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)

Severe cardiac arrhythmias (Lown class IVa), rapid loss of physical capacity and dyspnoea on the slightest exertion occurred in a 55-year-old man with idiopathic dilated cardiomyopathy. In the preceding year he had recurrent diarrhoea and lost 23 kg in weight. He was found to have hypercalcaemia (3-3.2 mmol/l). The heart failure significantly improved under treatment with twice daily 12.5 mg captopril, 100 mg spironolactone daily, furosemide 40 mg twice daily, and digitoxin 0.07 mg daily. The arrhythmia responded to verapamil 80 mg and quinidine 160 mg, both drugs three times daily. Primary hyperparathyroidism was found to be the cause of the hypercalcaemia (parathormone 84 pmol/l). After the parathyroid adenoma had been removed the patient's condition again improved markedly. There were only rare monotopic extrasystoles, cardiac size regressed, and diuretics were no longer necessary. His medication at present is verapamil (80 mg three times daily), captopril (12.5 mg three times daily) and digitoxin (0.07 mg daily). It is concluded that the hypercalcaemia influenced the severity of the cardiomyopathy. It would seem that both intra- and extracellular calcium homoeostasis is of great importance in dilated cardiomyopathy.
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PMID:[The coincidence of rapidly progressing dilated cardiomyopathy and primary hyperparathyroidism. The course before and after the removal of a parathyroid adenoma]. 173 86

A 34 year old male was hospitalized because of severe abdominal pain and diarrhea. An abdominal X-ray revealed multiple calculi in the head of pancreas and blood tests showed his serum calcium level to be high. He underwent surgery of the parathyroid gland and a parathyroid tumor was removed. Two months later, resection of the head of the pancreas was also performed. Eighteen months after his operation there has been no recurrence of abdominal pain or diarrhea and his serum calcium level is within the normal range. We report this case herein and also discuss the possible cause and effect relationship between primary hyperparathyroidism and pancreatitis, and the appropriate management, in relation to a review of the literature.
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PMID:Chronic calcifying pancreatitis associated with primary hyperparathyroidism--report of a case and review of the literature. 208 96

We studied 25-year-old HLA- and blood group-identical male twins who had multiple endocrine neoplasia, type I (MEN I). At the time of initial examination, one twin (case 1) had epigastric pain and diarrhea; he was cushingoid in appearance. Further evaluation revealed primary hyperparathyroidism, Zollinger-Ellison syndrome, Cushing's disease, and hyperprolactinemia. Immunostaining of a resected pituitary specimen demonstrated both prolactin and, to a lesser extent, growth hormone reactivity. The nontumorous adenohypophysis showed corticotropic hyperplasia. In contrast, the other twin (case 2) was asymptomatic. He had only primary hyperparathyroidism and hyperprolactinemia. An invasive pituitary adenoma was resected and showed similar proportions of cells with immunoreactive prolactin and those with growth hormone; no nontumorous gland was available for study. Apparently, factors other than heredity may play a role in the expression of MEN I.
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PMID:Nonidentical expressions of multiple endocrine neoplasia, type I, in identical twins. 287 27

Two cases of primary hyperparathyroidism in pregnancy are described. One was treated by parathyroidectomy in the late third trimester with good outcome. It appears that parathyroidectomy, although traditionally performed in the second trimester, may in selected cases be a reasonable treatment option in late pregnancy. The other patient was treated successfully with large doses of oral phosphosoda. Hypokalemia in association with this form of therapy, and not related to diarrhea or other known etiologies, is described for the first time.
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PMID:Hyperparathyroidism in pregnancy. 362 2

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

About 25% of patients with ZES have MEN-1. Except for diarrhoea, less frequent in patients with ZES MEN-1 than in sporadic ZES, and specific MEN-1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin level are also similar whether in the basal state or after secretin. Primary hyperparathyroidism (pHPT) exists in the majority of ZES MEN-1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25-30% have ECLomas: bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumours. The spread of the disease metastases to the liver (LM), mediastinum, bones, is evaluated best by Octreoscan. Associated endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in cases of associated life-threatening conditions such as insulinoma. Although the size of the tumour, when located in the pancreas >3 cm, favours metachronous LM occurrence, surgery in our experience has not been able to prevent LM development.
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PMID:Diagnostic and therapeutic criteria in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. 968 47

We report a case of a 35-year-old male, with a history of diarrhea, renal lithiasis with frequent expulsions of calculus and hypercalcemia during the last 2 years. The patient was studied and diagnosed with a multiple endocrine neoplasia type I (MEN I), familiar (mother with MEN I). A scintigraphic study with 99mTc-MIBI was performed in order to localize hyperfunctioning parathyroid glands because of biochemical diagnosis of primary hyperparathyroidism. Double phase 99mTc-MIBI scan detected one hyperfunctioning parathyroid gland and a large anterior mediastinal mass. Subsequent, plain radiograph and CT of the chest showed a soft-tissue mass in that localization. Punch biopsy of the lesion guided by CT revealed malignant cells of neuroendocrine tumor. The tumor was removed and histologically confirmed as a carcinoid within a thymus in a MEN type I syndrome. MEN I patients can benefit from the examination with this agent which can potentially localize not only parathyroid endocrine pathology but also unknown associated tumors.
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PMID:Thymic carcinoid and parathyroid hyperplasia detection with 99mTc-MIBI men type 1. 1061 32

In 65 jejunolleal (JI) bypasses done from 1973-1979, there were nine Scott and 56 Payne (with Y-shaped anastomosis). Preoperative excess body weight (EBW) translated to the 1983 Metropolitan Tables was 112 +/- 30%. Eight patients are lost to follow-up. We reversed seven patients for renal stones (12%) accompanied by a vertical banded gastroplasty (VBG) and one because she demanded a VBG. Five patients were reversed by surgeons elsewhere for minor problems (three with an accompanying gastric reduction operation), and all five regained and requested a JI bypass again, which we now refused to undertake. This leaves 44 JI bypass patients being followed: loss of EBW is 71 +/- 22% at 12-18 years. The eight reversed by us accompanied by a VBG regained some weight (loss of EBW from initial weight is 56 +/- 18%). Liver biopsies were done for 5 years in 31 patients, and showed improvement by 36 months. Patients took predigested collagen capsules plus high protein and multivitamins. Injections of B12 are indicated in 18 patients, given every 3 months. Liver dysfunction has not occurred in the long-term. Low serum carotene levels persist. Migratory arthraigias were controlled by oral metronidazole and did not occur after the fifth year. Oxalate crystals remain on urinalysis. Potassium and magnesium replacement is not required now, and a mean of 2.5 stools per day is not a problem, with infrequent diarrhea after greasy foods. Metronidazole is continued in 33 patients to prevent foul flatus. One patient developed a brain tumor, one myxedema, and one primary hyperparathyroidism, thought to be complications of the bypass until diagnosed. Most patients appear to be doing well.
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PMID:Long-term Outcome in a Series of Jejunoileal Bypass Patients. 1075 27

About 25% of patients with ZES have MEN 1. Except diarrhoea, less frequent in patients with ZES-MEN 1 than in sporadic ZES, and specific MEN 1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin levels are also similar whether in the basal state or after secretin stimulation. Primary hyperparathyroidism (PHPT) exists in the majority of ZES-MEN 1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25 to 30% have EC-Lomas; bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumors. The spread of the disease: metastases to the liver (LM), mediastinum, bones, is evaluated at best by Octreoscan. Endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in case of associated life-threatening condition such as insulinoma and has been advocated to prevent LM development in patients with large pancreatic tumor(s). However although, indeed, the size of the tumor, when located in the pancreas > 3 cm, favours metachronous LM occurrence, surgery, in our experience, has not been able to prevent LM development. Hepatic malignancies remain however the most pejorative prognostic determinant for survival and raise the most difficult therapeutic challenge. Surgery is the best option whenever feasible; specific chemotherapy and chemo-embolisation have not conclusively achieved definite successes. Long-term octreotide treatment, however, has been shown recently to obtain tumour stabilisation. Internal irradiation with 90 Ytrium-labelled octreotide is a new promising option, presently under evaluation (Novartis European trial). Preliminary results are promising.
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PMID:[Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. 1514 2

Parathyroid carcinoma constitutes less than 1 % of primary hyperparathyroidism. The male to female ratio is approximately equal and the mean age at presentation is 40 years. In about half of the patients there is a palpable cervical mass, and serum calcium level is usually above 14 mg/dl. In a case report we present a 21-year-old man with a non-tender, non-mobile bulging mandibular mass. He suffered from fatigue, recent weight loss, hoarseness, polydipsia, polyuria, hematuria, recurrent renal stones and bouts of constipation and diarrhea. A mandibular biopsy confirmed brown tumor. Serum calcium level was 15.4 mg/dl. Cervical ultrasound revealed a hypoecho area suspicious of parathyroid adenoma. Parathyroid carcinoma was later confirmed and en bloc resection was performed. During a two-year follow up there has been no evidence of recurrence or hypercalcemia. This unique case of parathyroid carcinoma in conjunction with brown tumor is the second reported case worldwide.
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PMID:Parathyroid carcinoma: A rare case with mandibular brown tumor. 1677 84


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