Friday, April 19, 2013

Clinchem5aC: Adrenal Cortex Diseases

“The physician should not treat the disease but the patient who is suffering from it”  ― Maimonides 

I prepared a short and easy game for you as a way of introducing the topic I will tackle. You just have to guess the word or term from the jumbled letters. Additional clues are also given.

  1. Ieedass  -  A pathological condition of a part, organ, or system of an organism that results from various causes, such as infection, hereditary defect, or environmental stress, and characterized by a particular group of signs or symptoms.

2. Iinlalcc Mtcysihre   -  is the area of clinical pathology that is mainly concerned with analysis of body fluids.

3. Daaernl Lndga - A small gland located on top of the kidney that produces hormones which facilitate in different activities of the body.

4. Daaernl Toecrx - The outer portion of the adrenal gland located on top of each kidney which produces steroid hormones.

5. Ssmmtyopany subjective evidence of disease perceived or felt by the patient.

6. Gsnis - Any objective evidence of a disease, such as blood in the stool, a skin rash that can be recognized by the doctor, nurse, family members and the patient.


7. Osnoehmr - A chemical substance produced in the body and secreted by glands that controls and regulates the activity of certain cells or organs.

8. Mteettarn - the application of medicines, surgery, therapy, etc., in treating a disease or disorder.



Now,that you've known some terms that are related in the topic let me introduce it to you. This blog is intended for all the readers who are not that familiar of the different diseases of Adrenal Cortex.I'll share the information I have learned from my previous subjects.


    I've learned that the adrenal cortex produces a hormone that regulates some type of organs and tissues within the body called the cortisol. It also produces aldosterone. Inside the body, it facilitates in maintaining appropriate proportions of water and salts.Disease results when there is deficiency or too much secretion of these hormones.A person with adrenal insufficiency may experience fatigue, poor appetite,dizziness, weight loss and nausea.

    The first disease that I will bring in, is Primary Aldosteronism/Primary Hyperaldosteronism. But before that, did you know that it is also referred to as Conn's syndrome? The disease is named after Jerome W. Conn,an American Endocrinologist who first described the condition at the University of Michigan in 1955. According to Paul Fitzgerald, M.D., Clinical Professor of Medicine at the University of California in Current Medical Diagnosis Treatment, five to 10 percent of people with hypertension have high blood pressure due to primary aldosteronism.

    What happen in your body when you have a Primary Aldosteronism? The outer zone of the adrenal cortex called the zona glomerulosa produces and secretes mineralocorticoids,a steroid hormone. The adrenal cortex secretes aldosterone which is the main mineralocoricoid. Too much secretion of aldosterone by the adrenal cortex happens. Aldosterone let the kidneys keep sodium. However, storing sodium means more water is reabsorbed. This results to increase in the blood volume, which increases the blood pressure. Based on research, the most common cause of this disorder is a benign tumor. 

       How will you know if you have this kind of disease? If you are having moderate to severe high blood pressure and that it takes several medications to control or a high blood pressure along with a low potassium level or hypokalemia, you might have a Primary Aldosteronism and must see a doctor.

       What are the possible treatments on this kind of disease? Treatment for primary aldosteronism depends on the underlying cause but its main purpose is to normalize, or block the effect of high aldosterone levels and prevent the probable complications of high blood pressure and low potassium levels. Like for instance,an adrenal gland tumor may be treated with surgery or medications and lifestyle changes.If the disease is caused by overactivity of both adrenal glands, a combination of medications and lifestyle modifications can effective. Also, the doctor may first prescribe spironolactone also known as aldactone.
Source:http://www.pharmabol.com/shop/images/large/aldactone.jpg

A medication facilitates in correcting high blood pressure and low potassium. In addition to blocking aldosterone receptors, spironolactone blocks androgen and progesterone receptors may inhibit the action of these hormones. Although there are side effects, such as male breast enlargement, decreased sexual desire , impotence, menstrual irregularities and gastrointestinal distress. And the easiest way is to work with the doctor to create a plan concerning your diet and maintain a healthy body weight. Getting regular exercise, limiting the amount of alcohol you drink and stopping smoking to improve your response to medications.


    Another disease of Adrenal Cortex is Addison's Disease. Did you know that the disease is named for its discoverer, Dr. Thomas Addison, a British surgeon who described adrenal insufficiency in 1849, though endocrine functions had yet to be explained. Addison described the condition from autopsies he performed. At the time, there was no cure for adrenal insufficiency, so victims died after contracting it. Addison also noted that 70-90% of patients with adrenal insufficiency had tuberculosis as well.
*Got the info. from: Addison's Disease - History Of Addison's Disease - Adrenal, Insufficiency, Tuberculosis, and Described - JRank Articles http://science.jrank.org/pages/74/Addison-s-Disease-History-Addison-s-disease.html#ixzz2QymNZhBb


     What happen in your body when you have Addison's disease? In Addison’s disease, the adrenal cortex has been destroyed. The adrenal gland secretes less glucocorticoids and mineralocorticoids. Next to the zona glomerulosa is the zona fasciculata, which makes the glucocorticoids, a steroid hormone. Cortisol, which is the main glucocorticoid increases the level of glucose in the bloodstream so people have energy to manage stress. People with Addison’s disease have low levels of cortisol and sodium. Instead of only attacking foreign substances, the immune system attacks its own tissues and organs.

Source: http://www.nsc.gov.sg/showpage.asp?id=220
       How will you know if you have this kind of disease? If you are experiencing muscle weakness and fatigue, and notice you're losing weight and appetite, your skin darkens, you have low blood pressure and blood sugar, you even experience fainting, salt craving nausea, diarrhea or vomiting, muscle or joint pain, irritability, depression, body hair loss or sexual dysfunction.

       What are the possible treatments on this kind of disease? Hormone replacement therapy involved all the treatment for Addison's disease in correcting the levels of steroid hormones the body isn't producing. The therapy is done in various ways, such as Oral corticosteroids, Corticosteroid injections, Androgen replacement therapy. Also, an ample amount of sodium is recommended, especially during heavy exercise, when the weather is hot, or if you have gastrointestinal upsets, such as diarrhea. If facing a stressful situation, the doctor will also suggest a temporary increase in the dosage such as an operation, an infection or a minor illness.




     One more disease of Adrenal Cortex is Cushing's Syndrome. Did you know that this syndrome was first described in 1912 when
Harvey Cushing
the neurosurgeon Harvey Cushing described a patient known as Minnie G. She showed all the characteristic signs and symptoms of the syndrome, including hyperglycaemia. Since cortisone and adrenocorticotrophic hormone (ACTH) were unknown at the time, Cushing suggested a polyglandular disorder. 
The term Cushing's syndrome was coined by Fuller Albright in 1943 to designate the consequences of overproduction of a hormone affecting carbohydrate metabolism by the adrenal cortex.

   
     What happen in your body when you have the Cushing's Syndrome? High levels of cortisol in the bloodstream are seen. People may have Cushing’s disease, where they have a benign tumor in the anterior pituitary gland of the brain. To tell the adrenal gland to secrete cortisol, this gland secretes the adrenocorticotropic hormone (ACTH). Without any regulation, a carcinoid tumor and small cell lung cancer secrete ACTH. Adrenal cancer and an enlarged adrenal gland secrete high levels of cortisol.

Source: http://www.tumblr.com/tagged/cushing's%20syndrome
     How will you know if you have this kind of disease? Progressive obesity and skin changes, weight gain and fatty tissue deposits, mainly around the midsection and upper back, in the face, and between the shoulders, pink or purple stretch marks on the skin of the abdomen, thighs, breasts and arms, thinning, fragile skin that bruises easily, slow healing of cuts, insect bites and infections and also acne are commonly seen on people with this kind of disease. But some signs and symptoms may vary. If you are a female and have this disease, you may have thicker or more visible body and facial hair. And an irregular or absent menstrual periods. But if you are a male, you may have a decreased libido, decreased fertility and an erectile dysfunction.

     What are the possible treatments on this kind of disease? Treatments for Cushing syndrome are intended to lower the high level of cortisol in the body. The best treatment depends on the cause of the syndrome. Options are reducing use of corticosteroid, surgery, radiation therapy, medications. Based on my readings the doctor may recommend surgical removal of the adrenal glands, if none of these treatment options is effective. This procedure will cure excess production of cortisol. But, the ACTH levels will remain high, possibly causing excess pigmentation of your skin. 

                             Story of Debbie who had Cushing's Disease

    The last disease I will tackle is Adrenogenital syndrome. Did you know that this disease is also referred to as Congenital adrenal hyperplasia (CAH). And the person that contributed to the discovery of the disease is Luigi De Crecchio, an italian anatomist. It is a family of autosomal recessive disorders of steroid hormone production in the adrenal glands resulting to a deficiency of cortisol, which is the stress fighting hormone.



     What happen in your body when you have the Adrenogenital syn drome? The pituitary which is the master hormonal regulatory gland, sensing the deficiency, secretes substantial amounts of the stimulating hormone corticotropin to bring the cortisol levels up to normal. At the same time, this hormone causes the adrenal glands to overproduce certain intermediary hormones which have testosterone-like effects on the fetus and child, leading to virilization, which means that there is enlargement of clitoris among females, and may resemble the male penis to the point that the sex of the child is questioned or mistaken. Males’ penis also enlarge.


     How will you know if you have this kind of disease? Newborn females with this condition usually have a swollen clitoris, their genitalia are indistinct which often appears more male than female. The internal features of the female's ovaries, uterus, and fallopian tubes are normal. As the girl ages, different other
*Source: http://imueos.wordpress.com/2010/04/29/
adrenal-disorders-%E2%80%93-adrenogenital-syndrome/
features will begin to appear more masculine, such as deepening of the voice, facial hair, and lack of menstruation during puberty. 
While newborn males do not present any obvious changes, signs of the condition will become noticeable before puberty begins. A boy will become increasingly muscular, the penis will enlarge, pubic hairs will become apparent and his voice will deepen. Based on my readings, these typical signs of puberty may occur as early as 2-3 years of age. The testes, at time of puberty, will be unusually small as well.


     What are the possible treatments on this kind of disease? Treatment of this condition involves hormone replacement. Treatment is monitored by measures of blood salt composition, by suppression of overly-rapid sexual maturation, and by monitoring of the skeletal maturation rate by bone age testing.


Thank you for visiting my blog. I hope you learned something from it:)

References: Donald Calbreath: Clinical Chemistry: A Fundamental                Textbook
Michael Bishop: Clinical Chemistry: Techniques, Principles and Correlations (6th ed.)
Henry's Clinical Diagnosis and Management by Laboratory Methods (22nd Ed.)