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)

The diagnostic value of pre-operative laboratory tests and current imaging methods was evaluated in a series of 65 patients operated upon for primary hyperparathyroidism. Among laboratory tests, nephrogenous cyclic AMP and PTH serum levels had sensitivities of 100% and 71.7% respectively. Total urinary cyclic AMP and bone biopsy were much less reliable. Imaging procedures were mainly real-time ultrasonography and thallium-technetium substraction scintigraphy with sensitivities of 50.8% and 46.7% and specificities of 86.3 and 76.3% respectively. Failure of cervical exploration occurred in only one case and was attributed to an incorrect diagnosis (1.5%). Thus the selective use of these preoperative investigations may be envisaged in the following cases: hypercalcemic crises, highly probable PHP, uncertain PHP and in secondary surgical exploration.
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PMID:[Diagnosis and localization of primary hyperparathyroidism]. 282 47

Parathyroid hormone (PTH) is strongly concerned with the pathogenesis of urinary stones. PTH is mainly regulated by the serum calcium concentration and not by other hormones, as is usually the case. We studied whether PTH is also regulated by adrenocorticotrophic hormone (ACTH) or not. ACTH (0.25 mg) was injected intravenously to 17 patients with primary hyperparathyroidism PHP, 7 patients with urolithiasis, 7 patients with malignant hypercalcemia, and 6 control subjects. Serum calcium was significantly increased in only PHP. The serum calcium increase rate showed a significant positive correlation with serum alkaline phosphatase, and a negative correlation with the preinjected serum calcium. PTH was slightly increased in all four groups. Serum cortisol and ACTH concentrations were not significantly different among the groups. PTH concentration in a culture medium of parathyroid tissues increased after ACTH addition. Serum calcium was significantly increased after ACTH injection in an adrenalectomized rat, and decreased in a parathyroidectomized rat. From our data and those of others, it appears that ACTH acts on the adrenal glands to decrease the serum calcium concentration, and might act directly on the parathyroid gland or bones to increase it.
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PMID:[Studies on the endocrinological metabolism of the parathyroid. II. Influence of ACTH on parathyroid function and calcium metabolism]. 300 38

Baseline levels and increases in urinary cyclic AMP excretion (UcAMP) and immunoreactive parathormone (iPTH) were studied before and during infusion of EDTA in euparathyroid patients with renal stones (n=11), patients with primary hyperparathyroidism (PHP; n=14) and patients with vitamin D deficiency (n = 12). In all three groups, EDTA evoked a significant rise in iPTH and UcAMP. In patients with PHP and in those with vitamin D deficiency, there was a sufficiently close relationship between increments in iPTH (delta iPTH) and in UcAMP (delta UcAMP) (r = 0.90, P less than 0.001 and r = 0.67, P less than 0.02, respectively) to use this model to assess renal sensitivity for changes to endogenous PTH levels. We quantified sensitivity of the kidney for PTH, by calculating the ratio delta UcAMP/delta TPTH for the three studied groups. The ratio was comparable in patients with renal stones (16.7 +/- 10.3) and PHP (13.8 +/- 4.9, P greater than 0.10), but was significantly increased in patients with vitamin D deficiency (33.2 +/- 17.9; P less than 0.01 versus patients with renal stones and P less than 0.01 versus patients with PHP). Within the group of patients with PHP there was no correlation between baseline serum calcium concentrations and the ratio delta UcAMP/delta TPTH. It is concluded that in patients with vitamin D deficiency, renal sensitivity to PTH is increased compared with patients with PHP and euparathyroid patients with renal stones, perhaps an expression of a teleological useful adaptation of end organ sensitivity.
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PMID:The renal sensitivity for endogenous parathormone in patients with primary hyperparathyroidism, vitamin D deficiency and renal stones. 301 31

The basal values of plasma vitamin D metabolites were evaluated in patients with primary hyperparathyroidism (1 degree HPT, n = 31), hypoparathyroidism (HP, n = 7), pseudohypoparathyroidism (PHP, n = 4) and normal controls (n = 21). Plasma 25-hydroxyvitamin D (25-OH-D) in 1 degree HPT (9.0 +/0 7.3 ng/ml, mean SD) was significantly lower than that of normal controls (17.9 +/- 5.5ng/ml)(p less than 0.001), and in particular 1 degree HPT classified as the skeletal type showed extremely low value (4.7 +/- 4.6 ng/ml). Plasma 1, 25-dihydroxyvitamin D [1, 25-(OH)2D] was significantly higher in 1 degree HPT (69.1 +/- 31.4pg/ml)(p less than 0.001) and significantly lower in Hp (15.2 +/- 11.0 pg/ml) (p less than 0.001) compared to normal controls (37.2 +/- 13.8pg/ml), although there was no significant difference in PHP (22.3 +/- 17.5 pg/ml). Plasma 24, 25-dihydroxyvitamin D [24,, 25-(OH)2D] in 1 degree HPT (1.06 +/- 0.55 ng/ml) was significantly lower than that of normal controls (1.73 +/- 0.62 ng/ml) (p less than 0.05), and particularly 1 degree HPT classified as the skeletal type showed a marked low value (0.85 +/- 0.27 ng/ml), whereas no significant differences were seen in HP (1.84 +/- 0.46 ng/ml) or PHP (1.34 +/- 0.22 ng/ml). There were slight but significant correlations between either plasma 25-OH-D and 1, 25-(OH)2D (r = -0.350, p less than 0.05), or plasma 25-OH-D and 24, 25-(OH)2D (r = 0.356, p less than 0.05), or plasma 1, 25-(OH)2D and 24, 25-(OH)2D (r = -0.444, p less than 0.01) in all subjects. In addition, relationships between plasma vitamin D metabolites and other indicators of parathyroid function in all subjects were analyzed. There were positive correlations between plasma 1, 25-(OH)2D and serum Ca (r = -0.621, p less than 0.001) or urinary cAMP (r = -0.671, p less than 0.001) or nephrogenous cAMP (r = -0.689, p less than 0.001), while negative correlations were seen between plasma 1, 25-(OH)2D and serum P (r = -0.680, p less than 0.001) or %TRP (r = -0.663, p less than 0.001). On the other hand, there were negative correlations between plasma 24, 25-(OH)2D and serum Ca (r = -0.457, p less than 0.01) or urinary cAMP (r = -0.562, p less than 0.005) or nephrogenous cAMp (r = -0.561, p less than 0.005), and a positive correlation was seen between plasma 24, 25-(OH)2D and %TRP (r = 0.519, p less than 0.005). After parathyroidectomy, a distinct depression of plasma 1, 25-(OH)2D and reciprocal elevation of plasma 24, 25-(OH)2D were observed in 1 degree HPT. Furthermore, there was a clear elevation of plasma 24, 25-(OH)2D as well as plasma 1, 25-(OH)2D after treatment with maintenance doses of 1 alpha-OH-D3 or 1 alpha, 25-(OH)2D3 in HP and PHP. It is concluded that plasma vitamin D metabolites are very useful as the indicators of parathyroid function.
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PMID:[Plasma vitamin D metabolites in parathyroid diseases (author's transl)]. 698 Jan 45

Hypercalcemia and a relative nonsuppressibility of parathyroid hormone by calcium are the biochemical hallmarks of primary hyperparathyroidism. An elevated PTH level should be defined in a highly sensitive and specific test system. Primary HPT is the single major cause of hypercalcemia in outpatients. A definitive medical option to manage PHP does not exist. Surgical neck exploration by an experienced surgeon has a very high success rate for cure in both symptomatic and asymptomatic disease forms. Secondary HPT is mostly due to renal failure. Nonsuppressibility of PTH by use of vitamin D sterols indicates parathyroid gland hyperplasia and autonomy of PTH secretion. Surgery should correct PTH excess and should lead to an optimal bone status.
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PMID:[Clinical and biochemical diagnosis of pHPT and sHPT, surgical indications from the internal medicine viewpoint]. 1182 51

Adrenomedullin (ADM) is a new potent vasorelaxant peptide identified originally in extracts of pheochromocytoma, and is widely distributed within the tissue. Although histopathological studies have demonstrated the presence of ADM-immunoreactivity (ir-ADM) in some human neuroendocrine tumors (such as insulinoma, pituitary adenoma, and gastrointestinal neuroendocrine tumors), data on the presence of ADM in normal and pathological parathyroid gland are not available. Plasma AM concentrations were recently reported to be elevated in patients with PHP (primary hyperparathyroidism). The aim of our study was to determine tissue distribution of ir-AM in 34 patients with PHP (27 female and 7 male, mean age 50 +/- 6 years) undergoing surgery. Six normal parathyroid samples incidentally found during thyroidectomy for neoplastic diseases and ten sections of human rectus abdominis muscle tissue were used as controls (C). Adenomatous parathyroids were found in 22 PHP and hyperplastic parathyroids in twelve PHP patients. Four hyperplastic parathyroids were found in three PHP patients and three parathyroids in 10 PHP patients. Eight parathyroids revealed a prevalent diffuse growth pattern and four showed a prevalent nodular growth pattern. Immunohistochemical ADM expression was seen in seven of twelve (58.3 %) hyperplastic parathyroids and in fourteen of twenty-two (66.6 %) adenomatous glands. Parathyroid chief cells showed strong cytoplasmatic staining, whereas oncocytic cells showed a faintly aspecific cytoplasmatic staining. Normal parathyroids were negative for ir-ADM. In conclusion, we found the presence of ADM in parathyroid chief cells of PHP patients using immunohistochemistry in our study.
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PMID:Adrenomedullin immunoreactivity tissue distribution in parathyroids of the patients with primary hyperparathyroidism. 1530 32

Primary hyperparathyroidism (PHP; serum calcium 2.75 mmol/L, PTH 226 pg/ml) had been the first clinical manifestation of MEN-2A in a female patient (aged 55 years) with a mutation (Y791F, TAT-->TTT) in exon 13 of the RET proto-oncogene. The patient has a pentagastrin-induced rise in serum calcitonin (up to 57 pg/ml) considered normal for noncarriers but abnormal in family members of MEN-2 patients. This is the first case of MEN-2 due to this specific mutation with primary hyperparathyroidism as the first manifestation of the disease. In addition, the patient harbored, within the Menin gene, a polymorphism (D418D) reportedly associated with sporadic primary hyperparathyroidism. This case report indicates that molecular biological tests in MEN- 2 may only suggest a certain phenotype but cannot predict it with certainty. It may also suggest that genetic screening for MEN-2 may be advisable in patients with primary hyperparathyroidism and a borderline-high pentagastrin stimulation test, even in the absence of a positive family history.
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PMID:Primary hyperparathyroidism as the leading symptom in a patient with a Y791F RET mutation. 1635 97

Objective. To report a case of primary hyperparathyroidism in a pregnant patient, report the obstetric and neonatal outcomes, and review the relevant literature. Results. A 29-year-old primigravida was successfully treated for PHP with minimally invasive resection of a parathyroid adenoma in the second trimester of pregnancy. A healthy baby girl was delivered at 37-week gestation with an unremarkable neonatal course. To the best of our knowledge, this is the second case report in the literature utilizing intraoperative PTH during a parathyroidectomy in a pregnant woman. Conclusions. Primary hyperparathyroidism is a rare life-threatening condition that can present during pregnancy. The diagnosis can be difficult to establish during pregnancy, given the nonspecific symptoms related to hypercalcemia. However, a better understanding of the condition, improved diagnostic studies, and well-organized multidisciplinary management decisions can significantly reduce the morbidity and mortality associated with the disease during pregnancy. This case report is presented to highlight the value of early diagnosis and appropriate management of PHP during pregnancy.
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PMID:Primary hyperparathyroidism in pregnancy-a rare cause of life-threatening hypercalcemia: case report and literature review. 2293 84