Bilateral Spontaneous Pneumothorax in a Child with Multisystem Inflammatory Syndrome Associated with COVID-19

Introduction: Spontaneous pneumothorax is a rare complication of coronavirus disease 2019 (COVID-19), primarily reported in adults. Pediatric cases with bilateral pneumothorax are much less reported. Case Presentation: We presented the case of a five-year-old previously healthy boy who developed persistent fever, abdominal pain, generalized maculopapular rash, and dyspnea before admission. His chest computed tomography (CT) showed a viral involvement pattern of pneumonia suggestive of COVID-19. Subsequently, he was confirmed with multisystem inflammatory syndrome in children (MIS-C). While he responded well to the therapies, on the fifth day of admission, he developed respiratory distress again. A chest roentgenogram showed bilateral spontaneous pneumothorax. Bilateral chest tubes were inserted, and his condition improved sig-nificantly after five days of admission to the intensive care unit. Two weeks later, he was discharged in good condition. Conclusion(s): Children with MIS-C associated with COVID-19 may develop primary spontaneous pneumothorax. Owing to the clinical picture overlapping with MIS-C associated with COVID-19, the timely diagnosis of pneumothorax may be challenging in such patients.Copyright © 2022, Author(s).

Rapid disease progression after enzyme replacement therapy interruption in a patient with cholesteryl ester storage disorder

Lysosomal acid lipase deficiency (LALD) has two clinical phenotypes: an infantile-onset form - Wolman disease (WD) presented by severe malabsorption, cholestasis, malnutrition, hepatosplenomegaly and early death, and a later-onset form - cholesteryl ester storage disorder (CESD) with hepatosplenomegaly, dyslipidemia, malabsorption and variable disease severity manifestation. Enzyme replacement therapy (ERT) with sebelipase alfa was approved by the Brazilian Health Regulatory Agency (ANVISA) in 2017. We report the deleterious effect of ERT interruption in a CESD patient. Male, 16y, diagnosed at 7y, positive familiar history with mother, grandfather and three siblings affected. He was enrolled in the phase 3 clinical trial from age 9-12y under the 3 mg/Kg dose regimen. Then he enrolled in the post-study donation program under the same dose. Due to personal, importation and supply issues, and the COVID-19 pandemic restrictions, he had a progressive decrease in treatment adherence rate: 68% at 12y, 27% at 13y, 30% at 14y and after that he had an interruption of 1 entire year. At 15y he presented with generalized edema, severe fatigue, tachycardia, was hospitalized, diagnosed low serum iron, albumin and protein, acute anemia (hemoglobin: 6.2 g/dL - normal range of 12,5-13,5), and received blood transfusion. In the next year, still without ERT, he was hospitalized again due to generalized edema, acute anemia (Hemoglobin = 6.9 g/dL) and worsening of hypoalbuminemia and iron deficiency, and received blood transfusion. Although our patient has the CESD phenotype, the ERT interruptions did not worsen the liver involvement nor the dyslipidemia, but caused a severe malabsorption which is classically described in WD phenotype. In conclusion, this case serves as an alert that when ERT is interrupted, there is a rapid clinical deterioration. Acknowledgements: to IGEIM, and Drs Felippe Raphael e Oliveira Previdi and Ana Eduarda Saraiva Pereira Campos for clinical assistance of the patient.

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.

A BLOODY MASQUERADE: A CURIOUS CASE OF HEMOPTYSIS

SESSION TITLE: Variety in Chest Infections Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Actinomyces is a Gram-positive anaerobic and micro aerophilic filamentous bacillus that normally colonize the human mouth and digestive and urogenital tracts. They most commonly cause cervical and abdominopelvic infections and rarely pulmonary actinomycosis. CASE PRESENTATION: 67-year-old female with past medical history of recurrent DVT with IVC filter placement, non- ischemic cardiomyopathy, atrial fibrillation, 40 pack year history, recent COVID19 infection, lung nodules & COPD presented with complaint of coughing up blood associated with chest pain for the past 2 days. She had a low-grade fever with stable vitals with preliminary labs showing she was anemic and had reactive leukocytosis. She was recommended to hold oral anticoagulation and follow-up outpatient during when her symptoms worsened. On admission she was started on tranexamic acid nebulization for hemostasis and underwent CTA chest which showed no evidence for pulmonary embolism but commented on a right lower lobe perihilar 12.5 mm mass which has increased in size compared to previous scans. Patient underwent bronchoscopy which showed generalized edema of the tracheobronchial tree with bleeding from superior segment of the right lower lobe bronchus with no visualization of mass. PET scan showed hyper-metabolic lung mass with concerns for malignancy. CT guided biopsy of nodule was done and was not staining for malignant cells, acid fast bacilli with no fungal or bacterial growth. Blood cultures and Karius Digital cultures were also negative. She began expectorating blood clots despite being on treatment and cardiothoracic surgery was consulted. A partial lobectomy with lysis of adhesions of the right lower lobe was done. Specimen sent to pathology showed no evidence for malignancy but instead elicited a contained pulmonary abscess containing filamentous bacteria with parenchymal inflammation with areas of chronic hemorrhagic fibrosing pleuritis and hilar thrombi. She was diagnosed with pulmonary actinomycosis and started on IV 24,000,000 IU penicillin. She underwent a panoramic dental x-ray which was read as suboptimal dentition with multiple missing teeth and did not identify a source. Patient symptoms resolved post lobectomy and since discharged on long course of antibiotics. She continued to have no more episodes of hemoptysis. DISCUSSION: Hemoptysis as a symptom of pulmonary actinomycosis is a rather rare presentation. Actinomycosis causes cavities, nodules, and pleural effusions. It is commonly mistaken for chronic suppurative lung disease and sometimes malignancy. Isolation and identification occur only a minority of cases with a high culture failure rate due to previous antibiotic therapy, inadequate incubation time or culture conditions. CONCLUSIONS: Due to it's variable presentation pulmonary actinomyces has a large overlap with other diseases but must be considered in the differential of unexplained hemoptysis. Reference #1: Hemoptysis secondary to actinomycosis: A rare presentation. PMID: 24778485 PMCID: PMC3999682 DOI: 10.4103/0970-2113.129864 DISCLOSURES: No relevant relationships by Victoria Famuyide No relevant relationships by rukhsaar khanam

COVID-19–Associated Nephrotic Syndrome

Since the outbreak of SARS-COV-2 (COVID-19) in late 2019, there is a wide variety of renal pathology that is most associated with collapsing focal segmental glomerulosclerosis and acute tubular injury. This case presents a patient with new-onset focal segmental glomerulosclerosis - not otherwise specified (FSGSNOS), in a previously healthy adult after a COVID-19 infection. A 30-year-old Caucasian male with no significant past medical history presented with increasing severe generalized edema for 6 months following COVID-19 infection. The patient was admitted to the hospital with a significant acute kidney injury and hypoalbuminemia. Subsequent kidney biopsy revealed focal segmental glomerulosclerosis (NOS variant) with widespread foot process effacement that strongly supported a primary podocytopathy. Serological work-up was unrevealing: C3/C4, HIV, Hepatitis B and C, UPEP, SPEP, and light chains were negative. COVID-19 anti-nucleocapsid and anti-spike antibodies were positive. The patient was started on high-dose prednisone, RAAS blockade, statin therapy, and diuresis. Outpatient follow-up has revealed significant symptomatic improvement of edema with a return to baseline creatinine with initial interventions. COVID-19 infections have been most closely associated with collapsing focal segmental glomerulosclerosis, termed COVAN (corona virus-associated nephropathy), and acute tubular injury. Although the incidence of a non-collapsing FSGS secondary to COVID -19 infection is much lower than COVAN, FSGS-NOS increases the risk of permanent damage to kidney parenchyma if untreated. At this time, management and outcomes of noncollapsing FSGS associated with COVID-19 are not thoroughly studied. Therefore, we present this case to make physicians aware of a rare association between non-collapsing FSGS and COVID-19, as early recognition and treatment can promote recovery.

Generalized Edema and Pseudothrombocytopenia After ChAdOx1 nCoV-19 COVID-19 Vaccination: A Case Report.

Reports of side effects of vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are increasing worldwide. Capillary leak syndrome and vaccine-induced immune thrombotic thrombocytopenia are very rare but life-threatening adverse events that should be identified early and treated. However, isolated thrombocytopenia can indicate pseudothrombocytopenia. In certain people, ethylenediaminetetraacetic acid (EDTA) induces an in vitro platelet aggregation, resulting in misleading underestimation of platelet counts. It is essential to recognize pseudothrombocytopenia to prevent diagnostic errors, overtreatment, anxiety, and unnecessary invasive procedures. We present a case who developed generalized edema and persistent pseudothrombocytopenia after the first dose of the ChAdOx1 nCoV-19 vaccine (AstraZeneca).

DE NOVO DOUBLE GLOMERULOPATHY (MEMBRANOUS NEPHROPATHY, MN AND COLLAPSING FOCAL SEGMENTAL GLOMERULOSCLEROSIS, CFSGS) ASSOCIATED TO POSITIVE MYELOPEROXIDASE-O (MPO) ANTIBODY FOLLOWING PFIZER - BIONTECH MRNA VACCINATION COVID 19

BACKGROUND AND AIMS: In the COVID 19 pandemic era, anti SARS-CoV-2 vaccination showed high efficacy at preventing the infection and its most severe complications. The aim of this report is to describe an unusual double glomerulopathy related to anti SARS-CoV-2 vaccination and the good results obtained with the immunosoppressive treatment. METHOD: An 80-year-old caucasian woman developed a nephrotic syndrome, progressive renal insufficiency and microhematuria. The patient presented a medical history of thrombocytopenic purpura treated and resolved by steroids in 2013, hypothyroidism, hypertension, ischaemic heart disease treated with surgical bypass in 2019 and pacemaker in 2020 for atrial ventricular block. Due to pandemic COVID 19 status, she received two doses of the Pfizer BioNTech mRNA COVID-19 vaccine in March 2021. Two weeks after the second dose her weight increased of 23 kg. The family physician added furosemide to her therapy for generalized edema with no diuretic effect. In April, creatinine was 1.38 mg/dL (versus 0.8 mg/dL 1 year before);urinalysis showed proteinuria (300 mg/dL) and microscopic hematuria;serum total cholesterol level was 218 mg/dL and triglycerides 178 mg/dL;then it was suggested to increase the doses of furosemide. In May 2021, creatinine resulted 2 mg/dL, serum albumin 2 g/dL, and urinalysis confirmed proteinuria and microscopic hematuria;proteinuria was 10 g/day. Abdomen ultrasound showed normal liver, kidneys and spleen, not ascites. Lower limb eco-Doppler showed right superficial femoral artery stenosis of 60% and absence of venous thrombosis. The physical examination evidenced anasarca. The patients were admitted to the nephrology unit;hepatitis B surface antigen, hepatitis C antibody and human immunodeficiency virus antigen and antibody were negative. Both complement C3 and C4 levels resulted within the normal range. Cryoglobulins were absent. Urinary Bence Jones, antinuclear antibody (ANA), anti-extractable nuclear antigen (ENA), anti-double stranded DNA (nDNA) antibodies were negative. Antineutrophil cytoplasm antibodies (ANCA) were 1:2560 with Perinuclear pattern and anti-MPO positivity (716 UA/mL);anti-proteinase-3 antibodies (PR3) were negative. Antiphosholipase A2 receptor antibody (PLA2R Ab) was positive with high titre. A kidney biopsy was performed showing a double nephropathy: a focal segmental glomerulosclerosis (FSGS) with some collapsing features, superimposed on membranous glomerulonephritis (Fig. 1). RESULTS: We started the Ponticelli regimen (alternate months steroids and cyclophosphamide). After the first month of therapy, blood tests revealed creatinine 1.7 mg/dL, haemoglobin 11.7 g/dL;serum albumin 2.7 g/dL and urinalysis without microscopic haematuria. At the third month of therapy, the patient developed atrial fibrillation and started anticoagulation;blood tests were as follows: creatinine 1.1 mg/dL, serum albumin 3.0 g/dL, Ab anti-MPO 7 UA/mL and PLA2R Ab was absent. A left ocular, frontal and parietal herpes zoster induced a short discontinuation of therapy and responded well to Acyclovir;then we concluded the fourth month of therapy. At the fifth month, a SARS CoV 2 RT PCR unexpectedly resulted positive;the patient remained asymptomatic, but we stopped definitively the therapy. One month later, blood tests showed: creatinine 1 mg/dL, serum albumin 4 g/dL, proteinuria 0.7 g/die, MPO 2 UA/mL and PLA2R Ab absent. CONCLUSION: To our knowledge, this is the first case of nephrotic sindrome secondary to a De novo MN and FSGS, associated with positive MPO antibody, following Pfizer-BioNTech mRNA vaccination COVID 19;the patient responded well to immunosoppression going in remission and regaining renal function. (Figure Presented).

EARLY DIAGNOSIS OF EOSINOPHILIC FASCIITIS IN A 16 YEAR-OLD FEMALE

Introduction: Eosinophilic fasciitis (EF) is an uncommon scleroderma-like disorder resulting from infiltration of eosinophils and other white blood cells into the fascia. The etiology of EF is frequently idiopathic, but triggers have included trauma, medications, infection, and autoimmune conditions. Case Description: We present a case of EF in a 16-year old female with a history of Hashimoto’s thyroiditis, food allergies, and allergic rhinitis. Two weeks following her 1st dose of the Pfizer mRNA SARS-CoV-2 vaccine, she presented with generalized edema, weight gain (for 2-3 months prior), and polyarthritis. She did not have any scleroderma-like skin changes. Laboratory analysis was remarkable for eosinophilia (2130 cells/uL), elevated aldolase (15.6 U/L), and normal creatinine kinase. Lupus testing was unremarkable. MRI of bilateral thighs showed fasciitis and muscle/fascial biopsy demonstrated inflammatory myofasciitis consistent with EF. Given pending parasite studies, she received ivermectin prior to IV methylprednisolone therapy. Our patient has demonstrated significant improvement on prednisone, methotrexate, intravenous immunoglobulin, and hydroxychloroquine. Infectious work-up revealed positive Toxocara IgG levels, which prompted albendazole treatment. Discussion: Our case demonstrates an interesting perspective on the rare diagnosis of eosinophilic fasciitis. The etiology of her EF is unclear with confounding factors including her history of Hashimoto’s thyroiditis, positive Toxocara serology (IgG positive, IgM testing not available), and temporal relationship to Pfizer SARS-CoV-2 vaccine. This vaccine could have triggered an inflammatory process in the patient’s already hyper-reactive immune system. It is important to promptly recognize EF, as prolonged symptoms prior to diagnosis is associated with a poor treatment response.