Pseudohyperparathyroidism

Hypercalcemia, normo- or hypophosphatemia, and increased urinary calcium clearance were observed in 8 dogs with malignant disease consisting of lymphosarcoma (n = 4), lymphogenous leukemia with bone involvement (n = 2), or carcinoma arising in the perianal region (n = 2). Parathyroid glands from these dogs had a normal or atrophic microscopic appearance. Ultrastructurally, the chief cells of the parathyroid glands had small cytoplasmic areas devoid of secretory granules and containing few organelles associated with hormonal synthesis, which is indicative of an inactive phase of the secretory cycle...

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Ability to successfully accomplish parathyroid surgery, as well as to understand the pathophysiology of parathyroid disease and hypercalcemia, is essential to the Otolaryngology Head and Neck surgeon (who must treat parathyroid disease), because of the more frequent identification of parathyroid adenomata and parathyroid hyperplasia (with their inherent hypercalcemia). Today many more cases are recognized because of hypercalcemia identified by now routine multiphasic biochemical screening tests. The etiology of hypercalcemia is discussed in considering the differential diagnosis of pseudohyperparathyroidism, and true hyperparathyroidism of parathyroid hyperplasia and parathyroid adenomata...

Pseudohyperparathyroidism to sarcomas is very rare. A 16-year-old boy had a rhabdomyosarcoma and severe hypercalcemia associated with an elevated serum parathyroid hormone (PTH) level and osteolytic bone metastases. The four parathyroid glands were normal. Both the hypercalcemia and the serum PTH level responded to chemotherapy.

We treated a case of hypercalcemia and primary liver tumor and reviewed a series of such cases treated at the Mayo Clinic (Rochester, Minn). Primary tumor of the liver was diagnosed in 192 patients (152 had hepatocellular carcinomas; 40, cholangiocarcinomas) between 1969 and 1980. Hypercalcemia of unknown cause was found in eight patients with hepatocellular carcinoma (5.3%) and seven with cholangiocarcinoma (17.5%). Five hypercalcemic patients had serum immunoreactive parathyroid hormone values consistent with ectopic hyperparathyroidism...

Postmortem bone and parathyroid gland histology in nine hypercalcemic cancer patients without bone metastases was compared to bone and parathyroid histology in ten normocalcemia patients. Parameters of parathyroid function, including serum immunoreactive parathyroid hormone, acid base status, serum phosphate, and nephrogenous cyclic AMP were measured in the hypercalcemic group and compared to normals and to patients with primary hyperparathyroidism. Bone histology in all nine hypercalcemic cancer patients showed increased osteoclastic bone resorption and increased fibrous connective tissue in the bone marrow...

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A study is reported of the estimation of plasma chloride concentration and acid-base status in the differentiation of primary hyperparathyroidism from all other causes of hypercalcaemia. In the two groups of patients studied, all of whom had hypercalcaemia, there was complete separation between the two groups on the basis of plasma chloride concentration and acid-base status. In 16 patients with primary hyperparathyroidism the increase in plasma chloride concentration and associated metabolic acidosis could have been accounted for by the known renal tubular effects of parathyroid hormone...

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