Sporadic primary hyperparathyroidism with multiple parathyroid adenomas

Multiple lesions of the parathyroid glands (PTG) in primary hyperparathyroidism (PHPT) can be sporadic or develop as part of hereditary syndromes, manifesting at young age. There the description of a severe sporadic PHPT with big parathyroid neoplasms in the young patient is presented. Clinical data made it possible to suspect MEN-1 syndrome or parathyroid carcinomas; however, mutations CDKN, CDC73, MEN1 were excluded. The patient underwent removal of three identified tumors: benign adenomas of the left PTG and hyperplasia of the right one. Postoperative hypocalcemia and severe hungry bone syndrome required the administration of vitamin D and calcium carbonate preparations. However, a year after the operation, a «mild» recurrent disease was confirmed. Taking into account the patient’s refusal to reoperation and a significant improvement of the target organs state, active observation was continued. The patient needs further careful dynamic monitoring by specialists in order to timely identify indications for repeated surgical treatment to improve the life quality and span.

From the Old, the Best: Parathyroidectomy in the Management of Soft-Tissue and Vascular Calcification in Patients with Chronic Renal Disease

Introduction. Bone mineral disease in patients with chronic kidney disease (CKD-MBD) is a clinical syndrome involving bone, biochemical changes, and extraosseous calcification. These complications increase morbidity and mortality. Prevalence reports are rare. Case Report. This case shows a young woman on peritoneal dialysis (PD) for 10 years with severe secondary hyperparathyroidism and soft-tissue calcifications in the hands, pelvis, and right knee, as well as severe vascular calcification, managed with calcimimetics without success. We decided to perform subtotal parathyroidectomy (STPTX). Three months after surgery, she had satisfactory evolution, despite notable hungry bone disease, without bone pain or functional limitation and almost no calcifications. Discussion. The benefit of hemodialysis has been shown with better volume management and improvement of calcium/phosphate products. STPTX allowed biochemical control and calcification improvement, with an evident better quality of life for our patient. Therapeutic alternatives need to be tailored to the patient’s characteristics in the calcimimetics era.

Brown tumour mimicking skeletal metastasis

Brown tumours of bone are highly vascular osteolytic lesions that depict a reparative cellular process instead of a neoplastic process in hyperparathyroidism (HPT) patients. These tumours have the potential to be aggressive and destructive. We report a case of a 30-year-old woman who presented with left thigh and lower back pain. The radiological evaluation showed multiple bony lesions in the pelvis and the spine, which mimicked multiple metastatic tumours. However, on biochemistry evaluation, serum calcium, alkaline phosphatase, and parathyroid hormone were all high, while serum phosphate was low, indicating primary HPT (PHPT), which was confirmed by parathyroid scintigraphy showing left parathyroid adenoma. Hence, the bony lesions were diagnosed as brown tumours secondary to PHPT. The patient underwent parathyroidectomy and developed severe hungry bone syndrome requiring parenteral calcium infusion along with oral calcium and active vitamin D supplementation. The clinical symptoms of bone pain improved after surgery.

ISSUES OF TREATMENT OF SECONDARY HYPERPARATHYROIDISM WITH THE EYES OF AN ANESTHESIOLOGIST. EXPERIENCE OF PERIOPERATIVE ANESTHESIOLOGICAL SUPPORT AND INTENSIVE THERAPY IN PARATHYROID SURGERY

Secondary hyperparathyroidism (SHPT) affects a majority of patients with chronic kidney disease (CKD) in the third and above stages with manifestations of chronic renal failure (CRF), especially in patients undergoing hemodialysis. Despite the temporary effects obtained from taking calcimimetics, there is no significant breakthrough in the treatment of SHPT, and most of the patients require parathyroid surgery (PTS). This review describes the main pathophysiological changes in SHPT, their correction, summarizes the indications for PTS, reflects preoperative preparation, anesthetic management, perioperative intensive care, and possible complications associated with SHPT and CRF. The experience of treating 214 patients with SHPT, 237 PTS (23 in connection with relapse of SHPT), algorithm of examination, preoperative preparation, anesthetic management and perioperative intensive care are presented. In the postoperative period, patients are at risk of severe hypocalcemia and hungry bone syndrome, which require careful monitoring and calcium replenishment.

Severe Refractory Hypocalcemia After Administering Zoledronic Acid for Osteoporotic Fracture in Primary Hyperparathyroidism That Is Complicated Into Hungry Bone Syndrome: A Case Report

Zoledronic acid is a very effective (IV) amino bisphosphonate which is indicated in osteoporosis, hypercalcemia of malignancy, multiple myeloma, Paget’s disease, and bone metastases from solid tumors. Bisphosphonate inhibits bone resorption through actions on osteoclast activity resulting in increasing bone density. Unfortunately, there are side effects associated with zoledronic acid one of those is mild to moderate hypocalcemia. Hungry Bone Syndrome (HBS) is defined as a severe drop in calcium levels less than 2.1 mmol/L and/or prolonged hypocalcemia for more than 4 days post parathyroidectomy. Most seen in patients’ who have secondary hyperparathyroidism compared to primary hyperparathyroidism. The sudden drop of parathyroid (PTH) levels post parathyroid resection after a prolonged duration of high PTH levels causes net calcium to move into the bone, this is the most proposed hypothesis. The authors report a case of a 32 years old female known case of severe osteoporosis presented to the emergency department complaining of left hip pain after a short height fall admitted as a case of pathological fracture. Furthermore, upon admission was vitally stable although on examination the patient had left side hip pain and tenderness otherwise unremarkable. Initial lab investigation showed a calcium level of 2.57 mmol/L and PTH level 37 pmol/L otherwise electrolytes and full blood count were unremarkable. Radiological investigations were done, and an X-ray has shown a left hip fracture where the patient underwent an operating room for fixation. In addition, a 99mTc-HDP bone scan revealed hyperparathyroidism and brown tumor. 99m Tc SESTA-MIBI whole body scan suggested metabolic bone disease. Furthermore, the parathyroid showed suggested typical parathyroid adenoma below and lateral to the lower pole of the left thyroid lobe. The ultrasound of the thyroid showing a well-defined hypoechoic nodule 2.5x1.15 cm with significant hypervascularity is seen, which is most likely to be enlarged left parathyroid gland. Thus, diagnosis of primary hyperparathyroidism has been reached with parathyroid adenoma which was resected during a hospital stay. The patient received pre-op zoledronic acid as a treatment for osteoporosis prior to the hip operation. After the parathyroid adenoma resection, the patient had a sudden drop of PTH and developed hypocalcemia which was 1.1 mmol/L. Thus, the complication of post operated parathyroid adenoma of HBS diagnosis has been made. Interestingly the patient needed to increase the length of stay of the hospital due to refractory hypocalcemia where the patient has been receiving IV calcium and vitamin D supplementation which hadn’t improved until a month of IV calcium gluconate. Although, the patient didn’t develop any signs or symptoms of hypocalcemia.

Calciphylaxis in Association With Hungry Bone Syndrome. A Possible Risk Factor?

Background: Calciphylaxis (calcific uremic arteriolopathy) is a rare complication seen in, although not limited to, patients with end-stage renal disease (ESRD). The abnormal regulation of calcium (Ca) and phosphorus (P) homeostasis in this patient group results in intravascular Ca deposition. These patients often develop secondary/tertiary hyperparathyroidism, presenting unique treatment challenges. When patients do not respond to medical therapy, parathyroidectomy is an option that may be complicated by hungry bone syndrome (HBS). We present a case of a patient with calciphylaxis with HBS post-parathyroidectomy. Case Report: The patient is a 41 y.o. male with ESRD on hemodialysis who presented with lower extremity ulcers complicated by calciphylaxis. On admission, the PTH was elevated at 2200 U with a normal corrected Ca 8.7 and P 8.5. He was found to have a non-displaced pathologic fracture of the right femoral neck. CT scan of the neck revealed nodular parathyroid hyperplasia affecting all four glands. His hyperparathyroidism was managed medically with cinacalcet, phosphate binder, and sodium thiosulfate to optimize his condition before surgery. He underwent a subtotal parathyroidectomy, with post-op course complicated by HBS. Repeat labs showed PTH 444, P 6.1, corrected Ca 7.4, and ionized Ca <4. Despite frequent repletion with IV Ca and addition of calcitriol, he remained persistently hypocalcemic with symptoms (paresthesia, perioral numbness) and prolonged QTc (560 ms) on 12-lead EKG. After weighing the more imminent risk of unstable arrhythmia versus exacerbation of HBS by targeting too high of a Ca level post-op, ICU admission was decided where Ca levels could be more closely monitored and titrated. The patient eventually demonstrated an improvement in his Ca level and symptoms. Discussion: This patient with ESRD complicated by hyperparathyroidism presented with ulcers and calciphylaxis refractory to medical therapy, requiring parathyroidectomy. HBS was a concern in this particular patient given his risk factors. Hypocalcemia is an expected electrolyte imbalance that usually resolves within 2–4 days after parathyroidectomy. Severe and persistent hypocalcemia on day 4 after the procedure should raise concern for HBS. It has been shown that the risk is higher in patients who have secondary hyperparathyroidism, prolonged duration of elevated PTH, age >60, and radiologic evidence of bone disease. ConclusionCalciphylaxis is an uncommon but potentially fatal illness seen in ESRD patients associated with Ca and P abnormalities. Whether this may increase the risk of HBS status-post parathyroidectomy is unknown; however, HBS requiring ICU management to treat refractory hypocalcemia has been reported in this group1. It is arguable that calciphylaxis may serve to identify such patients. (1) Hassanein M, et al. BMJ Case Rep 2018;11:e226696. doi:10.1136/bcr-2018–226696