1/18/17 Resident Morning Report – Hypercalcemia, MEN1

CC: Abdominal pain

ID: 39 yo woman presents with progressively worsening abdominal pain for the past 5 days. She first experienced LUQ abdominal pain a year ago, and since then has also had constipation,  decreased appetite, weight loss, and occasional nausea/vomiting. Initial labs were significant for an elevated calcium of 10.9 mg/dl. Further work-up was consistent with hyperparathyroidism (elevated parathyroid hormone, normal urine calcium and vitamin D level). CT Abdomen/Pelvis showed pancreatic lesions and MRI Brain showed a 11 cm pituitary mass. She was eventually found to be positive for the MEN1 gene.

 

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Diagnostic Approach to Hypercalcemia
Approach to Hypercalcemia (UpToDate)

Diagnostic Approach to Hypercalcemia (UpToDate)

Key Points:

  • We know to correct the calcium level in the setting of hypoalbuminemia, but don’t forget to also adjust the calcium value if a patient has hyperalbuminemia. Pseudohypercalcemia can be seen when the albumin is elevated in patients with multiple myeloma and dehydration.
  • After confirming true hypercalcemia, the first test to order in the workup is a parathyroid hormone (PTH) level. If the PTH level is inappropriately normal or elevated, order a urinary calcium level or Ca/Cr to rule out familial hypocalciuric hypercalcemia.
  • Other tests to order in non-PTH mediated hypercalcemia include 25 hydroxyvitamin D, 1,25 hydroxyvitamin D, and PTH-related protein. If those are negative, consider other causes of hypercalcemia (multiple myeloma, hyperthyroidism, vitamin A intoxication, etc.)

Pearls from morning report:

  • Suspect familial/genetic disorders when a young patient is diagnosed with primary hyperparathyroidism, especially if less than 30 years old.
  • Replete vitamin D deficiency in patients with hyperparathyroidism as low vitamin D levels will further stimulate the production the parathyroid hormone.
  • Don’t be fooled by the “3 Ps of MEN1A” (pituitary, pancreas, and parathyroid). Gastrinomas in MEN1 patients can also be found in the duodenum.

Random trivia:

MEN1 is also known as Wermer’s syndrome, named after the physician who first commented on the genetic transmission of the disorder in 1954. However, the first case of MEN1 was reported by Dr. Jacob Erdheim who noted an enlarged pituitary gland and parathyroid glands in a man with acromegaly.


Want to read more?

A Practical Approach to Hypercalcemia (American Family Practice)


References: 

  • Thakker R, Newey P and G Walls. Clinical Practice Guidelines fro Multiple Endocrine Neoplasia Type I (MEN1). J Clin Endocrinology and Metabolism. Sept 2012, 97(9):2990-3011.
  • Toledo S and D Lourenco. Multiple Endocrine Neoplasia Type I: Diagnosis, Management, and Treatment. Endocrine Society. Jun 2014.
  • Shane E. Diagnostic Approach to Hypercalcemia. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on January 18, 2017)

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