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Query: UMLS:C0020437 (hypercalcemia)
 10,293 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Forty-five patients with biochemically documented primary hyperparathyroidism as part of multiple endocrine neoplasia syndrome types 1 or 2 were surgically treated from 1960 through 1980. Hyperplasia occurred in 69% of the patients, single adenoma in 27%, and double adenomas in 4%. All but two patients with hyperplasia underwent subtotal parathyroidectomy. In this group, the cure rate was 93% and the incidence of permanent postoperative hypoparathyroidism 23%. In the adenoma group, treatment included excision of the adenoma and biopsy of at least one other gland. The cure rate was 76%, with no postoperative hypoparathyroidism. Analysis of patients with persistent hyperparathyroidism suggested that failure to recognize multiple gland disease was the principal cause of postoperative hypercalcemia. In view of the high incidence of hypocalcemia after subtotal parathyroidectomy, approximately 500 mg of tissue should be cryopreserved to allow transplantation should hypocalcemia ensue postoperatively.
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PMID:Primary hyperparathyroidism in patients with multiple endocrine neoplasia syndromes. Surgical experience. 613 93

A case of MEN type IIa is described showing among other symptoms, three attacks of transient neurological symptoms. These were attributed to a localized, transient cerebral ischemia. The latter probably developed as a consequence of the concomitant occurrence of different phenomena: the existence of hypercalcemia and on the other hand the secretion of vasoactive substances by one of the three tumours forming the MEN type IIa syndrome. The pheochromocytoma played an essential role because the symptoms did not recur after the bilateral adrenalectomy.
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PMID:An unusual cause of attacks of focal cerebral symptoms: multiple endocrine neoplasia type IIA. 613 63

A rare presentation of hypercalcemia secondary to the probable ectopic production of parathormone by a malignant melanoma associated with a pulmonary carcinoid is discussed. We suggest the association of melanoma with multiple endocrine neoplasia.
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PMID:Pulmonary carcinoid associated with a parathormone producing melanoma. 613 87

We report the case of a 43 year old man with multiple endocrine neoplasia (Zollinger-Ellison syndrome-hyperparathyroidism) diagnosed during investigations for a severe ulcer with diarrhoea. The history suggested Zollinger-Ellison syndrome, especially as basal acid production and blood gastrin levels were high. Hypercalcaemia and low blood phosphate levels suggested hyperparathyroidism, which was confirmed by ultra-sound of the neck, showing two parathyroid adenomas. The aim of treatment was two-fold: suppress the hyperparathyroidism and treat the Zollinger-Ellison syndrome. The approach is surgical in most cases, but the tumour is often impossible to remove and total gastrectomy has become rare with the progress in anti-secretory therapy. When the tumour behind a case of Zollinger-Ellison syndrome cannot be found, complementary chemotherapy is indicated. Other endocrine lesions must always be sought and the family investigated to exclude Wermer syndrome.
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PMID:[Multiple endocrine neoplasms associated with Zollinger-Ellison syndrome and hyperparathyroidism]. 615 41

During the period between 1967 and 1983, there were 28 WDHA patients in Japan. Clinically, these patients were not very different from those reported in western countries, but we have been able to add a few observations as follows. First, the WDHA patients with pancreatic endocrine tumors and those with neuroblastic tumors were present in almost equal numbers. Second, fluctuating hypercalcemia was often observed in patients with this syndrome, but it was not observed in any watery diarrhea due to the other etiologies. Third, the WDHA patients who have multiple endocrine neoplasia (MEN) type 1 have multiple tumors in the pancreas, indicating that the patients with MEN should be managed in a different manner in regard to determining tumor location as well as applying surgical treatment. When various types of tumors were examined for VIP content, it was found that many tumors have the potential to produce VIP, and when VIP is produced in large amounts, plasma VIP levels become elevated, resulting in the WDHA syndrome.
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PMID:The WDHA syndrome: clinical and laboratory data on 28 Japanese cases. 647 65

During a four-year period, 27 patients underwent total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm. In order to minimize the risk of persistent or recurrent hyperparathyroidism (HPT), a routine thymic resection and a wide excision of fat tissue around the parathyroids was performed to ensure excision of possible supernumerary glands or rudimentary parathyroid tissue. The indications for operation were HPT secondary to chronic renal failure in 24 patients (22 of whom had hypercalcaemia) and persistent or recurrent primary HPT in 2 cases. One further patient, who had a multiple endocrine neoplasia syndrome type I, underwent this procedure at the primary parathyroid operation. Preoperative hypercalcaemia was reversed in all patients but three during the first postoperative days, concomitantly with a fall in the parathyroid hormone (PTH) level. Fourteen patients showed marked hypocalcaemia postoperatively, necessitating calcium or vitamin D supplementation. This medication could later be discontinued in all of them. Thirteen patients, including two of those with primary HPT, never required any supplemental therapy. Survival of the grafts was documented by several observations. In all patients normal serum calcium values were being maintained without supplemental therapy at follow-up. During induced hypocalcaemia a PTH secretory response was demonstrated in all eight studied patients with a gradient between the grafted and non-grafted arm. In two patients in whom the grafts were examined histologically 19 and 28 months after the transplantation, viable parathyroid tissue was observed. In the initial part of the study excised tissue was cryopreserved. Since persistent hypocalcaemia did not occur in our patients, we have now abandoned this safety precaution. Thus, total parathyroidectomy with autotransplantation of parathyroid tissue is a valuable method for restoring long-term parathyroid function in patients with secondary HPT and uraemia. It also appears of value in selected cases of primary HPT.
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PMID:Parathyroid autotransplantation. An investigation of parathyroid autograft function. 665 65

Three patients with the watery diarrhea-hypokalemia-achlorhydria (WDHA) syndrome were studied. All had watery diarrhea, hypokalemia and hypercalcemia. Plasma vasoactive intestinal polypeptide (VIP) levels determined by radioimmunoassay were markedly elevated in these patients, indicating that they had VIP-producing tumors. Plasma VIP levels determined serially after the operation indicate that its determination is useful in estimating the effect of a treatment. As for multiple endocrine neoplasia type 1 (MEN1), two out of the three cases belonged to this category. Patient 1 had a brother with insulinoma, and in case 2, even though there was no family history, the autopsy revealed not only multiple tumors of the pancreas but also pituitary adenomas, chief cell hyperplasia of the parathyroid glands, thyroid adenomas and adrenocortical adenomas. VIP and other hormones in the tumors as well as in the plasma were examined extensively in these cases. In case 1, VIP, gastrin and calcitonin were produced in the tumor and only plasma VIP levels were elevated. In case 2, with multiple tumors, tumor 1 produced VIP, glucagon pancreatic polypeptide, gastrin and calcitonin, and tumor 2, VIP, pancreatic polypeptide, gastrin and beta-melanocyte stimulating hormone. In this case, plasma VIP, pancreatic polypeptide and glucagon levels were elevated. In case 3, VIP and calcitonin were produced in the tumor, and plasma VIP and calcitonin levels were elevated. These results indicate that (1) VIP is a good tumor marker for the WDHA syndrome due to VIP-producing tumors; (2) patients with the WDHA syndrome are sometimes associated with MEN1; and (3) VIP-producing tumors are multiple hormone-producing tumors, and VIP predominantly elevated in the plasma results in the WDHA syndrome, although other hormones such as pancreatic polypeptide, glucagon and calcitonin are sometimes found to be elevated in plasma without contributing to the clinical features.
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PMID:Clinical and hormonal aspects of the watery diarrhea-hypokalemia-achlorhydria (WDHA) syndrome due to vasoactive intestinal polypeptide (VIP)-producing tumor. 701 8

Four cases of neonatal severe primary hyperparathyroidism occurred in three families; familial hypocalciuric hypercalcemia was present in each kindred. The diagnosis of familial hypocalciuric hypercalcemia was based on the following features; hypercalcemia in many relatives (eight to 16 per kindred), without other features of the multiple endocrine neoplasia syndromes; recognition of hypercalcemia before the age of 10 in one to three relatives; hypocalciuric hypercalcemia in all relatives tested (five to 14 per kindred); and abnormal serum calcium levels despite parathyroidectomy in all additional relatives (one to five per kindred) undergoing this operation. The association of two uncommon syndromes (neonatal severe primary hyperparathyroidism and familial hypocalciuric hypercalcemia) in these kindreds suggests that the two syndromes share a common genetic cause within each kindred.
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PMID:An association between neonatal severe primary hyperparathyroidism and familial hypocalciuric hypercalcemia in three kindreds. 705 96

A series of 38 cases of primary hyperparathyroidism seen at a single hospital within a four a half year period is reported. The importance of hypercalcemia in the diagnosis of this syndrome and its screening in cases of arterial hypertension, gout, osteoporosis, and families with type I multiple endocrine neoplasia are underlined. The patients in the present series had a florid clinical history with a mean duration of 14 years. Main symptoms were urolithiasis (52%), arterial hypertension (28.9%), bone involvement and pain (23.7%), and peptic ulcer (18.4%). There were a high proportion of patients with hyperuricemia (26.3%), some with classical symptoms of gout. One patient presented simultaneous pituitary and pancreatic involvement. Surgical therapy was undertaken in 25 patients, of whom 24 (96%) were cured, one of them after reoperation. There were no cases of relapse, hypoparathyroidism, or postoperative death. Surgery is the only rational and definitive form of treatment of hyperparathyroidism; both experienced surgeons and pathologists are necessary to deal with the anatomic and histologic subtleties of this interesting endocrine disorder.
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PMID:[Comments on a series of 38 cases of primary hyperparathyroidism (author's transl)]. 724 69

Two women ahd acromegaly due to a pituitary adenoma associated with phaeochromocytoma. Eight additional patients with this combination of tumours have been described by others. Our first patient had sustained hypertension, mild hypercalcaemia, and elevated basal levels of parathyroid hormone and calcitonin associated with malignant phaeochromocytoma and parathyroid hyperplasia. The second patient had episodic hypertension and normal basal serum calcium, parathyroid hormone, and calcitonin levels with a benign cystic phaeochromocytoma. Four of the ten patients died from causes related to the phaeochromocytoma. Three patients had parathyroid hyperplasia. A separate group of four patients with phaeochromocytoma and pituitary adenoma without acromegaly has also been reported. These fourteen patients probably represent a non-familial variant of the multiple endocrine neoplasia syndrome. Our findings suggest that acromegalic patients with hypertension should be screened for phaeochromocytoma.
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PMID:Acromegaly and pituitary adenoma with phaeochromocytoma: a variant of multiple endocrine neoplasia. 729 6


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