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PubMed1991

Primary hyperparathyroidism and monoclonal gammopathy.

Coexistent primary hyperparathyroidism and monoclonal gammopathy, although rare, has been reported previously by a number of investigators. We report four patients with such an occurrence who were seen between 1976 and 1988. Another patient with primary hyperparathyroidism also had multiple myeloma and was in remission for 12 years. These patients represent approximately 1% of the 386 patients with primary hyperparathyroidism seen during the same 12-year period. Although several mechanisms have been proposed to explain this concurrence, we believe it is the result of a chance occurrence. A review of the literature, an estimate of the chance occurrence of coincidental monoclonal gammopathy, benign or malignant, in patients with primary hyperparathyroidism, and some practical implications of this interesting coexistence are presented.

Aged↗
PubMed1996

Long-term health risk of primary hyperparathyroidism: the effect of surgery.

Primary hyperparathyroidism is a common disease but 90% of sufferers remain undetected. In elderly women its prevalence is about 1%. The disease can cause severe hypercalcaemia and lead to life-threatening symptoms. However, the majority of cases represent milder forms of hyperparathyroidism with minimal symptoms or no symptoms at all, and slight hypercalcaemia which does not progress during follow-up. Surgery is the only effective treatment of primary hyperparathyroidism and most of the symptoms respond favourably to surgery. Nevertheless, the role of surgery has been controversial in the treatment of mini-symptomatic patients. Recently it has become evident that primary hyperparathyroidism is associated with increased mortality due mainly to cardiovascular diseases, and it has been shown that surgical treatment is able to diminish or even eliminate this risk of premature death. The extra mortality is connected also to the mild and nonprogressive forms of the disease, and it seems to have a strong correlation with the duration of the disease. This aspect favours active treatment at an early stage of the disease. Conservative treatment can be considered only in old patients with mild and stable clinical course. Primary hyperparathyroidism is an important risk factor causing morbidity and mortality.

Female↗
PubMed2001

[Clinical characteristics of 47 patients with primary hyperparathyroidism surgically treated].

BACKGROUND: The primary hyperparathyroidism is usually an asymptomatic disease. Actually, specialists are attempting to consider to what extent it can be necessary to undergo surgery in this disease. In this article, we are going to describe the clinical and biochemical characteristics of our patients with primary hyperparathyroidism as well as the diagnostic procedures and the results after operation. METHODS: Firstly, we have analysed 47 records of patients with primary hyperparathyroidism and intervened in our hospital during the last 5 years. Secondly, we assessed the biochemical results before and after the surgical intervention, the outcome of parathyroid echography, thoracico-cervical tomography, technietium-99-mm-sestamibi gammagraphy and bone densitometry. Finally, we followed up the assessment of the operation of the patients with hyperparathyroidism. RESULTS: 47 patients were intervened of hyperparathyroidism. 89.4% were women and average the age was 61.4 +/- 12.8 years. Most of them were asymptomatic (45.7%). The gammagraphy was the procedure with best sensibility and specificity, 75 and 100%. Bone densitometry showed that there were no patients with diminished calcification of the bones. The principal diagnostic of pathologic anatomy was adenoma (85.1%). Excluding one single case, the rest of patients healed of their illness after intervention. CONCLUSIONS: Primary hyperparathyroidism is asymptomatic in most of the cases. It is mainly an adenoma process. The best diagnostic procedure is technietium-99-mm-sestamibi gammagraphy. The most appropriate and successful treatment is surgery.

Aged↗
PubMedDate not supplied

Skeletal effects of primary hyperparathyroidism.

OBJECTIVE: To review the effect of primary hyperparathyroidism on bone mass and occurrence of fractures as well as the advances in medical management of this relatively common condition. METHODS: We conducted a MEDLINE search of peer-reviewed publications for the period from 1960 to 1999. Studies reviewed were those that evaluated bone mass and fracture risk in primary hyperparathyroidism in both male and female populations. Studies that assessed intervention with hormone replacement therapy, bisphosphonates, calcimimetic agents, and surgical treatment and their effect on bone mass and fracture were also reviewed. Preference was given to prospective studies, but retrospective, cross-sectional, and case-control studies were also evaluated. RESULTS: Most densitometry studies completed to date have been limited by their design. Some cross-sectional studies that used both single-photon absorptiometry and dual-energy x-ray absorptiometry have demonstrated preferential bone loss at cortical skeletal sites. Bone density seems to be relatively well preserved at cancellous bone sites. The absence of large prospective controlled trials makes it difficult to evaluate fracture incidence associated with hyperparathyroidism. Retrospective and case-control studies have found conflicting results relative to fracture incidence in patients with primary hyperparathyroidism. Parathyroidectomy has been shown to be of value in improving bone mineral density at both the lumbar spine and the femoral neck. Hormone replacement therapy in postmenopausal women with primary hyperparathyroidism has also been effective in improving bone mineral density and decreasing bone turnover. Bisphosphonates are currently being evaluated for effectiveness in improving bone mineral density and reducing the risk of fracture. Early results with alendronate treatment have demonstrated improvements in bone mass. Calcimimetic agents are a new treatment option that may become useful in the medical management of primary hyperparathyroidism. CONCLUSION: Although conflicting findings have been reported, bone loss has been noted in patients with primary hyperparathyroidism, especially at cortical skeletal sites. Medical management does not seem to be associated with increased morbidity or mortality in patients with asymptomatic primary hyperparathyroidism. Bone densitometry is advised, particularly for monitoring of bone mass at the midradius or femoral neck, in patients with primary hyperparathyroidism.

Bone Density↗
PubMed1991

Medical management of asymptomatic primary hyperparathyroidism.

Patients with mild asymptomatic primary hyperparathyroidism who do not meet currently accepted guidelines for surgery may be followed medically. General medical management of these individuals should be directed toward maintaining adequate hydration, therapy of hypertension, and avoiding immobilization. Diuretics should be used only with caution. Moderate dietary calcium intake (500-800 mg/day) should be encouraged. Propranolol and cimetidine are not useful in the therapy of primary hyperparathyroidism. Oral phosphate is efficacious in lowering serum and urinary calcium. However, because of concerns related to ectopic calcification, phosphate is usually reserved for those patients who meet surgical guidelines but who are not to undergo surgery. Bisphosphonates, potent inhibitors of osteoclast-mediated bone resorption, have been shown to lower serum and urinary calcium in patients with primary hyperparathyroidism. However, long-term data on their efficacy in this disorder are not yet available. The use of bisphosphonates at the present time is generally restricted to the research setting. More potent bisphosphonates as well as the design of newer agents that interfere with parathyroid hormone secretion may become very useful in future approaches to the medical management of primary hyperparathyroidism.

Administration, Oral↗
PubMed1980

Primary hyperparathyroidism: changes in the pattern of clinical presentation.

207 hypercalcaemic patients, discovered in an urban area of 1 million people over a period of 5 months, were examined to determine the current incidence and mode of presentation of primary hyperparathyroidism. Primary hyperparathyroidism was diagnosed in 111 patients and those most at risk were women over the age of 70 (55% of all patients). In 57% of all patients the initial indication of primary hyperparathyroidism was hypercalcaemia found unexpectedly on biochemical screening of the serum. The next most common mode of presentation was an acute hypercalcaemic syndrome in elderly patients, characterised by confusion and dehydration (14% of all patients). Modern diagnostic methods and routine estimations of serum calcium can be expected to yield at least 250 new cases of primary hyperparathyroidism per million population per year.

Acute Disease↗
PubMedDate not supplied

[Primary hyperparathyroidism in pregnancy--a case report].

BACKGROUND: Primary hyperparathyroidism during pregnancy is rare. The incidence of primary hyperparathyroidism in women of child-bearing age is estimated to be approximately 8 cases per 100,000 population per year. CASE REPORT: We provide a case report of a 33-year old pregnant woman, in whom primary hyperparathyroidism was diagnosed because of neonatal hypocalcemia two days after delivery. There was a history of recurrent urinary tract infections with hydronephrosis on the left side. The woman had been treated on an outpatient basis by an urologist, nephrostomy had been performed. After delivery, nephrolithiasis was diagnosed by x-ray and treated endoscopically. Parathyroid adenoma was found and removed successfully. DISCUSSION: Due to physiological changes of calcium metabolism primary hyperparathyroidism is often without symptoms during pregnancy. Operative elimination of adenoma as a frequent cause of primary hyperparathyroidism reduces maternal and fetal morbidity and mortality. CONCLUSIONS: In pregnant women with hydronephrosis on the left side primary hyperparathyroidism should be considered. If undetected, hyperparathyroidism causes considerable morbidity, not only for the mother but also for the child.

Adenoma↗
PubMed1999

Primary Hyperparathyroidism and Polymorphic Ventricular Tachycardia.

BACKGROUND: Primary hyperparathyroidism is a rare but important cause of ventricular arrhythmias. METHODS AND RESULTS: The medical records of a patient with primary hyperparathyroidism and polymorphic ventricular tachycardia was reviewed. The patient was serially interviewed and examined before and after parathyroidectomy. A literature search was performed. The association of primary hyperparathyroidism and polymorphic ventricular tachycardia is rare. Hypokalemia and hypomagnesemia occur infrequently in primary hyperparathyroidism. CONCLUSIONS: The potential for malignant ventricular arrhythmias in the setting of primary hyperparathyroidism must be recognized.

Journal Article↗
PubMed1988

[Surgery of primary hyperparathyroidism in 1988. Strategies during primary operations, in case of failure and in case of cancer].

In primary hyperparathyroidism, an operation is indicated when the calcemia exceeds 115 mg/l and phosphoremia is low in several successive instances, regardless of the symptoms, even if PTH levels and cervical ultrasonography are normal. In case of calcemia under 110 mg/l, the diagnosis must be confirmed by titration of the nephrogenic cyclic AMP and symptomatic patients must be operated upon as well as asymptomatic patients with a life expectancy exceeding 10 years. In case of acute hypercalcemia, the procedure must be performed as a semi-emergency without waiting for a definite diagnosis, since the course may rapidly be fatal in spite of all medical treatments. Ultrasonography mostly presents the advantage of detecting intrathyroid parathyroids glands. The experience of the surgeon is essential for the mandatory locating of 4 glands, and the choice of the surgical strategy. In front of a secondary indication for failure or recurrence, one must take into consideration what was seen and done during the first procedure, the calcemia levels and clinical, radiological or biological consequences. Finally, in case of cancer (2 p. cent), the best prognosis rests in wide excision of the thyroid compartment and nodes areas, since medical treatments and radiation therapy are ineffective.

Female↗
PubMed1990

[Primary hyperparathyroidism].

The diagnosis of primary hyperparathyroidism is based on laboratory tests showing high levels of calcium and parathyroid hormone. Primary hyperparathyroidism is treated by surgery, which is indicated in symptomatic as well as in asymptomatic disease, provided the latter patients are at low risk. Primary parathyroidectomy should be successful in at least 90% of the patients. Epidemiological studies show that pHPT occurs much more often than operation statistics imply. This situation can be improved by a more consequent differential diagnosis that detects up all cases of hypercalcemia.

Diagnosis, Differential↗
PubMed1986

Evidence that factors other than 1,25 dihydroxyvitamin D may play a role in augmenting intestinal calcium absorption in patients with primary hyperparathyroidism.

We examined 17 patients with primary hyperparathyroidism for their serum 1,25 dihydroxyvitamin D levels and for their fractional intestinal calcium absorption rates using a whole body counter and calcium-47. As controls, 10 normal volunteers were examined both before and after administration of 1 alpha-hydroxyvitamin D to increase serum 1,25 dihydroxyvitamin D. Values of serum 1,25 dihydroxyvitamin D were 71.6 +/- 37.6 pg/ml (mean +/- SD) in patients with primary hyperparathyroidism and 75.3 +/- 27.7 pg/ml (mean +/- SD) in normal volunteers after administration of 1 alpha-hydroxyvitamin D, while values of intestinal calcium absorption rate were 61.5 +/- 16.5% (mean +/- SD) in patients with primary hyperparathyroidism and 34.1 +/- 5.1% (mean +/- SD) in normal controls, respectively. There was a positive correlation between values of serum 1,25 dihydroxyvitamin D and intestinal calcium absorption in both groups. However, in patients with primary hyperparathyroidism, intestinal calcium absorption was more increased than that in normal volunteers when compared to their serum values of 1,25 dihydroxyvitamin D. This suggests that another factor than 1,25 dihydroxyvitamin D plays an important role in the intestinal calcium absorption in patients with primary hyperparathyroidism.

Adult↗
PubMed1989

Neuromuscular involvement in mild, asymptomatic primary hyperparathyroidism.

PURPOSE: The classic neuromuscular abnormalities of primary hyperparathyroidism are based on observations from an earlier time when the disease presented commonly with signs and symptoms. The purpose of the present study was to test for neuromuscular abnormalities among patients currently seen with primary hyperparathyroidism, in whom the vast majority are asymptomatic. PATIENTS, METHODS, AND RESULTS: Forty-two patients (30 women, 12 men) were studied. Serum calcium level was 11.1 +/- 0.1 mg/dL (normal, 8.7 to 10.7 mg/dL); phosphorus level was 2.8 +/- 0.1 mg/dL (normal, 2.5 to 4.5 mg/dL). Parathyroid hormone levels by radioimmunoassay for N-terminal and for mid-molecule regions were 32.6 +/- 2.4 pg/mL (normal, 8 to 24 pg/mL) and 831 +/- 127 pg/mL (normal, 50 to 330 pg/mL), respectively. Although generalized weakness and easy fatigability were common complaints, they were not seen on neurologic examination. No patient showed typical neuromuscular findings such as muscle weakness or atrophy, hyperreflexia, abnormal gait, tongue fasciculations, or objective changes in mental status. On the other hand, paresthesias and muscle cramps were reported by 22 patients (52%). Neurologic examination in 12 patients (29%) demonstrated findings consisting of either stocking-glove loss of pain sensation or loss of vibratory sensation with diminished reflexes. Creatine phosphokinase levels were normal in all patients. Electromyography and nerve conduction studies were performed in nine patients. None showed myopathy or signs of motor unit denervation. Isolated nerve abnormalities (n = 6) and nonspecific sensory neuropathy (n = 2) were observed. CONCLUSION: We conclude that the classic neuromuscular syndrome of primary hyperparathyroidism is rarely seen among a typical modern-day group of patients with primary hyperparathyroidism. The results suggest that the neurologic component of primary hyperparathyroidism should be included among those features of the disease that have changed along with other aspects of the clinical profile of primary hyperparathyroidism.

Aged↗
PubMed2006

How to localize parathyroid tumors in primary hyperparathyroidism?

The management of primary hyperparathyroidism (PHPT) has dramatically changed in the last 5 yr. Many more patients now undergo focused, limited or minimally invasive parathyroidectomy instead of traditional bilateral neck exploration. This change has taken place because of the improved accuracy of pre-operative localizing studies in selecting patients who have single-gland parathyroid disease (single adenoma) and can therefore have a minimally invasive parathyroidectomy. Sestamibi scanning followed by ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT) scans are most accurate for localizing parathyroid tumors in patients with PHPT. Selective venous catheterization for PTH levels is useful when other localizing studies are negative or discordant in patients with persistent or recurrent PHPT. The routine use of one or more localizing studies commonly identifies the parathyroid tumor in patients with single-gland disease; but if localizing studies are negative or discordant, patients should have intra-operative PTH levels monitored or have a bilateral neck exploration to ensure a high rate of biochemical cure.

Adenoma↗
PubMed2006

From mild to severe primary hyperparathyroidism: The Brazilian experience.

Primary hyperparathyroidism often presents as an asymptomatic disorder. In our institution, routine serum calcium measurements have now been used as part of medical examination for 23 years. Out of 124 patients consecutively seen at our institution, 47% presented with no symptoms related to the disease, while 25% presented with severe skeletal involvement and osteitis fibrosa cystica, 25% with renal stone disease without overt bone involvement, and 2% with the typical neuropsychiatric syndrome. This same pattern is seen in the city of São Paulo. In severe disease pathological fractures are frequently seen, especially in long bones of the lower extremities, and also loss of lamina dura of the teeth and salt-and-pepper appearance of the skull. Bone mineral density is extremely low in these patients but usually show remarkable recovery following surgical cure. Serum PTH and bone markers are considerable higher in severely affected patients, who also have a high rate of vitamin D deficiency, and the parathyroid lesion is easier located compared with asymptomatic patients. From pathological specimens 87% had histological confirmation of a single adenoma, 6.4% multiple gland hyperplasia and 3.8% carcinoma.

Adenoma↗
PubMed2005

[Surgical indications of primary hyperparathyroidism].

Clinical profile of primary hyperparathyroidism (PHPT) had shifted from symptomatic disorder, with hypercalcemic symptoms, kidney stones, overt bone disease, or a specific neuromuscular dysfunction toward a more asymptomatic state. Patients with symptomatic PHPT absolutely require parathyroidectomy (PTX). Surgical treatment is the well established and definitive treatment for PHPT. Consensus Conference at National Institute of Health (NIH) proposed the guidelines for PTX in asymptomatic PHPT. They included (1) serum calcium level was 1.0 mg/dL above upper limit of normal, (2) 24-h urinary calcium was over 400 mg, (3) creatinine clearance is reduced by 30%, (4) bone mineral density (T-score) reduced 2.5 SD below the peak bone mass at any site, and (5) patients under 50 years of age, and (6) patients for whom medical surveillance was either not desirable or not possible. Because in about 25% of patients asymptomatic PHPT will show progression, because it is important to monitor patients who are not undergoing PTX. Normocalcemic PHPT has been frequently found in patients whose bone mineral density is reduced. This diagnostic consideration requires that all potential causes of secondary HPT. The surgical procedure has been progressive induced by the developments of preoperative image diagnosis and intraoperative PTH assay. Minimally invasive PTX could expand surgical indications in patients with mild asymptomatic PHPT.

Humans↗
PubMed2002

Limited versus radical parathyroidectomy in familial isolated primary hyperparathyroidism.

BACKGROUND: Familial isolated primary hyperparathyroidism (FIHPT) is characterized by earlier onset, higher incidence of multiglandular disease, and higher recurrence rate when compared with sporadic primary hyperparathyroidism. Excision of 3.5 or 4 glands with autotransplantation has been recommended; however, these approaches lead to permanent hypoparathyroidism in 13% to 41% of patients. It is reported that many patients with FIHPT return to normocalcemia after single-gland excision. The use of preoperative localization and intraoperative parathyroid hormone assay permits limited resection of only hypersecreting glands. We report the outcome of this operative approach. METHODS: Fifteen consecutive patients with FIHPT underwent limited parathyroidectomy with resection guided by intact parathyroid hormone secretion in 2 university centers. Patients were followed up postoperatively for serum calcium and intact parathyroid hormone levels. RESULTS: With an operative success of 93%, 14 patients had only single-gland excision and 80% had unilateral neck exploration. All initial patients had their hypercalcemia corrected. In 4 reoperations, permanent hypoparathyroidism occurred in 2 patients. One recurrence was observed in 40 (8-122) months of follow-up. CONCLUSION: Limited parathyroidectomy allows successful single-gland excision in many patients with FIHPT, thus decreasing the risk of hypoparathyroidism. In these patients, a low incidence of hypoparathyroidism may be preferable to the possibility of late recurrence.

Adolescent↗
PubMed1987

Primary hyperparathyroidism and idiopathic hypercalciuria.

Primary hyperparathyroidism and idiopathic hypercalciuria are important causes of calcium stone disease. Hypercalcemia is usually the clue to the presence of primary hyperparathyroidism, but a minority of patients are intermittently or persistently normocalcemic. In these patients there appears to be a resistance to the effect of parathyroid hormone on renal calcium transport, to which calcitriol may contribute. Such patients mimic those with idiopathic hypercalciuria, the commonest metabolic cause of stone formation. The pathophysiology of idiopathic hypercalciuria remains controversial, but abnormalities of renal tubule function and disordered vitamin D metabolism are commonly present. The separation into so-called renal and absorptive types does not appear to be of practical importance, since thiazide diuretics provide effective prophylaxis regardless of type.

Calcium↗
PubMed2002

Normocalcemic primary hyperparathyroidism with fractures.

Normocalcemic primary hyperparathyroidism is an uncommon condition usually found among patients with urinary stones. To the best of our knowledge, this is the first case of a patient with normocalcemic primary hyperparathyroidism presenting with bilateral femoral neck fracture. A 45-year-old man had pain both hip joints and limping in for several months. Vertical fractures of bilateral femoral necks were found by radiographs. Laboratory findings showed a normal value of total serum calcium but elevated serum ionized calcium. Serum parathyroid hormone and alkaline phosphatase levels were elevated. The patient was treated with subtrochanteric valgus osteotomy fixed with a dynamic hip screw and total parathyroidectomy. Histopathologic examination showed hyperplasia of the parathyroid glands. At 1-year follow-up, the patient was doing well clinically.

Alkaline Phosphatase↗