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Primary Hyperparathyroidism (primary + hyperparathyroidism)
Kinds of Primary Hyperparathyroidism Selected AbstractsChondrosarcoma in Association With Primary Hyperparathyroidism,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 7 2004Ajay Bhatia Abstract We describe two female patients, 66 and 36 years of age, with both primary hyperparathyroidism and chondrosarcoma. Case 1 had a chondrosarcoma of the right scapula, and case 2 had chondrosarcoma of the left proximal tibia. Both patients underwent surgical resection of their chondrosarcoma and subsequent parathyroid surgery. Histological analysis of the excised parathyroid in case 1 showed a parathyroid carcinoma and in case 2 showed a parathyroid adenoma. Including these two patients, there is now a total of six cases that have been reported in the literature describing the association between hyperparathyroidism and bone malignancy. We believe that this small number makes it unlikely that there is an association between these two conditions, although we speculate that there may be an underlying genetic basis. [source] Primary Hyperparathyroidism and Monoclonal Gammopathy in a DogJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 1 2009G. Benchekroun First page of article [source] Primary hyperparathyroidism: new concepts in clinical, densitometric and biochemical featuresJOURNAL OF INTERNAL MEDICINE, Issue 1 2005J. P. BILEZIKIAN Abstract. 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. [source] Primary hyperparathyroidism in 29 dogs: diagnosis, treatment, outcome and associated renal failureJOURNAL OF SMALL ANIMAL PRACTICE, Issue 1 2005R. N. A. Gear Objectives: To review the records of 29 dogs diagnosed with primary hyperparathyroidism and see if any factors correlate with renal failure. Methods: Dogs were selected retrospectively from case files from the QVSH and the QMH. Results: The majority of dogs were middle-aged and four were keeshonds. The primary presenting complaints were polyuria and polydipsia. All dogs had an elevated total and ionised plasma calcium concentration. Plasma phosphate concentrations were variable. Ultrasonography of the parathyroid gland revealed nodular enlargement which was found to correlate well with surgical findings. The majority of dogs underwent surgical parathyroidectomy. Five cases were treated by ultrasound-guided chemical ablation of the parathyroid gland, of which only two cases showed a partial response. Three dogs were euthanased within a week of presentation. Seven other dogs had renal failure diagnosed either at presentation or up to six months after parathyroidectomy. The development of renal failure was correlated with total calcium concentration but did not correlate with any other factor, including the calcium phosphate product. Thirteen treated dogs were known to be alive at the time of writing, which was six months to 3.5 years after parathyroidectomy. Clinical Significance: Primary hyperparathyroidism cases with high total calcium were more likely to develop renal failure in this group of dogs; however, the calcium phosphate product did not seem to be a useful predictor. Ultrasound-guided chemical ablation seemed to have limited advantage over surgery. [source] Hypercalcemia in Cats: A Retrospective Study of 71 Cases (1991,1997)JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 2 2000Karine CM. A retrospective study was conducted to characterize the diseases, clinical findings, and clinicopathologic and ultrasonographic findings associated with hypercalcemia (serum calcium concentration >11 mg/dL) in 71 cats presented to North Carolina State University Veterinary Teaching Hospital. The 3 most common diagnoses were neoplasia (n = 21), renal failure (n = 18), and urolithiasis (n = 11). Primary hyperparathyroidism was diagnosed in 4 cats. Lymphoma and squamous cell carcinoma were the most frequently diagnosed tumors. Calcium oxalate uroliths were diagnosed in 8 of 11 cats with urolithiasis. Cats with neoplasia had a higher serum calcium concentration (13.5 ± 2.5 mg/dL) than cats with renal failure or urolithiasis and renal failure (11.5 ± 0.4 mg/dL; P <.03). Serum phosphorus concentration was higher in cats with renal failure than in cats with neoplasia (P < .004). Despite the fact that the majority of cats with uroliths were azotemic, their serum urea nitrogen and creatinine concentrations and urine specific gravity differed from that of cats with renal failure. Additional studies are warranted to determine the underlying disease mechanism in the cats we identified with hypercalcemia and urolithiasis. We also identified a small number of cats with diseases that are not commonly reported with hypercalcemia. Further studies are needed to determine whether an association exists between these diseases and hypercalcemia, as well as to characterize the underlying pathophysiologic mechanism for each disease process. [source] Primary hyperparathyroidism is a surgical conditionANZ JOURNAL OF SURGERY, Issue 3 2002Ian R. Gough No abstract is available for this article. [source] Primary hyperparathyroidism: Referral patterns and outcomes of surgeryANZ JOURNAL OF SURGERY, Issue 3 2002Richard S. Flint Background: Parathyroidectomy has long been established as an effective treatment for primary hyperparathyroidism (HPT). Methods: A 15-year retrospective audit was made by surgeons at North Shore Hospital, Auckland, of 33 patients with primary HPT who had parathyroidectomy. Results: There were 22 females and 11 males, ranging in age from 18 to 77 years (median 63 years). Initial diagnosis was predominantly by a general practitioner (72%), who invariably referred to a physician. Referral to surgery was made by general physicians (55%), endocrinologists (33%) and geriatricians (6%). Delay between diagnosis and referral for surgery ranged from 8 days to 10 years (median 7 months), and exceeded 2 years in 24% of patients. Twenty-eight (85%) were symptomatic: 13 (39%) had renal symptoms, 13 (39%) had bone disease, 10 (31%) had gastrointestinal complaints, seven (21%) had psychiatric illnesses and six (18%) had fatigue. The high incidence of symptoms was matched by high biochemical values (mean serum cal- cium level 2.97 mmol/L), and large parathyroid glands (mean weight 2001 mg). Twenty-nine patients (88%) had single adenomas, two (6%) had chief cell hyperplasia and two (6%) had carcinoma. Thirty-one (94%) were cured of their primary HPT. Conclusions: Parathyroidectomy is a safe and effective treatment for primary HPT but depends upon referral from non-surgical clinicians. A large proportion of patients have long delays before their surgery, and the group selected for surgery is referred with severe disease. [source] Increased plasma concentrations of N-terminal pro-B-type natriuretic peptide in patients with mild primary hyperparathyroidismCLINICAL ENDOCRINOLOGY, Issue 6 2006Erik G. Almqvist Summary Objective, Primary hyperparathyroidism (PHPT) is associated with heart disease. The aims of the present study were to evaluate how cardiac function and secretion of N-terminal pro-B-type natriuretic peptide (NT-proBNP) correlate in patients with mild PHPT, and how the plasma level of NT-proBNP is influenced by cure of the parathyroid disease. Design and patients, Forty-two patients with PHPT without symptoms of heart disease were examined before and 1 year after curative parathyroidectomy. Measurements, Plasma or serum concentrations of NT-proBNP, calcium, PTH, creatinine, oestradiol, testosterone and SHBG were measured. Cardiac function was evaluated by equilibrium radionuclide angiography (ERNA). Results, At baseline, NT-proBNP levels correlated negatively with systolic function [left ventricular ejection fraction (LVEF), P < 0·001]. Twelve per cent of the patients had NT-proBNP levels above normal reference values preoperatively. One year postoperatively, the corresponding proportion was 21%. The mean plasma concentration of NT-proBNP increased after parathyroidectomy (P < 0·01) in parallel with a dip in diastolic function (peak filling rate, P < 0·05) and a falling trend in systolic function (LVEF, P = 0·08). The postoperative percentage changes in circulating NT-proBNP and total oestradiol correlated positively (P < 0·05). Conclusions, Patients with mild PHPT and normal renal function may have high levels of circulating NT-proBNP despite the absence of symptomatic heart disease. Cure of the parathyroid disease is followed by a further increase in NT-proBNP secretion in parallel with ERNA measures, indicating subclinical changes in heart function. These results are in line with data indicating an association between PHPT and increased risk of premature death. [source] Update on genetic and clinical aspects of primary hyperparathyroidismCLINICAL ENDOCRINOLOGY, Issue 5 2003S. Miedlich Summary Primary hyperparathyroidism (pHPT) is a common endocrine disorder that predominantly affects postmenopausal women. It is mostly caused by solitary tumours within the parathyroid glands. Although the pathophysiology of pHPT is still incompletely understood, recent studies provide new clues on the development and cellular growth of tumours within the parathyroids associated with hypersecretion of parathyroid hormone and hypercalcaemia. The natural course of pHPT is rather benign. Nowadays, it has become an oligo- or asymptomatic disease often only detected by routine blood tests. These facts raise the question whether to perform parathyroidectomy on oligo- and asymptomatic patients with pHPT or whether it is possible to monitor these patients without surgery. The aim of this article is to review the literature as regards (i) the pathophysiological mechanisms that underlie parathyroid neoplasia and (ii) the defective calcium-sensing in patients with pHPT (iii) environmental and/or genetic risk factors that predispose to or promote parathyroid neoplasia, as well as (iv) alternative approaches to treat oligo- and asymptomatic patients with pHPT medically. [source] Insulin resistance is not coupled with defective insulin secretion in primary hyperparathyroidismDIABETIC MEDICINE, Issue 10 2009F. Tassone Abstract Aims, An increased frequency of both impaired glucose tolerance and Type 2 diabetes mellitus (DM) has been reported in primary hyperparathyroidism (pHPT), thus we sought to investigate insulin sensitivity and insulin secretion in a large series of pHPT patients. Subjects and methods, One hundred and twenty-two consecutive pHPT patients without known DM were investigated [age (mean ± sd) 59.3 ± 13.6 years, body mass index (BMI) 25.7 ± 4.2 kg/m2; serum calcium 2.8 ± 0.25 mmol/l; PTH 203.2 ± 145.4 ng/l]. Sixty-one control subjects were matched, according to the degree of glucose tolerance, in a 2 : 1 patient:control ratio. Fasting- and oral glucose tolerance test-derived estimates of insulin sensitivity and secretion were determined by means of the quantitative insulin sensitivity check index (QUICKI) and the insulin sensitivity index (ISI) composite. Results, Both the QUICKI and ISI composite were lower in pHPT patients than control subjects (P < 0.03 and P < 0.05, respectively) after adjusting for age, systolic blood pressure and BMI. Conversely, all insulin secretion estimates were significantly increased in pHPT patients than in control subjects (P < 0.04 and P < 0.03, respectively) and after adjusting for age, systolic blood pressure and BMI. Log serum calcium levels were negatively associated with the QUICKI and log ISI composite (R = ,0.30, P = 0.001; R = ,0.23, P = 0.020, respectively) in pHPT patients. Serum calcium levels significantly and independently contributed to impaired insulin sensitivity in multivariate analysis (QUICKI as dependent variable: , = ,0.31, P = 0.004, R2 = 0.15; log ISI composite as dependent variable: , = ,0.29, P = 0.005, R2 = 0.16). Conclusions, Our study confirms a reduction in both basal and stimulated insulin sensitivity in primary hyperparathyroidism, in spite of increased insulin secretion. Moreover, our data show for the first time a significant relationship between hypercalcaemia and insulin sensitivity in this condition. [source] Cytopathologist-performed ultrasound-guided fine-needle aspiration of parathyroid lesions,DIAGNOSTIC CYTOPATHOLOGY, Issue 5 2010David Lieu M.D., M.B.A. Abstract The gold standard to determine the cause of primary hyperparathyroidism (PHPT) is bilateral neck exploration. As most cases are caused by parathyroid adenoma, there is a movement toward preoperative localization of the abnormal gland by ultrasound and/or Tc99 -sestamibi scan and minimally invasive parathyroidectomy. Nonpalpable thyroid nodules are common and cannot be differentiated from parathyroid lesions by imaging alone. This study examines cytopathologist-performed ultrasound-guided fine-needle aspiration (UG-FNA) in diagnosis of parathyroid lesions. Between January 1, 2007 and December 31, 2008, seven patients with PHPT or other parathyroid lesions with one or more sonographically-visible thyroid masses underwent cytopathologist-performed UG-FNA with immediate cytological evaluation (ICE). One mass was palpable and nine were nonpalpable. Three parathyroid adenomas, two benign colloid nodules, one papillary carcinoma, three parathyroid cysts, and one thyroid cyst were diagnosed. The nodules in three patients with parathyroid adenomas were identified as follicular lesion/neoplasm on ICE. Additional UG-FNA passes were made to obtain tissue for immunohistochemistry stains, which confirmed parathyroid origin. Two of these patients had a separate benign colloid nodule and one had a thyroid cyst diagnosed by UG-FNA. The PHPT patient with papillary carcinoma on UG-FNA had the malignancy confirmed at surgery and a sonographically occult parathyroid adenoma. The three patients with thyroid cysts identified by radiology were suspected of being parathyroid cysts on the basis of real-time sonographic features at the biopsy table. The clear cyst fluid obtained by UG-FNA had markedly elevated PTH. Cytopathologist-performed UG-FNA can distinguish between parathyroid and thyroid nodules in patients with suspected parathyroid lesions. Diagn. Cytopathol. 2010. © 2009 Wiley-Liss, Inc. [source] Accuracy and definitive interpretation of preoperative technetium 99m sestamibi imaging based on the discipline of the reader,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2009Ayesha N. Khalid MD Abstract Background. Technetium 99m sestamibi scans have become a principal means of localizing parathyroid adenomas. Its accuracy and reliability has allowed for the proliferation of minimal access parathyroidectomy. Localizing interpretation of these scans often drives referral of hyperparathyroid patients for surgery. Interpretation of these scans may differ between nuclear medicine physicians and surgeons. Methods. We reviewed patients (N = 65) with digital images from an academic medical center with the diagnosis of primary hyperparathyroidism. We assessed the willingness to define an adenoma's location, the interrater reliability, and the accuracy of technetium (Tc-99m) sestamibi read by a surgeon and a nuclear medicine physician. Results. There was poor correlation between both readers for assessment of quality of images (k = 0.54, 0.07) but very good correlation for adenoma location (k = 0.81). Conclusion. Both readers had good accuracy in predicting the location of the parathyroid adenoma. The surgeon was more likely to call a scan positive. © 2008 Wiley Periodicals, Inc. Head Neck, 2009 [source] Comparison of perioperative management and outcome of parathyroidectomy between older and younger patientsHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 11 2008Gideon Bachar MD Abstract Background. The aim of this study was to compare the clinical status, surgical course, and outcomes of patients with primary hyperparathyroidism (PHPT), over the age of 70, with younger patients. Methods. Between 1996 and 2006, 951 patients underwent parathyroidectomies for PHPT, of whom 190 were over the age of 70. Patient data were collected from chart reviews and a computerized database. Results. Fewer older patients were asymptomatic at presentation. No between-group differences in serum calcium were seen; however, parathyroid hormone (PTH) levels were higher in the older group. Hospitalization time was longer for the elderly. Duration of surgery, surgical success rates, and postoperative complications were similar between the 2 groups. Conclusion. Surgical treatment of PHPT has both physiological benefits and helps to preserve quality of life. Our findings suggest that there is no practical difference in perioperative management and surgical outcomes for older patients. Surgeons should consider parathyroidectomy in PHPT patients regardless of age. © 2008 Wiley Periodicals, Inc. Head Neck, 2008 [source] Evolution of maxillofacial brown tumors after parathyroidectomy in primary hyperparathyroidismHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 11 2008Jaime Alonso Reséndiz-Colosia MD Abstract Background. Brown tumor occasionally affects the facial bones. Clinically, these lesions can be mistaken for a neoplasm. Opinions are divided on the course of management of the bony lesions once parathyroidectomy has been carried out. Methods. We treated 22 patients with primary hyperparathyroidism and osteitis fibrosa cystica and observed their clinical and biochemical recovery. Results. Fifteen patients (68.2%) had brown tumors in mandible, and 7 (31.8%) in maxilla. After parathyroidectomy, 21 patients had normal total serum calcium values. All brown tumors presented a spontaneous progressive regression; in 18cases, regression was total, with a mean time period of 10months. Two patients had partial regression after nearly 2years. Another 2 patients were lost to follow-up. Conclusions. After successful parathyroid surgery, the bony lesions tended to regress spontaneously, either partially or completely. However, if the lesion is disfiguring or symptomatic, surgical excision may be indicated. © 2008 Wiley Periodicals, Inc. Head Neck, 2008 [source] Spontaneous remission of primary hyperparathyroidism: A case report and meta-analysis of the literatureHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 1 2006Christopher T. Wootten MD Abstract Background. In a minority of patients, primary hyperparathyroidism spontaneously remits either by autoinfarction or by hemorrhage into or around the adenoma. We describe a case of autoparathyroidectomy occurring in a 63-year-old man 9 years after three parathyroid glands were removed during a total thyroidectomy. This case is compared with 50 previously reported cases of autoparathyriodectomy, and a meta-analysis is performed. Methods. Case report, literature review, and meta-analysis were done using statistical software (SigmaStat 2.0, SPSS, Chicago). Results. Fifty cases of autoparathyroidectomy were summarized according to the three etiologies. The greatest biochemical aberration was found in the acute intracapsular hemorrhage group, with [Ca++] falling from a mean 15.1 mg/dL to 8.9 mg/dL. The average drop in parathyroid hormone was 69% across all groups, comparing favorably to surgical resection. Conclusions. Autoparathyroidectomy is a rare but described outcome of unoperated primary hyperparathyroidism that may delay or supplant operative management. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX,XXX, 2005 [source] Preoperative evaluation of patients with parathyroid adenoma: Role of high-resolution ultrasonography,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 1 2002David Ulanovski MD Abstract Background Unilateral parathyroid exploration with adenoma removal and identification of a normal parathyroid gland is a controversial surgical approach to the treatment of primary hyperparathyroidism. The aim of this study was to evaluate the ability of high-resolution ultrasonography to localize adenomas preoperatively and to assess the effect of such localization on operative time. Methods One hundred twenty consecutive previously non-operated patients with primary hyperparathyroidism underwent ultrasonography before surgery, which consisted of unilateral neck exploration. The procedure was changed to bilateral exploration when justified by the surgical findings. Results The sensitivity and positive predictive value of the ultrasonographic examinations were 89% and 98%, respectively. These results were obtained regardless of the size of the adenoma. No significant difference was found in the presence of thyroid multinodular disease (p = .2). A positive sonographic examination decreased the operative time to an average of 59 minutes. The average size of the adenomas was 19 mm (range, 4,55 mm). A positive and highly statistically significant correlation was found between adenoma size and both preoperative calcium level (p = .01) and parathyroid hormone level (p = .0001). Conclusions In experienced hands, high-resolution ultrasonography can be a cost-effective means of localizing parathyroid adenomas when unilateral neck exploration is considered the acceptable surgical approach. © 2002 John Wiley & Sons, Inc. Head Neck 24: 1,5, 2002. [source] Oral calcium supplementation associated with decreased likelihood of nephrolithiasis prior to surgery for hyperparathyroidismINTERNATIONAL JOURNAL OF UROLOGY, Issue 12 2007Matthew R Cooperberg Abstract: We aimed to assess the impact of oral calcium supplementation (OCS) on the prevalence of nephrolithiasis among a cohort of patients undergoing surgery for primary hyperparathyroidism (PHPT). There were 339 patients undergoing surgery for PHPT with detailed past medical history data that were analyzed. 73 patients (22%) had a history of nephrolithiasis prior to parathyroid surgery. Nephrolithiasis was more common among men than women (40% vs 15%, P < 0.001), despite the predominance of women (73% of patients) with hyperparathyroidism. 83 patients (25%) used OCS. OCS was associated with a lower prevalence of nephrolithiasis (9.6% vs 25.4% without OCS, P = 0.002). This protective effect included both men and women (rates of nephrolithiasis with and without supplements: men 19% vs 46%, P = 0.027; women 7% vs 17%, P = 0.04). The mechanism for the apparent protective effect of OCS on rates of nephrolithiasis is unclear, and further research is required to elucidate the variable penetrance of nephrolithiasis among PHPT patients. [source] Usefulness of diagnostic imaging in primary hyperparathyroidismINTERNATIONAL JOURNAL OF UROLOGY, Issue 1 2003KAZUYA SEKIYAMA Abstract Background : In patients with primary hyperparathyroidism, prevention of urinary stone recurrence can be achieved by surgical removal of the enlarged parathyroid gland. To ensure the efficacy of surgery for primary hyperparathyroidism, preoperative localization of the enlarged gland is important. In the present study, usefulness of diagnostic imaging for localization of the enlarged gland was investigated in primary hyperparathyroidism. Methods : We retrospectively examined the findings of imaging studies and clinical records in 79 patients (97 glands) who underwent surgical treatment for primary hyperparathyroidism at Chiba University Hospital between 1976 and 2000. The detection rates of accurate localization were investigated for imaging techniques, such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI), thallium-201 and technetium-99m pertechnetate (Tl-Tc) subtraction scintigraphy and 99mTc-methoxyisobutylisonitrile (MIBI) scintigraphy, and analysed in relation to the size and weight of the gland and pathological diagnosis. Results : The detection rates by US, CT, MRI, Tl-Tc subtraction scintigraphy and MIBI scintigraphy were 70%, 67%, 73%, 38% and 78%, respectively. The overall detection rate changed from 50% to 88% before and after 1987. The detection rate of MIBI scintigraphy was superior to Tl-Tc subtraction scintigraphy. Conclusion : In primary hyperparathyroidism, improvement of accurate localization of an enlarged parathyroid gland was demonstrated along with recent advances in imaging techniques including MIBI scintigraphy. [source] Surveillance for Early Detection of Aggressive Parathyroid Disease: Carcinoma and Atypical Adenoma in Familial Isolated Hyperparathyroidism Associated With a Germline HRPT2 Mutation,,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 10 2006Thomas G Kelly Abstract Familial hyperparathyroid syndromes involving mutations of HRPT2 (also CDC73), a tumor suppressor, are important to identify because the relatively high incidence of parathyroid malignancy associated with such mutations warrants a specific surveillance strategy. However, there is a dearth of reports describing experience with surveillance and early detection informed by genetic insight into this disorder. Introduction: Familial isolated hyperparathyroidism (FIHP) is a rare cause of parathyroid (PT) tumors without other neoplasms or endocrinopathies. Germline mutations in CASR, MEN1, and rarely, HRPT2 have been identified in kindreds with FIHP. HRPT2 mutations may be enriched in FIHP families with PT carcinoma, underscoring the importance of identifying causative mutations. Materials and Methods: A 13-year-old boy, whose father had died of PT carcinoma, developed primary hyperparathyroidism. A left superior PT mass was identified by ultrasonography and removed surgically. Aggressive histological features of the boy's tumor included fibrous trabeculae, mitoses, and microscopic capsular infiltration. Two years later, under close biochemical surveillance, primary hyperparathyroidism recurred 5 months after documentation of normocalcemia and normal parathyroid status. Ultrasound and MRI identified a newly enlarged right superior PT gland but indicated no recurrent disease in the left neck. Histologic features typical of a benign adenoma were evident after surgical extirpation of the gland. Results: Leukocyte DNA analysis revealed a frameshift mutation in exon 2 of HRPT2. The initial tumor manifested the expected germline HRPT2 mutation, plus a distinct somatic frameshift mutation, consistent with the Knudson "two hit" concept of biallelic inactivation of a classic tumor suppressor gene. Genetic screening of the patient's 7 asymptomatic and previously normocalcemic siblings revealed three with the same germline HRPT2 mutation. One of the siblings newly identified as mutation-positive was noted to be hypercalcemic at the time of the genetic screening. He was found to have a PT adenoma with aggressive features. Two of the five children of another mutation-positive sibling also carry the same HRPT2 mutation. Conclusions: Despite the reported rarity of HRPT2 mutations in FIHP, a personal or family history of PT carcinoma in FIHP mandates serious consideration of germline HRPT2 mutation status. This information can be used in diagnostic and management considerations, leading to early detection and removal of potentially malignant parathyroid tumors. [source] Chondrosarcoma in Association With Primary Hyperparathyroidism,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 7 2004Ajay Bhatia Abstract We describe two female patients, 66 and 36 years of age, with both primary hyperparathyroidism and chondrosarcoma. Case 1 had a chondrosarcoma of the right scapula, and case 2 had chondrosarcoma of the left proximal tibia. Both patients underwent surgical resection of their chondrosarcoma and subsequent parathyroid surgery. Histological analysis of the excised parathyroid in case 1 showed a parathyroid carcinoma and in case 2 showed a parathyroid adenoma. Including these two patients, there is now a total of six cases that have been reported in the literature describing the association between hyperparathyroidism and bone malignancy. We believe that this small number makes it unlikely that there is an association between these two conditions, although we speculate that there may be an underlying genetic basis. [source] Familial Hypocalciuric Hypercalcemia Caused by an R648stop Mutation in the Calcium-Sensing Receptor Gene ,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 12 2002Mika Yamauchi Abstract In this study, we report an 84-year-old female proband in a Japanese family with familial hypocalciuric hypercalcemia (FHH) caused by an R648stop mutation in the extracellular calcium-sensing receptor (CaR) gene. At the age of 71 years, she presented with hypercalcemia (11.4 mg/dl), hypocalciuria (Cca/Ccr = 0.003), hypermagnesemia (2.9 mg/dl), and a high-serum parathyroid hormone (PTH) level (midregion PTH, 3225 [160,520] pg/ml). At the age of 74 years, a family screening was carried out and revealed a total of 9 hypercalcemic individuals (all intact PTH values <62 pg/dl) among 17 family members tested, thus, being diagnosed as FHH. Two and one-half of three clearly enlarged parathyroid glands were resected, because persistently high PTH levels (intact PTH, 292 pg/ml; midregion PTH, 5225 pg/ml) and the presence of a markedly enlarged parathyroid gland by several imaging modalities (ultrasonography, computed tomography [CT], magnetic resonance imaging [MRI], and subtraction scintigraphy) suggested coexistent primary hyperparathyroidism (pHPT); however, hypercalcemia persisted postoperatively. Histological and immunohistochemical examination revealed that the resected parathyroid glands showed lipohyperplasia as well as normally expressed Ki67, vitamin D receptor (VDR), and the CaR. Sequence analysis disclosed that the proband and all affected family members had a heterozygous nonsense (R648stop) mutation in the CaR gene. This mutation is located in the first intracellular loop; thus, it would be predicted to produce a truncated CaR having only one transmembrane domain (TMD) and lacking its remaining TMDs, intracellular loops, and C-terminal tail. Western analysis of biotinylated HEK293 cells transiently transfected with this mutant receptor showed cell surface expression of the truncated protein at a level comparable with that of the wild-type CaR. The mutant receptor, however, exhibited no increase in intracellular free calcium concentration (Ca2+i) when exposed to high extracellular calcium concentrations (Ca2+o). The proband's clinical course was complicated because of associated renal tubular acidosis (RTA) and nephrotic syndrome. However, it was unclear whether their association affected the development of elevated serum PTH and parathyroid gland enlargement. This report is the first to show that an R648stop CaR mutation yields a truncated receptor that is expressed on the cell surface but is devoid of biological activity, resulting in FHH. [source] Parathyroid Hormone-Related Protein Induced Coupled Increases in Bone Formation and Resorption Markers for 7 Years in a Patient With Malignant Islet Cell Tumors,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 5 2002Ph.D., Yasuhiro Takeuchi M.D. Abstract Parathyroid hormone-related protein (PTHrP) and PTH share the common PTH/PTHrP receptor. Although an elevated level of circulating PTHrP in patients with malignancies causes hypercalcemia as does PTH, chronic and systemic effects of PTHrP on bone metabolism in humans are not well understood because tumor-burden patients showing hypercalcemia usually have a poor prognosis. We investigated bone and calcium metabolism in a patient with malignant islet cell tumors showing hypercalcemia due to the elevated plasma PTHrP level for 7 years. Hypercalcemia and hypercalciuria continued throughout the clinical course in spite of frequent infusions of bisphosphonates. Bone resorption markers and a bone formation marker were consistently elevated as seen in primary hyperparathyroidism, a disease caused by an autonomous hypersecretion of PTH. Based on biochemical measurements including bone markers and serum 1,25-dihydroxyvitamin D, the clinical features of this case essentially are the same as those of primary hyperparathyroidism except for the elevated level of plasma PTHrP with suppressed intact PTH level. Therefore, it is suggested that chronic and systemic effects of PTHrP on bone as well as calcium metabolism are indistinguishable from those of PTH in human. [source] Cancellous Bone Remodeling Occurs in Specialized Compartments Lined by Cells Expressing Osteoblastic MarkersJOURNAL OF BONE AND MINERAL RESEARCH, Issue 9 2001Ellen M. Hauge Abstract We describe a sinus, referred to as a bone remodeling compartment (BRC), which is intimately associated with cancellous bone remodeling. The compartment is lined on its marrow side by flattened cells and on its osseous side by the remodeling bone surface, resembling a roof of flattened cells covering the bone surface. The flat marrow lining cells are in continuity with the bone lining cells at the margins of the BRC. We examined a large number of diagnostic bone biopsy specimens received during recent years in the department. Furthermore, 10 patients (8 women and 2 men, median age 56 [40,69] years) with the high turnover disease of primary hyperparathyroidism who were treated with parathyroidectomy and followed for 3 years were included in the histomorphometric study. Bone samples for the immuno-enzyme staining were obtained from an amputated extremity of child. The total cancellous bone surface covered by BRC decreases by 50% (p < 0.05) following normalization of turnover and is paralleled by a similar 50% decrease in remodeling surface (p < 0.05). The entire eroded surface and two-thirds of the osteoid surface are covered by a BRC. BRC-covered uncompleted walls are 30% (p < 0.05) thinner than those without a BRC. This indicates that the BRC is invariably associated with the early phases of bone remodeling, that is, bone resorption, whereas it closes during the late part of bone formation. Immuno-enzyme staining shows that the flat marrow lining cells are positive for alkaline phosphatase, osteocalcin, and osteonectin, suggesting that they are bone cells. The first step in cancellous bone remodeling is thought to be the lining cells digesting the unmineralized matrix membrane followed by their disappearance and the arrival of the bone multicellular unit (BMU). We suggest that the lining cell barrier persists during bone remodeling; that the old lining cells become the marrow lining cells, allowing bone resorption and bone formation to proceed under a common roof of lining cells; that, at the end of bone formation, new bone lining cells derived from the flattened osteoblasts replace the marrow lining cells thereby closing the BRC; and that the two layers of lining cells eventually becomes a single layer. The integrity of the osteocyte-lining cell system is reestablished by the new generation of lining cells. The BRC most likely serves multiple purposes, including efficient exchange of matrix constituents and minerals, routing, monitoring, or modulating bone cell recruitment, and possibly the anatomical basis for the coupling of bone remodeling. [source] Development of a Novel Immunoradiometric Assay Exclusively for Biologically Active Whole Parathyroid Hormone 1,84: Implications for Improvement of Accurate Assessment of Parathyroid FunctionJOURNAL OF BONE AND MINERAL RESEARCH, Issue 4 2001Ping Gao Abstract We developed a novel immunoradiometric assay (IRMA; whole parathyroid hormone [PTH] IRMA) for PTH, which specifically measures biologically active whole PTH(1,84). The assay is based on a solid phase coated with anti-PTH(39,84) antibody, a tracer of125I-labeled antibody with a unique specificity to the first N-terminal amino acid of PTH(1,84), and calibrators of diluted synthetic PTH(1,84). In contrast to the Nichols intact PTH IRMA, this new assay does not detect PTH(7,84) fragments and only detects one immunoreactive peak in chromatographically fractionated patient samples. The assay was shown to have an analytical sensitivity of 1.0 pg/ml with a linear measurement range up to 2300 pg/ml. With this assay, we further identified that the previously described non-(1,84)PTH fragments are aminoterminally truncated with similar hydrophobicity as PTH(7,84), and these PTH fragments are present not only in patients with secondary hyperparathyroidism (2° -HPT) of uremia, but also in patients with primary hyperparathyroidism (1° -HPT) and normal persons. The plasma normal range of the whole PTH(1,84) was 7,36 pg/ml (mean ± SD: 22.7 ± 7.2 pg/ml, n = 135), whereas over 93.9% (155/165) of patients with 1° -HPT had whole PTH(1,84) values above the normal cut-off. The percentage of biologically active whole PTH(1,84) (pB%) in the pool of total immunoreactive "intact" PTH is higher in the normal population (median: 67.3%; SD: 15.8%; n = 56) than in uremic patients (median:53.8%; SD: 15.5%; n = 318; p < 0.001), although the whole PTH(1,84) values from uremic patients displayed a more significant heterogeneous distribution when compared with that of 1° -HPT patients and normals. Moreover, the pB% displayed a nearly Gaussian distribution pattern from 20% to over 90% in patients with either 1° -HPT or uremia. The specificity of this newly developed whole PTH(1,84) IRMA is the assurance, for the first time, of being able to measure only the biologically active whole PTH(1,84) without cross-reaction to the high concentrations of the aminoterminally truncated PTH fragments found in both normal subjects and patients. Because of the significant variations of pB% in patients, it is necessary to use the whole PTH assay to determine biologically active PTH levels clinically and, thus, to avoid overestimating the concentration of the true biologically active hormone. This new assay could provide a more meaningful standardization of future PTH measurements with improved accuracy in the clinical assessment of parathyroid function. [source] Preoperative localization of parathyroid adenoma with sonography and 99mTc-sestamibi scintigraphy in primary hyperparathyroidismJOURNAL OF CLINICAL ULTRASOUND, Issue 4 2007Ilaria Grosso MD Abstract Purpose. To evaluate the sensitivity, specificity, and usefulness of dual-phase 99mTc-Sestamibi scintigraphy (SS) and sonography (US) of the neck, alone and in combination, as noninvasive adenoma localizing procedures in patients with primary hyperparathyroidism prior to parathyroidectomy. Methods. We retrospectively analyzed the charts of 79 patients with parathyroid (PT) adenomas and confirmed diagnosis of hyperparathyroidism who were evaluated with SS and US prior to successful parathyroidectomy. Results. Ninety-three adenomas were removed during bilateral neck exploration. SS alone showed a sensitivity of 76% and a specificity of 79% compared with 89% and 75%, respectively, for US performed after SS on the same day. Combination of the 2 procedures yielded a sensitivity of 89% and a specificity of 90%, with 22% discordant results. The differences in sensitivity and specificity between the 2 techniques alone or in combination were not statistically significant. Conclusions. No benefit was gained from using both SS and US for the preoperative localization of PT adenomas in patients with primary hyperparathyroidism. Each technique can be negatively affected by thyroid enlargment and nodularity. US, when performed by a skilled operator, is a reliable tool for PT adenoma localization. If the US findings are inconclusive, SS should be used. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2007 [source] Surviving extreme hypercalcaemia , a case report and review of the literatureJOURNAL OF INTERNAL MEDICINE, Issue 1 2005K. MARIENHAGEN Abstract. We report a case of extreme hypercalcaemia associated with a parathyroid adenoma in a young man. The patient presented with classical symptoms of a hypercalcaemic syndrome, and serum calcium and parathyroid hormone levels were 6.92 mmol L,1 and 70.2 pmol L,1 respectively. After stabilizing the patient and reducing the calcium level, a parathyroidectomy was performed. The postoperative course was uneventful with rapidly resolving clinical symptoms. Hypercalcaemic crisis is a rare but life-threatening complication of primary hyperparathyroidism. It should be suspected in acutely ill patients complaining of muscular weakness, gastrointestinal and cerebral symptoms. To reduce mortality, it is essential to correctly diagnose the condition without delay and provide appropriate emergency management correcting hypercalcaemia and dehydration. Successful parathyroidectomy quickly relieves symptoms and prevents recurrence. [source] Primary hyperparathyroidism in 29 dogs: diagnosis, treatment, outcome and associated renal failureJOURNAL OF SMALL ANIMAL PRACTICE, Issue 1 2005R. N. A. Gear Objectives: To review the records of 29 dogs diagnosed with primary hyperparathyroidism and see if any factors correlate with renal failure. Methods: Dogs were selected retrospectively from case files from the QVSH and the QMH. Results: The majority of dogs were middle-aged and four were keeshonds. The primary presenting complaints were polyuria and polydipsia. All dogs had an elevated total and ionised plasma calcium concentration. Plasma phosphate concentrations were variable. Ultrasonography of the parathyroid gland revealed nodular enlargement which was found to correlate well with surgical findings. The majority of dogs underwent surgical parathyroidectomy. Five cases were treated by ultrasound-guided chemical ablation of the parathyroid gland, of which only two cases showed a partial response. Three dogs were euthanased within a week of presentation. Seven other dogs had renal failure diagnosed either at presentation or up to six months after parathyroidectomy. The development of renal failure was correlated with total calcium concentration but did not correlate with any other factor, including the calcium phosphate product. Thirteen treated dogs were known to be alive at the time of writing, which was six months to 3.5 years after parathyroidectomy. Clinical Significance: Primary hyperparathyroidism cases with high total calcium were more likely to develop renal failure in this group of dogs; however, the calcium phosphate product did not seem to be a useful predictor. Ultrasound-guided chemical ablation seemed to have limited advantage over surgery. [source] Lymphomatoid papulosis associated with parathyroid nodular hyperplasia: report of a caseJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2004M Aguilar-Bernier ABSTRACT The association of cutaneous lymphoproliferative diseases and primary hyperparathyroidism (PHP) is infrequent, with only three cases reported to date. We present a patient with lymphomatoid papulosis (LyP) who developed hypercalcaemia secondary to parathyroid nodular hyperplasia. A review of the literature has revealed no similar cases; we therefore believe this to be the first description of the association between LyP and PHP. [source] Idiopathic Hypercalcemia in CatsJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 6 2000A.M. Midkiff Unexplained hypercalcemia has been increasingly recognized in cats since 1990. In some instances, hypercalcemia has been associated with calcium oxalate urolithiasis, and some affected cats have been fed acidifying diets. We studied the laboratory findings, clinical course, and treatment of 20 cats with idiopathic hypercalcemia. Eight (40%) of the cats were longhaired and all 14 cats for which adequate dietary history was available had been fed acidifying diets. Clinical signs included vomiting (6 cats), weight loss (4 cats), dysuria (4 cats), anorexia (3 cats), and inappropriate urinations (3 cats). Hypercalcemia was mild to moderate in severity, and serum parathyroid hormone concentrations were normal or low. Serum concentrations of phosphorus, parathyroid hormone-related peptide, 25-hydroxycholecalciferol, and calcitriol were within the reference range in most cats. Diseases commonly associated with hypercalcemia (eg, neoplasia, primary hyperparathyroidism) were not identified despite thorough medical evaluations and long-term clinical follow-up. Azotemia either did not develop (10 cats) or developed after the onset of hypercalcemia (3 cats), suggesting that renal failure was not the cause of hypercalcemia in affected cats. Seven of 20 cats (35%) had urolithiasis, and in 2 cats uroliths were composed of calcium oxalate. Subtotal parathyroidectomy in 2 cats and dietary modification in 11 cats did not result in resolution of hypercalcemia. Treatment with prednisone resulted in complete resolution of hypercalcemia in 4 cats. [source] Ultrasonography: Highly Accuracy Technique for Preoperative Localization of Parathyroid Adenoma,THE LARYNGOSCOPE, Issue 9 2008Bassam Abboud MD Abstract Objectives/Hypothesis: This study evaluates the accuracy of ultrasonography in guided unilateral parathyroidectomy to treat primary hyperparathyroidism. Study Design: Retrospective study. Methods: Two hundred fifty-three patients with primary hyperparathyroidism underwent preoperative ultrasonography. Two groups were defined. Group 1 included the patients in whom the preoperative cervical ultrasound localized one abnormal parathyroid gland; these patients underwent unilateral surgical exploration of the neck under local anesthesia. Group 2 included the patients who had a bilateral neck exploration under general anesthesia when the preoperative examination was equivocal or failed to localize the lesion, when concomitant thyroid pathology indicated thyroidectomy, and when justified by the surgical findings. Results: Sensitivity and positive predictive value of ultrasonography in detecting abnormal parathyroid gland were 96% and 98%, respectively. Cervical ultrasound correctly identified, 96% and 85% of abnormal glands in groups 1 and 2, respectively. The presence of thyroid nodular disease did not affect ultrasonographic accuracy. Sonographic examination decreased the operative time of parathyroidectomy to an average of 15 minutes. Mediastinal and retroesophageal localizations of abnormal parathyroid gland adversely affected the accuracy of the ultrasound. No cervical hematoma was noted. Transient recurrent laryngeal nerve palsy occurred in four patients. Twenty-three patients required postoperative calcium supplementation for 2 to 4 months, and all were normocalcemic at follow-up. Conclusions: Cervical ultrasound is a reliable preoperative exploration allowing parathyroidectomy via unilateral approach under local anesthesia. [source] | ||||||||||||||||||||||