Adrenergic Symptoms Research Articles

7148 Fake Fingerstick: Artifactual Hypoglycemia in a Hospitalized patient with Systemic Sclerosis

Abstract Disclosure: S. Shteyman: None. F. Mirza: None. Introduction: Artifactual hypoglycemia is a problem in hospitalized patients. It often leads to unnecessary use of resources and creates anxiety for the patient and medical team. We describe a case of a 60-year-old female with history of systemic sclerosis (Raynaud, telangiectasias, esophageal dysmotility, interstitial lung disease, pulmonary hypertension), rheumatoid arthritis, autoimmune hepatitis and hypothyroidism who initially presented to the hospital emergency department with bilateral lower extremity radiculopathy, and new onset urine incontinence. She underwent spine surgery initially, followed by emergent left upper extremity thrombectomy two days later. Postoperatively, she was noted to have fingerstick glucose of 34 mg/dL. Dextrose and juice were given, and endocrinology service was consulted. The patient was monitored with 2 hourly fingerstick glucose and was noted to have frequent hypoglycemia on fingerstick monitoring. Serum glucose in AM labs was consistently within normal limits. Patient denied both adrenergic and neuroglycopenic symptoms during periods of “hypoglycemia”. She was asked to fast till fingerstick glucose was noted to be at 43 mg/dl, when blood was drawn for serum glucose, insulin, proinsulin, C-peptide, B-hydroxybutyric acid, IGF-2, insulin AB, and sulfonylurea panel, prior to giving her any dextrose. She was asymptomatic at the time. All labs were within normal limits, with serum glucose at 119 mg/dL. At that point, artifactual hypoglycemia was suspected. Serum glucose was checked twice again when fingerstick glucose was noted to be <45mg/dL, and corresponding serum glucose was noted to be normal at both times (>100 mg/dL). Decision of artifactual hypoglycemia was made and fingerstick glucose monitoring was discontinued in this non-diabetic patient, with recommendation to check serum glucose only if symptoms of hypoglycemia occur, as fingerstick glucose values were not reliable. Fasting serum glucose continued to be normal for the remainder of the hospitalization. Discussion: There have been a handful cases of artifactual hypoglycemia reported in patients with CREST, systemic sclerosis, and Raynaud’s disease. It is thought to be due to abnormal small vasculature causing delayed transit of blood through capillaries, and increased uptake of glucose into the interstitial tissue. Some recommend using earlobe sticks as a replacement for fingerstick glucose monitoring in these patients. Future research can explore if continuous glucose monitoring can be used reliably in these patients. Presentation: 6/1/2024

5104 Ectopic Insulinoma- a Rare Presentation of a Rare Tumor

Abstract Disclosure: O. Lavrynenko: None. A. DeMarsilis: None. C. Jiang: None. R. Middelbeek: None. H. Rosen: None. M.E. Patti: None. Background. Insulinoma is a rare, functioning neuroendocrine tumor (NET) typically localized within the pancreas, that secretes insulin, resulting in hypoglycemia. Ectopic insulinoma is even more rare than pancreatic insulinoma and is more difficult to diagnose. Case report. This 31-year-old previously healthy woman, who had an uncomplicated pregnancy and a healthy baby in October 2021, presented with intermittent hypoglycemia for more than 8 months. She denied any prior use of insulin or antidiabetic medications and was not currently taking any medications at all. There is no history of bariatric or other GI surgery. While nursing her baby in early 2022, she was noted to have a capillary blood glucose (BG) between 20-30 mg/dl, and reported both adrenergic and neuroglycopenic symptoms of hypoglycemia; these usually occurred in the morning or before lunch, especially if lunch was delayed. She wore a Libre CGM which revealed episodic hypoglycemia, which correlated with her timeline of symptoms. She reported weight gain of 8 lbs during this period. Laboratory data obtained after a 10-12 hour fast revealed hypoglycemia (venous glucose 39 mg/dL) with inappropriately high plasma insulin (42.3 mIU/ml), C- peptide (3.78 ng/ml), and proinsulin (99 pmol/L). Beta-hydroxybutyrate was 0.05 mmol/L (reference <0.28) and insulin autoantibody and sulfonylurea screen were negative. Various imaging modalities for localization revealed a solitary omental mass outside the pancreas consistent with extra-pancreatic insulinoma. No pancreatic lesion or other findings were observed with MRCP, CT C/A/P or endoscopic ultrasound. The patient underwent CT-guided biopsy of the omental mass, which confirmed a well-differentiated NET, consistent with insulinoma. Due to persistent hypoglycemia, she was started on corn starch and diazoxide. She subsequently underwent laparoscopic resection of the mass. Postoperative pathology revealed a well-differentiated NET staining positive for insulin, consistent with an extra-pancreatic insulinoma. Following surgical resection of the insulinoma, her hypoglycemia resolved even off corn starch and diazoxide. Conclusion. Ectopic insulinoma is a rare insulin-secreting NET. Only 28 ectopic insulinomas have been described in the literature to date1. We describe a patient with symptomatic hypoglycemia who was found to have an omental neuroendocrine tumor; hypoglycemia completely resolved after laparoscopic enucleation of the ectopic insulinoma. 1Fernando Guerrero-Pérez, Nuria Vialarrasa, Lidia V. Huanuco et. al. Ectopic Insulinoma: a systemic review: Reviews in Endocrine and Metabolic Disorders, 2023 Presentation: 6/1/2024

12214 Metastatic Functional Thyroid Carcinoma As An Unusual Cause Of Hyperthyroidism

Abstract Disclosure: S. Jimenez Salazar: None. N. Aristizabal Henao: None. C. Aguilar Londoño: None. N. Buitrago Gómez: None. S. Saldarriaga Betancur: None. D.L. Beltran: None. J.L. Torres: None. Background: Differentiated thyroid carcinoma (DTC) represents the most prevalent endocrine neoplasm. Less than 10% present distant metastases, with the lung (50%), and skeleton (25%) being the most frequently affected sites. The location of bone metastases predominates in the spine and pelvis; usually as osteolytic lesions, being more frequent in follicular thyroid cancer (FTC) (7-28%) than in patients with papillary thyroid cancer (PTC) (1.4-7%). In rare cases this metastasis can be functional and trigger a thyrotoxicosis syndrome with or without recombinant human TSH injections prior to therapy with 131-I. Clinical case: 59-year-old woman with a history of bone metastatic follicular thyroid cancer (FTC) presented with 1 month of palpitations, 4 kg weight loss, non-dysenteric diarrhea and progressive dyspnea. Her FTC had been diagnosed 8 years prior her consultation. In that time iodine refractoriness was considered due to the progression of bone lesions, and Sorafenib 800 mg per day was initiated, which was not tolerated due to severe dermatological manifestations, and the patient decided not to continue with TKI therapy. Since then, she has been treated with zoledronic acid and local radiotherapy as part of palliative care. On physical examination, she was tachycardic (150 bpm), and hypertensive (BP 160/100 mmHg). A suppressed TSH (<0.01 uUI/mL, n: 0.4-4 uUI/mL) with elevation of thyroid hormones (free T4 3.99 ng/dL, n: 0.8-2 ng/dL and total T3 480 ng/dL, n: 45-140 ng/dL) was documented, associated with a patient who had required a dose adjustment of levothyroxine from 700 mcg to 350 mcg weekly 2 months prior to admission. Additionally, she had a significantly elevated thyroglobulin (>5000 ng/mL, n: 0.1-64.1 ng/mL) with undetectable antithyroglobulin antibodies (1.09 UI/mL, n: 0-4.11 UI/mL). Considering the history of total thyroidectomy and the high tumor burden, secondary thyrotoxicosis due to functioning bone metastases of DTC was considered. Levothyroxine was discontinued, and propranolol was initiated for the control of adrenergic symptoms, along with methimazole, cholestyramine, and systemic steroids to control thyroid hormone production. 3 days after therapy adjustment dyspnea, palpitations, and thyroid function tests improved significantly (free T4 1.43 ng/dL, n: 0.8-2 ng/dL and total T3 178 ng/dL, n: 45-140 ng/dL). A new administration of therapy with 131-I 200 mCi was indicated. Antithyroid therapy and beta-blockers were continued with sustained biochemical control. Conclusion: Functional bone metastases of FTC can lead to high morbidity and mortality. Treatment consists of surgical cytoreduction of any large accessible metastatic lesion, therapy with 131-I, and high-dose antithyroid drugs to control hyperthyroidism. It is important to avoid the use of recombinant human TSH due to the risk of secondary thyrotoxicosis following its administration. Presentation: 6/2/2024

Glycyrrhizic acid induced acquired apparent mineralocorticoid excess syndrome with a hyperadrenergic state: a case report

BackgroundSyndrome of apparent mineralocorticoid excess (AME) is characterized by excessive MR stimulation despite low levels of aldosterone. 11Beta-hydroxysteroid dehydrogenase-2 (11βDSH-2) inactivates cortisol to cortisone, preventing cortisol-induced MR activation. Genetic defects in 11βDSH-2 cause AME through accumulation of cortisol in the distal nephron, leading to MR activation induced hypertension, hypokalemia and metabolic alkalosis. Acquired AME can occur due to the ingestion of glycyrrhizic acid, found in licorice root, which inhibits 11βDSH-2 and has additional effects on cortisol homeostasis through inhibition of 11βDSH-1.Case reportWe present a case of acquired AME with a hyperadrenergic symptoms induced by ingestion of Advanced Liver Support, a nutritional supplement produced by Advanced BioNutritionals(R), in a 65-year-old Caucasian female who presented with accelerated hypertension, hypokalemia, metabolic alkalosis and adrenergic symptoms. Cessation of the licorice-containing supplement resulted in complete resolution of the patient's hypertension, symptoms and abnormal lab values. To our knowledge this is the first reported case of AME from this supplement, and the first to describe accompanying hyperadrenergic symptoms.ConclusionsGlycyrrhizic acid is increasingly being found in unregulated nutritional supplements and has the potential to induce a reversable syndrome of AME. Acquired AME should be suspected in individuals who present with hypertension along with hypokalemia, metabolic alkalosis and low plasma renin and serum aldosterone levels.

Clinical manifestations and management of Tityus species envenoming: A systematic review

Scorpionism, or a clinical picture of envenomation caused by a scorpion sting, is a public health problem in South America. The manifestations clinical and management are varied and complex and there is still no consensus in the literature on these aspects. The aim of this systematic review is to describe the clinical manifestations, as well as treatment after scorpion envenomation by Tityus species. The online databases of PubMed, Scopus, Web of Science, and SciELO were systematically searched for relevant publications until march 2022. No language and age restrictions were imposed. The literature search generated 476 references and 23 studies were included in the systematic review, according to the inclusion criteria of this review. The number of participants studied was 7,781. The specie Tityus serrulatus was found in more studies. The time elapsed between the accident and medical care ranged from 15 minutes to more than 6 hours. The treatments performed in selected studies include scorpion antivenoms, corticosteroids, anesthetics, antiemetics agents, inotropics, antihypertensives and benzodiazepines drugs. In six studies, fatal cases were observed in five children aged 1 to 6-y and one in children < 14 y. We concluded that management of Tityus species envenoming is supportive and focused on the patient’s symptoms. In severe cases, antivenoms, if available, are indicated. Medications to monitor vagal and adrenergic symptoms are encouraged. This study has the potential to help professionals who deal with envenomations caused by the genus Tityus.

Sex-Related Differences in Self-Reported Symptoms at Diagnosis in Pheochromocytomas and Paragangliomas.

Biological sex can play a role in the severity of certain diseases. Our objective was to evaluate whether sex-related differences affect the signs and symptoms of pheochromocytomas and paragangliomas (PPGLs) at presentation. We reviewed the records of patients with PPGLs at our center from 1995 to 2022. Our study included 385 patients with PPGLs: 118 (30.6%) head and neck paragangliomas (HNPGLs), 58 (15.1%) thoracoabdominal paragangliomas (TAPGLs) and 209 (54.3%) pheochromocytomas (PHEOs). The cohort consisted of 234 (60.8%) women and 151 (39.2%) men. At diagnosis, more women than men presented with headaches (47.5% vs 32.4%; P = .007); however, more men presented with diabetes (21.1% vs 12.5%; P = .039). When subdivided by tumor location, headaches occurred more often in women with HNPGLs and TAPGLs (31.0% vs 11.4%; P = .0499 and 60.0% vs 21.7%; P = .0167). More men presented with diabetes among patients with PHEOs (28.2% vs 11.2%; P = .0038). In regard to nonsecretory PPGLs, women presented with a higher prevalence of headaches (46.9% vs 3.6%; P = .0002), diaphoresis (16.3% vs 0.0%; P = .0454), and palpitations (22.4% vs 0.0%; P = .0057). In patients with secretory tumors, women presented with more headaches (58.9% vs 42.7%; P = .0282) and men with more diabetes (29.3% vs 12.5%; P = .0035). In our cohort, more women presented with headaches across all tumor types and secretory statuses. More men presented with diabetes among patients with PHEOs and secretory tumors. In nonsecretory PPGLs, women had more adrenergic symptoms. These findings can be explained by differences in adrenergic receptor sensitivity, self-reported symptoms, and possibly other vasoactive peptides and sex-hormone status.

SAT151 Assessments Of Body Composition In Women With Reactive Hypoglycemia

Abstract Disclosure: A. Adamska: None. A. Buczyńska: None. K. Łuka: None. A. Wiatr: None. K. Siewko: None. E. Duraj: None. A. Krętowski: None. A. Popławska-Kita: None. Introduction: Reactive hypoglycemia is characterized by low blood glucose level in non-diabetic patients. It manifests as a syndrome of adrenergic and neuroglycopenic symptoms in the postprandial period, and their resolution occurs after consuming carbohydrates. Reactive hypoglycaemia can also be seen in patients with insulin resistance and hyperinsulinism. In patients with reactive hypoglycemia can be observed disturbances in body composition. The aim of this study was to asses of body composition in women with reactive hypoglycaemia vs control group. Subjects and Methods: We examined 103 women: 49 subjects with RH and 54 control women matched for age and BMI. Four hours oral glucose tolerance test (OGTT) with 75 grams of glucose with estimation of serum glucose and insulin was performed. Body composition was analysed by dual-energy X-ray absorptiometry (DXA). Results: We did not observe differences in fat mass, fat free mass, android fat mass and fat free mass, gynoid fat mass and fat free mass and visceral adipose tissue estimated with DXA method between studied groups (all p>0.05). However, we have found positive relationship between fat mass and serum glucose concentration at 3rd hour of OGTT (r=0.29, p= 0.03) in the group of reactive hypoglycaemia. In the control group be observe relationships between android fat-free mass and serum glucose concentration at 3rd hour of OGTT (r=-0.3, p=0.02) and gynoid fat-free mass and serum glucose concentration at 3rd hour of OGTT (r=-0.27, p=0.04). Conclusions: Women with reactive hypoglycaemia is not differ in body composition estimated with DXA methods. However RH is connected in different manner with glucose homeostasis and fat and lean mass in comparison to control group. Presentation: Saturday, June 17, 2023

Anesthetic Management of Unexpected Retroperitoneal Paragonglioma: Case Report

Paragangliomas are rare tumors that arise from extra-adrenal chromaffine cells. Paraganglioma are classed as functional or non functional based on production of catecholamines, similar pheochromocytoma the clinical presentation is non- specific. We present a case perioperative anesthesic management of accidental intra-operatively diagnosed precave paragonglioma wich was confirmed as pheochromcytoma. A 48 women presented paroxysms of hypertension and adrenergic symptoms including classic triad of episodic headache, sweating, and tachycardia. Abdominal sonography detected an adrenal mass; Surgical treatment is the only radical treatment. Histological examination of the surgical specimen revealed a paraganglioma.

Anaesthetic challenges in surgical enucleation of pancreatic insulinoma

Insulinoma is a very rare tumour occurring in 1-4 persons per million per year. It is a functional endocrine tumour of the pancreas characterized by repeated attacks of hypoglycaemia presenting as adrenergic or neuroglycopenic symptoms. Surgical management of insulinoma is the mainstay of treatment which can be done by either laparoscopic or open approach. There is occurrence of rapid and wide fluctuations of blood glucose levels in the intraoperative as well as perioperative period. Intraoperative hypoglycaemia is critical due to masking of symptoms by the effect of anaesthesia and if not detected and treated promptly may lead to permanent neurological insult. We here-in describe the challenges of anaesthetic management in a case of functional pancreatic insulinoma undergoing open surgical enucleation with the help of intraoperative ultrasonography.

Pheochromocytomas Most Commonly Present As Adrenal Incidentalomas: A Large Tertiary Center Experience.

Pheochromocytomas are increasingly diagnosed in incidentally detected adrenal masses. However, the characteristics of incidental pheochromocytomas are unclear. We aimed to assess the proportion and clinical, biochemical, radiological, genetic, histopathological, and follow-up characteristics of incidental pheochromocytomas. A retrospective review was conducted of patients with pheochromocytoma seen between January 2010 and October 2022 at a large UK tertiary care center. The diagnosis was confirmed histologically or by the combined presence of increased plasma and/or urinary metanephrines (MN), indeterminate adrenal mass on cross-sectional imaging, and metaiodobenzylguanidine avidity. We identified 167 patients with pheochromocytoma; 144 (86.2%) underwent adrenalectomy, for 23 (13.8%) surgery was either awaited, deemed unsuitable due to frailty or other metastatic malignancy, or declined by the patients. Excluding pheochromocytomas diagnosed via screening genetically predisposed individuals (N = 20), 37 of 132 (28.0%) presented with adrenergic symptoms and/or uncontrolled hypertension, while 91 of 132 (69.0%) patients presented with an incidentally detected adrenal mass. Incidentally detected patients were older (median age 62 years) than those detected due to clinical suspicion (aged 42 years) or after genetic screening (aged 33 years) (all P < .05). Incidentally detected pheochromocytomas were smaller (median 42 mm) than tumors detected due to adrenergic symptoms/uncontrolled hypertension (60 mm), but larger than tumors identified by genetic screening (30 mm) (all P < .05). Increased MN excretion showed a similar pattern (symptomatic/uncontrolled hypertension > incidental > genetic screening) (all P < .05). Hereditary predisposition was detected in 20.4% of patients (incidental, 15.3%; symptomatic/uncontrolled hypertension, 42.9%). The majority of pheochromocytomas are diagnosed incidentally and have distinct clinical, radiological, biochemical, and genetic features. Their detection at older age but smaller size may point to a different underlying tumor biology.

Poisoning patients’ clinical features according to the blood level of propranolol

Purpose: Propranolol is widely prescribed to psychiatric patients to control adrenergic symptoms. However, propranolol poisoning can be fatal due to cardiovascular complications. We analyzed associations between blood levels of propranolol and patients’ clinical features, with the aim of predicting progression to severe complications.Methods: Data were collected from patients aged 18 years or older who presented to the emergency department with propranolol poisoning between January 2016 and May 2022. We retrospectively analyzed their medical records and compared blood levels of propranolol between those who had cardiovascular complications and those who did not.Results: Two hundred patients were included in this study. The blood levels of propranolol were significantly higher in patients with hypotension, bradycardia, and prolonged QT intervals, with median values of 247.0 ng/mL (interquartile range [IQR], 56.5–333.8 ng/mL), 275.8 ng/mL (IQR, 154.3–486.4 ng/mL), and 159.0 ng/mL (IQR, 33.9–310.8 ng/mL), respectively. In the predictive analysis of cardiovascular complications using a receiver operating characteristic curve, the area under the curve was 0.729 with a cut-off value of 72.40 ng/mL (sensitivity, 0.667; specificity, 0.819). In addition, the correlation coefficient between blood levels and the amount of drug described during the history-taking at the time of presentation was 0.634, which was found to have a significantly higher relationship.Conclusion: Because blood levels of propranolol can be used as predictors of exacerbation in patients with propranolol poisoning, patients with blood levels above 72.40 ng/dL require careful treatment and observation from their initial presentation at the emergency department.

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia.

Differential diagnosis of hypoglycemia in the non-diabetic adult patient is complex and comprises various diseases, including endogenous hyperinsulinism caused by functional β-cell disorders. The latter is also designated as nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). Clinically, this rare disease presents with unspecific adrenergic and neuroglycopenic symptoms and is, therefore, often overlooked. A combination of careful clinical assessment, oral glucose tolerance testing, 72 h fasting, sectional and functional imaging, and invasive insulin measurements can lead to the correct diagnosis. Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective. Therefore, nearly all patients currently undergo surgical resection of parts or the entire pancreas. Consequently, apart from faster diagnosis, more elaborate and less invasive treatment options are needed to relieve the patients from the dangerous and devastating symptoms. Based on a case of a 23-year-old man presenting with this disease in our department, we performed an extensive review of the medical literature dealing with this condition and herein presented a comprehensive discussion of this interesting disease, including all aspects from epidemiology to therapy.

Bladder paraganglioma treated with open partial cystectomy: a case report

BackgroundBladder paraganglioma is a neuroendocrine tumor that accounts for less than 0.1% of all bladder tumors. Symptoms caused by catecholamine release such as hypertension, palpitation, syncope, and macroscopic hematuria are the most common findings. Treatment modalities include transurethral resection, and partial or total cystectomy.Case presentationA 38-year-old Turkish female patient was examined for hematuria that had been persisting for 6 months. Among the clinical findings, only hematuria was present. Absence of adrenergic symptoms such as hypertension, palpitations, and syncope at the first presentation made it difficult to consider bladder paraganglioma in the differential diagnosis. Therefore, cystoscopy and transurethral resection were performed with the thought of urothelial cancer. Findings such as hypertension and bradycardia that developed during diagnostic transurethral resection suggested that it might be bladder paraganglioma. After the radiological evaluation and endocrinological preparation, the patient underwent partial cystectomy.ConclusionThe rarity of cases having been reported in the literature leads to uncertainties in the management of bladder paraganglioma. Adrenergic symptoms developing during transurethral resection should suggest paraganglioma in the differential diagnosis. A multidisciplinary approach and medical treatment are mandatory to prevent life-threatening complications such as hypertensive crisis, vascular collapse, and multiple-organ system failure. We aimed to report the clinical presentation that includes only macroscopic hematuria mimicking urothelial cancer and to emphasize the multidisciplinary approach in the treatment.

Abstract #1323855: Endoscopic Ultrasound: A Modality With High Sensitivity to Diagnose Pancreatic Insulinoma

An insulinoma is a rare endocrine tumor of the pancreas that causes hypoglycemia through its inappropriate secretion of insulin. Insulinomas are usually benign tumors. Symptoms of hypoglycemia include both adrenergic and neuroglycopenic symptoms. Whipple’s triad should be confirmed as part of diagnostic work-up, but be aware that over time, hypoglycemia unawareness may develop and potentially confound the clinical picture. Early localization of the disease is essential to prevent fatal hypoglycemia.

#1315712: A Case of Post Bariatric Surgery Hypoglycemia Requiring Percutaneous Gastrostomy (PEG) Tube Placement in the Remnant Stomach

As the prevalence of bariatric procedures increases, an important phenomenon to consider is post-gastric bypass hypoglycemia (PBH). PBH is defined as a post-prandial plasma glucose < 55 mg/dL accompanied by adrenergic and/or neuroglycopenic symptoms that are resolved by carbohydrate intake. PBH is explained by multiple mechanisms: increased GLP-1 secretion, inappropriate insulin release, reduced insulin clearance, and impaired glucagon counter-regulation. Although rare, PBH can be very serious and distressing to patients due to the symptom burden and difficulty to manage.

ODP041 Hearing Loss Reveals Dopamine-secreting Metastatic Paraganglioma

Abstract Background Paragangliomas are neuroendocrine tumors arising from chromaffin cells in extra-adrenal parasympathetic or sympathetic tissues. Frequently they produce catecholamines such as epinephrine and norepinephrine which cause adrenergic symptoms. Rarely, paragangliomas produce only dopamine. These tumors carry a worse prognosis than other types of paragangliomas, especially when associated with loss of SDH-B. Case A 41 year old African American female with developmental delay, allergic rhinitis, OSA, and obesity presented to a primary care clinic for evaluation of hearing loss and left ear pain. Patient was treated for acute otitis media but symptoms failed to resolve. She was referred to audiology where she was found to have a mixed conductive and sensorineural hearing loss on the left. Despite using hearing aids, a year later the patient presented to an ENT clinic with several weeks of worsening left aural fullness and decreased hearing. On otoscopy, a mass was visualized in the middle ear. CT scan identified a lytic lesion at the jugular foramen extending into the middle ear; MR of the inner auditory canal was concerning for a paraganglioma. PET CT revealed widely metastatic disease including FDG avid cervical and supraclavicular lymph nodes, pulmonary nodules, hepatic lesions, and sclerotic lesions in the vertebral column and right humerus. Biopsy of an hepatic lesion confirmed metastatic paraganglioma with loss of SDH-B. Labs were consistent with dopamine excess. A 24 hour urine dopamine was 1020 mcg/24 hour (normal 65-400) with plasma dopamine elevated at 191 pg/mL (normal &amp;lt;30). A 24 hour urine norepinephrine level was mildly elevated at 85 mcg/24 hours (normal 15-80) but plasma norepinephrine and epinephrine were both normal. The patient denied any history of palpitations, flushing, headaches, or chest pain. She was briefly started on doxazosin but this was later discontinued as metanephrines and normetanephrines were not significantly elevated. The tumor was not amenable for surgery. Ultimately after discussion with oncology, the patient opted to proceed with temozolomide and capecitabine chemotherapy. Discussion It is important to recognize differences in treating dopamine secreting paragangliomas compared with other catecholamine producing tumors. Preoperative alpha blockade is not routinely recommended as it may cause hypotension and postoperative cardiovascular collapse. While chemotherapy with Cyclophosphamide–dacarbazine–vincristine has historically been used for metastatic paragangliomas, temozolomide is now showing benefit in cases with SDH-B mutation. Sources cited: Foo, S. H., et al. "Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management." Singapore Med J 51.5 (2010): e89-e93. Hadoux, J., Favier, J., Scoazec, J. -Y., et al (2014),SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma. Int. J. Cancer, 135: 2711-2720. https://doi.org/10.1002/ijc.28913. Presentation: No date and time listed

ODP655 Apathetic Hyperthyroidism

Abstract Introduction Apathetic hyperthyroidism is a rare presentation of hyperthyroidism described in the elderly population. It is insidious in onset and symptoms are depression, lethargy, and weight loss. The adrenergic symptoms of hyperthyroidism are often absent. Furthermore, orbital disease associated apathetic hyperthyroidism has not been reported widely in literature. We present a clinical scenario of apathetic hyperthyroidism associated with orbitopathy. A 64-year-old lady presented with acute onset bilateral periorbital swelling that developed over 2 days. About 3 weeks prior, she began experiencing symptoms of brain fog, lethargy, intermittent nausea, anorexia, and headache. She experienced palpitations with exertion. Review of other systems was negative. Clinical exam: HR 70. Thyroid gland was enlarged, symmetric with no nodules and nontender, bilateral periorbital puffiness and mild conjunctival redness was noted. Lab data was significant for TSH at 0. 03 uIU/mL, elevated FT4 2.5ng/dL, total T3 of 222ng/dL, TSI 4.2 and sed rate of 10. Her thyroid ultrasound indicated hypervascular and heterogenous gland. Our patient was diagnosed with apathetic hyperthyroidism and thyroid eye disease. She was started on propranolol 60mg ER one tablet a day and methimazole 10mg a day. CT scan of the orbit indicated symmetric enlargement of extraocular muscles. Evaluation by the ophthalmologist confirmed the presence of thyroid eye disease with a clinical activity score of 6 out of 7 indicating severe thyroid eye disease and she was a candidate for treatment with teprotumumab. Discussion Apathetic hyperthyroidism was first described by Lahey in 1931, as a state of non-activated hyperthyroidism. It is manifested with gradual onset of apathy, depressed mood, lethargy, nausea, lack of appetite, weight loss, proximal muscle weakness, goiter, atrial fibrillation, and congestive heart failure. It lacks the typical presentation of a hyperadrenergic state of hyperthyroidism which include palpitations, anxiety, tremor, heat intolerance, and diaphoresis. Due to the lack of typical presentation of hyperthyroidism, timely diagnosis and treatment may be challenging. Concomitant occurrence of occult malignancy and hyperglycemia have been reported in case reports. Patients can also transition from apathetic state to hyperadrenergic state of hyperthyroidism and vice versa. Lahey emphasized the risk of unexpected death following surgery in apathetic hyperthyroidism. The absence of adrenergic symptoms could be supported by the theory that these patients are in a relative state of catecholamine deficiency or have a state of end organ catecholamine resistance. Significant orbital involvement in apathetic hyperthyroidism is not widely reported in the literature. Blepharoptosis has been mentioned in association with this form of hyperthyroidism. Our patient presented with acute onset eye symptoms which prompted testing of thyroid function. Further review of symptoms led to a diagnosis of apathetic hyperthyroidism with thyroid eye disease.

ODP023 Adrenal medullary hyperplasia as a differential diagnosis of paroxysmal hypertension: case report

Abstract Background Sporadic Adrenal Medullary hyperplasia (AMH) has been little described in the literature, being most often diagnosed during a genetic syndrome screening or the evaluation of an adrenal incidentaloma. Case report A 51-year-old female patient presented with progressive attacks of tachycardia, headache, nausea and mild paroxysmal hypertension, which started in December 2020. She had a history of smoking and she also presented Takotsubo syndrome in 2018. She denied other comorbidities. In March 2021, she was submitted to laboratorial testing which demonstrated indeterminate plasma fractionated metanephrines by HPLC/MS: metanephrines 0.3 nmol/L (n&amp;lt; 0.5) and normetanephrines 1.2 nmol/L (n&amp;lt; 0.9) and normal serum chromogranin A level: 82 ng/mL (n&amp;lt;93) by Directed Proteomic. Urinary metanephrines were collected in a spot sample after an adrenergic crisis episode, confirming the biochemical diagnosis: metanephrines 255 mcg/g creatinine (n 30-165), normetanephrine 824 mcg/g creatinine (n 105-375), total 1119 mcg/g creatinine (n 150- 510) by HPLC. She was submitted to an abdominal MRI, which showed bilateral adrenal thickening with medullary predominance with hyperintense sign on T2-weighted images. It was also performed a mIBG I 131 spect and a PET-CT DOTATATO-Galium 68 without abnormal uptakes. A genetic panel by NextGeneration Sequence (NGS) was also carried out for pheochromocytoma/paraganglioma (PHEO/PGL) without pathological findings. The patient underwent laparoscopic left adrenalectomy after the use of alpha and beta blockade for one month, with pathological findings suggestive of adrenal medullary hyperplasia. However, she remained symptomatic after surgery. Once again, new urinary metanephrines in a spot sample were collected after an adrenergic crisis: metanephrines 188 mcg/g creatinine (n 30-165), normetanephrines 1229 mcg/g creatinine (n 105-375), total 1417 mcg/g creatinine (n 150-510) by HPLC, which demonstrated remaining cathecolamine excess. She underwent a contralateral laparoscopic adrenalectomy without surgical complications. An anatomopathological analysis was performed in both adrenals, which confirmed bilateral adrenal medullary hyperplasia without associated PHEO. The patient showed improvement in adrenergic symptoms and normalization of plasma fractionated metanephrines levels postoperatively, currently using hydrocortisone and fludrocortisone for the treatment of adrenal insufficiency. Conclusion Adrenal Medullary hyperplasia is a rare condition, which usually presents in a milder form and is often associated with pheochromocytoma or genetic syndromes. However it should be considered in symptomatic patients without a personal or family history of PHEO/PGL, allowing an early diagnosis and treatment. Presentation: No date and time listed

Ca lâm sàng: Cơn cường giao cảm kịch phát sau đột quỵ nhồi máu não cấp

Background: Paroxysmal sympathetic hyperactivity (PSH) may occur after acute ischemic stroke. The clinical presentation of PSH results from increased sympathetic overdrive, including transient paroxysms of tachycardia, hypertension, hyperventilation, hyperthermia, dystonic posturing, and excessive sweating. The exact pathophysiology of PSH, however, remains unclear, and no definitive treatment is available. Case presentation: Herein, the authors report a case of PSH in a female patient who experienced an acute ischemic stroke, for which a good clinical outcome. An 88-year-old woman was admitted to 108 Military Central Hospital. She had a Glasgow Coma Scale (GCS) score of 9 (M4V2E3), right hemiplegia, NIHSS score of 29. At admission, computed tomography revealed a reduction in the density of the left hemisphere, the blood supply area of the left middle cerebral artery (ASPECT 2 points). 12 days after ischemic stroke, the patient was diagnosed with PSH based on PSH-AM score (19 points). Morphine, gabapentin, and baclofen were administered; the drug effect was sufficient. 17 days poststroke, the patient gradually recovered from the adrenergic symptoms of PSH, and head computed tomography performed 12 days after stroke revealed improvement. Ultimately, the patient recovered with GCS score of 12 (M5V3E4), NIHSS score of 16, and lived at home. Conclusion: The outcome of the present case demonstrates that PSH can be reversed if it is identified early and before it becomes irreversible, that is, post the development of hypoxic encephalopathy or hemorrhagic transformation in ischemic stroke. * Keywords: Paroxysmal sympathetic hyperactivity; Acute ischemic stroke.

P048 A young girl with myocardial infarction

Abstract Background/Aims Takayasu arteritis is a chronic granulomatous inflammatory disease of the aorta and its major branches. It is commonly seen in young women but has been reported in children as young as 6 months. Acute myocardial infarction due to coronary artery involvement is a rare presentation in Takayasu’s arteritis. Methods A previously healthy 13-year-old girl presented with a history of five episodes of short lasting exertional tightening type chest pain with adrenergic symptoms over a week which was relieved by rest. She didn't have any history of shortness of breath, limb claudication or constitutional symptoms. However, on two of the occasions she felt faint. She denied any recreational drug use. There was no family history of cardiovascular disease, connective tissue diseases or sudden cardiac death. On examination she was of average build with no peripheral stigmata of connective tissue diseases or hypercholesterolemia. Apart from a tachycardia of 110 bpm, her cardiovascular examination was normal. There was no BP difference between the arms and no audible bruits. All her peripheral pulses were felt. Results Her ECG showed ST segment depressions in V1-V5. High sensitivity Troponin I was strongly positive. An urgent CT coronary angiogram revealed aortitis involving the ascending and arch of the aorta extending to the left subclavian artery with both coronary arteries showing smooth short segment narrowing at the origin. Doppler USS abdomen showed wall thickening of proximal and mid abdominal aorta. Blood investigations showed a leucocytosis of 14,100/µl and thrombocytosis of 532000/µl. Her ESR was raised at 31 mm/hr and CRP was 5 mg/l. While further awaiting investigations she was started on treatment as for non-ST elevation myocardial infarction according to standard guidelines. Screening for tuberculosis and VDRL were negative . A CT aortic angiogram showed wall thickening involving the aortic root, coronary arteries, ascending aorta and the abdominal aorta with involvement of the origins of coeliac axis and the superior mesenteric artery. Appearance were suggestive of Takayasu arteritis Type v. She was pulsed with intravenous methylprednisolone 30 mg/kg for 3 days followed by oral prednisolone 2 mg/kg. As a steroid sparing agent methotrexate was added. Despite treatment, while in hospital she developed chest pain with ECG showing new onset ST elevation in aVR. An urgent coronary angiogram done showed severe LMCA disease for which stenting was done. At this stage Tocilizumab was initiated. A year later she remains well controlled in terms of her disease activity. Conclusion Though rare, Takayasu’s arteritis should be considered in any adolescent female presenting with ischemic type symptoms and positive inflammatory response. High index of suspicion can lead to early imaging and aggressive immunosuppression that will reduce the morbidity and mortality of this disease. Disclosure S. Janagan: None. M.P.M.L. Gunathilaka: None. L. Rajagopala: None. J.D. Jagoda: None.