age or either gender may be affectedcitation needed

Hypoadrenocorticism is treated with fludrocortisone or with monthly injections of desoxycorticosterone pivlate DOCP and daily prednisone Routine blood work is necessary in the initial stages until a maintenance dose is established Most of the medications used in the therapy of hypoadrenocorticism can cause excessive thirst and urination if not prescribed at the lowest effective dose, making it important to provide enough drinking water If the owner knows about an upcoming stressful situation shows, traveling, etc, patients generally need an increased dose of prednisone to help deal with the added stress

Referencesedit

  1. ^ a b c d e f g h i j k l m n o p q r s t "Adrenal Insufficiency and Addison's Disease" NIDDK May 2014 Retrieved 13 March 2016 
  2. ^ a b Napier, C; Pearce, SH June 2014 "Current and emerging therapies for Addison's disease" Current Opinion in Endocrinology, Diabetes and Obesity 21 3: 147–53 PMID 24755997 doi:101097/med0000000000000067 
  3. ^ a b c Brandão Neto, RA; de Carvalho, JF 2014 "Diagnosis and classification of Addison's disease autoimmune adrenalitis" Autoimmunity reviews 13 4-5: 408–11 PMID 24424183 doi:101016/jautrev201401025 
  4. ^ a b Adam, Andy 2014 Grainger & Allison's Diagnostic Radiology 6 ed Elsevier Health Sciences p 1031 ISBN 9780702061288 
  5. ^ Napier, C; Pearce, SH December 2012 "Autoimmune Addison's disease" Presse medicale Paris, France : 1983 41 12 P 2: e626–35 PMID 23177474 doi:101016/jlpm201209010 
  6. ^ Rajagopalan, Murray Longmore, Ian B Wilkinson, Supraj R 2006 Mini Oxford handbook of clinical medicine 6 ed Oxford: Oxford University Press p 312 ISBN 9780198570714 
  7. ^ Rose, Noel R; Mackay, Ian R 2014 The autoimmune diseases 5 ed San Diego, CA: Elsevier Science p 605 ISBN 9780123849304 
  8. ^ a b c d e f g h Ten S, New M, Maclaren N 2001 "Clinical review 130: Addison's disease 2001" The Journal of Clinical Endocrinology and Metabolism 86 7: 2909–2922 PMID 11443143 doi:101210/jc8672909 
  9. ^ Nieman LK, Chanco Turner ML 2006 "Addison's disease" Clinics in Dermatology 24 4: 276–280 PMID 16828409 doi:101016/jclindermatol200604006 
  10. ^ de Herder WW, van der Lely AJ May 2003 "Addisonian crisis and relative adrenal failure" Reviews in Endocrine and Metabolic Disorders 4 2: 143–7 PMID 12766542 doi:101023/A:1022938019091 
  11. ^ a b Freeman HJ 2016 "Endocrine manifestations in celiac disease" World J Gastroenterol Review 22 38: 8472–8479 PMID 27784959 
  12. ^ Zhernakova A, Withoff S, Wijmenga C 2013 "Clinical implications of shared genetics and pathogenesis in autoimmune diseases" Nat Rev Endocrinol Review 9 11: 646–59 PMID 23959365 doi:101038/nrendo2013161 CS1 maint: Multiple names: authors list link
  13. ^ Denham JM, Hill ID 2013 "Celiac disease and autoimmunity: review and controversies" Curr Allergy Asthma Rep Review 13 4: 347–53 PMC 3725235  PMID 23681421 doi:101007/s11882-013-0352-1 
  14. ^ "Autoimmune polyglandular syndrome type 1 | Genetic and Rare Diseases Information Center GARD – an NCATS Program" rarediseasesinfonihgov Retrieved 26 June 2017 
  15. ^ "Autoimmune polyglandular syndrome type 2 | Genetic and Rare Diseases Information Center GARD – an NCATS Program" rarediseasesinfonihgov Retrieved 26 June 2017 
  16. ^ "Addison's Disease" National Endocrine and Metabolic Diseases Information Service Retrieved 26 October 2007 
  17. ^ Winqvist O, Karlsson FA, Kämpe O June 1992 "21-Hydroxylase, a major autoantigen in idiopathic Addison's disease" The Lancet 339 8809: 1559–62 PMID 1351548 doi:101016/0140-67369291829-W 
  18. ^ Husebye ES, Perheentupa J, Rautemaa R, Kämpe O May 2009 "Clinical manifestations and management of patients with autoimmune polyendocrine syndrome type I" Journal of Internal Medicine 265 5: 514–29 PMID 19382991 doi:101111/j1365-2796200902090x 
  19. ^ Kennedy, Ron "Addison's Disease" The Doctors' Medical Library 
  20. ^ Laureti S, Casucci G, Santeusanio F, Angeletti G, Aubourg P, Brunetti P 1996 "X-linked adrenoleukodystrophy is a frequent cause of idiopathic Addison's disease in young adult male patient" The Journal of Clinical Endocrinology and Metabolism 81 2: 470–474 PMID 8636252 doi:101210/jc812470 
  21. ^ Quinkler M, Dahlqvist P, Husebye ES, Kämpe O Jan 2015 "A European Emergency Card for adrenal insufficiency can save lives" Eur J Intern Med 26 1: 75–6 PMID 25498511 doi:101016/jejim201411006 
  22. ^ a b c Michels A, Michels N 1 Apr 2014 "Addison disease: early detection and treatment principles" Am Fam Physician 89 7: 563–8 PMID 24695602 
  23. ^ White, Katherine 28 July 2004 "What to do in an emergency -Addisonian crisis" Addison's Disease Self Help Group 
  24. ^ "Adrenal Insufficiency and Addison's Disease" National Endocrine and Metabolic Diseases Information Service Retrieved 26 November 2010 
  25. ^ "Addison Disease" MedicineNet Archived from the original on 24 June 2007 Retrieved 2007-07-25 
  26. ^ a b Odeke, Sylvester "Addison Disease" eMedicine Archived from the original on 7 July 2007 Retrieved 2007-07-25 
  27. ^ Volpé, Robert 1990 Autoimmune Diseases of the Endocrine System CRC Press p 299 ISBN 0-8493-6849-9 
  28. ^ "Addison's disease - Treatment" NHS Choices Retrieved 2016-10-08 
  29. ^ Bergthorsdottir, Ragnhildur; Leonsson-Zachrisson, Maria; Odén, Anders; Johannsson, Gudmundur 2006-12-01 "Premature Mortality in Patients with Addison’s Disease: A Population-Based Study" The Journal of Clinical Endocrinology & Metabolism 91 12: 4849–4853 ISSN 0021-972X doi:101210/jc2006-0076 
  30. ^ Dimitrios Chantzichristos; Anders Persson; Björn Eliasson; Mervete Miftaraj; Stefan Franzén; Ragnhildur Bergthorsdottir; Soffia Gudbjörnsdottir; Ann-Marie Svensson; Gudmundur Johannsson 2016-04-01 Cushing Syndrome and Primary Adrenal Disorders Meeting Abstracts Endocrine Society pp OR25–4–OR25–4 doi:101210/endo-meetings2016ahpaa9or25-4 
  31. ^ Addison, Thomas 1855 On The Constitutional And Local Effects Of Disease Of The Supra-Renal Capsules London: Samuel Highley 
  32. ^ Patnaik MM, Deshpande AK May 2008 "Diagnosis–Addison’s Disease Secondary to Tuberculosis of the Adrenal Glands" Clinical Medicine & Research 6 1: 29 PMC 2442022  PMID 18591375 doi:103121/cmr2007754a 
  33. ^ Bishop PM 1950 "The history of the discovery of Addison's disease" Proceedings of the Royal Society of Medicine 43 1: 35–42 PMC 2081266  PMID 15409948 

External linksedit

Classification
  • ICD-10: E271-E272
  • ICD-9-CM: 2554
  • DiseasesDB: 222
External resources
  • MedlinePlus: 000378
  • eMedicine: med/42
  • Patient UK: Addison's disease



  • Addison's disease at DMOZ

addison's disease diet, addison's disease in children, addison's disease in dogs, addison's disease in humans, addison's disease jfk, addison's disease pathophysiology, addison's disease pictures, addison's disease symptoms, addison's disease symptoms in dogs, addison's disease treatment


Addison's disease Information about

Addison's disease


  • user icon

    Addison's disease beatiful post thanks!

    29.10.2014


Addison's disease
Addison's disease
Addison's disease viewing the topic.
Addison's disease what, Addison's disease who, Addison's disease explanation

There are excerpts from wikipedia on this article and video

Random Posts

Picts

Picts

The Picts were a tribal confederation of peoples who lived in what is today eastern and northern Sco...
Visual prosthesis

Visual prosthesis

A visual prosthesis, often referred to as a bionic eye, is an experimental visual device intended to...
Mini rugby

Mini rugby

Mini rugby, also known as New Image Rugby, is a form of rugby union designed to introduce the sport ...
List of synthetic polymers

List of synthetic polymers

Synthetic polymers are human-made polymers From the utility point of view they can be classified int...