Many patients with primary aldosteronism (PA) exhibit a decline in renal function after adrenalectomy. Excessive aldosterone secretion causes glomerular hyperfiltration, and cancellation of this excessive secretion manifests the masked renal dysfunction. Considering the mineralocorticoid effect of cortisol as with aldosterone, excessive cortisol secretion may also mask the renal dysfunction of patients with adrenal Cushing’s syndrome (CS). However, postoperative changes in renal function in patients with CS have not been evaluated. We evaluated changes in renal function after adrenalectomy in patients with functional adrenal tumor.
METHODS
A total of 164 consecutive patients underwent adrenalectomy for unilateral functional adrenal tumor at our institution between January 2004 and October 2014. Of the 164 patients, we retrospectively analyzed 118 patients (PA/CS/subclinical Cushing’s syndrome [SCS]/pheochromocytoma [PCC]: n = 51/21/13/33, respectively) who were followed up for over 6 months and whose change in renal function was evaluable. Laboratory data, including baseline hormone levels before medical treatment, were collected from medical records. Estimated glomerular filtration rate (eGFR) values were obtained using the 3-variable Japanese equation. Renal dysfunction was defined as a 20% reduction in eGFR from baseline to within 3 times average during the 6 months post-operation. The change in eGFR before and after the operation was analyzed in each adrenal tumor.
RESULTS
The mean change in eGFR before and after the operation was -14% (range, -55%-18%) in patients with PA, -9% (-48%-23%) in patients with CS, -2% in patients with SCS, and -2% in patients with PCC. The eGFR decline in patients with PA and CS was significant (p < 0.0001 and p = 0.0171, respectively), while there was no significant change in patients with SCS and PCC. After the operation, 39% (20/51) and 24% (5/21) of patients with PA and CS manifested renal dysfunction, respectively, while none of the patients with SCS and PCC manifested renal dysfunction. Preoperative renal function was not correlated with manifested renal dysfunction. Multivariate analysis identified older age and higher levels of plasma aldosterone concentration as independent predictors of renal dysfunction manifestation in patients with PA, while no clinical predictor was identified in patients with CS.
CONCLUSIONS
This is the first report that has shown a decline in eGFR after adrenalectomy in patients with CS. Possible masked renal damage should be considered in patients with CS as well as PA.
R Correa, M Zilbermint, A Demidowich, F Faucz, A Berthon, J Bertherat, M Lodish, C Stratakis
Summary: Researchers conducted this study to describe the different phenotypical characteristics of patients with armadillo repeat containing 5 (ARMC5) mutations, located in 16p11.2 and a likely tumor-suppressor gene. They determined that patients with bilateral adrenal enlargement, found on imaging tests, should be screened for ARMC5 mutations, which are associated with subclinical Cushing’s syndrome (CS) and primary hyperaldosteronism (PA).
Methods:
Researchers identified 20 patients with ARMC5 mutations (germline and/or somatic) who were enrolled in a National Institutes of Health (NIH) protocol.
They obtained sociodemographic, clinical, laboratory, and radiological data for all participants.
Results:
Three families (with a total of 8 patients) were identified with ARMC5 germline mutations; the rest of the patients (13/20) had sporadic mutations.
The male to female ratio was 1.2:1; mean age was 48 years and 60% of patients were African American.
Forty percent of patients were diagnosed with CS, 20% with subclinical CS, 30% with hyperaldosteronism, and 10% had no diagnosis.
The mean serum cortisol (8 am) and Urinary Free Cortisol were 13.1 mcg/dl and 77 mcg/24 hours, respectively.
Nearly all patients (95%) had bilateral adrenal enlargement found on CT or MRI.
Patients underwent the following treatments: Bilateral adrenalectomy (45%), unilateral adrenalectomy (25%), medical treatment (20%), and no treatment (10%).
ARMC5 mutations are associated with primary macronodular adrenal hyperplasia (PMAH) and are also seen in patients with PA, especially among African Americans.
Cushing’s and Addison’s Disease. An endocrine NCLEX review on how to differentiate between Cushing’s Syndrome/Disease vs Addison’s and Addisonian Crisis. In this video, I will discuss the pathophysiology, signs & symptoms, and nursing interventions for these endocrine disorders of the adrenal cortex and pituitary glands.
Addison’s Disease and Cushing’s Syndrome/Disease review notes for nursing school and NCLEX exam. In nursing school and for the NCLEX exam, you will need to know how to provide care to a patient with either Addison’s Disease or Cushing’s.
However, many students get these two endocrine disorders confused, but these review notes will help you differentiate between them.
These NCLEX review notes will cover:
Signs and Symptoms of Addison’s Disease vs Cushing’s
Causes of Addison’s Disease and Cushing’s
Nursing Management of Addison’s Disease and Cushing’s
Role of Adrenal Cortex: releases steroid hormones and sex hormones
Role of Aldosterone: regulates blood pressure through renin-angiotensin-aldosterone system, helps retain sodium and secretes potassium (balances sodium and potassium levels).
Role of Cortisol: “STRESS Hormone” helps the body deal with stress such as illness or injury, increases blood glucose though glucose metabolism, break downs fats, proteins, and carbs, regulates electrolytes.
Cushing’s (Syndrome & Disease)
Cushing’s: hyper-secretion of CORTISOL (watch video for clever ways to remember this)
Cushing’s Syndrome vs Cushing’s Disease
Cushing’s Syndrome: caused by an outside cause or medical treatment such as glucocorticoid therapy
Cushing’s Disease: caused from an inside source due to the pituitary gland producing too much ACTH (Adrenocorticotropic hormone) which causes the adrenal cortex to release too much cortisol.
Signs & Symptoms of Cushing’s
Remember the mnemonic: “STRESSED” (remember there is too much of the STRESS hormone CORTISOL)
Skin fragile
Truncal obesity with small arms
Rounded face (appears like moon), Reproductive issues amennorhea and ED in male(due to adrenal cortex’s role in secreting sex hormones)
Ecchymosis, Elevated blood pressure
Striae on the extremities and abdomen (Purplish)
Sugar extremely high (hyperglycemia)
Excessive body hair especially in women…and Hirsutism (women starting to have male characteristics), Electrolytes imbalance: hypokalemia
Dorsocervical fat pad (Buffalo hump), Depression
Causes of Cushing’s
Glucocorticoid drug therapy ex: Prednisone
Body causing it: due to tumors and cancer on the *pituitary glands or adrenal cortex, or genetic predisposition
Nursing Management for Cushing’s Syndrome
Prep patient for Hypophysectomy to remove the pituitary tumor
Prep patient for Adrenalectomy:
If this is done educate pt about cortisol replacement therapy after surgery
Risk for infection and skin breakdown
Monitor electrolytes blood sugar, potassium, sodium, and calcium levels
Addison’s Disease
Addison’s: Hyposecretion of Aldosterone & Cortisol (watch the video for a clever way on how to remember this and not get it confused with Cushing’s)
Signs & Symptoms of Addison’s Disease
Remember the phrase: “Low STEROID Hormones” (remember you have low production of aldosterone & cortisol which are STEROID hormones)
Sodium & Sugar low (due to low levels of cortisol which is responsible for retention sodium and increases blood glucose), Salt cravings
Tired and muscle weakness
Electrolyte imbalance of high Potassium and high Calcium
Reproductive changes…irregular menstrual cycle and ED in men
lOw blood pressure (at risk for vascular collapse)….aldosterone plays a role in regulating BP
Increased pigmentation of the skin (hyperpigmentation of the skin)
Diarrhea and nausea, Depression
Causes of Addison’s Disease
Autoimmune due to the adrenal cortex becoming damaged due to the body attacking itself:
Tuberculosis/infections
Cancer
Hemorrhaging of the adrenal cortex due to a trauma
Nursing Management of Addison’s Disease
Watching glucose and K+ level
Administer medications to replace the low hormone levels of cortisol and aldosterone
For replacing cortisol:
ex: Prednisone, Hydrocortisone
Education: Patient needs to report if they are having stress such as illness, surgery, or extra stress in life ( will need to increase dosage), take medication exactly as prescribed….don’t stop abruptly without consulting with MD.
For replacing aldosterone:
ex: Fludrocortisone aka Florinef
Education: consume enough salt..may need extra salt
Wearing a medical alert bracelet
Eat diet high in proteins and carbs, and make sure to consume enough sodium
Avoid illnesses, stress, strenuous exercise
Watch for Addisonian Crisis
This develops when Addison’s Disease isn’t treated.
In addisonian crisis, the patient has extremely LOW CORTISOL levels (life threatening).
Remember the 5 S’s
Sudden pain in stomach, back, and legs
Syncope (going unconscious)
Shock
Super low blood pressure
Severe vomiting, diarrhea and headache
NEED IV Cortisol STAT:
Solu-Cortef and IV fluids (D5NS to keep blood sugar and sodium levels good and fluid status)
Watch for risk for infection, neuro status (confusion, agitation), electrolyte levels (sodium and potassium, glucose)
Adrenal insufficiency, a rare disorder which is characterized by the inadequate production or absence of adrenal hormones, may be classified as primary adrenal insufficiency in case of direct affection of the adrenal glands or secondary adrenal insufficiency, which is mostly due to pituitary or hypothalamic disease.
Primary adrenal insufficiency affects 11 of 100,000 individuals. Clinical symptoms are mainly nonspecific and include fatigue, weight loss, and hypotension. The diagnostic test of choice is dynamic testing with synthetic ACTH.
Patients suffering from chronic adrenal insufficiency require lifelong hormone supplementation. Education in dose adaption during physical and mental stress or emergency situations is essential to prevent life-threatening adrenal crises.
Patients with adrenal insufficiency should carry an emergency card and emergency kit with them.
Dear Dr. Roach: I had apoplexy, a ruptured pituitary tumor, developed panhypopituitarism, then adrenal insufficiency. I am doing fairly well with cortisol replacement, thyroid supplement and oral diabetic medicine.
My problem is exhaustion that comes on very easily. I have other ailments to blame, too — chronic pain from fibromyalgia and tendinitis. I am 67. I am still able to work. Is adrenal fatigue a real issue, and if so, what can be done about it? — S.M.
Answer: The term “adrenal fatigue” is increasingly used, and not always correctly — or, at least, it is used in cases where it’s not clear if that is actually the case. But let me start by discussing what has happened to you. Pituitary apoplexy is bleeding into the pituitary gland, usually into a pituitary tumor, as in your case. This may cause severe headaches and vision changes, and often it prevents the pituitary from making the many important hormones that control the endocrine glands and regulate the body.
For example, without TSH from the pituitary gland, the thyroid won’t release thyroid hormone, and importantly, the adrenal gland can’t make cortisol without the influence of ACTH from the pituitary.
Rather than trying to replace TSH, ACTH and the other pituitary hormones, it is easier to directly replace the hormones made by the adrenal, thyroid and gonads. That’s why you are taking cortisol and thyroid hormone, and why younger women take estrogen and men testosterone. Although there is nothing wrong with your thyroid and adrenal glands, they simply won’t work unless stimulated.
Inadequate adrenal function from any cause leads to profound fatigue, and in the presence of severe stress, such as surgery or major infection, the body’s need for cortisol increases dramatically. Unless enough adrenal hormone is given in response, the result can be an immediate life-threatening condition called an Addisonian crisis.
Readers may email questions to ToYourGoodHealth@med.cornell.edu.
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