A Rare Manifestation of Unknown Hyperparathyroidism as a Perforated Peptic Ulcer.

Hypercalcemia is a common electrolyte abnormality with different causes. Hypercalcemia is most often associated with malignancy and primary hyperparathyroidism and malignancy together account for most cases. Primary hyperparathyroidism manifests as hypercalcemia owing to the overproduction of parathyroid hormone. In most cases, primary hyperparathyroidism manifests due to a solitary parathyroid adenoma. Based on calcium levels, hypercalcemia can be classified as mild, moderate, and severe. Hypercalcemia typically presents with non-specific clinical features. Here, we present the case of a 38-year-old male patient who presented to the emergency department (ED) with acute abdominal pain and a tender abdomen with absent bowel sounds. He had chest radiography and blood tests initially. Chest radiography showed left-sided pneumoperitoneum, and the patient was suspected to have a perforated peptic ulcer due to hypercalcemia secondary to a parathyroid adenoma during the second wave of the coronavirus disease 2019 (COVID-19) pandemic. The findings were confirmed by a computerized tomography scan of the abdomen, and the patient was treated with intravenous fluids for hypercalcemia and was managed conservatively for a sealed perforated peptic ulcer following discussion in the multi-disciplinary team meeting (MDT). The COVID-19 pandemic led to a long waiting list and delays in the timely management of patients requiring elective surgical intervention, such as parathyroidectomy. The patient made a complete recovery and had parathyroidectomy of the inferior right lobe two months later.

Primary hyperparathyroidism in young patients is associated with metabolic disorders: a prospective comparative study.

BACKGROUND: Components of metabolic syndrome can be observed in patients with primary hyperparathyroidism (PHPT). The link between these disorders remains unclear due to the lack of relevant experimental models and the heterogeneity of examined groups. The effect of surgery on metabolic abnormalities is also controversial. We conducted a comprehensive assessment of metabolic parameters in young patients with PHPT. METHODS: One-center prospective comparative study was carried out. The participants underwent a complex biochemical and hormonal examination, a hyperinsulinemic euglycemic and hyperglycemic clamps, a bioelectrical impedance analysis of the body composition before and 13 months after parathyroidectomy compared to sex-, age- and body mass index matched healthy volunteers. RESULTS: 45.8% of patients (n = 24) had excessive visceral fat. Insulin resistance was detected in 54.2% of cases. PHPT patients had higher serum triglycerides, lower M-value and higher C-peptide and insulin levels in both phases of insulin secretion compared to the control group (p < 0.05 for all parameters). There were tendencies to decreased fasting glucose (p = 0.031), uric acid (p = 0.044) and insulin levels of the second secretion phase (p = 0.039) after surgery, but no statistically significant changes of lipid profile and M-value as well as body composition were revealed. We obtained negative correlations between percent body fat and osteocalcin and magnesium levels in patients before surgery. CONCLUSION: PHPT is associated with insulin resistance that is the main risk factor of serious metabolic disorders. Surgery may potentially improve carbohydrate and purine metabolism.

Familial hypocalciuric hypercalcaemia type 1 caused by a novel heterozygous missense variant in the CaSR gene, p(His41Arg): two case reports.

BACKGROUND: Familial hypocalciuric hypercalcaemia (FHH) is a rare, inherited disorder of extracellular calcium sensing. It is clinically characterised by mild to moderate parathyroid hormone dependent hypercalcaemia, an autosomal dominant pattern of inheritance, and a normal to reduced urinary calcium excretion in spite of high serum calcium. CASE PRESENTATION: We report two cases of FHH in a family caused by a novel pathogenic missense variant in the CaSR gene, p. His41Arg. Case 1, describes a 17 year old female with no significant past medical history, admitted with acute appendicitis requiring laparoscopic appendectomy and reporting a six month history of polydipsia. Routine investigations were significant for hypercalcaemia, corrected calcium 3.19 mmol/L (2.21-2.52mmol/L), elevated parathyroid hormone of 84pg/ml (15-65pg/ml) and a low 24-hour urine calcium of 0.75mmol/24 (2.50-7.50mmol/24). She was initially managed with intravenous fluids and Zolendronic acid with temporary normalisation of calcium though ultimately required commencement of Cinacalcet 30 mg daily for persistent symptomatic hypercalcaemia. Genetic analysis was subsequently positive for the above variant. Case 2, a 50-year-old female, was referred to the endocrine outpatient clinic for the management of type 2 diabetes and reported a longstanding history of asymptomatic hypercalcaemia which had not been investigated previously. Investigation revealed hypercalcaemia; corrected calcium of 2.6 mmol/L (reference range: 2.21-2.52 mmol/L); PTH of 53.7ng/L (reference range: 15-65 ng/L) and an elevated 24-hour urine calcium of 10 mmol/24 (2.50-7.50 mmol/24hr) with positive genetic analysis and is managed conservatively. Despite sharing this novel mutation, these cases have different phenotypes and their natural history is yet to be determined. Two further relatives are currently undergoing investigation for hypercalcaemia and the family have been referred for genetic counselling. CONCLUSION: Accurate diagnosis of FHH and differentiation from classic primary hyperparathyroidism can be challenging, however it is essential to avoid unnecessary investigations and parathyroid surgery. Genetic analysis may be helpful in establishing a diagnosis of FHH in light of the biochemical heterogeneity in this population and overlap with other causes of hypercalcaemia.

Multiple Brown Tumors Secondary to Parathyroid Carcinoma: A Challenging Diagnosis.

Parathyroid carcinoma is an extremely rare endocrine neoplasm that accounts for less than 1% of the cases of primary hyperparathyroidism (PHPT). Continuous exposure to high levels of parathyroid hormone (PTH) induces an increase in bone remodeling and patients may present with osteitis fibrosa cystica, which is characterized by subperiosteal resorption of the phalanges, diffuse osteopenia, salt and pepper appearance of the skull, bone cysts, and brown tumors. Brown tumors occur in less than 5% of all patients with any form of hyperparathyroidism. Due to similar clinical, radiographic, and histological appearance, differential diagnosis of brown tumors includes primary and secondary bone tumors. We report a case of a 67-year-old female diagnosed with multiple osteolytic lesions initially thought to be bone metastasis of thyroid carcinoma. Further work-up led to the diagnosis of brown tumors due to parathyroid carcinoma. We want to emphasize the inclusion of osteitis fibrosa cystic in the differential diagnosis of osteolytic lesions and the need to perform serum calcium and PTH measurements when investigating these lesions.

Stress and Adherence During the COVID-19 Pandemic in an Inner-City Population of Dialysis Patients: Relationship to Age

It has been reported that older pts adapt better to dialysis than younger pts. We investigated in response by age to various stressors encountered during the COVID-19 pandemic in a population of inner-city dialysis patients. A survey was conducted in a random sample of 32 dialysis patients. Patients were asked about their fluid intake, general attitudes towards medical recommendations, and changes in their wellbeing due to COVID19. The PSS (Perceived Stress Scale) and KAS-R (Kim Alliance Scale Revised) were also used. Mean age was 56.8 ± 18.2 years. 15 pts (46.9%) were <60 yrs (younger) and 17 (53.1%) were ≥60 yrs (older). Mean dialysis time was 88.0 ± 104.0 months. There were 20 (62.5%) male, 29 (90.6%) identified as black, 18 (56%) had a high school diploma or less, and 14 (44%) completed some college or more. 7% (1) of older and 46% (6) of younger pts reported “some of the time” or “never” rather than "most of the time" when asked how often they followed the fluid restriction recommendations (p=0.034). 29% (4) of younger pts reported fluid restrictions were difficult to follow, vs. none of the older pts (p=0.037). 33% (5) of younger pts reported “poor” or “average” when asked about wellbeing prior to the pandemic and 100% (15) of older patients reported “good” (p=0.05). When asked to rate their stress level over the last year, 64% (9) of younger pts reported being somewhat or very stressed and 79% (11) of older pts reported not at all or a little stressed (p=0.015). 29% (4) of younger pts stated they sometimes work well with their provider and 100% (15) of older patients stated always (p=0.026). There were no statistically significant differences between the groups for sex, race, or education. In our population during the pandemic: 1. Younger pts were less adherent to fluid restriction and found them more difficult to follow. 2. Older pts were more likely to report feeling good prior to the pandemic and were less stressed following it. 3. Older pts were more likely to report a good relationship with their provider. 4. Younger pts may need more support through the pandemic as they appear to be coping less well, feel less connected, and are less able to follow important dietary restrictions. (Table Presented) This case highlights the uncommon sequelae of untreated primary hyperparathyroidism which is rare since the introduction of automated chemistry analyzers [2]. Pancreatitis is reported in < 3% of patients with hyperparathyroidism and is seldom seen in current practice. Nephrocalcinosis and pancreatitis are rare complications of untreated hyperparathyroidism and could have been averted with the definitive treatment of parathyroidectomy.

AS SEEN ON TV: A CAUTIONARY TALE OF VITAMIN D SUPPLEMENTATION FOR COVID-19 PREVENTION

CASE: An 80-year-old woman with untreated osteoporosis and suspected primary hyperparathyroidism presents to establish care. Review of systems and physical examination are normal. She has mild hypercalcemia (11.2), and normal albumin and phosphorous. Parathyroid hormone (PTH) is elevated (71). Bone density testing demonstrates osteoporosis at the hip and spine (Tscore -2.9 and -3.0). She reports self-medicating with 12,000 IU of vitamin D daily to prevent COVID-19 infection, which she learned about from a popular news source;she is unvaccinated for COVID-19. Her vitamin D 25-OH level is 172 (normal 30-100). The patient was instructed to stop vitamin D supplementation. Additional work up for hyperparathyroidism was initiated, including 24-hour urine collection for calcium, and she was referred for a parathyroidectomy. IMPACT/DISCUSSION: Adequate vitamin D supplementation has been postulated to reduce the risk and severity of the COVID-19 infection through its immunomodulatory effects that augment the immune cell response, decrease inflammation, and prevent RAAS system dysregulation, which is associated with more severe coronavirus infection. However, trials and metaanalyses have yielded inconclusive data, with most reporting no associations between adequate or high-dose vitamin D supplementation and COVID-19 morbidity and mortality. Nonetheless, popular news sources and social media have called for high-dose vitamin D supplementation, which can result in hypervitaminosis D through patient self-medication. Both hypervitaminosis D and primary hyperparathyroidism present with signs and symptoms of hypercalcemia, including nephrolithiasis, osteoporosis, bone pain, weakness, and neuropsychiatric changes. Hypervitaminosis D is caused by ingestion of too much exogenous vitamin D (normally more than 10,000 IU/day), dysregulation of the vitamin D pathway, or overproduction of vitamin D. Primary hyperparathyroidism is caused by parathyroid adenomas, hyperplasia, and carcinomas. Distinguishing between the two conditions involves a thorough history and physical, laboratory measurements, and occasionally imaging. Hypervitaminosis D patients have suppressed PTH levels, serum 25(OH)D > 150ng/mL, and hyperphosphatemia while primary hyperparathyroidism patients have normal/elevated PTH levels, low/normal 25(OH)D levels, and hypophosphatemia. Primary hyperparathyroidism is the most common cause of hypercalcemia, but this case highlights the importance of screening for and identifying other etiologies of hypercalcemia. This patient's vitamin D toxicity can be treated by stopping vitamin D supplementation. Her primary hyperparathyroidism meets criteria for a parathyroidectomy due to the presence of osteoporosis. CONCLUSION: 1. High dose vitamin D supplementation is ineffective as prophylaxis against the COVID-19 infection. 2. Hypercalcemia secondary to vitamin D toxicity is distinguished from primary hyperparathyroidism by PTH, 25(OH)D, and phosphorus levels.

COVID-19 pneumonia detected by parathyroid scintigraphy.

We report a case of incidental diagnosis of COVID-19 pneumonia by parathyroid scintigraphy. A 53-year-old woman who had severe fatigue, and mild dyspnea underwent parathyroid scintigraphy due to increased serum parathyroid hormone (PTH) and serum calcium levels. Parathyroid scan was negative for abnormal parathyroid tissue. Although the patient had three negative results of COVID-19 PCR tests, significant 99m Tchexakis-2-methoxyisobutylisonitrile ([99mTc]MIBI) uptake is noticed in both lungs that was suspicious for Covid-19 pneumonia. The patient underwent CT scan of the chest for further evaluation. Diffuse groundglass opacities were identified in both lungs which were interpreted as typical feature for COVID-19 pneumonia.

A sustainable outpatient transformation project for metabolic stone assessment: A specialist stone nurse led non-face-to-face (F2F) clinic

Introduction: Recent NHSEI policy and the COVID-19 pandemic are increasing the proportions of consultations occurring non-face-to-face (F2F). Here we describe a nurse-led non-F2F clinic for the metabolic assessment of kidney stone patients. Method: A metabolic assessment may be indicated in patients forming urate stones, CaPO4 stones, or recurrent stones or with clinical features suggesting a metabolic cause. In otherwise uncomplicated clinical scenarios, these patients are reviewed in a non-F2F clinic run by an endo-urological specialist nurse. A stone history is taken by telephone. Blood tests are arranged in primary care. A collapsible 24-hour urine collection container is posted to the patient and returned via the primary care sample collection service. The cases are reviewed at the Metabolic Stone MDT by the nurse, nephrologist and urologist. Results: A total of 145 patients were eligible with six DNAs, leaving 139 patients reviewed through the non-F2F clinic between March 2020 and June 2021. Demographics were 81 males: 58 females, age range 17-83. About 126 of 139 (91%) patients completed the tests, which is a significantly higher rate than completion rates typically reported. Stone analysis was also available in 97 patients (28 CaOx;54 CaPO4;15 urate). Around 102 patients (81%) were discharged with dietary advice, while 24 patients (19%) were referred for consultant review. Two patients had primary hyperparathyroidism. Nineteen patients had hypercalciuria, all requiring consultant review. Conclusion: Nurse-led non-F2F review streamlines the metabolic assessment of stone-formers, reducing the need for hospital attendances and reducing consultant workload.

A single-centre retrospective analysis of cinacalcet therapy in primary hyperparathyroidism.

Primary hyperparathyroidism (pHPT) is a common endocrine disorder that can be cured by parathyroidectomy; patients unsuitable for surgery can be treated with cinacalcet. Availability of surgery may be reduced during COVID-19, and cinacalcet can be used as bridging therapy. In this single-centre retrospective analysis, we investigated the utility and safety of cinacalcet in patients with pHPT receiving cinacalcet between March 2019 and July 2020, including pre-parathyroidectomy bridging. We reviewed and summarised the published literature. Cinacalcet dosages were adjusted by endocrinologists to achieve target calcium < 2.70 mmol/L. Eighty-six patients were identified, with the most achieving target calcium (79.1%) with a mean dose of 39.4 mg/day (±17.1 mg/day) for a median duration of 35 weeks (1-178 weeks). Calcium was normalised in a median time of 5 weeks. The majority of patients commenced cinacalcet of 30 mg/day (78 patients) with the remainder at 60 mg/day (8 patients). Forty-seven patients commencing lower dose cinacalcet (30 mg/day) achieved target calcium without requiring 60 mg/day. Baseline PTH was significantly higher in patients requiring higher doses of cinacalcet. 18.6% of patients reported adverse reactions and 4.7% discontinued cinacalcet. Patients treated with cinacalcet pre-parathyroidectomy required a higher dose and fewer achieved target calcium compared to medical treatment with cinacalcet alone. Post-operative calcium was similar to patients who were not given pre-parathyroidectomy cinacalcet. In summary, cinacalcet at an initial dose of 30 mg/day is safe and useful for achieving target calcium in patients with symptomatic or severe hypercalcaemia in pHPT, including those treated for pre-parathyroidectomy. We propose a PTH threshold of >30 pmol/L to initiate at a higher dose of 60 mg/day.

Management of Primary Hyperparathyroidism With Severe Hypercalcemia During the COVID-19 Pandemic.

PURPOSE: In patients with primary hyperparathyroidism (PHPT) and severe hypercalcemia, parathyroidectomy remains the only curative therapy. During the coronavirus disease 2019 (COVID-19) pandemic, when many hospital visits are suspended and surgeries cannot be performed, the management of these patients represents a challenging clinical situation. This article presents a literature review and discussion of the pharmacologic management of PHPT and severe hypercalcemia, which can be used as a temporary measure during the COVID-19 pandemic until parathyroidectomy can be performed safely. METHODS: This narrative review was conducted by searching literature on the PubMed, Medline, and Google Scholar databases using the terms primary hyperparathyroidism, hypercalcemia, cinacalcet, bisphosphonates, denosumab, vitamin D, raloxifene, hormone replacement therapy, coronavirus, and COVID-19. FINDINGS: Appropriate monitoring and remote medical follow-up of these patients are essential until the resolution of the pandemic. Cinacalcet is the drug of choice for controlling hypercalcemia, whereas bisphosphonate or denosumab is the drug for improving bone mineral density. Combined therapy with cinacalcet and bisphosphonates or cinacalcet and denosumab should be considered when the effects on serum calcium and bone mineral density are simultaneously desired. IMPLICATIONS: Medical management of PHPT and severe hypercalcemia presents a reasonable alternative for parathyroid surgery during the COVID-19 outbreak and should be instituted until the pandemic ends and surgery can be performed safely.