Changes in Kawasaki disease incidence and phenotype during the COVID-19 pandemic

INTRODUCTION. The aetiology of Kawasaki disease (KD) remains unknown. Changes in infectious exposure during the COVID-19 pandemic owing to infection prevention measures may have affected the incidence of KD, supporting the pathogenic role of an infectious trigger. The purpose of this study was to evaluate the incidence, phenotype and outcome of KD before and during the COVID-19 pandemic in Denmark. METHODS. This was a retrospective cohort study based on patients diagnosed with KD at a Danish paediatric tertiary referral centre from 1 January 2008 to 1 September 2021. RESULTS. A total of 74 patients met the KD criteria of whom ten were observed during the COVID-19 pandemic in Denmark. Alof these patients were negative for SARS-CoV-2 DNA and antibodies. A high KD incidence was observed during the first six months of the pandemic, but no patients were diagnosed during the following 12 months. Clinical KD criteria were equally met in both groups. The fraction of intravenous immunoglobulin (IVIG) non-responders was higher in the pandemic group (60%) than in the in the pre-pandemic group (28.3%), although the rate of timely administered IVIG treatment was the same in both groups (>= 80%). Coronary artery dilation was observed in 21.9% in the pre-pandemic group compared with 0% in KD patients diagnosed during the pandemic. CONCLUSION. Changes in KD incidence and phenotype were seen during the COVID-19 pandemic. Patients diagnosed with KD during the pandemic had complete KD, higher liver transaminases and significant IVIG resistance but no coronary artery involvement.Copyright © 2023, Almindelige Danske Laegeforening. All rights reserved.

Clinical profile and outcomes of COVID-19 infection during the first wave in children with hematological illnesses and cancer: An observational study from a tertiary care center in North India

Background: Patients with cancer are at a higher risk of getting infected with the severe acute respiratory syndrome coronavirus 2 owing to their immunocompromised state. Providing care to these patients amidst the first wave of the coronavirus disease-2019 (COVID-19) pandemic was extremely challenging. Objective(s): This study was aimed at evaluating the clinical profile and disease-related outcomes of pediatric patients with hematological illnesses and cancer. Material(s) and Method(s): This retrospective study was conducted at a tertiary care center in North India during the first wave of the pandemic from March 2020 to December 2020. Children aged up to 18 years, who were treated for a hematological illness or malignancy or underwent hematopoietic stem cell transplantation (HSCT) and tested positive for COVID-19 regardless of symptoms were included in the study. Baseline demographic data related to the age, diagnosis, treatment status, and chemotherapy protocol used were collected. Outcomes including the cure rates, comorbidities, and sequelae were recorded. Result(s): A total of 650 tests for COVID-19 were performed for 181 children;22 patients were found to be COVID-19 positive. The most common diagnosis was acute leukemia (63.6%). None of the patients developed COVID-19 pneumonia. The majority of patients had asymptomatic infection and were managed at home. Among those with a symptomatic infection, the most common symptoms were fever and cough. A total of 3 (13.6%) patients needed oxygen therapy, one developed multisystem inflammatory syndrome of children leading to cardiogenic shock. Three patients required intensive care or respiratory support;all the patients had favorable clinical outcomes. The median time from the onset of COVID-19 to a negative result on the reverse transcription-polymerase chain reaction test was 21.3 days. Cancer treatment was modified in 15 patients (68.2%). Conclusion(s): Our results suggest that children with hemato-oncological illnesses rarely experience severe COVID-19 disease. The impact of the first wave of COVID-19 primarily manifested as disruptions in the logistic planning and administration of essential treatment to these children rather than COVID-19 sequelae.Copyright © 2021 Cancer Research, Statistics, and Treatment Published by Wolters Kluwer - Medknow.

Clinical Features, Laboratory Findings Outcomes in Multisystem Inflammatory Syndrome (Mis-C) Following Covid-19 Infection - a Multicenter, Observational, Ambidirectional Cohort Study in Rio De Janeiro State, Brazil

Objectives: As of March 5th, 2022, around 1.585 cases of MIS-C and 98 deaths (6,4%) were reported in Brazil. The state of Rio de Janeiro State (RJ) having 94 cases (5,9%) and 4 deaths (4,2%)1.Our aim was to evaluate clinical and laboratory features, and management of MIS-C in seven pediatric hospitals in RJ, Brazil. Method(s): Multicenter, observational, ambidirectional cohort study in seven tertiary hospitals in RJ(Brazil), assessing medical charts of pediatric inpatients (0-18 years) diagnosed with MIS-C according to WHO/CDC criteria, from August, 2020 to February, 2022. Descriptive statistics were used to analyze distributions of continuous variables, frequencies, and proportions. Result(s): A total of 112 cases of MIS-C were enrolled. The mean age was 4.2 years and thre was male predominance (59,8%). All cases had a SARS-CoV-2 contact (29.5% close contact;31.3%:positive PCR;serology:43.8%).Only 12.5% had comorbidities. Length of stay (LOS) was 7 days.Median duration of fever was 8 days. Most common symptoms were: rash(67%);gastrointestinal (67%);conjunctivitis (42%);neurological(39.6%);cardiovascular(37.5%);cervical lymphadenopathy (36.6%), and shock/hypotension(28.6%).Co-infection occurred in 3 patients. Forty-four patients fulfilled criteria for Kawasaki disease. Most patients were admitted to PICU(12;62,5%) for amedian of 2 days. Respiratory distress was seen in 18,7%;hypotension:28,6%, and shock in 23,2%. Main laboratory findings were: high C-reactive protein in 95%;D-dimer:77%, anemia:77%, thrombocytosis:63%;transaminitis:43.8%, lymphopenia:38%;hypoalbuminemia:34%;thrombocytopenia: 29%;hypertriglyceridemia:28%, and high pro-BNP in 27%. Echocardiogram was performed in 91/112 patients;abnormal in 70,3%;exhibiting myocardial dysfunction( 25%);pericardial effusion(21%);coronary dilation/aneurysms(11%) and, valvulitis (14.5%). IVIG+corticosteroids (CTC) were administered in 59.8%(67/ 112);18.6%(18/112) IVIG only;10.7%(12/112) CTC only;3.4%(4/112)biologics, and 15(13.3%) received no treatment. ASA low dose in 77.7% (87/112) and moderate/high dose in 34.8%. Oxygen support was needed in 27,7%;vasoactive amines:18,7%;dialysis:5,3%, and transfusion:18,7%.One patient died from a cytokine storm syndrome. Conclusion(s): Our study reports a higher number of MIS-C cases in RJ than the number reported to Brazilian authorities, highlighting underreporting. Our patients were younger, had fewer comorbidities, cardiovascular/gastrointestinal/renal involvement, shortest LOS in ICU, and a higher frequency of myopericarditis.

Sarcoidosis-Associated Immune Thrombocytopenic Purpura and Focal Segmental Glomerulosclerosis

Purpose of study A 32-years old male with known multi-system sarcoidosis in remission for 5 years off treatment presented to the emergency room with complaints of generalized weakness, hematemesis, epistaxis, and bruises. Physical examination was notable for petechiae, ecchymosis along with papules and plaques suggestive of active sarcoid skin lesions on his extremities. Laboratory workup was significant for thrombocytopenia 3000/uL, acute kidney injury with sub-nephrotic proteinuria. Peripheral blood smear did not show evidence of hemolysis and direct Coombs test was negative. Infectious workup including COVID-19, HIV, and hepatitis serologies were negative. Computed Tomography (CT) of chest, abdomen, and pelvis showed mild splenomegaly and an increased number of sub-centimeter hilar and mediastinal lymph nodes. The patient was treated with dexamethasone 40 mg daily for 4 days and intravenousimmunoglobulins (IVIG-2 gm/kg) for possible Immune Thrombocytopenic Purpura (ITP) with improvement in platelet count to 42000/uL by day 3. His workup for AKI and sub-nephrotic proteinuria was negative apart from a positive ANA (1: 160) with low complements. The anti-phospholipid antibody panel was negative. The ACE level was markedly elevated (>80U/L). The patient could not get a renal biopsy due to severe thrombocytopenia. He was discharged but was re-admitted in 15 days for severe thrombocytopenia of 1000/uL, epistaxis, and bruising. We continued high dose steroids along with IVIG 1 gm/kg for refractory ITP with minimal response and started anti-CD20 agent (Rituximab) 375 mg/m2 weekly with thrombopoietin-receptor agonist (Eltrombopag). His platelets count improved in response to treatment and subsequent renal biopsy showed focal and segmental glomerulosclerosis along with mild interstitial fibrosis, tubular atrophy thought to be from long standing sarcoidosis. There was also evidence of focal arteriosclerosis with no evidence of granulomas, immune complex, complement, or IgG4 deposition. Given skin lesions, thrombocytopenia, extensive lymphadenopathy, and renal involvement with markedly elevated ACE levels the overall picture was consistent with active multi-system sarcoidosis. His platelet count increased to 177,000/uL at the time of discharge. Currently, the patient is on slow steroid taper along with Eltrombopag 25 mg every other day without any recurrence of his symptoms so far. Methods used We described one case of sarcoidosis with hematologic and renal involvement. Summary of results Our patient developed hematologic and renal complications approximately 6 years after being diagnosed with sarcoidosis. Initially, he did not demonstrate sufficient clinical response to IVIG and high dose steroids. However, after a course of anti-CD20 agent (Rituximab) and with the addition of thrombopoietin-receptor agonist (Eltrombopag) he showed improvement of platelet count and stabilization of the renal function. Currently, the patient is receiving maintenance therapy with Prednisone 7.5 mg daily along with Eltrombopag 25 mg twice weekly with no recurrence of ITP and stable renal function. A further decision on whether the patient needs another cycle of Rituximab will be determined by the patient's clinical course. Conclusions Highly variable manifestations of Sarcoidosis can pose a significant diagnostic and therapeutic challenge as can be seen from our case. ITP is a rare hematological manifestation of sarcoidosis and addition of anti-CD20 agents should be considered in refractory cases.

27th Asia and Oceania Federation of Obstetrics and Gynecology Congress

The proceedings contain 358 papers. The topics discussed include: role of early prophylactic aspirin on Covid-19 outcome in antenatal patients - an audit of a hospital in India;partial intestinal obstruction complicating pregnancy: diagnostic dilemma and management;a case report of uterine rupture recognized during cesarean section at the site of a previous hysteroscopy-related perforation;menstrual characteristics and its related morbidities among adolescent girls living in North Borneo, Malaysia: a questionnaire-based study;the volume of posterior cervical varicose correlates with intraoperative blood loss in placenta previa;implications of large fibroids in pregnancy: a multidisciplinary approach;unexpected ovarian malignancy in postmenopausal women following laparoscopic surgery for adnexal masses - a review of 5 years;post radiotherapy outcome on cervical cancer stage IIIB patients with and without paraaortic lymph nodes enlargement;and evaluation of the relationship between thrombocytosis and clinico-pathological factors of patients with epithelial ovarian cancer.

Cerebellar syndrome as a complication of COVID-19 disease

COVID-19 disease is caused by the new coronavirus SARS-CoV-2. The disease first appeared in China in 2019 and quickly spread throughout the world. It primarily affects the respiratory tract, manifested by fever, cough and the devel-opment of dyspnoea, but the symptoms and complications can affect any organ system. Neurological symptoms include headaches, muscle and joint pain, taste and smell disorders. Complications include inflammatory diseases of the central nervous system, ataxia, peripheral nerve and muscle diseases, worsening of extra-pyramidal diseases, and neuropsychiatric disorders. This paper presents a case report of a 62-year-old man with cere bellar syndrome, ataxia, intentional tremor and hypermetria when dealing with COVID-19 disease.Copyright © 2021 Neuroendocrinology Letters.

Heyde Syndrome Complicated by Essential Thrombocythemia: A Case Report.

Heyde syndrome is a multisystem disorder characterized by the triad of aortic stenosis (AS), gastrointestinal bleeding, and acquired von Willebrand syndrome. Age-related degeneration is the most common cause of aortic stenosis and is frequently encountered in today's aging society. Approximately 20% of patients with severe aortic stenosis have Heyde syndrome. We encountered an older patient with primary thrombocytosis who was brought to a rural community hospital with bloody stools and was diagnosed with bleeding from an intestinal arteriovenous malformation. A final diagnosis of Heyde syndrome was made based on the presence of severe aortic stenosis and the presence of schistocytes in peripheral blood smears. Valvular diseases can complicate chronic hematological diseases. When the rapid progression of anemia and segmented red blood cells in the peripheral blood are observed in patients with severe aortic stenosis, Heyde syndrome should be considered based on peripheral blood smears and clinical course.

Hematologic Parameters of Patients with COVID-19 Infection

SARS-CoV-2 causes primarily respiratory tract infection, but can also affect hematopoietic and immune systems. Quantitative and qualitative changes in lymphocytes, neutrophils, monocytes and platelets, are reported in infected patients. These changes are related to the severity of the disease. Lymphopenia is the most common finding in adult patients infected with SARS-CoV-2, while it is much less common in children. Leukocytosis can be detected in patients with severe infection, but rare in patients with mild to moderate infection. Thrombocytopenia or thrombocytosis can also be seen in accordance with the clinic. Dysplastic morphological changes in neutrophils and platelets can be detected in peripheral smear of patients.

Rare case of post-COVID transverse myelitis

Case Report: Acute transverse myelitis (TM) is a rare inflammatory disease that typically presents asweakness, sensory alterations, and bowel or bladder dysfunction. Among the causes of TM are infections, paraneoplastic syndromes, or autoimmune conditions of CNS. Postinfectious TM can develop secondary to a viral or bacterial infection. SARS-CoV-2 is a recently discovered viral illness, and sequelae due to COVID-19 infection are still being studied. There is scarce literature relating the two conditions, and it is imperative to raise awareness. A 72-year-old man with hypertension and GERD, completely independent in ADL, was brought to the ED with sudden onset of bilateral lower extremity weakness. He reported symptoms started with difficulty climbing stairs that rapidly progressed to inability to ambulate independently and were associated with bilateral thigh soreness. Nine days prior, he developed fever and generalized malaise, and two days later, SARS-CoV-2 PCR and Ag tests were positive. He received azithromycin, Paxlovid, and dexamethasone as treatment. Upon evaluation, the patient was afebrile and hemodynamically stable. Neurological examination was remarkable for spasticity and hyperreflexia at bilateral lower limbs, clonus, preserved motor strength with adequate sensation to soft touch, and intact vibration and proprioception in all extremities. Cranial nerves were intact. These findings were consistent with an upper motor neuron lesion. On imaging, the Head CT scan was unremarkable. Thoracic/Lumbar Spine MRI was significant for distal thoracic and conus areas with central homogeneous brightness compatible with nonspecific myelitis. Laboratories showed leukocytosis without neutrophilia or bandemia, thrombocytosis, and elevated CRP. HIV and RPR tests were negative. A lumbar puncture for CSF analysiswas remarkable for mild monocytic pleocytosis (7 cell/muL), an increased level of total proteins (56 mg/dL), and normal glucose (57 mg/dL). CSF culture and gram stain were negative. CSF cytology yielded few lymphocytes and few monocytes and was negative for malignant cells. The meningoencephalitis panel was negative. Based on these findings, a clinical diagnosis of postinfectious myelitis secondary to COVID-19was made. The patient was treated with intravenous Methylprednisolone 1 g daily for five days. On follow-up, lower extremity weakness resolved completely, and he resumed his daily physical activities. Patients with COVID-19 infection can present with neurologic manifestations such as headache, myalgias, dizziness, dysgeusia, and anosmia. This case hopes to raise awareness of less commonly known neurological manifestations of SARS-CoV-2 infection and how the early recognition of symptoms can help expedite the diagnosis and treatment of the condition to avoid long-term sequelae. [Figure presented] Copyright © 2023 Southern Society for Clinical Investigation.

Tsukamurella and mycobacterium tuberculosis pneumonia co-infection

Case Report: Tsukamurella species are aerobic, partially acid fast saprophytes commonly isolated from soil and water. They are opportunistic pathogens known to infect multiple organs and can contribute to significant pathologies such as bacteremia, peritonitis, and respiratory tract infections. Moreover, Tsukamurella shares certain characteristic properties to Mycobacterium tuberculosis and Actinomyces species, including the acid fast stain, which can contribute to misdiagnosis of patients. A 68 year old female patient presented to the ED for shortness of breath, fatigue, and weight loss for 6 months. The patient's past medical history includes pulmonary fibrosis, type 2 diabetes, coronary artery disease with stent, hyperlipidemia, hypertension, and M. tuberculosis infection when she was 3 years old in Finland. On admission, labs revealed thrombocytosis (reactive 555 000/microL), leukocytosis (14 450/microL), and microcytic anemia (9.4 microg/dl). Moreover, C reactive protein was elevated and procalcitonin was normal (0.06 microg/l);a COVID-19 PCR was negative. An X-ray revealed severe patchy and interstitial infiltrates throughout both lungs with parenchymal scarring and pleural thickening in the periphery of the left mid-lung zone with multifocal pneumonia. Blood and sputum cultures were performed under the impression of pneumonia, and treatment with azithromycin and ceftriaxone was started. A M. tuberculosis infection was suspected due to a positive AFS. Further chest CT suggested multifocal pneumonia within the left lung in addition to apparent cavitary lesions versus bulla, a chronic interstitial lung disease with traction bronchiectasis, calcified right lower lung nodule, and calcified hilar lymph nodes suggesting a history of granulomatosis diseases. A bronchoscopy with Bronchoalveolar lavage was performed. The initial sputum specimen direct smear showed acid-fast stain positive with Actinomyces growth, and Penicillin G was added to the treatment. Samples were sent to the state department lab, and biopsy revealed granulomatous inflammation negative for malignant cells. One month later, the patient's sputum culture showed Tsukamurella for High-performance liquid chromatography (HPLC). Moreover, a rifampicin sensible M. tuberculosis complex by NAA was also positive six weeks later. The patient was started on a complete TB regimen and continued in the outpatient pulmonology clinic with the addition of levofloxacin for three months and rifampicin substituted for rifabutin. As demonstrated in the case above, a Tsukamurella infection can present similarly to a Mycobacterium infection. Patients may be misdiagnosed or potentially be co-infected. Our patient was further tested and appropriately treated for Tsukamurella after further extensive diagnostic screenings. Due to a high rate of missed cases, it is important to keep Tsukamurella infection on the differential diagnosis as the patient presentation may initially appear to be a Mycobacterium or other pulmonary infection. Copyright © 2023 Southern Society for Clinical Investigation.

Should COVID-Positive Pregnant Patients with Thrombocytosis get Prophylactic Anticoagulation for Stroke in Labor?

Clinical manifestation of coronavirus (COVID-19) is known to be associated with a hypercoagulable state and has a correlation of stroke as observed during the COVID pandemic. Pregnancy augments physiologic estrogenic effects on coagulation, making pregnant patients hypercoagulable. Management of hypercoagulability includes anticoagulation, which poses a contraindication to neuraxial anesthesia based on current guidelines. Management of primary labor anesthesia modality in patients with thrombocytosis can be challenging, particularly when presented with concurrent COVID infection. There is no guideline on the administration of anti-coagulation in this populace. The risk-benefit stratification of neuraxial anesthesia versus general anesthesia needs to be reviewed in such a patient population. We present a case of a parturient in labor who had thrombocytosis with a platelet of 576, COVID infection, and received therapeutic anticoagulation based on an automatic EMR best practices protocol. In this report, we examine literature surrounding the intersection of COVID, pregnancy, thrombocytosis and neuraxial anesthesia with respect to coagulation status to better guide anesthetic management in patients.

Unusual Presentation of Acute Myeloid Leukemia (AML-M2): A Case Report

Context: Acute myeloid leukemia (AML) is rarely presented with thrombocytosis and marked thrombocytosis with a platelet count over 1.0×1012/L is an extremely rare phenomenon. Objective: A case of de novo AML with unusual presentation by extreme thrombocytosis. Design: A case report. Setting: Hematology Unit at the Oncology Center Mansoura University, Egypt. Patient: A 37-year-old male patient with a history of sleeve gastrectomy in 2020, presented with oral mucositis, recurrent abscess, fever, and bilateral axillary lymphadenopathy. Initial complete blood count (CBC) showed a Hb level of 6 g/dL, a platelet count of 1.685×109/L, and a white blood cell (WBC) count of 19×109/L. Diagnosis of de novo AML (FAB-M2 AML) was confirmed by bone marrow aspiration, biopsy, and immunophenotyping. Cytogenetic study showed negative t(8;21)/inv 16/ t(9;22). A molecular study showed positive FLT-3 mutation and negative BCP/ABL1, JAK2, V617F, and CALR exon 9 mutations excluding blast transformation on top of myeloproliferative neoplasm (MPN) and myelodysplastic syndrome (MDS). Interventions: The patient received induction chemotherapy including a course of 7-day-cytarabine along with 3-day-anthracycline on September 20th, 2021 but the patient was refractory as BMA showed blast cells 75%. The patient started salvage HAM (high-dose cytosine arabinoside and mitoxantrone) + Sorafenib on November 6th, 2021. Again, he could not achieve a response and received FLAG (high-dose cytosine arabinoside, fludarabine, and granulocyte colony-stimulating factors) + Sorafenib on December 18th, 2021, after recovery from COVID-19 infection. Main Outcome Measures: To shed light on fact that myeloid neoplasms as MPN, MDS, and de novo AML can share overlapping features. Results: On January 25th, 2022, the last CBC showed a Hb level of 8.4 g/dL, a platelet Count of 395×109/L, and a WBC Count of 2.41×109/L with a differential Neutrophil count of 0.24×109/L. The patient lost follow-up since then. Conclusions: Only a few AML cases have been reported with thrombocytosis. Detailed molecular studies are mandatory to confirm the diagnosis of de novo AML patients with unusual presentation. Careful follow-up of those cases could help in establishing management guidelines for better outcomes as those patients usually have a poor prognosis.

Differences in Prevalence of Haematological Abnormalities on Presentation to Hospital in COVID 19 Infected adult and Paediatric Patients: A Retrospective Multicenter Descriptive Study

Background: It is well known that COVID 19 infection affects multiple systems in the body. Reports have documented many changes in the hematopoietic system in the pathophysiology of the disease. Aim: The aim of the study was to find out the prevalence and any significant difference in routine haematological parameters on presentation in Paediatric and adult patients with COVID 19 infection. Methodology: We conducted a multicenter retrospective descriptive observational study and investigated the prevalence of haematological abnormalities at presentation of 1000 PCR swab confirmed COVID 19 infected randomly selected adult and Paediatric patients admitted to 3 tertiary hospital in Dubai. Data was gathered through their electronic medical records and all analysis was done using the Statistical Package for the Social Sciences software (SPSS). Results: The prevalence of at least one abnormal haematological parameter was 95.1% (794/835) on first presentation to the hospital. After adjusting of age and gender the prevalence of any white cell abnormality was 34.7% (290/835) (5.7% leukopenia, 9.6% leucocytosis, 25.4% lymphopenia, 5.5% neutropenia, 16.4% had neutrophilia, 7.3% monocytosis, and 1.2% eosinopenia). A prevalence of 15.3% (128/835) anaemia, 9.5% (79/835) thrombocytopenia and 4.3% (36/ 835) thrombocytosis was also observed. The prevalence of other abnormal blood parameters: C reactive protein 69.5%(573/835), D dimer 57.5% (280/835), high LDH 52%(383/835), high ferritin 72.1%(452/835), high INR 5.1%(38/835), prolonged PT 32.2% (240/835), and prolonged APTT 35.6%(264/835). A significant difference in prevalence of these abnormalities was evident between adult and Paediatric population, these abnormalities were much more prevalent in adults but interestingly paediatric population tended to have higher incidence of neutropenia, eosinophilia and monocytosis (p<0.001). Conclusion: The effects of COVID 19 infection are different in adult and paediatric patients. Many mechanisms have been hypothesized for this observation. This study revealed another less studied and interesting variation in the manifestation among the two populations.

A CASE of SEVERE ANCA ASSOCIATED VASCULITIS after COVID-19 VACCINATION

Background: The world is currently rocking to and fro in the midst of the COVID-19 viral storm and vaccinations have played a pivotal role in calming this.Although COVID-19 vaccines have been thoroughly assessed and studied before being rolled out to the general population, there have been reports of post vaccination complications in limited number of subjects strongly associated with COVID-19 vaccinations[1]. Objectives: To report a case of severe ANCA associated vasculitis after COVID-19 vaccination. Methods: A case report and discussion. Results: In view of this, we report the case of a 77 year old caucasian male who developed severe ANCA associated vasculitis (AAV) after two doses of Astra-Zeneca vaccine and one booster dose of Pfzer COVID-19 booster. He presented with acute onset infammatory arthritis with mononeuritis multiplex with bilateral foot drop and left radial and ulnar nerve forearm weakness in typical asymmetrical pattern two weeks after the Pfzer vaccination. He had a raised MPO-ANCA titre of 66 IU/ml, C-reactive protein of 131mg/L and reactive thrombocytosis of 458 X 10 9/L. Nerve conduction study confrmed mononeuritis multiplex in the bilateral peroneal nerves and left radial and ulnar nerve. A total body CT had excluded malignancy and paraneoplastic associations and Gullian-Barre diagnosis was also excluded. The patient was treated with 3 days of intravenous methylprednisolone 1g daily then given intravenous Rituximab 1g, two weeks apart. He is currently undergoing rehabilitation in view of the vasculitic neuropathy from his diagnosis. Conclusion: Diagnosis of AAV is often delayed or missed by other medical specialties due to its varied presentation. AAV should be suspected in a patient with paraesthesia/weakness in keeping with mononeuritis multiplex or other peripheral neuropathy in the absence of an alternative explanation (e.g. diabetes,B12 defciency) and in particular with a wrist or foot drop.Exposure to certain drugs and substances of abuse such as cocaine, hydralazine and propylthiouracil has been implicated with AAV.While short-term side effects of COVID-19 vaccine resemble those of other vaccines, long-term side effects remain unknown[2]. Rare side effects continue to surface as millions of people receive COVID-19 vaccines around the world.

A Crick in theNeck: Lemierre Syndrome in a 4-year Old Female Presenting as Fever and Torticollis

Introduction: Described by Dr. Andre Lemierre in a 1936 case series of 20 patients, Lemierre Syndrome (LS) is defined as a septic thrombophlebitis of the internal jugular vein (IJV). LS typically begins as an oropharyngeal infection that advances to the IJV through direct extension through the fascial planes of the neck, or indirect lymphatic or hematogenous spread from the peritonsillar vessels. We present a case of LS in a 4-year-old patient who presents much younger than the typical age range of affected individuals, and who recovered well without any longterm sequelae. Case Description: A 4-year-old ex-27 week female presented with a near 3 week history of intermittent fevers and progressive right-facing torticollis. She had multiple interactions with the health care system over her illness course, and was given diagnoses ranging from general viral syndrome to gingivostomatitis and acute otitis media. Around the 2 week mark, her caretaker described her as having developed a “crick” in her neck while consistently favoring a rightward tilt. On illness day 16, she presented to her pediatrician for routine visit, and was noted to have fever, right tonsillar enlargement, and cervical lymphadenopathy, thereby prompting referral to the emergency department. Her physical exam on admission was additionally significant for a 30 degree rightward head rotation, a swollen and tender right sternocleidomastoid, and submandibular lymphadenopathy. She was resistant to active or passive neck rotation due to discomfort, but was able to traverse the midline with coaxing. Laboratory workup was notable for leukocytosis and thrombocytosis with elevated inflammatory markers, as well as mild transaminitis. Infectious serologic workup was negative for: SARS-CoV-2, Bartonella henselae, Bartonella quintana, EBV, and Mycoplasma. Blood culture showed no growth, but was drawn after antibiotics were given. A CT neck with contrast demonstrated intrinsic occlusion vs compression of the right IJV, and ultrasound and MRI confirmed IJV thrombophlebitis. Discussion: LS is typically associated with Fusobacterium necrophorum infection, a gram-negative anaerobe, with incidence estimated to be around 1 to 3.6 per million per year and mortality rate around 5 to 9%. Significant morbidity is often present, due to dissemination of septic thromboemboli, potentially affecting the CNS, bones/joints, and lungs. The typical age range for LS in pediatric patients clusters around adolescence, but infants as young as 6 months of age have been reported. As oropharyngeal infections most often precede LS, it is important to keep this rare but serious infection on any differential. Conclusion: This patient was diagnosed with Lemierre Syndrome. She was treated with an inpatient course of ampicillin/sulbactam before transitioning to oral amoxicillin/clavulanic acid to complete a total of 4 weeks of antibiotic therapy. All elevated laboratory markers normalized prior to hospital discharge, and the patient had complete resolution of symptoms at outpatient follow up.

A Clinical Differentiation of Multi system Inflammatory Syndrome in Children (MISC) & Kawasaki Disease (KD)

Background: With the emergence of the COVID 19 pandemic, a new disease, Multisystem Inflammatory Syndrome in Children (MIS-C), has evolved. Increasing numbers of children are being reported to have MIS-C in the U.S. & worldwide. In the U.S., there are currently 2617 MISC cases reported. MIS-C & Kawasaki Disease (KD) have almost the same presentation, making clinical differentiation difficult. This study aims at differentiating KD & MIS-C, which could assist clinicians to determine which disease they could be dealing with in their practices. Methods: Clinical features & laboratory values were collected from published studies found by queries on PubMed & other websites. Reported values were selected from published systemic reviews, metaanalyses, & large retrospective chart studies. Results: In KD, the most prevalent clinical features are fever (100%) & the 5 KDdefining clinical features: oral mucosal changes (96.5%), rash (96%), non-purulent conjunctivitis (89%), extremity changes (75.6%), and cervical lymphadenopathy (62.7%). MIS-C also presents with fever (100%) but has lower prevalence of oral mucosal changes (23%), rash (38.2%), non-purulent conjunctivitis (44.0%), extremity changes (2.5%), & cervical lymphadenopathy (4%). MIS-C leads to higher rates of ventricular dysfunction (39.3%), myocarditis (23%), & shock. For cardiac biomarkers, MISC has elevated troponin I (x6 normal) & Beta Natriuretic Peptide (BNP) (x414 normal), while KD has elevations of troponin I (x1.9 normal) & BNP (x15 normal). MIS-C has higher elevations in ESR, CRP, and D-Dimer at x6, x30, and x40 from the normal values, respectively, while KD has elevations of x2.8, x2.1, x7.3 from the normal values, respectively. MIS-C is associated with neutrophilia, thrombocytopenia, & anemia in 22% of cases. KD is associated with mild neutrophilia & anemia. KD has thrombocytosis in the subacute phase (x1.46 normal). Conclusion: Our results demonstrated that there are overlaps & differences in clinical and laboratory features. Fever is present in both KD & MIS-C, however the 5 KD defining clinical features of KD are less frequent in MIS-C. MIS-C induces higher levels of troponin I & BNP, findings that could potentially explain for higher rates of ventricular dysfunction & myocarditis. MIS-C causes higher elevations in inflammatory markers & D-Dimers compared to KD. Uniquely, thrombocytopenia is commonly present in MISC rather than in KD. Differentiating KD & MIS-C can be challenging, but by focusing closely on the clinical & laboratory features, clinicians may be able to distinguish between the two &, therefore, deliver the most appropriate care to patients in their practices.

DON'T LOSE SIGHT OF GPA

CASE: A 48-year-old female with no medical history presented with 2 days of decreased vision in the right eye. She reported painless blurry vision that progressed to near complete vision loss. The vision loss was accompanied by one month of progressively worsening cough, body aches, and subjective fevers. She denied smoking and reported no sick contacts. Physical exam was notable for submandibular lymphadenopathy, bilateral conjunctival injection, and grossly decreased vision of the right eye. She also endorsed decreased sensation in bilateral lower extremities distally. Her initial labs showed leukocytosis (13), thrombocytosis (754), and elevated inflammatory markers (ESR 105 and CPR 359). A chest CT showed bilateral upper lobe consolidations and scattered mass like opacities bilaterally. Ophthalmic exam of the right eye revealed multiple small retinal infarctions consistent with paracentral acute middle maculopathy. A CT head was negative and TTE showed no vegetation. Additional testing revealed negative TB, COVID, and normal complements. Initial ANCA testing was negative, however a repeat test was strongly positive for ANCA with PR3 significantly elevated to 428. She was diagnosed with granulomatosis with polyangiitis (GPA) vasculitis and treated with prednisone and started induction therapy of Rituximab. IMPACT/DISCUSSION: GPA is a small-medium vessel necrotizing vasculitis and the most common anti-neutrophil- cytoplasmic-antibody (ANCA) associated vasculitis. GPA classically involves the upper respiratory tract, lungs, and kidneys referenced by the ELK criteria (ENT, Lung, Kidney) commonly used for diagnosis. ENT findings are present in 70-100% of cases with the nasal cavity and paranasal sinuses most commonly involved. Roughly 50% have pulmonary involvement on presentation, as in this patient, while only 10-20% have initial renal involvement. A prodrome of systemic symptoms including body aches and fevers is often present. GPA is closely associated with c-ANCA, with autoantibodies to proteinase 3 (PR3) positive in over 80% of cases. This patient did have prodromal symptoms yet her primary presenting symptom of vision loss was atypical. Eye involvement is not part of the diagnostic triad yet it can occur in GPA. When it does present, it usually manifests as scleritis, conjunctivitis, or uveitis. Retinal infarctions, as seen in this patient, are uncommon and make this case an atypical presentation of GPA. Additionally, ANCA positivity is related to disease activity and a negative ANCA should not exclude GPA from a differential. Not all patients will be ANCA positive on initial presentation and 10% of patients with GPA will remain ANCA negative. CONCLUSION: Providers should consider atypical presentations of GPA in addition to the classic triad of ENT, Lungs, and Kidneys. Renal manifestations are often missing initially and involvement of other systems, such as ocular, can take place. With a positive c-ANCA and high clinical suspicion, treatment should not be delayed.

HUMAN IMMUNODEFICIENCY VIRUS (HIV) INFECTION PRESENTING WITH PROGRESSIVE RASH

CASE: A 25-year-old female with no medical history presented with progressive petechial rash at the chest, trunk, bilateral forearms, and thighs. Patient had the COVID-19 vaccine three weeks prior. However, denied recent travel or tick bites. No home medications nor recent hospitalizations, other than a tooth extraction a month earlier. On physical exam, the patient's vitals were unremarkable and had non-blanching petechial rash noted on her torso and bilateral extremities. Labs were significant of platelet count 1,000/mcL, hemoglobin 12.9 mg/dL, WBC 7.52 x103 /mcL, absolute lymphocytes 3.33x103 /mcL. Patient was administered two units of platelets followed by intravenous immunoglobulin (IVIG) and dexamethasone. No bleeding or hemodynamic instability was identified. Platelet count improved to 100,000/mcL over the next 24 hours. Further work-up revealed a positive HIV-1 antibody, absolute CD4 256 cells/mcL, viral load 27,300 copies/mcL. Once starting antiretroviral therapy (ART);bictegravir, emtricitabine, and tenofovir alafenamide, platelet count increased within a month to more than 200,000/mcL. IMPACT/DISCUSSION: Thrombocytopenia is defined as platelet count below 150,000/mcL. HIV-induced cytopenias are common, mainly neutropenia. However, sentinel events of thrombocytopenia are very rare in otherwise healthy individuals. A review of 5,290 HIV patients at the University of British Columbia from 1996 to 2012 revealed only 0.6% incidence of severe thrombocytopenia which they defined as platelet count <20,000/mcL. The exact pathophysiology is not clearly understood, but it is possible that antibodies against HIV cross-react with platelets or possible immune alteration. This is suggested by the prompt resolution of thrombocytopenia once ART is initiated. Immune thrombocytopenia (ITP) is a diagnosis of exclusion typically presenting with thrombocytopenia while other cell lines are normal. The greatest concern is when platelet counts drop less than 20,000/mcL due to fears of intracranial bleeding. Literature is not decisive in a correlation between platelet counts and risk of bleeding, yet it is suggested that circulating platelets are younger and more effective to maintain hematopoiesis in ITP when compared to other causes of thrombocytopenia. Treatment approach for ITP depends on the bleeding risk. In the presence of bleeding, urgent platelet transfusion, glucocorticoids, and IVIG are the mainstay of treatment. In absence of bleeding, individualized assessment of the condition is recommended to either monitor or treat. Platelet counts below 20,000-30,000/mcL require steroids or IVIG. In our case, she surprisingly presented only with minor petechial bleeding. Prompt initiation of ART, close monitoring of platelet response and CD4 count, as well as identifying resistant thrombocytopenia is indicated once patient is medically stable. CONCLUSION: Sole presentation of ITP due to HIV infection is rare. Risks of critical bleeding and further management are crucial to prevent fatal outcomes.

Persistent Median Artery Thrombosis as Complications of Vaccination Against SARS-CoV-2

Purpose or Learning Objective: Due to the COVID-19 pandemic, people all over the world have been vaccinated. Among the huge benefits of the vaccines, the risk of thrombosis in both young and older people is rarely present. This case report describes an incidentally diagnosed thrombosis of the persistent median artery in the wrist shortly after the patient received a second dose of the Comirnaty vaccine. Methods or Background: A 34-year-old woman presented with pain in the right wrist and numbness in her fingers. Ultrasonography (US) and neurography were performed for diagnostic purposes. Results or Findings: Neurography of the median nerve was within the normal range. Doppler US detected a 15-mm-long area of median artery thrombosis. There were no signs of median nerve pathology on US. Due to a good collateral system distally, the blood flow was not affected. Hemostatic and an autoantibody blood test were checked. The antinuclear antibody immunoglobulin G (ANA IgG) was elevated, but no signs of thrombocytosis were detected. Conclusion: It is very important to remember possible vessel problems and thrombosis in patients with joint pain, especially if the patient has received the COVID-19 vaccine or recovered recently from a disease.

Post Dengue Reactive Thrombocytosis-A Case Report

Dengue fever is common in tropical countries like India. Typically, dengue fever and dengue haemorrhagic fever is characterized by high grade fever with arthralgia, myalgia and headache. Leukopaenia, thrombocytopaenia, and fluid leak is the hallmark of dengue which normalises with recovery. However, it is very uncommon in these patients to progress to severe thrombocytosis. Authors hereby report an unusual case of dengue followed by severe reactive thrombocytosis. A 13-year-old male child was admitted with complaints of fever and headache for the past two days and vomiting for one day. There was right hypochondrium tenderness. Investigations revealed leucopaenia (total leucocyte count was 3500/μL), platelets count was 150×103/μL, haematocrit was 34.5%, and dengue virus antigen detection (NS1) was positive. Coronavirus Disease 2019 (COVID-19) Reverse Transcription-Polymerase Chain Reaction (RT-PCR) was negative. The patient was managed conservatively with intravenous fluid ringer lactate, acetaminophen, antiemetics and antacids. After 72 hours of discharge, patient came back with thrombocytosis. He was medicated on aspirin and the platelet count was monitored on a daily basis. The platelet count kept on increasing, and on 4th day it reached 1350×103/μL, but declined to normal (450×103/μL) over the next 10 days. Hence, aspirin was stopped. The patient was later discharged, and on follow-up the blood counts were normal.