Addison hastalığı (kronik adrenal yetmezlik) bir ve altı her 100.000 kişi arasında etkileyen nadir ve ilerleyici bir hastalıktır. Her iki cinste ve her yaştan insan etkiler.
İnsan vücudunda iki böbreküstü bezleri, her bir böbreğin üst üste bulunmaktadır. sinir sistemi ve bağışıklık sistemi ile çalışan endokrin sisteminin Bu bezler bölümünü oluşturan, vücudun farklı etkinlikler ile baş gerilmeler ve yardım etmek. Addison hastalığı düzenleyen hormonlar yeterli miktarda yapmak böbreküstü bezlerinin yetersizliği nedeniyle oluşur.
Adrenalin en iyi (bezinin orta kesiminde) adrenal medulla olarak, böbreküstü bezleri tarafından salgılanan hormonların bilinmektedir. Adrenal korteks (dış kısmı) da kortikosteroidler önemli hormonları üretir. Onlar, aldosteron kortizol ve ek cinsiyet hormonları bulunur. Addison hastalığı olan bir kişinin, sadece adrenal korteks etkilenir. Kişi bazen de yeterli mineralokortikoid veya aldosteron üretmek için başarısız yeterli glukokortikoid veya kortizol ve üretemez.
NEDENLERİ
Addison hastalığı vakalarının çoğu otoimmün yanıt neden olduğu saldırı ve zamanla zarar böbreküstü bezleri. Diğer nedenler şunlardır:
1.Enfeksiyon
2.Kanser
3.Adrenal veya hipozif bezi
SEMPTOMLAR
Addison hastalığı belirtileri aşağıdakilerden herhangi birini veya tümünü içerebilir:
1.iştah ve kilo kaybı.
2.Bulantı, kusma veya ishal.
3.Kas güçsüzlüğü.
4.Kronik yorgunluk kötüleşmesi.
5.Düşük kan basıncı.
6.Tuz istek.
7.Dehidratasyon.
8.Hipoglisemi veya düşük kan şekeri (özellikle çocuklarda).
9.izleri ve kemikli bölgelerde özellikle çevresinde, deri pigmentasyon artışı.
10.Düzensiz veya kadınlarda hiç adet dönemleri.
11.Ruh hali, zihin bulanıklığı ya da bilinç kaybı.
Bu belirtilerin 20 yıl veya daha fazla yavaş veya (çocuklar ve gençler başta olmak üzere) hızla ilerleme gelişebilir. Birçok belirtiler diğer hastalıkları taklit edebilir, bu nedenle tanı gecikebilir.
Kortizol hormonu
Kortizol, adrenal bezin dış tabakası tarafından üretilen adrenal korteks denir. böbreküstü bezleri tarafından yayımlanan kortizol miktarı yakından endokrin sistem, beyinde bulunan hipofiz, ustası bezi tarafından izlenmektedir. hipofizin çalışmasını başka bir beyin yapısı, hipotalamus tarafından yönetilir. kortizol düzeyi çok düşük olduğunda, hipofiz (ACTH) uyarıcı hormon adrenokortikotropin salgılar. Tersine, kortizol düzeyi yüksek kortizol üretimini yavaşlatır ACTH salınımı azaltmak için hipofiz istemi.
Kortizol birçok hayati rol oynar, çünkü bu birçok vücut fonksiyonları için gereklidir:
1.adrenalin ile çalışır beden fiziksel ve duygusal stres yönetmenize yardımcı olmak için.
2.glikoz dönüştürür protein işaretleme kan şekeri düzeylerini artırmak için.
3.hormon insülin ile tandem İşleri sürekli kan şekeri düzeylerini korumak için.
4.İltihaplanmayı azaltır.
5.vücut sürekli kan basıncı korur.
6.bağışıklık sisteminin işleyişini yardımcı olur.
Hormon aldosteron
Aldosteron da adrenal korteksi tarafından üretilen bir mineralokortikoid vardır. Vücutta aldosteron miktarı salgıladıkları hormonlar artırmak veya aldosteron üretimini azaltmak böbrekler tarafından izlenir. Aldosteron kandaki elektrolit (sodyum ve potasyum gibi) düzenlemektedir. Bu kan basıncı ve kalp fonksiyonlarının korunmasına yardımcı olur. Çok fazla sodyum böbrekler tarafından atılır ise, vücut sıvısının önemli bir miktarı da kaybolur. Bu kan hacmi azalır ve kan basıncı düşer. Çok fazla veya çok az potasyum şekilde kalp fonksiyonlarını etkileyebilir.
Primer adrenal yetmezlik
Addison hastalığı ve yavaş yavaş oluşabilir (lar) hasar olduğunda yaklaşık 90 böbreküstü bezi yüzde tanımlanır. Bu birincil adrenal yetmezlik olarak bilinir. Addison hastalığı 10 olgunun yedisi Around vücudun kendi bağışıklık hücreleri saldırı ve böbreküstü bezleri yok otoimmün yanıt kaynaklanır. bir durum polyendocrine eksikliği sendromu denilen bazı durumlarda, endokrin sistemin diğer bezler, otoimmün yanıt etkilenir.
Polyendocrine eksikliği sendromu
Hastalığın iki tipi vardır ve her iki tip ailelerde çalıştırmak eğilimindedir:
Tip I - Daka sık çocuklarda görülür. Semptomlar geliştirme dahil underactive paratiroid, pernisiyöz anemi, tekrarlayan kandida enfeksiyonları, kronik aktif hepatit ve yavaş cinsel.
Tip II - (Schmidt sendromu) erişkinler ve gençlerde daha yaygındır. Semptomlar geliştirme dahil tiroid, diyabet ve yavaş cinsel.
Birincil Addison hastalığı ile ilgili diğer şartlar şunlardır:
1.Adrenomiyelonöropati (AMN) - Bazı erişkinlerde ortaya çıkar. Omurga etkiler ve zaman içinde dejeneratif olur.
2.Adrenolökodistrofi (ALD) - (çocuk)nadiren görülür(100.000 de biri), özellikle erkek. Beyine zarar verir ve ölümcül olabilir. Kurtulanların çoğu AMN dir.
Birincil Addison hastalığı tedavisi ömür boyu glukokortikoid ve mineralokortikoid replasmanı.
Sekonder adrenal yetmezlik
Bazen, Addison hastalığı böbrek üstü bezleri kortizol salgılar istenmez demektir ACTH, yeterli miktarda üretmek için hipofiz bezi yetersizliği neden olur. Bu ikincil adrenal yetersizliği olarak bilinir. Nedenleri şunlar olabilir:
Bazı ilaçlar - artrit ve astım gibi romatoid inflamatuar bozukluklar gibi) bulunan yedek glukokortikoid genellikle tedavi ile uzun süreli veya yüksek doz steroid (. ya da varsa, doz durdu aniden önlemler sivrilen uygun değil azaltılmış tarafından, hipofiz bezi ACTH yeterli üretmek için başarısız olabilir cevap. Bu durum tersine olabilir bazen.
Cushing hastalığı - üretir ACTH bezinin hipofiz tümör iyi huylu. çok kortizol de bu sonuç üretiliyor. Tedavi) ve tümörün bazı kaldırma gerektiren cerrahi durumlarda s (kaldırma hasarlı böbreküstü bezi.
Diğer nedenler - enfeksiyonları, azalmış kan akımı, radyoterapi ve üretmek ACTH veya bez belli nöroşirürji gelebilecek hasarlar hipofiz hipotalamus yeteneği azaltmak.
İkinci Addison hastalığı tedavisi sadece glukokortikoid replasmanı.
Addison Krizi
Ani, akut belirtilerin kötüleşmesine bir Addison krizi olarak bilinir. O neden olabilir:
1.Aşırı stres - Bir kaza, aşırı sıcak ya da fiziksel zorlama.
2.Şiddetli hastalık -ishal veya kusma özellikle susuz.
3.Ani şok - örneğin, önemli bir kişinin ölümü.
Addison krizi belirtileri şunlardır:
karın, sırt ve bacaklarda şiddetli ağrı.
Bulantı, kusma veya ishal.
Düşük tansiyon, düşük kan şekeri, yüksek potasyum, düşük sodyum ve hızlı bir nabız.
Mümkün şuur bozukluğu ve bilinç kaybı.
Isteminde acil hastane tedavisi ilaç ve serum fizyolojik steroid artmış, intravenöz sıvılar dahil aranmalıdır. Birçok Addisonians bir tıbbi uyarı bilezik aşınma ya da bilgi ve kimlik ile kolye ve hidrokortizon acil durumlarda kullanmak için enjektabl taşırlar. Hala hastanede ve sürekli izlenmesi gerekir.
NOT : "Eğer tedavi edilmezse, bir Addison krizi ölümcül olabilir."
YAZININ İNGİLİZCESİ İÇİN TIKLAYIN
22 Nisan 2011 Cuma
Addison's Disease
Addison's disease (chronic adrenal insufficiency) is a rare and progressive disorder that affects between one and six in every 100,000 people. It affects people of both sexes and all ages.
The human body has two adrenal glands, one on top of each kidney. These glands form part of the endocrine system, which works with the nervous system and the immune system to help the body cope with different events and stresses. Addison's disease is caused by the inability of the adrenal glands to make sufficient amounts of regulating hormones.
Adrenaline is the most well known of the hormones that are secreted by the adrenal glands, in the adrenal medulla (the central part of the gland). The adrenal cortex (the outer part) also produces important hormones, the corticosteroids. They include cortisol, aldosterone and supplementary sex hormones. In a person with Addison's disease, only the adrenal cortex is affected. The person cannot produce enough glucocorticoid or cortisol and, occasionally, also fails to produce sufficient mineralocorticoid or aldosterone.
Causes
Most cases of Addison's disease are caused by an autoimmune response that attacks and damages the adrenal glands over time. Other causes include:
* Infection
* Cancer
* Surgical removal of particular tumours in the adrenal or pituitary glands or the hypothalamus.
Symptoms
The symptoms of Addison's disease can include any or all of the following:
* Loss of appetite and weight.
* Nausea, vomiting or diarrhoea.
* Muscle weakness.
* Chronic, worsening fatigue.
* Low blood pressure.
* Salt cravings.
* Dehydration.
* Hypoglycaemia, or low blood sugar levels (especially in children).
* Increased pigmentation of the skin, particularly around scars and bony areas.
* Irregular or no menstrual periods in women.
* Mood swings, mental confusion or loss of consciousness.
These symptoms can develop quickly (especially in children and teenagers), or progress slowly for 20 years or more. Many symptoms can mimic other diseases, so diagnosis can be delayed.
The hormone cortisol
Cortisol is produced by the outer layer of the adrenal gland, called the adrenal cortex. The quantities of cortisol released by the adrenal glands are closely monitored by the master gland of the endocrine system, the pituitary, which is located in the brain. The workings of the pituitary are governed by another brain structure, the hypothalamus. When cortisol levels are too low, the pituitary secretes the stimulating hormone adrenocorticotropin (ACTH). Conversely, high levels of cortisol prompt the pituitary to decrease ACTH secretion, which slows cortisol production.
Cortisol plays many vital roles and is essential to many body functions because it:
* Works with adrenaline to help the body manage physical and emotional stress.
* Converts protein into glucose to boost flagging blood sugar levels.
* Works in tandem with the hormone insulin to maintain constant blood sugar levels.
* Reduces inflammation.
* Helps the body maintain a constant blood pressure.
* Helps the workings of the immune system.
The hormone aldosterone
Aldosterone is a mineralocorticoid, also produced by the adrenal cortex. The amount of aldosterone in the body is monitored by the kidneys, which secrete hormones to increase or decrease aldosterone production. Aldosterone regulates electrolytes (such as sodium and potassium) in the blood. This helps to maintain blood pressure and heart function. If too much sodium is excreted by the kidneys, a considerable amount of body fluid is also lost. This reduces blood volume and drops blood pressure. Too much or too little potassium can affect the way the heart functions.
Primary adrenal insufficiency
Addison's disease can occur gradually, and is defined when approximately 90 per cent of the adrenal gland(s) is damaged. This is known as primary adrenal insufficiency. Around seven out of 10 cases of Addison's disease are caused by an autoimmune response, where the body's own immune cells attack and destroy the adrenal glands. In some cases, other glands of the endocrine system are affected by an autoimmune response, in a condition called polyendocrine deficiency syndrome.
Polyendocrine deficiency syndrome
There are two types of this disorder and both types tend to run in families:
* Type I - is more common in children. Symptoms include underactive parathyroid, pernicious anaemia, recurring candida infections, chronic active hepatitis and slow sexual development.
* Type II - (Schmidt's syndrome) is more common in younger adults. Symptoms include underactive thyroid, diabetes mellitus and slow sexual development.
Other conditions related to primary Addison's disease are:
* Adrenomyeloneuropathy (AMN) - which can occur in some adults. It affects the spine, and is degenerative over time.
* Adrenoleukodystrophy (ALD) - which occurs (rarely) in some children (one in 100,000), especially males. It can cause brain damage and can be fatal. Survivors often develop AMN.
Treatment for primary Addison's disease is with glucocorticoid and mineralocorticoid replacement for life.
Secondary adrenal insufficiency
Sometimes, Addison's disease is caused by the pituitary gland's inability to produce sufficient amounts of ACTH, which means the adrenal glands aren't prompted to secrete cortisol. This is known as secondary adrenal insufficiency. Causes may include:
* Some medications - inflammatory disorders, such as rheumatoid arthritis and asthma, are often treated with prolonged or high dose steroids (glucocorticoid replacements). If the dose is suddenly stopped, or not reduced by appropriate tapering measures, the pituitary gland may respond by failing to produce enough ACTH. This situation can sometimes be reversed.
* Cushing's disease - a benign tumour of the pituitary gland that produces ACTH. This results in too much cortisol being produced. Treatment requires surgical removal of the tumour and, in some cases, removal of damaged adrenal gland(s).
* Other causes - infections, reduced blood flow, radiotherapy and some neurosurgery can damage the pituitary gland or hypothalamus, and decrease ability to produce ACTH.
Treatment for secondary Addison's disease is with glucocorticoid replacement only.
Addisonian crisis
A sudden, acute worsening of symptoms is known as an Addisonian crisis. It can be caused by:
* Extreme stress - an accident, excessive heat or physical exertion.
* Severe illness - especially dehydration from vomiting or diarrhoea.
* Sudden shock - for example, the death of a significant person.
The symptoms of Addisonian crisis include:
* Violent pain in the abdomen, back and legs.
* Nausea, vomiting or diarrhoea.
* Low blood pressure, low blood sugar, high potassium, low sodium and a rapid heart rate.
* Possible mental confusion and loss of consciousness.
Prompt emergency hospital treatment must be sought, including intravenous fluids, increased steroid medication and saline. Many Addisonians wear a medical alert bracelet or pendant with information and identification, and carry a hydrocortisone injectable for use in emergencies. They will still need hospitalisation and ongoing monitoring.
If untreated, an Addisonian crisis can be fatal.
Diagnostic tools
Diagnosis of Addison's disease may involve:
* Complete detailed family history, with special attention to any other endocrine disorders.
* Biochemical tests, which measure cortisol levels before and after a challenge injection of synthetic ACTH, known as a 'short synacthen test'. A 'long synacthen test' is done over several days in hospital. Synacthen tests will indicate the person's baseline level of cortisol production and their response to an increased need for cortisol in the body. An Addisonian may show a flat or reduced response.
* Blood electrolyte and plasma renin tests will indicate if there is a need for mineralocorticoid replacement.
* Anti-adrenal antibody test. If the result is positive, primary Addison's disease is definitively diagnosed. However, even if these antibodies do not exist, the person may still have Addison's disease.
* X-rays, ultrasounds and CAT scans of the abdominal region to check for visual signs of damage and the size of adrenal glands.
Treatment options
Treatment aims to bolster or replace insufficient or absent steroid components. Glucocorticoid replacement is essential for primary and secondary Addisonian patients, and must be taken for life. Treatment should:
* Be tailored to each person over the course of their life.
* Be altered, in consultation with a doctor, during illness or other stressful events.
* Allow for the different needs of children and young adults.
Where to get help
* Your doctor
* Endocrinologist
* Australian Addison's Disease Association Tel. (02) 6652 4761
* Australian Pituitary Foundation Tel. 1300 331 807 email: support@pituitary.asn.au
Things to remember
* Addison's disease is a progressive disorder, characterised by the inability of the adrenal glands to produce sufficient hormones.
* Causes can include infection, damage, and an autoimmune response that prompts the immune system to attack and destroy the adrenal glands.
* Treatment includes steroid replacement therapy that must be managed for life.
* An Addisonian crisis can be fatal unless treated quickly and appropriately.
ALINTIDIR.
ORGINAL
The human body has two adrenal glands, one on top of each kidney. These glands form part of the endocrine system, which works with the nervous system and the immune system to help the body cope with different events and stresses. Addison's disease is caused by the inability of the adrenal glands to make sufficient amounts of regulating hormones.
Adrenaline is the most well known of the hormones that are secreted by the adrenal glands, in the adrenal medulla (the central part of the gland). The adrenal cortex (the outer part) also produces important hormones, the corticosteroids. They include cortisol, aldosterone and supplementary sex hormones. In a person with Addison's disease, only the adrenal cortex is affected. The person cannot produce enough glucocorticoid or cortisol and, occasionally, also fails to produce sufficient mineralocorticoid or aldosterone.
Causes
Most cases of Addison's disease are caused by an autoimmune response that attacks and damages the adrenal glands over time. Other causes include:
* Infection
* Cancer
* Surgical removal of particular tumours in the adrenal or pituitary glands or the hypothalamus.
Symptoms
The symptoms of Addison's disease can include any or all of the following:
* Loss of appetite and weight.
* Nausea, vomiting or diarrhoea.
* Muscle weakness.
* Chronic, worsening fatigue.
* Low blood pressure.
* Salt cravings.
* Dehydration.
* Hypoglycaemia, or low blood sugar levels (especially in children).
* Increased pigmentation of the skin, particularly around scars and bony areas.
* Irregular or no menstrual periods in women.
* Mood swings, mental confusion or loss of consciousness.
These symptoms can develop quickly (especially in children and teenagers), or progress slowly for 20 years or more. Many symptoms can mimic other diseases, so diagnosis can be delayed.
The hormone cortisol
Cortisol is produced by the outer layer of the adrenal gland, called the adrenal cortex. The quantities of cortisol released by the adrenal glands are closely monitored by the master gland of the endocrine system, the pituitary, which is located in the brain. The workings of the pituitary are governed by another brain structure, the hypothalamus. When cortisol levels are too low, the pituitary secretes the stimulating hormone adrenocorticotropin (ACTH). Conversely, high levels of cortisol prompt the pituitary to decrease ACTH secretion, which slows cortisol production.
Cortisol plays many vital roles and is essential to many body functions because it:
* Works with adrenaline to help the body manage physical and emotional stress.
* Converts protein into glucose to boost flagging blood sugar levels.
* Works in tandem with the hormone insulin to maintain constant blood sugar levels.
* Reduces inflammation.
* Helps the body maintain a constant blood pressure.
* Helps the workings of the immune system.
The hormone aldosterone
Aldosterone is a mineralocorticoid, also produced by the adrenal cortex. The amount of aldosterone in the body is monitored by the kidneys, which secrete hormones to increase or decrease aldosterone production. Aldosterone regulates electrolytes (such as sodium and potassium) in the blood. This helps to maintain blood pressure and heart function. If too much sodium is excreted by the kidneys, a considerable amount of body fluid is also lost. This reduces blood volume and drops blood pressure. Too much or too little potassium can affect the way the heart functions.
Primary adrenal insufficiency
Addison's disease can occur gradually, and is defined when approximately 90 per cent of the adrenal gland(s) is damaged. This is known as primary adrenal insufficiency. Around seven out of 10 cases of Addison's disease are caused by an autoimmune response, where the body's own immune cells attack and destroy the adrenal glands. In some cases, other glands of the endocrine system are affected by an autoimmune response, in a condition called polyendocrine deficiency syndrome.
Polyendocrine deficiency syndrome
There are two types of this disorder and both types tend to run in families:
* Type I - is more common in children. Symptoms include underactive parathyroid, pernicious anaemia, recurring candida infections, chronic active hepatitis and slow sexual development.
* Type II - (Schmidt's syndrome) is more common in younger adults. Symptoms include underactive thyroid, diabetes mellitus and slow sexual development.
Other conditions related to primary Addison's disease are:
* Adrenomyeloneuropathy (AMN) - which can occur in some adults. It affects the spine, and is degenerative over time.
* Adrenoleukodystrophy (ALD) - which occurs (rarely) in some children (one in 100,000), especially males. It can cause brain damage and can be fatal. Survivors often develop AMN.
Treatment for primary Addison's disease is with glucocorticoid and mineralocorticoid replacement for life.
Secondary adrenal insufficiency
Sometimes, Addison's disease is caused by the pituitary gland's inability to produce sufficient amounts of ACTH, which means the adrenal glands aren't prompted to secrete cortisol. This is known as secondary adrenal insufficiency. Causes may include:
* Some medications - inflammatory disorders, such as rheumatoid arthritis and asthma, are often treated with prolonged or high dose steroids (glucocorticoid replacements). If the dose is suddenly stopped, or not reduced by appropriate tapering measures, the pituitary gland may respond by failing to produce enough ACTH. This situation can sometimes be reversed.
* Cushing's disease - a benign tumour of the pituitary gland that produces ACTH. This results in too much cortisol being produced. Treatment requires surgical removal of the tumour and, in some cases, removal of damaged adrenal gland(s).
* Other causes - infections, reduced blood flow, radiotherapy and some neurosurgery can damage the pituitary gland or hypothalamus, and decrease ability to produce ACTH.
Treatment for secondary Addison's disease is with glucocorticoid replacement only.
Addisonian crisis
A sudden, acute worsening of symptoms is known as an Addisonian crisis. It can be caused by:
* Extreme stress - an accident, excessive heat or physical exertion.
* Severe illness - especially dehydration from vomiting or diarrhoea.
* Sudden shock - for example, the death of a significant person.
The symptoms of Addisonian crisis include:
* Violent pain in the abdomen, back and legs.
* Nausea, vomiting or diarrhoea.
* Low blood pressure, low blood sugar, high potassium, low sodium and a rapid heart rate.
* Possible mental confusion and loss of consciousness.
Prompt emergency hospital treatment must be sought, including intravenous fluids, increased steroid medication and saline. Many Addisonians wear a medical alert bracelet or pendant with information and identification, and carry a hydrocortisone injectable for use in emergencies. They will still need hospitalisation and ongoing monitoring.
If untreated, an Addisonian crisis can be fatal.
Diagnostic tools
Diagnosis of Addison's disease may involve:
* Complete detailed family history, with special attention to any other endocrine disorders.
* Biochemical tests, which measure cortisol levels before and after a challenge injection of synthetic ACTH, known as a 'short synacthen test'. A 'long synacthen test' is done over several days in hospital. Synacthen tests will indicate the person's baseline level of cortisol production and their response to an increased need for cortisol in the body. An Addisonian may show a flat or reduced response.
* Blood electrolyte and plasma renin tests will indicate if there is a need for mineralocorticoid replacement.
* Anti-adrenal antibody test. If the result is positive, primary Addison's disease is definitively diagnosed. However, even if these antibodies do not exist, the person may still have Addison's disease.
* X-rays, ultrasounds and CAT scans of the abdominal region to check for visual signs of damage and the size of adrenal glands.
Treatment options
Treatment aims to bolster or replace insufficient or absent steroid components. Glucocorticoid replacement is essential for primary and secondary Addisonian patients, and must be taken for life. Treatment should:
* Be tailored to each person over the course of their life.
* Be altered, in consultation with a doctor, during illness or other stressful events.
* Allow for the different needs of children and young adults.
Where to get help
* Your doctor
* Endocrinologist
* Australian Addison's Disease Association Tel. (02) 6652 4761
* Australian Pituitary Foundation Tel. 1300 331 807 email: support@pituitary.asn.au
Things to remember
* Addison's disease is a progressive disorder, characterised by the inability of the adrenal glands to produce sufficient hormones.
* Causes can include infection, damage, and an autoimmune response that prompts the immune system to attack and destroy the adrenal glands.
* Treatment includes steroid replacement therapy that must be managed for life.
* An Addisonian crisis can be fatal unless treated quickly and appropriately.
ALINTIDIR.
ORGINAL
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