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Block 9 Board Review Part 2 Endocrine 28Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14.

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Presentation on theme: "Block 9 Board Review Part 2 Endocrine 28Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14."— Presentation transcript:

1 Block 9 Board Review Part 2 Endocrine 28Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

2 Why are we STILL reviewing ENDOCRINE???

3 3.5% of Initial Certifying Exam!!!

4 Pediatrics In Review Articles Congenital Adrenal Hyperplasia Addison Disease Turner’s Syndrome

5 Congenital Adrenal Hyperplasia

6 What are the signs and symptoms of CAH (adrenal crisis)??

7 Genital Ambiguity (severe form/females) Decreased activity/fatigue Altered sensorium/unresponsiveness Poor feeding/weak suck Dry mucus membranes Hyperpigmentation Abdominal pain Vomiting Hyponatremia Hyperkalemia Hypoglycemia Metabolic Acidosis Hypothermia Hypotension Dehydration Lack of Weight Gain

8 What is the lab evaluation for CAH?

9 CMP Dex ABG Cortisol ACTH 17OHP Pelvic Ultrasound Karyotype

10 Can CAH be diagnosed prenatally? How?

11 Yes Amniocentesis (14-18 wks) Chorionic Villous Sampling (10-12wks)

12 How do you treat adrenal crisis in a patient with CAH??

13 Fluid resuscitation (20ml/kg bolus 0.9% NaCl) Fluids at 1.5-2x maintenance Hydrocortisone (stress dose-100mg/m 2 ) Central Access Pressors (with higher glucose concentrations) HyperK?? Consider C BIGK

14 What is the value in screening for salt losing CAH in male infants with normal genitalia?

15 What is the value in neonatal screening for salt losing CAH in male infants with normal genitalia? Prevent adrenal crisis in cases of male infant that might not have otherwise been suspected

16 Maternal exposure to what can lead to virilization in female infants?

17 Androgens

18 Addison Disease

19 What are the signs and symptoms of Addison Disease?

20 Similar to adrenal crisis – Abdominal pain, nausea, vomiting, fatigue, muscle weakness, poor weight gain, loss of pubic and axillary hair …BUT – No elevated adrenal androgens – Response of Cortisol and its precursors to ACTH is blunted or absent

21 How can you use lab tests to diagnose Addison disease?

22 Electrolytes AM Cortisol Plasma Renin ACTH ACTH stim test

23 How do you treat adrenal crisis in a patient with Addison Disease?

24 Fluid resuscitation (20ml/kg bolus 0.9% NaCl) Fluids at 1.5-2x maintenance Hydrocortisone (stress dose-100mg/m 2 ) Central Access Pressors (with higher glucose concentrations) HyperK?? Consider C BIGK

25 What are the complications of sudden withdrawal of steroids in patients with adrenal insufficiency?

26 Addisonian crisis – sharp leg pain – Lower back or abdominal pain – nausea – Vomiting – hyponatremic dehydration – hyperkalemia – metabolic acidosis – hypotension – Hypoglycemia – Shock – sudden death

27 Turner’s Syndrome

28 What are the signs and symptoms of Gonadal Dysgenesis (Turner’s Syndrome)?

29 congenital lymphedema (swelling of the hands or feet) webbed neck Low hairline shield chest with inverted and widely spaced nipples Deformity of the ears nail dysplasia cubitus valgus short metacarpals micrognathia

30 What is the lab evaluation for gonadal dysgenesis?

31 Karyotype Serum LH Serum FSH Serum Estradiol

32 What is the importance of evaluating for renal and cardiac disorders in gonadal dysgenesis (Turner Syndrome)

33 What is the importance of evaluating for renal and cardiac disorders in gonadal dysgenesis (Turner Syndrome)? High incidence of: – Aortic valve defects (bicuspid Aortic valve) (20- 30%) – Coarctation of the Aorta (3-10%) – Horshoe Kidney – **Increased risk of aortic dissection, EKG abnormalities, and HTN – Initial Cards workup with 4pt BP, EKG, Echo, or Echo and MRI

34 PREP

35 A 4-year-old girl in your practice was noted to have clitoromegaly at birth and was diagnosed with congenital adrenal hyperplasia (CAH). She was diagnosed as having classic CAH caused by 21- hydroxylase deficiency and has been doing well on glucocorticoids and mineralocorticoids prescribed by her pediatric endocrinologist. Her mother is considering another pregnancy and wishes to know what she needs to be aware of in a future pregnancy. Of the following, you are MOST likely to tell her that

36 A. at birth, an affected female would be expected to have a similar degree of virilization as her sister B. at birth, an affected male is likely to have ambiguous genitalia C. at birth, the majority of affected males will not require glucocorticoids and mineralocorticoids D. prenatal administration of dexamethasone is important for both male and female fetuses E. prenatal diagnosis for CAH can be accomplished using molecular technologies through amniocentesis

37 A. at birth, an affected female would be expected to have a similar degree of virilization as her sister B. at birth, an affected male is likely to have ambiguous genitalia C. at birth, the majority of affected males will not require glucocorticoids and mineralocorticoids D. prenatal administration of dexamethasone is important for both male and female fetuses E. prenatal diagnosis for CAH can be accomplished using molecular technologies through amniocentesis

38 A mother of a patient in your practice reports that she is currently 8 weeks pregnant and just discontinued use of danazol, which she was taking for treatment of severe endometriosis. She has been told that use of this medication in the first trimester of pregnancy can be associated with birth defects and wishes to know what possible birth defects can be seen with use of this medication in pregnancy. Of the following, you are MOST likely to tell her that use of danazol in pregnancy is associated with an increased risk for

39 A. anterior abdominal wall defects B. congenital heart defects C. neural tube defects D. renal malformations E. virilization of a female fetus

40 A. anterior abdominal wall defects B. congenital heart defects C. neural tube defects D. renal malformations E. virilization of a female fetus

41 A 5-year-old girl with classic 21-hydroxylase deficiency (congenital adrenal hyperplasia) develops gastroenteritis with fever (up to 38.9°C), vomiting, and diarrhea. Her regular medications include hydrocortisone and fludrocortisone. Upon presentation to the emergency department, she is tired-appearing and remains febrile. Her pulse rate is 162 beats/min, blood pressure is 62/40 mm Hg, and capillary refill is poor. Laboratory tests drawn in the emergency department are still pending, but fingerstick glucose level is 42 mg/dL (2.3 mmol/L). The patient is treated with a bolus of normal saline to restore circulatory support. Of the following, the MOST important therapy to administer to this patient next is

42 A. cortisone acetate intramuscularly and aldosterone intravenously B. cortisone acetate intramuscularly and dextrose intravenously C. dopamine and dextrose intravenously D. hydrocortisone hemisuccinate and aldosterone intravenously E. hydrocortisone hemisuccinate and dextrose intravenously

43 A. cortisone acetate intramuscularly and aldosterone intravenously B. cortisone acetate intramuscularly and dextrose intravenously C. dopamine and dextrose intravenously D. hydrocortisone hemisuccinate and aldosterone intravenously E. hydrocortisone hemisuccinate and dextrose intravenously

44 During a regularly scheduled visit, the mother of a 10-year-old boy who has had type 1 diabetes mellitus for 5 years expresses concern about her son’s increased frequency of hypoglycemic episodes over the past 3 months. She also notes that he is increasingly tired and that his skin is getting tanner even though he has not had any sun exposure. Of the following, the MOST appropriate next step in this boy’s management is to order a

45 A. cortisol level drawn at 7 am B. cortisol level drawnat 4 pm C. dexamethasone (1 mg) suppression test D. high-dose (250 μg) corticotropin stimulation test E. low-dose (1 μg) corticotropin stimulation test

46 A. cortisol level drawn at 7 am B. cortisol level drawnat 4 pm C. dexamethasone (1 mg) suppression test D. high-dose (250 μg) corticotropin stimulation test E. low-dose (1 μg) corticotropin stimulation test

47 Quick Associations

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