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23. 1. Cushing Syndrome a) any factor that causes  cortisol (i.e., glucocorticoids) i) mainly due to administration of glucocorticoids (iatrogenic) ii) hypersecretion of ACTH iii) hyperplasia or neoplasia iv) ectopic secretion of ACTH www.freelivedoctor.com

26. c) known affects of glucocorticoids i) hypertension ii) weight gain (“moon face” “buffalo hump”) iii) atrophy of fast (type II) twitch myofibers  muscle atrophy and limb weakness iv) hyperglycemia and glucosuria v) proteolysis and bone resorption (osteoporosis) vi) suppresses immune system   risk for infections vii) hirsuitism viii) CNS  mood changes www.freelivedoctor.com

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31. d) adrenal causes of  androgens i) neoplasms (more likely to be carcinomas) ii) adrenal hyperplasia - group of autosomal recessive disorders causing  cortisol production with feedback  in ACTH with resultant adrenal hyperplasia (e.g., congenital) - the most common enzymatic defect in congenital adrenal hyperplasia is 21-hydroxylase deficiency which accounts for ~95% of cases. www.freelivedoctor.com

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35. d) 21-hydroxylase deficiency causes excessive androgenic activity i) masculinization in females - hirsutism - oligomenorrhea ii) in males, enlargement of external genitalia e) some rare forms of congenital adrenal hyperplasia i) 17-  -hydroxylase deficiency ii) in other forms of congenital adrenal hyperplasia (e.g., 11  -hydroxylase deficiency) www.freelivedoctor.com

36. - accumulated intermediates steroids have sodium retention properties and subsequent hypertension f) congenital adrenal hyperplasia should be suspected in neonate with ambiguous genitalia i) severe enzyme deficiency in infancy can be life threatening - vomiting - diarrhea - sodium loss (dehydration) ii) in all cases, an androgen producing neoplasm in the ovary must be R/O www.freelivedoctor.com

39. - Hashimoto - Pernicious anemia - Type I diabetes - idiopathic Hypoparathyroidism iv) Type I and II polyglandular syndrome - Type I : - autosomal recessive; mutations located on chromosome 21q - Type II : - strong link to histocompatibility antigens (HLA-B8, HLA- DR3 and HLA-DQ5) www.freelivedoctor.com

40. v) circulating antibodies to several steroidal enzymes (e.g., 21 and 17  -hydroxylase) have been found in all types of autoimmune adrenalitis b) Infections i) TB (accounted for ~90% of primary chronic adrenocortical insufficiency) antituberculosis drugs have  the incidence of Addisons disease ii) Fungi (Histoplasma capsulatum and coccidioides immitis) iii) AIDS, due to contracting a variety of infections www.freelivedoctor.com

41. c) Metastatic neoplasms i) common site of metastatic disseminated carcinomas ii) carcinomas of breast and lung are source of majority of metastases in the adrenals iii) other neoplasms from GI, melanomas and hematopoietic neoplasms may also metastasize to the adrenals www.freelivedoctor.com

43. d) Primary adrenal insufficiency i)  aldosterone   Na + loss;  K + retention; volume depletion  hypotension ii) heart smaller than normal (? Chronic hypovolemia) iii) hypoglycemia (via glucocorticoid deficiency) and impaired gluconeogenesis iv) acute stress in the patients (e.g., infections, surgical procedures, trauma) may initiate an “ acute adrenal crisis ” www.freelivedoctor.com

45. iii) DIC iv) overwhelming sepsis (i.e., Waterhouse-Friderichsen syndrome) - classically associated with Nisseria meningitides septicemia - also can be caused by pneumococci, Haemophilus influenza and pseudomonas sp. - pathogenesis unclear (may involve ETX induced vascular damage?) - DIC, shock, skin purpura - more common in children - massive adrenal clots www.freelivedoctor.com

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51. i) MEN2A and MEN2B ii) type I neurofibromatosis iii) Von Hippel-Lindau disease iv) Sturge-Weber syndrome c) Extra-adrenal source (~10%) i) carotid body ii) organ of Zuckerkandl d) are bilateral (~10%) i) may be as high as 50% in familial cases e) malignant (~10%) i) more common when arise in extra- adrenal sites www.freelivedoctor.com

52. f) clinical i) hypertension! - chronic, elevated BP (~70% of cases) ii) other hormones can be secreted - ACTH - somatostatin iii) dx   urinary excretion of catecholamines and metabolites - vanillylmandelic acid - metanephrines iv) isolated pheochromocytomas treated surgically - multifocal  medically treated www.freelivedoctor.com