Hypoparathyroidism and biochemical screening.
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Biomedical subjects
Publications and source records attributed to B Frame.
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A 51-year-old man has had absorptive hypercalciuria and corticosteroid-responsive hypercalcemia for at least 12 years. There has been no clinical or laboratory proof of primary hyperparathyroidism, hypervitaminosis D, or other known causes of hypercalcemia and absorptive hypercalciuria. Hypercalciuria as well as the elevated serum level of calcium and 1 alpha, 25(OH)2D fell to normal during treatment with corticosteroids. The disturbed calcium metabolism in this patient is characteristic of that observed in sarcoidosis, but extensive studies have failed to uncover evidence of this condition.
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A history of head and neck irradiation in childhood or adolescence was found in 22 of 130 patients with primary hyperparathyroidism compared with only 12 of 400 control patients, a significant difference (P less than .025). Among 200 patients with a known history of childhood irradiation, biochemical or surgical evidence of hyperparathyroidism was found in ten, a prevalence of 5%. This is at least 30 times the prevalence of hyperparathyroidism in the general population (P less than .025). The data indicate that head and neck irradiation should be regarded as an important risk factor in the subsequent development of hyperparathyroidism.
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Recently acquired knowledge about vitamin D metabolism has improved our understanding in different varieties of osteomalacia. Many new causes of osteomalacia continue to be found. Radiologic and biochemical changes are not always characteristic and may occasionally be misleading. Bone biopsy after a double tetracycline label is helpful in differentiating osteomalacia from high bone turnover conditions and is recommended in most patients with a generalized rarefying skeletal disorder. Even if the underlying disease state cannot be corrected, effective therapy is available in most varieties of osteomalacia. The newer metabolites of vitamin D should soon be generally available to the medical profession. Their use will make treatment of osteomalacia more individualized and specific.
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Three patients with nephrolithiasis were found to have both medullary sponge kidney (MSK) and primary hyperparathyroidism. In all cases, urine calcium excretion returned to normal after parathyroidectomy. The passage of stones was abolished for more than 20 years in one case and for more than 12 years in another. The available data suggest that many patients with MSK are asymptomatic and that the risk of stone formation is increased by an associated metabolic abnormality such as hypercalciuria or hyperparathyroidism.
A 57-year-old woman with Turner syndrome had severe recurrent gastrointestinal bleeding. Exploratory laparotomy at the age of 26 showed an extensive telanglectasia of the entire small intestine. Following death due to myocardial infraction at age 57, postmortem examination revealed only a 0.2-cm residual telangiectasia in the mucosa of the distal part of the ileum. Spontaneous regression of the intestinal telangiectasia observed in Turner syndrome may occur and account for the improved prognosis with age.
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In two of 182 patients with verified primary hyperparathyroidism, microscopical hyperplasia was present in all parathyroid glands that were normal in size or only slightly enlarged. All parathyroid glands in another two patients showed microscopical hyperplasia and varied from a normal size of 190 mg. In seven additional patients, microscopical hyperplasia was present in one, several, or all parathyroid glands, which varied in weight from normal to 350 mg. Familial hyperparathyroidism or multiple endocrine neoplasia was evident in five of 11 patients. Contributing to difficulties was the experience in five patients in whom removal of mildly enlarged parathyroid glands corrected hypercalcemia, but definite microscopical abnormalities were not evident by routine histologic study of the glands. Thus, there appears to be a spectrum of abnormalities relative to size and microscopical changes in parathyroid glands of patients with primary hyperparathyroidism. The surgeon should be aware of these patterns of parathyroid hyperplasia that require a search for a fifth parathyroid gland and a subtotal parathyroidectomy.
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