Anemia in primary hyperparathyroidism

Although anemia has not been widely appreciated as a complication of primary hyperparathyroidism, 5.1% of the individuals with this disorder seen at the Massachusetts General Hospital since 1962 had a normochromic, normocytic anemia that could not be related to blood loss,a deficiency state, or uremia. The anemic group had more advanced bone disease and higher levels of serum calcium, alkaline phosphatase, and parathyroid hormone than the nonanemic group. Results of bone marrow biopsies performed in five patients showed variable degrees of myelofibrosis. However, none of the patients had hepatosplenomegaly, a myelophthisic peripheral blood smear, leukopenia, or thrombocytopenia. Removal of the abnormal parathyroid glands led to improvement or correction of the anemia.     

Surgery in primary hyperparathyroidism

Data were reviewed on 26 patients suffering from primary hyperparathyroidism (PHPT). The diagnosis of PHPT is increasing in frequency, due to greater awareness and better methods of detection. Delay in recognition has gradually decreased, thus permitting earlier treatment. No single test or any combination of tests can be considered satisfactorily pathognomonic of PHPT. Hypercalcemia is the most satisfactory finding suggestive of PHPT. Cervical exploration should be an integral part of the diagnostic work-up. Removal of a distinct adenoma is adequate therapy if the other parathyroid glands are normal. Subtotal parathyroidectomy should be performed only in cases of hyperplasia of all parathyroid glands.     

Asymptomatic primary hyperparathyroidism

The prevalence of hypercalcaemia in the adult population is probably between 0.6 and 1.1%, sufferers being predominantly women over 50 years of age. Most apparently asymptomatic hypercalcaemic patients are found to have primary hyperparathyroidism, and may in fact show some symptoms of the condition (lowered bone mineral density, cardiovascular disease and/or neuropsychiatric symptoms). The criteria for surgical intervention in these cases are discussed in the light of the high success rate of parathyroidectomy in experienced hands and the lack of effective alternative treatments.     

Geriatric primary hyperparathyroidism

Primary hyperparathyroidism occurs in a significant number of patients over 60 years of age; about one-third of the 182 patients we studied were in this age group. Primary hyperparathyroidism has been recognized with greater frequency in recent years, particularly in patients without manifestations of renal lithiasis or osteitis fibrosa cystica and in older patients. Age alone is not critical in the occurrence of serious complications from hypercalcemia, the presence of other life-endangering disease, or the development of major postoperative complications in the management of primary hyperparathyroidism. A liberal but selective policy is advocated in advising operation for primary hyperparathyroidism in the elderly. Different clinical patterns of primary hyperparathyroidism are recognized in all age groups. The operative procedure for primary hyperparathyroidism is determined primarily by the surgeons at the time of operation, on the basis of gross characteristics of the parathyroid glands. A precise correlation is not found between microscopic changes, gross changes, and clinical features of primary hyperparathyroidism.     

Neonatal primary hyperparathyroidism

The authors report on a case of severe primary hyperparathyroidism with clinical signs from birth. The boy was admitted because of poor somatomental development, hypotony, hepatosplenomegaly and osseous abnormalities, resembling those of rachitis. Laboratory data showed the typical findings of primary hyperparathyroidism. The authors performed parathyroidectomy with simultaneous heterotopic parathyroid tissue autotransplantation. Histology revealed parathyroid chief cell hyperplasia. Because of the hypercalcaemia and clinical signs persisting after the operation they removed the parathyroid autografts. Since this later was ineffective they performed a left sided neck dissection on the side of the excessive parathormone production. The child became hypocalcaemic necessitating calcium and vitamin D administration. He is now 17 months after the last operation. His somatomental development is accelerated. In connection with the case the authors surveyed the literature of this rare entity.     

Preoperative instrumental diagnosis in secondary hyperparathyroidism

There is a very high incidence of hyperparathyroidism secondary to chronic renal insufficiency in dialysed patients (67%). When medical therapy can no longer control the evolution of disease, an appropriate surgical strategy must be defined for the clinical case. Instrumental tests play an important role during the preoperative phase in the possible "localisation" of ectopic parathyroids (10% of cases) and the identification of those glands with a more pronounced hyperplastic appearance. The authors examine and describe the most useful tests for a preoperative study, namely ultrasonography, echo-colour Doppler, scintigraphy using a dual tracer and image subtraction, computerised xial tomography (CT), magnetic resonance (MR), digital angiography and intraoperative ultrasonography. In conclusion, it can be said that all the methods examined present similar sensitivity levels for the study of parathyroid, but the combination of more than one technique considerably improves diagnostic accuracy.     

Familial isolated primary hyperparathyroidism

Familial primary hyperparathyroidism (PHPT) is usually encountered in the context of multiple endocrine neoplasia (MEN) syndromes. Few families have been reported in the literature where PHPT was the only abnormality. However, in these families no long-term follow-up data were reported and no genetic linkage studies were performed. OBJECTIVE: We investigated a large family with a familial primary hyperparathyroidism for biochemical and genetic markers of multiple endocrine neoplasia syndromes. DESIGN: A family screening study. PATIENTS: Thirty-seven family members participated in this study including 7 patients who had been previously operated upon for PHPT. MEASUREMENTS: Serum calcium (albumin adjusted), was measured in all family members. Hypercalcaemic subjects and patients who had been operated upon for PHPT were assessed for biochemical markers of MEN syndromes (serum gastrin, prolactin, calcitonin, fasting plasma glucose and 24-hours urinary excretion of adrenaline, noradrenaline and vanillylmandelic acid (VMA)). Genetic linkage analysis was performed using DNA markers linked to chromosome 11q13, the presumed MEN type 1 (MEN-1) locus. RESULTS: Four new patients with PHPT and two with probable PHPT were discovered. No clinical or biochemical evidence of MEN syndromes could be detected. DNA marker pMS51(D11S97) was informative, maximum two-point lodscore of 2.12 at a recombination fraction of 0.05 confirming linkage to chromosome 11q13. CONCLUSIONS: Familial PHPT can exist as a separate clinical entity. Isolated familial PHPT is caused by mutation in a gene located in the MEN-1 region on chromosome 11q13, possibly the MEN-1 locus.     

Technetium 99m tetrofosmin parathyroid imaging. Results with double-phase study and SPECT in primary and secondary hyperparathyroidism

RATIONALE AND OBJECTIVES: The aim of our study was to evaluate the sensitivity, specificity, and positive predictive value (PPV) of technetium 99m (99mTc) tetrofosmin double-phase scintigraphy and single-photon emission computer tomography (SPECT) in preoperative localization of parathyroid adenoma in case of primary and secondary hyperparathyroidism (HPT). METHODS: Sixty-eight consecutive patients biochemically or sonographically suspected of parathyroid adenoma were included in our study. Apart from biochemical analysis of serum calcium, phosphate, and intact parathyroid hormone, double-phase scintigraphy was performed in each patient 5 and 45 minutes after injection of 370 MBq 99mTc tetrofosmin, followed by SPECT imaging. In consciousness of the scintigraphic results, ultrasound of the neck was performed as well to exclude false-positive results due to thyroid adenomas. RESULTS: Depending on the results of the biochemical analysis in combination with the results of the scintigraphic and ultrasound examination, the patients were classified retrospectively into three groups: group A with primary HPT (n = 35), group B with secondary HPT (n = 13), and group C without any biochemical suspicion of primary or secondary HPT (n = 20). In group A, double-phase study localized 25 of 36 (69.2%) parathyroid adenomas (one double adenoma) as against 34 of 36 (94.4%) with SPECT. Nine adenomas could be visualized only by SPECT. The reason for nonvisualization on planar scans was suspected to be an ectopic location in 2 cases (retrotracheal dislocation, retrovascular dislocation), a maximal diameter less than 15 mm (9-13 mm) in 6 cases, and oxyphilic-cell-poor cellularity in 1 case. Four false-positive retention (3 thyroid adenomas and 1 papillary thyroid carcinoma) were observed. SPECT showed a sensitivity of 94.4%, a specificity of 85%, and a PPV of 91.9% in biochemically suspected primary HPT. In group B, planar scintigraphy demonstrated 12 hyperplastic glands in 5 of 13 patients, and SPECT demonstrated 20 hyperplastic parathyroid glands in 8 out of 13 patients, which corresponds to a sensitivity of 38% and 61.5%, respectively. CONCLUSIONS: Technetium 99m tetrofosmin seems to be a promising alternative tracer with similar capabilities to 99mTc sestamibi in localization of parathyroid adenoma. SPECT showed clear advantages in terms of sensitivity over planar scintigraphy and should be used at least in cases with poor or no uptake in double-phase study. In endemic goiter areas, ultrasound of the neck should be performed to exclude false positive retention in thyroid adenomas. Technetium 99m tetrofosmin, like 99mTc sestamibi, is not ideal for localization of hyperplastic glands in secondary hyperparathyroidism because of low sensitivity.     

Topical diagnostic methods in primary hyperparathyroidism

The efficiency of USI of the neck, scintigraphy of the parathyroid glands (PTG) with selen-methionine and selective catheterization of veins (SCV) of the neck and mediastinum for preoperative determination of the localization of the source of hyperproduction of the parathyroid hormone was analyzed on the basis of results of examination of 149 patients including 51 patients with primary hyperparathyroidism confirmed during the operation. It was noted that none of the methods of topical diagnostics used in the work is thought to be universal for different clinical situations. A method of their complex use is proposed which foresee USI of the neck as the first step. In case of insufficient information of the results of USI the authors propose SCV of the neck and mediastinum as a method of choice which allows the size and level of the altered PTG to become more exact and which makes further search with the help of other methods of topical diagnostics more purposeful and effective.     

Primary hyperparathyroidism in the primary care setting

A series of 36 patients with surgically proved primary hyperparathyroidism is reported. From this group a clinical profile consisting of obesity, anxiety, and/or depression in a mildly hypertensive, middleaged female was derived. Women constituted 92 percent of the patients. The serum calcium was confirmed again as the single most valuable test in the evaluation of this disorder.     

Biochemical studies and bone tissue biopsy in primary hyperparathyroidism

Insomnia in 11 general hospital in-patients was treated by a placebo. The conditions of administration of the placebo were experimentally varied, and a hypothesis derived from Festinger's cognitive dissonance theory was tested. Consistent with this hypothesis, it was found that patients who were required to make meaningful decisions concerning their treatment derived significantly greater hypnotic (therapeutic) value from it than did patients whose treatment was simply administered to them and who were not required to make such decisions. The power of cognitive dissonance theory to explain the placebo effect, and the therapeutic potential of this approach are discussed.     

New strategies in the surgery of primary hyperparathyroidism

Although a remarkable pathology in terms of morbidity and life expectancy, primary hyperparathyroidism remains nonetheless a poorly recognized state despite highly suggestive clinical polymorphism, reliable biological signs and performing imaging for localisation. Through a retrospective study of a series of 129 patients surgically treated for primary hyperparathyroidism over a period of 12 years, helped by a review of the literature, we are able to propose an adapted surgical strategy for every different clinical aspect of this pathology (adenoma, benign hyperplasia, carcinoma). This strategy is guided by specific pre-operative findings and the Sestamibi scintigraphy.