Wednesday, 13 November 2013

CHUSING'S SYNDROME




DEFINITION
  • Cushing's syndrome is a disorder caused by prolonged exposure of the body's tissues to high levels of the hormone cortisol.
  • Sometimes called " hypercortisolism " 
INCIDENCE
  • Occurs 10 times more in women 
  • Average age - 30 and 50 years old 
ETIOLOGY
  • Adrenal tumor ( responsible for approximately 30% of all cushing's syndrome cases ) 85% - benign, 15% - malignant.
  • Adrenal hyperplasia - caused by overproduction of ACTH. Two sources of excessive ACTH secretions are : 
a) Pituitary hypersection and pituitary tumors.
b) Ectopic secretion of ACTH - some benign or malignant tumors that arise outside the pituitary can produce ACTH. Lung tumors cause over 50% of these cases. Men are affected 3 times more than women. 

RISK FACTORS
  • Administration of exogenous steroids. 
          Example : Prednisolone 

PATHOPHYSIOLOGY

 Hypothalamus sends Corticotropin releasing hormone CRH > Pituitary Gland > secrete ACTH ( adrenocorticotropin ) > stimulates the adrenal glands to release cortisol into the bloodstream > exaggerated physiologic action of glucocorticoids 
           
         

                                                                                 
CLINICAL MANIFESTATION



   Persistent hyperglycemia 
- Protein tissue wasting resulting in :
  • Weakness due to muscle wasting 
  • Capillary fragility resulting in ecchymosis 
  • Osteoporosis due to bone matrix wasting 
- Potassium depletion leading to hypokalemia, arrythmias, muscle weakness and renal disorders.
- Sodium and water retention which causes edema and hypertension > CVA, left ventricular failure leading to CCF.           
- Abnormal fat distribution due to edema resulting in moon shaped face, dorsocervical fat pad on neck ( buffalo hump ) 
- Truncal obesity with slender limbs. 
- Pink and purple striae appears on breasts, axillary areas, abdomen, leg due to thinning of skin. 
- Increases susceptibility to infection and lowered resistance to stress increase chances of invasion of micro organism. 
- Poor wound healing due to suppression of inflammatory response.
- Virilism ( musculinization - acne, thinning of sclap, hirsutism, voice - musculine )
- Mental changes include memory loss, poor concentration, depression. 

DIAGNOSTIC TESTS
  • 24-Hour Urinary Free Cortisol Level ( Levels higher than 50 - 100 mcg/day for an adult suggest Cushing's syndrome ) 
  • Blood Test - cortisol level, ACTH, Potassium low
  • X-ray of the skull detect erosion of the sella turcica by pituitay tumor.
  • CT scan and ultrasonography detect location of tumors. 
TREATMENT
Surgical and Radiation
  • Surgery is performed via transsphenoidal hypophysectomy, craniotomy - if pituitary tumor has enlarged beyond sella turcica, adrenalectomy - hyperplasia of adrenals.
  • Radiation can be externally or internally.
  • Internal radiation can be applied through a transsphenoidal implant.
  • Radiation must be done with care as the optic nerve is nearby. 


TRANSSPHENOIDAL HYPOPHYSECTOMY 

MANAGMENT

Replacement therapy postoperatively 
Require a lifelong replacement therapy such as :
  • Glucocorticoid - cortison ( cortef )
  • Mineralcorticoid - fludrocortisone ( florinef ) 
MEDICAL TREATMENT
  • Interferes with the ACTH production or adrenal hormone synthesis. 
  • Example : Mitotane ( Lysodren ) - a cytotoxic antihormonal agent that inhibits corticosteroid synthesis without destroying corticol cells. 
NURSING DIAGNOSIS

1. Impaired skin integrity related to edema, impaired healing, and thin and fragile skin. 
Obj : Patient is able to maintain intact skin. 
Intervention
- Assess skin for early detection of trauma. 
Example : redness, excoriation, infection, edema
- Protect patient from bumping and bruising.
- Change patient's position every two hourly 
R : to prevent skin breakdown. 
- Do not use the adhesive tape. 
R : to avoid irritate the skin and tear the fragile tissue when the tape is removed. 
- Give meticulous skin care.
R : to avoid traumatizing the pateint's fragile skin.
- Serve low salt diet. 

2.  Self-care deficit related to weakness, fatigue, muscle wasting, altered sleep patterns. 
Obj : Patient is able to self care with minimal support. 
Intervention
- Assess patient ability in carrying out the daily activities. 
R : to help patient plan and space rest periods throughout the day to prevent complications of immobility.
- Assist when patient is weak. 
R : to encourage the moderate activity. 
- Encourage patient to rest.
R : to promote relaxing for rest and sleep.
- Increase the activity gradually. 
R : to promote increased self-esteem. 

3. Risk for infection related to altered protein metabolism and inflammatory response. 
Obj : Patient has no infection.
Intervention
- Assess for inadequate protein stores, proteinuria, muscle wasting and poor wound healing. 
- Monitor vital sign. 
R : to detect any sign and symptoms of infection.
- Assess potential site infection site eg: skin and urinary tract.
- Screen and limit visitor who may have infections
R : to protect patient from sources of infection.
- Teach patient and staff effective handwashing.
R : to limits patient's exposure to potential infection cause agents.
- Promote adequate rest and nutrition.
R : to promote rest and adequate nutrition limits fatigue and enhance immune system natural defence mechanism.
- Refer patient to dietitian. 
R : to teach patient selecting appropriate foods that are low in sodium and calories. 

SUMMARY CUSHING'S SYNDROME YOUTUBE

REFERENCES

                                                         

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