Category: Calcium/Bone Disorders
Primary hyperparathyroidism (PHP) is the most common cause of outpatient hypercalcemia affecting 0.15% of the population. Hypercalcemia in pregnancy is rare and is under recognized and can lead to severe maternal and fetal complications. We report a case of hypercalcemia in pregnancy associated with PHP presenting with fetal demise.
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29-year-old female presented at 39 weeks of gestation with decreased fetal movement and diagnosed with intrauterine fetal demise. She reported increasing polydipsia, nausea and vomiting for a few weeks. Laboratory evaluation showed severely elevated calcium 15.9 mg/dl (8.7-10.5), albumin 3.4 g/dl (3.5-5), ionized calcium 2.04 mmol/L(1.17-1.33), 25 OH D 22 ng/ml (30-100) and PTH 657 pg/ml (15-65). She also had pre-eclampsia with acute renal failure. Supportive treatment was started with IV fluids and the fetus was delivered vaginally. She was treated with cinacalcet 90 mg BID and denosumab for persistent hypercalcemia. US neck showed 1.4 cm left parathyroid lesion but Sestamibi scan did not show any increased uptake. CT scan neck with contrast showed sub-cm nodularities in right thyrohyoid membrane. She had left parathyroid adenoma removal with post-operative decline in PTH to 7 pg/ml. 2 months later PTH levels were noted to be 108 mg/dl with normal calcium and vitamin D levels. Parathyroid 4 D CT scan showed 2 sub-cm arterial phase enhancing nodules in right strap muscle and left upper pole of thyroid. She underwent repeat neck exploration and bilateral superior parathyroidectomies which showed hyperplasia with improvement in PTH to 53 mg/dl. She continues to have close monitoring.
PHP is found in 0.05% of reproductive age woman but is significantly underdiagnosed in pregnancy. The diagnosis can be confounded due to pregnancy related symptoms and physiologic changes; elevated estrogen, hypoalbuminemia, increased GFR, and placental transfer of calcium. It is associated with serious maternal morbidity and mortality including pre-eclampsia, pancreatitis, hypercalcemic crisis and more than 2 fold increase in hyperemesis and nephrolithiasis. Adverse fetal outcomes include fetal demise, low birth weight, neonatal hypocalcemia, and mental retardation. Surgery in second trimester is treatment of choice if diagnosis is made in a a timely manner.
PHP in pregnancy is a rare diagnosis, is under recognized and is associated with life threatening maternal and fetal complications with up to 3.5-fold higher-than-normal rate of miscarriages. Timely diagnosis, prompt treatment and surgical intervention where indicated can reduce adverse maternal and fetal outcomes.
Starling Physicians Endocrinology, Hartford, CT.
West Hartford, Connecticut
I went to Aga Khan University Medical College, where I obtained Bachelors in Medicine and Surgery. I completed my internal medicine residency at the University of Connecticut, and an Endocrinology fellowship at the Cleveland Clinic Foundation. I like the diversity of Endocrinology and I have a special interest in thyroid disorders and academic medicine.
Stoughton Hospital, Stoughton, WI.
I completed my medical school in Aga Khan University. I trained at University of Connecticut for Internal medicine residency and am currently working as a hospitalist. I will be starting my fellowship in Endocrinology, Diabetes and metabolism in University of Wisconsin in 7/2019.