What is Addison’s Disease?

April 22 22:42 2019 Print This Article

It is defined as a disorder in which there is not an underlying disease process. It is named after Dr. Thomas Addison who was the first to describe this condition. He was a British physician. He described the condition in on the constitutional and local effects of the disease in a supra renal capsules in the year 1849. The adjective addisonian describes the features and conditions of the patients suffering from this disorder. In the year 1855 6 patients of this disorder had adrenal tuberculosis.

What are the signs and symptoms of Addison’s disease?

It includes the sudden onset of symptoms. It takes some time to get recognized. It includes the fatigue, dizziness, muscle weakness, loss of weight, difficulty in standing up, anxiety, diarrhea, head ache, sweating, change in the personality, and sudden drop in the blood pressure. It happens when a person changes its position from seated to standing. There is a joint and muscle pain. The patients have a marked carving for a salty food due to the urinary loss of sodium. There is an adrenal insufficiency which is manifested by the hyper pigmentation.

The clinical signs include the hypo thalamus, pituitary gland and adrenal cortex. There is a negative feedback loop for gluco corticoids as primary hypo cortislism is manifested as a deficiency in the release of gluco corticoid. It occurs from the adrenal cortex in which there is an increase in the ACTH secretion. It is released by the pituitary which initiates the release of gluco corticoid which are absent. It occurs due to the over stimulation of ACTH which makes the skin to look bronze. There is a secondary or tertiary hypo cortisolism in which there is a deficiency of the CRH or ATH release. It occurs with the help of hypo thalamus or pituitary gland. It is manifested as no ACTH release and the later will manifest as a normal response. There is no over production of ACTH. On an examination one notices a low blood pressure which falls when an individual stands and is referred as an ortho static hypo tension.

The people with the primary disorder have a hyper pigmentation of the area which is present on the shades and does not include the exposure to sun. It includes the skin creases present on the hands, nipples and inside of the cheek. It involves the buccal mucosa. The old scars become dark in the color. They occur due to the melanocyte stimulating hormones which are referred as a MSH and adrenocoorticotrophic hormone which is referred as the ACTH. They share the same precursor molecule known as pro opio melano cortin referred as POMC. After it is produced in the anterior pituitary the POMC gets cleaved into gamma MSH, acth and beta lipotropin. The sub unit adrenocoorticotrophic hormone which is referred as the ACTH goes into the cleavage to form a melanocyte stimulating hormones which are referred as a MSH. It is the most important for the pigmentation of skin. There is a secondary or tertiary form of Addison’s disease in which the darkening of skin does not occur. There are certain medical conditions which occur in association with the Addison’s disease which include the goiter and vitiligo.

What is the Addisonian crisis?

It is also known as the adrenal crisis. It is defined as a disorder in which there is a collection of symptoms and indicates a severe insufficiency of the adrenal. It may occur due to the result of previous undiagnosed disease of this disorder. It is sudden in onset and involves the function of adrenal and is known as the adrenal hemorrhage. There may also occur a inter current problem known as the infection or trauma. This condition develops in the people who use long term oral gluco corticoids and have suddenly left it. They also suffer from long use of synthetic gluco corticoids. They cause further atrophy of the adrenal glands. It occurs by a negative feedback.

It may also cause a myxedema coma, thyroxine and without a glucose one can precipitate a crisis. If this condition is not treated it can become fatal too. It is mainly a medical emergency which requires the hospitalization. The symptoms included are the fever, low blood pressure, syncope, convulsions, hypo glycemia, hyper calcemia, confusion, slurred speech and psychosis. There is a sudden penetrating pain in the legs, lower back and abdomen. There is a severe vomiting and diarrhea which results in the dehydration. The blood pressure is low which is followed by the syncope. There is a severe lethargy also.

What is the diagnosis of Addison’s disease?

It includes the routine investigations. There is a hypo glycemia with a low blood sugar, hyper calcemia with a raised blood calcium levels. There is a hypo natraemia with a low blood sodium levels. It occurs due to the loss of production of aldosterone. There is a hyper kalemia in which there is a raised blood potassium levels. It occurs due to the loss of production of aldosterone. There is an eosinophilia in which there is an increased number of eosinophils and lymphocytosis in which there is an increased number of white blood cells. There is a metabolic acidosis in which there is an increased acidity of the blood. It also occurs due to the loss of production of aldosterone.

The sodium re absorption occurs in the distal tubule and is linked with the secretion of hydrogen ion or acids. The low levels of aldosterone stimulate the renal distal tubules which may lead to the sodium wasting in the urine and hydrogen ion retention in the serum. In the suspected cases of Addison’s disease we need to demonstrate that the adrenal hormones are low even after the appropriate stimulation. It is referred as the ACTH stimulation test. It occurs with the synthetic pituitary ACTH hormone known as the tetracosactide. There are two tests which are done and include the short and long test. The short test compares the blood cortisol levels. They are compared before and adter the 250 micro grams of tetracosactide. It is given intra muscularly or intra venously.

After one hour if the plasma cortisol level is more than 170 n mol per litre. It may rise up to the 330 to 690 n mol per litre. It may be considered as an adrenal failure. The short test is abnormal and the long test is used to differentiate between the primary and secondary adrenal failure. The long test uses the 1 mg tetracosactide intra muscularly. Blood is taken 1 4 8 and 24 hours later. The normal plasma cortisol levels must reach 1000 n mol per litre by 4 hours. In the primary Addison disease the cortisol level is reduced at all stages and in the case of secondary Addison’s disease a delayed but normal response is seen. There are other tests which are done to differentiate the various causes of hypo adrenalism.

These include the rennin and adreno corticotropic hormone levels. It also includes the medical imaging in the form of ultra sound and magnetic resonance imaging which is referred as the MRI. There is computed tomography also. There is adreno leuko dystrophy and a milder form adreno myelo neuropathy leads to the adrenal insufficiency. It is combined with the neurological symptoms. These disorders lead to the adrenal insufficiency in one third of the males with idiopathic Addison’s disease. It must be consider with the differential diagnosis of male with adrenal insufficiency. The diagnosis is made by the blood test to detect very long chain fatty acids.

What are the causes of Addison’s disease?

It includes the grouping by the way in which there is a way by which adrenals have to produce insufficient cortisol. It is known as the adrenal dysgenesis which means the gland has not formed fully during development. There is an impaired steroid genesis in which there is a presence of the gland but is not able to form cortisol during the development. There is an adrenal destruction in which the gland is damaged due to the progression of disease. It is referred as an adrenal destruction.

What is adrenal dysgenesis?

It is defined as a disorder in which there is a rare occurrence and is genetic in nature. It includes the mutations in the SF1 transcription factor and congenital adrenal hypo plasia. It occurs due to the mutations in DAX 1 gene. The mutations to ACTH receptor gene can lead to the related gene which is known as the All grove syndrome. The mutations in DAX 1 gene may cluster in a syndrome with a glycerol kinas deficiency. It occurs with other symptoms when DAX 1 gene is removed along with the number of other genes.

What is impaired steroid genesis?

It is defined as a disorder in which there is adrenal gland needs cholesterol which is changed into the steroid hormones and form a cortisol. There is an interruption in the delivery of cholesterol which includes the smith lemli optiz syndrome along with the abeta lipo proteinemia. The congenital adrenal hyper plasia is the most common form of synthetic problems. There is a lipoid CAH which occur due to the deficiency of St AR and mitochondrial DNA mutations. There are some medicines which interfere with the steroid synthesis enzymes. It includes the ketoconazole. There are some medicines which accelerate the normal breakdown of hormones. It occurs by the liver and includes the rifampicin and phenytoin.

What is adrenal destruction?

It is defined as a disorder in which there is an auto immune adrenalitis. In this there is an auto immune destruction of the adrenal cortex due to the antibodies against the enzyme 21 hydroxylase. It is a common cause of this disorder mainly in the teenagers and adults. It can be isolated or may be present in the context of auto immune poly endocrine disorder which is referred as APS type 1 or 2. It is also a feature of adreno leuko dystrophy which is referred as the ALD. The adrenal glands can be involved in a metastasis in which the cancer cells are spread everywhere from the lungs. There is a hemoorhage which occur in the case of water house syndrome or anti phosphor lipid syndrome. It occurs in the certain infections like tuber culosis and histo plasmosis. One can also observe the deposition of abnormal protein. It is also known as amyloidosis.

What is the epidemiology of Addison’s disease?

It includes the frequency rate of this disorder in the human population to be 1 in 100,000. There are few researches which have put the number up to 50 cases per 1 million populations. It is very difficult to determine the exact number of cases of this disorder. Some of the figures are not correct. This disorder can affect the person of any age, gender or ethnicity. It is mainly present in the people between ages 30 to 50 years of age. There is no significant predisposition based on the ethnicity.

What is the prognosis of Addison’s disease?

It includes the proper medicines and a person can live a healthy and a normal life. The person with an adrenal insufficiency carries an identification stating his condition in the case of emergency. The card must alert the emergency personnel about its need to inject 100 mg of cortisol. It is needed if the bearer is severely injured and he is unable to answer the questions. The card must contain the doctor’s name and address with a telephone number. It must also contain the nearby relatives name and address with a telephone number. When the patient travels he must carry a needle, syringe and an injectable form of cortisol. The person with this disorder must also carry an increased medicine in case of stress and mild respiratory tract infections. There is a need for immediate medical attention when there is a diarrhea, infection or a vomiting. This condition can precipitate an addisonian crisis. The patient who vomits needs an injection of hydro cortisone as oral hydro cortisone are not adequately metabolized.

What is Canine hypoadrenocorticism?

It is defined as a disorder in which there is an under diagnosis and one has the suspicion of this disease. It occurs mostly in all breeds of the dogs. The patient represents many complaints. It occurs mainly in the females with age mainly from 4 to 7 years. It can occur in any age and at any gender. The genetic continuity between the dogs and humans helps to know about this disorder in both the species. This disorder is treated with the fludro cortisone or florinef. It is done with a monthly injection by percorten V. One can also use the prednisolone. There is a need of rotine blood work in the initial stages and there requires a maintenance dose for it. The medicines used in the treatment of this disorder leads to more of thrust and urination. It is necessary to provide the fresh drinking water to the patients who suffer from this disorder. If the patient knows that he has to travel than he needs an increased dose of prednisone which helps to deal with the stress. The dogs with this disorder must also avoid the stress.

What is Famous Addisonians?

It is defined as a disorder in which there were famous personalities which were suffering from this disorder. It included the John F Kennedy the former president of America was having this disorder. He was the first person to have this disorder and survived a surgery. His health was a secret issue during the time he was a president. The 25th amendment of the US constitution was introduced to maintain secrecy. There was an E K Shriver the sister of J F Kennedy who was also suffering from this disorder. The popular singer H Reddy and a scientist E M Shoemaker who was the co discoverer of comet shoe maker levy 9 also suffered from this disorder. There was a French Carmelite nun and a religious writer Blessed Elizabeth of the Trinity who also suffered from this disorder. There was Jane Austin also suffered from this disorder. A Canadian medical researcher Charles dickens also suffered from this disorder as suggested by Dr. C Abbot. This disorder may have also affected the Osama bin laden. A L Wright noticed that the Osama had all the features which included the low blood pressure, fatigue of muscles, irritation in the stomach, sharp craving for salt, loss of weight, back pains and dehydration. He is known to intake a large amount of drug known as the sulbutamine. There were other famous personalities like S Arana, a Spanish writer and A Herndon, famous black American educator who was an aspiring actress who was married to A Herndon and a mother of N Herndon. There was a T Cansever who was a Turkish architect who was a renowned for the one to receive an Aga Khan award for the architecture three times.

What is the treatment of Addison’s disease?

It includes the maintenance treatment in which the missing cortisol is replaced. It occurs in the form of hydro cortisone tablets as a dose regimen. It mimics the physiological concentrations of cortisol. Around 25 percent as much as prednisolone is used for the equal gluco corticoid effect like hydro cortisone. The treatment is continued for the life time. There are many patients which require fludro cortisone as a replacement for the missing aldosterone. One must be cautious using it and also when the patient becomes unwell and requires a surgery or is pregnant. The medicines are required at the time of stress, infection or any injury.

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