Prior Episodes Research Articles

A Case of Recurrent Cerebral Cortical Encephalitis in MOG Antibody-Associated Disease

Objective NA. Background Cerebral cortical encephalitis is a recently recognized syndrome of myelin-oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD), yet most descriptions report single episodes without recurrence. Design/Methods Case report of recurrent cerebral cortical encephalitis in MOGAD. Results This is a case report of a 32-year-old patient who initially developed meningoencephalitis when he was three. He was stable until age 31, when he developed headaches followed by status epilepticus secondary to cerebral cortical encephalitis with accompanying unilateral right temporal cerebral cortical T2-hyperintensity with leptomeningeal enhancement. Cerebrospinal fluid analysis revealed 505 white cells/µl (normal, 0-5) but oligoclonal bands were negative. Over the subsequent two weeks he developed bilateral severe vision loss (20/400 bilaterally) accompanied by bilateral optic disc edema, and MRI orbits showed bilateral anterior segment optic nerve enhancement extending >50% of the length of each nerve, confirming bilateral optic neuritis. MOG-IgG was tested in serum and was positive at a titer of 1:100. He was treated with intravenous methylprednisolone 1 gram daily for 5 days and a subsequent slow oral prednisone taper along with anti-epileptic treatment with levetiracetam, later transitioned to oxcarbazepine, although he later self-discontinued all anti-epileptics. He received maintenance intravenous immunoglobulins (IVIg) 0.4 g/Kg weekly, that was later weaned to 1 g/Kg every 4 weeks. He then developed recurrent cerebral cortical encephalitis 18 months after his prior episode manifesting with seizures and left occipito-temporal T2-hyperintensity and swelling with leptomeningeal enhancement. He was treated with high dose IV steroids, an oral prednisone taper and his IVIg dose was changed back to 0.4 g/Kg once weekly and anti-seizure medications reinitiated. Conclusions Cerebral cortical encephalitis in MOGAD can be recurrent. Close scrutiny of the MRI in patients with MOGAD who develop seizures after prior cerebral cortical encephalitis is important as it can discriminate a recurrent cerebral cortical encephalitis attack from a breakthrough seizure related to prior damage.

Special Circumstances: Agitation with Dementia in Emergency Departments

AbstractBackgroundOlder persons presenting to the emergency department (ED) with agitation have a high prevalence of cognitive impairment which may not have been previously diagnosed.. Agitation in the ED may have many causes, one of the most prominent of which is delirium which occurs in 8–10% of older persons in the ED, and is often not diagnosed till hospital admission . The recently developed IPA algorithm for diagnosis and management of agitation in dementia is applicable to the ED environment particularly as it applies to delirium.MethodThis abstract describes the application of the IPA algorithm to the ED settingResultsOne of the major challenges of agitation management in the ED is taking a history, particularly for patients coming from a LTC environment. LTC staff can readily describe the index episode of agitation, but what is often missing is understanding the timeframe of agitation, including provoking and mitigating factors and any history of prior episodes and their causes. This makes it difficult to know if cognitive impairment is chronic or acute, the hallmark of delirium. A systematic approach to diagnosis and management is required. EDs are making increased use of well‐validated delirium screening assessments such as the Confusion Assessment Method (CAM). Other useful tools for management of delirium in the ED include ADEPT and the Delirium Triage Screen (DTS).Nonpharmacologic strategies have an important role in treatment of agitated patients in the ED. Interventions to prevent or reduce delirium in acute care environments are becoming increasingly effective: a recent Cochrane review supported the effectiveness of interventions including re‐orientation (including use of familiar objects), cognitive stimulation, sleep hygiene, attention to nutrition and hydration, oxygenation, medication review, assessment of mood, and bowel and bladder care. EDs increasingly incorporate these interventions into routine care including the use of delirium‐focused order sets and deployment of geriatric management teams which have been shown to reduce 30‐day ED readmission rates.ConclusionFurther studies should integrate diagnostic workflows and algorithms for behavioral interventions developed for both agitation and delirium management in the ED.

Recurrent Guillain-Barré syndrome presenting as pharyngeal-cervical-brachial variant with three species of ganglioside antibodies:case report

BackgroundGuillain-Barré syndrome (GBS) is generally considered to be monophasic, and recurrent GBS (RGBS) is very rare. Pharyngeal-cervical-brachial (PCB) is a less common variant of GBS. There have been no cases reported describing RGBS showing different phenotype presenting as PCB variant with three species of ganglioside antibodies.Case presentation We report a case of a 77-year-old female patient with GT1a, GD1a and sulfatide-seropositive PCB-GBS after prior episode of AMAN-GBS 13 years ago. Our patient showed oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs and partially improved after 5 days of IVIG and physiotherapy.ConclusionThis study reports a rare case characterized as recurrent GBS after a long period, showing different phenotypes in different episodes with three different species of ganglioside antibodies. Further studies are required to obtain better understanding of RGBS and PCB variant.

Study of JAK2 V617F allele Burden By Droplet Digital PCR in BCR-ABL1 Negative Myeloproliferative Neoplasms and Its Implications on Disease Phenotype

JAK2V617F mutation is the most common mutation seen among the BCR-ABL1 negative myeloproliferative neoplasms, the incidence of which is 90-95% in PV, 50-70% in ET and 40-50% in PMF. The discovery of the association of JAK2 V617F mutation with all the above mentioned myeloproliferative neoplasms and MPN-U boggled the minds of many scientists on how a single gene mutation can cause different disorders, and it was later reported that the allele burden determines the clinical phenotype of the disease. With the advent of droplet digital polymerase chain reaction(ddPCR), even a small quantity of JAK2 V617F allele burden could be detected and measured, facilitating an earlier definitive diagnosis and hence early treatment. In addition, with the rise in use of JAK2 inhibitors such as Ruxolitinib for treating high risk patients, the quantification of allele burden could probably play a role in defining treatment guidelines for usage of these drugs. Method: After examination of bone marrow aspirate smears and biopsies in clinically suspected cases of MPN, molecular testing and allele burden quantification of JAK2 V617F mutation was done using ddPCR. The objectives of our study was to correlate the allele burden with the clinical phenotype, it's implication on the symptom burden and hematological parameters and it's correlation with the prognosis of patients. The symptom burden was measured using MPNTEN Score, whereas the prognosis for PV and ET was done based on Prognostic risk score for thrombosis. Prognostication of PMF was done using IPSS and DIPSS. Results: In our study, among 50 patients, 50% (n=25) were diagnosed as PV, 30% (n=15) were diagnosed as Primary myelofibrosis, 16 % as ET (n=8) and 4% (n=2) as MPN-U. The mean allele burden of PV was highest (64.5%), closely followed by PMF at 61.65% and lowest in ET (31.17%). The allele burden showed significant (p<0.05) positive correlation with the symptom burden in patients. It was also found that a significant association between presence of para trabecular megakaryocyte localization and allele burden exists (p value of 0.02). It was observed that a significant correlation exists between previous history of thrombosis with increased allele burden at presentation (p<0.05). However, the allele burden quantification at presentation and prognostic scores of PV, ET and PMF did not show significant correlation. Conclusion: To conclude, our study reports that the allele burden varies according to the clinical phenotype, has a bearing on the symptom burden of patients, has morphological implications and has a strong correlation with prior thrombotic episodes. Follow up of these patients based on remission criteria for PV, ET and PMF are being done and will be presented.

Abstract 11724: Echocardiographic Predictors and Outcomes of Multiple Vegetations in Infective Endocarditis

Introduction: Infective endocarditis (IE) is a life-threatening infection with an annual mortality of 40%. Embolic events reported in up to 80% of patients. Vegetations of &gt; 10 mm size are associated with increased embolic events and poor prognosis. There is a paucity of literature on the association of multiple vegetations with outcomeHypothesis: We aimed to study the echocardiographic (ECHO) features and outcomes associated with the presence of multiple vegetationsMethods: In this retrospective, single-center, case-control study patients diagnosed with IE were recruited from June 2017 to June 2019. A total of 84 patients were diagnosed to have IE, of whom 67 with vegetation were identified. Baseline demographic, clinical, laboratory, and ECHO parameters were reviewed. Outcomes that were studied included recurrent admission, embolic phenomenon, and mortality. Results: Twenty-three (34%) patients were noted to have multiple vegetations, 13 (56.5 %) were male and 10 (43.5%) were female. The mean age of these patients was 50. Eight (35%) had a prior episode of IE. ECHO features of moderate to severe valvular regurgitation (OR:4), presence of pacemaker lead (OR:4.8), impaired LV relaxation, (OR:4), and elevated PASP (OR:2.2) are associated with higher odds of multiple vegetations. Of these moderate to severe valvular regurgitation (p: 0.028), pacemaker lead (p-0.039) and impaired relaxation (p-0.028) were statistically significant. These patients were noted to have an increased association of recurrent admissions (OR:3.6), recurrent bacteremia (OR:2.4), embolic phenomenon (OR:2.5), ICU stay (OR:2.8), hypotension (OR:2.1), surgical intervention (OR:2.8) and device removal (OR:4.8). Of this device removal (p-0.039) and recurrent admissions (0.017) were statistically significantConclusions: This study highlights the associations of echocardiographic predictors and outcomes in patients with IE having multiple vegetations. ECHO features of moderate to severe regurgitation, presence of pacemaker lead, impaired LV relaxation, and elevated PASP and outcomes including recurrent admissions and device removal were found to be associated with multiple vegetations

Abstract 10594: Cardiac Tamponade in a Covid-19 Positive Patient With Idiopathic Capillary Leak Syndrome: A Perilous Combination

Introduction: Clarkson’s disease, more known as idiopathic systemic capillary leak syndrome (SCLS) is a rare condition characterized by hypotensive episodes due to capillary hyperpermeability and can manifest as fluid accumulation in any organ or potential space. This may cause a range of complications: respiratory failure (from pulmonary edema or pleural effusion), mesenteric hypoperfusion (from hepatic congestion and severe intestinal edema), compartment syndrome and rhabdomyolysis, and even shock from hypovolemia or cardiac tamponade. Very few cases of cardiac tamponade in Clarkson's disease and COVID-19 have been reported, and when present, is certainly a serious and life-threatening complication. Case Summary: A 44-year-old female - with prior episodes of angioedema, pleural and pericardial effusions aggravated by menstruation and infection - presented with hypotension and diaphoresis. She was exposed to a COVID patient and eventually tested positive for COVID-19 infection. Persistent hypotension warranted triple inotropic support. Initial laboratories revealed hemoconcentration and hypoalbuminemia. Urinalysis was normal. 2D echocardiography showed normal left ventricular ejection fraction but with large pericardial effusion and signs of cardiac tamponade, hence, she underwent emergency pericardial windowing. She then developed pleural effusion which was drained surgically. Hepatitis, mycoplasmal and Epstein-Barr virus were negative. Direct and indirect Coomb’s test, and Lupus panel were unremarkable. Serum free light chain panel, immunopathology and C1 inhibitor results were non-diagnostic. Patient was managed as a case of idiopathic SCLS or Clarkson's disease. Methylprednisolone pulse therapy (MPPT) and intravenous immunoglobulin (IVIg) were administered. Patient was discharged with tapering doses of oral steroids and scheduled monthly IVIg infusion. Conclusions: Cardiac tamponade is a very rare but life-threatening complication of Clarkson’s disease, and on top of a COVID-19 infection, warrants emergent intervention. The pathogenesis is not yet fully elucidated. Diagnosis of SCLS is challenging thus recurrent pericardial and pleural effusions should raise the clinician’s index of suspicion.

ODP043 Hypokalemia in Autoimmune Polyglandular Syndrome: A Case of Newfound Type I (Distal) Renal Tubular Acidosis

Abstract Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy Type 1 (APECED) is a rare autosomal recessive disorder predisposing to early development of chronic mucosal candidiasis and progressive development of various endocrinopathies, such as Addison's disease, Primary Hypoparathyroidism, and Type 1 Diabetes (T1D). Few cases described in literature regarding renal involvement in this disorder, with most common manifestation being tubulo-interstitial nephritis, however, there is limited evidence regarding presentation of APECED with hypokalemia due to concomitant Type I Renal Tubular Acidosis (RTA). Case of a 47-year-old female with past medical history of APECED Type 1 (consisting of hypoparathyroidism, adrenal insufficiency, and chronic mucocutaneos candidiasis), T1DM on honeymoon, hypothyroidism, and nephrolithiasis presenting to the emergency department complaining of bilateral upper and lower extremity weakness of one week in evolution, but worsening on day of admission. Denies associated constitutional symptoms. Strong positive first-degree relative history for autoimmune diseases. Physical examination: 2/5 strength in all four extremities, deep tendon reflexes grossly intact, no gross sensory or motor deficits. Patient on oral steroid therapy for her Addison's, calcitriol supplementation for hypoparathyroidism, and potassium chloride supplementation since a prior hypokalemic episode 17 years ago. Laboratory workup results remarkable for sodium: 140 mmol/L (N: 135-144 mmol/L), chloride: 110 mmol/L (N: 95-105 mmol/L), potassium: 2. 0 mmol/L (N: 3.4-4.5 mmol/L), central bicarbonate: 18mmol/L (N: 23-31 mmol / L), anion gap: 12 (N: 10-12), glycosylated hemoglobin: 5.2% (N: 4.7-6.2%) and anti-glutamic acid decarboxylase antibodies &amp;gt;25,000 U/mL (N: &amp;lt;5 U/mL). Adrenal insufficiency was ruled out by laboratory findings, but she had normal anion gap metabolic acidosis (NAGMA) and hypokalemia. Urinalysis was negative for urinary tract infection but with pH: 7. 0 (N: 4.5-8. 0). Urine spots, measured to find the cause of her NAGMA, showed urine sodium: 92 mmol/L, urine potassium: 10 mmol/L, urine chloride: 90 mmol/L, and urine anion gap of 12 mEq/L, which is positive and suggests low urinary NH4+, consistent with Type I (Distal) RTA as the main etiology. Patient started on IV potassium replacement, which increased potassium to 3.8 mmol/L, and improved acidotic state, with central bicarbonate of 29mmol/L, and overall symptoms. Patient was discharged home with alkali therapy (Sodium citrate) for Type I (Distal) RTA. RTA is an underrecognized condition that may be inherited or acquired. Diagnosis is difficult but can be verified with a thoughtful workup. RTA should be considered for any patient with otherwise unexplained hyperchloremic metabolic acidosis and may be the presenting manifestation of autoimmune diseases. Regular renal monitoring for any APECED patient should be performed. We present this case as evidence for the coexistence of several different immune-mediated diseases in the clinical context of APECED with an unusual concomitant Type I (Distal) RTA, where there is few documented evidence of association with this disorder. Presentation: No date and time listed

POST COVID RECURRENT FEVER IN A CHILD WITH GENETIC SUSCEPTIBILITY TO FAMILIAL MEDITERRANEAN FEVER

<h3>Introduction</h3> Children and adults with post-acute sequelae of COVID (PASC) sometimes report recurrent fevers. We present the case of 13-year-old male with PASC recurrent fever who was found to have a heterozygous mutation in the gene associated with Familial Mediterranean fever (FMF). <h3>Case Description</h3> Our patient presented with recurrent fever 6 months after acute SARs-CoV-2 infection in January 2022. He endorsed prior episodes of unexplained fever and flushing prior but his recurrent fevers (39 C) post COVID had become more frequent occurring every few days. Laboratory analysis when he was afebrile demonstrated normal serum ESR, CRP, and CMP and normal cytokine levels. Genetic (In-Vitae) panel testing for autoinflammatory syndromes revealed a pathogenic heterozygous missense mutation in MEFV (p.A744S), the gene that controls pyrin production. A trial of colchicine was recommended, and he was referred to pediatric rheumatology. <h3>Discussion</h3> FMF is a genetic disorder that causes recurrent episodes of fever and almost always presents before age 20. Clinically significant FMF gene mutations lead to poorly functional pyrin; a protein that controls inflammation. Most children look and feel well between episodes but some children have such frequent episodes that they do not fully recover. In such cases, daily use of Colchicine should be considered as a safe and effective medication to control fever episodes. This report highlights the utility of genetic testing to identify potential causes of recurrent fever after COVID. Children with PASC related recurrent fevers should have genetic testing for susceptibility to FEF as this is a potentially treatable condition.

A DISCUSSION OF THE REFRACTORY PERIOD IN VENOM IMMUNOTHERAPY

<h3>Introduction</h3> Refractory periods are inconsistently observed phenomena that follow hypersensitivity reactions wherein a reaction is followed by a period of time during which additional reactions cannot be triggered. We discuss a case that challenges this theory. <h3>Case Description</h3> A 56-year-old male with Diabetes Mellitus II, Hypertension, and HIV presented with history of two prior episodes of fire ant stings leading to anaphylaxis. His baseline tryptase level was 6.1 ug/mL and sIgE to fire ant (Solenopsis invicta) was 42.3 kU/L. The patient completed the immunotherapy ultrarush induction protocol using 1:100wt/v Solenopsis invicta whole body extract (WBE) as the maintenance (1:1v/v) concentration. One hundred minutes after completion of the protocol, he developed throat tightness, nasal congestion, diffuse pruritus, and urticaria. Symptoms resolved after two injections of 0.5mL intramuscular epinephrine. One week later, he received 0.2 mL of 1:1 v/v and developed stridor, productive cough, and injected sclera 30 minutes after injection. Symptoms resolved with intramuscular epinephrine. The following week, he received another injection (decreased to 0.5 mL of 1:10 v/v) without incident. He continued weekly buildup to maintenance dose and subsequent monthly injections without additional reactions. <h3>Discussion</h3> In this case, completion of an ultrarush induction protocol and anaphylaxis failed to result in a refractory period of unresponsiveness, allowing for a second anaphylactic reaction to S. invicta WBE one week after desensitization should have been achieved. Additional research is needed to reliably identify patients experiencing a refractory period following an anaphylactic event. This would improve the accuracy of allergy testing and has the potential to make desensitization safer.

A NOVEL IKBKG MUTATION IDENTIFIED IN A PATIENT WITH RECURRENT SKIN LESION

<h3>Introduction</h3> Pathogenic variants in the inhibitory kB kinase g gene (<i>IKBKG</i>), which encodes nuclear factor kB essential modulator (NEMO), have a wide variety of presentations. Patients with NEMO deficiency have varying degrees of ectodermal dysplasia (ED), anhidrosis, and immune deficiency. Herein, we describe the initial presentation of a patient with a novel pathogenic variant in <i>IKBKG</i>. <h3>Case Description</h3> A 6-month-old male with two prior episodes of cellulitis was admitted for fever and right lower extremity swelling. Blood cultures grew <i>Enterobacter cloacae</i> and remained positive despite appropriate antimicrobial coverage. Brain MRI was obtained and demonstrated multiple microhemorrhages concerning for septic emboli. Right extremity biopsy was not consistent with cellulitis. Immunology evaluation (Table 1) was significant for hypogammaglobinemia, abnormal proliferation assays, and diminished NK cell cytotoxicity. Over time, findings of ED became evident including lymphedema, anhidrosis, and delayed tooth eruption with conical incisors. Whole exome sequencing (WES) was obtained but failed to identify an underlying cause. Since findings were concerning for NEMO deficiency, <i>IKBKG</i> sequencing was obtained, which identified a previously unreported duplication in exon 7 c.815_817dup (p.E272dup) in both the patient and his mother. <h3>Discussion</h3> Patients who present with ED and immunodeficiency should raise high clinical suspicion for NEMO deficiency syndrome. This patient also demonstrates how WES may not detect all significant variants. The <i>IKBKG</i> pseudogene<i>, IKBKGP1</i>, limits the accuracy of WES. While findings in NEMO deficiency syndrome can vary, few immune deficiencies are associated with ED, and disease phenotype must remain a consideration in selecting and interpreting genetic testing.

PSUN236 Previously Undiagnosed Hereditary Pancreatitis Presenting as New Onset Type 3c Diabetes Mellitus

Abstract Background Type 3c Diabetes Mellitus (DM3c) or Pancreatogenic Diabetes is defined as diabetes secondary to pancreatic disease. The most common cause of Type 3c Diabetes is chronic pancreatitis, accounting for 80% of cases.1 Hereditary pancreatitis, with mutations such as SPINK1, CFTR, CTRC, is an underdiagnosed cause of chronic pancreatitis leading to Type 3c Diabetes Mellitus.1 The typical disease course for hereditary pancreatitis is recurrent episodes of acute pancreatitis that leads to chronic pancreatitis.1 The average time between hereditary pancreatitis diagnosis and development of DM3c has been shown to be 80 +/- 24 months.1 We present a case of previously undiagnosed hereditary pancreatitis presenting as new onset Type 3c Diabetes Mellitus. Clinical Case A 33-year-old male presents with a two month history of diarrhea, epigastric pain, and weight loss. Vital signs on presentation were blood pressure 105/74, heart rate 59, BMI 13. Physical exam revealed a cachectic appearing male with epigastric tenderness. Lab-work showed blood glucose 819 mg/dL, SOsm 313 mOsm/kg, lipase 21 U/L, bicarbonate 33 mmol/L. A computed tomography (CT) of the abdomen revealed punctate pancreatic parenchymal calcifications, suggestive of chronic pancreatitis. He was admitted to general medicine for management of HHS, where he was treated with IV hydration and insulin therapy. Workup for new onset diabetes revealed a negative anti-GAD Ab &amp;lt;5.0 U/mL, and C peptide level 0.5 ng/mL which suggested β cell dysfunction and decreased endogenous insulin secretion. With these lab values, along with radiographic findings consistent with chronic pancreatitis, the likely driving factor for his diabetes was pancreatic disease and he was diagnosed with DM3c. Severe pancreatic disease was further shown by a reduced fecal elastase level of &amp;lt;50, suggesting exocrine pancreatic insufficiency. Investigating the etiology of his chronic pancreatitis, history revealed no prior episodes of acute pancreatitis or abdominal pain, and no family history of pancreatitis. Workup for common causes of pancreatitis, including alcohol use, hypertriglyceridemia, and gallstones was negative, and genetic workup was pursued. The patient was found to be heterozygous for a SPINK1 mutation, which is a known mutation in hereditary pancreatitis.1 Therefore, hereditary pancreatitis was determined to be the likely cause of his chronic pancreatitis which led to Type 3c Diabetes Mellitus. He was started on daily insulin therapy and pancreatic enzymes for his endocrine and exocrine pancreatic dysfunction respectively, with improvement in symptoms. Conclusion This is a unique case of hereditary pancreatitis presenting as new onset Type 3c Diabetes Mellitus with no prior episodes of acute pancreatitis or established diagnosis of chronic pancreatitis. References Ramalho, G. X., &amp; Dytz, M. G. (2020). Diabetes of the exocrine pancreas related to hereditary pancreatitis, an update. Current Diabetes Reports, 20(6). Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

The perfect storm: Thrombotic thrombocytopenic purpura (TTP) associated with COVID-19, a clinical case series and review.

This is a case series of three patients in our hospital system who developed acquired thrombotic thrombocytopenic purpura (aTTP) after testing positive for COVID-19 infection. Two patients had acute COVID-19 infections, and one had COVID-19 IgG antibodies consistent with prior COVID-19 infection. Twelve additional cases of aTTP after COVID-19 infection were found in the literature. COVID-19 creates alterations in the vWF-ADAMTS-13 axis with reduced ADAMTS-13 in acute illness that may lead those patients who are predisposed into fulminant aTTP. Further genetic studies are necessary to uncover why some patients with COVID-19 can have concurrent aTTP. For those with a prior COVID-19 infection, molecular mimicry with autoantibodies to ADAMTS-13 is likely the predominant trigger, but having an underlying predisposition (prior episode of TTP, genetic predisposition to autoimmune conditions, or breast cancer history) creates an environment that could be a possible trigger for aTTP.

Ultrasonography in Tubo-Ovarian Abscess: A Case Study

Tubo-ovarian abscess (TOA) is one of acute complications of pelvic inflammatory disease that usually occur in woman of childbearing age. Factors associated with TOA are reproductive age, Intra-Uterine Device (IUD) insertion, multiple sexual partners, and a history of a prior episode of PID. The classic presentation of a TOA includes abdominal pain, pelvic mass on examination, fever, and leukocytosis. However, prompt diagnostic evaluation must be undertaken to specify the diagnosis. Tubo-ovarian abscess can be found on imaging with ultrasonography, computed tomography (CT), or magnetic resonance imaging (MRI). Ultrasonography as the cheaper of all could be used as first-line diagnostic modality. This study presents an example of ultrasonography examination of 42-year-old woman presenting with stomach pain and fever. Abdominal ultrasonography was performed and we found abscess formation in adnexa. Emergency surgery was performed and antibiotics were given after surgery. This study showed us how abdominal ultrasound could diagnose tubo-ovarian abscess.

Lithospheric thinning and ignition of a Cordilleran magmatic flare-up: Geochemical and O-Hf isotopic constraints from Cretaceous plutons in southern Korea

Northeast Asian continental margins contain the products of magma emplacement driven by prolonged subduction of the (paleo-)Pacific plate. As observed in many Cordilleran arcs, magmatic evolution in this area was punctuated by high-volume pulses amid background periods. The present study investigates the early evolution of the Cretaceous magmatic flare-up using new and published geochronological, geochemical, and O-Hf isotope data from plutonic rocks in the southern Korean Peninsula. After a long (∼50 m.y.) magmatic hiatus and the development of the Honam Shear Zone through flat-slab subduction, the Cretaceous flare-up began with the intrusion of monzonites, granodiorites, and granites in the inboard Gyeonggi Massif and the intervening Okcheon Belt. Compared to Jurassic granitoids formed during the former flare-up, Albian (∼111 Ma) monzonites found in the Eopyeong area of the Okcheon Belt have distinctly higher zircon εHf(t) (−7.5 ± 1.3) and δ18O (7.78‰ ± 0.25‰) values and lower whole-rock La/Yb and Sr/Y ratios. The voluminous coeval granodiorite and granite plutons in the Gyeonggi Massif are further reduced in Sr/Y and to a lesser extent, in La/Yb, and have higher zircon εHf(t) values (−13 to −19) than the Precambrian basement (ca. −30). These chemical and isotopic features indicate that Early Cretaceous lithospheric thinning, most likely resulting from delamination of tectonically and magmatically overthickened lithospheric keel that was metasomatized during prior subduction episodes, and consequent asthenospheric upwelling played vital roles in igniting the magmatic flare-up. The O-Hf isotopic ranges of synmagmatic zircons from the Albian plutons and their Paleoproterozoic and Jurassic inheritance attest to the involvement of lithospheric mantle and crustal basement in magma generation during this decratonization event. Arc magmatism then migrated trenchward and culminated in the Late Cretaceous, yielding widespread granitoid rocks emplaced at shallow crustal levels. The early Late Cretaceous (94–85 Ma) granites now prevalent in Seoraksan-Woraksan-Sokrisan National Parks are highly silicic and display flat chondrite-normalized rare earth element patterns with deep Eu anomalies. Synmagmatic zircons in these granites mimic their host rock’s chemistry. Delamination-related rejuvenation of crustal protoliths is indicated by zircon εHf(t) values of granites (−6 to −20) that are consistently higher than the Precambrian basement value. Concomitant core-to-rim variation in zircon O-Hf isotopic compositions reflects a typical sequence of crustal assimilation and fresh input into the magma chamber.

WORK HARD OR SMART? A CASE OF EFFORT-RELATED SUBCLAVIAN THROMBOSIS

WORK HARD OR SMART? A CASE OF EFFORT-RELATED SUBCLAVIAN THROMBOSIS

S2686 A Rare but Potentially Fatal Cause of Abdominal Pain: Segmental Arterial Mediolysis

Introduction: Segmental arterial mediolysis (SAM) is a rare disease, defined as a nonatherosclerotic, noninflammatory disruption of the arterial medial layer of a medium- to large-sized artery. We describe a case of a healthy woman with acute onset abdominal pain subsequently found to have spontaneous peritoneal hemorrhage from SAM. Case Description/Methods: A 52-year-old former professional female athlete developed abrupt onset lower abdominal pain with nausea and vomiting. Pain was 10/10 in severity with a sharp and crampy quality. She denied fevers, diarrhea, melena, bloody stool, ill contacts, recent travel, or prior episodes. Past medical history was significant for endometrial polyp, menopause, and iron deficiency. She had no prior surgeries. Family history included colon cancer in her father and ovarian cancer in her mother. Her abdomen was soft with tenderness in the lower quadrants with the remainder of the exam unremarkable. Labs including CBC, BMP, LFT, lipase, beta hCG, troponin, ESR, CRP, serum acetone, urinalysis, and lactic acid were reassuring. CT abdomen and pelvis with and without contrast demonstrated hyperenhancing hepatic lesions suggestive of hemangiomas. Ultrasound of the pelvis with duplex was normal with arterial and venous duplex waveforms to both ovaries. On the second day of hospitalization, she had another episode of abdominal pain with labs demonstrating a hemoglobin drop from 13.7 g/dL to 10.2 g/dL. Repeat CT abdomen pelvis with contrast demonstrated large volume hemorrhagic ascites up to 16 cm in size with concerns for active hemorrhage in the left upper quadrant. Diagnostic angiography was performed by Interventional radiology which did not show active arterial hemorrhage although demonstrated fusiform dilatation of the distal middle colic artery and short segment focal fusiform dilatation of the left colic artery at the splenic flexure suggestive of SAM. She subsequently underwent diagnostic laparoscopy and evacuation of hemoperitoneum with over 1L of unclotted blood evacuated with a mesenteric hematoma noted in the distal transverse and proximal descending mesocolon. The spontaneous peritoneal hemorrhage was thought to be secondary to underlying arterial abnormalities triggered by minor trauma. (Figure) Discussion: Vascular causes of abdominal pain such as segmental arterial mediolysis are uncommon. Awareness of noninflammatory and nonatherosclerotic artery diseases such as SAM need to be recognized since they can be fatal if not treated promptly.Figure 1.: Fig 1 demonstrates a bleeding artery as seen on CT abdomen and pelvis with intravenous contrast. Fig 2 demonstrates the large hemoperitoneum in the left upper quadrant seen on CT abdomen and pelvis.

S2363 Pagetoid Spread of Esophageal Adenocarcinoma

Introduction: Pagetoid spread refers to the “upward spreading” of cancer cells into the upper dermis from below. In the esophagus, it is a rare finding seen with poorly differentiated adenocarcinomas and is associated with deeper invasion. Case Description/Methods: A 75-year-old male with history of diabetes, hypertension, and hyperlipidemia presented to the gastroenterology clinic for dysphagia to solids. He reported that his symptoms started intermittently approximately 3 weeks prior and had gradually progressed. He denied a history of acid reflux, heartburn, or prior episodes of dysphagia. He also reported an unintentional loss of 10 pounds over the past 5 months. His medications included lisinopril, metformin, pioglitazone, and simvastatin. He denied a history of tobacco or alcohol use and had no family history of gastrointestinal malignancy. Physical exam was unremarkable. To further evaluate his dysphagia, an esophagogastroduodenoscopy was performed, revealing a small fungating mass in the distal esophagus near the gastroesophageal junction. Superior to this in the distal and mid-esophagus, there was a long segment of circumferential induration with irregular, reticulated erosions (Figure). Biopsies of the lesion revealed esophageal adenocarcinoma with pagetoid spread into overlying squamous epithelium, arising in Barrett mucosa with high-grade dysplasia. Endoscopic ultrasound was subsequently performed, revealing a mass measuring 12 mm in thickness with sonographic evidence of invasion into the adventitia and one malignant appearing lymph node in the lower paraoesophageal mediastinum (staged T3, N1). Discussion: Upper endoscopy is highly sensitive and specific for diagnosing esophageal cancer. However, in cases where there may not be an obvious fungating tumor, the presence of indurated mucosa with reticular erosions should alert the endoscopist to the presence of malignancy, possibly with pagetoid spread and deep invasion, as was seen in this patient.Figure 1.: Circumferential induration with irregular, reticulated erosions found in the mid esophagus during upper endoscopy.

S363 Successful Treatment of Distal Intestinal Obstruction Syndrome With N-acetylcysteine and Polyethylene Glycol via Colonoscopy

Introduction: Cystic fibrosis (CF) can lead to obstructive gastrointestinal disease, such as meconium ileus in children and distal intestinal obstruction syndrome (DIOS) in adults. DIOS is characterized by the accumulation of inspissated stool and viscous secretions in the terminal ileum and proximal colon, leading to complete or incomplete obstruction. Treatment strategies for DIOS are still essentially empirical as there are no randomized controlled trials to guide therapy. We describe a case of a CF patient with DIOS successfully treated with 4% N-acetylcysteine (NAC) and polyethylene glycol (PEG) administered via colonoscopy. Case Description/Methods: A 46-year-old woman with cystic fibrosis and prior episodes of DIOS presented with several days of abdominal pain and constipation. On exam, her abdomen was distended and diffusely tender to palpation. Abdominal CT showed extensive debris in the distal small bowel with resultant dilation proximally, thickening at the terminal ileum, and a decompressed colon (Fig A). She was initially managed with frequent enemas and oral laxatives including Lubiprostone and PEG via nasogastric tube. Her symptoms worsened and she was taken for urgent colonoscopy. With the patient in supine position, the colonoscope was meticulously advanced to the cecum, traversing several matted, mucoid-adherent folds (Fig B). A mucoid plug was seen extending from the ileocecal (Fig B) valve to the walls of the cecum (Fig C). The mucoid plug was injected with 4% NAC and PEG. The scope was then advanced into the terminal ileum approximately 20 cm to the proximal end of a large, actively dissolving fecalith with administration of NAC and PEG. A CRE 8-9-10 mm balloon was used to sweep the stool from the distal ileum. Following this maneuver, peristalsis was observed with inspissated stool emanating from the distal ileum towards the IC valve. Discussion: Optimal management of DIOS in CF patients remains a challenge. Although most cases are relieved with conservative treatments, select patients will need surgery, which is associated with a higher peri- and postoperative morbidity. However, the administration of 4% N-acetylcysteine and polyethylene glycol via colonoscopy offers a viable remedy that may obviate the need for surgery. Watch the video at https://tinyurl.com/ACGAbstractS363AFigure 1.: (A) Abdominal CT showing extensive debris in the distal small bowel. (B) Matted, mucoid-adherent folds in the colon. (C) A mucoid plug extending from the ileocecal valve to the walls of the cecum.

S2659 Splenic Artery Pseudoaneurysm as a Rare Cause of Upper Gastrointestinal Bleeding

Introduction: Upper gastrointestinal bleeding (UGIB) is one of the most common causes for hospital admission in the US. Common etiologies for UGIB include peptic ulcer disease, erosive esophagitis, varices, and less commonly malignancies. Splenic artery pseudoaneurysm (PSA) is a known complication of pancreatitis. It may be asymptomatic or present with left upper quadrant or epigastric pain, and rarely as hemorrhagic shock. Splenic artery PSA presenting with UGIB is extremely unusual. Case Description/Methods: We present the case of a 57-year-old male with alcohol use disorder complicated by a prior episode of alcoholic pancreatitis, who presented with 3 days of hematemesis, melena, and recurrent syncopal episodes. In the ED his vitals and physical exam were notable for tachycardia, hypotension, and melena. Labs revealed a hemoglobin of 4.2. Non-contrast CT of the abdomen and pelvis showed evidence of blood in the antrum and a 2.0 x 1.6 cm out-pouching from the greater curvature of the stomach which was postulated to be a gastric diverticulum (image 1). Upper endoscopy revealed fresh blood in the antrum and duodenum, as well as a massive adherent clot in the fundus. Since no single site of active bleeding was identified, the patient was started on IV PPI with continued close monitoring in the ICU. He continued to have melena, and increased PRBC requirements. Repeat EGD was performed but was unchanged from prior. A CT angiogram of the abdomen and pelvis demonstrated a 1.5 cm actively bleeding PSA arising from a first divisional branch of the splenic artery communicating with the fundus of the stomach (images 2 & 3). The patient underwent successful coil embolization (image 4). Hemoglobin remained stable throughout the remainder of his admission with no further evidence of bleeding. Discussion: This case demonstrates PSA as a rare cause of UGIB. Lesions are most commonly caused by chronic pancreatitis, pancreaticobiliary surgery, and trauma. This patient’s PSA was likely secondary to his prior episode of alcoholic pancreatitis. Prompt diagnosis of PSA is essential given the high mortality rate associated with rupture. Clinicians should maintain a high index of suspicion for gastrointestinal PSA in patients with a history of pancreatitis who present with UGIB for whom no obvious source of bleeding can be identified on endoscopy.Figure 1.: CT Thx/Abd/Pel demonstrating a 2.0 x 1.6 cm out-pouching from the greater curvature of the stomach (arrow), postulated to be a gastric diverticulum, 2. CT Angiogram Abd/Pel demonstrating the splenic artery PSA (arrow), 3. Fluoroscopic imaging re-demonstrating the splenic artery PSA with extravasation of contrast into the gastric lumen, 4. Splenic Artery PSA after successful coil embolization.

S838 Health-Related Quality of Life in Patients With One Prior Episode of Recurrent Clostridioides difficile Infection (rCDI): Results from the RBX2660 Phase 3 Randomized, Placebo-Controlled rCDI Trial (PUNCH CD3)

S838 Health-Related Quality of Life in Patients With One Prior Episode of Recurrent Clostridioides difficile Infection (rCDI): Results from the RBX2660 Phase 3 Randomized, Placebo-Controlled rCDI Trial (PUNCH CD3)