The Addison's disease (AD) or the Primary adrenal insufficiency It is an autoimmune pathology that is characterized by a dysfunction or hypofunction of the adrenal cortex (Royo Gómez et al., 2013).
This disorder therefore occurs when the body begins to produce an insufficient amount of several hormones that produce the adrenal glands. Specifically, these glands produce high amounts of cortisol and reduced aldosterone (Mayo Clinic, 2015).
It is a rare disease in childhood, but it can be potentially fatal if not diagnosed early (Royo Gómez et al., 2013).
In general, symptoms progressively develop with asthenia , anorexy , Weight loss, vomiting, Hypoglycemia , Among others (Royo Gómez et al., 2013) and are usually the result of infectious diseases (Cassama et al., 2006).
What are the functions of the adrenal glands?
The adrenal glands are located in the back of the two kidneys (NHI, 2014).
The outer region, the cortex, is responsible for the production of different steroid hormones such as Cortisol , Aldosterone And different hormones that can be transformed into Testosterone (NHI, 2014).
On the other hand, the inner region, the medulla, is responsible for the production of Epinephrine Y Norepinephrine (NHI, 2014).
The efficient production of this type of hormone is essential for the optimal functioning of our body. When these glands or other structures alter the production of one or several essential hormones for the body, various pathologies may develop (NHI, 2014).
Primary disorders such as infectious, autoimmune, Neoplasms or autoimmune diseases Can significantly alter the hormonal production of the adrenal glands (NHI, 2014).
The Adrenal hormones Play an essential role in the functioning of the body, regulation of the blood pressure , The metabolism, the use of nutrients, or the different responses of the organism stress (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
In relation to the main hormones that are altered in Addison's disease:
Cortisol
This hormone belongs to the group of Glucocorticoids Which affects almost all body tissues and organs almost universally (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
He Cortisol It is a substance that promotes the body's response to stress. On the other hand, it also helps maintain blood pressure, heart function, blood glucose level (Cleveland Clinic, 2015).
On the other hand, cortisol also contributes to the regulation of immune system , When the body recognizes a foreign agent and defends itself against bacteria, viruses and other harmful substances (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
The production of optimal and balanced amounts of cortisol is regulated by the hypothalamus and pituitary gland (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
He Hypothalamus Releases a hormone called corticotropin-releasing hormone (HLC), which signals the pituitary gland to release adrenocorticotropic hormone (ACTH) that stimulates the adrenal glands to produce cortisol (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Aldosterone
The Aldosterone Is a type of hormone that belongs to the group of mineralcorticoids and is also produced by the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Aldosterone is related to balance of sodium and blood potassium. In addition, it controls the amount of fluids that the kidneys must remove (urine) (Cleveland Clinic, 2015).
Decreased sodium in the blood can cause both a decrease in blood pressure and total blood volume. In addition, it can also lead to Hyponatraemia (Decreased sodium causing symptoms of confusion, fatigue, fatigue, muscle spasms and / or convulsions) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Dehydroepiandrosterone
Although it is a substance that is less affected by the development of Addison's disease, dehydroepiandrosterone (DHEA) is another hormone produced by the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Our body uses the Dehydroepiandrosterone (DHEA) for the production of sex hormones, androgens and extrrogens. When adrenal insufficiency occurs, sufficient amounts of dehydroepiandrosterone (DHEA) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014) may not be produced.
Although healthy men and women derive most androgen and estrogen from sexual structures, specifically in women and adolescents, dehydroepiandrosterone (DHEA) deficiencies can lead to loss of pubic hair, depression or loss of sexual interest (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Characteristics of Addison's Disease
Adrenal insufficiency is an endocrine / hormonal disorder that occurs when Kidney glands Do not produce enough of certain hormones (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Adrenal insufficiency can be primary or secondary. However, the term Addison's disease is commonly used to denote primary adrenal insufficiency (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Adisson's disease or primary adrenal insufficiency is a pathology that is related to hypofunction of the adrenal glands (Cleveland Clinic, 2015). This alteration can lead to a low production of cortisol and aldosterone or even the inactivation of the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
However, we can also distinguish a type of secondary adrenal insufficiency that affects the Functioning of the pituitary gland (Gládula located at cerebral level). There is a decrease in the secretion of adrenocorticotropic hormone, which is responsible for activating the adrenal glands to produce the hormones mentioned above (Cleveland Clinic, 2015).
About us
Secondary adrenal insufficiency is much more frequent than Addison's disease or primary adrenal insufficiency (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Approximately 110-144 individuals out of every one million people suffer from Addison's disease (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
The incidence of Addison's disease is estimated at 0.8-1.4 cases per 100,000 population / year. In addition, it is a rare disease in the pediatric age (Royo Gómez et al., 2013)
In the United States, Addison's disease affects 1 in 100,000 people and occurs in both men and women in a similar way.
Although it can occur in all age groups, it is more common to appear between 30 and 50 years of age (Cleveland Clinic, 2015).
Symptoms and signs
Usually the symptoms of Addison's disease develop gradually over several months (Mayo Clinic, 2015).
The most frequent symptoms of adrenal insufficiency are (National Institute of Diabetes and Digestive and Kidney Diseases, 2014):
- Chronic and long-term fatigue.
- Muscular weakness.
- Abdominal pain.
Other symptoms that are also commonly seen in individuals with Addison's disease are (Mayo Clinic, 2015):
- Loss of weight and significant decrease in appetite.
- Hyperpigmentation or darkening of the skin.
- Reduced blood pressure, fainting.
- Hypoglycemia or low blood glucose levels.
- Need or desire to ingest salt.
- Nausea, diarrhea, vomiting.
- Abdominal pain and discomfort
- Muscle and joint pain and discomfort.
- Irritability.
- Depressive symptomatology.
- Loss of hair (when there is a dysfunction of sex hormones in women.
In general, slowly progressing symptoms have to be ignored until an event with a high level of stress, such as surgery, illness, serious injury or pregnancy, worsens its clinical course (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
In other cases, the symptoms of this pathology may occur suddenly, leading to an acute Addison's disease crisis or Addison's crisis (Mayo Clinic, 2015):
- Acute renal failure.
- Pain in the abdomen, lower back and legs.
- Vomiting and severe diarrhea.
- Dehydration.
- Significant reduction in blood pressure.
- High levels of potassium (hyperkalemia) and low sodium content (hyponatremia).
Causes
Autoimmune disorders and disorders are the main cause of most cases of Addison's disease.
However, some infections and / or drugs may also contribute to the development of this pathology (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Autoimmune Disorders
Addison's disease can occur as a consequence of an autoimmune response of the organism (Cleveland Clinic, 2015).
Approximately 80% of cases of Addison's disease are caused by an immunological type disorder (National Institute of Diabetes and Digestive and Kidney Diseases, 2014), which occurs when the immune system"attacks". To their own organs and tissues (Cleveland Clinic, 2015).
In Addison's disease, the immune system attacks the outside of the adrenal glands where cortisol and aldosterone are produced (Cleveland Clinic, 2015).
The autoimmune cause of Addison's disease occurs mainly in middle-aged women (National Institute of Diabetes and Digestive and Kidney Diseases, 2014)
Infections
Some infectious causes that may lead to the development of Addison's disease have also been described (Cleveland Clinic, 2015).
Tuberculosis is one of these infections that can damage or destroy the adrenal glands. Approximately 10-15% of cases of Addison's disease have their origin in tuberculosis (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
On the other hand, recent clinical investigations have indicated an increase of the cases of Addison's disease as a result of the cytomegalovirus (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Cytomegalovirus does not usually cause symptoms in healthy people, however, if it affects babies during gestation or people who have a immune system (VHI) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
One of the causes of meningitis (Neisseria meningitidis) or fungal infections can also lead to the development of Addison's disease (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).
Other (less common) causes that may lead to the development of Addison's disease have also been described (Cleveland Clinic, 2015):
- Injuries to the adrenal glands.
- Oncologic metastasis.
- Bleeding from the adrenal glands.
- Surgical removal of the adrenal glands.
- Amyloidosis (abnormal accumulation of certain proteins)
- Genetic defects.
Treatments
All therapeutic interventions for Addison's disease focus on the use of hormone replacement therapies to compensate and correct hormone levels (Mayo Clinic, 2015):
Some of the treatment options are (Mayo Clinic, 2015):
- Oral corticosteroids or injections : the Hydrocortisone , Prednisone Or cortisone acetate are often used to replace cortisol. In the case of aldosterone deficiency, some specialists recommend the use of fludrocostisone.
On the other hand, abundant amounts of sodium are also recommended, especially during intense exercise, warm weather, or intense hormonal diarrhea and vomiting (Mayo Clinic, 2015).
In addition, it should be noted that when an Addison crisis occurs, the survival of the person is seriously threatened: low levels of blood pressure , Blood glucose, or high levels of blood potassium (Mayo Clinic, 2015).
When a Addison crisis develops, getting medical care is essential. Treatment in the acute phase generally includes (Mayo Clinic, 2015):
- Hydrocortisone.
- Saline solutions.
- Glucose.
References
- ASHG. (2002). What is Addison's disease? Obtained from Addison's self help group: http://www.addisons.org.uk/info/
- Cassama, C., Pieri, C., Macedo, B., & Teixeira, J. (2006). Addison's disease.
- Cleveland Clinic. (2015). Addison's Disease . Retrieved from the Cleveland Clinic: https: //my.clevelandclinic.org/health/
- Mayo Clinic. (2015). Addison's Disease . Retrieved from the May Clinic: http://www.mayoclinic.org/
- NHI. (2014). Adrenal Insufficiency and Addison's Disease . Retrieved from the National Institute of Diabetes and Digestive and Kidney Diseases: http://www.niddk.nih.gov/
- NHI. (2016). Addison Disease . Retrieved from MedlinePlus: https://www.nlm.nih.gov/
- Royo Gómez, M., Olmos Jiménez, M., Rodríguez Arnao, M., & Roldán Martín, M. (2013). Addison's disease. Forms of presentation in pediatrics. An Pediatr, 78 (6), 405-408.