- Severe hypercalcemic hyperparathyroidism developing in a patient with hyperaldosteronism and renal resistance to parathyroid hormone. [Case Reports]
- JBJ Bone Miner Res 2013; 28(3):700-8
- We evaluated an African American woman referred in 1986 at age 33 years because of renal potassium and calcium wasting and chronic hip pain. She presented normotensive, hypokalemic, hypocalcemic, nor...
We evaluated an African American woman referred in 1986 at age 33 years because of renal potassium and calcium wasting and chronic hip pain. She presented normotensive, hypokalemic, hypocalcemic, normophosphatemic, and hypercalciuric. Marked hyperparathyroidism was evident. Urinary cyclic adenosine monophosphate (cAMP) excretion did not increase in response to parathyroid hormone (PTH) infusion, indicating renal resistance to PTH. X-rays and bone biopsy revealed severe osteitis fibrosa cystica, confirming skeletal responsiveness to PTH. Renal potassium wasting, suppressed plasma renin activity, and elevated plasma and urinary aldosterone levels accompanied her hypokalemia, suggesting primary hyperaldosteronism. Hypokalemia resolved with spironolactone and, when combined with dietary sodium restriction, urinary calcium excretion fell and hypocalcemia improved, in accord with the known positive association between sodium intake and calcium excretion. Calcitriol and oral calcium supplements did not suppress the chronic hyperparathyroidism nor did they reduce aldosterone levels. Over time, hyperparathyroid bone disease progressed with pathologic fractures and persistent pain. In 2004, PTH levels increased further in association with worsening chronic kidney disease. Eventually hypercalcemia and hypertension developed. Localizing studies in 2005 suggested a left inferior parathyroid tumor. After having consistently declined, the patient finally agreed to neck exploration in January 2009. Four hyperplastic parathyroid glands were removed, followed immediately by severe hypocalcemia, attributed to "hungry bone syndrome" and hypoparathyroidism, which required prolonged hospitalization, calcium infusions, and oral calcitriol. Although her bone pain resolved, hyperaldosteronism persisted.
- An unusual case of diffuse focal osteolysis with disseminated osteoclastosis. [Journal Article]
- JCJ Clin Rheumatol 1997; 3(1):58-62
- We report the unusual case of a 31-year-old woman referred for inflammatory pain of the long bones associated with diffuse focal osteolysis, elevated erythrocyte sedimentation rate (ESR) and hypercal...
We report the unusual case of a 31-year-old woman referred for inflammatory pain of the long bones associated with diffuse focal osteolysis, elevated erythrocyte sedimentation rate (ESR) and hypercalcemia. Corticosteroid therapy produced dramatic symptomatic improvement, and biological parameters returned to normal. Biochemical markers of bone remodeling, particularly those of resorption, were strikingly elevated. Parathyroid hormone, parathyroid hormone-related protein, nephrogenous cyclic adenosine monophosphate and calcitriol levels were normal. Investigations for infectious disease and malignancy were negative. The histology of the lesions consisted of cortical osteolysis caused by intense osteoclastic resorption, with several foci of osteogenesis, without malignant cells. The patient died 9 months after the onset of symptoms, following a "malignant" course: severe hypercalcemia, extension of lytic bone lesions and spontaneous fractures. To our knowledge, such a peculiar picture with quite unusual radiologic findings, disseminated focal cortical lesions and a progressive course, without evidence for a tumoral process, has never been described. This case suggests either the presence of an unknown systemic osteoclast-activating factor from an occult tumor or a primary malignant hyperosteoclastosis.