Severe Hypokalemia Research Articles

Thyrotoxic Periodic Paralysis Precipitated by Viral Hepatitis

Thyrotoxic hypokalaemic Periodic Paralysis (TPP) is a medical emergency usually seen in Grave’s disease, commonly in persons of oriental descent. The present case is a rare first time presentation of TPP in association with acute viral hepatitis A. A 46-yearold female presented with weakness, vomiting, fever, altered sensorium and yellow discolouration for 20 days. She was diagnosed to have hepatic failure due to viral hepatitis A. Her serum potassium level was 1.79 mmol/L. Treatment for hepatic failure and i.v. potassium chloride replacement was started. Sensorium improved but severe hypokalaemia and flaccid paralysis persisted. Thyroid functions were assessed and thyrotoxicosis due to Grave’s disease was diagnosed. Carbimazole and systemic corticosteroids were also started and potassium infusion was continued. Serum potassium became normal only after thyroid hormones reached normal limits followed by improvement in muscle weakness. TPP can be precipitated in conditions of stress, thyroid functions should be assessed in cases of hypokalaemic periodic paralysis even in absence of overt thyroid disease.

JS-NG-8: CASE REPORT: POSSIBLE NEW CHANNELOPATHY ASSOCIATED WITH ELEVATED ALDOSTERONE LEVELS

Background: Potassium channelopathies are associated with prolonged QT cardiac syndrome. New potassium channelopathies in somatic cells, including KCNJ5 mutations, also underlie some forms of primary hyperaldosteronism. We present a first-in-human report of an association between a KCNQ1 loss of function mutation manifesting with life threatening-ventricular tachycardia (VT), severe hypokalemia and presumed hyperaldosteronism. Case Presentation: The patient is a 51-year-old Asian woman known for obesity, Wolff-Parkinson-White post ablation, and recurrent syncopal episodes with polymorphic VT due to prolonged QTc, requiring an implantable cardioverter defibrillator. On initial presentation for syncope, spontaneous hypokalemia was discovered at 3.1 mmol/L. She had multiple hospitalizations for torsade storms and persistent hypokalemia resistant to aggressive potassium supplementation until addition of spironolactone. Investigations revealed a suppressed plasma renin activity < 0.05 ng/L/s, serum aldosterone concentration at 302 pmol/L, and an aldosterone to renin ratio (ARR) > 6040. Repeated ARR was > 10980. Confirmatory saline suppression testing or adrenal vein sampling were not performed due to risk of recurrent VT associated with spironolactone cessation. A comprehensive evaluation for the etiology of this patient's clinical presentation revealed a mutation in the KCNQ1 gene type 1. Literature review: The voltage-dependent K(+) channel responsible for activating delayed K(+) current is composed of pore-forming KCNQ1 and regulatory KCNE1/3 subunits which loss of function predispose to sustained torsade de pointes. Animal models demonstrate that KCNQ1 and KCNE1 mRNAs are expressed in the zona glomerulosa of adrenal glands where potassium channels directly participate in the control of aldosterone production. Although KCNE3 is not expressed in mouse adrenals, its deletion is thought to cause activation of lymphocytes targeting adrenal glands, leading to hyperaldosteronism. Furthermore, administration of spironolactone to KCNE3 knockout mice ameliorates their QT prolongation and predisposition to VT. There have been no case reports of an association between KCNQ1/E1/E3 loss of function and hyperaldosteronism in humans. Conclusion: This raises the question if this mechanism can also be a component of human KCNQ1 and KCNE1/3-linked arrhythmogenesis and hyperaldosteronism. Alternatively, we can further speculate that patients with hyperaldosteronism who develop malignant arrhythmias during hypokalemia are subjects with impaired repolarization reserve as a consequence of variants in genes known to cause long QT syndrome. The variants, leading to subclinical ion channel dysfunction, are typically silent until unmasked by electrolytes abnormalities such as hypokalemia. To our knowledge, this case is a first report of a potentially novel channelopathy associated with hyperaldosteronism in humans. These findings are hypothesis generating and require further investigations.

The imbalance of body electrolytes in HIV patients using highly active antiretroviral therapy

The retrovirus named HIV (Human Immunodeficiency Virus) targets the immune system, which makes it more vulnerable to numerous illnesses and some cancers that healthy immune systems can combat. The worst phase of HIV infection, AIDS (Acquired Immunodeficiency Syndrome), can take a variety of people between one and twenty years to emerge if untreated. HIV is frequently spread by unprotected sexual contact, blood transfusions, mother-to-child transmission, etc. HIV/AIDS symptoms include weight loss, skin problems, achy muscles, recurrent fevers. Prevention of HIV/AIDS includes use of condom, avoidance of exposure to implicated body fluids, HIV prevention programs to stop vertical HIV transmission. Highly active antiretroviral therapy (HAART), which reduces the disease's progression, is the main form of treatment. The HAART's effect on the kidney results in electrolytes imbalance. Electrolytes are substances that, when dissolved in water, transmit electricity and control blood pressure, pH level, neuron and muscle activity, and maintain tissues hydrated. Irregularity in the body's electrolyte content is known as an electrolyte imbalance which can be caused by fluid lose, inadequate water consumption, certain medications. Symptoms of electrolyte imbalances include, irregular heartbeat, fatigue, headaches, confusion, numbness and tingling. Diagnosis of Electrolyte Imbalance in HIV/AIDS Patients are done using kidney function test, pinch test and electrocardiogram. Treatment and management of electrolytes imbalance in HIV/AIDS patients include drinking water, oral rehydration drinks, sodium chloride, intravenous fluids, oral medications and supplements such as gluconate, magnesium oxide, use of dialysis. HAART induced electrolytes imbalance are seen in Tenofovir, which is associated with severe hypokalemia and hypophosphatemia.

Ectopic adrenocorticotrophic hormone syndrome secondary to treatment-related neuroendocrine differentiation of metastatic castrate-resistant prostate cancer.

A 64-year-old man with progressive metastatic castrate-resistant prostate adenocarcinoma presented with recurrent fluid overload, severe hypokalaemia with metabolic alkalosis and loss of glycaemic control. Clinical features were facial plethora, skin bruising and proximal myopathy. Plasma adrenocorticotrophic hormone (ACTH), serum cortisol and 24-h urinary cortisol levels were elevated. Low-dose dexamethasone failed to suppress cortisol. Pituitary MRI was normal and 68Gallium-DOTATATE PET-CT scan showed only features of metastatic prostate cancer. He was diagnosed with ectopic ACTH syndrome secondary to treatment-related neuroendocrine prostate cancer differentiation. Medical management was limited by clinical deterioration, accessibility of medications and cancer progression. Ketoconazole and cabergoline were utilised, but cortisol remained uncontrolled. He succumbed 5 months following diagnosis. Treatment-related neuroendocrine differentiation of prostate adenocarcinoma is a rare cause of ectopic ACTH syndrome. Neuroendocrine differentiation following prostate adenocarcinoma treatment with androgen deprivation has been described. Ectopic adrenocorticotrophic hormone (ACTH) syndrome should be considered where patients with metastatic prostate cancer develop acute electrolyte disturbance or fluid overload. Ketoconazole interferes with adrenal and gonadal steroidogenesis and can be used in ectopic ACTH syndrome, but the impact may be insufficient. Inhibition of gonadal steroidogenesis is favourable in prostate cancer. More data are required to evaluate the use of cabergoline in ectopic ACTH syndrome. Ectopic ACTH syndrome requires prompt management and is challenging in the face of metastatic cancer.

PS-R07-5: A CASE OF PRIMARY ALDOSTERONISM WITH DECLINED GLOMERULAR FILTRATION RATE AFTER ADRENALECTOMY

Background: Primary aldosteronism (PA) has been known to cause organ damage, such as renal and cardiovascular disorders more frequently than essential hypertension (EH), independently of blood pressure. Among renal disorders, albuminuria is more prevalent in patients with PA than EH, while GFR tends to be higher in PA due to glomerular hyperfiltration. Surgical and medical treatments for PA correct these effects of excess aldosterone, which sometimes uncover preexisting chronic kidney disease (CKD). Here we present a case of PA with CKD who manifested decline of glomerular filtration rate (GFR) and overt renal failure following resection of aldosterone-producing adenoma. Case report: A 53-year-old woman was diagnosed with hypertension at the age of 40. Her primary care physician started treatment with a calcium channel blocker (CCB). At the age of 49, she was diagnosed with Sjogren's syndrome (SS). Blood tests revealed severe hypokalemia and eGFR of 50 mL/min/1.73m2, while 24-hr urine collection showed urinary protein of 0.5 g per day. SS was suspected to underlie her renal disease. A renal biopsy was performed, and suggested nephrosclerosis and IgA nephropathy. On the other hand, a 11mm-sized tumor in the left adrenal gland was discovered during the investigation of secondary hypertension. PA was confirmed based on active renin concentration (ARC) of 4.3 pg/mL, plasma aldosterone concentration (PAC) of 342 pg/mL, an aldosterone-to-renin ratio of 79.5 (> 40), and 24-hr urinary aldosterone levels of 15.9 μg per day under the adequate salt-loaded condition. Adrenal venous sampling yielded aldosterone hypersecretion from the left adrenal vein, which indicated aldosterone-producing adenoma. Laparoscopic partial left adrenalectomy was performed and fully normalized ARC, PAC, and urinary aldosterone levels, which indicated biochemical cure of PA. Consequently, blood pressure had been well controlled with a single CCB. However, while her preoperative serum creatine level ranged from 0.9 to 1.1 mg/dL and eGFR was around 40–50 mL/min/1.73m2, her postoperative serum creatine level increased to 1.4 to 1.9 mg/dL and eGFR decreased to 25 to 35 mL/min/1.73m2 on average. In contrast, urinary protein level improved to less than 0.1 g/day, suggesting that post-treatment increase in creatine level indicated unveiling CKD rather than deterioration of renal function. Conclusion: PA treatment may unmask CKD through improving glomerular hyperfiltration from aldosterone excess.

High-dose intravenous hydrocortisone for the treatment of hyperthyroidism: a rare precipitant of thyrotoxicosis periodic paralysis.

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism triggered by precipitants that increase the activity of the sodium-potassium pump in the skeletal muscle. In our case study, a previously healthy 34-year-old male presented to the emergency department with new onset thyrotoxicosis, secondary to Graves' disease. Given the severity of his triiodothyronine (T3) thyrotoxicosis, he was admitted and started on a high dose of beta-blocker, thioamides, and intravenous hydrocortisone. On the second day of his hospitalization, he developed acute flaccid paralysis of his lower extremities. Subsequent stroke workup was negative, and his electrolytes revealed severe hypokalemia and hyperglycemia consistent with TPP. He was treated with potassium and had a complete recovery of his paralysis and hypokalemia within hours. The patient has not had any recurrence since this singular episode in the hospital. This case highlights the scenario where the treatment of hyperthyroidism with high-dose corticosteroids to reduce the conversion of thyroxine to T3 inadvertently resulted in TPP. Clinicians should be aware of this potentially rare but serious consequence of using steroids to manage hyperthyroidism. High-dose steroids used to treat hyperthyroidism in hospitalized patients may rarely precipitate thyrotoxic periodic paralysis (TPP) by inducing hypokalemia and hyperglycemia. TPP should be included in the differential diagnosis for acute flaccid paralysis in hospitalized patients with hyperthyroidism. Since TPP is associated with trans-cellular shifts in potassium instead of total body potassium depletion, conservative repletion of potassium is recommended to avoid rebound hyperkalemia.

Hypokalemia Duration in the First Year Associated with Subsequent Peritoneal Dialysis-Associated Peritonitis: A Multicenter Retrospective Cohort Study.

The association of hypokalemia (LK) with peritoneal dialysis-associated peritonitis (PDAP) risk remains uncertain. Here, we calculated LK duration in the first PD year and evaluated its association with PDAP. A multicenter, retrospective, incident cohort study of 1633 participants was conducted from January 2008 to October 2020 in China. The duration of LK and severe hypokalemia (SLK) was calculated as the total number of months that a patient's serum potassium (SK) level was less than 3.5 or 3.0 mEq/L during the first PD year. The study outcome was the risk of subsequent PDAP started in the second year and later. Cox proportional hazards models and competing risk models were used to assess the association. The subsequent PDAP occurred in 420 (25.7%) participants during a median of 28 months of follow-up. Overall, LK duration in the first year was positively associated with a subsequent PDAP risk (per 3-month increments, adjusted HR, 1.13; 95%CI: 1.05-1.23). After categorization, patients with LK duration longer than 6 months had the highest adjusted HR of 1.53 (p = 0.005 vs. those without LK) for subsequent PDAP risk. A similar trend was also found for SLK duration. In a competing risk model, a similar trend was also observed. None of the variables, including demographic and PD characteristics, diabetes history, and several clinical measurements, significantly modified this association. The causative organisms of PDAP were similar to those previously reported. PD patients with longer LK duration in the first year had a higher subsequent PDAP risk.

Gitelman syndrome diagnosed in the first trimester of pregnancy: a case report and literature review

Abstract Objectives Gitelman syndrome is a rare salt-losing tubulopathy caused by inactivating mutations in the SLC12A3 gene, which is expressed in the distal convoluted tubule and accounts for 5–10% of renal sodium reabsorption. Atypical symptoms and insidious conditions generally delay diagnosis until childhood or even adulthood. Here, we report the case of a 22-year-old Chinese woman who was admitted to our endocrinology department for severe hypokalemia during pregnancy. Case presentation The patient had no specific symptoms but exhibited hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria, hyperreninemia, hyperaldosteronism, and normal blood pressure. Together, these symptoms indicated the clinical diagnosis of Gitelman syndrome, which was confirmed by genetic analysis. Many drugs have limited safety data during early pregnancy, and optimum potassium and magnesium levels are necessary for a successful pregnancy. Conclusions Diagnosis and management of Gitelman syndrome are crucial during pregnancy to ensure the safety of the mother and fetus, especially during the first trimester.

600: RETROSPECTIVE REVIEW OF OUTCOMES IN PEDIATRIC DKA: EARLY VERSUS LATE LONG-ACTING INSULIN

Introduction: In patients with diabetic ketoacidosis (DKA), the American Diabetes Association guidelines support administering long-acting insulin (LAI) at the time of conversion from IV insulin to subcutaneous rapid acting insulin (RAI). Challenges with LAI timing in children may complicate this transition, and LAI may be administered earlier in the DKA course at some institutions. Limited evidence currently exists assessing the value of this early LAI administration while the patients are still in DKA. The purpose of this study was to assess the benefit and safety of early LAI in pediatric DKA. Methods: This single-center retrospective cohort study included patients < 21 years old admitted to the PICU in DKA on an IV insulin drip. Patients meeting inclusion criteria were divided into two groups: early LAI (administered >4 hours prior to conversion to RAI) and late LAI (administered within 2 hours of RAI). Each group was subdivided into DKA severity (moderate vs severe) and new onset vs known diabetes. The primary outcome of the study was to compare the time to DKA resolution, and secondary outcomes evaluated the total IV insulin doses received, and incidence of adverse effects and complications: hypoglycemia, hypokalemia, cerebral edema, and recurrence of DKA. Results: A total of 372 encounters were included in the preliminary assessment (224 in early LAI and 148 in late LAI groups). Baseline characteristics were similar between both groups. The time to DKA resolution was significantly longer (13 vs. 9.6 hours, p< 0.0001) and total dose of IV insulin significantly higher (65.7 vs. 44 units, p=0.0018) in the early LAI group. Rates of mild hypoglycemia and hypokalemia were significantly higher in the early LAI groups (33.5% vs. 21.6%, p=0.0134 and 42.4% vs. 14.2%, p< 0.0001, respectively). The trends in longer duration of DKA and rates of these adverse effects for the early LAI were also seen in the subgroups of new onset patients and with both moderate vs severe DKA. No differences were observed with severe hypoglycemia or hypokalemia, rates of cerebral edema or recurrence of DKA. Conclusions: Use of early LAI in pediatric DKA demonstrated no reduction in the time to resolution of DKA or total IV insulin dose regardless of severity of presentation, and increased risk of mild hypoglycemia and hypokalemia.

224: ALTERNATIVE MANAGEMENT OF DIABETIC KETOACIDOSIS DUE TO REFRACTORY HYPOKALEMIA

Introduction: In a hypoglycemic state, potassium levels are falsely elevated due to potassium shifting extracellularly. Once insulin is initiated, potassium will reverse, returning intracellularly, and leading to a lower potassium level. It is this reason that the standard protocol of diabetic ketoacidosis (DKA) treatment is to not begin insulin until potassium is replenished above a designated level. Below we present a patient with DKA that required treatment outside of standard protocol due to refractory hypokalemia. Description: The patient is a 25-year-old male that presented to the emergency department (ED) with elevated glucose for the past few weeks. He is a type-2 diabetic, controlled with diet and exercise for the last year. Prior to which, he had been on insulin. The only exacerbating factor he can think of is receiving the J&J vaccine around the same time. Other associated symptoms are difficulty breathing, abdominal pain, mild nausea, but no vomiting. In the ED, he was found to be in DKA with labs significant for severe acidosis and critical hypokalemia of 1.6 mmol/L. A central line was placed and potassium repletion began prior to initiating insulin drip, with plan to start insulin once potassium corrected. Following aggressive potassium repletion with up to 40 mEq per hour, it persistently remained below 3 mmol/L. Thus, it was decided to start the patient on the lowest rate of the insulin drip at 0.02 units/kg/hr until his potassium rose above 3.3 mmol/L as per our protocol. While being on the insulin drip, he was also receiving 20 mEq potassium hourly via IV, 40 mEq orally every four hours, with frequent basic metabolic profiles every two hours. The patient remained on the insulin drip for three days until he was finally able to be transitioned to long-acting insulin, 73-hours after admission. Discussion: The treatment of refractory hypokalemia in the setting of DKA is intricate. Insulin, while imperative for the treatment of DKA, can worsen hypokalemia, therefore sequence of medication administration is important in treatment. It is important for providers to recognize and identify the side effects of these treatments. In this case, multidisciplinary rounds were crucial in both monitoring and replacing potassium while on an insulin drip.

294 BEYOND BRUGADA SYNDROME: A COMMON ECG PATTERN IN AN UNCOMMON CLINICAL SCENARIO

Abstract Propofol infusion syndrome (PRIS) is a rare but potentially lethal side effect of propofol. In most cases it shows various combinations of signs such as unexplained metabolic acidosis, rhabdomyolysis, hepatomegaly, renal failure, hypertriglyceridemia, malignant arrhythmia and rapidly progressive cardiac failure. The development of coved ST elevation in the right precordial leads of the electrocardiogram (ECG), similar to that seen in the type I Brugada syndrome may be the first sign of cardiac instability. There is no specific treatment for PRIS. Successful management consists of an early recognition of its signs followed by a prompt propofol infusion termination. We present the case of a 35-year-old male affected by mild hypertension. He was found by his wife during a transitory loss of consciousness episode. He had resulted positive to Sars Cov 2 infection a day before and was symptomatic for fever and myalgia. An ambulance was immediately called and the patient was transferred to the emergency department for a suspected out-of-hospital-cardiac arrest. The initial one-lead ECG performed by the emergency physician was unremarkable. On arrival he was in a coma state but with stable hemodynamics. ECG showed only an asymmetric T wave inversion in V4-V6 leads. The cardiac echocardiogram did not show any major alterations. In the meantime, due to worsening of respiratory function, orotracheal intubation was performed and the patient was sedated with propofol, midazolam and fentanyl. Subsequently, an episode of atrial fibrillation was documented. Amiodarone infusion was started and the patient reverted to sinus rhythm after a few hours. The following day two episodes of Torsade de Pointes during prolonged QTc (660 ms) occurred. These arrhythmias were treated successfully with magnesium sulfate infusion. Blood analysis showed severe hypokalemia that was immediately corrected. After the hemodynamic stabilization the ECG showed a pattern highly resembling the Brugada pattern type 1 in the right precordial leads. Moreover CPK, myoglobin, high sensitivity troponin I levels started to rise, along with creatinine, triglycerides and markers of hepatic injury. Propofol had been administered continuously for eight days, so PRIS was suspected as the primum movens of this clinical scenario. Propofol infusion was immediately interrupted. Thereafter, the patient gradually improved and was extubated. As soon as the patient's hemodynamic conditions allowed it, a coronary CT and a cardiac MRI were performed, but were unremarkable. To further evaluate the case, a flecainide challenge test was performed, but no significant ECG change was induced. Nonetheless, given both the history of ventricular arrhythmia, the young age of the patient and the unexplained transitory loss of consciousness a subcutaneous defibrillator was implanted as a form of secondary prevention.

Management of hypokalemia in patients with thyrotoxicosis periodic paralysis in Soetomo general hospital: A case report

The prevalence of Graves' disease varies widely between 21 and 80% of all cases of hyperthyroidism. Research conducted in 2018 at the Dr. Soetomo Regional General Hospital Surabaya found Graves' disease in as many as 66.7% of all cases of hyperthyroidism. Thyrotoxicosis Periodic Paralysis (TPP) is a disorder characterized by reversible muscle weakness and paralysis, accompanied by hypokalemia, which usually accompanies hyperthyroidism, mostly caused by Graves' disease. Management of severe hypokalemia in TPP is challenging. male, 29 years patient complained that both lower legs felt weak in the last 6 hours before coming to the hospital emergency department. The patient was diagnosed with hyperthyroidism in 2018. A fine tremor was found, and the patient was admitted to the hospital for 4 days and routinely controlled at the endocrine polyclinic. The underlying disease or causative etiology of thyrotoxicosis must be determined before treatment is conducted. The main concern when performing potassium replacement therapy is the occurrence of rebound hyperkalemia because this hypokalemia condition is caused not by total potassium depletion. The principles of management for thyrotoxicosis periodic paralysis are proper diagnosis, exclusion of other causes of paralysis, and other causes of hypokalemia, slow and gradual correction of hypokalemia, and close and careful clinical monitoring, ECG, and laboratory.

Acute Hypokalemic Paralysis Secondary to Distal Renal Tubular Acidosis Presenting as Guillain Barre Syndrome

A 52 year old female patient presented to emergency casualty with Acute onset quadriparesis with respiratory muscle involvement in. During initial work up Serum electrolytes showed severe hypokalemia. Arterial blood gas showed normal anionic gap metabolic acidosis with positive urine anion gap and urine examination showed alkaline PH in the presence of systemic acidosis. All these findings suggest Distal Tubular Acidosis. Further evaluation revealed strongly positive Anti nuclear antibodies with SS-A also being positive suggesting Sjogren’s Syndrome.

ODP245 Syndrome of Diabetic Ketoalkalosis due to Severe Hypercortisolemia: A Case Series

Abstract Introduction Decompensated diabetes (DM) precoma/comas states are major causes of hospital morbidity and mortality. These commonly include diabetic ketoacidosis (DKA), diabetic hyperglycemic hyperosmolar syndrome (DHHS), diabetic hypoglycemia coma (DHC) and diabetic lactic acidosis (DLA). Another less common decompensated DM precoma/coma state to be aware of is Diabetic ketoalkalosis (DKAL). We present two cases of DKAL due to severe hypercortisolemia (HCS) from ectopic Cushing's syndrome (ECS). Clinical cases; Case 1 was a 61 yr old AA woman with suboptimally managed type 2 DM who had been admitted for DKA 2 months before. She had marked hyperglycemia (blood glucose &amp;gt;500mg/dl) on repeated measures (RMs) and chronic diarrhea with tachypnea and altered mental status (AMS). She had heavy tobacco abuse and work up revealed a right lung mass biopsy of which showed a malignant neuroendocrine tumor presumed to be malignant bronchial carcinoid. Her initial lab tests showed marked metabolic alkalosis with severe hypokalemia and elevated anion gap, ketonemia and ketonuria. Presentation was typical of DKAL and subsequent work up showed ECS with serum cortisol &amp;gt; 110 (n; 4.3-22.4ug/dl) on RMs and serum ACTH 377-480 (n; 0/46pg/ml). Her metabolic derangements were corrected and HCS managed with mild impact using ketaconazole and octreotide but she died within a month of admission with intractable septic shock despite parenteral antimicrobials. Case 2 was a 46 yr old Hispanic man with heavy tobacco abuse admitted on account of AMS, tachypnea and upper abdominal pain. He had severe hyperglycemia (blood glucose &amp;gt;750mg/dl on RMs) though not known to have DM. HBA1c was &amp;gt; 15% indicating undiagnosed DM. Initial lab tests showed marked respiratory and metabolic alkalosis, marked hyponatremia, hypokalemia, hypo-osmolality but with ketonemia and ketonuria. Presentation was consistent with DKAL. Work up showed a left lung lesion with pleural effusion shown to be small cell carcinoma of the lung. He was also found to have profound HCS (serum cortisol &amp;gt;150ug/d; on RMs) confirmed due to ECS with serum ACTH, 1500-1800pg/ml. Metabolic derangements were corrected and he was started on ketoconazole and etomidate to correct HCS with little response. Elective bilateral adrenalectomy was planned but patient abruptly discharged himself AMA. He was readmitted ∼ 3 weeks later with a massive pulmonary embolism and acute respiratory failure from which he never recovered. He died ∼ 2 months later. Discussion + Conclusions DKAL is an uncommon but important potential acute presentation of decompensated DM. Early recognition and management requires close attention to the acid-base and electrolyte derangements that often accompany it. Among the many possible causes of DKAL severe HCS is an important possibility to be consider. Rapid diagnosis and institution of aggressive therapy of HCS is crucial as it often has high morbidity and mortality potential. Presentation: No date and time listed

ODP621 Hypertension with Hypokalemia in Pregnancy? Think "Cushing's Syndrome"

Abstract Introduction Pregnancy is uncommon in women with Cushing's syndrome (CS) as hypercortisolism and hyperandrogenism suppress gonadotropins leading to anovulatory cycles. Such pregnancies carry a higher rate of fetal morbidity and mortality. Therefore, it is of paramount importance to diagnose and treat CS in pregnancy. The diagnosis of CS in pregnancy is challenging due to the physiologic changes of the hypothalamic-pituitary-adrenal axis during pregnancy. We describe a case of CS in pregnancy that was a successfully treated with surgery. Case description An asymptomatic 39-year-old pregnant female patient with gestational hypertension was admitted to the hospital after she was found to have uncontrolled hypertension (while on 3 anti-hypertensive medications) and severe hypokalemia with potassium level of 2.8 mEq/L. She was 14 weeks pregnant at the time. She has a family history of primary aldosteronism in her father successfully treated with adrenalectomy at the age of 60. Her work up included blood work (done at 13: 42) which showed sodium level of 146 mEq/L, plasma aldosterone concentration of &amp;lt;1 ng/dL, plasma renin activity of 1.0 ng/mL/hr, total testosterone of 46 ng/dL, DHEA-S of 31 mcg/dL, plasma metanephrines &amp;lt;50 pg/mL, 24-hour urine metanephrines 70 ug, 24-hour urine normetanephrines 164 ug/24 hrs, ACTH 5 pg/mL, and cortisol 28.2 mcg/dl. MRI abdomen without contrast showed a 3.5×3.0×4.2 cm left adrenal nodule consistent with adenoma. Patient was diagnosed with non-ACTH dependent subclinical CS based on her elevated cortisol and low ACTH levels. She underwent left laparoscopic adrenalectomy and was discharged on oral hydrocortisone. Pathology showed adrenal cortical adenoma. Her blood pressure normalized shortly after surgery and her anti-hypertensive medications were stopped. Discussion Pregnancy is associated with multiple changes in the hypothalamic-pituitary-adrenal axis making diagnosis of CS in pregnancy challenging. Those changes include increased Cortisol binding globulin production leading to increased serum cortisol level and lack of suppression with Dexamethasone beside placental production of CRH and ACTH, all leading to increased salivary and urinary free cortisol levels, as well. This case of hypertension and hypokalemia in pregnancy presented a diagnostic dilemma. Furthermore, the clinical presentation was confounded by a family history of primary aldosteronism. However, the low serum ACTH level highly suggested the diagnosis of CS. Unfortunately, there are no pregnancy-specific guidelines for the diagnosis of CS. The dexamethasone suppression test is not recommended as it often yields false-positive results. The use of urinary free cortisol and late-night salivary cortisol is recommended, and the diagnosis is established when results are three to four times the upper limit of normal. ACTH levels &amp;lt;10 pg/ml are suggestive of adrenal CS. MRI without contrast can be used for imaging. To date, there have been over 260 reported cases of CS in pregnancy with more than half of them being. Presentation: No date and time listed

ODP477 Ectopic Cushing's Syndrome Due to Medullary Thyroid Cancer

Abstract Introduction Medullary thyroid carcinoma (MTC) is an uncommon aetiology of ectopic ACTH syndrome. Ectopic ACTH secretion has been reported in 0.7% of MTCs; it usually occurs in metastatic MTC and has been associated with increased mortality. Case presentation A 65-year-old male with a history of diabetes, hypertension and schizophrenia presented with confusion and upper respiratory symptoms. Initial evaluation showed hypokalaemia, hypocalcaemia, hypomagnesaemia and metabolic alkalosis with random plasma cortisol of 1045 nmol/L. He did not have any cushingoid features on examination. Clubbing of the fingers and proximal myopathy were noted on examination. 24-hour urinary free cortisol was elevated at 3536 nmol/day [NR 50-270] and midnight plasma cortisol was high 856 nmol/L. Low-dose dexamethasone suppression testing showed a cortisol of 1195 nmol/L at baseline and 983 nmol/L [NR &amp;lt;50] at 48 hours. ACTH was significantly elevated at 275 ng/. Plasma metanephrines were normal. A pituitary MRI was normal and an adrenal CT showed bilateral hyperplastic adrenal glands with no discrete nodules. FDG PET/CT revealed nodularity in both upper lung lobes and 2 cm metabolically active left supraclavicular lymph node. He was started on potassium chloride, calcium carbonate with Ergocalciferol. Hypokalaemia worsened in spite of potassium replacement. He was commenced on Metyrapone and prednisolone as a block-and-replace. Ketoconazole has been avoided due to derangement of liver biochemistry. Normalisation of cortisol led to resolution of his confusion. Rivaroxaban 10mg once daily was started as DVT prophylaxis. Eplerenone was added for better control of the hypokalaemia. He underwent ultrasound-guided biopsy of his supraclavicular/cervical mass which showed findings consistent with metastatic MTC. Calcitonin was significantly elevated at 28,400 ng/L(NR &amp;lt;11.8) and CEA level was 340ug/L (NR 0-5). Cytogenetics showed a somatic RET mutation. An attempted total thyroidectomy had to be abandoned as his disease was found to be too locally advanced with fixed area of tumour in the left root of the neck encasing the great vessels and extending down into the sternum. He was commenced on Cabozantinib with plan to re-explore total thyroidectomy option if the tumour shrinks. At follow up, biochemical and radiological assessment showed good response to treatment. The patient remains in good physical condition and is continuing treatment for his mental health problems. Conclusion MTC may infrequently present with paraneoplastic syndrome such as ectopic ACTH secretion. A high index of clinical suspicion is essential in diagnosing atypical cases of Cushing syndrome presenting with rapidly progressive clinical features of hypercortisolaemia, severe hypokalaemia, normal or high ACTH in the absence of a pituitary lesion on imaging. Targeted therapy such as Tyrosine kinase inhibitors (TKI) or selective RET inhibitors have led to improved treatment outcomes. Presentation: No date and time listed

ODP442 Posaconazole-induced Pseudohyperaldosteronism (PIPH) in Patient Treated for Acute Myeloid Leukemia

Abstract Case summary A 62-year-old Caucasian female presented to our institute with mechanical fall complicated by cervical spine fracture. She had known history of essential hypertension and recently diagnosed acute myeloid leukemia with myelodysplasia related changes (AML-MRC). She had undergone induction chemotherapy with combination of daunorubicin and cytarabine, resulting in chemotherapy induced prolonged pancytopenia. She was initially started on primary prophylaxis with fluconazole, but was switched to voriconazole due to persistent neutropenia. Patient then developed bilateral upper and lower extremity erythematous popular rash on voriconazole. Decision at that time was to switch to Posaconazole 300 mg orally daily. Three months after initiating Posaconazole, patient presented with severe hypokalemia (2.1 mmol/L) and hypertension. Previously her blood pressure was under control with amlodipine, lisinopril and nadolol. Hypokalemia persisted despite aggressive oral and intermittent intravenous potassium replacement therapy. No sources of potassium loss were identified on clinical history. She had persistently elevated blood pressure (systolic blood pressure of 150-180 mmHg) despite being on her home anti-hypertensive regimen. Further work up showed undetectable plasma aldosterone of less than 1 ng/dL (normal &amp;lt; 28 ng/dL) and plasma renin activity level was suppressed to 0.14 ng/mL/h (normal 0.25–5.82 ng/mL/h) with concurrent potassium of 2.9 mmol/L (normal 3.5-5.3 mmol/l). Diagnosis of pseudohyperaldosteronism was made at this time. Posaconazole gradually stopped and eplerenone 25 mg daily was commenced to selectively block mineralocorticoids receptors to control blood pressure. Subsequently hypokalemia resolved after stopping posaconazole and blood pressure normalized with her original home anti-hypertensive regimen. Eplerenone was eventually stopped. Her potassium level and blood pressure remained normal 2 weeks after discharge. Discussion Recent studies have shown the presentation of hypokalemia and hypertension in nearly a quarter of patients on Posaconazole. The clinical presentation of hypokalemia and hypertension with apparent mineralocorticoid excess was evident in our patient and was consistent with posaconazole-induced pseudohyperaldosteronism (PIPH). Posaconazole interference with the steroid synthesis pathway has been described in various literature. Both cortisol and aldosterone activate aldosterone receptors. In the case of posaconazole, excess mineralocorticoid effect is due to relative selectivity of posaconazole for 11ΒHSD2 inhibition. 11ΒHSD2 inhibition causes increased serum cortisol level by preventing the conversion to inactive cortisone. The high cortisol level cross-reacts with the mineralocorticoid receptor leading to apparent mineralocorticoid excess with low serum aldosterone level and low plasma renin activity. The degree of pseudohyperaldosterone effect on systolic blood pressure and hypokalemia has been strongly associated with serum posaconazole level in some studies. Our patient had resolution of uncontrolled hypertension and hypokalemia after withholding the posaconazole and short term treatment with selective mineralocorticoid receptor blocker. Conclusion Early diagnosis and to allow for early intervention with cessation of the offending agent or treatment with mineralocorticoid antagonists, such as spironolactone and eplerenone is necessary in PIPH. Presentation: No date and time listed

PMON87 A Case of Mineralocorticoid Induced Hypertension Secondary to Cushing's Syndrome

Abstract We present a case of mineralocorticoid induced hypertension secondary to Cushing's syndrome in a hospitalized patient with uncontrolled hypertension and hypokalemia. A 78-year-old female with hypertension, hypothyroidism, and known pituitary (1.6 cm) and left-sided adrenal mass (2.9 cm) since 2012, presented to the hospital with altered mental status and rectal bleeding with hemoglobin of 5.8 g/dL. On arrival, she was afebrile, blood pressure 87/51 mm Hg, pulse 95 bpm, and saturating at 97% on room air. The patient was altered and had bloody stool positive for fecal occult blood test, with the rest of the examination being normal. Her hospital course was complicated by uncontrolled hypertension (on lisinopril and amlodipine) and severe hypokalemia. During her hospital stay, her potassium ranged 2-2.5 mEq/L (3.5-5.1) and she had persistent metabolic alkalosis. Aldosterone &amp;lt;1 ng/dL, plasma renin activity (PRA) 0.38 ng/mL/hr and aldosterone/PRA ratio 2.6 (0.9-28.9). Thyroid function: TSH 0.709 uIU/mL (0.30-5.00), free T4 1.4 ng/dL (0.7-1.7). Catecholamines were not significantly elevated: free metanephrines &amp;lt;25 pg/mL (&amp;lt;57), normetanephrines 158 pg/mL (&amp;lt;148). Further investigation revealed severe hypercortisolism: 24-hour urinary free cortisol 422 mcg/24 hours, cortisol AM 29.2 mcg/dL (3.7-19.4), and cortisol 31 mcg/dL with 1 mg dexamethasone suppression test. Late-night salivary cortisol was not obtained. ACTH was 138 pg/mL (9-46) and cortisol level after 8 mg dexamethasone suppression test was elevated at 17.2 ug/dL. MRI brain showed a pituitary macroadenoma sized 1.6×1.4×1.1 cm, stable since 2012. CT abdomen showed a left adrenal nodule measuring 2.3×3.3×2.8 cm. Further workup revealed free testosterone 10.6 pg/mL(0.2-3.7), total testosterone 22 ng/dL (2-45), DHEA-Sulfate 533 mcg/dL (45-430), IGF-1 81 ng/mL(34-245), Human growth hormone 0.2 ng/mL (&amp;lt;10), FSH 0.3 mIU/mL, LH 0.1 mIU/mL, prolactin 18.9 ng/mL(0-29), and deoxycorticosterone &amp;lt;16 ng/dL (&amp;lt;16). Given the patient's clinical presentation (rectal bleeding, altered mental status) with associated laboratory abnormalities, the plausible explanation for her hypertension and hypokalemia was ACTH-mediated hypercortisolism resulting in increased mineralocorticoid activity. Other possible etiologies considered were syndrome of apparent mineralocorticoid excess, Liddle's syndrome, deoxycorticosterone tumors, and exogenous intake of steroids. The patient was started on spironolactone and amiloride, both blood pressure and potassium levels improved. A decision was made to proceed with inferior petrosal sinus sampling, to determine the exact source of ACTH, however, prior to completion patient developed COVID pneumonia and expired. Our case highlights the importance of considering hypercortisolism in patients presenting with hypertension, hypokalemia, and metabolic alkalosis with suppressed aldosterone and renin levels. Furthermore, Cushing's syndrome should be a consideration even in the absence of classic cushingoid features as in this patient. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PSAT203 Acquired Resistance to Medical Management in a Patient with Parathyroid Crisis due to a Parathyroid Adenoma

Abstract Introduction Parathyroid crisis is a characterized by severe hypercalcemia associated with multiple organ dysfunction. Case 90 years-old Caucasian man was hospitalized for progressive confusion over 3 days and was noted to have a critically elevated serum calcium (S Ca) 16.8 mg/dl (8.6-10.0) with albumin 4.1 mg/dl. Patient had a good functional status prior to the hospitalization. On further evaluation, patient was diagnosed with primary hyperparathyroidism with a high suspicion for parathyroid cancer, with labs showing a markedly elevated PTH 1183 pg/ml (15-65) and ionized Ca &amp;gt;1.85 mmol/L (1.15-1.35). Of note, S Ca was normal at 9.5 mg/dl (no albumin) approximately 20 months prior to the hospitalization. Parathyroid ultrasound showed a 1.8×1.7×1.1 cm left superior parathyroid mass with ill-defined margins. Sestamibi scan confirmed a left superior parathyroid mass. CT neck/chest/abdomen/pelvis with contrast showed a 1.8×1.1×1.9 cm left superior parathyroid mass with enhancement and punctate non-obstructing right kidney stones. Hypercalcemia responded extremely well to medical management (calcitonin, 4 mg of zoledronic acid infusion and 13 days of cinacalcet 30 mg daily) resulting in normalization of S Ca. However, patient required a prolonged hospitalization for aspiration pneumonia and severe electrolyte disturbances. Since patient was not a good surgical candidate and hypercalcemia resolved on medical management, patient was discharged to a rehab facility with the plan to pursue parathyroidectomy as outpatient. S Ca worsened progressively and was 10.4 mg/dl (albumin 3.6 mg/dl) with PTH 130 pg/ml on day 15 after discharge. Cinacalcet was added when hypercalcemia further worsened to 11.7 mg/dl (albumin 3.6 mg/dl) on day 38. However, patient was hospitalized again for altered mental status, recurrent severe hypercalcemia (S Ca 15.7 mg/dl, albumin 3.8 mg/dl, PTH 1299 pg/ml), hypokalemia and urinary tract infection. In the ED, patient developed complete heart block that responded to atropine. Interestingly, hypercalcemia was recalcitrant to aggressive medical management during this hospitalization (zoledronic acid 4 mg infusion, calcitonin IM- increased up to 6 mg IM q6hr, cinacalcet- increased to 60 mg PO BID and IV fluid resuscitation with furosemide as needed). S Ca was 14.2 mg/dl (albumin 3.4 mg/dl) on day 8 of hospitalization. Therefore, urgent left superior parathyroidectomy was performed. Surgical pathology revealed a 3.1 gm parathyroid adenoma. Post-operative day#1 labs showed normal PTH 17.3 pg/ml, S Ca 12.1 mg/dl and albumin 3.2 mg/dl. S Ca and patient's mental status normalized over next few days. Discussion Urgent parathyroidectomy has shown to have excellent outcomes in patients with parathyroid crisis. Our patient's severe hypercalcemia initially responded excellently to medical management but recurred within few weeks and developed resistance to medical management. Our case highlights the importance of performing surgical exploration as soon as possible after initial medical stabilization in patients with parathyroid crisis. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

LBSUN345 Diagnostic Dilemma In A Case of Vipoma

Abstract Introduction Vasoactive intestinal polypeptide secreting tumors (VIPoma) are rare neuroendocrine tumors that have nonspecific features of watery diarrhea, hypokalemia and achlorhydria. With an incidence of 1 in a million per year, it is diagnosed based on serum VIP levels (&amp;gt;75pg/ml) followed by imaging studies to localize the tumor and look for metastases. We present a challenging case of VIPoma that was successfully managed with surgical exploration despite classic yet nonspecific clinical features and poor evidence on imaging. Case Discussion A 78-year-old Caucasian woman presented with persistent secretory diarrhea for 2 months. Past medical history was significant for intractable peptic ulcer disease and Billroth II reconstruction. Laboratory investigations showed severe hypokalemia (2.1 mmol/l), serum bicarbonate 13 and chloride 117. Blood glucose and calcium levels were within normal range. Stool studies confirmed secretory diarrhea with stool osmotic gap of 10 mOsm/kg but no growth. Serum gastrin, parathyroid hormone, chromogranin and urine 24-hour 5-hydroxy indoleacetic acid (5-HIAA) levels were within normal limits. Plasma VIP levels were elevated at 79 pg/mL. Computed tomography of the abdomen and pelvis did not show any evidence of pancreatic tumor or biliary ductal dilatation. Endoscopic ultrasound could not fully evaluate the head of pancreas but showed unremarkable liver, pancreatic body and tail. Capsule endoscopy was unremarkable as well. An octreotide scan, however, demonstrated increased activity near the head of the pancreas and abdominal aorta. As clinical picture and octreotide scan findings were consistent with VIPoma, operative exploration for possible pancreaticoduodenectomy was pursued. No evidence of palpable or occult metastatic disease was found; but intraoperative color doppler ultrasound localized two lesions less than 1 cm in size at the level of the uncinate process and ampulla. An open pancreaticoduodenectomy was performed and the existing Billroth II anatomy was modified to accommodate pancreatojejunostomy, gastrojejunostomy and choledochojejunstomy. A jejunostomy tube was placed for enteral access. At 2-month post-operative follow-up, the patient had significant improvement of his diarrhea. Radiographic resolution was noted on repeat octreotide scan 4 months following resection. Conclusion VIPomas are usually diagnosed by elevated VIP levels and imaging studies are used to localize the tumor for further treatment. However, this case highlights that in secretory diarrhea with elevated VIP level and no alternate diagnosis, despite negative imaging studies further exploration with octreotide scan is warranted. Surgical exploration may show the site of lesion and appropriate surgical intervention may lead to resolution of VIPoma. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.