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BY ADDISU TEFERA( BSC STUDENT) MEDICAL _SURGICAL NURSING ENDOCRINE DISORDER Cushing’s Syndrome Addison's disease Diabetes Insipidus

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BY ADDISU TEFERA( BSC STUDENT)

MEDICAL _SURGICAL NURSINGENDOCRINE DISORDER Cushing’s Syndrome Addison's disease Diabetes Insipidus

IntroductionCushing’s syndrome (hypercortisolism) is a hormonal disorder caused by prolonged exposure high levels of steroid hormones called glucocorticoids.Exogenous Cushing’s syndrome: caused by taking excessive amounts of medications e.g. prednisone, dexamethasone for chronic asthma, rheumatoid arthritis, lupus, to suppress immune system after transplant to prevent rejection, other inflammatory diseases.Endogenous Cushing’s syndrome: excess cortisol produced by the adrenal glands. This is far rarer, but if left undiagnosed & untreated it can result in a shorter lifespan.

Cushing’s Syndrome

1932: Harvey Cushing described a series of seven pts with basophilic adenomas of the pituitary .Cushing’s disease Cushing’s syndrome when caused by a tumor of the pituitary gland.Cushing’s syndrome Caused by overproduction of cortisol for any reason. Cushing’s syndrome is a result of excessive circulating free corticosteroids, caused by unnecessary adrenocortical activity.

Cushing’s syndrome

Cushing’s syndrome is a rare condition that occurs when there is excess cortisol in the body. Cortisol is a hormone normally made by the adrenal glands and is necessary for life. It allows us to respond to stressful situations such as illness or injury, and has effects on almost all body tissues. It is produced in varying amounts over the course of the day, most in the early morning, with very little at night.Cushing’s syndrome refers to the condition caused by excesscortisol in the body, regardless of the cause. When Cushing’ssyndrome is caused by a pituitary tumor, it is called Cushing’sdisease.Cushing’s syndrome is more often found in women than in men and often occurs between the ages of 20 and 40.

Disease vs. Syndrome

Cushings Disease is the result of a pituitary tumour secreting inappropriate ACTH.Cushings Syndrome causes the same symptoms, but is caused by overproduction of adrenal hormones. It encompasses all other forms of Cushings.Basically:Disease = increased cortisol from pituitarySyndrome = all other sources

For example,Cushing’s Syndrome

• Hypercortisolism of any causeCushing’s Disease

• Corticotropin (ACTH) secreting pituitary adenoma

Cushing Syndrome

• Cushing Syndrome refers to disorder causinghypersecretion of adrenal corticoid hormones fromwhatever cause. Due to an Increased glucocorticoid exposure.• Cushing Syndrome if untreated produces seriousmorbidity, or death.There are 4 causes• Pituitary hypersecretion of ACTH from a pituitarytumor (classic “Cushing Disease”)• Adrenal hypersecretion of corticoid hormonesdue to an adrenal tumor (benign or malignant)• Ectopic Cushing Syndrome from paraneoplasticsyndrome (secretion of ACTH from tumors e.g. small celllung carcinoma).• Iatrogenic Cushing Syndrome from long termglucocorticoid therapy.

What causes Cushing’s syndrome and Cushing’s disease?oCushing’s syndrome can be caused by cortisol-likemedications (called glucocorticoids) or by a tumor. Sometimes, there is a tumor of the adrenal gland(s) that makes too much cortisol. Cushing’s syndrome may also be caused by a tumor in the pituitary gland (a small gland under the brain that produces hormones that in turn regulate the body’s other hormone glands).oSome pituitary tumors produce a hormone called adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands and causes them to make too much cortisol. This is termed Cushing’s disease. ACTH-producing tumors can also originate elsewhere in the body and these are referred to as ectopic tumors.

Causes:

Most commonly caused by prolonged use of corticosteroid medications for chronic illnesses such as asthma or rheumatoid arthritis.Pituitary AdenomasPituitary adenomas cause 70 % of endogenous Cushing’s syndrome cases. Benign tumours of the pituitary gland secrete extra ACTH. This form of the syndrome (Cushing’s disease), affects women five times more often than men.Ectopic ACTH SyndromeEctopic ACTH syndrome: benign/ cancerous tumours producing ACTH outside of the pituitary. Lung tumors cause more than half of these cases, and men are affected three times more often than women. Most common forms of ACTH-producing tumours: small cell lung cancer, (13% of all lung cancer cases)2 and carcinoid tumours (small, slow-growing tumours that arise from hormone-producing cells in various parts of the body). Other less common types of tumors that can produce ACTH: thymomas, pancreatic islet cell tumours, and medullary carcinomas of the thyroid.

Causes:

Adrenal TumorsAn abnormality of the adrenal glands- usually the presence of a tumour.Adrenal tumors are four to five times more common in women than men. Average age of onset is about 40 yrs. Most of these cases involve noncancerous tumours of adrenal tissue adrenal adenomas, which release excess cortisol into the blood.Adrenocortical carcinomas—adrenal cancers—are the least common cause of Cushing’s syndrome. With adrenocortical carcinomas, cancer cells secrete excess levels of several adrenocortical hormones, including cortisol and adrenal androgens. Usually cause very high hormone levels and rapid development of symptoms.Familial Cushing’s SyndromeRarely, Cushing’s syndrome results from an inherited tendency to develop tumours in one or more endocrine glands. e.g.With primary pigmented micronodular adrenal disease, children or young adults develop small cortisol-producing tumors of the adrenal glands. With multiple endocrine neoplasia type 1 (MEN1), hormone-secreting tumors of the parathyroid glands, pancreas, and pituitary develop.

In general it has the following causes

Endogenous Causes:65% = pituitary 25% = adrenals10% = ectopic source (small cell lung ca), non-pituitary, ACTH producing tumourExogenous Causes:Iatrogenic Steroids (Asthma, RA, palliative)Higher incidence in people with: DM, HTN, Obesity and Osteoporosis

Notes:causes of Cushing syndromeThe use of corticosteroid medication. Excessive secretion of adrenocorticotropic hormone (ATCH). Primary hyperplasia. Ectopic production of ACTH.

Symptoms Cardiovascular◦ Hypertension and heart failure. Endocrine and metabolic◦ Truncal obesity.◦ Moon face.◦ Buffalo hump.◦ Sodium retention.◦ Metabolic alkalosis.◦ Hyperglycemia. Gastrointestinal◦ Peptic ulcers and pancreatitis.

Symptoms cont.

Muscular◦ Myopathy and muscle weakness. Dermatologic◦ Thinning of skin.◦ Ecchymoses.◦ Striae. Psychiatric:◦ Mood alterations and psychoses. Immune functions◦ Impaired wound healing.◦ Increased susceptibility to infections.◦ Decreased inflammatory response

Signs & SymptomsWomen with Cushing syndrome often have:Excess hair growth on the face, neck, chest, abdomen, and thighs Menstrual cycle becomes irregular or stops Men may have:Decreased fertility Decreased or no desire for sex Impotence Other symptoms that may occur with this disease:Mental changes, such as depression, anxiety, or changes in behavior Fatigue Headache High blood pressure Increased thirst and urination

Cushing’s SyndromeClinical features

General Central obesity Proximal muscle weakness HTN Headaches

Dermatologic Wide purple striae Spontaneous ecchymoses Facial plethora Hyperpigmentation Acne, hirsutism Fungal skin infections

..

Cushing’s SyndromeClinical features continued

Neuropsychiatric Insomnia Depression, frank psychosis Impaired cognition and short-term memory

Endocrine/Metabolic Hypokalemic alkalosis Hypokalemia Osteopenia Hypogonadism Glucose intolerance Hyperlipidemia Hyperhomocysteinemia Kidney stones Polyuria Hypercoagulability

S&S

SWEDISH S – Spinal tenderness W – Weight gain E – Easily bruise D – Diabetes I – Intercapsular fat pad S – Striae H – HTN

Cushing Syndrome - Manifestations- 1

Manifestations in Cushing Syndrome aremainly due to excess glucocorticoids with exaggeration of cortisol actions (major glucocorticoid) & include: “Buffalo hump”, moon face & protruding abdomen from redistribution of body fat Muscle weakness & thin extremities from proteinbreakdown with muscle wasting. Thin “parchment skin” in arms, legs from protein & fat breakdown Purple striae (stretch marks) on breasts, thighs, abdomen from protein & fat breakdown. Osteoporosis & weak bones from protein breakdown& Ca mobilisation.

Cushing Syndrome - Manifestations - 2

• Disturbed glucose metabolism with diabetes mellitus occuring in 20% of cases.• Hypokalemia, Na retention & hypertension all from mineralocorticoid properties of glucocorticoids.• Increased infections & diminished inflammatory & immune responses.• Gastric ulceration & bleeding (cortisol stimulates gastric secretions)• Hirsutism (body hair), acne & menstrual irregularities in females due to increased levels of androgens.• Emotional lability (mild euphoria to psychoticbehaviour).

Cushing Syndrome - Manifestations

COMMON FEATURES LESS COMMON FEATURES

Weight gain Insomnia

Hypertension Recurrent infection

Poor short-term memory Thin skin and stretch marks

Irritability Easy bruising

Excess hair growth (women)

Depression

Red, ruddy face Weak bones

Extra fat around neck Acne

Round face Balding (women)

Fatigue Hip and shoulder weakness

Menstrual irregularity Swelling of feet/legs

Poor concentration Diabetes

Diagnostic Findings

Urinalysis. Dexamethasone suppression test. Serum cortisol testing. Corticotropin testing.Blood chemistry analysis. Salivary free cortisol analysis

DiagnosisDiagnosis is based on a review of a person’s medical history, a physical examination, and laboratory tests. X rays of the adrenal or pituitary glands can be useful in locating tumours.

Tests to Diagnose Cushing’s Syndrome24-hour urinary free cortisol level: Patient’s urine is collected several times over a 24-hour period and tested for cortisol. Levels higher than 50 to 100 micrograms a day for an adult suggest Cushing’s syndrome. Midnight plasma cortisol and late-night salivary cortisol measurements: The midnight plasma cortisol test also measures cortisol concentrations in the blood. Cortisol production is normally suppressed at night, but in Cushing’s syndrome, this suppression doesn’t occur. If the cortisol level is more than 50 nanomoles per litre (nmol/L), Cushing’s syndrome is suspected. The test generally requires a 48-hour hospital stay to avoid falsely elevated cortisol levels due to stress.

Continued…Low-dose dexamethasone suppression test (LDDST): Patient is given a low dose of dexamethasone (a synthetic glucocorticoid) orally every 6 hours for 2 days. Urine is collected before dexamethasone is administered and several times on each day of the test.Cortisol and other glucocorticoids signal the pituitary to release less ACTH, so the normal response after taking dexamethasone is a drop in blood and urine cortisol levels. If cortisol levels do not drop, Cushing’s syndrome is suspected.NB:The LDDST may not show a drop in cortisol levels in people with depression, alcoholism, high estrogen levels, acute illness, or stress, falsely indicating Cushing’s syndrome. On the other hand, drugs such as phenytoin and phenobarbital may cause cortisol levels to drop, falsely indicating that Cushing’s is not present in people who actually have the syndrome. For this reason, physicians usually advise their patients to stop taking these drugs at least 1 week before the test.

continuedDexamethasone- corticotropin-releasing hormone (CRH) test: Some people have high cortisol levels but do not develop the progressive effects of Cushing’s syndrome (e.g. muscle weakness, fractures, thinning of the skin). These people may have pseudo-Cushing’s syndrome, a condition sometimes found in people who have depression or anxiety disorders, drink excess alcohol, have poorly controlled diabetes, or are severely obese. Pseudo-Cushing’s does not have the same long-term effects on health as Cushing’s syndrome and does not require treatment directed at the endocrine glands.The dexamethasone-CRH test rapidly distinguishes pseudo-Cushing’s from mild cases of Cushing’s. This test combines the LDDST and a CRH stimulation test. In the CRH stimulation test, an injection of CRH causes the pituitary to secrete ACTH. Pre treatment with dexamethasone prevents CRH from causing an increase in cortisol in people with pseudo-Cushing’s. Elevations of cortisol during this test suggest Cushing’s syndrome.

How is Cushing’s syndrome diagnosed Because not all people with Cushing’s syndrome have all signs and symptoms, and because many of the features of Cushing’s syndrome, such as weight gain and high blood pressure, are common in the general population, it can be difficult to make the diagnosis of Cushing’s syndrome based on the symptoms alone. As a result, doctors use laboratory tests to help diagnose Cushing’s syndrome and, if that diagnosis is made, go on to determine whether it is caused by Cushing’s disease (i.e., from a pituitary tumor). These first diagnostictests determine if too much cortisol is being made spontaneously or if the normal control of hormones isn’t working properly.The most commonly used tests measure the amount of cortisol in the saliva or urine. It is also possible to check whether there is over-production of cortisol by giving a medication called dexamethasone that mimics cortisol. This is called a dexamethasone suppression test. If the body is regulating cortisol correctly, the cortisol levels will decrease, but this will not happen in someone with Cushing’s syndrome.These tests are not always able to definitively diagnose Cushing’s syndrome because other illnesses or problems can cause excess cortisol or abnormal control of cortisol production. These conditions that mimic Cushing’s syndrome are called ‘pseudo-Cushing’s states.

Diagnosis involves• determination of 24 hr cortisol levels in blood, saliva,urine to determine any late night cortisol increase.• Plasma ACTH levels to determine cause of disorder(ACTH increased in ectopic source & in Cushing Disease).

Prognosis

If untreated, Cushing’s syndrome can be life-threatening.If the cause can be treated, whether it means weaning the patient off cortisteroids, or surgically removing a tumour, the patient will fully recover. ComplicationsComplications of untreated Cushing's syndrome include:Diabetes Enlargement of any tumours that are the underlying cause. Fractures due to osteoporosis High blood pressure Kidney stones Serious infections

What are the treatment options for Cushing’s disease?The best way to cure the tumor is by surgically removing it, but there are other ways to control the tumor and effects of excess cortisol. In addition, there are other complementary approaches that may be used to treat some of the symptoms. For example, diabetes, depression and high blood pressure will be treated with the usual medicines used for these conditions. Also, doctors may prescribe calcium or vitamin D supplements, or other medicine to prevent thinning of the bone. Removal of the pituitary tumor by surgery is recommended for those who have a tumor that is not extending into areas outside of the pituitary gland, and who are well enough to have anesthesia. This approach, known as transsphenoidal surgery, is usually carried out by going through the nose or underneath the upper lip and then through the sphenoid sinus to reach the tumor recovery.

TreatmentCushing’s syndrome caused by corticosteroid use:Slowly decrease the drug dose (if possible) under medical supervision. If you cannot stop taking the medication because of disease, high blood sugar, high cholesterol levels, and bone thinning or osteoporosis should be closely monitored. Cushing’s syndrome caused by a pituitary tumour or tumour that releases ACTH:Surgery to remove the tumour Radiation after removal of a pituitary tumour in some cases (curative in 50-100% of patients with Cushing’s disease) You may need hydrocortisone (cortisol) replacement therapy after surgery, and possibly continued throughout your life Cushing’s syndrome due to an adrenal tumor or other tumors:Surgery to remove the tumour, followed by radiotherapy/ chemotherapy if requiredIf the tumour cannot be removed, medications to help block the release of cortisol

In short the treatment is..• Cushing’s disease:- trans-s phenoidal surgery• Cushing’s Syndrome -- resent ectopic site of ACTH secretion- adrenalectomy (+ steroid replacement)- anti-glucocorticoids (e.g. RU486) +anti-androgens (e.g. flutamide)

Surgical Treatment

Pituitary tumours: trans- sphenoidal microsurgery. Radiation therapy may

be used as an adjunct for patients who are not cured. Bilateral adrenalectomy may be necessary to control toxic cortisol levels.

Adrenocortical tumours: require surgical removal – can develop Nelsons

Syndrome Removal of neoplastic tissue is indicated for ectopic ACTH production. Metastatic spread makes a surgical cure unlikely or impossible. Bilateral adrenalectomy is indicated if necessary to control toxic cortisol levels. PreventionPatients should be weaned off corticosteroid medication for their conditions as soon as possible.Awareness early detection.

Assessment

Diabetes mellitus. Muscle weakness and loss of muscle mass. Hypertension. Redistribution of fat. Poor wound healing. Emotional lability. Insomnia.

Hallmark signs of Cushing’s Syndrome:Rounded “moon face”Fatty “Buffalo Hump” between shouldersTruncal obesityHirsutism (excess facial hair)Acne, PetechiaeSkin becomes susceptible to trauma, infection,bruising, edemaWounds are slow to healOsteoporosisHigh Blood Pressure

.

Assessment cont ..

Nursing Diagnoses

•Risk for infection related to altered protein metabolism andinflammatory response•Risk for injury related to weakness•Self-care deficit related to weakness, fatigue, and muscle wasting•Disturbed body image related to changes in physical appearanceand decreased activity•Impaired skin integrity related to thin and fragile skin and impairedhealing•Disturbed thought process related to mood swings, irritability anddepression

Diagnosis Actions/Interventions Risk for injury related to: Decreased bone density Increased capillary fragility Poor wound healingAction/interventions Increase calcium, vitamin D and protein in diet Assess skin for signs of bruising, breakdown, wounds not healing,changes in height Instruct patient about safety measures to reduce risk for falls and

Medical Management

Surgery for tumors of the adrenal and pituitary glands. Radiation therapy. Drug therapy.

Evaluation

1. Decreases risk of injury2. Decreases risk of infection3. Increases participation in self-careactivities4. Attains/maintains skin integrity5. Achieves improved body image6. Exhibits improved mental functioning7. Exhibits absence of complications