Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Under observation were 131 patients with primary hyperparathyroidism, 22 of them (16.8%) suffering its visceral form. Seventeen patients aged from 31 to 60 years. The duration of the disease from the onset of initial signs till establishment of the final diagnosis was from 3 to 21 years. In two-thirds of patients this period lasted from 4 to 7 years. The clinical picture of the disease is described. All patients were subjected to surgical therapy--adenomas were removed in 18 patients, hyperplastic bodies --in 3, parathyroid cancer--in 1. No lethal issues were observed. The follow-up terms were from 1 to 7 years.
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PMID:[Diagnosis and treatment of visceral form of hyperparathyroidism]. 101 80

Parathyroid carcinoma is a rare tumor responsible for 0.5-5% of primary hyperparathyroidism. It is usually small (not more than 27 g) and the precise diagnosis of malignancy is made when local or distant metastases are found. We describe a case of a 37 yr old male presenting with a substernal goiter and no specific symptoms except hypertension. This mass had cysts and calcifications and it was in the anterior upper mediastinum. The patient had severe hypercalcemia (Ca greater than 14 mg/dl), high PTH levels and mild renal failure. Bone scanning showed signs of hyperparathyroidism. The patient was subjected to total thyroidectomy and removal of the mass en block. The tumor was circumscribed lobulated and mostly cystic. It weighed 1,200 g (380 g after evacuation of cysts) and measured 12 x 9 x 4.5 cm. Histologic examination showed a highly differentiated adenocarcinoma of parathyroid with metastasis in a regional lymph node. Almost 4 years later the patient is alive and well without hypercalcemia and without evidence of distant metastases.
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PMID:Large parathyroid functioning carcinoma (1,200 g) presenting as a substernal goiter. 156 Jan 89

Among 1819 patients with renal stone disease 44 cases with primary hyperparathyroidism (p.h.p.) were diagnosed. In all cases the diagnosis of php was confirmed by histomorphological examination. In 34 patients with php solitary adenoma was found, in 5 patients an adenoma with concomitant hyperplasia of the parathyroid glands, in 2 patients hyperplasia and in 3 patients carcinoma of the parathyroid glands. Hypercalcemia was found in 86% of patients, while elevated plasma levels of PTH in 90% of patients with php. Not in all patients PTH secretion was entirely autonomous. No significant correlation was found between plasma levels of PTH and kind of pathology of the parathyroid glands as well as clinical feature of php respectively.
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PMID:[Hypercalciuria and primary hyperparathyroidism in patients with kidney calculi. II. Primary hyperparathyroidism]. 164 64

Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. However, to our best knowledge, at least 163 cases of functioning parathyroid carcinoma appeared in the English literature from 1981 to 1989. We summarize the available information obtained from the reports of those patients and compare it with previous descriptions of the disease. The etiology of parathyroid carcinoma is usually obscure, but the possibility of a radiation-induced malignant change in the parathyroid gland became evident in a few patients. Clinical manifestations, including age, sex, symptoms, and biochemical findings in this review were comparable to those in previous reviews. Noninvasive localization studies such as ultrasonography may offer a diagnostic clue to parathyroid carcinoma. Measurement of DNA content is a useful adjunct for making the histologic diagnosis of parathyroid carcinoma and prediction of the clinical outcome. Since the initial operation offers the best chance for cure, pre-operative suspicion and intra-operative recognition of the parathyroid cancer are essential. The initial operation should be en bloc resection of the tumor, avoiding rupture of the tumor capsule and spillage of tumor cells. As parathyroid carcinoma is a slow-growing but tenacious malignancy, repeated resection of local recurrent tumors or even distant metastases is effective for palliation of recurrent hypercalcemia and occasional cure. When hypercalcemia is refractory to surgical therapy or no recurrent tumor can be identified, other modalities of therapy must be considered. New drugs to control hypercalcemia by inhibiting bone resorption may hold promise in patients with recurrent parathyroid carcinoma.
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PMID:Diagnosis and treatment of patients with parathyroid carcinoma: an update and review. 176 40

Nine patients (median age, 81 years) with primary hyperparathyroidism were treated with intravenous infusions of disodium pamidronate (APD), which is a bisphosphonate drug. Six patients had severe hypercalcemia (serum calcium concentration, greater than 3 mmol/L) persisting after rehydration with saline and treatment with furosemide; three patients had moderate hypercalcemia with pronounced symptoms (serum calcium concentration 2.8 to 2.9 mmol/L). Three of the patients were considered to have hypercalcemic crises. In all patients, the raised serum calcium levels were lowered by the disodium pamidronate infusions. One week after a single infusion of 15 to 60 mg disodium pamidronate, six of the nine patients had serum calcium concentrations within the normal reference interval and two patients had slightly raised values. Transient asymptomatic hypocalcemia was noted in one patient. All patients tolerated the infusions well, and no side effects were noted. In the patients with verified parathyroid adenomas, a temporary increase in parathyroid hormone levels were observed concomitant with the drop in serum calcium level. The patient with parathyroid cancer displayed no such effect indicating an autonomous parathyroid hormone secretion from the parathyroid carcinoma tumor. The good effect of treatment with the osteoclast inhibitor disodium pamidronate on hypercalcemia caused by primary hyperparathyroidism suggests that this hypercalcemia is mainly due to an increased osteoclast activity. The number of patients in this series is yet too small to allow general conclusions. But the case histories in this series show that disodium pamidronate promises to be of value in different clinical situations for the treatment of severe hypercalcemia in patients with hyperparathyroidism. It can be used (1) preoperatively to investigate whether the patient's symptoms are related to the hypercalcemia, (2) in the treatment of hypercalcemic crises when "forced diuresis" has failed to normalize the serum calcium, (3) after unsuccessful parathyroid surgery when it can be used as a long-term treatment before reoperation, giving time for localization studies and healing of the scar reaction, and (4) in aged and fragile patients where it can be tried as an alternative to surgery.
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PMID:Disodium pamidronate in the preoperative treatment of hypercalcemia in patients with primary hyperparathyroidism. 848 82

Parathyroid carcinoma is rare, occurring in less than 2-3% of the patients with clinical features of primary hyperparathyroidism. In haemodialysis patients parathyroid carcinoma has only once been described, although secondary hyperparathyroidism in these patients is common. We discuss two female haemodialysis patients with parathyroid carcinoma. Both were treated surgically: in one patient only local excision of the malignancy was performed; the other patient underwent a modified neck dissection on the side of the tumour as well. Physical, biochemical and radiological evaluation for 4-7 years after operation gave no evidence of recurrence of the malignancy.
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PMID:Successful surgical treatment of parathyroid carcinoma in two haemodialysis patients. 185 30

Parathyroid tumors account for only a small percentage of all head and neck neoplasms. The overwhelming majority of these are parathyroid adenomas that result in primary hyperparathyroidism. From 0.5% to 4% of hyperparathyroid patients, however, will be found to have a parathyroid carcinoma. In this paper, the authors relate their recent experience with such a patient and with two other such cases. Parathyroid carcinoma patients usually present with striking hyperparathyroidism and hypercalcemia, with the resultant related symptoms being more severe than those associated with parathyroid adenomas. Parathyroid carcinomas also tend to be large and may be detectable by current imaging techniques. The surgical appearance of these lesions is also distinct; the tumors are frequently multilobulated, gray-tan in color, quite firm, and often invasive. These physical findings are important since frozen section diagnosis may be difficult. The final histologic diagnosis depends on the presence of mitotic figures and capsular and vascular invasions. Preoperative medical problems, surgical approach, and prognostic factors are also discussed.
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PMID:Parathyroid carcinoma: clinical presentation and treatment. 224 Apr 16

Hyperplasia of the parathyroid glands is a central feature of familial multiple endocrine neoplasia type 1. We used cultured bovine parathyroid cells to test for mitogenic activity in plasma from patients with this disorder. Normal plasma stimulated [3H]thymidine incorporation, on the average, to the same extent as it was stimulated in a plasma-free control culture. This contrasted with the results of the tests with plasma from patients with familial multiple endocrine neoplasia type 1, in which parathyroid mitogenic activity increased 2400 percent over the control value (P less than 0.001). Plasma from these patients also stimulated the proliferation of bovine parathyroid cells in culture, whereas plasma from normal subjects inhibited it. Parathyroid mitogenic activity in plasma from the patients with familial multiple endocrine neoplasia type 1 was greater than that in plasma from patients with various other disorders, including sporadic primary hyperparathyroidism (with adenoma, hyperplasia, or cancer of the parathyroid), sporadic primary hypergastrinemia, sporadic pituitary tumor, familial hypocalciuric hypercalcemia, and multiple endocrine neoplasia type 2 (P less than 0.05). Parathyroid mitogenic activity in the plasma of patients with familial multiple endocrine neoplasia type 1 persisted for up to four years after total parathyroidectomy. The plasma also had far more mitogenic activity in cultures of parathyroid cells than did optimal concentrations of known growth factors or of any parathyroid secretagogue. This mitogenic activity had an apparent molecular weight of 50,000 to 55,000. We conclude that primary hyperparathyroidism in familial multiple endocrine neoplasia type 1 may have a humoral cause.
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PMID:Parathyroid mitogenic activity in plasma from patients with familial multiple endocrine neoplasia type 1. 287 88

Since the adrenal or parathyroid cancer is a clinically rare entity. We often have difficulty in its diagnosis and treatment. The adrenocortical cancer is usually classified into two categories--endocrinologically functioning or non-functioning. The incidence is not different between them. It is often found in an advanced stage as it does not show clinical manifestation before it has grown up to a large tumor. Only an effective agent for the adrenal cancer is op'-DDD so far. Recently, cisplatin, VP-16 (etoposide) and others are administered as trial use. Most of malignant pheochromocytomas are endocrinologically active and they often cause hypertension leading to death. Therefore it is important to control hypertension in malignant pheochromocytoma. Chemotherapy and irradiation are not effective for it. Recently, 131I-MIBG (metaiodobenzylguanidine) is found to be useful not only for diagnosis but also treatment of malignant pheochromocytoma. 131I-MIBG is accumulated specifically in the chromaffin cells and with helpful to find out metastatic foci. It is also used in a large amount as a specific irradiation therapy for this malignancy. Parathyroid cancer is found in approximately 3 percent of primary hyperparathyroidism. Clinically it usually reveal serum calcium level higher than 14 mg/dl, bone lesions and renal dysfunction in addition to palpable cervical tumors adhering with skin. Sometimes it is difficult to differentiate malignancy from adenoma in histology. Most cases develop local recurrences and distant metastases in due course and dies of hypercalcemia. It is very important to control hypercalcemia in inoperable cases. As both chemotherapy and radiation therapy render no effect on this malignancy. Surgery is a sole strategy for it.
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PMID:[Current therapy of endocrine organ tumors (adrenal and parathyroid glands)]. 334 84

An experience with 316 patients operated upon with a presumptive diagnosis of primary hyperparathyroidism is presented. Of the 316 patients, 291 (92.1 per cent) were cured after the initial cervical exploration without using any technique for preoperative localization of parathyroid tissue. Persistent and recurrent hyperparathyroidism occurred in 4.0 and 3.7 per cent, respectively. The success rate for secondary operations (cervical and mediastinal) was 82 per cent. Permanent unilateral vocal cord paralysis occurred in three patients and persistent symptomatic hypercalcemia occurred in another two. Twelve (4 per cent) of the patients had hyperparathyroid crisis and five (1.8 per cent) had carcinoma of the parathyroid gland. The mean follow-up time was six years. Removal of a single macroscopically enlarged gland, if the other glands are normal, is all that needs to be done in most instances. Subtotal parathyroidectomy should be preserved for those patients who have diffuse glandular hyperplasia.
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PMID:Primary hyperparathyroidism. 335 30


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