1. Block 9 Board Review Endocrine/Rheum 14Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

2. Pediatrics In Review Articles Type 1 DM Hypothyroidism in Children Kawasaki Disease Index of Suspicion

3. Quiz(zes)!!!

4. Type 1 DM

5. 1. You see a 12-year-old boy with a complaint of frequent urination. His mother is concerned because her father has had type 2 diabetes mellitus for several years. Urinalysis in the office shows the presence of glucose. You suspect that the boy may have type 1 diabetes mellitus because he also has A. Acanthosis nigricans. B. An elevated body mass index. C. Hispanic heritage. D. Hypertension. E. Weight loss.

6. 1. You see a 12-year-old boy with a complaint of frequent urination. His mother is concerned because her father has had type 2 diabetes mellitus for several years. Urinalysis in the office shows the presence of glucose. You suspect that the boy may have type 1 diabetes mellitus because he also has A. Acanthosis nigricans. B. An elevated body mass index. C. Hispanic heritage. D. Hypertension. E. Weight loss.

7. 2. A 6-year-old girl is admitted for new-onset hyperglycemia (ie, a fasting blood glucose level greater than 250 mg/dL). She has had symptoms for approximately 2 weeks and looks ill. The most appropriate of the following serum levels to measure first is A. Autoantibodies. B. Electrolytes. C. Insulin. D. lipids. E. Transaminases.

8. 2. A 6-year-old girl is admitted for new-onset hyperglycemia (ie, a fasting blood glucose level greater than 250 mg/dL). She has had symptoms for approximately 2 weeks and looks ill. The most appropriate of the following serum levels to measure first is A. Autoantibodies. B. Electrolytes. C. Insulin. D. lipids. E. Transaminases.

9. 3. You are reviewing dosage and management of insulin with a 9-year- old girl who weighs 35 kg and has had type 1 diabetes mellitus for several years. You calculate her total daily dose of insulin; she states she is receiving 25 units per day. The following statement that is true and relevant to her dosage is that A. After diagnosis of type 1 diabetes mellitus, the “honeymoon phase” lasts approximately 24 months. B. Approximately 50% of the total daily insulin prescribed is long acting, with the other 50% being short acting. C. The “mixed-split” regimen of insulin dosing allows for greater flexibility in diet than the “basal–bolus” regimen. D. Prepubertal children tend to require a higher total daily dose of insulin. E. The usual total daily dose of insulin is between 2 and 3 units/kg per day.

10. 3. You are reviewing dosage and management of insulin with a 9-year- old girl who weighs 35 kg and has had type 1 diabetes mellitus for several years. You calculate her total daily dose of insulin; she states she is receiving 25 units per day. The following statement that is true and relevant to her dosage is that A. After diagnosis of type 1 diabetes mellitus, the “honeymoon phase” lasts approximately 24 months. B. Approximately 50% of the total daily insulin prescribed is long acting, with the other 50% being short acting. C. The “mixed-split” regimen of insulin dosing allows for greater flexibility in diet than the “basal–bolus” regimen. D. Prepubertal children tend to require a higher total daily dose of insulin. E. The usual total daily dose of insulin is between 2 and 3 units/kg per day.

11. 4. A 3-year-old boy presents to the emergency department in diabetic ketoacidosis (ie, a blood sugar level of 450mg/dL). You resuscitate him with isotonic solution and admit him to your intensive care unit for monitoring. He is treated with normal saline and insulin at a rate of 0.1 unit/kg per hour. His blood glucose level gradually falls to 180 mg/dL, but ketosis persists, and the serum bicarbonate concentration is 13 mg/dL. At this time, the most appropriate medical management is to A. Add bicarbonate to IV fluids, continue insulin infusion. B. Add bicarbonate to IV fluids, discontinue insulin infusion. C. Add dextrose to IV fluids, continue insulin infusion. D. Add dextrose to IV fluids, discontinue insulin infusion. E. Discontinue IV fluids, change to subcutaneous insulin.

12. 4. A 3-year-old boy presents to the emergency department in diabetic ketoacidosis (ie, a blood sugar level of 450mg/dL). You resuscitate him with isotonic solution and admit him to your intensive care unit for monitoring. He is treated with normal saline and insulin at a rate of 0.1 unit/kg per hour. His blood glucose level gradually falls to 180 mg/dL, but ketosis persists, and the serum bicarbonate concentration is 13 mg/dL. At this time, the most appropriate medical management is to A. Add bicarbonate to IV fluids, continue insulin infusion. B. Add bicarbonate to IV fluids, discontinue insulin infusion. C. Add dextrose to IV fluids, continue insulin infusion. D. Add dextrose to IV fluids, discontinue insulin infusion. E. Discontinue IV fluids, change to subcutaneous insulin.

13. 5. A 5-year-old girl is undergoing treatment for diabetic ketoacidosis. Her level of consciousness fluctuates, she is vomiting repeatedly, and her diastolic blood pressure is 105 mm Hg. You should immediately A. Add bicarbonate to the intravenous fluids. B. Administer mannitol. C. Administer potassium. D. Infuse hypotonic saline solution. E. Obtain neuroimaging to look for cerebral edema

14. 5. A 5-year-old girl is undergoing treatment for diabetic ketoacidosis. Her level of consciousness fluctuates, she is vomiting repeatedly, and her diastolic blood pressure is 105 mm Hg. You should immediately A. Add bicarbonate to the intravenous fluids. B. Administer mannitol. C. Administer potassium. D. Infuse hypotonic saline solution. E. Obtain neuroimaging to look for cerebral edema

15. Type I DM Content Specs

16. What are the signs and symptoms of Type I DM?

17. Classically, polydypsia, polyuria, polyphagia, and weight loss – Also may see nocturia or enuresis

18. What is the best way to achieve good control of Type 1 DM??

19. Insulin Diet Exercise Psychologic acceptance of the disease

20. What is the value of Hemoglobin A1C in the management of T1DM?

21. – Give a snapshot into glucose control for the last 3-4 months

22. What is the honeymoon period?

23. When endogenous insulin secretion from remaining b-cells continues, and in many cases, insulin doses must be lowered to prevent hypoglycemia.

24. How do you manage sick days in T1DM?

25. Check BG and ketone levels q 3-4h Correct with short acting insulin q 3-4 hrs even if not eating DO NOT WITHOLD INSULIN Encourage fluid intake (1oz per year of age per hr in sips) Glc >200 sugar free fluids Glc <200 sugar containing fluids

26. What are some long term complications for T1DM? When should we screen for them?

27. Nephropathy – Age 10 and disease x5yrs Neuropathy – Annual foot exam starting at puberty Retinopathy – Age 10 and disease 3-5yrs Macrovascular disease

28. What other Autoimmune Diseases are associated with T1DM?

29. Thyroid Disease – (20% prevalence) Celiac Disease – (4.5% prevalence)

30. How do you manage hypoglycemia in diabetic patients?

31. Glc <70g/dL; give 15g carbs (glucose tabs or candy) Goal to get Glc >100g/dL If not, REPEAT Give 0.5 to 1mg Glucagon IM if unconscious or PO intolerant

32. What criteria justifies DKA?

33. pH 7.3 and below Bicarbonate of 15 or less Glucose >200

34. DKA treatment??

35. Plan to rehydrate over 48hrs Fluid resuscitate with isotonic fluids Fluids at 1.5 or 2x maintenance – 1/2NS +KCl and 1/2NS +Kphos Add Glucose to fluids once BG <300 Insulin drip (NO BOLUS) – 0.1u/kg/hr Frequent labs (ABG, Glucose, Electrolytes Q2)

36. DKA Complications??

37. Hypokalemia Hypoglycemia Cerebral Edema Shock

38. What’s the risk of using Bicarbonate in DKA?

39. Use has been associated with cerebral edema

40. What is the major cause of recurrent DKA??

41. NONCOMPLIANCE!!!!

42. Hypothyroidism

43. 5. Which of the following statements regarding congenital hypothyroidism is true? A. Goiter is a common feature seen in infants who have hypothyroidism. B. Males are affected more commonly than females. C. Most neonates show clinical features of hypothyroidism at birth. D. Preterm infants often have abnormal screening results due to delayed rise in T4 values. E. The most common cause is maternal antibody- mediated hypothyroidism.

44. 5. Which of the following statements regarding congenital hypothyroidism is true? A. Goiter is a common feature seen in infants who have hypothyroidism. B. Males are affected more commonly than females. C. Most neonates show clinical features of hypothyroidism at birth. D. Preterm infants often have abnormal screening results due to delayed rise in T4 values. E. The most common cause is maternal antibody- mediated hypothyroidism.

45. 6. A 2-week-old neonate born at 36 weeks’ gestation is receiving antibiotic therapy and ventilator support for presumed pneumonia and sepsis. Thyroid studies are performed because the neonatal screening result was abnormal. These studies reveal low total T4, low T3, low TSH, and normal free T4 values. Of the following, the most likely cause of these findings is: A. Autoimmune thyroiditis. B. Central hypothyroidism. C. Nonthyroidal illness (euthyroid sick syndrome). D. Thyroid aplasia. E. Thyroxine dyshormonogenesis.

46. 6. A 2-week-old neonate born at 36 weeks’ gestation is receiving antibiotic therapy and ventilator support for presumed pneumonia and sepsis. Thyroid studies are performed because the neonatal screening result was abnormal. These studies reveal low total T4, low T3, low TSH, and normal free T4 values. Of the following, the most likely cause of these findings is: A. Autoimmune thyroiditis. B. Central hypothyroidism. C. Nonthyroidal illness (euthyroid sick syndrome). D. Thyroid aplasia. E. Thyroxine dyshormonogenesis.

47. 7. Which of the following clinical features is most likely to be present at birth in a neonate who has congenital hypothyroidism? A. Bradycardia. B. Increased muscle tone. C. Jitteriness. D. Microcephaly. E. Normal for gestational age weight and length.

48. 7. Which of the following clinical features is most likely to be present at birth in a neonate who has congenital hypothyroidism? A. Bradycardia. B. Increased muscle tone. C. Jitteriness. D. Microcephaly. E. Normal for gestational age weight and length.

49. 8. You are evaluating a 10-year-old girl who has constipation and a recent decline in school performance. Thyroid studies ordered as part of your evaluation reveal the following: low total T4, low free T4, low TSH, and low T3 values. A TRH stimulation test results in elevation of the TSH to normal values. Which of the following is the most likely cause of her symptoms? A. Ectopic thyroid gland. B. Hashimoto thyroiditis. C. Hypothalamic tumor. D. Inborn error of thyroid metabolism. E. Thyroid-binding globulin deficiency.

50. 8. You are evaluating a 10-year-old girl who has constipation and a recent decline in school performance. Thyroid studies ordered as part of your evaluation reveal the following: low total T4, low free T4, low TSH, and low T3 values. A TRH stimulation test results in elevation of the TSH to normal values. Which of the following is the most likely cause of her symptoms? A. Ectopic thyroid gland. B. Hashimoto thyroiditis. C. Hypothalamic tumor. D. Inborn error of thyroid metabolism. E. Thyroid-binding globulin deficiency.

51. Hypothyroidism Content Specs

52. What are the consequences of untreated hypothyroidism in the neonate?

53. Lower IQ/decreased intellectual development

54. What are the signs and symptoms of untreated congenital and aquired hypothyroidism?

55. Lethargy Hypotonia Hoarse cry Feeding problems Constipation Macroglossia Umbical hernia Dry skin Hypothermia Prolonged jaundice

56. What are causes of congenital hypothyroidism? Aquired?

57. Congenital – Thyroid dysgenesis – Inborn errors of thyroxine synthesis – Maternal antibiody mediated hypothyroidism – Central hypothyroidism – Transient hypothyroidism – Iodide deficiency Aquired – Primary Autoimmune Postablation Irradiation Medications Late onset Congenital – Secondary Pituitary – Tertiary Hypothalamus – Miscellaneous Thyroid hormone resistance

58. How do you treat congenital and aquired Hypothyroidism?

59. Depends on cause: Synthroid

60. What is the prognosis of patients with hypothyroidism?

61. Depends on duration of illness and age at which treatment was started but it is generally good

62. What lab findings are present in TBG deficiency?

63. Low T4 Low or normal free T4 Normal TSH

64. Kawasaki Disease

65. 1. A 3-year-old boy has had an unremitting fever for 4 days. Which of the clinical findings below best supports Kawasaki disease (KD) as the explanation for his fever? A. Bilateral cervical lymph node enlargement B. Bilateral nonexudative conjunctivitis C. Periungual peeling of fingers and toes D. Tonsillar exudate E. Vesicles on the palms and soles

66. 1. A 3-year-old boy has had an unremitting fever for 4 days. Which of the clinical findings below best supports Kawasaki disease (KD) as the explanation for his fever? A. Bilateral cervical lymph node enlargement B. Bilateral nonexudative conjunctivitis C. Periungual peeling of fingers and toes D. Tonsillar exudate E. Vesicles on the palms and soles

67. 2. You are aware that other conditions can cause a similar clinical pattern. In your evaluation of this child, which of the following conditions is initially most likely to be confused with KD? A. Adenoviral infection B. Pauciarticular juvenile arthritis C. Rubella D. Staphylococcal scarlet fever E. Varicella-zoster

68. 2. You are aware that other conditions can cause a similar clinical pattern. In your evaluation of this child, which of the following conditions is initially most likely to be confused with KD? A. Adenoviral infection B. Pauciarticular juvenile arthritis C. Rubella D. Staphylococcal scarlet fever E. Varicella-zoster

69. 3. You order laboratory tests to add further diagnostic insights. Which of the following findings strengthen your impression that the child has KD? A. Elevated erythrocyte sedimentation rate B. Lymphocytosis C. Microcytic anemia D. Neutropenia E. Thrombocytopenia

70. 3. You order laboratory tests to add further diagnostic insights. Which of the following findings strengthen your impression that the child has KD? A. Elevated erythrocyte sedimentation rate B. Lymphocytosis C. Microcytic anemia D. Neutropenia E. Thrombocytopenia

71. 4. A 3-year-old girl meets clinical criteria for KD. You realize that the greatest threat to her is coronary artery disease. The best choice for initial imaging of the coronary arteries is A. Cardiac catheterization B. Computed tomography C. Magnetic resonance angiography D. Radionuclide imaging E. Two-dimensional echocardiography

72. 4. A 3-year-old girl meets clinical criteria for KD. You realize that the greatest threat to her is coronary artery disease. The best choice for initial imaging of the coronary arteries is A. Cardiac catheterization B. Computed tomography C. Magnetic resonance angiography D. Radionuclide imaging E. Two-dimensional echocardiography

73. 5. Although her echocardiography shows no coronary artery lesions, you realize that the girl is at risk for developing coronary artery disease and requires preventive therapy. The treatment that lowers the incidence of coronary artery disease in KD the most is high-dose A. Aspirin B. Corticosteroids C. Cyclosporine A D. Infliximab E. Intravenous immune globulin

74. 5. Although her echocardiography shows no coronary artery lesions, you realize that the girl is at risk for developing coronary artery disease and requires preventive therapy. The treatment that lowers the incidence of coronary artery disease in KD the most is high-dose A. Aspirin B. Corticosteroids C. Cyclosporine A D. Infliximab E. Intravenous immune globulin

75. Kawasaki Disease Content Specs

76. What are the clinical manifestations of Kawasaki disease?

78. What is the differential diagnosis for Kawasaki disease?

80. What lab abnormalities might you see in Kawasaki Disease?

81. Leukocytosis Normocytic, normochromic anemia Thrombocytosis Elevated ESR/CRP Elevated transaminases Elevated GGT Sterile pyuria

82. What is the value in IV IG and high dose Aspirin in Kawasaki disease?

83. IVIG is protective for CALs