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)

A 62-year-old woman had primary hyperparathyroidism due to parathyroid carcinoma. In spite of surgical removal of the enlarged parathyroid gland, intractable hypercalcemia persisted. 99mTc-methoxyisobutylisonitrile scanning revealed bone metastatic lesions, although conventional 201T1-chloride scanning failed to demonstrate those lesions. Octreotide inhibited parathyroid hormone (PTH) secretion, although various means such as bisphosphonates, calcitonin and hydration were effective to reduce serum calcium level to some extent but failed to reduce PTH secretion at all. The CT-guided transcutaneous tumor marking facilitated the subsequent orthopedic surgery for successful removal of the metastatic focus of PTH secretion from the iliac bone.
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PMID:Successful treatment by surgical removal of bone metastasis producing PTH: new approach to the management of metastatic parathyroid carcinoma. 784 85

No satisfactory medical therapy exists for the treatment of primary hyperparathyroidism. Calcitonin and diphosphonates do not give good long-term control. This work examines the efficacy of the somatostatin analogue octreotide in the management of hyperparathyroidism. Twenty-one patients were admitted before parathyroid surgery and were treated for 6 days with subcutaneous octreotide 100 micrograms twice daily. Fasting blood samples were taken for determination of serum levels of calcium and parathyroid hormone, and 24-h urinary calcium excretion was measured before and after treatment. A significant decrease in urinary calcium was demonstrated, but the reduction in serum calcium level was not statistically significant and there was no change in serum parathyroid hormone concentration. Octreotide may provide therapy for patients with hyperparathyroidism and for those who have undergone unsuccessful neck exploration or who are unfit for operation. It may ameliorate hypercalciuria and reduce stone formation. The octapeptide may also have a potential role as a diagnostic test in primary hyperparathyroidism by determining the symptomatic effect of reducing raised levels of serum and urinary calcium.
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PMID:Somatostatin and primary hyperparathyroidism. 795 42

Multiple endocrine neoplasia type 2 (MEN 2) is a rare syndrome of medullary thyroid carcinoma (MTC) with pheochromocytoma and/or primary hyperparathyroidism (PHP), usually due to multigland hyperplasia. MEN 2 is associated with several RET protooncogene mutations. A 61-year-old woman with a family history of RET-positive MTC presented with a solitary thyroid nodule. Fine-needle aspiration biopsy was suspicious for neoplasm. Biochemical studies revealed basal hypercalcitoninemia (116 pg/mL [normal <26]) and PHP (serum calcium, 10.9 mg/dL; intact PTH, 113.2 pg/mL [10.0-65.0]). Pheochromocytoma screening was negative. A provisional diagnosis of MEN 2 was made, but at surgery, a single parathyroid adenoma was resected and frozen sections of several lymph nodes revealed papillary thyroid carcinoma (PTC). A total thyroidectomy was performed. Final histological diagnosis was PTC and parathyroid adenoma with no evidence of MTC. Postoperatively, RET mutation testing was positive. The basal calcitonin (CT) fell to 25 pg/mL, but peaked at 935 (normal <105) after pentagastrin infusion, consistent with occult MTC. After radioiodine ablation, CT decreased further. Octreotide scanning was negative. Faced with PHP, a thyroid nodule, and a family history of MTC, clinicians tend to diagnose MEN 2. This patient had a single parathyroid adenoma and nonmedullary thyroid cancer, which the literature actually suggests to be an association more frequent than MEN 2. Yet, there remains compelling data in favor of occult MTC, leaving open the possibility of an MEN 2 variant with the rare association of PTC.
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PMID:Papillary thyroid carcinoma, parathyroid adenoma, and unexplained hypercalcitoninemia: an unusual presentation of multiple endocrine neoplasia type 2A? 977 49

A 30-year-old woman had a history of prolactinoma and primary hyperparathyroidism. She was diagnosed as having multiple endocrine neoplasia type 1 with gastrinoma and liver metastases. Octreotide therapy was started and the serum gastrin level decreased immediately. Octreotide continued to suppress gastrin secretion over the next 7 years. The Ki67/MIB1 proliferation index of this tumor was only 0.5 % and somatostatin receptor (SSTR) 2 expression was very strong in both 2002 and 2009. This case suggests the importance of investigating the Ki67/MIB1 index and SSTR expression in patients with metastatic gastrinoma.
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PMID:Long-term suppressive effect of octreotide on progression of metastatic gastrinoma with multiple endocrine neoplasia type 1: seven-year follow up. 2082 41