Acute adrenal failure/adrenal crisis

describe CAH:

AR disease deficiency of 21-hydroxylase 90-95% deficiency of 11beta-hydroxylase: 5% the cortisol deficiency that comes from the lack of final end product stimulates the pituitary to produce ACTH. 80% with deficiency of aldosterone develops salt loss (present with crisis at day 7) an accumulation of the adrenocortical steroids proximal to the enzyme block (testosterone like activity) leads to virilization in affected females. when the 21-hydr.does not function, pathways turn to the right and the end product will be androgens.

etiology of acute adrenal failure/crisis?

acute: waterhouse friedrichsen syndrome tb of adrenals dissemintated fungal infection (histoplasmosis) drugs: ketoconazole,metyparone Acute exacerbation of chronic insufficiency: addison disease: so called addisonian crisis: addison disease may exacerbate by trauma,illness or surgery. may also occur in patients who failed to ↑dose with infections. -hypopituitarism

imaging?

chest xray : may be signs of tb infection abdominal x ray: adrenal calcification

description

is a severe life threathening condition it is the result of adrenocorticoid insufficiency (cortisol mainly but also aldosterone) may be the result of an acute event or acute exceberation of a chronic disease.

treatment of adrenal crisis?

may be required before diagnosis o2,ecg monitoring,urinary catheter placement treat shock: IV normal saline or colloid for hypotension give dextrose 50% iv (50ml)if hypoglycemic 8mg of dexmethasone or hydrocortisone once hydrocortisone levels ahve stabilised administer fluodrocortisone

clinical feautes of adrenal crisis

patient is hypotensive and in shock→tachycardia fainting: postural hypotension may appear dehydrated may be symptoms/signs of causative agents;fever,night swaeats,flank pain gi: anorexia,N/V,abdominal pain,diarrhea weight loss,myalgia,fatigue,weakness. confusion

therapy of CAH

replacement hydrocortisone along with fudrocortisone to supress ACTH and surgical correction in females. replacement with electrolyts if there is salt loss lifelongsteroid replacement;needs to be ↑during illness or times of stress monitor growth,blood levels (↑glucocorticoids leads to skeletal decay)

clinical symptoms of cah?

salt loss infants present in the 2nd week of life in crisis:vomiting,↓wight,circulatory collapse due to the steroids with testosterone like activity. newborn females: virilization of external genitalia,and the penis in male is enlarged later features: tall stature,odour,pubic hair,acne,clitroral hypertrophy, variable fusion of labia

investigations in an adrenal crisis?

this is a medical emergency,so avoid long procedures. lab: urea and electrolytes: -hyponatremia and hyperkalemia -urea:creatinine ratio is high FBC: normocytic anemia,neutropenia,lymphocytosis and eosinophilia hypoglycemia hypercalcemia metabolic acidosis and respiratory fialure

diagnosis of CAH

↑17-alpha hydroxyprogesterone ↑urinary metabolites in salt loss:↓ Na,↑K+,metabolic acidosis,hypoglycemia