(IEMR6-7)Regulation of Endocrine Function/Autoimmunity Flashcards

1
Q

A 33-year-old man who is the father of two children (ages 3 and 7) comes to your office complaining of a generalized lack of energy and a waning interest in sex. Laboratory studies demonstrate a low testosterone level. Measuring which of hormone can assist you in determining if the hypogonadism is due to testicular versus central defects?

A

Measuring LH is appropriate. If LH is high, then hypothalamus and pituitary are working.
IF LH is low, then hypothalamus-pituitary is the problem.

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2
Q

A boy presents at age 10 with a height that is appropriate for a 3 year-old. In addition, he exhibits a prominent forehead, a depressed nasal bridge, underdevelopment of the mandible, truncal obesity, and a very small penis. These features could represent severe Growth Hormone deficiency but when measured, serum Growth hormone concentrations are noted to be well above the normal range. Further studies showed a marked deficiency in Insulin-Like-Growth factor. Discuss the possible causes and suggest a most likely scenario.

A

GH typically exerts some of its actions through IGF-1. If GH is high and IGF-1 is low, GH is not exerting its effect, a possible cause is a lack of a functioning GH receptor – this is a rare condition called Larons dwarfism which is treated by the IGF1 agonist MECASERMIN.

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3
Q

At the age of 12, shortly after the onset of menstruation, Sally X. suddenly began to experience polyuria and polydipsia and she lost approximately 10 pounds in 4 days. Nausea and vomiting developed and became so severe that her parents brought her to your emergency department, where plasma glucose was determined to be 396 mg% (normal fasting is 70-110 mg%) and her urine was positive for ketones. Therapy with IV fluids and insulin was begun, and 2 days later Sally was home and back to her usual self. She quickly mastered the use of insulin and finger- stick glucose monitoring. What is a likely cause for her type 1 diabetes mellitus?

A

Autoimmune destruction of the pancreatic beta cells.

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4
Q

At age 16, Sally started to have frequent insulin reactions. Her daily insulin dose was decreased, but she functioned reasonably until one day when she missed a meal, prompting a major motor seizure (glucose was determined to be 20 mg%). At the emergency department her blood pressure was 90/60 mm Hg with a pulse of 100/minute supine, and 60/?? Mm Hg with a pulse of 125/minute standing. Her skin was tanned with pigmentation of the palmar creases and areolae. Other aspect of the physical exam were non-contributory. She was given 50 mL of 50% dextrose and started on normal saline IV. What is the likely diagnosis?

A

adrenal failure

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5
Q

At age 16, Sally started to have frequent insulin reactions. Her daily insulin dose was decreased, but she functioned reasonably until one day when she missed a meal, prompting a major motor seizure (glucose was determined to be 20 mg%). At the emergency department her blood pressure was 90/60 mm Hg with a pulse of 100/minute supine, and 60/?? Mm Hg with a pulse of 125/minute standing. Her skin was tanned with pigmentation of the palmar creases and areolae. Other aspect of the physical exam were non-contributory. She was given 50 mL of 50% dextrose and started on normal saline IV. What is the likely cause of her adrenal deficiency?

A

Most common cause of adrenal failure in US is autoimmune.

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6
Q

At age 16, Sally started to have frequent insulin reactions. Her daily insulin dose was decreased, but she functioned reasonably until one day when she missed a meal, prompting a major motor seizure (glucose was determined to be 20 mg%). At the emergency department her blood pressure was 90/60 mm Hg with a pulse of 100/minute supine, and 60/?? Mm Hg with a pulse of 125/minute standing. Her skin was tanned with pigmentation of the palmar creases and areolae. Other aspect of the physical exam were non-contributory. She was given 50 mL of 50% dextrose and started on normal saline IV. Adrenal failure was discovered to be the cause. What else can go wrong? Are there any other endocrine deficiencies that are associated with diabetes and addsions?

A

Polyglandular Failure due to Autoimmune Mechanisms

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