Background:
Primary hyperparathyroidism is a disorder of parathyroid glands in which one or more of these glands is/are overactive. As a result, the overactive gland releases too much parathyroid hormone (PTH) which increases serum calcium levels from thinning of the bones. Osteoporosis occurs in patients with hyperparathyroidism, but it is usually reversible post parathyroidectomy. In the United States, about 100,000 people develop primary hyperparathyroidism each year. The disorder is diagnosed most often in people between age 50 and 60, and women are affected about three times as often as men.
Case:
This is the case of a 71-year-old lady with history of primary hyperparathyroidism treated with left parathyroidectomy for severe hypercalcemia and osteoporosis. However, although quiescent for few months, the hyperparathyroidism recurred and the osteoporosis worsened. She was referred to our Osteoporosis clinic for evaluation and management. Patient had multiple Sestamibi scans for re-localization of the abnormal parathyroid which were all unsuccessful. Also, she went through a second neck exploration which failed to find the abnormal parathyroid gland. She even underwent fine needle aspiration of intrathyroidal cysts with PTH washouts for identification of possible parathyroid adenoma with negative results. Patient was then treated with bisphosphonates for 5 years and her DEXA scan T score of (-) 2.9 at the radius never improved. Bisphosphonates were discontinued, and patient was treated with Denosumab infusions every 6 months for the last two years in an attempt to help with both osteoporosis and hypercalcemia. Repeated DEXA scan revealed significant improvement of bone mineral density at the level of the lumbar spine, with worsening of osteoporosis at the femurs and radius. Hypercalcemia normalized while PTH remained inappropriately high normal.
Discussion:
Osteoporosis from hyperparathyroidism affects predominantly long bones. Aside from parathyroidectomy of parathyroid adenomas, no other therapy has been shown to improve cortical bone osteoporosis due to hyperparathyroidism. Denosumab is a human monoclonal antibody which is used for the treatment of osteoporosis in postmenopausal women who have high risk of bone fracture. It has been used lately also for treatment of hypercalcemia of malignancy. Although our patient’s hypercalcemia and trabecular bone osteoporosis improved with Denosumab, cortical bone osteoporosis worsened. This case illustrates the power of continuous PTH secretion on cortical bone resorption which is able to counteract the beneficial effect of Rank ligand blockade at the trabecular bone level. It also demonstrates the need for more research on potential therapies to address this PTH specific action.
Hypertriglyceridemia is a commonly encountered condition for health care providers, it is defined by a fasting serum triglyceride level of more than 150 mg/dl. However, more severe hypertriglyceridemia >1000 mg/dl is rare and found usually in less 1/5000 individuals. (Ford ES, et al, 2009) hypertriglyceridemia can be secondary to few diseases among which is familial lipodystrophy. Triglyceride level of more than 1000 mg/dl is associated with acute pancreatitis, and the risk increases dramatically with increasing triglyceride level to above 2000 mg/dl (reaching 20%) (Scherer J, et al,2014). We present a 39-year-old Caucasian male patient who started to have recurrent acute pancreatitis at the age of 18, investigations for the common causes of acute pancreatitis was unremarkable, except for triglyceride level of 20,000 mg/dl. Genetic testing was consistent with familial partial lipodystrophy. Management initially consisted of plasmapheresis to control the acute episode, in addition to lipid lowering agents (Statin, fenofibrate, and omega-3-acid) .despite being on maximal medical therapy, he required plasmapheresis on weekly basis.His physical exam was remarkable for loss of adipose tissue in the lower extremities and excess adipose tissue deposit in the supra-clavicular area. other metabolic complications included severe insulin resistance and difficult to control DM, and NASH that is progressing to chronic liver disease. The hallmark of lipodystrophy syndromes in general is the selective loss of adipose tissue, which can be partial or complete. Lipodystrophy syndromes are divided into congenital and acquired. Acquired lipodystrophy is most commonly seen is the lipodystrophy secondary to HIV antiretroviral medications (protease inhibitors)(Garg A et.al ,2011). Congenital lipodystrophy remains rare. Our patient has Partial familial lipodystrophy (PFLD), as evident by his physical exam findings. FPLD is also associated with other metabolic abnormalities include hyperglycemia, insulin resistance, increased metabolic rate, and hypertriglyceridemia and that can be severe as in this patient. Conclusion: untreated severe hypertriglyceridemia is an independent risk factor for CAD, as well as a cause of recurrent pancreatitis, which is associated with significant morbidity and mortality, evaluation for secondary causes helps guide the management and prevent serious complications and recurrence.
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