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).

Inflammatory and oxidative stress markers in intracerebral hemorrhage: Relevance as prognostic markers for quantification of the edema volume.

We aimed to analyze the inflammatory and oxidative stress (OS) markers after intracerebral hemorrhage (ICH) and their temporal changes, interaction effects, and prognostic values as biomarkers for the prediction of the edema volume. Our prospective, longitudinal study included a cohort group of 73 conservatively treated patients with ICH, without hematoma expansion or intraventricular bleeding, which were initialized with the same treatment and provided with the same in-hospital care during the disease course. Study procedures included multilevel comprehensive analyses of clinical and neuroimaging data, aligned with the exploration of 19 inflammatory and five OS markers. White blood cells (WBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), neutrophilia, and lymphopenia peaked 3 days post-ICH, and they showed much stronger correlations with clinical and neuroimaging variables, when compared to the admission values. An intricate interplay among inflammatory (WBC, CRP, neutrophils, neutrophil-to-lymphocyte ratio [NLR], interleukin (IL)-6, and IL-10) and OS mechanisms (catalase activity and advanced oxidation protein products [AOPP]) was detected operating 3-days post-ICH, being assessed as relevant for prediction of the edema. The overall results suggested complex pathology of formation of post-ICH edema, via: (A) Not additive, but statistically significant synergistic interactions between CRP-ESR, neutrophils-CRP, and neutrophils-IL-6 as drivers for the edema formation; (B) Significant antagonistic effect of high protein oxidation on the CRP-edema dependence, suggesting a mechanism of potential OS-CRP negative feedback loop and redox inactivation of CRP. The final multiple regression model separated the third-day variables NLR, CRP × AOPP, and WBC, as significant prognostic biomarkers for the prediction of the edema volume, with NLR being associated with the highest effect size. Our developed mathematical equation with 3D modeling for prediction and quantification of the edema volume might be beneficial for taking timely adequate strategies for prevention of delayed neurological deteriorations.

Acquired hemophilia in a patient with SARS-CoV-2 infection, a case report

Acquired hemophilia (AH) is a potentially life-threatening hemorrhagic disorder. We report the second confirmed case of COVID-19-associated AH in a 45-year-old female which, unfortunately, expired as her treatment failed. She presented to the emergency department with abnormal bleeding and spontaneous hemoptysis about ten days after a removal surgery of her epiglottis tumor. Aggregation tests, such as partial thromboplastin time (PTT), are recommended in patients with COVID-19 infection that have bleeding episodes.Copyright © 2022 The Author(s);Published by Society of Diabetic Nephropathy Prevention.

Severe course of diabetic ketoacidosis due to new coronavirus infection in older children (clinical cases).

The risk of severe type I diabetes mellitus in children with new coronavirus infection (COVID-19) is extremely high, which is associated, with a high risk of intracranial hypertension, cerebral edema and multiple organ dysfunction, syndrome. On the example of a clinical case, the features of the course of diabetic ketoacidosis and. intensive care measures in children with COVID-19 were considered. The main data of the history and clinical and laboratory examination are reflected, specialattention is paid to the applied aspects of therapy, it was noted that with a severe course of a new coronavirus infection and diabetic ketoacidosis, the risk of developing cerebral injury, acute kidney injury and. thromboembolic complications is quite high, which, may require artificial lung ventilation, for the purpose of cerebral protection, renal replacement therapy and. the use of anticoagulants. The new coronavirus infection is a risk factor for the severe course of diabetic ketoacidosis in children with type I diabetes, regardless of the age of the child, which is the basis for clinicalalertness in order to timely identify and treat potential life-threatening complications.Copyright © 2022 Authors. All rights reserved.

Severe course of diabetic ketoacidosis due to new coronavirus infection in older children (clinical cases).

The risk of severe type I diabetes mellitus in children with new coronavirus infection (COVID-19) is extremely high, which is associated, with a high risk of intracranial hypertension, cerebral edema and multiple organ dysfunction, syndrome. On the example of a clinical case, the features of the course of diabetic ketoacidosis and. intensive care measures in children with COVID-19 were considered. The main data of the history and clinical and laboratory examination are reflected, specialattention is paid to the applied aspects of therapy, it was noted that with a severe course of a new coronavirus infection and diabetic ketoacidosis, the risk of developing cerebral injury, acute kidney injury and. thromboembolic complications is quite high, which, may require artificial lung ventilation, for the purpose of cerebral protection, renal replacement therapy and. the use of anticoagulants. The new coronavirus infection is a risk factor for the severe course of diabetic ketoacidosis in children with type I diabetes, regardless of the age of the child, which is the basis for clinicalalertness in order to timely identify and treat potential life-threatening complications.Copyright © 2022 Authors. All rights reserved.

Nasopharyngeal Myiasis in Intensive Care Unit (ICU) Patients: Report of Two Cases

Nosocomial myiasis is a rare event that has a higher incidence in the hospitals of poor and developing countries. The presence of nosocomial myiasis reflects the need for improved medical facilities and increased awareness among healthcare personnel. Severely ill patients are more susceptible, such as those with impaired consciousness, paralysis, and underlying diseases. The two cases here in described represent the first report of nosocomial myiasis in the Kurdistan Province, in Western Iran and one of them is the first report of myiasis involving a COVID-19-infected patient. The causal agent was Lucilia sericata. The taxonomical identification of the larvae of the second and third instar was based on the morphology of the cephaloskeleton, anterior spiracles, and peri-treme plaques.Copyright © 2023 Zobairy et al.

Neuropsychiatric Manifestations of COVID-19 in Hospitalized Pediatrics: A Multicenter Cross-sectional Study

Background: The large proportion of coronavirus disease 2019 (COVID-19) patients has been associated with a large number of neu-ropsychiatric manifestations. Despite the high prevalence of COVID-19, few studies have examined such manifestations, especially in children and adolescents. Objective(s): This study investigated neuropsychiatric manifestations in hospitalized children and adolescents admitted for COVID-19 infection in Iran. Method(s): This prospective observational study included admitted children and adolescents (4-18 years old) diagnosed with COVID-19 infection, pediatric neurologists, child and adolescent psychiatrists, and infectious disease specialists, and assessed 375 infected patients during August and December 2021. Result(s): Of the 375 patients, 176 (47%) were female, with a mean age of 9.0 +/- 3.39 years. Psychiatric and neurological manifestations were reported in 58 (15.5%) and 58 (15.5%) patients, respectively. The most prevalent psychiatric disorders were separation anxiety disorder (SAD) (5.1%), major depressive disorder (MDD) (3.5%), generalized anxiety disorder (GAD) (2.7%), insomnia (2.4%), and op-positional defiant disorder (ODD) (2.4%). Regarding neurological complications, seizures were the most prevalent (13.1%), followed by encephalitis (1.9%), transverse myelitis (0.3%), acute ischemic stroke (0.3%), and Guillain-Barre syndrome (0.3%). There was no significant relationship between the duration of COVID-19 infection (P = 0.54) and ICU admission (P = 0.44) with the emergence of psychiatric symptoms. Conclusion(s): The most prevalent neurologic and psychiatric complications among children and adolescents with COVID-19 infection were seizures and the symptoms of anxiety/mood disorders, respectively.Copyright © 2023, Author(s).

Immunotherapy-responsive recurrent extensive white matter disease without neuronal or glial surface antibodies: A case report

Background: Acute disseminated encephalomyelitis (ADEM) is classically considered as a monophasic immune-mediated demyelinating disorder. A relapse can occur in children but extremely rare in adults. Case-report: A 57-year-old man presented with fulminant ADEM-like episode without proceeding viral illness. Neurological deficits rapidly developed associated with extensive demyelinating brain lesions with vasogenic edema. After the initiation of aggressive immunotherapy, his symptoms resolved, but he relapsed twice during 26-month observation period;one was a mild episode characterized by rapidly evolving MRI lesions without development of symptoms, and the other was a fulminant ADEM-like episode similar to the first one. The second fulminant episode occurred only 2 days after getting a flu shot despite no clinical or radiological relapse when he received COVID-19 vaccinations. The patient's symptoms and extensive brain MRI lesions improved after the initiation of aggressive immunotherapy at the early stage. No autoantibodies against neuronal surface (such as GABA A receptor) or glial surface antigens (aquaporin 4, or myelin oligodendrocyte glycoprotein) were identified in either serum or CSF. Conclusion(s): Extensive white matter lesions can occur without neuronal or glial surface antibodies, recurrent fulminant ADEM-like episode can develop even in an adult patient, and flu shot may provoke fulminant ADEM-like episode.Copyright © 2022

Maternal COVID-19 Infection: A Cause of Neonatal Stroke?

Perinatal arterial ischemic stroke (PAIS) is a rare cause of neonatal seizures, with an incidence of 1 in 2500 to 4000 live births, globally. This is a case of a neonate with PAIS due to transpla-cental passage of COVID-19 IgG antibodies from the mother. A term, male neonate, born to a primigravida with an unevent-ful antenatal history was presented on the second day of life with multiple episodes of focal clonic seizures involving the right upper and lower limbs. Magnetic resonance imaging revealed an acute infarct in the left frontal lobe, extending into the parietal region, anterior limb, and genu of internal capsule suggestive of arterial ischemic stroke. The known causes of PAIS were evaluated and ruled out. The result of reverse transcription polymerase chain reaction analysis for SARS-CoV-2 antigen was negative for both the mother and the neonate. COVID-19 IgG antibodies in the mother and neonate were elevated. Seizures were controlled with antiepileptics. The neonate had no further seizure episodes and was discharged on oral levetiracetam. The infant was developmentally and neurologically normal at 3 months of age. PAIS is a rare cause of neonatal seizures, and maternal COVID-19 infection may be associated with neonatal stroke.Copyright © 2022, Himalaya Wellness Company. All rights reserved.

A successful thrombectomy in a case of progressive cerebral sinus thrombosis

Introduction: Cerebral venous sinus thrombosIs (CVST) is a rare condition compared to other categories encountered in stroke medicine.It occurs more frequently in patients with conditions that predispose them to other venous thrombosis, such as thrombophilias, acute malignancies, nephrotic syndrome, and COVID-19. CVST was identified as one of the rare causative of stroke. The exact mechanism of the stroke is not fully understood. However, a commonly agreed pathophysiology is that a dysfunction in arachnoid granulation can lead to sinus occlusion. Subsequently, this leads to a reduction in cerebral fluid drainage, which can increase intracranial pressure, causing capillary hypertension, cerebral oedema, decrease in cerebral perfusion pressure and venous haemorrhage. The European Stroke Organisation (ESO) supports using both MRI/MR Venogram and CT venogram as modalities for diagnosis of CVST, with no particular preference of one over the other. The standard practice in the management of cerebral venous sinus thrombosis includes treating the clot and its precipitating factors and treating the sequela of the clot as in the case we are reporting. Yet, there is no clinical guideline for the more aggressive measures to break down the clot in either AHA or European Stroke Organization, but they are used in clinical practice, with promising results in certain cases. Our case is an example of a successful mechanical thrombectomy with a lifesaving outcome. Method(s): We are reporting an unusual case of a 27- year- old lady who presented to the hyperacute stroke unit with dense right- sided weakness and expressive dysphasia. After an initial CT (Computerised Tomography) scan confirming extensive cerebral venous sinus thrombosis, she went for urgent mechanical thrombectomy. The clinical assessment after the procedure showed significant recovery in power of the right limbs and speech. She was discharged 7 days later with near full recovery. Venous thrombectomy is a rarely performed procedure. However, in this case, it was potentially lifesaving and resulted in an excellent clinical outcome. Result(s): An MRI/MRV follow up in a month demonstrated that the lesion on left centrum semiovale had regressed compared to the first scan. Also, there was some evidence of recanalization of her transverse sinuses. She was assessed by the therapist two months from the event. The patient reported some word finding difficulties and clumsiness in the right hand and leg. However, no further major event since her thrombectomy, and now aiming to get back to work. Conclusion(s): Mechanical thrombectomy in cerebral venous sinus thrombosis can be an effective, life-saving, and safe procedure with an extremely rewarding outcome. It should be considered in patients with acute neurological deterioration despite anticoagulant therapy.

Isolated cerebral mucormycosis in post-COVID-19 pneumonia

Background: Rhino-orbital-cerebral and isolated cerebral involvement of basal ganglia by mucormycosis are two different manifestations of CNS mucormycosis. The former variant caused by inhaled fungal spores and is common with immunosuppressive conditions. The latter form is caused by intravascular inoculation of spores as seen in intravenous drug abusers. Case report: Here we describe a case of young, non-addict patient with a history of recent mild COVID-19 pneumonia who presented with isolated cerebral mucormycosis involving bilateral basal ganglia. Discussion(s): The pulmonary vasculitis associated with COVID-19 is probably the cause of direct intravascular entry of inhaled fungal spores leading to direct isolated cerebral involvement. Such condition may rapidly turn fatal. Conclusion(s): This is the first reported case of isolated cerebral mucormycosis following post-COVID-19 infection. Early tissue diagnosis and intravenous amphotericin B is the key management.Copyright © 2022

Frequency of Life-Threatening Diabetic Ketoacidosis Related Complications among Pediatric Icu Patients Admitted Pre- and Postcovid- 19 Pandemic: A Single Center Experience

BACKGROUND AND AIM: The COVID-19 pandemic has been associated with an increase in the frequency and severity of new-onset diabetic ketoacidosis (DKA) in children (<18). We aimed to compare the incidence of life-threatening DKA complications among patients admitted to our pediatric intensive care unit (PICU) with new-onset DKA presenting pre- and post-the COVID-19 pandemic. METHOD(S): Retrospective observational study of children (0-18 years) admitted to our PICU with a diagnosis of newonset DKA between March 2018 and December 2020. Life-threatening DKA complications were defined as cerebral edema (defined as requiring hyperosmolar therapy and/ or cerebral imaging), respiratory failure requiring invasive mechanical ventilation, shock or hypotension requiring vasopressors, cardiac arrhythmias, cardiac arrest or death. Data was obtained by review of medical records. Analysis was performed using Chi-squared test, and Fisher's exact test where appropriate. RESULT(S): A total of 274 pediatric patients were admitted to our PICU with new-onset DKA between March 2018 and December 2020. There was a total of 12 life-threatening DKA complications among the pre-pandemic cohort (n=157) who presented between March 2018-February 2020. In comparison, there were 27 life-threatening complications among the post-pandemic cohort (n=117) who presented between March-December 2020. The difference was statistically significant (p= 0.0018). However, when analyzed individually, differences among cerebral edema (p=0.066), respiratory failure requiring mechanical ventilation (p=0.77) and shock (p=0.17) failed to reach statistical significance. This is likely due to the low overall incidence in which these individual complications occur. CONCLUSION(S): Following the COVID-19 pandemic we observed an overall increase in the incidence of lifethreatening DKA complications among pediatric ICU patients.

Cerebral Vasculopathy-Associated Ischemic Stroke in the Setting of Bacterial Meningitis

INTRODUCTION: Bacterial Meningitis is known to have high morbidity and mortality rates. A less recognized complication from this disease includes acute ischemic stroke, which conveys a worse prognosis. DESCRIPTION: A 37-year-old previously healthy woman presented to the hospital with progressive encephalopathy associated with ataxia and dysarthria. Her immediate past travel history revealed a trip to Europe where she endured a COVID19 infection three weeks before her admission. Nevertheless, she recovered without any complications. However, she developed fatigue and headaches, prompting a diagnosis of post-COVID19 syndrome by her primary care physician. Over the course of several days, her ability to carry out her normal daily activities and perform work-related duties deteriorated as she developed severe fatigue accompanied by a painless diffuse skin rash. She presented to the ED once she started having symptoms of dysarthria, abasia, and truncal ataxia. An emergently obtained CSF sample was consistent with bacterial meningitis. Standard empiric antibiotics and steroids were administered. The patient's condition acutely decompensated soon after antibiotics administration. A follow-up head CT showed global cerebral edema and hydrocephalus, triggering an EVD placement for ICP monitoring. An MRI brain showed multiple bilateral acute ischemic strokes in the brainstem and basal ganglia. A head CT angiography showed diffuse narrowing of the cerebral arteries. The patient ultimately completed a course of antibiotics (Neisseria PCR was positive). We used TCD-guided blood pressure augmentation to prevent the progression of cerebral ischemia. The patient was discharged on long-term steroid therapy for presumed post-infectious vasculopathy. A follow-up MRI brain did not reveal a progression of cerebral ischemia. DISCUSSION: Bacterial Meningitis is a severe disease with significant complications. One such complication is ischemic stroke. However, the exact pathophysiology is unknown. Understanding the risks of developing cerebral ischemia and the related pathophysiology could help improve patient outcomes with better treatment modalities. The interplay between COVID19 infection and conventional infectious pathogens is an ongoing area of interest.

Neonatal Diabetes Mellitus: A 5-Week-Old Male Presenting With Diabetic Ketoacidosis Complicated by Cerebral Venous Thrombosis and Status Epilepticus

Introduction: Neonatal diabetes mellitus (NDM) a rare cause of hyperglycemia in the neonatal phase, occurring in 1/300,000 case is characterized by a genetic mutation causing hyperglycemia in the first 6 months of age. We will present the case of a 5-week-old male with NDM, presented with hyperventilation, dehydration and complicated by status epilepticus and venous sinus thrombosis to review the pathophysiology, the complications and management options. Case Description: A 5-week-old male born at term appropriate for gestational age, presented for dehydration and lethargy. Urine analysis to rule out urinary tract infection showed glycosuria and ketonuria unmasking severe hyperglycemia of 1400 mg/dL. Sepsis workup was negative. There is no history of consanguinity nor family history of diabetes, but we note COVID infection 4 weeks ago. Intravenous insulin and hydration were started, then our patient complicated by respiratory distress, metabolic acidosis and status epilepticus. Three days later, clinical improvement was seen. An ultrasound of the head and abdomen were normal, and brain MRI showed a venous sinus thrombosis treated with anticoagulation. A1C was 5%, insulin level 1 mUi/mL, C peptide 1.36 ng/mL. Genetic analysis showed no mutation in the parents, but a de novo heterozygous mutation of the ABCC8 gene in our patient. Our patient was switched to subcutaneous insulin, and a decrease in the insulin need was seen over 3 months without the need of sulfonylureas. And the final diagnosis of our patient was transient NDM with the need of regular follow-ups to detect any relapse in the future. Discussion(s): NDM is characterized by a severe hyperglycemia in the first 6 months of age. The most frequent genetic causes of NDM with abnormal pancreatic function are aberrations in the 6q24 locus and mutations of the ABCC8 or KCNJ11 genes. We will focus on the ABCC8 gene coding for the ATP-potassium channel, which has an essential function in the cascade of insulin secretion. When glucose is low this channel is hyperpolarized, and when glucose increases, this channel is depolarized. This depolarization activates the calcium channels, enabling the release of insulin. Activating mutations will cause hyperpolarization of this channel, blocking the cascade of insulin release. NDM is stratified based on its progression as transient or permanent NDM. Transient NDM resolves within months and may relapse years later. The chief complaint can be dehydration and difficulty to thrive. The presence of a polyuria with frequent heavy wet diapers, in the setting of dehydration should raise the suspicion of NDM. Many complications can be seen like cerebral edema, and hypercoagulable state. Our patient developed a venous sinus thrombosis prevalent in 0.67 case/100000 cases/year. The initial treatment would be insulin injections, and in the presence of endogenous insulin production, estimated by C peptide levels, starting sulfonylureas is an option. The high relapse frequency suggests the need of regular follow-up during childhood and adolescence. Copyright © 2022

A Case of Cortical Blindness Secondary to Pres

Blindness brought on by an intact anterior visual pathway but due to bilateral occipital lobe involvement is known as cortical blindness. Compared to partial blindness, it is less frequent. The posterior reversible encephalopathy disease has a well-documented history of reversible cortical visual blindness (PRES). The neurological condition PRES is characterised by reversible subcortical vasogenic brain oedema and sudden onset neurological symptoms. This illness can be reversed with prompt diagnosis and treatment. It frequently occurs in conjunction with disorders like eclampsia, cancer, kidney disease, hypertension, and hypertension. This case involves a male teenager who was infected with COVID and later developed PRES. The presence of Anton's blindness complicated PRES. Utilizing radiological investigation, an early diagnosis was obtained, and once therapy was started, all symptoms disappeared. Copyright © 2023 Ubiquity Press. All rights reserved.

HYPERAMMONEMIA POSTLUNG TRANSPLANTATION: AN UNCOMMON BUT LIFE-THREATENING COMPLICATION

SESSION TITLE: Lung Transplantation Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Hyperammonemia is an uncommon yet serious complication that has been described in patients after solid organ transplantation, most commonly after lung transplantation. It has an incidence of about 2-4 % and a high fatality rate. Given the myriad of etiologies that can lead to encephalopathy post lung transplantation, hyperammonemia can easily be missed unless we have a high index of suspicion. Unlike in hepatic cirrhosis, non cirrhotic hyperammonemia can result in rapidly rising high levels of ammonia which can result in cerebral edema, seizures and long term neurological deficits. Hence, quick diagnosis and a multi faceted treatment approach is required for a favorable outcome CASE PRESENTATION: 37 year old man with COVID pneumonia and respiratory failure on ECMO support underwent bilateral orthotopic lung transplant. He had no significant past medical history. ECMO was decannulated on post op day 4 and by day 6 he was progressing well and working with physical therapy. On post op day 11 he had an abrupt decline in mental status and had an episode of seizure. Initial ammonia level was 181 uMol/L (Normal < 45 uMol/L) with a peak level of 248 uMol/L. Bronchial wash was positive for Ureaplasma species by PCR. CT head did not reveal any signs of cerebral edema. Management included daily hemodialysis, Sodium phenyl butyrate, Levocarnitine, Rifaximin, Lactulose and Doxycycline. Mental status started improving and ammonia levels normalized in the next 4 days. He was subsequently discharged home from the hospital without any neurological deficits. DISCUSSION: The etiology of post lung transplant hyperammonemia is not very clear. The etiology with the most evidence is an infection with urease producing bacteria as in our patient. Based on this, obtaining a PCR screening for these organisms in the Donor/recipient has been proposed. Obtaining a screening ammonia level at around day 7 post transplant has also been suggested. Given the high mortality and morbidity associated with this condition an aggressive multimodal treatment approach is required that includes renal replacement therapy, Nitrogen scavengers, bowel decontamination and empiric antibiotics. Hemodialysis has been shown to be more effective than continuous veno-venous hemodialysis for ammonia clearance. Antibiotics such as Azithromycin and Doxycycline that would be effective against urease producing organisms should be administered. In patients with signs of raised intracranial pressure, prompt neuroimaging and also measures to reduce cerebral edema must be instituted. CONCLUSIONS: Clinical signs of hyperammonemia should be promptly recognized in post lung transplant patients and managed aggressively given high mortality rates without treatment. A multi-pronged treatment approach with Intermittent high flux hemodialysis, bowel decontamination and agents targeting the urea cycle should be used to rapidly decrease the ammonia levels. Reference #1: Krutsinger D, Pezzulo A, Blevins AE, Reed RM, Voigt MD, Eberlein M. Idiopathic hyperammonemia after solid organ transplantation: Primarily a lung problem? A single-center experience and systematic review. Clin Transplant. 2017 May;31(5). doi: 10.1111/ctr.12957. Epub 2017 Apr 7. PMID: 28295601. Reference #2: Leger RF, Silverman MS, Hauck ES, Guvakova KD. Hyperammonemia Post Lung Transplantation: A Review. Clin Med Insights Circ Respir Pulm Med. 2020 Oct 26;14:1179548420966234. doi: 10.1177/1179548420966234. PMID: 33192115;PMCID: PMC7594252. Reference #3: Anwar S, Gupta D, Ashraf MA, Khalid SA, Rizvi SM, Miller BW, Brennan DC. Symptomatic hyperammonemia after lung transplantation: lessons learnt. Hemodial Int. 2014 Jan;18(1):185-91. doi: 10.1111/hdi.12088. Epub 2013 Sep 2. PMID: 23998793. DISCLOSURES: Research Grant relationship with Alung Please note: $1001 - $5000 by Bindu Akkanti, value=Grant/Research Support No relevant relationships by Soma Jyothula no disclosure on file for Manish Patel;No relevant relationships by Sandeep Patri

Mucormycosis, Acute Necrotising Pancreatitis and Haemorrhagic Stroke Occuring Simultaneously in an Immunocompetent Male: The Unwarned Apocalypse

Mucormycosis or zygomycosis is a life threatening invasive fungal infection, usually seen in patients with alteration of their immune system. It is a lethal and an aggressive fungal infection caused by the fungi of the order Mucorales. The angioinvasive property of mucormycosis can lead to fatal complications such as intracranial bleed. Acute pancreatitis refers to inflammation of the pancreas which presents mainly as acute pain in the abdomen and is a potentially fatal condition. The association of mucormycosis with acute pancreatitis is rare but dangerous. This case report highlights a case of 32-year-old male patient, with no co-morbidities, who was admitted to an rural central Indian hospital with four days of abdominal pain and two days of headache. Patient appeared to be in good health prior to this event. He was ultimately diagnosed with mucormycosis of paranasal sinus with acute pancreatitis. The patient was treated with intravenous antifungals, antibiotics and fluid therapy along with other supportive measures. Patient later developed intracranial bleed five days after admission, and ultimately succumbed on day seven of admission. After an extensive review of literature it was found that this is the first article to report mucormycosis, acute pancreatitis and intracranial bleed all occurring at once in an immunocompetent male.

EXPERIENCES with COVID-19 INFECTIONS in GERMAN PEDIATRIC RHEUMATOLOGY CENTERS

Background: Although the risk for severe COVID-19 progression in children is low, this may be aggravated by the underlying disease and/or immunosuppres-sive drugs. Objectives: We analyzed clinical data of COVID-19 cases among paediatric patients with rheumatic diseases reported to the BIKER registry. Methods: The main task of the German BIKER (Biologics in Pediatric Rheumatology) registry is to monitor the safety of biologics therapies in JIA. After the onset of the COVID-19 pandemic, the survey was expanded with a standardized form to proactively interview all participating centers about the occurrence, presentation, and outcome of SARS-CoV-2-infections in children with rheumatic diseases. Interviews were conducted with 68 centers initially weekly and later biweekly. Results: A total of 68 centres participated in the survey. Clinical data from 194 COVID-19 cases reported to the BIKER registry from 41 German and 1 Austrian pediatric rheumatology institutions between February 2020 and December 2021 were analyzed. Juvenile idiopathic arthritis (JIA, n=144) was the most common diagnosis followed by genetic autoinflammation (n=18;i.e. FMF, TRAPS, CAPS, HIDS, DADA2), systemic autoimmune diseases (n=11;i.e. SLE, dermatomyositis, vasculitis) and 16 with other rheumatic diseases (i.e. CRMO, Uveitis). 5 patients with no rheumatic disease were excluded. 104 (54%) patients were receiving conventional DMARDs, 81 (43%) received biologics, mainly TNF inhibitors (n=66 (35%)). Of the 189 rheumatic patients with SARS-CoV2 infection, 123 (63%) were female. The mean age was 12.4+/-4.4 years in females and 13.2+/-4.1 in males. The duration of SARS-Co2 infection associated symptoms was 13.8+/-15.3 days (max. 113 days), in 35 (43%) patients they lasted for > 12 days. 46 (24%) were asymptomatic. Patients with autoinflammation and systemic autoimmunopathies reported more symptoms such as fever, head and throat ache. 4 patients only complained about dyspnea. Only 3 patients were hospitalized and received Oxygen-supplementation. The only patients admitted to ICU, received ventilation but succumbed. This 3/-year-old patient, initially diagnosed with systemic JIA, developed fatal disease with intracranial edema and respiratory failure, as well as typical pulmonary texture changes. Prior to her SARS-CoV-2 infection, the patient was treated with MTX and low-dose steroids. Genetic testing revealed a so far unrecognized congenital immunodeficiency. In the total JIA cohort, treatment with corticosteroids, conventional DMARDs, biologics or combinations did not influence the number of reported symptoms or the favorable outcome of the cohort. However, the duration of symptoms was lower in the TNF-treated cohort (10.4+/-6.4 days vs. 15.7 +/-19.7 days). In the cohort with autoinflammation, fever was observed in 11 (61%). Those 6 who received IL-1-inhibitors did not show a different outcome than those 12 who did not. No case of PIMS/MISC in children with rheumatic diseases was reported. Conclusion: Except for one patient with congenital immunodefciency who died from her COVID-19 infection, no case of severe COVID-19 was reported in our cohort. At the time of infection, over 80% of patients in our cohort had been treated with conventional DMARDs and/or biologics. This did not appear to have a negative impact on the severity or outcome of SARS-CoV2 infection. Interestingly, no case of PIMS/MISC was observed.