Comparison of Comorbidities between Adults and Children with Glycogen Storage Disease Type Ia (GSDIA)

Objectives: Glycogen Storage Disease Type Ia (GSDIa) is a rare inherited disorder resulting in acute hypoglycemia due to impaired release of glucose from glycogen. Despite dietary management practices to prevent hypoglycemia in patients with GSDIa, complications still occur in children and throughout adulthood. This retrospective cohort study compared the prevalence of complications in adults and children with GSDIa. Method(s): Using ICD-10 diagnosis codes, the IQVIA Pharmetrics Plus database was searched for patients with >=2 GSDI claims (E74.01) from January 2016 through February 2020, with >=12 months continuous enrollment beginning prior to March 2019 (for one year of follow-up before COVID-19), and no inflammatory bowel disease diagnoses (indicative of GSDIb). Complication prevalence in adults and children with GSDIa was summarized descriptively. Result(s): In total, 557 patients with GSDIa were identified (adults, 67%;male, 63%), including 372 adults (median age, 41 years) and 185 children (median age, 7 years). Complications occurring only in adults were atherosclerotic heart disease (8.6%), pulmonary hypertension (3.0%), primary liver cancer (1.9%), dialysis (0.8%), and focal segmental glomerulosclerosis (0.3%). Other complications with the greatest prevalence in adults/children included gout (11.8%/0.5%), insomnia (10.0%/1.1%), osteoarthritis (22.0%/2.7%), severe chronic kidney disease (4.3%/0.5%), malignant neoplasm (10.8%/1.6%), hypertension (49.7%/8.7%), acute kidney failure (15.3%/2.7%), pancreatitis (3.0%/0.5%), gallstones (7.8%/1.6%), benign neoplasm (37.4%/8.1%), hepatocellular adenoma (7.0%/1.6%), neoplasm (41.1%/9.7%), and hyperlipidemia (45.2%/10.8%). Complications with the greatest prevalence in children/adults included poor growth (22.2%/1.9%), gastrostomy (29.7%/3.2%), kidney hypertrophy (2.7%/0.8%), seizure (1.6%/0.5%), hypoglycemia (27.0%/11.3%), hepatomegaly (28.7%/15.9%), kidney transplant (1.6%/1.1%), diarrhea (26.5%/18.6%), nausea and/or vomiting (43.8%/35.8%), acidosis (20.0%/17.2%), and anemia due to enzyme disorders (43.8%/40.6%). Conclusion(s): GSDIa is associated with numerous, potentially serious complications. Compared with children, adults with GSDIa had a greater prevalence of chronic complications, potentially indicating the progressive nature of disease. Children with GSDIa had more acute complications related to suboptimal metabolic control.Copyright © 2023

Syphilis: "The Great Imitator" Presenting as Acute Hepatitis

Introduction: Syphilis is a multi-systemic disease caused by spirochete Treponema pallidum. Very rarely, it can affect the liver and cause hepatitis. Since most cases of hepatitis are caused by viral illnesses, syphilitic hepatitis can be missed. Here, we present a case of syphilitic hepatitis in a 35-year-old male. Case Description/Methods: Patient was a 35-year-old male who presented to the hospital for jaundice and mild intermittent right upper quadrant abdominal pain. His medical history was only significant for alcohol abuse. His last drink was 4 weeks ago. He was sexually active with men. On exam, hepatomegaly, mild tenderness in the right upper quadrant, jaundice, and fine macular rash on both hands and feet were noted. Lab tests revealed an ALT of 965 U/L, AST of 404 U/L, ALP of 1056 U/L, total bilirubin of 9.5 mg/dL, direct bilirubin of 6.5 mg/dL, INR of 0.96, and albumin of 2.0 g/dL. Right upper quadrant ultrasound showed an enlarged liver but was negative for gallstones and hepatic vein thrombosis. MRI of the abdomen showed periportal edema consistent with hepatitis without any gallstones, masses, or common bile duct dilation. HIV viral load and Hepatitis C viral RNA were undetectable. Hepatitis A & B serologies were indicative of prior immunization. Hepatitis E serology and SARS-CoV-2 PCR were negative. Ferritin level was 177 ng/mL. Alpha-1-antitrypsin levels and ceruloplasmin levels were normal. Anti-Smooth muscle antibody titers were slightly elevated at 1:80 (Normal < 1:20). Anti-Mitochondrial antibody levels were also slightly elevated at 47.9 units (Normal < 25 units). RPR titer was 1:32 and fluorescent treponemal antibody test was reactive which confirmed the diagnosis of syphilis. Liver biopsy was then performed which showed presence of mixed inflammatory cells without any granulomas which is consistent with other cases of syphilitic hepatitis. Immunohistochemical stain was negative for treponemes. Patient was treated with penicillin and did have Jarisch-Herxheimer reaction. ALT, AST, ALP, and total bilirubin down trended after treatment. Repeat tests drawn exactly 1 month post treatment showed normal levels of ALT, AST, ALP, and total bilirubin (Figure). Discussion(s): Liver damage can occur in syphilis and can easily be missed because of the non-specific nature of presenting symptoms. In our patient, the fine macular rash on both hands and feet along with history of sexual activity with men prompted us to test for syphilis which ultimately led to diagnosis and treatment in a timely manner. (Figure Presented).

A Case Report of Ivermectin-Induced Liver Failure

Introduction: Ivermectin is an antiparasitic medication that is primarily metabolized by the liver. During the COVID-19 pandemic, researchers demonstrated that Ivermectin successfully inhibited the replication of SARS-COV-2 in vivo, but current research has failed to demonstrate clinical benefit for treatment of COVID-19. Despite this, misinformation campaigns have misled patients to ingest Ivermectin at concentrations meant for domestic animals. Here, we present a case of acute liver failure secondary to the use of Ivermectin. Case Description/Methods: A 61-year-old man with medical history of ischemic cardiomyopathy with last echocardiogram showing ejection fraction at 21%, atrial fibrillation on warfarin for oral anticoagulation, and previously treated Hepatitis C presented with generalized weakness and yellowish discoloration of the skin worsening over the last two weeks. The patient denied significant alcohol use, acetaminophen use, or illicit drugs. He admitted to injecting himself with two doses of weight-based horse ivermectin, for COVID prophylaxis, two weeks prior to his presentation. Physical exam was pertinent for scleral icterus and hepatomegaly with no abdominal tenderness. Initial labs revealed elevated liver chemistries in a mixed pattern (Figure 1). Acute hepatitis panel, HSV, and CMV were negative. Hepatitis C antibodies were positive, but the patient was in sustained virologic response. Full workup for chronic liver disease was unremarkable. Ultrasound revealed hepatosplenomegaly with patent portal and hepatic vasculature. Subsequently, the patient developed hepatic encephalopathy along with his coagulopathy, raising concern for acute hepatic failure. The patient was transferred to the ICU and started on NAcetylcysteine, rifaximin, and supportive care. The patient recovered well and fortunately did not require liver transplant. Discussion(s): While the FDA recommends against the use of Ivermectin for COVID-19, many continue to inappropriately consume it. Ivermectin-induced liver failure is a rare but deadly side effect. Given our patient's rapid onset of symptoms post-self injection of Ivermectin, his liver injury was presumed to be related to Ivermectin. The drug interaction between Ivermectin and warfarin had worsened the patients coagulopathy. Physicians should be aware of the ways Ivermectin overdose may clinically present to avoid delayed treatment. This case demonstrates the detriments of perpetuation of medical misinformation to care.

Acute myeloid leukemia complicated by post COVID-19 multi-system inflammatory syndrome-children

Corona Virus disease 2019 (COVID-19) pandemic has presented a huge challenge to the health care system in terms of magnitude of cases and to pediatric oncology units with varied clinical presentations. Acute myeloid leukemia(AML) is a rare heterogenous cancer of childhood with an induction mortality around 15% in our country due to neutropenic sepsis. Multisystem inflammatory syndrome in children(MIS-C) is an hyperinflammatory syndrome seen 4–6 weeks after COVID-19 infection. COVID infection in some of these children would have gone unnoticed. Here we report a two year eight months old boy diagnosed with AML on induction chemotherapy developed post COVID MIS-C. © 2022

L-Ornithine-L-Aspartate and Intermittent Renal Replacement Therapy in Fulminant Hepatitis A: A Case Report

Hepatitis A is a common viral infection worldwide that is transmitted via the fecal-oral route. Since the introduction of an efficient vaccine, the incidence of infection has decreased but the number of cases has risen due to widespread community outbreaks among unimmunized individuals. Classic symptoms include fever, malaise, dark urine, and jaundice, and are more common in older children and adults. People are often most infectious 14 days prior to and 7 days following the onset of jaundice. We will discuss the case of a young male patient, diagnosed with acute hepatitis A, leading to fulminant hepatitis refractory to conventional therapy and the development of subsequent kidney injury. The medical treatment through the course of hospitalization was challenging and included the use of L-ornithine-L-aspartate and prolonged intermittent hemodialysis, leading to a remarkable outcome. Hepatitis A is usually self-limited and vaccine-preventable;supportive care is often sufficient for treatment, and chronic infection or chronic liver disease rarely develops. However, fulminant hepatitis, although rare, can be very challenging to manage as in the case of our patient.Copyright © 2023 The Author(s).

Granulomatous Interstitial Nephropathy : When the Kidney Spills the Bean

Introduction: Sarcoidosis is a rare granulomatosis. The absence of well-defined criteria for definition and the existence of differential diagnosis makes the positive diagnosis difficult. Method(s): We report a case of sarcoidosis that illustrates the difficulty of this diagnosis in the presence of atypical clinical manifestations and a strong suspicion of tuberculosis. Ultimately, renal histology allowed the positive diagnosis and the response to corticosteroids confirmed it retrospectively. Result(s): Our patient was a 66 years-old female with a history of hypertension who presented with a sensory and motor polyneuropathy a couple of months after a mild COVID-19 pneumonia, hospitalized for exploration of a worsening renal function due to a tubulointerstitial neuropathy (creatinine upon admission at 250 micromol/l, eGFR = 16 ml/min/1,73m2 -MDRD). Kidney biopsy revealed an interstitial infiltrate of monocytes and fibrosis alongside non-necrotic and giant-cell epithelioid interstitial granulomas. Extra-renal signs consisted of the above-mentioned neuropathy, bilateral mediastinal adenopathies with no signs of a pulmonary disease at the bodyscan, a hepatomegaly, splenomegaly, a pleural and pericardial effusion of low abundance, and a peritoneal thickening. Bronchoscopy and bronchoalveolar washing found no evidence for malignancies and screening for mycobacterial infections by polymerase chain reaction was negative. No granulomas were found at the hepatic biopsy. Digestive tract endoscopy and biopsies showed no abnormalities. During hospitalization, the patient presented an episode of acute polyradiculonevritis confirmed by cerebral-spine fluid study and nerve conduction study results. Our patient received intraveinous immunoglobulins (IgIV) with a favorable outcome but relapsed one month later, showing signs of respiratory failure. Upon the second relapse of the chronic polyradiculonevritis and based on the absence of bacteriological and histological evidence for a mycobacterial infection and the results or the renal biopsy, the patient received high-dose corticosteroids alongside a second course of IgIV. The neuropathy regressed totally within a month with a decrease of creatinine level to 140 micromol/l (eGFR = 35ml/min/1,73m2) alongside the polyserositis and organomegaly. The final diagnosis was that of a sarcoidosis with pulmonary and renal involvement. Although the neuropathy could be considered a manifestation of sarcoidosis, its origin was intricated as post-viral original could not be formally excluded. Conclusion(s): The etiological diagnosis for granulomatous interstitial nephropathies can be challenging due to similar clinical presentations and the need to start specific treatments especially in the presence of life-threatening situations and the absence of clear criteria defining sarcoidosis further enhances the level of difficulty. No conflict of interestCopyright © 2023

Liver involvement in patients with COVID-19 infection: A comprehensive overview of diagnostic imaging features.

During the first wave of the pandemic, coronavirus disease 2019 (COVID-19) infection has been considered mainly as a pulmonary infection. However, different clinical and radiological manifestations were observed over time, including involvement of abdominal organs. Nowadays, the liver is considered one of the main affected abdominal organs. Hepatic involvement may be caused by either a direct damage by the virus or an indirect damage related to COVID-19 induced thrombosis or to the use of different drugs. After clinical assessment, radiology plays a key role in the evaluation of liver involvement. Ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) may be used to evaluate liver involvement. US is widely available and it is considered the first-line technique to assess liver involvement in COVID-19 infection, in particular liver steatosis and portal-vein thrombosis. CT and MRI are used as second- and third-line techniques, respectively, considering their higher sensitivity and specificity compared to US for assessment of both parenchyma and vascularization. This review aims to the spectrum of COVID-19 liver involvement and the most common imaging features of COVID-19 liver damage.

Features of Gastrointestinal Disorders in Children with Multisystem Inflammatory Syndrome Associated with Covid-19

Gastrointestinal tract damage is a part of the course of multisystem inflammatory syndrome in children (MVS-D) associated with the new COVID-19 coronavirus infection. According to the results of a retrospective study, gastrointestinal tract damage was detected in 77% of patients with MVS-D and is represented by signs such as abdominal pain, vomiting, diarrhea and peritoneal symptoms. In children with gastrointestinal tract lesions, significant differences were noted in the frequency of occurrence of the following signs: hepatomegaly, splenomegaly, hypotension/shock, as well as conjunctivitis and facial swelling. Among laboratory abnormalities, hypoalbuminemia is more characteristic, but the level of CRP and troponin is higher. The article shows that gastrointestinal tract damage is an important early predictor of the severity of MVS-D.

Multisystem inflammatory syndrome associated with COVID-19 in children: Risk factors of severe life-threatening course of the disease

Background/Purpose: Multisystem inflammatory syndrome in children (MIS-C), associated with COVID-19 infection is a life-threatening condition, required intensive care. The aim of this study was to determine risk factors for severe/life-threatening course of MIS-C. Method(s): The retrospective study included 166 children (99 male, 67 female), aged from 4 months to 17 years (median 8.2 years), who met the WHO criteria for MIS-C. The criterion of severity was the fact of the ICU admission. The analysis of the obtained data was performed using the STATISTICA software package, version 10.0 (StatSoft Inc., USA). Result(s): To assess the factors associated with the severe course of MIS-C, patients were divided into two groups: those who were hospitalized in the ICU (n = 84;50.6%), and those who did not (n = 82;49.4%). Patients with a more severe course of MIS-C were significantly older. They had a high frequency of signs such as rash, edema, hepatomegaly, splenomegaly, neurological and respiratory symptoms. Hypotension/shock and myocardial damage were much more common in patients hospitalized in the ICU. Among the laboratory changes there were significant differences in the levels of hemoglobin, leukocytes and platelets, CRP, creatinine, troponin and D-dimer. The presence of macrophage activation syndrome was higher in patients, admitted in the ICU. Children, required intensive care required high dose corticosteroids and IVIG more often (table 1). FIGURE: 1) The first symptoms of progeria in infancy: scleroderma-like changes in the skin of the lower extremities and stiffness of knee joints at the age of 2 months. 2) Girl at the age of 3 years 5 months. Almost total alopecia with the absence of eyebrows and eyelashes. Pronounced venous pattern in the forehead, nasal bridge and nasolabial triangle. Conclusion(s): MIS-C is potentially a severe life-threatening condition, in which more than half (50.6%) of patients needed the ICU admission. The main factors determining the severity of MIS-C were: cardiovascular, resiratory and central nervous system disorders. It has been found that factors such as hepatomegaly, splenomegaly, D-dimer >2568 ng/ml, troponin >10 pg/ml, make it possible to identify a group of patients with high risk of severe MIS-C who may potentially need hospitalization in the ICU.

Tocilizumab as a promising treatment for MIS-C in JIA: Implications for countries with inaccessible anti-IL1 agents

Background: Multisystem inflammatory syndrome in children (MIS-C), causing high morbidity and mortality, is the hyperinflammatory response following COVID-19 infection (CI). According to the MISC management guideline, Anakinra (anti-IL1) is the preferable agent among other biologic agents: Infliximab, Tocilizumab (TCZ), and baricitinib if the patient is refractory to intravenous immunoglobulin (IVIG) and systemic corticosteroid (CS). However, these are not available in a number of countries, including Thailand. Our case represents refractory MIS-C in a systemic juvenile idiopathic arthritis (SJIA) patient responding well to TCZ. Method(s): Diagnostic investigations, including basic and immunological blood tests, and echocardiography assessment, were conducted. Result(s): A 12-year- old boy has been diagnosed with SJIA since he was 2 years old, according to the presentation of prolonged fever, hepatomegaly, and evanescent rash. CS, cyclosporin-a, and TCZ have been prescribed, and he has been in clinical remission off medication for two years. He experienced acute fever, rash, shortness of breath, nausea and vomiting for few days. Physical examination revealed a febrile boy with respiratory failure, compensated shock, and a generalized persistent maculopapular rash. The other was unremarkable. MIS-C was one of the possible diagnoses according to fever accompanied by more than two systems involved and his previous CI four weeks prior. Laboratory investigation revealed an elevated inflammatory response (Figure 1). The echocardiography was done by an experienced cardiologist with concern for myocardial dysfunction in MIS-C and showed a significant poor ejection fraction of the left ventricle of 42% under dobutamine, milrinone, and norepinephrine. Broad spectrum antibiotics and IVIG (1 g/kg/dose for two days) were initiated. After hemoculture did not report bacteria growth, pulse intravenous methylprednisolone (IVMP) 1000 mg for 3 days was given for the MIS-C treatment. After initial aggressive treatment with IVIG and pulse IVMP, the patient still has a high grade fever with laboratory revealed ongoing elevated inflammatory markers. The other possible causes of fever, such as infection and active SJIA were suspected. Immunological profiles returned with positive SAR-COV2 IgG, negative SAR-COV2 IgM, which confirmed the diagnosis of MIS-C with refractory to IVIG and CS. After multidisciplinary team discussion, TCZ was given. He had neither fever, dyspnea, nor heart failure. His clinical condition gradually improves together with laboratory parameters (Figure 1). Conclusion(s): In conclusion, our case demonstrated TCZ as a potential therapeutic agent in refractory MIS-C patients living in countries with limited access to anti-IL1 agents. The multidisciplinary care team together with prompt management is advisable to the best benefit of the patient. (Figure Presented).

Using HScore for the evaluation of the signs of hemophagocytosis in multisystem inflammatory syndrome associated with covid-19 in children

Background/Purpose: Multisystem inflammatory syndrome associated with COVID-19 in children (MIS-C) is a rare but severe disease associated with coronavirus infection, in which various systems and organs are affected, including the heart, lungs, kidneys, brain, skin, eyes and gastrointestinal tract. One of the most severe features of this disease can be hemophagocytosis. The aim of this study is to assess the features of hemophagocytosis in MIS-C. Method(s): The retrospective study included 166 children (99 male, 67 female), aged from 4 months to 17 years (median 8.2 years), who met the WHO criteria for MIS-C. The analysis of the obtained data was performed using the STATISTICA software package, version 10.0 (StatSoft Inc., USA). Result(s): To study the signs of hemophagocytosis in patients with MIS-C they were divided into 2 equal groups: with HScore<=91 (n = 79) and with a HScore value >91 (n = 79). This division was done, since this value was associated with the severe life-threatening course of MIS-C and need in ICU admission (70.9% vs. 32.3%, P = 0.000002). Patients with HScore > 91 were more likely to have symptoms such as cervical lymphadenopathy (80.6% vs 54.1%, P = 0.0007), red dry cracked lips (63% vs 34.3%, P = 0.0007), face swelling (66.7% vs 34.7%, P = 0.001), hepatomegaly (84.2% vs 43.1%, P = 0.000000), splenomegaly (54.7% vs 43.1%, P = 0.0003), hypotension/shock (63.3% vs 25.3%, P = 0.000002), had higher levels of ESR (47 mm/h vs 34 mm/h, P = 0.0001), CRP (175.5 mg/L vs 125.8 mg/L, P = 0.01), D-dimer (2135 ng/mL vs. 1079 ng/mL, P = 0.0003), but lower levels of fibrinogen (3.1 g/L vs 5.6 g/L, P = 0.000002) erythrocytes (3.6 x 1012/L vs 4.0 x 1012/L, P = 0.000005), hemoglobin (98 g/L vs 112 g/L, P = 0.000000), and a tendency to thrombocytopenia (110 x 109/l vs 192 x 109/L, P = 0.0002) in 63.3% of patients. According to EchoCG data, signs of myocardial (45.5% vs 15.6%, P = 0.00006) and pericardial (45.5% vs 14.3%, P = 0.00002) lesions were more common in patients with HScore > 91. Patients with HScore > 91 more often needed treatment with IVIG (66.2% vs 24%, P = 0.000000), acetylsalicylic acid (65.7% vs. 47.1%, P = 0.027) and biological drugs (9.1% vs. 1.6%, P = 0.061). The average duration of hospitalization was also much longer in patients with HScore > 91 (23 days vs 14 days, P = 0.000000). Also, the identification of clinical and laboratory signs that were more common in the group of patients with HScore > 91 was performed using sensitivity and specificity analysis, and calculation of odds ratio. Results are presented in Table 1. Conclusion(s): Hemophagocytic syndrome is one of the most severe manifestations of MIS-C occuring in 35.4% of patients. It was found that HScore > 91 is associated with such a severe signs of MIS-C as myocarditis, pericarditis, hypotension/shock, and ICU admission. HScore is a simple tool that can also be used to assess the severity of MIS-C and dynamic monitoring.

Gastrointestinal symptoms of COVID-19 in children

Introduction: The COVID-19 pandemic progresses. The clinical manifestation of the disease and the severity of its course vary significantly. There are considerable differences between symptoms of SARS-CoV-2 infection in the child and adult populations. The gastrointestinal (GI) symptoms are an essential element in understanding the pathophysiology of the disease and in drawing conclusions concerning the diagnostic, therapeutic, and epidemiological management of COVID-19. The aim of the study was to characterize the gastroenterological symptoms of COVID-19 in the paediatric population and to find differences in the course of the disease between paediatric patients with and without GI symptoms of COVID-19. Material(s) and Method(s): We report the clinical characteristics of 321 children with COVID-19 (age 0-215 months) hospitalized between March 2020 and April 2021. The following division was used when processing the data: the first wave of cases in Poland lasted from the beginning of the pandemic to June 2020, the 2nd wave September-November 2020, and the 3rd wave February-May 2021. We specifically compared the differences between patients with and without GI symptoms. Result(s): Among all included patients, 95 (29.5%) had GI symptoms - the most common included abdominal pain (15.27%) and diarrhoea (14%). Approximately 3% of patients with GI symptoms required surgical intervention. As the pandemic progressed, GI symptoms were reported with increasing frequency - during the first wave 9%, the second wave 25%, and the third wave 38%. Patients with GI symptoms had more frequent and statistically significantly higher inflammatory parameters. During treatment, GI patients more often required the administration of antibiotics. The most common abdominal ultrasound abnormalities were liver enlargement, a slight amount of free fluid in the peritoneal cavity, and moderately enlarged individual lymph nodes. Conclusion(s): Gastrointestinal symptoms form an image of COVID-19, which is a possible prognostic risk factor for severe course of the disease. Gastrointestinal symptoms should be treated as a possible isolated image of COVID-19. Copyright © 2022 Termedia Publishing House Ltd.. All rights reserved.

Mixed Nephrotic/Nephritic Syndrome in a Pediatric Patient With SARS-CoV-2 Infection

Introduction: Glomerular diseases in children generally present as a variety of findings that include hematuria, proteinuria, edema, and hypertension. Glomerular diseases can be isolated to the kidney or present as a component of a systemic disorder. Emerging reports show that SARS-CoV-2 infection precedes the appearance of various autoimmune diseases. Case Description: Twelve-year-old Hispanic female with history of migraines, obesity, hypercholesterolemia, and NAFLD, who presented with anasarca, fever, and fatigue. On exam, she had hypertension (138/89 mmHg) and anasarca with ascites. Labwork was remarkable for K+ 5.6 mmol/L, Cr 1.07 mg/dL, albumin 2.1 gm/dL, AST 48 IU/L, cholesterol 265 mg/dL, triglycerides 178 mg/dL, CRP 2.6 mg/dL and ESR 80 mm/hr. UA with >500 proteinuria, moderate blood, 100 RBCs, 49 WBCs, and negative leukocyte esterase and nitrites. A rapid strep test and Hepatitis Panel were negative. ANA <1:10, C3 complement level low at 36 mg/dL, C4 normal. Abdominal US showed hepatomegaly, echogenic bilateral kidneys, and a small pleural effusion. SARS-CoV2 antigen test was positive. She was managed with antihypertensives, albumin infusions, and furosemide. She was readmitted 2 weeks later from the Nephrology clinic since her creatinine was 1.5 mg/dL and she persisted with generalized edema. Repeat SARS-CoV2 PCR was negative. At this time, she presented ANA titer in >=1:1280, strongly positive SSA, SSB, and SM antibodies, low C3 (43.0 mg/dL) and low C4 (7.4 mg/dL). Renal biopsy specimens showed more than 50 percent of glomeruli affected with mesangial and extracapillary hypercellularity, segmental cellular crescents, interstitial fibrosis, and marked deposition of immunoglobulins and complement, consistent with lupus nephritis Grade IV. Discussion(s): Systemic Lupus Erythematosus (SLE) is an autoimmune condition that has been described in correlation with SARS-CoV-2 infection in adult patients. Our patient fulfills SLICC criteria for SLE and a temporal relationship exists between SARS-CoV-2 infection and the development of SLE antibodies. SARS-CoV2 has also been reported to directly cause nephritis, although with more tubular than glomerular involvement. A renal biopsy is required for accurate diagnosis.

COVID-19 AS A CONFOUNDER IN A CASE OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

SESSION TITLE: Pulmonary Involvement in Critical Care Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Hemophagocytic Lymphohistiocytosis (HLH) is a condition in which the body's natural ability to end an immune or inflammatory response is defective1. COVID-19 also presents with severe inflammation, and like HLH, leads to significantly elevated ferritin2. We present a case that was initially thought to be COVID-19, but the patient was diagnosed with HLH in the setting of S. aureus endocarditis. CASE PRESENTATION: A 62-year-old male with a history of atrial fibrillation, mechanical mitral valve on warfarin, type II diabetes, chronic obstructive pulmonary disease, and recently diagnosed COVID-19 presented to the hospital with progressive dyspnea. In the emergency department, he was found to be hypoxemic and in atrial fibrillation with rapid ventricular response. He had a fever of 39.3°C and his initial laboratory workup revealed hemoglobin of 11.9 g/dL, leukocytes of 5,700, platelets of 83,000, AST 35 U/L, ALT 34 U/L, CRP of 31.89 mg/dL, and ferritin of 1994 ug/L. The patient was admitted and started on dexamethasone 6 mg daily. The following day, the patient's blood work revealed a significant worsening of AST and ALT to 7280 U/L and 3319 U/L, respectively. D-dimer increased to 11861 ng/mL (DDU) and ferritin to 36,470 ug/L. On the third day of admission, his clinical status declined acutely as he became significantly bradycardic, progressing to a cardiac arrest after which he required cardiopulmonary resuscitation, intubation, and was transferred to the intensive care unit. A CT scan obtained revealed hepatomegaly of 22 cm and blood cultures were positive for S. aureus requiring vancomycin treatment. The patient was kept on dexamethasone due to concerns for HLH. Ferritin continued to worsen, reaching 50,749 ug/L. His sCD25 came back positive. Unfortunately, the patient expired on his fifth day of hospitalization after discussing with his family their goals for his care and switching his care to comfort only. DISCUSSION: HLH is a challenging condition since diagnosis is difficult and mortality is high. There are a few methods used to diagnose HLH. Usually, 5 of 8 criteria must be met, which was achieved with this patient. However, often the patient only fulfills 4 of 8 since many criteria are difficult to obtain such as bone marrow biopsy, sCD25, and CXCL9. A useful tool is the H-calculator3. Our patient scored a 180 indicating a 50-75% likelihood of HLH. Assessing the likelihood of disease is important since sCD25 and CXCL9 take time and if the patient is clinically deteriorating treatment should not be delayed. CONCLUSIONS: HLH is catastrophic and rare. Physicians should always have it as a differential diagnosis in patients with severe inflammatory states and elevated ferritins to avoid anchoring bias. If suspicion is high based on clinical evaluation and scores, treatment should not be delayed. Reference #1: Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010;16(1 Suppl):S82-S89. doi:10.1016/j.bbmt.2009.11.014 Reference #2: Cheng L, Li H, Li L, et al. Ferritin in the coronavirus disease 2019 (COVID-19): A systematic review and meta-analysis. J Clin Lab Anal. 2020;34(10):e23618. doi:10.1002/jcla.23618 Reference #3: Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-2620. doi:10.1002/art.38690 DISCLOSURES: No relevant relationships by Areeka Memon No relevant relationships by Carissa Monterroso No relevant relationships by Carson Oprysko No relevant relationships by Eduardo Padrao No relevant relationships by Mouna Penmetsa

ASA 2022 Abstracts

The proceedings contain 114 papers. The topics discussed include: hepatitis: current treatments and what we need from ultrasound;new tools for grading hepatic steatosis;a new standard for measuring the liver using ultrasound and determining hepatomegaly;getting to the heart of liver disease;1st trimester loss: communication and collaborate;recurrent miscarriage: clinical management and the role of ultrasound;fetal orofacial anomalies in the first trimester;anterior wall defects in the first trimester: from ectopia cordis to bladder exstrophy;the fatty liver: more than the crappy image;COVID in kids: are the children ok? the RCH experience and the Australian context;how to scan the unscannable: tips and tricks to help you navigate your way through a pediatric examination;ultrasound diagnosis of hypertrophic pyloric stenosis - time to change the criteria;plethora of pediatric abdominal pathology;and medullary pyramid thickness: a novel ultrasound metric that predicts the need for pyeloplasty in infants.

Multisystem Inflammatory Syndrome in Children: A Diagnostic Conundrum in a Young Kenyan Boy, October 2021

Introduction/ Background: Multisystem inflammatory syndrome in children is a severe manifestation of COVID-19 infection in children and adolescents. It causes a significant hyper inflammatory response in children and is related to SARS-CoV-2 infection. There is paucity of data on this subject, especially in Sub-Saharan Africa, leading to challenges and delays in diagnosis. Methods: A case of a 17-year Kenyan boy who presented to a tertiary-level facility in Nairobi with abdominal pain and diarrhea for five days, difficulty in breathing and conjunctival injection for 1 day. Three weeks prior to this he had a dry cough and associated sore throat. He hadn't received Covid-19 vaccination. There had been a COVID-19 outbreak at school. Examination at admission revealed he was hypotensive, tachycardic, tachypnoeic, afebrile with normal oxygen saturations. He had distended neck veins with hyperactive precordium and elevated jugular venous pressure, a distended abdomen, tender in the right upper quadrant and a hepatomegaly of 16cm. Results: Investigations revealed multiple organ dysfunction (MOD) including heart failure with reduced ejection fraction (LVEF-30%), acute kidney injury, acute congestive hepatopathy, coagulopathy, elevated inflammation markers and positive SARS-CoV-2 IgG and IgM and a negative COVID 19 PCR test. He received IV antibiotics, daily hemodialysis sessions, inotropic support, high dose steroid therapy and Tocilizumab. He succumbed 8 days after admission. A postmortem revealed necrosis of the glomeruli and tubules, acute hemorrhagic necrosis of hepatocytes with fatty change, hyaline covering alveoli sac inkeeping with acute respiratory distress syndrome. Impact: MIS-C presents a diagnostic challenge and is often mistaken for other medical conditions. This often leads to inappropriate or delayed treatment, hence poor outcomes. A high index of suspicion is warranted. This may present a wakeup call for consideration of extending vaccination to the pediatric age group. Conclusion: Multi-system inflammatory syndrome is a rare COVID 19 complication affecting children and adolescents. It presents difficulty in diagnosis in Kenya considering most adolescents are managed as adults. This case hopes to increase vigilance among health care workers and that more preventive interventions can be implemented to reduce infection in children.

Coronavirus-2019 Disease with Pancytopenia with Leptospirosis and Herpes Simplex Virus Co-infection

Aim and background: COVID-19 pandemic has affected the whole world. Besides COVID, many infections may emerge during the course of the disease. Lymphopenia, use of immunosuppressants underlying comorbidities, and immune dysregulation secondary to SARS-CoV-2 could be the likely cause of the emergence such infections. We hereby describe a case of COVID-19 disease which presented with pancytopenia and was found to have Leptospirosis and Herpes Simplex Virus co-infection. Case summary: A 23-yearold postpartum female with no comorbidities and uneventful obstetric history was referred to our hospital 2 weeks after a full-term normal vaginal delivery. She developed generalized convulsive status epilepticus on the 10th day of her delivery, which was managed elsewhere with anti-epileptic drugs (AEDs). During her hospital stay, RTPCR for COVID-19 turned out to be positive but she remained asymptomatic throughout the course of her illness and seizures remained well-controlled on AEDs. On admission to our hospital, she was fully conscious, alert with no focal neurological deficits. Notable findings on evaluation were pancytopenia with megaloblastic features, bilateral pedal edema, and hepatosplenomegaly. NCCT brain was done which was suggestive of subarachnoid hemorrhage (SAH) along bilateral parietooccipital region for which conservative management was planned. 2D echocardiography was normal. Ultrasonography of abdomen revealed gross splenomegaly and mild hepatomegaly with mesenteric lymphadenopathy. NCCT thorax and abdomen were unremarkable apart from hepatosplenomegaly. In the panel sent for pancytopenia workup, IgM anti-HSV 1 antibodies turned out to be positive in blood. In addition, tropical workup was suggestive of Leptospirosis (IgM antibodies were positive). Workup for tuberculosis was negative. Bone marrow workup revealed features of trilineage hematopoiesis with micronormoblastic maturation consistent with iron deficiency anemia with no evidence of hemophagocytosis. Subsequently, IV acyclovir, IV doxycycline, and iron replacement were added. She improved clinically after these therapies and was subsequently discharged in a stable condition. MRI brain with MR angiography and venography done before discharge showed T1 sulcal hyperintensities along bilateral parietooccipital regions suggestive of SAH which was not progressing (as compared to NCCT brain scan done at admission). On day 60 of telephonic follow-up, patient was doing well and leading normal life without any persistence or emergence of symptoms.

A Case Report of Coronavirus (COVID-19) Disease and Herpesviridae Viral Co-Infections: A Lethal Combination? ABS0138)

Introduction: COVID-19 pandemic has affected the whole world. Besides COVID, other viral infections may emerge during the course of the disease owing to lymphopenia, use of immunosuppressants, underlying comorbidities, and immune dysregulation, which may pose additional threats.1 We hereby describe two cases of COVID- 19 with viral co-infections belonging to the Herpesviridae family with undulating clinical course. Case 1: Cytomegalovirus (CMV) Co-infection: A 55-year-old male, COVID unvaccinated, chronic smoker, overweight and hypertensive was admitted to our ICU with a 1-week history of fever, cough, and breathlessness. SARSCoV- 2 reverse transcriptase-polymerase chain reaction (RT-PCR) test was positive. At admission, he had hypoxaemia (SpO2 86%on room air), respiratory rate 35-40/minute, and ground-glass opacities in chest X-ray involving 50% of bilateral lung parenchyma suggestive of severe COVID-19 pneumonia. He was managed with lung-protective invasive mechanical ventilation, restrictive fluid strategy, 16-18 hour/day proning sessions (4-5), intravenous (IV) remdesivir, IV dexamethasone 6 mg 12 hourly, and enoxaparin thromboprophylaxis. After 2 weeks of ICU stay, weaning was attempted but the weaning attempts failed due to underlying neuromuscular weakness. On examination, bilateral (B/L) cranial nerve palsies, areflexia, and motor power 0/5 in bilateral upper and lower limbs were noticed. possibility of Guillain-Barre syndrome (GBS) was kept and IV immunoglobulin therapy was empirically administered for 5 days with some improvement in power up to 1/5 in upper limbs. On day 35 of hospitalization, he developed pancytopenia along with features of deranged liver function and gut dysfunction. In evaluation, PCR for CMV turned out to be positive in blood. Bone marrow aspiration and biopsy showed hemopoiesis with viral inclusion bodies and hemophagocytosis (HLH) [Figs 1 and 2]. A diagnosis of secondary HLH related to CMV was contemplated and IV ganciclovir was initiated along with steroids. Histological evidence of CMV co-infection was present and moreover, the quantitative viral load of CMV showed a decreasing trend after initiating IV gancyclovir. However, the patient continued to deteriorate and succumbed to his illness in the 8th week of the ICU stay. Case 2: Herpes Simplex Virus (HSV) Co-infection: Twenty-three years postpartum female with no comorbidities and uneventful obstetric history was referred to our hospital two weeks after a full-term normal vaginal delivery. She developed generalized status epilepticus on the 10th day of delivery, which was managed with anti-epileptic drugs (AEDs). During the hospital stay, RTPCR for COVID-19 turned out to be positive but she remained asymptomatic and seizures were well-controlled on AEDs. On admission to our hospital, she was fully conscious and alert with no neurological deficits. Notable findings were pancytopenia with megaloblastic features, B/L pedal edema, and hepatosplenomegaly. NCCT brain revealed mild subarachnoid hemorrhage (SAH) along the bilateral parietooccipital region for which conservative management was planned. 2D echocardiography was normal. Ultrasonography of the abdomen showed gross splenomegaly and mild hepatomegaly with mesenteric lymphadenopathy. NCCT thorax and abdomen were unremarkable apart from hepatosplenomegaly. In pancytopenia workup, IgM anti-HSV-1 antibodies turned out to be positive in blood. In addition, tropical workup was suggestive of Leptospirosis (IgM antibodies positive). Serological evidence was suggestive of acute HSV-1 infection (based on antibody titers). Bone marrow workup had features of trilineage hematopoiesis with micronormoblastic maturation consistent with iron deficiency anemia without any evidence of hemophagocytosis. IV acyclovir, IV doxycycline, and iron replacement were added, after which she improved clinically and was discharged in stable condition. Tables 1 and 2 show a detailed description of these cases. Discussion: Herpesviridae family is the most important group of viruses responsible for persistent vi al infections in humans, of which CMV contributes to 60-90% of infections in adults, especially in developing countries.2 In healthy individuals, these viruses are kept dormant by the body's immune mechanisms but in an immunocompromised population, reactivation from the latent state can occur. SARS-CoV-2 infection predisposes patients to concomitant viral co-infections, owing to T-cell lymphopenia, decreased NK cell number, and use of immunosuppressive medications.3,4 The first case of CMV co-infection was first reported by D'Ardes and co-workers in 2020.5 Since then, many studies have been emerging in this area. In an observational study from France, 38 COVID-19 patients on >7 days of MV were studied for HSV and CMV pulmonary co-infections (by quantitative real-time PCR in tracheal samples) out of which 47% of patients had one of these infections (24% HSV, 5% CMV, 18% both).6 Another study looking for HSV-1 in patients on invasive MV found HSV-1 reactivation between days 11 and 40, which correlated with immunological markers of decreased innate immunity.7 A case series looking for CMV infection (by PCR in plasma or BAL) in COVID-19, also found CMV reactivation between day 7 and 45 of illness. Most of these patients were above 60 years of age and immunosuppressed (HIV, diabetes mellitus, medications).8 Although immunocompromised individuals are more vulnerable, healthy immunocompetent adults who are critically ill or on prolonged MV may also be susceptible to these infections.9-12 This may be explained by a state of immunoparalysis inherent to prolonged critical illness. In case 1, an ICU stay of around 9 weeks complicated with recurrent nosocomial infections, multiple blood product transfusions, and steroid usage could have the likely triggers. Whether viral co-infections are merely bystanders or truly pathogenic is difficult to comment but timely management is essential to avoid end-organ damage (EOD) which may occur directly (by enhanced viral load secondary to compromised host immunity) or indirectly (by inflammatory changes consequent to prolonged cell-mediated immunity required to maintain viral dormancy).2-4,13 It also seems imperative to study if a viral co-infection has a proclivity to develop more severe hematological anomalies (besides the inherent risk of HLH with COVID) as was seen in case 1, in which the patient had a downward spiral of illness with multiorgan dysfunction.14-15 Limitations: Dynamics of PCR trends and virology studies of samples from trachea, gut, and urine could not be analysed in our patients. Conclusion: Viral co-infections can occur in COVID-19 disease as these patients are often immunocompromised and critically ill. A high index of suspicion and prompt management is needed to improve the outcome of patients. Patients with organ dysfunctions especially hematologic abnormalities with bone marrow involvement should be worked up in detail to look for concomitant viral co-infections. In the future, large-scale research is needed to better elucidate the relationship between SARS-CoV-2 and other viral co-infections.

Neonatal Acute Liver Failure in a Preterm Neonate with Gestational Alloimmune Liver Disease and Congenital Portosystemic Shunts

Introduction: Gestational alloimmune liver disease (GALD) is a leading cause of neonatal acute liver failure (NALF), a rare but important diagnosis. Congenital portosystemic shunts (CPSs) are rare vascular anomalies that leave patients at risk for developing a wide spectrum of complications and have not been previously associated with GALD. In this case, we present a newborn male with NALF secondary to GALD complicated by intrahepatic shunts. Case Description: The patient is a 30 weeks gestational age male born to a 28-year-old gravida 2 para 0 mother via urgent cesarean section for severe placental malperfusion. The pregnancy was complicated by severe intrauterine growth restriction, oligohydramnios, and maternal COVID-19 infection. The patient's initial NICU course was remarkable for respiratory distress requiring ventilatory support, hypotension requiring inotropes and stress dose steroids, and coagulopathy with bleeding requiring transfusion of multiple blood products. An abdominal ultrasound showed large congenital intrahepatic portosystemic shunts. Over the course of the hospitalization, the infant progressed to fulminant hepatic failure with associated coagulopathy, hypoalbuminemia, direct hyperbilirubinemia, and hyperammonemia. There was persistent anasarca in addition to elevated ferritin (1,922 ng/dL) and alpha-fetoprotein (97,855 ng/mL). Serial SARS-CoV-2 NAAT were negative. In consultation with the hepatologist there was high clinical suspicion for GALD, and treatment with intravenous immunoglobulin was initiated, however, no clinical or laboratory improvement was noted. Abdominal MRI showed progression of the large CPSs and enlargement of the hepatic arteries. The infant continued to deteriorate, was transitioned to comfort care, and died on day of life 82. A limited autopsy revealed a markedly edematous and jaundiced male with grossly enlarged liver with hepatocellular cholestasis, portal fibrosis, diffuse hepatobiliary iron depositions, and C5b9 positivity within hepatocytes confirming a diagnosis of GALD. Discussion: Neonatal hemochromatosis is the phenotypic result of severe liver injury leading to iron overload and extrahepatic siderosis, the mechanism of hepatic injury now recognized in GALD. Liver failure in newborns with GALD often presents with marked coagulopathy, hypoalbuminemia, and edema with and without ascites. The establishment of the diagnosis is crucial given without treatment, the prognosis is very poor. There have been no case reports of neonates with acute liver failure from CPSs or CPSs occurring with GALD. We hypothesize that the presence of CPSs worsens the clinical course of GALD through an unknown mechanism that further expedites hepatocellular damage. Furthermore, the role of SARS-CoV-2 infection and transmission in the neonatal population is still unknown. Conclusion: Neonatal acute liver failure caused by GALD is a rare but potentially fatal diagnosis. CPSs associated with GALD have not been previously documented. This case demonstrates the interplay of these disease entities likely contributing towards a more severe course of NALF and highlights the importance of early identification for guiding management. (Figure Presented).