{'fraction_non_alphanumeric': 0.03671820098383696, 'fraction_numerical': 0.027406886858749122, 'mean_word_length': 4.838461538461538, 'pattern_counts': {'":': 0, '<': 1, '<?xml version=': 0, '>': 1, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
MON-105 A Case Report of Neonatal Hyperinsulinism in an Infant with Turner Syndrome Successfully Treated with Diazoxide

Abstract BACKGROUND: Only four previous cases of hyperinsulinemic hypoglycemia have been reported in association with Turner Syndrome. All of these cases involved young females with a mosaic form of Turner Syndrome involving a ring X chromosome; an abnormality found in just 16% of Turner Syndrome karyotypes. Two of these cases showed responsiveness to diazoxide which stabilized their blood glucose levels and allowed for a longer fasting tolerance. Clinical Case: A 5 day old female who was born at 36 weeks and 1 day with Turner Syndrome was referred to pediatric endocrinology for persistent episodes of hypoglycemia. Patient was started on D10W with electrolytes at an outside hospital due to hypoglycemia and was transferred to the NICU at day of life 3. D10W was discontinued on day of life 5 due to adequate PO feeding and reassuring blood glucose readings. Blood glucose was monitored every 4 hours and infant continued to have episodes of asymptomatic hypoglycemia typically in the mid-low 40’s, with one episode of low blood sugar to 36 mg/dl. Due to a concern for hyperinsulinism, labs were drawn at a serum blood glucose of 46mg/dl. The infant was found to have a beta-hydroxybutyrate of 0.2 (0.0-0.8mmol/L) and insulin of 1.0 (2.0–19.6uIU/mL). Hyperinsulinism was thus confirmed by the presence of inappropriately low ketones and inappropriately normal insulin in the setting of hypoglycemia. We initiated the patient on 7.5mg diazoxide BID (5.9mg/kg/day total) and was highly responsive to diazoxide. After her third dose (36 hours into treatment), her hypoglycemia had resolved and blood sugar readings had normalized. Patient was discharged home with a glucometer and instructions for the mother to call if blood glucose was less than 60. The patient has been stable on diazoxide with no reported episodes of hypoglycemia. Interestingly, her genetic testing demonstrated a complex chromosomal microarray pattern on chromosome X, a mosaicism with 2 cell lines: 1 with monosomy X and 1 cell line with a normal X chromosome with a ringed X chromosome. Conclusion:This is a case demonstrating the efficacy of diazoxide in the management of asymptomatic hyperinsulinemic hypoglycemia in a neonate with Turner Syndrome with a ring X chromosome. References: Cappella M, Graziani V, Pragliola A, et al. Hyperinsulinemic Hypoglycaemia in a Turner Syndrome with Ring (X). Case Rep Pediatr 2015; 2015:561974.


SAT-338
Background: Brown tumor of hyperparathyroidism (BTHPT) are non-neoplastic bony lesions that arise from increased osteoclastic activity in the setting of uncontrolled hyperparathyroidism, (HPT). The prevalence is low, with a frequency of 3% in primary HPT and 2% in secondary HPT. It is rare as earlier detection and treatment of HPT prevents the progression and development of BTHPT. BTHPT is more common when HPT is untreated. BTHPT are benign tumors which usually show high 18Fludeoxyglucose-PET/ CT (FDG) uptake. The mechanism of elevated FDG uptake has been suggested to be the presence of giant cells, and intracellular glucose metabolism of the macrophages may also play a role. We describe a case of a patient who presents with FDG uptake-negative BTHPT of the left femur. Clinical Case: 62-year-old male with end-stage renal disease (ESRD) and secondary HPT presented with severe left hip pain for many months. The patient had poor access to health care and had not received routine medical treatments at an outside facility prior to presentation at our facility. Laboratory studies showed PTH 1942 pg/ml (14-72), Calcium 8.7 ng/dl (8.4-10.2), phosphorus 3.6 mg/ dl (2.5-4.9), Vitamin D total 19.7 ng/ml (30-140), Vit D 1,25 9 pg/ml (18-72), and creatinine 7.75 mg/dl (.66-1.28), alkaline phosphatase 57 u/L (33-94). FDG scan revealed several lucencies in the left proximal femur, with no uptake. The patient underwent left total hip arthroplasty due to impending pathological fracture. Surgical pathology revealed fragments of fibrous tissue with fibroblasts, hemosiderin and interstitial hemorrhage, consistent with brown tumor. Conclusion: We describe a unique case of BTHPT of the left femur which was the unrecognized cause of hip pain. BTHPT occurs late in the setting of HPT and is considered as a sign of poorly controlled disease. This is the first case of BTHPT to our knowledge that is not FDG-avid. This is unusual given the vascularity seen in these tumors and highlights that glucose utilization may not necessarily reflect the degree of osteoclastic activity in these tumors. Medical treatment has been shown to be sufficient in helping resolve these lesions, although the process is usually slow. Conversely, in cases of refractory disease, subtotal parathyroidectomy may be required. Our case describes an FDG-negative BTHPT, and highlights that although rare, this should be considered in the management of patients with ESRD, secondary HPT and hip pain. Patients can present with various imaging characteristics. Despite being rare, this case underscores the importance of recognition of BTHPT, so that intervention may be rendered early.

MON-105
Background: Only four previous cases of hyperinsulinemic hypoglycemia have been reported in association with Turner Syndrome. All of these cases involved young females with a mosaic form of Turner Syndrome involving a ring X chromosome; an abnormality found in just 16% of Turner Syndrome karyotypes. Two of these cases showed responsiveness to diazoxide which stabilized their blood glucose levels and allowed for a longer fasting tolerance.
Clinical Case: A 5 day old female who was born at 36 weeks and 1 day with Turner Syndrome was referred to pediatric endocrinology for persistent episodes of hypoglycemia. Patient was started on D10W with electrolytes at an outside hospital due to hypoglycemia and was transferred to the NICU at day of life 3. D10W was discontinued on day of life 5 due to adequate PO feeding and reassuring blood glucose readings. Blood glucose was monitored every 4 hours and infant continued to have episodes of asymptomatic hypoglycemia typically in the mid-low 40's, with one episode of low blood sugar to 36 mg/dl. Due to a concern for hyperinsulinism, labs were drawn at a serum blood glucose of 46mg/dl. The infant was found to have a betahydroxybutyrate of 0.2 (0.0-0.8mmol/L) and insulin of 1.0 (2.0-19.6uIU/mL). Hyperinsulinism was thus confirmed by the presence of inappropriately low ketones and inappropriately normal insulin in the setting of hypoglycemia. We initiated the patient on 7.5mg diazoxide BID (5.9mg/kg/ day total) and was highly responsive to diazoxide. After her third dose (36 hours into treatment), her hypoglycemia had resolved and blood sugar readings had normalized. Patient was discharged home with a glucometer and instructions for the mother to call if blood glucose was less than 60. The patient has been stable on diazoxide with no reported episodes of hypoglycemia. Interestingly, her genetic testing demonstrated a complex chromosomal microarray pattern on chromosome X, a mosaicism with 2 cell lines: 1 with monosomy X and 1 cell line with a normal X chromosome with a ringed X chromosome. Conclusion:This is a case demonstrating the efficacy of diazoxide in the management of asymptomatic hyperinsulinemic hypoglycemia in a neonate with Turner Syndrome with a ring X chromosome.

SUN-189
Background: Hypoaldosteronism occurs in 6-30% of patients following unilateral adrenalectomy for primary aldosteronism. The Endocrine Society guidelines recommend discontinuing potassium supplementation and spironolactone postoperatively with repeat renin and aldosterone after surgery to monitor for cure. Clinical Case: A 69-year-old male with a 15-year history of hypertension on amlodipine 10 mg daily, atenolol 100 mg daily, terazosin 5 mg daily, valsartan 160 mg daily, spironolactone 50 mg three times daily, with longstanding hypokalemia on potassium chloride 20 mEq four times daily presented with an ischemic stroke and persistent hypertension (BP 182/79). Following discontinuation of spironolactone, evaluation revealed aldosterone concentration of 214 ng/dL (normal 4.0 -31) and plasma renin activity of 0.1 ng/mL/hr (normal 0.5 -4.0), giving an aldosterone-to-renin ratio of 2,140. CT of the abdomen showed a 3 cm right adrenal mass. He underwent uncomplicated right adrenalectomy for primary aldosteronism. Postoperative potassium was 3.4 mEq/L (normal 3.5-5.0) and hypertension persisted, so he was discharged on potassium chloride 10 mEq, losartan 100 mg daily, amlodipine 10 mg daily, and labetalol 200 mg twice daily. Two weeks later potassium level was 5.1 mEq/L and potassium chloride supplement was discontinued. Six months postoperatively, potassium was 5.7 mEq/L with well-controlled blood pressure, so losartan was discontinued. Labs over the subsequent several weeks showed persistent hyperkalemia up to 6.2 mEq/L and new hyponatremia to 128 mEq/L (normal 134 -150). Repeat plasma renin activity was 0.51 ng/mL/ hr and aldosterone concentration <1.0 ng/dL. Morning cortisol concentration was 18.3 ug/dL (normal 6.7 -22.6) and ACTH 38 pg/mL (normal 6.0 -50 pg/mL). He was diagnosed with postsurgical hypoaldosteronism. Potassium stabilized at 5.1 mEq/L and sodium stabilized at 134 mEq/L, so he was monitored without treatment for hypoaldosteronism. One year postoperatively his labs showed: potassium 5.1 mEq/L, sodium 135 mEq/L, renin 1.0 ng/mL/hr, and aldosterone 5.7 ng/dL. Conclusion: This patient had primary aldosteronism leading to suppression of aldosterone secretion from the contralateral healthy adrenal gland. This resulted in postoperative hypoaldosteronism once the affected adrenal gland was resected. This case demonstrates the need for continued monitoring of potassium, sodium, renin, and aldosterone following unilateral adrenalectomy for primary aldosteronism, especially in the setting of postoperative angiotensin receptor blocker use or other medications which can affect the renin-angiotensin-aldosterone system.

LC-MS/MS Measurements of Parathyroid Hormone-Related Protein (PTHrP): Association Between Ca, PTH and PTHrP in Clinical Samples
Mark M. Kushnir, PhD 1 , Joely A. Straseski, PhD 2 . 1 ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA, 2 Univ of Utah School of Medicine, Salt Lake City, UT, USA.

SUN-720
Parathyroid hormone related protein (PTHrP) is involved in intracellular calcium (Ca) regulation and homeostasis. The main clinical utility of PTHrP as a biomarker is to assist with determining the cause of unexplained hypercalcemia (UH). Considering that PTHrP, along with parathyroid hormone (PTH), is involved in Ca homeostasis, we evaluated associations between concentrations of Ca, PTH, PTHrP and age, and the between-sex differences in clinical patient samples. We reviewed historic data on Ca, PTH and PTHrP concentrations measured in routine patient samples (n=2,701) submitted for testing to a large clinical diagnostic laboratory. Among the analysed samples 31.1% were from adults (18-59y), 52.9% from older adults (60-79y) and 16.9% from elderly (>79y). Out of the samples, 60.5% were from women (W) and 39.5% from men (M). PTHrP was measured using a validated LC-MS/MS method; Ca and parathyroid