Block 9 Board Review Part 3 Endocrine 7March2014 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

Why are we STILL reviewing ENDOCRINE???

3.5% of Initial Certifying Exam!!!

Pediatrics In Review Articles Type 2 Diabetes Mellitus Cushing’s Disease Grave’s Disease Puberty/Delayed Puberty

PIR Quizzes

Among the following factors, which is most likely to protect a 13-year-old patient against type 2 diabetes mellitus (DM)? A. Adolescent age. B. Female sex. C. History of being breastfed. D. Mother who had gestational diabetes. E. Native American race

Among the following factors, which is most likely to protect a 13-year-old patient against type 2 diabetes mellitus (DM)? A. Adolescent age. B. Female sex. C. History of being breastfed. D. Mother who had gestational diabetes. E. Native American race

It can be difficult to distinguish between type 1 and type 2 DM in a child who presents with ketoacidosis. Among the following, which clinical finding is most suggestive of type 2 DM? A. Acanthosis nigricans. B. Polydipsia. C. Polyphagia. D. Polyuria. E. Weight loss

It can be difficult to distinguish between type 1 and type 2 DM in a child who presents with ketoacidosis. Among the following, which clinical finding is most suggestive of type 2 DM? A. Acanthosis nigricans. B. Polydipsia. C. Polyphagia. D. Polyuria. E. Weight loss

In your management of a 7-year-old boy with type 1 DM, which of the following parameters are you likely to assess every 3 months? A. Albuminuria. B. Hemoglobin A1c. C. Lipid profile. D. Liver function tests. E. Signs of sleep apnea

In your management of a 7-year-old boy with type 1 DM, which of the following parameters are you likely to assess every 3 months? A. Albuminuria. B. Hemoglobin A1c. C. Lipid profile. D. Liver function tests. E. Signs of sleep apnea

In addition to diet and exercise, you are considering pharmacotherapy for a 15-year-old girl who has had type 2 DM for the past year. Among the following, which is the only drug approved by the US Food and Drug Administration for someone her age? A. Acarbose. B. Exenatide. C. Glipizide. D. Metformin. E. Pioglitazone.

In addition to diet and exercise, you are considering pharmacotherapy for a 15-year-old girl who has had type 2 DM for the past year. Among the following, which is the only drug approved by the US Food and Drug Administration for someone her age? A. Acarbose. B. Exenatide. C. Glipizide. D. Metformin. E. Pioglitazone.

In a patient with newly diagnosed type 2 DM, initiation of insulin therapy is recommended if which one of the following findings is present? A. Fasting blood glucose level of 140 mg/dL (7.8 mmol/L). B. Hemoglobin A1c level of 8.0% (0.08). C. Hemoglobin A1c level of 8.5% (0.09). D. Random blood glucose level of 200 mg/dL (11.1 mmol/L). E. Random blood glucose 275 mg/dL (15.3 mmol/L).

In a patient with newly diagnosed type 2 DM, initiation of insulin therapy is recommended if which one of the following findings is present? A. Fasting blood glucose level of 140 mg/dL (7.8 mmol/L). B. Hemoglobin A1c level of 8.0% (0.08). C. Hemoglobin A1c level of 8.5% (0.09). D. Random blood glucose level of 200 mg/dL (11.1 mmol/L). E. Random blood glucose 275 mg/dL (15.3 mmol/L).

1. Which of the following statements about normal puberty in children is true? A. Bone age correlates better with pubertal development than chronologic age. B. Gonadotropin-releasing hormone (GnRH) secretion in response to negative feedback from sex steroids is constant throughout life. C. Growth hormone secretion is the sole determinant of the pubertal growth spurt. D. Menarche is the first stage of puberty in girls. E. Normal pubertal development is unrelated to nutritional status.

1. Which of the following statements about normal puberty in children is true? A. Bone age correlates better with pubertal development than chronologic age. B. Gonadotropin-releasing hormone (GnRH) secretion in response to negative feedback from sex steroids is constant throughout life. C. Growth hormone secretion is the sole determinant of the pubertal growth spurt. D. Menarche is the first stage of puberty in girls. E. Normal pubertal development is unrelated to nutritional status.

2. Which of the following statements best describes adrenarche. A 2. Which of the following statements best describes adrenarche? A. Breast development becomes evident in girls. B. Hypothalamic production of adrenocorticotropin hormone increases. C. Maternal estrogens are withdrawn, causing neonatal acne. D. Spermatozoa begin to appear in seminal fluid. E. The adrenal gland increases production of dehydroepiandrosterone sulfate.

2. Which of the following statements best describes adrenarche. A 2. Which of the following statements best describes adrenarche? A. Breast development becomes evident in girls. B. Hypothalamic production of adrenocorticotropin hormone increases. C. Maternal estrogens are withdrawn, causing neonatal acne. D. Spermatozoa begin to appear in seminal fluid. E. The adrenal gland increases production of dehydroepiandrosterone sulfate.

3. Which of the following is the primary action of luteinizing hormone 3. Which of the following is the primary action of luteinizing hormone? A. Secretion of follicle-stimulating hormone. B. Secretion of GnRH from the pituitary gland. C. Stimulation of gametogenesis in the testes. D. Stimulation of the gonads to produce androgens. E. Stimulation of the ovarian follicle to produce estrogen

3. Which of the following is the primary action of luteinizing hormone 3. Which of the following is the primary action of luteinizing hormone? A. Secretion of follicle-stimulating hormone. B. Secretion of GnRH from the pituitary gland. C. Stimulation of gametogenesis in the testes. D. Stimulation of the gonads to produce androgens. E. Stimulation of the ovarian follicle to produce estrogen

4. At which of the following phases of the menstrual cycle is the concentration of progesterone the highest? A. The beginning of the follicular phase. B. The beginning of the luteal phase. C. The end of the luteal phase. D. The middle of the follicular phase. E. The middle of the luteal phase.

4. At which of the following phases of the menstrual cycle is the concentration of progesterone the highest? A. The beginning of the follicular phase. B. The beginning of the luteal phase. C. The end of the luteal phase. D. The middle of the follicular phase. E. The middle of the luteal phase.

6. Normal pubertal development varies according to a child’s weight and ethnicity. Which of the following clinical findings indicates premature pubertal development? A. Penile enlargement in a 10-year-old African American male of normal weight. B. Stage 3 pubic hair in a 7-year-old Mexican American girl of normal weight. C. Testicular enlargement in a 9-year-old white boy who is obese. D. Thelarche in a 7-year-old African American girl of normal weight. E. Thelarche in a 9-year-old white girl who is obese.

Normal pubertal development varies according to a child’s weight and ethnicity. Which of the following clinical findings indicates premature pubertal development? A. Penile enlargement in a 10-year-old African American male of normal weight. B. Stage 3 pubic hair in a 7-year-old Mexican American girl of normal weight C. Testicular enlargement in a 9-year-old white boy who is obese. D. Thelarche in a 7-year-old African American girl of normal weight. E. Thelarche in a 9-year-old white girl who is obese.

7. You are seeing a 13-year-old girl who experienced menarche 3 months ago. Her physical examination shows that the areolae form a secondary mound above the contour of her breasts. Her pubic hair is curly and coarse and covers the lower portion of her mons pubis. Which of the following is the most accurate description of her Sexual Maturity Rating? A. Breast: stage 2, pubic hair: stage 3. B. Breast: stage 3, pubic hair: stage 3. C. Breast: stage 3, pubic hair: stage 4. D. Breast: stage 4, pubic hair: stage 4. E. Breast: stage 4, pubic hair: stage 5.

You are seeing a 13-year-old girl who experienced menarche 3 months ago. Her physical examination shows that the areolae form a secondary mound above the contour of her breasts. Her pubic hair is curly and coarse and covers the lower portion of her mons pubis. Which of the following is the most accurate description of her Sexual Maturity Rating? A. Breast: stage 2, pubic hair: stage 3. B. Breast: stage 3, pubic hair: stage 3. C. Breast: stage 3, pubic hair: stage 4. D. Breast: stage 4, pubic hair: stage 4. E. Breast: stage 4, pubic hair: stage 5.

8. Which of the following is a true statement regarding normal pubertal development? A. Behavioral changes in adolescence are a direct manifestation of increases in sex hormone concentrations. B. Bone age is an accurate determinant of height potential in boys and girls. C. Most girls miss periods for 90 days within 1 year of menarche. D. Pubertal gynecomastia is rare and should prompt an investigation. E. The pubertal growth spurt in girls typically occurs within 2 to 3 months after menarche.

8. Which of the following is a true statement regarding normal pubertal development? A. Behavioral changes in adolescence are a direct manifestation of increases in sex hormone concentrations. B. Bone age is an accurate determinant of height potential in boys and girls. C. Most girls miss periods for 90 days within 1 year of menarche. D. Pubertal gynecomastia is rare and should prompt an investigation. E. The pubertal growth spurt in girls typically occurs within 2 to 3 months after menarche.

9. Which of the following patients should undergo an evaluation to rule out organic pathology as a cause for abnormal pubertal development at this time? A. A 6-year-old white girl who has unilateral thelarche, normal growth velocity, and no pubic hair development. B. A 7-year-old Mexican American boy who has breast development and testicular enlargement. C. A 13-year-old African American girl who has a recent growth spurt and no menarche. D. A 13-year-old white boy who has no testicular enlargement and normal growth velocity. E. A 16-year-old African American girl who has excessive pubic hair and normal menstrual cycles.

9. Which of the following patients should undergo an evaluation to rule out organic pathology as a cause for abnormal pubertal development at this time? A. A 6-year-old white girl who has unilateral thelarche, normal growth velocity, and no pubic hair development. B. A 7-year-old Mexican American boy who has breast development and testicular enlargement. C. A 13-year-old African American girl who has a recent growth spurt and no menarche. D. A 13-year-old white boy who has no testicular enlargement and normal growth velocity. E. A 16-year-old African American girl who has excessive pubic hair and normal menstrual cycles.

Match the clinical scenario with the most likely diagnosis. 6 Match the clinical scenario with the most likely diagnosis. 6. 13–11/12-year-old girl who has secondary amenorrhea and a body mass index of 13 kg/m2. 7. 14–10/12-year-old girl who has primary amenorrhea and is a competitive gymnast. 8. 15–1/12-year-old tall boy who has small testes and Sexual Maturity Rating 5 pubic hair. 9. 16–9/12-year-old short girl who has primary amenorrhea and a webbed neck. 10. 17–5/12-year-old boy who has small testes and anosmia. A. Constitutional delayed puberty. B. Functional gonadotropin deficiency. C. Kallman syndrome. D. Klinefelter syndrome. E. Turner syndrome.

Match the clinical scenario with the most likely diagnosis Match the clinical scenario with the most likely diagnosis. 13–11/12-year-old girl who has secondary amenorrhea and a body mass index of 13 kg/m2. B 14–10/12-year-old girl who has primary amenorrhea and is a competitive gymnast. B 15–1/12-year-old tall boy who has small testes and Sexual Maturity Rating 5 pubic hair. D 16–9/12-year-old short girl who has primary amenorrhea and a webbed neck. E 10. 17–5/12-year-old boy who has small testes and anosmia. C A. Constitutional delayed puberty. B. Functional gonadotropin deficiency. C. Kallman syndrome. D. Klinefelter syndrome. E. Turner syndrome.

CONTENT SPECS!!!

Type 2 Diabetes Mellitus

How can you tell the difference between Type 1 and Type 2 DM?

What the difference between Type 1 and Type 2 DM? Sometimes the difference is hard to distinguish Type 1: Autoimmune destruction of B cells, thus no insulin Type 2: Increased insulin resistance with relative insulin deficiency

What is Acanthosis Nigricans a marker for?

What is Acanthosis Nigricans a marker for? Insulin Resistance

What are the treatment approaches to Type 2 DM?

What are the treatment approaches to Type 2 DM? Diabetes “team” Diet and Exercise modification Insulin in for random glc on >250 Metformin in >10yrs

What screening tests should you do for a patient with Type 2 DM?

What screening tests should you do for a patient with Type 2 DM? Hbgb A1C q3mo BP qvisit LFTs at diagnosis and annually Eye exam at diagnosis and annually Lipid panel at diagnosis and annually UA at diagnosis and annually Assess for symptoms of sleep apnea at diagnosis and annually

What are long term complications of Type 2 DM?

What are long term complications of Type 2 DM? Microvascular (retinopathy, neuropathy, nephropathy) Macrovascualr (HTN, hyperlipidemia) May be present at diagnosis

Cushing’s Syndrome

What are the signs and symptoms of Cushing Syndrome?

What are the signs and symptoms of Cushing Syndrome? Obesity Delayed or absent linear growth May or may not be present Acne Virilization Hirsuitism Round Facies HTN Lethargy/Depression Osteoporosis Myopathy Striae Buffalo hump

What is the most common cause of Cushing Syndrome?

What is the most common cause of Cushing Syndrome? Protracted use of Exogenous Corticosteroids (including topical, oral, and inhaled)

What is the lab evaluation for a patient with suspected Cushing Syndrome?

What is the lab eval for a patient with suspected Cushing Syndrome? 24hr urine free cortisol Midnight Plasma and Salivary Cortisol Low dose Dexamethasone suppression 1mg Dexamethasone suppression

Hyperthyroidism

What are the signs and symptoms of Grave’s Disease?

What are the signs and symptoms of Grave’s Disease? tachycardia weight loss increased appetite heat intolerance Tremor Exophthalmos eyelid lag moist skin fine hair Restlessness emotional lability short attention span declining school performance fatigue

What are the signs and symptoms of Neonatal Hyperthyroidism?

What are the signs and symptoms of Neonatal Hyperthyroidism? Irritability Tremor Flushing Hyperactivity Increased bowel motility Thrombocytopenia Cardiac dysfunction hepatosplenomegaly

What are the treatment options for hyperthyroidism?

What are the treatment options for hyperthyroidism? Antithyroid medications Radioiodine Ablation Surgery

Puberty

Distinguish between Normal and Abnormal Gynecomastia in males

Distinguish between Normal and Abnormal Gynecomastia in males Occurs after the onset of genital development in 50% Usually last less than 1 year Abnormal Any variation from this Prior to puberty is ABNORMAL

What is the significance of a breast mass in an adolescent girl?

What is the significance of a breast mass in an adolescent girl? Likely benign

What is the differential diagnosis for precocious puberty?

What is the differential diagnosis for precocious puberty? CNS tumors Ovarian tumors Ovarian Cysts Feminizing Adrenal tumors McCune Albright CAH Leydig Cell tumors Familial Male Precocious Puberty

What is premature thelarche?

What is premature thelarche? Early breast development without other signs of puberty in girls Common in ages 1-4yrs Regresses spontaneously Breast development before puberty (without other signs of puberty) in boys is ABNORMAL! RULE OUT NEOPLASM!

What tumors may produce precocious puberty?

What tumors may produce precocious puberty? Liver CNS Ovary Testes Adrenal Glands

What are the causes of precocious puberty?

What are the causes of precocious puberty? early activation of the HPG axis, with gonadotropins stimulating sex hormone production More common in girls than boys Idiopathic CNS disorders (more common in boys) Overweight/obesity Gonadotropin Independent (adrenal/gonad disorders) or exogenous hormones McCune Albright Syndrome (non classic CAH) Neoplasms (rare)

What are the signs and symptoms of delayed puberty?

What are the signs and symptoms of delayed puberty? Delayed puberty is defined as lack of breast development by age 13.0 years in girls and lack of pubertal testicular development (genital stage 2) by age 14.0 years in boys Slowing of linear growth

What are the causes of delayed puberty?

What are causes of delayed puberty?

What is the natural history of constitutional delayed puberty?

What is the natural history of constitutional delayed puberty? Once puberty begins, its course and tempo are normal, and catch-up growth to target height occurs No puberty by age 18 in boys= PATHOLOGIC

When is treatment for constitutional delayed puberty indicated?

When is treatment for constitutional delayed puberty indicated? If a negative self image is present

What are the therapeutic options for delayed puberty??

What are the therapeutic options for delayed puberty?? Testosterone IM Oral Transdermal Gels Patch Estrogen

Quick Associations

McCune-Albright syndrome McCune-Albright syndrome. A, Irregular café-au-lait pigmentation over right anterior chest, shoulder, and right arm. B, Polyostotic fibrous dysplasia. Multiple areas of fibrous dysplasia, most commonly found in long bones and pelvis.

Premature thelarche. Isolated bilateral breast enlargement in a toddler with premature thelarche.

Premature adrenarche. Pubic hair development in a prepubertal girl with premature adrenarche

Cushing syndrome. These photographs show how dramatic the changes associated with Cushing syndrome are and how rapidly they occur. A, Patient before the onset of Cushing syndrome. B, Patient 4 months after photograph in A was taken. Note the centripetal obesity of the trunk compared with the extremities after the onset of Cushing syndrome. C, Moon facies, clearly demonstrated, should raise the diagnostic index of Cushing syndrome. D, Buffalo hump. Excessive adipose tissue over the lower cervical and upper thoracic spine is characteristic of Cushing syndrome.