Presentation Of Primary Hyperparathyroidism Research Articles

Multiple pathological fractures with a hidden aetiology

Bone disease is a well-recognised complication of primary hyperparathyroidism (PHPT). With early diagnosis of PHPT, florid bony changes such as brown tumours, lytic lesions and pathological fractures are rarely seen now. Here, we report an unusual presentation of PHPT in the current era. A 47-year-old lady was evaluated for multiple fragility fractures. Biochemical assessment confirmed primary hyperparathyroidism. DEXA scan showed severe osteoporosis. Left parathyroid adenoma was localised with imaging. She had multiple brown tumours. She underwent parathyroid adenoma excision and recovered after surgery with improved quality of life. Proper evaluation for secondary causes of osteoporosis is vital especially in premenopausal women. Early diagnosis and treatment of PHPT prevent disastrous complications.

The clinical presentation of primary hyperparathyroidism: effects of obesity and vitamin D deficiency

The clinical presentation of primary hyperparathyroidism: effects of obesity and vitamin D deficiency

An uncommon manifestation in the common presentation of primary hyperparathyroidism

An uncommon manifestation in the common presentation of primary hyperparathyroidism

Unveiling the Hidden Stroke Threat in Patients With Atrial Fibrillation and Primary Hyperparathyroidism

Recent American College of Cardiology (ACC), American Heart Association (AHA), American College of Clinical Pharmacy (ACCP), and Heart Rhythm Society (HRS) guidelines suggest that patients with atrial fibrillation (AF) at intermediate to low annual risk of ischemic stroke can benefit from consideration of factors that might modify their risk of stroke. The role of nontraditional risk factors, such as primary hyperparathyroidism (PHPT), remains unexplored. In our study, we investigated the potential association between PHPT and the risk of ischemic stroke in patients with AF. Using data from the Nationwide Inpatient Sample Database, a retrospective cohort study focused on the adult population with AF, we stratified the participants based on PHPT presence. Demographic information, co-morbidities, and hospitalization details were extracted using International Classification of Diseases, Tenth revision codes. Propensity score matching was applied, encompassing over 20 confounding variables, including the risk factors outlined in the CHA2DS2-VASc (Congestive heart failure (C), Hypertension (H), Age ≥75 years (A₂), Diabetes Mellitus (D), Stroke/Transient Ischemic Attack (TIA)/Thromboembolism (S₂), Vascular disease (V), Age 65-74 years (A), Sex category [female] (Sc)) score. Multivariate logistic regression analysis was performed after matching to assess the independent impact of PHPT as an ischemic stroke risk factor. A total of 2,051 of the identified 395,249 patients with AF had PHPT. The PHPT group had an average age of 74 years and consisted of more women (66.1% vs 53.0%). After matching, it was observed that the PHPT group had longer hospital stays (5 vs 4 days) and higher hospitalization charges ($45,126 vs $36,644). This group exhibited higher rates of ischemic stroke (6.0% vs 4.4%) and mortality (6.3% vs 4.9%). The adjusted outcomes showed a 1.4-fold increased risk for ischemic stroke and a 1.32-fold increased risk for mortality in the PHPT cohort. The subgroup analysis showed a higher incidence of mortality in men with a high CHA2DS2-VASc score. In conclusion, this study highlights a marked association between PHPT and ischemic stroke in patients with AF, independent of the conventional CHA2DS2-VASc score. The potential mechanisms implicated include vascular changes, cardiac dysfunction, and coagulation cascade alterations. The presence of PHPT should be taken into consideration when deciding the assessment of thromboembolic risk.

Calcium/phosphate ratio: an additional tool for the clinical management of asymptomatic primary hyperparathyroidism?

Serum calcium/phosphate ratio (Ca/P) has been recently proposed as an additional tool to identify primary hyperparathyroidism (PHPT), especially in patients with subclinical presentation, with a proposed cut-off of 3.3 when both values are expressed in mg/dL. No data are available on the relationship between Ca/P and the clinical presentation of PHPT. We thus evaluated this relationship in a large, single-center, unselected series. 515 consecutive PHPT patients (mean age 65 ± 13.15years, 77.1% females) were retrospectively evaluated at diagnosis. Mean Ca/P was 4.54 ± 1.5 (range 2.36-13.9), being higher than 3.3 in 88.5% of patients. Ca/P was significantly higher in (1) males, (2) symptomatic PHPT, (3) patients with 25-hydroxy vitamin D levels lower than 20μg/L, (4) patients with osteitis fibrosa cystica, (5) patients with T score < - 2.5 at the radial site. In a multivariate regression analysis, Ca/P resulted significantly associated with PTH levels. After the exclusion of 57 patients with asymptomatic PHPT (aPHPT) patients and serum Ca higher than 1mg/dL above the upper limit of normal range, no differences were found in Ca/P between aPHPT meeting or not surgical criteria. In PHPT Ca/P ratio is associated with increased biochemical and clinical severity of disease and represents a direct indicator of clinical bone damage. However, it does not seem an additional tool to identify aPHPT patients reaching surgical indication.

Vitamin D Deficiency and the Presentation of Primary Hyperparathyroidism: A Mini Review.

The clinical presentation of primary hyperparathyroidism (PHPT) has evolved over the years from a symptomatic disorder to a predominantly asymptomatic condition. Altered vitamin D metabolism seems to play a role in the presentation of PHPT and may exacerbate the severity of disease. The epidemiology of PHPT differs in the developing versus the developed world, where more severe phenotypes occur in regions where vitamin D deficiency is common. Although it has been validated that patients with PHPT should be vitamin D sufficient, the threshold to supplement in relation to the severity of PHPT and the degree of vitamin D deficiency remains controversial. This review will highlight some of the controversy regarding vitamin D deficiency and the different phenotypes of PHPT.

Rare Classic Presentation of Primary Hyperparathyroidism: A Case Report and Literature Review

Rare Classic Presentation of Primary Hyperparathyroidism: A Case Report and Literature Review

Brownish limb tumor as a presentation of primary hyperparathyroidism

43-year-old male, with tumor in the anterior region of the right lower limb (figure 1) and biopsy compatible with brown tumor. He presented total serum calcium 12.3 mg/dl and 13 mg/dl (NR: 8.3-10.3), parathormone (PTH) 1057 pg/ml (NR 15-65). With the diagnosis of primary hyperparathyroidism, a 99mTc-MIBI parathyroid scintigraphy with SPECT was requested, which showed hyperfunctioning parathyroid tissue in the projection of the left inferior parathyroid gland. Hand radiographs showed a lytic lesion in punch in the distal phalanx of the first finger of the left hand (figure 2); in the clavicle radiograph: lytic lesion of 10 mm x 8 mm in the right clavicle, no alterations were evidenced in the skull radiograph.

Rare Presentation of Primary Hyperparathyroidism in a Young Woman.

Even though primary hyperparathyroidism (PHPT) is a common endocrine disorder, due to better and more regular screening, the usual presentation is only seen in less than 15% of cases of PHPT. The authors present the case of a young female patient with a previous medical history of depression and nephrolithiasis, with one year of bone pain, that had become progressively worse and disabling. In the initial work-up, several lytic bone lesions and moderate hypercalcemia were found, leading to admission of the patient in the Internal Medicine ward for investigation and treatment. The ensuing investigation revealed PHPT due to hyperfunctioning parathyroid adenoma. The patient underwent a parathyroidectomy and at the follow-up assessment two months after discharge, she reported no symptoms and a computer tomography scan showed regression of the lytic lesions. This case is a reminder that severe symptomatic PHPT, a rare form in developed countries nowadays, still exists, and even though it is a medical condition, collaboration with surgical specialties is necessary to ensure the best possible treatment and prognosis.

Widespread presentation of brown tumors mimicking multiple myeloma

Brown tumors or osteitis fibrosa cystica has become a rare presentation of primary hyperparathyroidism in up-to-date clinical practice. Here, we describe a case of longstanding untreated hyperparathyroidism presenting itself with brown tumors in a 65-year-old patient. During the diagnostic work-up of this patient, bone SPECT/CT and 18F-FDG-PET/CT revealed multiple widespread osteolytic lesions. Differentiating from other bone tumors such as multiple myeloma is challenging. In this case, the final diagnosis was made by integrating the medical history, biochemical diagnosis of primary hyperparathyroidism, pathological findings and medical imaging.

Submandibular Ectopic Parathyroid Adenoma: A Case Report.

Primary hyperparathyroidism (PHPT) is a common endocrine disorder characterized by hypercalcemia and elevated or abnormally normal parathyroid hormone (PTH) levels, due to excessive secretion of PTH by 1 or more parathyroid glands. In this report, we discuss the diagnostic and therapeutic challenges posed by ectopic parathyroid adenomas, a rare but atypical presentation of PHPT. We present the case of a 36-year-old female with PHPT due to an ectopic parathyroid adenoma located in the submandibular region. The patient presented with bone pain and was initially evaluated with routine imaging studies, which were negative. [18F] F-choline positron emission tomography (PET)/Computed tomography revealed the ectopic adenoma, leading to successful surgical management. Ectopic parathyroid adenomas are rare but can occur in various locations, and functional imaging modalities such as choline PET can aid in their detection. Surgical resection remains the definitive treatment for parathyroid adenomas, with intraoperative PTH monitoring guiding the extent of resection. Proper evaluation and management of PHPT is essential to avoid significant morbidity. Our case adds to the growing body of literature on the importance of considering ectopic locations of parathyroid adenomas in patients with PHPT.

Abstract #1373163: A Rare Case of Ectopic Parathyroid Adenoma in the Left Axillary Region

A parathyroid adenoma is a benign neoplasm that arises from parathyroid parenchymal cells. Parathyroid adenomas are the most common cause of primary hyperparathyroidism (PHPT). Ectopic parathyroid adenomas arise within the parathyroid glands located at the four poles of the thyroid [2]. Ectopic parathyroid adenomas result from aberrant migration of the gland during the early stages of development and can be identified in up to 16% of cases of PHPT [3]. They are commonly found in the mediastinum, within the thyroid, and along the path of the vagus or recurrent laryngeal nerves [4]. 45% of ectopic parathyroid adenomas are found in the paraesophageal region, 25% in the cervical thymus, 20% in the cervical mediastinum, 5% in the anterior portion of the mediastinum, and 5% at the level of the thymus [5]. A 64-year-old man with a past medical history of over 20-year history of recurrent nephrolithiasis, type II diabetes mellitus, dyslipidemia, hypertension, and pancreatic cancer presented to our outpatient endocrine clinic for evaluation of hyperparathyroidism. Further investigation of his history revealed that he had struggled with several bouts of urinary calculi throughout his life. These episodes required multiple surgical interventions to treat, including left nephrectomy and ureteral stenting. He had been evaluated for high serum calcium in the past and was diagnosed with PHPT by his urologist, who referred him to our clinic. Also complicating his health was a recent diagnosis of prostate cancer for which, he was undergoing radiation treatment. He had a family history of thyroid disorder in his mother but no family history of parathyroid disorders. Physical examination was unremarkable. Laboratory investigations revealed high serum calcium and high serum parathyroid hormone. Parathyroid scintigraphy with 99mTc-sestamibi revealed a subtle persistent focal region of increased uptake in the left axilla with no other areas of increased uptake, raising the suspicion for parathyroid adenoma in the left axilla. As discussed earlier, ectopic parathyroid adenomas result from aberrant migration of parathyroid tissue during embryological development. Additionally, ectopic parathyroid adenomas are a cause of failed surgery for hyperparathyroidism as well as persistent and recurrent hyperparathyroidism [3, 7-9]. Ectopic parathyroid tissue has a prevalence of 2-43% in anatomic series and has been detected in up to 16% of patients undergoing evaluation for hyperparathyroidism [3, 7, 10]. Ectopic parathyroid adenomas arising within the axilla may present with hypercalcemia and associated skeletal and renal involvement [11, 12]. The case that has been reported had a presentation of PHPT with recurrent nephrolithiasis. As mentioned earlier, 45% of ectopic parathyroid lesions are found in the paraesophageal region with 38% arising within the thymus [7, 10]. However, in this case, the patient presented with a possible ectopic parathyroid adenoma localized to the left axilla and no other areas of increased uptake on tc99m-sestamibi parathyroid scintigraphy.

Recurrent Acute Pancreatitis as the First Presentation of Primary Hyperparathyroidism

Recurrent Acute Pancreatitis as the First Presentation of Primary Hyperparathyroidism

PHPT with Pancreatitis: Atypical Presentation of PHPT.

Primary hyperparathyroidism (PHPT) is rarely associated with the occurrence of acute or chronic pancreatitis, requiring complex perioperative management. This study aimed to assess the prevalence and disease characteristics of pancreatitis in PHPT. This study is a clinicopathological analysis of the medical records of patients who were diagnosed with PHPT with pancreatitis between 1989 and 2021 in the Endocrine Surgery department, SGPGI, Lucknow. Out of 548 PHPT cases, 44 (8.03%) were found to be associated with pancreatitis. The mean age was 33.57 years (15-65 years); 5 were ≤20 years, while 26 were ≤30 years of age. There were 27 males and 17 females. Twenty-one cases were of acute (11 acute, nine recurrent acute, one acute on chronic), whereas 23 were of chronic pancreatitis (six chronic calcific pancreatitis). The major clinical presentation of PHPT with pancreatitis was abdominal pain (65.91%). The mean number of attacks per patient in recurrent acute pancreatitis was two. Mean PTH levels were 68.19 pmol/L. The mean tumor size (in the largest dimension) was 2.79 ± 1.4 cm while the mean tumor weight was 4.91 g. Nephrolithiasis was associated with 25 cases. An association with multiple endocrine neoplasia type 1 syndrome was seen in one case. The final histopathological diagnosis was parathyroid carcinoma in two, hyperplasia in three, and parathyroid adenoma in 39 cases. Normocalcemia was seen in 27.2%, hypercalcemic crisis in 15.9%, and 25% of patients required semi-emergency parathyroidectomy. The outcome was favorable in all, as none had any further attacks of pancreatitis. In our study, the prevalence of pancreatitis in PHPT cases was 8.03%. The majority of patients were young. Normocalcemia was seen in 12 patients, so even if calcium levels are normal, PHPT should be suspected in young patients with pancreatitis. Parathyroidectomy resulted in the complete resolution of symptoms of pancreatitis in all 44 patients.

Late-onset hypocalcemia seizure: An interesting presentation of sporadic maternal primary hyperparathyroidism with hypovitaminosis D

Abstract The cardinal manifestation of primary hyperparathyroidism is secondary to hypercalcemia and high bone turnover disease due to sustained elevation of parathyroid hormone. A high index of suspicion for maternal primary hyperparathyroidism should be kept in the setting of neonatal hypocalcemia. Delayed presentation of hypocalcemic seizure secondary to maternal primary hyperparathyroidism is infrequent. Herein, we report a case of a 2-month-old infant who presented with late-onset (&gt;72 h) hypocalcemic seizure, biochemically mimicking pseudohypoparathyroidism, which eventually led to the diagnosis of maternal hyperparathyroidism with vitamin D deficiency.

PRIMARY HYPERPARATHYROIDISM REVEALED BY ACUTE ALITHIASIC PANCREATITIS: A RARE CASE

Acute pancreatitis is a rare presentation of Primary hyperparathyroidism (PHPT), its prevalence during PHPT is about 1%. The physiopathology of the implication of PHPTs hypercalcemia is not completely elucidated. Several hypotheses plead for the direct or indirect role of hypercalcemia via the activation of pancreatic proteases. Acute alithiasic pancreatitis can be a revealing feature of PHPT wich is a curable disease with the mastery of parathyroid surgery and hypercalcemia treatment. Herein, the authors report a rare case of a patient with PHPT revealed by acute alithiasic pancreatitis with a dreaded complication.

Prevalence and associated risk factors of simple renal cysts in primary hyperparathyroidism.

Previous studies have pointed to the role of parathormone (PTH) in the development of simple renal cysts. However, there is insufficient evidence related to simple renal cysts in primary hyperparathyroidism (PHPT). Therefore, this study aimed to evaluate the prevalence and risk factors associated with renal cysts in PHPT patients. In this retrospective study, we included 235 patients treated surgically for PHPT and 147 control subjects. The clinical and physical examination findings, laboratory test results, and neck and urinary system ultrasonography examination findings were evaluated. Postoperative parathyroid adenoma volume and weight were also recorded. Simple renal cysts were found in 53 PHPT patients (22.6%) and in 15 control subjects (10.2%) (p = 0.002). Kidney stones were found in 33 PHPT patients (14%) and in only seven control subjects (4.8%) (p = 0.004). The presence of PHPT and high PTH levels independently supported the development of a simple renal cyst. Advanced age was determined as a risk factor for the development of simple renal cysts, and the presence of kidney stones was an independent predictor of simple renal cysts in PHPT patients. There was no relationship between the presence of simple renal cysts and the volume and weight of parathyroid adenoma. A diagnosis of PHPT, high PTH levels, advanced age, and kidney stones are independent risk factors for simple renal cysts in PHPT patients. These patients should be evaluated regularly for complications of renal cysts and nephrolithiasis.

PRIMARY HYPERPARATHYROIDISM AS A RARE INTRINSIC CAUSE OF CHRONIC URTICARIA AND ANGIOEDEMA

<h3>Introduction</h3> Chronic urticaria and angioedema(CUA) has an incidence of approximately 0.05%,²of which autoimmune conditions contribute about 50% of the cases.³ Immune mediated Primary hyperparathyroidism, has been linked to CUA due to autoantibodies against the calcium-sensing receptors.^3,4 We present a patient with hyperparathyroidism induced CUA, who experienced four episodes of urticaria with angioedema. She was diagnosed with hyperparathyroidism after the second episode. <h3>Case Description</h3> A 70-year-old female with a history of recurrent kidney stones, Hashimoto's thyroiditis, lymphedema, SVT, osteoarthritis, and nonsmoker presented to the Emergency Department(ED) with facial and upper extremities urticaria and erythema associated with tongue swelling, dyspnea and syncope after eating crab. Since that visit, she had 3 additional episodes of urticaria and angioedema without eating crab or suspected allergens. Extensive allergy and immunology workup was negative for common CUA causes. She was prescribed Fexofenadine, Cetirizine and Famotidine daily with Epi-pen as needed. Despite this regimen, she had recurrent CUA. During the second episode, her calcium was elevated in the ED, and follow-up outpatient hyperparathyroidism workup was positive. Parathyroid scan showed an inferior right pole adenoma. She underwent partial parathyroidectomy, then experienced resolution of episodes to date and negative crab food challenge test. <h3>Discussion</h3> Primary hyperparathyroidism very rarely causes CUA through an autoimmune process. Our patient's clinical manifestations, treatment course, and improved outcomes after surgery, suggest hyperparathyroidism induced CUA. Previous reported similar presentations of primary hyperparathyroidism induced CUA resolved after partial parathyroidectomy.³ Our aim is to increase awareness of hyperparathyroidism as a potential cause of CUA and curative treatment with parathyroidectomy.

ODP121 Recurrent atypical parathyroid adenoma: a case report

Abstract Background Atypical parathyroid adenoma is a rare cause of hyperparathyroidism, with an incidence ranging from 0.5 to 4.4%. Its recurrence rate is low, around 3%. We describe a young patient with an atypical parathyroid adenoma that recurred twice at the right neck. Case presentation A 39-year-old woman presented to the ER with low back pain, hyporexia, weight loss, and an oral lesion with purulent discharge. She was diagnosed with nephrolithiasis, severe hypercalcemia (serum calcium concentration: 21,2 mg/dl (Reference range 8,5-10,5) and hyperparathyroidism (serum PTH: 703 pg/mL; reference range 15-45pg/mL). Parathyroid scintigraphy identified an uptake at the site of the lower right parathyroid. A cervical US confirmed the presence of a 3 cm markedly hypoechoic nodule in that location. Fine needle aspiration (FNA) suggested parathyroid adenoma, and PTH washout levels were 1900 pg/ml. Screening for multiple endocrine neoplasia was negative. Biopsy of the mandibular alveolar bone, around the oral lesion, showed giant cell lesion. Lower right parathyroidectomy was performed, and the histopathology showed parathyroid neoplasm with features favoring atypical parathyroid adenoma (lesion measuring 3×2×1.8 cm). Fifteen months after the surgery, the patient presented symptoms of hypercalcemia, with a new elevation of serum PTH levels (738 pg/ml), confirmed with radiotracer uptake at the site of the upper right parathyroid gland. She underwent right parathyroidectomy and hemithyroidectomy. Histopathology revealed an atypical parathyroid adenoma. Immunohistochemistry was positive to galectin 3 in rare cells; Ki 67 positive (2%). After nine months, the patient noticed a subcutaneous nodule in the right anterior cervical region associated with symptomatic hypercalcemia, and a new increase in serum PTH levels (469.5 pg/mL). Parathyroid scintigraphy confirmed radiotracer activity at the nodule described, that was surgically resected. Histopathology was compatible with an atypical parathyroid neoplasm (uncertain malignant potential). The patient remains in regular follow-up, asymptomatic and normocalcemic. Conclusion While 96% of patients with atypical parathyroid adenomas are cured with parathyroidectomy, this case shows a very rare description of two recurrences in a short time of follow-up, with an ectopic location in the last one. It should be noted that atypical paratyroid adenomas are already an uncommon presentation of primary hyperparathyroidism. Genetic investigation is warranted in such cases. Presentation: No date and time listed

PSAT173 Weekly Supplementation with Vitamin D3 reduces 1,25(OH)2 vitamin D3 Measured by Mass Spectrometry, and increases Fibroblast-like growth factor 23 (FGF23) in Individuals with and without Primary Hyperparathyroidism (PHPT)

Abstract Introduction Vitamin D deficiency is a very common condition, which has been associated with more severe presentations of PHPT. It has been suggested that the Vitamin D replacement improves this prognosis, but there are few data regarding its effect on mineral homeostasis. The aim of this research was to evaluate the effects of weekly Vitamin D supplementation on the mineral metabolism in this population.Material and methods: In this prospective case-control trial, we evaluated the effects of 14,000 IU/week of oral Vitamin D3, for 12 weeks, on albumin-adjusted total Calcium (TCa), Parathormone (PTH), 25 hydroxyvitamin D3 (25OHD), intact FGF23 and 1,25dihydroxyvitamin D3 (1,25(OH)2D), in addition to other biochemical markers. Volunteers with active PHPT (n=55) and paired controls (n=64) collected fasting blood samples before and after 12 weeks of the Vitamin D supplementation. 1,25(OH)2D was measured by liquid chromatography and mass spectrometry, and other hormones by commercial immunometric assays. P&amp;lt;0.05 was considered significant. Results At baseline there were no differences between groups regarding 25OHD, FGF23 and clearance of creatinine. As expected, the concentrations of TCa, PTH, 1,25(OH)2D were higher in the PHPT. After supplementation, TCa and PTH did not change despite the significant increase on 25OHD in both groups, more markedly in the controls (from 22.7±6.1 to 31.5±6.8 ng/mL in PHPT; and from 20.4±5.8 to 32.5±6.4 ng/mL in controls). FGF23 levels significantly increased after supplementation in both groups, more markedly in the PHPT. (from 47.9 ± 27.1 to 76.3 ± 33.3 pg/mL in PHPT and from 40.5 ± 13.9 to 59.8±19.8 pg/mL in controls). A significant reduction in the concentrations of 1,25(OH)2D was seen in both groups, (time effect p-value&amp;lt;0.001), with more intensity in the PHPT group (time x group effect p-value=0.015) (from 94.8±34.6 to 68.9±25.3 pg/mL in PHPT and from 68.7±23.5 to 56.4±20.7pg/mL in controls). The delta of 1,25(OH)2D was inversely correlated with the delta of FGF23 in both the PHPT (r=-0.302, p-value=0.028) and control (r=-0.278, p-value=0.027) groups. Conclusion In both patient groups (with and without PHPT), weekly administration of 14,000IU of Vitamin D3 reduced 1,25(OH)2D concentrations measured by mass spectrometry, probably due to the inhibition of 1α-hydroxylase induced by the increase in FGF23. This phenomenon could explain the absence of hypercalcemia after supplementation, even in individuals with active PHPT, appearing to be a protective mechanism against Vitamin D excess. Future studies are needed to see if this increase in FGF23 could explain the paradoxical effects observed with the high bolus doses of vitamin D described by others.*This research received public grants from FAPESP (Fundação de Amparo a Pesquisa do Estado de Sao Paulo), with technical support from Laboratorio Fleury Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.