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)

Primary hyperparathyroidism (pHPT) is associated with osteopenia. However, the individual variation in recovery in bone mass after surgery is large. Therefore, modes of prediction of the increase in bone mass after parathyroid surgery were investigated. Preoperatively and at one year after surgery bone mineral content (BMC) in the distal radius was measured with single photon absorptiometry technique in 40 patients with pHPT. Serum levels of calcium, intact parathyroid hormone (PTH), alkaline phosphatase, osteocalcin and Vitamin D metabolites were also determined. Preoperatively, Z-score of BMC was -0.85 +/- 1.20 SD below the normal mean. There was a modest association between BMC and serum levels of osteocalcin (r = -0.34; P < 0.05), and dihydroxycholecalciferol (r = -0.35; P < 0.05). At one year after surgery, mean BMC increased by 2% (P < 0.05), but with a wide dispersion. Preoperative Z-score of BMC correlated with the relative change in BMC (r = -0.33; P < 0.05). An increase in BMC with 95% confidence was evident in 10 of the patients. None of these patients had a preoperative Z-score of BMC above the mean expected for age and sex. We conclude that the increase in bone mass after surgery for pHPT is small and evident only in a portion (approximately 25%) of patients. Hence, a decrease in bone mass should not be a major indication for surgery in pHPT.
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PMID:Prediction of changes in bone density after operation for primary hyperparathyroidism. 812 72

A 27-year-old man presented to his dentist with a swelling in his lower jaw. Histology revealed this to be a brown tumour associated with primary hyperparathyroidism and severe but asymptomatic hypercalcaemia. A large parathyroid adenoma was removed and the serum calcium fell to normal. Hypercalcaemia recurred and re-exploration of the neck revealed parathyroid metastases in cervical lymph nodes. A modified radical neck dissection was performed and he has remained normocalcaemic on Vitamin D analogues for 2 years. Bone disease of the mandible is a very rare presentation of primary hyperparathyroidism. The diagnosis of parathyroid malignancy is often difficult histologically, and the optimum treatment is uncertain.
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PMID:Parathyroid carcinoma presenting with a brown tumour of the mandible in a young man. 847 74

Persistent hypercalcemia attributable to parathyroid gland hyperplasia was identified in 6 dogs with primary hyperparathyroidism. Clinical signs included polydipsia (n = 4), polyuria (n = 4), and signs caused by cystic calculi (n = 3). Abnormal clinical pathologic findings included hypercalcemia (mean, 13.6 mg/dl; range, 12.6 to 14.7 mg/dl; n = 6), hypophosphatemia (mean, 2.2 mg/dl; range, 1.4 to 2.9 mg/dl; n = 6), high serum alkaline phosphatase activity (mean, 222 IU/L; range, 161 to 286 IU/L; n = 3), and isosthenuria (mean, 1.012; range, 1.006 to 1.017; n = 6). Serum parathyroid hormone concentration was within the reference range or high (mean, 23 pmol/L; range, 7 to 119 pmol/L; reference range, 1.5 to 13 pmol/L) in all dogs. At surgery, the number of large parathyroid glands was variable, being limited to 1 gland in 3 dogs, 2 glands in 2 dogs, and 4 glands in 1 dog. All visibly large parathyroid glands were surgically removed from each dog. Serum calcium concentration decreased into or below the reference range within 72 hours of surgery in all dogs, confirming the diagnosis of primary parathyroid disease. Multiple nodules of adenomatous hyperplasia were identified in each dog. All 6 dogs were treated with vitamin D and calcium carbonate following surgery. The dog from which all 4 parathyroid glands were removed has remained eucalcemic for more than 1 year with vitamin D supplementation. Vitamin D and calcium administration was discontinued within 4 to 12 weeks of surgery in the remaining 5 dogs. These dogs remained eucalcemic without vitamin D supplementation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Primary parathyroid gland hyperplasia in dogs: six cases (1982-1991). 847 30

During the period 1983-1995, 200 chronic renal failure patients (115 males and 85 females) were parathyroidectomized for hyperparathyroidism in our Department. In all of them, the presenting clinical symptoms, physical signs, biochemical and radiological tests were typically those of hyperparathyroidism. One hundred ninety patients were operated for the first time whereas 10 were re-operated due to relapse of the disease; 3 of these cases were primary hyperparathyroidism, 182 secondary and 5 tertiary. All three primary hyperparathyroidism cases underwent removal of the adenoma; in the group of secondary hyperparathyroidism, 50 underwent removal of all the parathyroid glands found, 25 underwent total parathyroidectomy with forearm or deltoid autograft and 60 subtotal parathyroidectomy whereas in 39 and 8 patients only 3 and 2 parathyroid glands were found respectively. In the group of tertiary hyperparathyroidism, we removed only the hyperplastic gland detected as the operative detection of the rest was not possible. Ten cases were re-operated for removal of the remaining glands. No complications were noted postoperatively, apart from severe hypocalcemia in 20 cases, treated successfully by Calcium and Vitamin D administration. The highest relapse rate was noted among the 8 patients with only the 2 parathyroid glands removed. It seems that total or subtotal parathyroidectomy represents the most successful methods for surgical treatment of hyperparathyroidism complicating chronic renal failure.
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PMID:Parathyroidectomy in the treatment of secondary hyperparathyroidism in chronic renal failure. 918 11

The measurement of calciotropic hormones may be useful in metabolic bone disease. Assays of intact parathyroid hormone are essential to differentiate between primary hyperparathyroidism and nonparathyroid-mediated hypercalcemia. Vitamin D status is best assessed by measuring serum 25(OH)D. Concentrations of calciotropic hormones should be measured in osteoporotic patients if the degree of osteopenia does not correlate with the risk factors. The decrease in circulating parathyroid hormone in osteoporotic patients treated with vitamin D reflects the success of the vitamin D treatment. Parathyroid hormone is also a potential anabolic agent.
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PMID:Diagnostic and therapeutic aspects of calciotropic hormones. 922 85

Vitamin D regulates parathyroid cell proliferation and secretion of PTH. Increased prevalence of the polymorphic vitamin D receptor (VDR) alleles b, a, and T has been reported in sporadic primary hyperparathyroidism (PHPT), suggesting that these genetic variants may predispose to the disease. Recently, another polymorphism in the VDR gene was related to bone mineral density, and this VDR-FokI polymorphism causes different lengths of the VDR, implying possible functional consequences. The VDR-FokI polymorphism was studied in 182 postmenopausal women with sporadic PHPT and in matched controls. No significant differences in distribution of the VDR-FokI genotypes could be detected between the groups, although there was a tendency toward overrepresentation of the F allele in the PHPT patients (P = 0.05). There were no significant associations with age, serum calcium, serum PTH, bone mineral density, or parathyroid tumor weight. The VDR genotypes were unrelated to VDR and PTH messenger ribonucleic acid levels in the parathyroid adenomas of 42 PHPT patients. In 23 PHPT patients, the Ca2+-PTH set-points were determined in vivo and were unrelated to the VDR alleles. We suggest that the VDR-FokI polymorphism has at most a minor pathogenic importance in the development of PHPT.
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PMID:The vitamin D receptor (VDR) start codon polymorphism in primary hyperparathyroidism and parathyroid VDR messenger ribonucleic acid levels. 1032 1

The typical manifestations of severe hypercalcemia with osteitis fibrosa cystica have become exceedingly rare. We describe the case of a woman hospitalized for a tibial tumor with functional impotence, leading to a diagnosis of primary hyperparathyroidism (HPT I) associated with profound vitamin D deficiency. This 31-year-old woman was admitted, after two pregnancies complicated by the HELLP syndrome. Preoperative laboratory values were as follows: calcemia 4.05 mmol/l (2.2-2.6); urinary calcium 30 mmol/24 h (1.25-7.5); parathormone (PTH) 1 195 pg/ml (10-60); and 25 OH-vitamin D 13 nmol/l (22-120). Specific MIBI uptake by the tibial lesion oriented the diagnosis towards a brown tumor. After surgical excision of a parathyroid adenoma and the brown tumor (associated with tibial fracture), calcemia fell to 1.55 mmol/l and normalized after three months. Urinary calcium fell to 0.1 mmol/24 h and remained low during the 2 years following surgery. Vitamin D levels rapidly normalized on supplementation (87 nmol/l). PTH levels fell markedly after surgery but remained higher than normal till 2 years after surgery despite normalization of calcemia three months after. Bone repair, estimated by means of bone densitometry, improved from preoperative Z-score values of - 6.54, - 5.20 and - 3.50 in the left femoral neck, right femoral neck and lumbar spine, respectively, to - 0.20, - 1.55 and - 0.28, respectively, one year after surgery. In conclusion, this case illustrates: 1) the severe osseous expression of HPT probably related to vitamin D deficiency; 2) specific MIBI uptake by the bone lesion, orientating the diagnosis towards a brown tumor; 3) the consequences of vitamin D deficiency on postoperative outcome, with transient severe initial hypocalcemia related to bone calcium avidity; 4) a possible link between HPT and the HELLP syndrome.
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PMID:Vitamin D deficiency and severe hyperparathyroidism. 1252 57

The simultaneous occurrence of hyperthyroidism and primary hyperparathyroidism is quite rare. A case is reported here of Graves' disease and primary hyperparathyroidism. Treatment of these concomitant diseases consists in combined thyroidectomy and parathyroidectomy. Histological examination confirmed Graves' disease and revealed the presence of a parathyroid adenoma in one gland and parathyroid hyperplasia in another one. After surgical treatment, secondary hyperparathyroidism due to 25-OH Vitamin D deficiency appeared. To our knowledge, this is the first case in which a Vitamin D absorption deficit was found in presence of hyperthyroidism and hyperparathyroidism without other intestinal absorption deficits. Diagnostic and follow-up difficulties related to these three concomitant pathological conditions are discussed.
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PMID:Concomitant primary hyperparathyroidism, Graves' disease and vitamin D deficiency. 1467 15

We report the case of a young woman with hyperparathyroidism due to a large parathyroid adenoma associated with severe vitamin D deficiency. The case is noteworthy for the size of the parathyroid adenoma and for the young age at presentation, and is more typical of the presentation of hyperparathyroidism seen in developing countries where the prevalence of vitamin D deficiency is high. Vitamin D is known to have a suppressive effect on parathyroid cell proliferation and parathyroid hormone synthesis. Vitamin D deficiency may result in a compensatory increase in the secretion of parathyroid hormone (secondary hyperparathyroidism) which involves hyperplasia of all four parathyroid glands. Secondary hyperparathyroidism can become autonomous and this has been termed tertiary hyperparathyroidism, the underlying pathology of which has been variably described in the literature as adenoma formation or four gland hyperplasia. The pathogenesis of parathyroid adenoma formation in vitamin D deficiency remains unclear. It is possible that a proportion of cases represent the coincidence of primary hyperparathyroidism in patients with vitamin D deficiency. Alternatively, we hypothesise that autonomous four gland hyperplasia or tertiary hyperparathyroidism may progress to adenoma formation and that this should be termed 'quaternary hyperparathyroidism'.
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PMID:Hypothesis: the case for quaternary hyperparathyroidism. 1508 92

Primary hyperparathyroidism (PHPT) is characterized most commonly now as an asymptomatic disorder with hypercalcaemia and elevated levels of parathyroid hormone (PTH). The elevation in PTH is detected by both the standard immunoradiometric assays (IRMA) and a more recent IRMA that detects only the 1-84 full-length PTH molecule. The serum calcium concentration is usually <1 mg dL(-1) above normal. Recently, another variant of PHPT (normocalcaemic PHPT) has been described in which the serum calcium is normal but the serum PTH is elevated, in the absence of any secondary cause for PTH elevation. Although usually sporadic, PHPT also occurs in inherited syndromes. Skeletal manifestations are appreciated by densitometry showing a typical pattern in which cancellous bone of the lumbar spine is reasonably well preserved whilst the cortical bone of the distal third of the radius is preferentially reduced. Although reduced in incidence, renal stones remain the most common overt complication of PHPT. Other organs are theoretical targets of PHPT such as the neurobehavioural axis and the cardiovascular system. Vitamin D looms as an important determinant of the activity of the PHPT state. The 2002 NIH Workshop on asymptomatic PHPT has led to revised guidelines to help doctors determine who is best advised to have parathyroid surgery and who can be safely followed without surgery. New information about the natural history of PHPT in those who did not undergo surgery has helped to define more precisely who is at-risk for complications. At the NIH workshop, a number of items were highlighted for further investigation such as pharmacological approaches to controlling hypercalcaemia, elevated PTH levels and maintaining bone density.
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PMID:Primary hyperparathyroidism: new concepts in clinical, densitometric and biochemical features. 1560 72


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