Hypercalcemic Crisis in Pregnancy: A Rare Case of Primary Hyperparathyroidism Complicated by Wernicke's Encephalopathy and Multi-Organ Involvement

A Rare Case of Primary Hyperparathyroidism, Hyperemesis Gravidarum, and Wernicke Encephalopathy

To investigate the etiological diagnosis and treatment of hypercalcemic crisis,the clinical data from one case of primary hyperparathyroidism first represented with hypercalcemic crisis were retrospectively analyzed.In this case,hypercalcemic crisis recurrent attacked and the etiological diagnosis was twists and turns.It was turned out to be parathyroid adenoma after operation.The clinical manifestations of primary hyperparathyroidism were hypercalcemia,hypophosphatemia,elevated alkaline phosphatase,bone lesions,urinary calculi,peptic ulcer and so on,more than 90％ of which were caused by parathyroid adenoma.Resection of tumor is the best choice after diagnosis. Key words: Hypercalcemic crisis ; Primary hyperparathyroidism ; Parathyroid adenoma ;

Variability of clinical presentations in three cases of parathyroid carcinoma.

A case of primary hyperparathyroidism(PHPT) complicated with Graves' disease was reported. The parathyroid lesion(s) could not be identified by repeated MIBI and ultrasonography tests. With the control of hyperthyroidism, medical therapies of hypercalcemia were still not effective, the serum calcium levels continued to be high. Thus, the decision to operate was made. The pathological findings confirmed the diagnosis of parathyroid adenoma. For PHPT patients with clear surgical indications, even though the pre-operative localizing tests are negative, operation is still worth to try. (Chin J Endocrinol Metab, 2016, 32: 146-148) Key words: Hyperthyroidism; Primary Hyperparathyroidism; Hypercalcemia

Background: Primary hyperparathyroidism is a common disease in adults, but extremely rare in the pediatric age group. To our knowledge, pediatric primary hyperparathyroidism has never been reported in Thailand. Objectives: To describe 3 cases of primary hyperparathyroidism presenting in Thai children with different clinical manifestations. Methods: Cases of primary hyperparathyroidism in 3 Thai pediatric patients are reported herein, with a brief literature review. Results: Three patients are described. The first patient, a 14-year-old Thai girl, presented with progressively worsening bowlegs and lordosis of the spine for 1.5 years. The second patient, a 15-year-old Thai boy, presented with a history of hip pain and constipation for 3 years. The third patient, an obese 11-year-old Thai boy, presented with acute abdominal pain, which was initially incorrectly diagnosed as acute pancreatitis. Conclusions: The clinical symptoms of hyperparathyroidism in Thai children are nonspecific at an early stage of the disease. The diagnoses are usually delayed and the patients therefore suffer complications of long-term hypercalcemia such as bone deformities or renal nephrocalcinosis. We recommend initial laboratory screening in pediatric patients with nonspecific symptoms and complaints of persistent bone pain. Appropriate investigations to confirm or eliminate primary hyperparathyroidism should be made, as an early diagnosis can facilitate early treatment and prevent the organ damage that can result from delayed diagnosis. Keywords: Hypercalcemia, hyperparathyroidism, parathyroid adenoma, parathyroid hyperplasia

Primary hyperparathyroidism (PHPT) during pregnancy is rare. Hypercalcemia (HCa) may be undetected due to hypoalbuminemia and increased active transport of calcium via the placenta. Undiagnosed HCa may present with life threatening maternal hypercalcemic crisis and fetal hypocalcemia. In patients with moderate and severe HCa, parathyroidectomy (PTx) is recommended during the second trimester. Cinacalcet is approved for treatment of secondary hyperparathyroidism in patients with ESRD, HCa due to parathyroid carcinoma, and in patients with severe HCa due to PHPT unable to undergo PTx. However, cinacalcet use in pregnancy is limited to a few case reports. We present a case of PHPT presenting with severe HCa and cardiomyopathy where PTx was contraindicated due to risk of cardiac decompensation. To our knowledge this is the first case of cinacalcet use in PHPT where therapeutic doses were well tolerated resulting in a safe delivery without maternal and fetal complications. A 38 year old female at 25 weeks gestation (gravida 7, para 4) presented from an outside hospital for treatment of pyelonephritis and new onset cardiomyopathy with reduced ejection fraction of 20%. The patient had a history of preeclampsia but denied a personal or family history of nephrolithiasis and calcium abnormalities. Present medication included a prenatal multivitamin. Physical exam revealed tachycardia, hypotension and no palpable neck mass. Laboratory evaluation revealed a corrected calcium 13.8 (8.7-10.7mg/dL), ionized calcium (iCa) 1.7 (0.95-1.32 mmol/L), phosphate 1.7 (2.5-4.6 mg/dL), magnesium 1.5 (1.6-2.7 mg/dL), creatinine 0.83 mg/dL. Treatment with normal saline 0.9 % was started with IV furosemide to prevent fluid overload. Further evaluation showed an elevated intact parathyroid hormone 383 (8-85 pg/mL), elevated 1,25 dihydroxyvitamin D 147 (18-78 pg/mL), normal parathyroid hormone related peptide 14 (14-27 pg/mL), fractional excretion of calcium of 1.7%, and fractional excretion of phosphate of 25% confirming the diagnosis of PHPT. A neck ultrasound showed a 2 cm left thyroid nodule; FNA biopsy confirmed a parathyroid adenoma. Evaluation for MEN 1 was negative. Due to severe cardiomyopathy, the patient was deemed a poor surgical candidate. Treatment with cinacalcet 30 mg daily was started and slowly titrated to 90 mg twice daily. On discharge, iCa had normalized to 1.26. The patient was readmitted to the hospital four days prior to delivery for discontinuation of cinacalcet and prevention of fetal parathyroid gland suppression. The patient delivered a healthy male at 37 weeks of gestation without hypocalcemia. Treatment of PHPT during pregnancy is challenging especially in those patients who are unable to undergo PTx. Cinacalcet may be a safe option to prevent maternal and fetal complications of HCa in patients with PHPT. Further studies are needed to evaluate the safety of cinacalcet in pregnancy.

Clinical manifestations of cancer-related hypercalcemia.

Hypercalcemic Crisis in the Era of Targeted Parathyroidectomy

Giant parathyroid adenomas represent a distinct clinical entity forming a subset under primary hyperparathyroidism (PHPT). They comprise lesions weighing more than 3.5g. Our study aims to determine the clinical presentation, biochemical profile, outcomes, and pathological characterization of patients with giant parathyroid adenoma. Retrospective review of data of patients who underwent parathyroid surgery between January 1995 and December 2022 by a single surgeon. A total of 561 patients were identified, and out of these, 78 had glands measuring more than 3.5g. The mean age was 42years, and 51 (65%) were females. The most common complaint was bone pain (46%), followed by renal calculi (38%), with 15 (19%) patients presenting with hypercalcemic crisis. Median calcium value was 12.3mg/dL, with median PTH, vitamin D, and ALP of 1025pg/mL, 23ng/mL, and 664U/L, respectively. Fifty-three (68%) patients underwent focused parathyroidectomy, and 22 (28%) underwent neck exploration, with median postoperative calcium and PTH being 8.35mg/dL and 37.8 pg/mL, respectively. The median weight was 7g with a range from 3.58g to a whopping 31g. Symptomatic postoperative hypocalcemia was seen in 54 (69%), with 28 (36%) requiring intravenous calcium supplementation, and this was positively correlated with the preoperative ALP and PTH values. Giant parathyroid adenomas present with florid symptoms with hypercalcemia and often hypercalcemic crisis. The biochemical workup, localization, and surgical management are similar to any other case of PHPT. Postoperatively these patients are prone to hypocalcemia, and the intravenous requirement of calcium can be predicted by preoperative ALP levels.

Als hyperkalzamische Krise bezeichnet man ein akut lebensbedrohliches Krankheitsbild, das auf dem Boden einer dekompensierten Hyperkalzamie entsteht. Die Hyperkalzamie ist kein eigenstandiges Krankheitsbild, sondern immer ein Symptom einer Grunderkrankung. Die Tumorhyperkalzamie (ca. 60–70%) und der primare Hyperparathyreoidismus (ca. 20%) sind im stationaren Bereich die haufigsten Ursachen; seltener sind iatrogene, entzundliche und sonstige endokrin bedingte Hyperkalzamien. Drei verschiedene pathogenetische Grundprinzipien konnen zu erhohtem Serumkalzium fuhren: vermehrte enterale Aufnahme, vermehrter Knochenabbau und verminderte renale Kalziumausscheidung. Kennzeichnend fur eine schwere akute Hyperkalzamie ist das gemeinsame Auftreten von gastrointestinalen, renalen, kardialen und neuropsychiatrischen/neuromuskularen Symptomen. Nach Verifizierung des tatsachlichen Vorliegens einer echten Hyperkalzamie (Albuminkorrektur) und ggf. Einleitung der notwendigen akutmedizinischen Masnahmen (s. u.) ist zur Planung der weiteren Therapie die Diagnose der Grunderkrankung notig. Fur die symptomatische Therapie der Hyperkalzamie gibt es verschiedene Ansatzpunkte: Erhohung der Kalzium- Ausscheidung, Hemmung der Kalziumfreisetzung und Hemmung der Kalzium-Absorption. An erster Stelle steht hierbei die Volumensubstitution und die kalziumarme Ernahrung. Weitere Masnahmen sind die Gabe von Diuretika, Kalzitonin und Bisphosphonaten. Bei Patienten mit granulomatosen Erkrankungen kann durch die Gabe von Glukokortikoiden eine Senkung des Kalziumspiegels erreicht werden. Liegt ein akutes oder chronisches Nierenversagen vor, oder konnte durch die o. g. Masnahme keine suffiziente Senkung des Kalziumspiegels erreicht werden, kann die Hamodialyse eine schnelle Senkung des Serumkalziums erzielen.

The authors report a case of hypercalcemia in a 79-year-old woman that led to multiple hospitalizations. The case was challenging because the hypercalcemia was ultimately found to have 2 distinct causes. The patient initially presented with an elevated parathyroid hormone (PTH) level, consistent with primary hyperparathyroidism. She underwent parathyroidectomy and her hypercalcemia resolved in the immediate postoperative period. Four days after the operation, she was found to have recurrent hypercalcemia with a suppressed PTH level, raising suspicion for hypercalcemia of malignancy. After an extensive workup, the patient was found to have lymphoma, which was causing hypercalcemia and suppressing PTH. The authors also review the literature on hypercalcemia of malignancy.

Hypercalcemic crisis (HC) is a rare and severe complication of primary hyperparathyroidism (PHPT), characterized by markedly elevated serum calcium levels and diverse clinical manifestations. PHPT is the primary cause of hypercalcemia and is often asymptomatic. The factors that trigger HC in PHPT are not fully understood. Here we report a patient who was diagnosed with HC resulting from previously undetected PHPT. Remarkably, the patient exhibited no symptoms of PHPT before the HC episode. The patient presented with neurological symptoms, including altered behavior and slurred speech, which were attributed to hypercalcemia. The probable trigger for HC in this case appears to be an acute febrile illness related to a urinary tract infection (UTI). The occurrence of an acute febrile illness or UTI as a precipitating factor for HC in PHPT is a rare and infrequently documented phenomenon in the existing medical literature.

Background. The relevance of the study is due to the fact that undiagnosed primary hyperparathyroidism (PHPT) significantly increases the risk of osteoporosis and fractures, nephrolithiasis and renal failure, reducing the quality of life of patients. All this necessitates the improvement of the organizational system of disease detection and monitoring. The purpose of the study was to assess the incidence of hypercalcemia (HCa) in patients with endocrine disorders and to determine the sex and age composition in the subgroup with HA. Materials and methods. The frequency of HCa (by total calcium and ionized calcium levels) in 107 patients with endocrine pathology examined in endocrine clinic during November and December 2021 was determined, gender and age characteristics, the nature of endocrine pathology in the subgroup with HCa were characterized. The level of total calcium was determined by photocolorimetric method using a photometer ”Solar PM 2111“, set ”SpL“ (Ukraine), the level of ionized calcium was determined by a calculated method depending on the concentration of albumin. Results. Among patients with endocrine pathology, HCa occurs in approximately 30 % of cases. The ratio between men and women is similar in the subgroups with normo- and hypercalcemia. The presence of endocrine comorbidity in patients increases the risk of HCa. The obtained results show that the existing organization of work in Ukraine to identify and monitor patients with HCa needs to be optimized. As a sample, we can use the conceptual model of action of a primary care physician developed by American authors, aimed at optimizing the work on the detection and monitoring of PHPT. Conclusions. For Ukraine, the problem of improving the organizational system for detection and monitoring of HCa, which should include deadlines for monitoring blood calcium levels, periodicity of research, further monitoring of people with HCa, provide communications to primary and specialized physicians.

BackgroundThere is no consensus or management algorithm for primary hyperparathyroidism (PHPT) in pregnancy.MethodsThis study comprises a retrospective case series. From August 2014 to December 2020, 9 cases of PHPT in pregnancy were diagnosed by a multidisciplinary team (MDT) consultation center of obstetrics in our hospital. Their clinical manifestations, treatment strategies, and maternal and infant outcomes were analyzed.ResultsThe median onset age of the patients was 32 (25 ~ 38) years. PHPT was diagnosed in two cases before pregnancy, in six cases during pregnancy and in one case postpartum. The main clinical manifestations were nausea, vomiting, and other nonspecific symptoms, with anemia as the most common maternal complication. Hypercalcemia crisis was developed in one case. The median levels of preoperative serum calcium and parathyroid hormone (PTH) were 3.08 (2.77 ~ 4.21) mmol/L and 300.40 (108.80 ~ 2603.60) pg/ml, respectively. The parathyroid ultrasonography tests were positive in eight cases and negative in one patient who had an ectopic lesion localized by 99mTc-MIBI. Parathyroidectomy was conducted in 7 cases during the 2nd trimester, including 2 patients diagnosed before pregnancy who refused surgery, 1 patient during the 1st trimester, and 1 patient postpartum, with a significant reduction in serum concentrations of calcium and PTH. A management algorithm was developed.ConclusionThis case series suggests that pregnant women with PHPT should be managed by MDT according to the algorithm. If PHPT is confirmed in fertile women before pregnancy, parathyroidectomy should be strongly suggested and performed. If PHPT is diagnosed during pregnancy, even in its mild form, surgical treatment, optimally during the 2nd trimester, is effective and safe for pregnancy and neonatal outcome.

Most cases of primary hyperparathyroidism (PHP) are diagnosed when mild hypercalcemia is noted incidentally on blood chemistry panels. An international