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)

In vitro studies have demonstrated that secretin can stimulate the release of parathyroid hormone (PTH), but reports concerning its effects on PTH and calcium in vivo are contradictory. To examine this question further, a bolus injection of secretin (75 IU) was given to 12 normal subjects and 10 patients with primary hyperparathyroidism (HPT). Six of the patients had multiple endocrine neoplasia and five had endocrine pancreatic tumours (EPT). Three normocalcaemic patients with EPT were also included in the study. The mean serum gastrin level rose significantly (from 19 to 40 pmol/l, p less than 0.01) within 15 min of secretin injection in the normal subjects. HPT patients without EPT had a somewhat higher mean basal level of gastrin (39 pmol/l, p less than 0.05 compared with controls), but it did not increase significantly after the secretin bolus. In six EPT patients the gastrin concentrations rose by more than 300 pmol/l. Although secretin had a biological capacity to release gastrin, it had no discernible effects on either serum PTH or serum calcium in any of the groups studied. Nor were any changes in PTH or calcium observed when secretin was given as a continuous infusion (3 IU/kg/h) over 90 min. Thus, our data do not support the concept that secretin, in vivo, is a secretagogue of PTH.
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PMID:Effects of secretin on parathyroid hormone and calcium in normal subjects, patients with hyperparathyroidism and patients with gastrinoma. 399 34

It has been suggested that primary hyperparathyroidism (HPT) may occasionally be caused by "multiple adenomas." If this is true, then selective excision of the enlarged glands should be curative in such cases. To examine this proposition, we reviewed the records of 1,962 patients with surgically proved HPT and culled all those who met the following criteria: (1) identification and excision of two enlarged and histologically hypercellular parathyroid glands, each weighing more than 70 mg, and (2) identification and preservation of two other normal-sized parathyroid glands. There were 38 such patients (1.9% of the total group). Thirty-seven patients were euparathyroid at follow-up (an average of 4.6 years postoperatively). Only one individual, a member of a kindred with multiple endocrine neoplasia (MEN), type 1, had persistent HPT; but four other patients with MEN syndromes were among the group cured by surgery. The findings of this study support the notion that more than one adenoma may occasionally be found in primary HPT, and that selective excision of the enlarged glands with preservation of the normal parathyroid glands constitutes adequate treatment. One possible exception to this rule is the patient with either familial HPT or one of the MEN syndromes; subtotal parathyroidectomy is preferred in this situation.
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PMID:Parathyroid "double adenomas": fact of fiction? 611 78

In about 80% of the cases, primary hyperparathyroidism (pHPT) is caused by a single parathyroid adenoma. However, the disease may be complicated by involvement of more than one parathyroid gland or by the combination with other endocrine tumors (syndrome of multiple endocrine neoplasia = MEN). This presentation deals with our experience in such conditions. During 11 years, 98 cases of pHPT were seen (90 in Ulm from 1968 to 1979, 8 since then in Heidelberg). In 9 patients, 2 to 4 parathyroids were in hyperfunction. A recurrence of pHPT was diagnosed after symptomfree intervals of 2 - 13 years in 5 patients. Data are presented of 4 patients suffering from MEN type I (Wermer syndrome): 3 had Zollinger-Ellison syndrome and pHPT, and the 4th insulinoma and pHPT. Whereas pHPT is the most frequent endocrinopathy in MEN type I, it is rarely seen in MEN type II, the Sipple syndrome (combination of medullary thyroid carcinoma, MTC, and pheochromocytoma). Among 20 own cases with MTC and 10 others with pheochromocytoma, no pHPT was observed. The common basis for the development of MEN syndromes is Pearse's concept of the diffuse neuroendocrine system (DNES).
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PMID:Primary hyperparathyroidism and multiple endocrine neoplasia (MEN). 612 57

In approximately 20% of the cases the Zollinger-Ellison syndrome (ZES) is associated with primary hyperparathyroidism (HPT). In view of this frequent association, serum calcium and phosphorus levels should be measured in all patients with ZES. Conversely, all patients with HPT I accompanied or preceded by peptic ulcer and/or diarrhoea should have their gastric acid secretion and serum gastrin level measured. Since the association may reflect a type I multiple endocrine neoplasia (MENI), involvement of other endocrine systems, notably the pituitary gland, should be investigated in the patients and their family. A rise in basal plasma pancreatic polypeptide has been observed in about 50% of cases of familial MEN I (Wermer's syndrome) and appears to be a good index of pancreatic endocrine tumour. When ZES is associated with HPT I, the latter should be treated first for three reasons: (7) lethal acute hypercalcaemia may occur after abdominal surgery; (2) HPT I itself may increase the gastric acid secretion and hypergastrinaemia of the ZES, and (3) parathyroidectomy and medical treatment with gastric antisecretory drugs may postpone the need for total gastrectomy.
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PMID:[Hyperparathyroidism associated with Zollinger-Ellison syndrome. 4 cases (author's transl)]. 612 5

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

Circulating human pancreatic polypeptide (hPP) concentrations in the plasma of 61 patients were determined by radioimmunoassay and compared with concentrations in normal age-matched subjects to assess the role of plasma hPP in diagnosis and detection of pathologic dysplasias in endocrinopathies. Basal fasting plasma hPP concentrations greater than 3.0 times normal values were found in six of six patients with multiple endocrine adenopathy syndrome, type I (MEA I) who had islet cell tumors that contained hPP and in only three of 15 nonfamilial patients with sporadic islet cell tumors. An exaggerated plasma hPP response to meal stimulation that exceeded greater than 4.5 times the basal value was found in 15 of 18 patients with MEA I, which indicated the presence of endocrine cell hyperplasia as the underlying genetic trait. The abnormal plasma hPP response to meal stimulation correlated most strongly with islet cell hyperplasia in both the genetic and sporadic endocrinopathies and to some extent with antral G cell hyperplasia. In patients with primary hyperparathyroidism, an exaggerated plasma hPP response to meal stimulation is highly indicative of genetic parathyroid hyperplasia in MEA I and not of sporadic parathyroid adenoma. Informed consent was obtained from all patients who were subjects of these investigations.
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PMID:Pancreatic polypeptide update: its roles in detection of the trait for multiple endocrine adenopathy syndrome, type I and pancreatic polypeptide-secreting tumors. 613 85

Five members of one family had been operated on for primary hyperparathyroidism. One of them also had Cushing's disease (i.e. pituitary tumor). An association between familial hyperparathyroidism and Cushing's disease is quite unusual. Such a combination of rare diseases is not fortuitous though; it probably is but a special type of multiple endocrine neoplasia.
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PMID:[Association of familial hyperparathyroidism and Cushing's disease]. 615 May 31

The concentration of subunits of hCG (hCG alpha and hCG beta) was determined in plasma or serum from 70 patients with primary hyperparathyroidism (1 degrees HPT). Two of three patients with parathyroid carcinoma showed elevation in plasma concentrations of both subunits, which fell after surgical removal of the tumor. An extract prepared from the tumor of 1 of these patients contained the subunits in high concentrations, whereas in extracts similarly prepared from tissues removed from 7 patients with benign 1 degrees HPT, the subunits were not detectable or were present in much lower concentrations. In 42 cases of benign 1 degrees HPT, samples from veins containing a 10-fold gradient of parathyroid hormone obtained during selective venous catheterization and peripheral samples from the same patients were analyzed for hCG subunits. Only 1 patient demonstrated a mild elevation of hCG alpha in parathyroid venous effluent alone that may have represented subunit release by apparently benign parathyroid tissue. Thirty patients with multiple endocrine neoplasia type I were tested; 8 evidenced clinically active islet cell tumors, and 6 of these 8 showed high circulating concentrations of hCG alpha (and hCG beta in 1 case). Patients in the multiple endocrine neoplasia type I group with 1 degrees HPT or pituitary tumors, but no evident pancreatic islet disease, did not show elevations in subunit concentrations. Thus, in patients with 1 degrees HPT, determination of subunits of hCG may be helpful in making the diagnosis of parathyroid carcinoma or in screening for associated (and probably malignant) pancreatic islet disease.
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PMID:Human chorionic gonadotropin subunit measurement in primary hyperparathyroidism. 627 98

We have experienced 186 patients with proven primary hyperparathyroidism operated on during past 16 years. In this series, 147 patients (79%) had adenoma, 24 patients (12.9%) had carcinoma and only 15 (8.1%) had hyperplasia. Because of a 92% of single gland involvement, the removal of an enlarged gland is adequate, if the remaining gland are grossly normal. Besides, interestingly enough, an incidence of parathyroid carcinoma is rather high in Japan, so that surgeons should be alert to recognize the parathyroid carcinoma on the basis of clinical and operative findings. In an effort to minimize recurrence, en bloc resection of the parathyroid carcinoma is important. When a patient with parathyroid carcinoma has a local recurrence or distant metastases, an aggressive surgical approach is recommended to alleviate hypercalcemic symptoms. The majority of patients with hyperplasia belong to multiple endocrine neoplasia, type 1. Recent development of the non-invasive diagnostic methods is remarkable. The last consecutive 31 patients underwent preoperative localization study by 201Thallium (201TI-CI) scanning and ultrasonography. 201TI-CI scanning gave the most accurate results and 100% correct localization was obtained when the parathyroid tumor was more than 1 g. However, it is noteworthy that the parathyroid tumor was less than 1 g in 64% of patients who had no evidence of generalized fibrous osteitis.
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PMID:[Surgical management of primary hyperparathyroidism]. 650 56

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


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