ACUTE HEPATIC SEQUESTRATION CRISIS: AN OFTEN UNDERDIAGNOSED AND FATAL SYNDROME

SESSION TITLE: Critical Renal and Endocrine Disorders Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Sickle Cell Disease (SCD) is an autosomal recessive disease characterized by an abnormal beta-globin chain of hemoglobin (Hb) that leads to malformed sickled cells with a multitude of downstream microvascular occlusions and anemia. While splenic infarction is by far the most common gastrointestinal (GI) manifestation, vaso-occlusion may occur in the liver, leading to an acute hepatic crisis. Acute hepatic sequestration of sickled erythrocytes is an exceedingly rare manifestation. CASE PRESENTATION: A 43-year-old man with homozygous sickle cell disease complicated by End-Stage renal disease was admitted with generalized malaise, right upper quadrant (RUQ) abdominal pain, nausea and vomiting. He was febrile with a temperature of 38.1°C, hypotensive with a blood pressure of 93/61 mmHg and tachycardic with a heart rate of 120 bpm. He was lethargic and uncomfortable with diffuse abdominal tenderness without guarding. Due to concern for septic shock, blood cultures, COVID PCR and influenza were obtained, and the patient was rapidly transferred to the intensive care unit for closer monitoring. Empiric vancomycin and cefepime were started promptly. The initial hemoglobin level was 6.1mg/dL with a leukocytosis of 31.2 K/CUMM and absolute neutrophil count of 21.8 K/CUMM;total hyperbilirubinemia of 17.45 mg/dL, direct hyperbilirubinemia of 11.46mg/dL and elevated INR at 1.66. Computed tomography of the abdomen and pelvis without contrast showed a known 4 cm cystic lesion of the right hepatic lobe and atrophic kidneys. Duplex flow of the abdomen and pelvis showed no portal vein thrombosis and patent flow in the portal vein and artery. Over the course of several hours, the patient's hemoglobin dropped to 3.8mg/dL with a steep rise in LDH and total bilirubin to 632 U/L and 27.04 mg/dL, respectively consistent with hepatic sequestration crisis. Patient was transfused with two units of packed red blood cells, fluid hydration and initiation of erythrocyte exchange transfusion. Prior to receiving exchange transfusion, the patient experienced rapid clinical deterioration with subsequent pulseless electrical activity. Return of spontaneous circulation was achieved transiently however patient's family at this point opted for palliative measures and the patient passed away shortly thereafter. DISCUSSION: Complications of SCD manifest in multiple organ systems. One of the few acute manifestations, hepatic sequestration crisis, is often unfamiliar to many clinicians and left unrecognized, results in poor clinical outcomes. It is rarely encountered and treatment options with blood and, more importantly, exchange transfusion remains often underutilized. CONCLUSIONS: Acute hepatic sequestration crisis is an often-unrecognized manifestation of SCD in which delay in diagnosis and prompt treatment with exchange and blood transfusions may impart a significant risk of mortality in an already prone patient population. Reference #1: Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: a review World J Gastrointestinal Pathophysiology 2017;8(3): 108-116 Reference #2: Norris W. Acute hepatic sequestration in sickle cell disease. J of the National Medical Association 2004;96: 1235-1239 Reference #3: Praharaj D, Anand A. Sickle Hepatopathy J of Clinical and Experimental Hepatology 2021;11: 82-96 DISCLOSURES: No relevant relationships by Karim Dirani No relevant relationships by Georgiana Marusca No relevant relationships by Aryan Shiari

DEVELOPMENT OF METHEMOGLOBINEMIA IN A PATIENT WITH COVID-19 PNEUMONIA

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: The World Health Organization officially declared COVID-19 as a pandemic in March 2020. Since then, it has caused more than 1.5 million deaths and has affected more than 68 million people worldwide (1). Methemoglobinemia is a very rare and potentially life-threatening condition. Congenital methemoglobinemia is caused by a deficiency in cytochrome b5 reductase (Cyb5R) while acquired is caused by exposure to an oxidizing agent such as chloroquine or dapsone. We present a case of a critically ill patient with COVID-19 pneumonia who developed methemoglobinemia without an identifiable cause. CASE PRESENTATION: A 64-year-old gentleman with past medical history of hypertension presents to the emergency department (ED) with chief complaints of shortness of breath and myalgias for the past 3 days. In the ED, patient was found to tachycardic at 121 beats/minute, tachypneic at 40 breaths/minute, and febrile at 38.6° Celsius. He was found to have elevated COVID-19 labs which included C-reactive protein, lactate dehydrogenase, ferritin, creatine phosphokinase, d-dimer in addition to elevated BUN 53 mg/dL and creatinine 2.92 mg/dL. His COVID-19 PCR resulted positive and was placed on bilevel positive airway pressure (BPAP) for his respiratory distress and transferred to the medical intensive care unit (MICU) for further monitoring. He was started on broad spectrum antibiotics with vancomycin and cefepime along with dexamethasone and remdesivir. Within 24 hours of admission, patient required mechanical ventilation for worsening hypoxemia. Furthermore, his kidney function continued to decline and required continuous renal replacement therapy. His oxygen requirements improved until hospital day 10;where he developed methemoglobinemia measuring 11.8%. Patient was then given methylene blue 1mg/kg and 1.5 grams of ascorbic acid. Hematology was consulted for emergent exchange transfusion as he developed hemolysis but was deemed too unstable and unlikely to benefit. Patient subsequently went into multisystem organ failure and died on hospital day 12. DISCUSSION: To date, there has only been one reported case of COVID-19 complicated by methemoglobinemia without an identifiable cause (2). Our patient tested negative for glucose-6-phosphate dehydrogenase (G6PD) deficiency and a full medication reconciliation was performed without an identifiable cause. However, there have been 10 cases reported that were either associated with use of an oxidizing agent such as hydroxychloroquine or G6PD deficiency (3–6). Therefore, clinicians should be cognizant of the rising cases of methemoglobinemia and COVID-19. CONCLUSIONS: In the setting of COVID-19 pneumonia requiring mechanical ventilation with refractory hypoxemia, clinicians should be mindful that methemoglobinemia may be a contributing factor. Therefore, in the setting of refractory hypoxemia in COVID-19, measuring methemoglobin levels should be considered. REFERENCE #1: 1) Home - Johns Hopkins Coronavirus Resource Center. Accessed December 6, 2020. https://coronavirus.jhu.edu/2) Lopes DV, Lazar Neto F, Marques LC, Lima RBO, Brandão AAGS. Methemoglobinemia and hemolytic anemia after COVID-19 infection without identifiable eliciting drug: A case-report. IDCases. 2021;23:e01013. doi:10.1016/j.idcr.2020.e01013 REFERENCE #2: 3) Faisal H, Bloom A, Gaber AO. Unexplained Methemoglobinemia in Coronavirus Disease 2019: A Case Report. A&A Pract. 2020;14(9):e01287. doi:10.1213/XAA.00000000000012874) Choo SY. Letters to the Editor in Therapeutic Apheresis and Dialysis Rapidly rising methemoglobinemia in a patient with severe COVID-19 treated successfully with red cell exchange transfusion Running Title: Red Cell Exchange for COVID-19 Methemoglobinemia. doi:10.1111/tap.13598 REFERENCE #3: 5) Naymagon L, Berwick S, Kessler A, Lancman G, Gidwani U, Troy K. The emergence of methemoglobinemia amidst the COVID-19 pandemic. Am J Hematol. 2020;95(8):E196-E197. doi:10.1002/ajh.258686) Palmer K, Dick J, French W, Floro L, Ford M. ethemoglobinemia in patient with g6pd deficiency and sars-cov-2 infection. Emerg Infect Dis. 2020;26(9):2279-2281. doi:10.3201/eid2609.202353 DISCLOSURES: No relevant relationships by Paul Nguyen, source=Web Response No relevant relationships by Jonathan Pickos, source=Web Response No relevant relationships by Aryan Shiari, source=Web Response No relevant relationships by Ayman Soubani, source=Web Response

Five cases of angioedema in patients diagnosed with COVID-19: A single-center case series

INTRODUCTION: In this case series, five African American patients admitted with Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) infection or coronavirus 2019 syndrome (COVID-19) manifested signs and symptoms of angioedema. METHODS: All five patients were tested positive for COVID-19. Three of the five patients presented with shortness of breath requiring mechanical ventilation with subsequent prolonged and complicated hospital courses in the intensive care unit. Each developed angioedema of their tongue and lips during their hospital stay without identifying a clear inciting factor. The other two patients presented with angioedema to the emergency department and were discharged after a short observation period and evaluation by an ear, nose, throat (ENT) specialist. None had prior known episodes of angioedema. RESULTS: All five patients with COVID-19 and angioedema were of African American ethnicity and three of the five had severe sequelae, including prolonged ICU hospitalization for acute respiratory distress syndrome and acute renal failure. CONCLUSIONS: To our knowledge, this is the first report of a possible association of angioedema with COVID-19. Clinicians may consider monitoring for angioedema in patients with COVID-19 either early or late in the disease course as it may impact the management of disease. Angioedema (AE) is a potentially life-threatening condition that presents to the emergency department (ED) and frequently requires hospitalization and monitoring in the intensive care unit (ICU)1,2. AE has been has been classified as hereditary or acquired types3 and may be mediated via release of histamine, bradykinin or both. This case series is the first report on the development of AE in patients with the severe acute respiratory syndrome-coronavirus-2 (SARSCoV- 2) or COVID-19 syndrome. While COVID-19 presents with varying clinical symptomatology, some of which were recognized only recently4,5, the natural progression of the disease remains under investigation. Awareness of the many clinical presentations of this novel coronavirus will allow early diagnosis, triage and management of COVID-19 and its complications.