Discussion w5 652

Q-1

Adrenal Insufficiency (Addison’s Disease)

Etiology

The most common cause of Addison’s disease (80 to 90%) in developed countries is by dysfunction of autoimmune system with antibodies directed against the adrenal cortex or 21-hydroxylase, a steroid involved in biosynthesis of aldosterone and cortisol, present in patients with autoimmune dysfunction (Huecker, 2021). Tuberculosis is another cause in endemic countries. Less common origins are infectious disease that results in destruction of the adrenal glands, systemic fungal infection, opportunistic infections secondary to HIV, metastatic malignancies, medications, and some conditions that interfere with cortisol synthesis (Huecker, 2021).

Clinical Manifestations

Substantial weakness associated with mucocutaneous hyperpigmentation, hypotension and/or postural hypotension and salt craving (Huecker, 2021).

Common Presentation

Fatigue is commonly reported by patients maybe described as weakness or tiredness, anorexia, weight loss, hyperpigmentation evident in mucosa and sun-exposed areas notable in palmar crease, scars and areas of friction. Hyponatremia is a result of decreased aldosterone production. Salt craving is specific to Addison’s disease but is only demonstrated by 16% of patients (Huecker, 2021).

Consultant orders for diagnostic test

The first diagnostic test I will order is the morning plasma cortisol level between 8 am and 9 am when the cortisol levels peak. If the patient’s cortisol level is 3 to 18 mcg/dL, I will order further testing with ACTH stimulation test also known as Cosyntropin test. It is ordered when morning serum cortisol does not confirm or exclude adrenal insufficiency (Mongioì et al., 2019). This test can be performed at any time of the day. Cosyntropin is a synthetic derivative of adrenocorticotropic hormone (ACTH) that is used in the evaluation and diagnosis of patients with adrenocortical insufficiency. The pharmacologic profile of Cosyntropin is similar to that of purified natural ACTH. If cortisol levels below 18 micrograms/dL are found at either 30 or 60-minutes after ACTH stimulation, the diagnosis of adrenal insufficiency is highly likely (Mongioì et al., 2019).

Treatment

Oral glucocorticoid and mineralocorticoid replacement therapy is given in physiologic doses for life. I will prescribe a short-acting glucocorticoid such as Hydrocortisone 15-30 mg/day PO in 2 divided doses 8am and 4pm. I will provide education so they know when to increase replacement doses appropriately when febrile, vomiting or in preparation for elective surgical procedures, stress, trauma, infection and pregnancy (Huecker, 2021). Persistent fatigue is evident of insufficient dosage. Excessive weight gain or facial plethora is present of over-replacement (Huecker, 2021).

Preventive Care

Patient education is important in the prevention of adrenal crisis. A regular review by an endocrinologist, in the months following diagnosis and then six to 12 months thereafter, is recommended after a pertinent acute presentation with an adrenal crisis. Patients need to be encouraged to make relevant adjustments to their own medication, in the absence of their treating physician, and to adapt their dose according to individual needs (Dineen et al., 2019).

 

Reference:

Dineen, R., Thompson, C. J., & Sherlock, M. (2019). Adrenal crisis: Prevention and management in adult patients. Therapeutic Advances in Endocrinology and Metabolism, 10, 204201881984821. doi:10.1177/2042018819848218

Huecker, M. (2021, February 07). Adrenal insufficiency. Retrieved April 06, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK441832/

Mongioì, L. M., Condorelli, R. A., Barbagallo, F., Cannarella, R., La Vignera, S., & Calogero, A. E. (2019). Accuracy of the low-dose acth stimulation test for adrenal insufficiency diagnosis: A re-assessment of the cut-off value. Journal of Clinical Medicine, 8(6), 806. doi:10.3390/jcm8060806

Q-2

Hyperprolactinemia is a condition resulting from elevated serum prolactin that occurs from prolactinomas and from a pituitary adenoma. Transient mildly elevation from transient factors such as food, exercise, stress, and chest wall stimulation and moderate elevation can be caused by pregnancy, primary hypothyroidism, renal failure, cirrhosis, and medications such as metoclopramide, calcium-channel blockers, phenothiazines, SSRIs, and risperidone (Jain, & Katznelson, 2019). Any sellar tumors may compress the pituitary stalk, reduce the dopamine delivery to the gland leading to elevation of prolactin levels up to 150 ng/ml.

The majority of patients are asymptomatic due to concomitance of other diseases and some may develop hypogonadism symptoms such as galactorrhea, menstrual disorders, fertility problems, gynecomastia in men, and neuroradiological abnormalities (Vilar, Vilar, Lyra, & da Conceicao Freitas, 2019). Diagnosis by history to rule out other causes of pregnancy, drug-induced or secondary diseases such as hypothyroidism, and renal failure. Lab test for elevated prolactin levels, imaging study CT or MRI and differentiate from other pituitary tumors by gel filtration or polyethylene glycol precipitation (Kasum, Orešković, Čehić, Šunj, Lila, & Ejubović, 2017). Macroprolactinomas are a tumor size more than >10 mm in greatest diameter and produce prolactin levels of > 150 ng/ml (Jain, & Katznelson, 2019).

Consult to an endocrinologist is appropriate for the management of prolactinoma and for neurosurgery for surgical treatment and oncologist for radiotherapy for failed medical and surgical therapy and OB/GYN consultation if the condition coexists with pregnancy and lactation.

Treatment is by dopamine agonist such as bromocriptine started at 1.25 mg at bedtime and slowly titrated up to 2.5 mg twice a day and cabergoline is expensive than bromocriptine and better tolerated at a dose of 0.25 mg once weekly and titrated up to 1 mg twice a week (Jain, & Katznelson, 2019). Lab monitoring of prolactin levels and intermittent MRI is needed to monitor the effectiveness of therapy. Transsphenoidal surgery is indicated when the medical treatment fails or due to cystic tumors which do not respond to dopamine agonist. A radiotherapy is also an option who fails medical and surgical therapy. Management of postoperative complications such as anterior pituitary insufficiency and diabetes insipidus, sinusitis and perforation of nasal septum and CSF leakage, meningitis, intracranial hemorrhage, epistaxis, and visual deficits. Follow-up visits with endocrinology and neurosurgery. Education and counseling regarding monitoring of those complications and to report on time and have extra glucocorticoid tablets and medical identification alert for them.

Reference.

Jain, S.H., & Katznelson, L. (2019). Pituitary disorders. In S.C. McKean, J.J. Ros, D.D. Dressler & D.B. Scheurer. (2nd ed.), Principles and practice of hospital medicine, 2e. Vol.1 (PP.1248-1251). Retrieved from https://lccn.loc.gov/2016022668

Vilar, L., Vilar, C. F., Lyra, R., & da Conceicao Freitas, M. (2019). Pitfalls in the diagnostic evaluation of hyperprolactinemia. Neuroendocrinology, 109(1), 7-19. Retrieved from https://www.karger.com/Article/Abstract/499694

Kasum, M., Orešković, S., Čehić, E., Šunj, M., Lila, A., & Ejubović, E. (2017). Laboratory and clinical significance of macroprolactinemia in women with hyperprolactinemia. Taiwanese journal of obstetrics and gynecology, 56(6), 719-724. Retrieved from https://www.sciencedirect.com/science/article/pii/S1028455917302413

Q-3

Adrenal crisis is a life-threatening condition that is caused by low levels of cortisol that is produced by the adrenal gland (Elshimy, et al.,2020). Cortisol is essential to maintain glucose, suppress immune response in relation to the body response to stress. Generally, there is something that stresses the body to go into a crisis such as infection, dehydration, stress, etc. It generally occurs in patients with known adrenal insufficiency from primary or secondary causes. In stressful situations the patients cannot replace the used cortisol sending them into a crisis. Most are caused by undiagnosed Addison’s disease, followed by infiltrative diseases, iatrogenic causes, pituitary causes, and medications. Symptoms of Adrenal crisis are headache, weakness, fatigue, nausea vomiting, low blood pressure, confusion, electrolyte abnormalities, abdominal pain, and fever.

Patients present usually very sick and appear in shock and will need to get collateral information such as adrenal insufficiency issues. Initial symptoms are nausea/vomiting, fever, abdominal pain, hypotension, altered mental status. They may have hyperpigmentation of the skin, have Cushingoid symptoms from chronic steroid use, fevers, and could slip into a coma if cortisol levels are depleted (Rathbun, et al.,2020). Obtaining labs such as CMP, CBC, cortisol levels, ACTH, aldosterone, renin, and thyroid function. The electrolytes may show dehydration with hyponatremia, hyperkalemia, hypoglycemia, and acute renal injury. Treatment includes fluid replacement (2-3L of NS or 5% dextrose) glucocorticoid replacement (hydrocortisone 100mg bolus first followed by 100-300mg per day for another 2-3 days (Puar, et al., 2016). The rapid correction of hyponatremia should be avoided, and treatment of fluids should be titrated to urine output. Long term treatment includes hydrocortisone, prednisone, or methylprednisolone, or fludrocortisone acetate (if aldosterone is low), and making sure the patient gets enough salt in the diet.

 

Elshimy G, Alghoula F, Jeong JM. Adrenal Crisis. [Updated 2020 Nov 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499968/

 

Rathbun KM, Nguyen M, Singhal M. Addisonian Crisis. [Updated 2020 Dec 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441933/

 

Puar, T. H., Stikkelbroeck, N. M., Smans, L. C., Zelissen, P. M., & Hermus, A. R. (2016). Adrenal crisis: Still a deadly event in the 21st century. The American Journal of Medicine, 129(3). doi:10.1016/j.amjmed.2015.08.021