Debulking surgery after muscular paraffin oil injections: Effects on calcium homeostasis and patient satisfaction.

OBJECTIVE: Cosmetic paraffin oil injections can lead to granuloma formation causing hypercalcemia and kidney failure. This study explores whether debulking surgery is an effective treatment for improving calcium homeostasis, inflammation and clinical symptoms. MATERIALS AND METHODS: In a retrospective study, we reviewed 33 patients undergoing debulking surgery. Changes in calcium, inflammatory markers, and renal function from baseline up to twelve months post-surgery were assessed. Patients were interviewed post-surgery. RESULTS: The patients were 34.6 years (SD 6.9) and had 1,104 grams (SD 591) of granuloma tissue removed following injection of 1,329 mL (SD 803) paraffin oil 7.9 years (SD 3.2) earlier. Seventeen patients had hypercalcemia and experienced a significant decline in ionized calcium from 1.48 mmol/L (SD 0.16) at baseline to 1.33 mmol/L (SD 0.03) at twelve months (p<0.002), although only four men (23.5%) became normocalcemic. Serum ferritin was reduced by 50% after twelve months (p=0.048). Sixteen patients were normocalcemic and had no change in calcium homeostasis but experienced a 20% drop in serum ferritin levels (p=0.025) after surgery. Fifteen patients completed all their planned surgeries within the study period and experienced a decline in serum ionized calcium (p=0.031), ferritin (p=0.011), and interleukin 2-receptor (p=0.037). A patient satisfaction survey showed that 55% of patients reported post-operative satisfaction scores of 10/10, and 59% of the patients reported reduced pain. CONCLUSION: Surgery improved calcium homeostasis in a fraction of patients, reduced inflammation and subjective symptoms such as pain and mental well-being in a patient group left with few treatment options except high-dose prednisolone.

[Familial Hypocalciuric Hypercalcemia Type 1 Likely Secondary to a New Inactivating Mutation of CASR].

Familial Hypocalciuria Hypercalcemia (FHH) is an inherited disease with autosomal dominant transmission characterized by the presence of usually mild-to-moderate hypercalcemia, hypophosphatemia, hypocalciuria, and normal or moderately increased PTH values. Generally, FFH is asymptomatic although symptoms related to elevated plasma calcium values such as asthenia, intense thirst, polyuria, polydipsia or confusional state may occur. Three types of FHH, which differ in the genetic alterations underlying the condition, are described. The majority of FHH cases are classified as type 1 (about 65 percent of cases), due to mutation in the gene for the calcium-sensitive receptor CASR, expressed on chromosome (Chr) 3q13.3-21, which encodes for a calcium-sensitive receptor G-protein-coupled protein of the plasma membrane. FHH types 2 and 3 are due to GNA11 and AP2S1 mutations, respectively, and other genes involved in the pathogenesis of the disease have likely yet to be identified. Rarely, familial hypocalciuric hypercalcemia may not recognize a genetic cause but be caused by autoantibodies directed against CASR. The frequency of the disease is not known and is estimated, probably by default, because of paucisymptomatic presentation of the disease, to be around 1:80000 cases. Recognition of FHH is especially important for differential diagnosis with primary hyperparathyroidism, which has a much higher incidence, about 1:1000 cases. This allows for the identification of patients at risk for chondrocalcinosis and/or pancreatitis. Clinical suspicion must be raised in cases of hypercalcaemia associated with hypocalciuria, and genetic analysis is fundamental in the differential diagnosis toward forms of primary hyperparathyroidism that might result in unnecessary surgical interventions. We describe a clinical case in which a novel inactivating mutation of CASR leading to FHH type 1 was found.

Diagnosing Primary Hyperparathyroidism in Pregnancy: A Case of Altered Parathyroid Hormone Degradation in Pregnancy.

Diagnosing primary hyperparathyroidism in pregnancy is difficult due to pregnancy-related changes in parathyroid hormone (PTH); calcium; 1,25 vitamin D; and renal calcium excretion. Parathyroid hormone-related peptide (PTHrP) produced by the placenta adds additional complexity. Our case is the first to demonstrate an increased rate of PTH degradation within a pregnant individual who returned unexpectedly low PTH levels. We describe a 27-year-old female patient who presented at 25 weeks gestation with pancreatitis and hypercalcemia. Primary hyperparathyroidism was suspected but variable PTH results led to uncertainty and an assay error was considered. PTH samples were collected in both serum-separating tubes (SST) and EDTA tubes and compared to controls (5 nonpregnant and 5 pregnant individuals). Samples were retested every 2 hours for a period of 10 hours. A rapid decline in the measured PTH was noted in the index case, an observation which differed from controls. We postulated that internal and/or external factors influenced the PTH measurement obtained from our patient. From our observations, rapid PTH degradation in pregnancy, and individual variation in PTH stability and laboratory processes, can influence PTH results and impact on interpreting hypercalcemia in pregnancy.

Outcome of Primary Hyperparathyroidism: Retrospective Tertiary Center Experience From Basrah, Iraq.

BACKGROUND: Primary hyperparathyroidism is regarded as a common endocrine disorder that is biochemically identified and could be symptomatic or asymptomatic. A detailed history and a thorough evaluation with regular follow-ups are required until a definite diagnosis is made. The study aims to evaluate the characteristics of patients and the performance of a tertiary endocrine center in managing the disease in Basrah, Iraq. MATERIAL AND METHODS: A retrospective study was conducted at the Faiha Specialized Diabetes, Endocrine, and Metabolism Center in Basrah, southern Iraq, on 106 patients diagnosed with primary hyperparathyroidism between 2012 and 2023. The patients' general characteristics were assessed, and those who underwent parathyroidectomy were evaluated post-surgery, and the cure rate was determined. RESULTS: The mean age of presentation was 47.5 ± 14.6 years, with a median of 50 years. The highest occurrence is in the sixth decade. Females comprised 79 (75%) of the patients, and the female-to-male ratio was 3:1. Symptomatic patients were 84 (90%), 30 (70%) of the patients had nephrolithiasis, and 52 (68%) had osteoporosis. The cure rate was 15 (83%). CONCLUSION: In our single-center study, the frequency of primary hyperparathyroidism has increased with time. The disease's highest occurrence was seen in the sixth decade. Females were substantially higher than males. Most patients were symptomatic. The cure rate was 83%.

Ectopic Production of Parathyroid Hormone and Production of Parathyroid Hormone-related Protein in Dedifferentiated Endometrial Carcinoma Induced Severe Hypercalcemia.

Hypercalcemia is a significant complication in cancer patients, primarily caused by parathyroid hormone-related peptide (PTHrP) and, rarely, by parathyroid hormone (PTH) production from tumors. We report a case of severe hypercalcemia in a woman with uterine cancer who exhibited elevated PTH and PTHrP levels. Surgical intervention revealed dedifferentiated endometrial carcinoma. Postoperatively, PTH and PTHrP levels normalized but subsequently increased due to metastases. A molecular analysis confirmed the expression of the PTH gene and protein within the tumor, indicating ectopic PTH production. In diagnosing and treating cancers, it is necessary to consider not only PTHrP production but also ectopic PTH production.

[Analysis of the prognostic factors in primary plasma cell leukemia in the era of novel agents].

Objective: To explore the prognostic factors of primary plasma cell leukemia (pPCL) in the era of novel agents. Methods: The clinical data of 66 patients with pPCL treated at the Department of Haematology, Beijing Chao-Yang Hospital, Capital Medical University from 2011 to 2022 were retrospectively collected to analyze their prognostic factors. Results: Among the 66 patients with pPCL, the median age was 59 (range: 29-79) years. The median overall survival (OS) duration was 19.0 (95% CI 10.4-27.6) months, and the median progression-free survival (PFS) duration was 11.0 (95% CI 6.5-15.6) months. The median OS and PFS were significantly longer in patients with the best post-treatment response of very good partial remission (VGPR) or better than in patients with a response of partial remission (PR) or worse (median OS: 33.0 months vs 6.0 months, P<0.001; median PFS: 16.0 months vs 3.0 months, P<0.001). OS was significantly longer in patients who underwent autologous hematopoietic stem cell transplantation than in those who did not undergo transplantation (49.0 months vs 6.0 months, P=0.002), and there was a trend toward a longer PFS in patients who underwent transplantation than in those who did not undergo transplantation (19.0 months vs 8.0 months, P=0.299). The median OS and PFS were significantly longer in patients who received maintenance therapy than in those who did not receive maintenance therapy (median OS: 56.0 months vs 4.0 months, P<0.001; median PFS: 20.0 months vs 2.0 months, P<0.001). Multivariate analysis showed that hypercalcemia was an independent risk factor (HR=3.204, 95% CI 1.068-9.610, P=0.038) for patients with pPCL, while receiving maintenance therapy (HR=0.075, 95% CI 0.022-0.253, P<0.001) and post-treatment response of VGPR or better (HR=0.175, 95% CI 0.048-0.638, P=0.008) were independent protective factors for patients with pPCL. Conclusions: In the era of novel agents, hypercalcemia, receiving maintenance therapy, and post-treatment response of VGPR or better are independent prognostic factors for pPCL.

Novel Mutation in the Calcium-Sensing Receptor Gene Associated With Familial Hypocalciuric Hypercalcemia.

We present a case of a 75-year-old woman with persistent hypercalcemia (serum calcium 10.7 mg/dL, ionized calcium 1.37 mmol/L), elevated parathyroid hormone levels (86.6 pg/mL), and significantly low 24-hour urinary calcium excretion (<11 mg/24 hours). Genetic testing identified a novel heterozygous variant in the calcium-sensing receptor (CaSR) gene, c.3166G>C (p. Val1056Leu). The patient's biochemical profile and the identification of the CaSR variant support the diagnosis of familial hypocalciuric hypercalcemia (FHH). The novel c.3166G>C (p.Val1056Leu) variant has not been previously reported in FHH or other CaSR-associated conditions. Its presence in this patient suggests a potential role in the clinical manifestation of FHH. However, it is currently classified as a variant of undetermined significance (VUD) in the ClinVar database, necessitating further research on the clinical relevance of this variant in FHH. This case highlights the significance of genetic testing in diagnosing FHH and the potential clinical impact of discovering novel CaSR gene mutations. Further research on the genetics associated with FHH is necessary to better understand the condition, detect it early, and manage it effectively, thereby improving patient care and outcomes.

A retrospective case series on bisphosphonate related osteonecrosis of the jaw in 20 cats.

Introduction: This retrospective study highlights the salient aspects of a series of feline patients affected with bisphosphonate related osteonecrosis of the jaw. Though more commonly published in human literature, this presentation is rare in cats. The authors hope that this study will assist in making this a more globally known entity with subsequent improved prognosis. Methods: Data was retrospectively obtained from the medical records between 2015 and 2021 of 20 cats with Medication Related Osteonecrosis of the Jaw. Data included patient information, clinical history, presenting complaint, systemic diseases, details referable to hypercalcemia and treatment thereof, bisphosphonate specifics (dose and duration), clinical presentation of the lesion, diagnostic testing including radiographic and histopathologic descriptions, treatment, and outcome. Results: Pertinent results include that all 20 cats who developed Medication Related Osteonecrosis of the Jaw had been treated for idiopathic hypercalcemia with the bisphosphonate medication alendronate. Eighty-five percent of the cases had prior dental extractions at the site of MRONJ lesion. Ninety-five percent of the affected cats required a surgical procedure to control the disease. Thirty-five percent of cases required at least one revision surgery after the initial procedure was performed. Diagnosis of MRONJ was made by a correlation of diagnostic findings and patient history. No single diagnostic, or combination was pathognomonic for lesion diagnosis. As well, there were no statistically significant associations between patient variables assessed and the overall patient outcome. Discussion: The case series reveals that cats with feline idiopathic hypercalcemia treated with alendronate may be at a risk for development of MRONJ, a serious oral condition with significant morbidity. Prior dental extraction sites in patients concurrently treated with bisphosphonate medications were often associated with MRONJ lesions. Therefore, any needed dental surgery should be performed prior to the use of bisphosphonates where possible. The authors have also included a relevant comparative literature review.

An Infrequent Histopathological Diagnosis in a Prevalent Systemic Disease: A Case of Renal Sarcoidosis.

Sarcoidosis is a multisystemic granulomatous disease that results from an aberrant immunological response to certain antigens. Although pulmonary involvement predominates, renal involvement may also occur. We present the case of a 59-year-old woman with a recent diagnosis of hepatic sarcoidosis and a history of non-compliance with treatment who was admitted to the hospital for study of acute kidney injury. Renal dysfunction was assumed to be a result of acute interstitial nephritis, as revealed on renal biopsy, and alterations in glomerular hemodynamics due to hypercalcemia. Renal function recovered and serum calcium levels normalized with the introduction of corticosteroid therapy.

Electrolyte and Acid-Base Abnormalities After Kidney Transplantation.

Kidney transplantation is the optimal therapeutic approach for individuals with end-stage kidney disease. The Scientific Registry of Transplant Recipients has reported a continuous rise in the total number of kidney transplants performed in the United States, with 25,500 new kidney recipients in 2022 alone. Despite an improved glomerular filtration rate, the post-transplant period introduces a unique set of electrolyte abnormalities that differ from those encountered in chronic kidney disease. A variety of factors contribute to the high prevalence of hypomagnesemia, hyperkalemia, metabolic acidosis, hypercalcemia, and hypophosphatemia seen after kidney transplantation. These include the degree of allograft function, immunosuppressive medications and their diverse mechanisms of action, and metabolic changes after transplant. This article aims to provide a comprehensive review of the key aspects surrounding the most commonly encountered electrolyte and acid-base abnormalities in the post-transplant setting.

[Primary hyperparathyroidism: From diagnosis to treatment]. / L'hyperparathyroïdie primaire : du diagnostic à la prise en charge thérapeutique.

Primary hyperparathyroidism (PHPT) is the leading cause of hypercalcemia. It is secondary to hypersecretion of parathyroid hormone (PTH) by the parathyroid glands. Today, PHTP is asymptomatic in 80-90% of cases. Its repercussions are mainly renal (nephrolithiasis, nephrocalcinosis, decline in renal function) and skeletal (osteoporosis, fractures), and should be systematically investigated. Diagnosis is only biological, and in its classic form relies on the association of hypercalcemia, inappropriate PTH (normal or elevated) and hypercalciuria. Diagnosis of normocalcemic forms, where only PTH is elevated, requires elimination of secondary hyperparathyroidism and confirmation of elevated PTH on two consecutive samples, over a 3 to 6 months period. Imaging evaluation, which combines neck ultrasound with scintigraphy or 18F-choline PET/CT, is of interest only if surgery is indicated. Surgical management of the hyperfunctioning parathyroid gland(s) is the only curative treatment for HPTP. Medical management concerns patients for whom surgery is not indicated, who present a surgical contraindication or who refuse surgery. The diagnosis of HPTP warrants contact with an endocrinologist to ensure its management.

Calcium chloride poisoning: an autopsy case report and histologic features.

Calcium chloride, which is commonly used in de-icing agents and desiccants, is known for the potential harm it can cause through skin contact, oral ingestion, or intravenous administration. The current paper reports on an autopsy case of a 68-year-old woman who died after ingesting an unknown substance. Histopathological examination revealed coagulative necrosis in the esophagus and ionized blood calcium levels that were significantly higher than normal. Calcium chloride was detected in the contents of the water bottle collected at the scene. These results are expected to make a significant contribution to the limited literature on fatal outcomes from calcium chloride ingestion, while emphasizing the need for heightened awareness and education about the risks of commonly available chemicals.

Parathyroid Hormone-Related Protein-Producing Adenocarcinoma Suspicious of Lung Cancer: A Case Report.

Introduction: Lung adenocarcinoma with parathyroid hormone (PTH)-related hypercalcemia is uncommon. Case Presentation: A 69-year-old man was admitted to our hospital due to anorexia and fatigue. Serum calcium (15.0 mg/dL) and carcinoembryonic antigen (361.7 ng/mL) were extremely high, and PTH-related protein (PTH-rP) also elevated (16.7 pmol/L). Systemic computed tomography revealed multiple enlarged lymph nodes and disseminated peritoneal nodules, with irregularly shaped nodules in the upper lobe in the left lung. Ultrasound-guided biopsy from the axillary lymph node revealed adenocarcinoma. Immunohistological staining showed the tumor cells to be positive for cytokeratin 7 and PTH-rP and negative for cytokeratin 20 and thyroid transcription factor-1. Although the primary origin remains undetermined despite detailed examinations, possible primary tumor was considered to be lung adenocarcinoma in the present case. The serum calcium level was reduced by denosumab, but the patient died 20 days after admission. Conclusion: The present case demonstrated the importance of considering oncological emergency, such as hypercalcemia and/or PTH-rP-producing hypercalcemia, in patients with adenocarcinoma.

Survival Outcomes in Malignancy-related Hypercalcemia: A Tertiary Care Single-center Experience.

Introduction: Malignancy-related hypercalcemia is commonly observed in patients with advanced stages of cancer. It is intricately linked with an unfavorable prognosis among oncology patients. This study aimed to evaluate survival outcomes among individuals diagnosed with hypercalcemia associated with malignancy. Materials and Methods: This retrospective analysis of 173 cancer patients with hypercalcemia who sought treatment at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan, between July 2019 and June 2020. This cohort of patients underwent a longitudinal follow-up for 2.5 years. To assess survival outcomes, the Kaplan-Meier tool was used to construct survival curves and estimate the survival probability over time. The significance of potential survival factors was evaluated using the log-rank test. Results: All patients exhibited elevated levels of calcium. At admission, the cohort demonstrated varying degrees of hypercalcemia severity attributable to malignancy: Mild hypercalcemia was observed in approximately 61.3% of patients, moderate hypercalcemia in 23.7%, and severe hypercalcemia in 15% of cases. Among the total sample, most patients were female (54.9%), with a median age of 54. The primary tumor site most frequently observed was in cases of breast cancer (35.3%), wherein the prevalent histological subtype was lobular/ductal invasive carcinoma (34.1%). Most of the patients (93.6%) had an Eastern Cooperative Oncology Group (ECOG) performance status (ECOG) >1. In addition, the median overall survival for patients diagnosed with hypercalcemia was 51 days. Notably, there was a significant association between survival factors, including the primary site of malignancy (P = 0.001), bone metastasis (P = 0.04), severity and symptoms of hypercalcemia (P = 0.001), altered mental state (P = 0.001), albumin levels (P = 0.001), and ECOG (P = 0.001). Conclusion: Malignancy-related hypercalcemia in patients with cancer is a significant predictor of an unfavorable prognosis. The aforementioned survival factors may have the potential to influence patient survival outcomes. Further studies on larger cohorts are warranted.

Concomitant manifestations of systemic lupus erythematosus flare-up and nodal marginal zone B-cell lymphoma in a 41-year-old male patient: A challenging case report.

Key Clinical Message: Malignancy may be a possible cause of systemic lupus erythematosus (SLE) flare-ups, and it is necessary to consider it in the context of treatment resistance. In this case, we present a challenging instance of concomitant nodal marginal zone B-cell lymphoma (NMZL) and SLE flare-up in a 41-year-old male patient. Abstract: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can cause various symptoms and affect multiple organs in the body. It is also associated with the development of malignancies, especially lymphomas. This case report discusses a patient who experienced a flare-up of SLE along with hypercalcemia, which led to the diagnosis of nodal marginal zone B-cell lymphoma (NMZL). This is the first case of its kind to be reported. A 41-year-old man with a 10-year history of SLE and antiphospholipid syndrome (APS) was referred to our center due to several symptoms, including fatigue, oral lesions, dyspnea, bilateral wrist pain and inflammation, mild pericardial effusion, organ enlargement, pancytopenia, high erythrocyte sedimentation (ESR) level, high anti-double stranded DNA (anti-dsDNA) level, low complement level, resistant hypercalcemia, and high brain natriuretic peptide (pro-BNP) level. After further testing, it was discovered that the patient had NMZL, which was the ultimate diagnosis. He underwent six cycles of the R-CHOP chemotherapy regimen, and his clinical and laboratory conditions improved during follow-ups. The initial case of SLE flare-up, with concomitant NMZL is being reported as the final diagnosis. In simpler terms, it is possible for lymphoma to manifest as a potential cause of SLE flare-ups, and clinicians should be mindful that they need to consider malignant conditions when faced with treatment resistance.

The Use of Steroids to Treat Hypercalcemia Due to Granulomatous Disease From Disseminated Coccidioidomycosis.

The clinical course and treatment of hypercalcemia from a granulomatous disease in the setting of an infectious etiology, namely disseminated coccidioidomycosis, remains incompletely understood. The mechanism and treatment of hypercalcemia have been documented in most granulomatous disorders, with sarcoidosis being the most well-understood so far. We discuss a case of a patient with a recent diagnosis of disseminated coccidioidomycosis who presented with hypercalcemia despite adequate infection control. The treatment course involved combinatorial-calcitonin, low-dose bisphosphonates, and corticosteroids, which led to a favorable outcome.

Unveiling the hidden culprit: Parathyroid adenoma induced recurrent renal calculus and pancreatitis-A case report.

This case report describes a 52-year-old patient presenting with recurrent episodes of pancreatitis and renal stones. Further investigation revealed hypocalcemia and elevated parathyroid hormone (PTH) levels, leading to diagnosis of a parathyroid adenoma. This case highlights the importance of considering primary hyperparathyroidism in patients with recurrent pancreatitis and renal stones, as early diagnosis and surgical intervention can prevent recurrence and reduce morbidity. Abstract: Primary Hyperparathyroidism secondary to Parathyroid adenoma, rarely presents as acute pancreatitis. A 38-year-young male with a history of recurrent renal stones referred from a local center, presented to the emergency services, with a diagnosis of acute pancreatitis and bilateral renal stones. Laboratory evaluation showed an elevated calcium level, elevated PTH levels, low vitamin D, and low phosphorus levels. CT scan done outside was suggestive of acute pancreatitis along with bilateral renal calculi. USG neck and MIBI scan done as a part of hypercalcemia evaluation showed presence of a right parathyroid adenoma. Parathyroid adenoma was later removed, and calcium and parathyroid levels were normal on subsequent follow ups.

Expanding the Clinical Features of Schimke Immuno-Osseous Dysplasia: A New Patient with a Novel Variant and Novel Clinical Findings.

Schimke Immuno-Osseous Dysplasia (SIOD) (MIM:242900) is an ultra-rare autosomal recessive pan-ethnic pleiotropic disease. Typical findings of this syndrome are steroid-resistant nephrotic syndrome, cellular immunodeficiency and spondyloepiphyseal dysplasia and facial dysmorphism. Biallelic variants in the SMARCAL1 gene cause SIOD. The five-and-half-year-old female patient was evaluated because of short stature, dysmorphism, hypercalcemia, hypophosphatemia and elevated FSH levels. Karyotype analysis and array-CGH testing were normal. Clinical Exome Sequencing was performed via next-generation sequencing to analyze genes associated with hypophosphatemia. No pathogenic variant was detected. The subsequent detection of proteinuria during her follow-up for cross-fused ectopic left kidney ultimately facilitated the diagnosis of SIOD, although no obvious spondyloepiphyseal dysplasia was detected. Re-analysis of CES revealed a novel homozygous c.2422_2427+9delinsA pathogenic variant in the SMARCAL1. One hundred twenty-five SIOD cases from 38 literature reporting SMARCAL1 gene pathogenic variants were reviewed to investigate whether hypercalcemia, hypophosphatemia and elevated FSH levels had been previously reported in SIOD patients. This review revealed that this was the first time these findings had been reported in a SIOD patient. This report expands not only the phenotypic but also genotypic spectrum of SIOD.

A Case Report of the Atypical Presentation of Multiple Myeloma Manifesting as a Sternal Mass.

The diagnosis of multiple myeloma (MM) is made based on the presence of either marrow clonal plasma cells > 10% or an extramedullary or bony plasmacytoma confirmed by biopsy. Additionally, at least one of the SLiM (sixty years, light chain ratio, magnetic resonance imaging)-CRAB (calcium elevation, renal insufficiency, anemia, and bone lesions) myeloma-defining events must be present. MM typically presents with symptoms such as fatigue due to anemia, kidney failure, hypercalcemia, and bone pain. It is uncommon, though, for MM to manifest as a single bone mass. We report the case of a 65-year-old male who did not fit the criteria for solitary bone plasmacytoma and presented with an unusual sternal tumor. The patient was diagnosed with MM despite not having any of the traditional symptoms such as low back pain, weight loss, anemia, or hypercalcemia. The diagnosis was based on a bone marrow examination, which showed 50% plasma cells. Radiation therapy and systemic chemotherapy were then used to treat him. The patient's symptoms, radiological findings, and biopsy results are described in detail, emphasizing the difficulty and intricacy of correctly diagnosing this uncommon manifestation of MM. This case highlights the need for a comprehensive and multidisciplinary strategy to diagnose and treat atypical presentations of MM, making sure that all possible diagnostic pathways are investigated in order to achieve accurate and timely diagnosis and treatment.

Neonatal Severe Hyperparathyroidism Causing Life-Threatening Hypercalcemia Treated With Medical and Surgical Management.

A 3-day-old male presented to a peripheral remote hospital in New South Wales, Australia, with tachypnea. He was found to have hypercalcemia, with ionized calcium >2.5 mmol/L (>10 mg/dL) (0.97-1.5 mmol/L or 1.14-1.3 mg/dL) and serum calcium of 3.85 mmol/L (15.43 mg/dL) (2.2-2.8 mmol/L or 8.5-10.5 mg/dL). Peak serum calcium was 5.4 mmol/L (21.64 mg/dL). He was transferred to a tertiary pediatric intensive care unit. Medical management (including hyperhydration, diuretics, corticosteroids, bisphosphonates, cinacalcet, and calcitonin) failed to maintain normocalcemia; therefore, total parathyroidectomy was performed on day 16 of life. Hungry bones syndrome developed postoperatively, requiring high doses of calcium, calcitriol, and phosphate supplementation. Genetic testing identified compound heterozygosity for 2 likely pathogenic variants in the calcium-sensing receptor gene. He is now 3 years old and is growing and developing without any concerns. This case highlights the importance of aggressive initial management in addressing severe hypercalcemia through perioperative management principles as well as the prolonged nature of hungry bones syndrome.