148 results on '"IVIg, intravenous immunoglobulin"'
Search Results
2. Invasive Pulmonary Aspergillosis and Tuberculosis Complicated by Hemophagocytic Lymphohistiocytosis - Sequelae of COVID-19 in a Liver Transplant Recipient
- Author
-
Akash Gandotra, Rohit Mehtani, Madhumita Premkumar, Ajay Duseja, Arka De, Nabhajit Mallik, S. Durgadevi, Ashim Das, and Naveen Kalra
- Subjects
sHLH, Secondary hemophagocytic lymphohistiocytosis ,Case Report ,PAS, Periodic acid Schiff ,HRCT, High resolution computed tomography ,COVID-19, Coronavirus disease of 2019 ,Lenvatinib ,Liver transplant ,DEB-TACE, Drug eluting bead trans-arterial chemoembolization ,mRECIST, modified response evaluation criteria in solid tumors ,AKI, Acute kidney Injury ,IVIg, Intravenous immunoglobulin ,GM, Galactomannan ,Hepatology ,SARS-CoV-2 ,IDSA, Infectious Diseases Society of America ,COVID-19 ,hepatocellular carcinoma ,RT-PCR, Reverse transcriptase polymerase chain reaction ,AFB, Acid fast bacilli ,hemophagocytic lymphohistiocytosis ,disseminated tuberculosis ,BDG, Beta-D Glucan ,Invasive aspergillosis ,ATT, Anti-tubercular therapy ,SARS-CoV-2, Severe acute respiratory syndrome corona virus 2 ,NODAT, New onset diabetes after transplant ,IPA, Invasive pulmonary aspergillosis ,HCC, Hepatocellular Carcinoma ,HLH, Hemophagocytic Lymphohistiocytosis - Abstract
Liver transplant recipients are at an increased risk of opportunistic infections due to use of immunosuppression. Coronavirus disease of 2019 (COVID-19) increases the risk of these infections further due to associated immune dysfunction and use of high dose steroids. We present a case of liver transplant recipient who developed disseminated tuberculosis and invasive pulmonary aspergillosis complicated by acquired hemophagocytic lymphohistiocytosis after recovering from severe COVID-19.
- Published
- 2022
3. Advances in clinical outcomes: What we have learned during the COVID-19 pandemic
- Author
-
Amer Al-Musa, Brenna LaBere, Saddiq Habiballah, Alan A. Nguyen, and Janet Chou
- Subjects
BMI, body mass index ,RR, relative risk ,Risk Factors ,SOCS1, Suppressor of Cytokine Signaling ,Immunology and Allergy ,TNF-α, tumor necrosis factor alpha ,COVID-19, Coronavirus disease 2019 ,Sulfonamides ,LRBA, Lipopolysaccharide-Responsive Beige-Like Anchor Protein ,Antibodies, Monoclonal ,Prognosis ,MCP, monocyte chemotactic protein ,Treatment Outcome ,CXCL, CXC motif chemokine ligand ,JAK, Janus kinase ,CVID, common variable immunodeficiency ,TLR, Toll-like receptor ,CYBB, beta subunit of cytochrome b ,MIS-C, Multisystem Inflamatory Syndrome in Children ,COVID-19 Vaccines ,BAL, bronchial lavage fluid ,Immunology ,XIAP, X-linked Inhibitor of Apoptosis ,primary immunodeficiency ,ACE2, angiotensin-converting enzyme 2 ,Antiviral Agents ,Article ,EUA, emergency use authorization ,PIDs, primary immunodeficiencies ,MHC, major histocompatibility complex ,Humans ,biologics ,IFN, interferon ,CCL, C-C motif ligand ,RCT, randomized control trial ,PEG, polyethylene glycol ,Biological Products ,SARS-CoV-2 ,Interleukin-6 ,Immunologic Deficiency Syndromes ,COVID-19 ,asthma ,HR, hazard ratio ,Survival Analysis ,CI, confidence interval ,OR, odds ratio ,IVIG, intravenous immunoglobulin ,Interleukin 1 Receptor Antagonist Protein ,Purines ,Azetidines ,Pyrazoles ,CTLA-4, Cytotoxic T-lymphocyte-Associated Protein 4 ,ECMO, extracorporeal membrane oxygenation ,Biomarkers - Abstract
Our understanding of risk factors and interventions influencing outcomes from coronavirus disease 2019 (COVID-19) has continued to evolve, revealing advances emerging from hypotheses formed at the start of the pandemic. Epidemiologic studies have shown that asthma control, rather than a diagnosis of asthma, is a determinant of COVID-19 severity. Clinical outcomes in patients with primary immunodeficiencies, even in those with impaired cellular immunity, are variable. IL-6 has emerged as a reliable biomarker of COVID-19 severity, and large clinical trials have shown the potential for improving outcomes through inhibition of IL-6 signaling in some patients. Studies of genetic risk factors for severe COVID-19 have also revealed the importance of interferon homeostasis in the defense against severe acute respiratory syndrome coronavirus 2. Because COVID-19 vaccines constitute the primary tool for ending this pandemic, strategies have been developed to address potential allergic and immune-mediated reactions. Here, we discuss advances in our understanding of COVID-19 risk factors and outcomes within the context of allergic and immunologic mechanisms.
- Published
- 2022
4. Repetitive Immune-Mediated Noninfectious Endocarditis Necessitating 5 Mitral Valve Replacements
- Author
-
Maarten J. Cramer, Leo Koenderman, Steven A Muller, Helen L. Leavis, Karim Taha, Willem J.L. Suyker, Steven A. J. Chamuleau, Cardiology, ACS - Atherosclerosis & ischemic syndromes, and ACS - Heart failure & arrhythmias
- Subjects
TIA, transient ischemic attack ,medicine.medical_specialty ,medicine.medical_treatment ,Case Report ,MV, mitral valve ,Immune system ,Clinical Case ,CTD, connective tissue disease ,Internal medicine ,Mitral valve ,medicine ,Endocarditis ,Anakinra ,MVR, mitral valve replacement ,business.industry ,Mitral valve replacement ,autoimmune ,TEE, transesophageal echocardiography ,medicine.disease ,mitral valve replacement ,IVIG, intravenous immunoglobulin ,repetitive ,medicine.anatomical_structure ,endocarditis ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Dacron, polyethylene terephtelate ,medicine.drug - Abstract
We present a young patient who had to undergo 5 mitral valve replacements (MVR) because of a repetitive immune-mediated noninfectious endocarditis. The patient was treated with multiple anti-inflammatory drugs and high-dose prednisone. After the fifth MVR, the patient remained in stable condition using Anakinra after 22 months of follow-up. (Level of Difficulty: Advanced.), Central Illustration
- Published
- 2021
5. Giant Coronary Aneurysms in Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 Infection
- Author
-
Meghan Corrigan Nelson, Matthew E. Oster, Sassan Hashemi, Kelly Rouster-Stevens, D. Sofia Villacis-Nunez, Fred H. Rodriguez, Preeti Jaggi, Sampath Prahalad, Amit Thakral, and Elaine R. Flanagan
- Subjects
Pathology ,medicine.medical_specialty ,2019-20 coronavirus outbreak ,Coronavirus disease 2019 (COVID-19) ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,IgG, immunoglobulin G ,Case Report ,IVMP, intravenous methylprednisolone ,Clinical Case Series ,LAD, left anterior descending ,PCR, polymerase chain reaction ,Coronary Aneurysms ,giant coronary aneurysms ,otorhinolaryngologic diseases ,Medicine ,cardiovascular diseases ,multisystem inflammatory syndrome in children ,biology ,SARS-CoV-2 ,business.industry ,COVID-19 ,SARS-CoV-2, severe acute respiratory distress syndrome-coronavirus-2 ,KD, Kawasaki disease ,Biologic Agents ,IVIG, intravenous immunoglobulin ,cardiovascular system ,biology.protein ,MIS-C, multisystem inflammatory syndrome in children ,Antibody ,Cardiology and Cardiovascular Medicine ,business ,RCA, right coronary artery - Abstract
Multisystem inflammatory syndrome in children (MIS-C) can cause a myriad of cardiac manifestations, including coronary dilation and aneurysms; giant aneurysms are infrequent. We describe 3patients with giant coronary aneurysms associated with MIS-C, including the youngest case reported to date, treated with intravenous immunoglobulin, corticosteroids, and biologic agents. (Level of Difficulty: Intermediate.), Central Illustration
- Published
- 2021
6. Toxic epidermal necrolysis-like toxic erythema of chemotherapy: 2 illustrative cases
- Author
-
Ryan Arakaki, Lindy P. Fox, Daniel M. Klufas, Anna Haemel, Sally Y. Tan, Alyson A. Endicott, Eric Dean Merrill, Angela Lu, and Philip E. LeBoit
- Subjects
medicine.medical_specialty ,medicine.medical_treatment ,Case Report ,TEN - Toxic epidermal necrolysis ,Dermatology ,paraneoplastic pemphigus ,Stevens-Johnson syndrome ,CLS, capillary leak syndrome ,CLs upper limits ,toxic epidermal necrolysis ,capillary leak syndrome ,Acute graft versus host disease ,aGVHD, acute graft-versus-host disease ,medicine ,TEN, toxic epidermal necrolysis ,toxic erythema of chemotherapy ,Chemotherapy ,business.industry ,acute graft-versus-host disease ,PNP, paraneoplastic pemphigus ,TEC, toxic erythema of chemotherapy ,medicine.disease ,Toxic epidermal necrolysis ,IVIG, intravenous immunoglobulin ,Paraneoplastic pemphigus ,drug reactions ,RL1-803 ,oncology ,Toxic erythema ,business ,Capillary Leak Syndrome - Published
- 2021
7. Early Immunosuppression and Rapid Recovery of Cardiogenic Shock in Multisystem Inflammatory Syndrome From Convalescent COVID-19
- Author
-
Maxwell D. Coll, Mounica Yanamandala, Enrico G. Ferro, Mandeep R. Mehra, Eric Q. Wei, Cameron T Nutt, and Christine J. Wang
- Subjects
MIS-A, multisystem inflammatory syndrome in adults ,medicine.medical_specialty ,2019-20 coronavirus outbreak ,Coronavirus disease 2019 (COVID-19) ,acute heart failure ,medicine.medical_treatment ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Case Report ,Biventricular function ,Clinical Case ,Internal medicine ,Medicine ,biology ,treatment ,business.industry ,Cardiogenic shock ,Immunosuppression ,autoimmune ,medicine.disease ,MRI - Magnetic resonance imaging ,IVIG, intravenous immunoglobulin ,biology.protein ,Cardiology ,Antibody ,MIS-C, multisystem inflammatory syndrome in children ,Cardiology and Cardiovascular Medicine ,business ,MRI, magnetic resonance imaging - Abstract
A previously healthy 39-year-old man presented in cardiogenic shock with evidence of multisystem inflammatory syndrome of adults 2 months after a mild case of coronavirus disease 2019. He was treated with intravenous immunoglobulin and pulse-dose corticosteroids with rapid resolution of his symptoms and normalization of biventricular function. (Level of Difficulty: Intermediate.), Central Illustration
- Published
- 2021
8. The immune response to SARS-CoV-2 and COVID-19 immunopathology – Current perspectives
- Author
-
A. Morais, Luís Delgado, Inês Chora, and José Laerte Boechat
- Subjects
PRR, pattern recognition receptor ,Chemokine ,ICs, immune complexes ,viruses ,Review ,Adaptive Immunity ,medicine.disease_cause ,Severity of Illness Index ,IRFs, interferon regulatory factors ,0302 clinical medicine ,S protein, spike protein ,Immunopathology ,Medicine ,030212 general & internal medicine ,SARS-CoV-2, coronavirus-related second severe acute respiratory syndrome ,COVID-19, coronavirus disease 2019 ,Coronavirus ,Innate immunity ,Respiratory Distress Syndrome ,TNF, tumor necrosis factor ,biology ,RBD, receptor-binding domain ,Middle Aged ,Acquired immune system ,ICU, intensive care unit ,MASP-2, MBL-associated serine protease-2 ,VEGF, vascular endothelial growth factor ,NF-kB, nuclear factor kB ,Disease Progression ,Immunotherapy ,Antibody ,Adult ,ACE-2, angiotensin-converting enzyme 2 ,Pulmonary and Respiratory Medicine ,ILCs, innate lymphocytes or innate lymphoid cells ,Antiviral Agents ,Virus ,Diseases of the respiratory system ,03 medical and health sciences ,Immune system ,Humans ,IFN, interferon ,SARS, severe acute respiratory syndrome ,ARDS, acute respiratory distress syndrome ,Aged ,Inflammation ,Innate immune system ,RC705-779 ,SARS-CoV-2 ,business.industry ,COVID-19 ,biochemical phenomena, metabolism, and nutrition ,Respiration, Artificial ,Immunity, Innate ,IL, interleukin ,MAS, macrophage activation syndrome ,SARS-CoV-1, coronavirus-related first severe acute respiratory syndrome ,IVIG, intravenous immunoglobulin ,MERS-CoV, coronavirus-related Middle Eastern Respiratory syndrome ,030228 respiratory system ,DAMPs, damage-associated molecular patterns ,Immunology ,MBL, mannose binding lectin ,biology.protein ,MoAb, monoclonal antibody ,Lung Diseases, Interstitial ,C, complement factor (e.g. C3a, C3b, C4, C5, C5a) ,business ,CoV, coronaviruses - Abstract
SARS-CoV-2 is a new beta coronavirus, similar to SARS-CoV-1, that emerged at the end of 2019 in the Hubei province of China. It is responsible for coronavirus disease 2019 (COVID-19), which was declared a pandemic by the World Health Organization on March 11, 2020. The ability to gain quick control of the pandemic has been hampered by a lack of detailed knowledge about SARS-CoV-2-host interactions, mainly in relation to viral biology and host immune response. The rapid clinical course seen in COVID-19 indicates that infection control in asymptomatic patients or patients with mild disease is probably due to the innate immune response, as, considering that SARS-CoV-2 is new to humans, an effective adaptive response would not be expected to occur until approximately 2–3 weeks after contact with the virus. Antiviral innate immunity has humoral components (complement and coagulation-fibrinolysis systems, soluble proteins that recognize glycans on cell surface, interferons, chemokines, and naturally occurring antibodies) and cellular components (natural killer cells and other innate lymphocytes). Failure of this system would pave the way for uncontrolled viral replication in the airways and the mounting of an adaptive immune response, potentially amplified by an inflammatory cascade. Severe COVID-19 appears to be due not only to viral infection but also to a dysregulated immune and inflammatory response. In this paper, the authors review the most recent publications on the immunobiology of SARS-CoV-2, virus interactions with target cells, and host immune responses, and highlight possible associations between deficient innate and acquired immune responses and disease progression and mortality. Immunotherapeutic strategies targeting both the virus and dysfunctional immune responses are also addressed.
- Published
- 2021
9. Successful Treatment of Pembrolizumab-Induced Severe Capillary Leak Syndrome and Lymphatic Capillary Dysfunction
- Author
-
Nelson Leung, Sandra M. Herrmann, Svetomir N. Markovic, Itunu Owoyemi, Brittany Vlaminck, and Haixia Qin
- Subjects
medicine.medical_specialty ,Medicine (General) ,business.industry ,Immune checkpoint inhibitors ,Case Report ,Pembrolizumab ,030204 cardiovascular system & hematology ,CLS, capillary leak syndrome ,Tailored treatment ,VEGF, vascular endothelial growth factor ,Lymphatic Capillary ,Immune checkpoint ,IL, interleukin ,IVIG, intravenous immunoglobulin ,03 medical and health sciences ,0302 clinical medicine ,CLs upper limits ,R5-920 ,Internal medicine ,medicine ,Cardiology ,030212 general & internal medicine ,Adverse effect ,business ,Capillary Leak Syndrome - Abstract
Although capillary leak syndrome has a high mortality rate, its trigger, diagnosis, and treatment remain a challenge to clinicians because of the poor understanding of its mechanism and lack of treatment guidelines. With the extended use of immune checkpoint inhibitors in modern oncology, immune checkpoint inhibitor–associated immune-related adverse events have also expanded. We present a case of pembrolizumab-induced capillary leak syndrome and lymphatic capillary dysfunction in which the patient had an excellent clinical response to a tailored treatment strategy.
- Published
- 2021
10. Severe acute respiratory syndrome coronavirus 2-related multisystem inflammatory syndrome in children mimicking Kawasaki disease
- Author
-
Isabelle Melki, Romain Basmaci, Luigi Titomanlio, Ulrich Meinzer, Naim Ouldali, Constance Beyler, Michael Levy, Jean-Christophe Mercier, Agence Régionale de Santé Ile-de-France [Paris] (ARS IDF), Hôpital Robert Debré Paris, Hôpital Robert Debré, Centre de Référence pour les Maladies Rhumatologiques Auto-Immunes et Systémiques [Paris] (Institut IMAGINE/RAISE), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut Imagine [Paris Necker] (2I), Service de Pédiatrie-Urgences [Hopital Louis Mourier - APHP, Colombes], Hôpital Louis Mourier - AP-HP [Colombes], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Infection, Anti-microbiens, Modélisation, Evolution (IAME (UMR_S_1137 / U1137)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Université Sorbonne Paris Nord, Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Comets, Emmanuelle, Centre de Référence pour les Maladies Rhumatologiques Auto-Immunes et Systémiques [CHU Necker] (RAISE), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Université Sorbonne Paris Nord, and Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité)
- Subjects
Male ,Pediatrics ,Review ,030204 cardiovascular system & hematology ,Procalcitonin ,PCR, polymerase chain reaction ,0302 clinical medicine ,Vasoplegia ,030212 general & internal medicine ,Multisystem inflammatory syndrome temporally associated with SARS-CoV-2 ,Respiratory system ,skin and connective tissue diseases ,Child ,Children ,COVID-19, coronavirus disease 2019 ,[SDV.BIBS] Life Sciences [q-bio]/Quantitative Methods [q-bio.QM] ,Immunoglobulins, Intravenous ,TSS, toxic shock syndrome ,General Medicine ,KD, Kawasaki disease ,Shock, Septic ,[SDV.BIBS]Life Sciences [q-bio]/Quantitative Methods [q-bio.QM] ,Rash ,Systemic Inflammatory Response Syndrome ,3. Good health ,Viral pneumonia ,Shock (circulatory) ,Female ,Disease Susceptibility ,Symptom Assessment ,medicine.symptom ,PICU, paediatric intensive care unit ,Cardiology and Cardiovascular Medicine ,Syndrome inflammatoire multi-systémique temporellement associé à SARS-CoV-2 ,Enfant ,medicine.medical_specialty ,Adolescent ,education ,Shock, Cardiogenic ,Mucocutaneous Lymph Node Syndrome ,SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 ,Diagnosis, Differential ,03 medical and health sciences ,Diabetes mellitus ,otorhinolaryngologic diseases ,medicine ,Humans ,Immunologic Factors ,PIMS-TS, paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 ,KDSS, Kawasaki disease shock syndrome ,Cardiogenic shock ,IQR, interquartile range ,SARS-CoV-2 ,business.industry ,fungi ,ComputingMilieux_PERSONALCOMPUTING ,Syndrome de Kawasaki ,COVID-19 ,Kawasaki syndrome ,Length of Stay ,medicine.disease ,IL, interleukin ,body regions ,CI, confidence interval ,IVIG, intravenous immunoglobulin ,Kawasaki disease ,MIS-C, multisystem inflammatory syndrome in children ,business ,ECMO, extracorporeal membrane oxygenation ,Biomarkers ,Choc cardiogénique - Abstract
Graphical abstract Schematic distribution of the clinical signs in multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2., The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has been characterized by high transmission rates and high mortality in adults with predisposing factors, including age > 70 years, obesity, diabetes, systemic hypertension and other underlying diseases. During the second week of viral pneumonia, acute respiratory distress syndrome can occur and carries high mortality. Unlike most common respiratory viruses, children seem to be less susceptible to SARS-CoV-2 infection, and generally develop mild disease with low mortality. However, clusters of severe shock associated with high levels of cardiac biomarkers and unusual vasoplegia requiring inotropes, vasopressors and volume loading have recently been described. Both the clinical symptoms (i.e. high and persistent fever, gastrointestinal disorders, skin rash, conjunctival injection and dry cracked lips) and the biological signs (e.g. elevated C-reactive protein/procalcitonin and high levels of ferritinaemia) mimicked Kawasaki disease. In most cases, intravenous immunoglobin therapy improved cardiac function and led to full recovery within a few days. Adjunctive steroid therapy and sometimes biotherapy (e.g. anti-interleukin 1Ra and anti-interleukin 6 monoclonal antibodies) were often necessary. Although almost all children fully recovered within a week, some of them later developed coronary artery dilation or aneurysm. Thus, a new “multisystem inflammatory syndrome in children” related to SARS-CoV-2 has recently been described. Similarities with Kawasaki disease and the physiopathology of this syndrome still need further exploration.
- Published
- 2021
11. SARS-CoV-2 infection in children, clinical characteristics, diagnostic findings and therapeutic interventions at a tertiary care center in Riyadh, Saudi Arabia
- Author
-
Musaed Alharbi, Yasser M. Kazzaz, Hamad Alkhalaf, Tahir Hameed, Jubran Theeb Alqanatish, Maria Alayed, Mohammed Al Shaalan, Shahad Abu Hussien, Sameera M. Al Johani, and Alaa Alsadoon
- Subjects
Male ,China ,medicine.medical_specialty ,Pediatrics ,SARS-CoV, severe acute respiratory syndrome coronavirus ,Saudi Arabia ,Article ,WHO, World Health Organization ,law.invention ,lcsh:Infectious and parasitic diseases ,Tertiary Care Centers ,chemistry.chemical_compound ,Tocilizumab ,Severe acute respiratory syndrome coronavirus-2, epidemiological characteristics, children, COVID-19, Saudi Arabia ,law ,IVIG, Intravenous Immunoglobulin ,ICU, Intensive Care Unit ,Epidemiology ,CDC, Center for Disease Control and Prevention ,medicine ,Humans ,lcsh:RC109-216 ,Child ,Retrospective Studies ,Coronavirus disease 2019 ,Novel coronavirus ,business.industry ,SARS-CoV-2 ,lcsh:Public aspects of medicine ,Public Health, Environmental and Occupational Health ,COVID-19 ,Infant ,COVID-19, 2019 novel coronavirus disease ,MIS, C Multiinflammatory ,Retrospective cohort study ,lcsh:RA1-1270 ,General Medicine ,medicine.disease ,Intensive care unit ,Systemic Inflammatory Response Syndrome ,Systemic inflammatory response syndrome ,Pneumonia ,Infectious Diseases ,chemistry ,Supportive psychotherapy ,Child, Preschool ,Cohort ,Female ,business - Abstract
Background Coronavirus disease 2019 (COVID-19) pandemic caused by SARS-CoV-2 was first identified in Wuhan, China. All ages are susceptible to SARS-CoV-2 infection. Few studies had reported milder course in children however, severe course of illness has been reported. We aimed to describe the clinical features of COVID-19 in pediatric patients including diagnostic findings and therapeutic interventions in sever disease manifestation. Methods We retrospectively reviewed 742 patients with SARS-CoV-2 proven infection at King Abdullah Specialist Children’s Hospital, from April 2020 and July 2020. Inpatients, outpatient, including those with sever manifestation treated at the Intensive Care Unit (ICU) were included. We collected data including demographic data, comorbidities, symptoms, imaging data, laboratory findings, treatments and clinical outcomes of patients with COVID-19. Results Among of 742 patients, 71 (9.6%) were hospitalized. The median age of patients was 75 months old and 53.6 were male. A total of 461 (62.1%) had close contact with confirmed cases, 45 (6.1%) had no contact history, and 236 (31.8%) with unknown exposure risk. The most common symptoms at the onset of illness were fever (32.5%), respiratory symptoms (21%) and gastrointestinal symptoms (10.3%). Among the entire cohort, 7 patients were admitted to PICU with COVID-19 related symptoms, five patients diagnosed with MIS-C, one patient with Kawasaki, and one patient with pneumonia. All patients received supportive therapy, no antiviral treatment had been used however, in MIS-C patients IVIG had been given to all patients, five patients received Anakinra; and one patient received tocilizumab. Conclusions In this study, children infected with SARS-CoV-2 are less likely to develop symptomatic or serious diseases. Among symptomatic children, the most common clinical features were fever and respiratory symptoms followed by gastrointestinal manifestations. The majority of infected children have reported contact with an infected individual. MIS-C associated with COVID-19 is a severe presentation of SARS-CoV-2 infection and of a major concern as an overlapping features with other diseases could happen, making the diagnosis challenging.
- Published
- 2021
12. Acute Adult-Onset Kawasaki Disease Complicated by Coronary Artery Aneurysms, Thrombosis, and ST-Segment Elevation Myocardial Infarction
- Author
-
Andras Bratincsak, Trudy Hong, and Marian E. Melish
- Subjects
0301 basic medicine ,medicine.medical_specialty ,coronary aneurysm ,medicine.medical_treatment ,030105 genetics & heredity ,Chest pain ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,LVEF, left ventricular ejection fraction ,medicine ,ST segment ,Diseases of the circulatory (Cardiovascular) system ,Mini-Focus Issue: Imaging ,Myocardial infarction ,cardiovascular diseases ,Cardiac catheterization ,Kawasaki disease ,business.industry ,STEMI, ST-segment elevation myocardial infarction ,Emergency department ,medicine.disease ,KD, Kawasaki disease ,Thrombosis ,CT, computed tomography ,IVIG, intravenous immunoglobulin ,LAD, left anterior descending coronary artery ,medicine.anatomical_structure ,myocardial infarction ,RC666-701 ,Cardiology ,cardiovascular system ,ED, emergency department ,Case Report: Clinical Case ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,030217 neurology & neurosurgery ,RCA, right coronary artery ,Artery - Abstract
A 21-year-old man who had an initial misdiagnosis of chest wall cellulitis and sepsis presented to the emergency department with chest pain. Electrocardiogram demonstrated ST-segment elevation in the inferior leads. Cardiac catheterization identified diffuse aneurysmal dilation and thrombosis of the distal right coronary artery. Clinical signs were consistent with acute Kawasaki disease. (Level of Difficulty: Intermediate.), Graphical abstract
- Published
- 2021
13. Thymoma-associated multiorgan autoimmunity treated with multimodal therapy including extracorporeal photopheresis
- Author
-
John G. Zampella, Abhishek Gami, Manisha J. Loss, Chikoti M. Wheat, and Judy Doong
- Subjects
medicine.medical_specialty ,Thymoma ,ECP, extracorporeal photopheresis ,extracorporeal photopheresis ,medicine.medical_treatment ,Case Report ,chemical and pharmacologic phenomena ,Dermatology ,GVHD, graft-versus-host disease ,medicine.disease_cause ,Gastroenterology ,Autoimmunity ,Prednisone ,hemic and lymphatic diseases ,Internal medicine ,Extracorporeal Photopheresis ,IVIg, intravenous immunoglobulin ,medicine ,immunosuppression ,business.industry ,autoimmunity ,Multimodal therapy ,Immunosuppression ,thymoma ,medicine.disease ,TAMA, thymoma-associated multiorgan autoimmunity ,thymoma-associated multiorgan autoimmunity ,Radiation therapy ,surgical procedures, operative ,Graft-versus-host disease ,RL1-803 ,business ,medicine.drug - Abstract
Patients with malignant thymomas are susceptible to the development of graft-versus-host disease (GVHD)–like disease known as thymoma-associated multiorgan autoimmunity (TAMA). The proposed mechanism of this paraneoplastic phenomenon is a breakdown in the immune regulatory function of the thymus and subsequent activation of T cells. Here, we report a novel case of a patient with TAMA who, following surgical resection and radiation therapy, successfully underwent treatment with a combination of extracorporeal photopheresis (ECP), intravenous immunoglobulin (IVIg), cyclosporine, and prednisone.
- Published
- 2021
14. A Unique Case of Systemic Lupus Erythematosus Myocarditis Complicated by Plasmapheresis-Responsive Cardiogenic Shock
- Author
-
Rachel E. Dougherty, Carson Smith, Roopa Rao, and Maya Guglin
- Subjects
0301 basic medicine ,medicine.medical_specialty ,Myocarditis ,Cyclophosphamide ,medicine.medical_treatment ,Cardiomyopathy ,030105 genetics & heredity ,SLE, systemic lupus erythematosus ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,LVEF, left ventricular ejection fraction ,medicine ,Biventricular failure ,ECMO, extra-corporeal membrane oxygenation ,Diseases of the circulatory (Cardiovascular) system ,Ventricular function ,business.industry ,Cardiogenic shock ,systolic heart failure ,autoimmune ,Oxygenation ,Mini-Focus Issue: Heart Failure ,VA-ECMO, venoarterial extracorporeal membrane oxygenation ,medicine.disease ,IVIG, intravenous immunoglobulin ,RC666-701 ,Cardiology ,Plasmapheresis ,Case Report: Clinical Case ,Cardiology and Cardiovascular Medicine ,business ,cardiomyopathy ,030217 neurology & neurosurgery ,medicine.drug - Abstract
A 25-year-old woman with systemic lupus erythematosus complicated by biventricular failure with a history of multiple admissions presented with cardiogenic shock unresponsive to steroids, intravenous immunoglobulin, cyclophosphamide, and required extra-corporeal membrane oxygenation. Left ventricular function eventually recovered after plasmapheresis. (Level of Difficulty: Advanced.), Graphical abstract
- Published
- 2020
15. Management of Multisystem Inflammatory Syndrome in Children Associated With COVID-19: A Survey From the International Kawasaki Disease Registry
- Author
-
Supriya Jain, Ashraf S Harahsheh, Matthew D. Elias, Michael A. Portman, Audrey Dionne, Geetha Raghuveer, Therese M. Giglia, Brian W. McCrindle, Guillermo Larios, Cedric Manlhiot, Nagib Dahdah, and Nadine F. Choueiter
- Subjects
Pediatrics ,medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,Coronavirus disease 2019 (COVID-19) ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,MEDLINE ,Systemic inflammation ,medicine ,Dosing ,BNP, B-type natriuretic peptide ,COVID-19, coronavirus disease 2019 ,SARS-CoV-2, Severe acute respiratory syndrome coronavirus 2 ,business.industry ,ASA, Acetylsalicylic acid ,Multisystem inflammatory syndrome in children (MIS-C) ,IKDR, International Kawasaki Disease Registry ,KD, Kawasaki disease ,medicine.disease ,IVIG, intravenous immunoglobulin ,medicine.anatomical_structure ,MIS-C, Multisystem inflammatory syndrome in children ,lcsh:RC666-701 ,Original Article ,Kawasaki disease ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,ECMO, extracorporeal membrane oxygenation ,Venous thromboembolism ,Artery - Abstract
Background Since April 2020, there have been numerous reports of children presenting with systemic inflammation, often in critical condition, and with evidence of recent infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This condition, since defined as the multisystem inflammatory syndrome in children (MIS-C), is assumed to be a delayed immune response to COVID-19, and there are frequently cardiac manifestations of ventricular dysfunction and/or coronary artery dilation. Methods We surveyed the inpatient MIS-C management approaches of the members of the International Kawasaki Disease Registry across 38 institutions and 11 countries. Results Among the respondents, 56% reported using immunomodulatory treatment for all MIS-C patients, regardless of presentation. Every respondent reported use of intravenous immunoglobulin (IVIG), including 53% administering IVIG in all patients. Steroids were most often used for patients with severe clinical presentation or lack of response to IVIG, and only a minority used steroids in all patients (14%). ASA was frequently used among respondents (91%), including anti-inflammatory and/or anti-platelet dosing. Respondents reported use of prophylactic anticoagulation, especially in patients at higher risk for venous thromboembolism, and therapeutic anticoagulation, particularly for patients with giant coronary artery aneurysms. Conclusions There is variation in management of MIS-C patients with suboptimal evidence to assess superiority of the various treatments; evidence-based gaps in knowledge should be addressed through worldwide collaboration to optimize treatment strategies., There is a delayed and often critical presentation of a multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Cardiac manifestations can include ventricular dysfunction and/or coronary artery dilation. We surveyed the management approaches of the membership of the International Kawasaki Disease Registry. Management often includes immunomodulatory therapies and thromboprophylaxis with variation in indications across institutions.
- Published
- 2020
16. West Nile virus encephalitis: A report of two cases and review of neuroradiological features
- Author
-
Shitiz Sriwastava, Medha Tandon, Dhairya A. Lakhani, John C Mach, Christopher Bailey, and Saurabh Kataria
- Subjects
lcsh:Medical physics. Medical radiology. Nuclear medicine ,Pathology ,medicine.medical_specialty ,viruses ,lcsh:R895-920 ,Case Report ,Disease ,Arbovirus ,030218 nuclear medicine & medical imaging ,White matter ,03 medical and health sciences ,PCR, polymerase chain reaction ,0302 clinical medicine ,Cerebrospinal fluid ,Meningoencephalitis ,Basal ganglia ,medicine ,Radiology, Nuclear Medicine and imaging ,biology ,medicine.diagnostic_test ,business.industry ,virus diseases ,Magnetic resonance imaging ,biology.organism_classification ,medicine.disease ,IVIG, intravenous immunoglobulin ,Flavivirus ,WNV, West Nile virus ,medicine.anatomical_structure ,WNND, West Nile neuroinvasive disease ,business ,MRI, magnetic resonance imaging ,West Nile virus ,030217 neurology & neurosurgery - Abstract
West Nile virus (WNV) is a single-stranded RNA arbovirus of Flavivirus genus that is endemic to the United States and known to cause neuroinvasive disease. Diagnosis is confirmed by the presence of WNV-specific IgM antibodies within serum or cerebrospinal fluid (CSF). Radiologically, it presents as hyperintense T2 signal within deep brain structures (ie, thalami and mid-brain) with or without cerebral peduncle and substantia nigra involvement. On diffusion-weighted imaging, restricted diffusion is reported in basal ganglia and disseminated throughout the white matter. In this report, we describe the imaging findings for 2 cases of WNV from our institution; a 56-year-old female and a 34-year-old female. Increased vigilance for WNV is warranted, particularly in immunosuppressed patients presenting with a clinical picture of viral meningoencephalitis despite initial negative magnetic resonance imaging or CSF analysis. A high suspicion for WNV disease should prompt repeat imaging or laboratory workup.
- Published
- 2020
17. A review of potential treatments to date in COVID-19 patients according to the stage of the disease
- Author
-
Tamim Alsuliman, Banan Alkharat, Lugien Alasadi, Micha Srour, Ali Alrstom, Laboratoire d'Hématologie et d'Immunologie [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Damascus University, and Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)
- Subjects
0301 basic medicine ,medicine.medical_specialty ,Coronavirus disease 2019 (COVID-19) ,[SDV]Life Sciences [q-bio] ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Pneumonia, Viral ,Disease ,Antiviral Agents ,Pathophysiology ,Article ,General Biochemistry, Genetics and Molecular Biology ,Betacoronavirus ,03 medical and health sciences ,0302 clinical medicine ,IVIG, Intravenous Immunoglobulin ,Health care ,Pandemic ,medicine ,Humans ,Effective treatment ,CP, Convalescent Plasma ,Intensive care medicine ,Pandemics ,SARS-CoV-2 ,business.industry ,COVID-19 ,Viral Vaccines ,Timeline ,General Medicine ,COVID-19 Drug Treatment ,LMWH, low molecular weight heparin ,3. Good health ,Emergent disease ,Treatment ,Treatment Outcome ,030104 developmental biology ,LPV/RTV, Lopinavir/Ritonavir ,030220 oncology & carcinogenesis ,Coronavirus Infections ,business ,Hydroxychloroquine - Abstract
International audience; Introduction and motivation: Since the end of 2019, the COVID-19 pandemic has affected millions of people worldwide. With the rapid spread of this virus, an immense burden has fallen upon both healthcare and economic systems. As a consequence, there is an unprecedented urgency for researchers and scientific committees from all over the world to find an effective treatment and vaccine.Review structure: Many potential therapies are currently under investigation, with some, like Hydroxychloroquine, being authorized for emergency use in some countries. The crucial issue is now clearly to find the suitable treatment strategy for patients given comorbidities and the timeline of the illness. Vaccines are also under development and phase 1 clinical trials are rolling. Despite all efforts, no single drug or vaccine has yet been approved. In this review, we aim at presenting the proposed pathophysiological mechanisms of SARS-CoV-2 and to provide clinicians with a brief and solid overview of the current potential treatments classified according to their use at the three different currently proposed disease stages. In light of pathogenesis and proposed clinical classification, this review's purpose is to summarize and simplify the most important updates on the management and the potential treatment of this emergent disease.
- Published
- 2020
18. Elucidation of the Effects of a Current X-SCID Therapy on Intestinal Lymphoid Organogenesis Using an In Vivo Animal Model
- Author
-
Daiichiro Fuchimoto, Minoru Kitago, Hisashi Aso, Yoshihisa Suyama, Akira Onishi, Osamu Itano, Ayumi Matsuo, Kanae Niimi, Yoshifumi Sakai, Yoji Sasahara, Tomonori Nochi, Fuller W. Bazer, Shotaro Morita, Sachiko Matsuda, Shunichi Suzuki, Katsuki Usami, Kouichi Watanabe, Shun Ito, Guoyao Wu, and Mutsumi Furukawa
- Subjects
0301 basic medicine ,Male ,Swine ,Organogenesis ,X-SCID, X-linked severe combined immunodeficiency ,X-Linked Combined Immunodeficiency Diseases ,NARO, National Agriculture and Food Research Organization ,Animals, Genetically Modified ,Gene Knockout Techniques ,Peyer's Patches ,0302 clinical medicine ,PCR, polymerase chain reaction ,ILFs, isolated lymphoid follicles ,Medicine ,Intestinal Mucosa ,Child ,Original Research ,GFP, green fluorescent protein ,Bone Marrow Transplantation ,PP, Peyer’s patch ,Gastroenterology ,ELISA, enzyme-linked immunosorbent assay ,X-SCID ,medicine.anatomical_structure ,Treatment Outcome ,Child, Preschool ,RT, room temperature ,030211 gastroenterology & hepatology ,Female ,IgA ,Interleukin Receptor Common gamma Subunit ,Adult ,Adolescent ,Microflora ,03 medical and health sciences ,Immune system ,Animals ,Humans ,In vivo animal model ,lcsh:RC799-869 ,Gene ,Immunity, Mucosal ,Feces ,Severe combined immunodeficiency ,PCA, principal component analysis ,Hepatology ,Peyer’s Patches ,business.industry ,BMT ,medicine.disease ,LTi, lymphoid tissue inducer ,Ig, immunoglobulin ,WT, wild-type ,Small intestine ,BMT, bone marrow transplantation ,IL, interleukin ,Gastrointestinal Microbiome ,IVIG, intravenous immunoglobulin ,Disease Models, Animal ,030104 developmental biology ,Immunoglobulin G ,Immunology ,lcsh:Diseases of the digestive system. Gastroenterology ,Immune disorder ,business - Abstract
Background & Aims Organ-level research using an animal model lacking Il2rg, the gene responsible for X-linked severe combined immunodeficiency (X-SCID), is clinically unavailable and would be a powerful tool to gain deeper insights into the symptoms of patients with X-SCID. Methods We used an X-SCID animal model, which was first established in our group by the deletion of Il2rg gene in pigs, to understand the clinical signs from multiple perspectives based on pathology, immunology, microbiology, and nutrition. We also treated the X-SCID pigs with bone marrow transplantation (BMT) for mimicking a current therapeutic treatment for patients with X-SCID and investigated the effect at the organ-level. Moreover, the results were confirmed using serum and fecal samples collected from patients with X-SCID. Results We demonstrated that X-SCID pigs completely lacked Peyer’s patches (PPs) and IgA production in the small intestine, but possessed some dysfunctional intestinal T and B cells. Another novel discovery was that X-SCID pigs developed a heterogeneous intestinal microflora and possessed abnormal plasma metabolites, indicating that X-SCID could be an immune disorder that affects various in vivo functions. Importantly, the organogenesis of PPs in X-SCID pigs was not promoted by BMT. Although a few isolated lymphoid follicles developed in the small intestine of BMT-treated X-SCID pigs, there was no evidence that they contributed to IgA production and microflora formation. Consistently, most patients with X-SCID who received BMT possessed abnormal intestinal immune and microbial environments regardless of the presence of sufficient serum IgG. Conclusions These results indicate that the current BMT therapies for patients with X-SCID may be insufficient to induce the organogenesis of intestinal lymphoid tissues that are associated with numerous functions in vivo., Graphical abstract
- Published
- 2020
19. The development of mucous membrane epidermolysis bullosa acquisita in a pediatric patient
- Author
-
Kathryn Rentfro and Megan Arthur
- Subjects
Epidermolysis bullosa acquisita ,medicine.medical_specialty ,EBA, epidermolysis bullosa acquisita ,Case Report ,Dermatology ,medicine ,Drug rash ,Eosinophilia ,mucous membrane epidermolysis bullosa acquisita ,Unusual case ,integumentary system ,business.industry ,IIF, indirect immunofluorescence ,Autoantibody ,Mucous membrane ,IIf ,MM-EBA, mucous membrane epidermolysis bullosa acquisita ,medicine.disease ,drug rash with eosinophilia and systemic symptoms ,DRESS, drug rash with eosinophilia and systemic symptoms ,IVIG, intravenous immunoglobulin ,Pediatric patient ,medicine.anatomical_structure ,RL1-803 ,medicine.symptom ,business ,DRESS - Abstract
Autoimmune diseases have been reported to develop months to years following drug rash with eosinophilia and systemic symptoms (DRESS) syndrome. However, the development of autoimmune bullous diseases in a patient with a history of DRESS syndrome is extremely rare. We here report the unusual case of mucous membrane epidermolysis bullosa acquisita (MM-EBA) presenting 2 years after lamotrigine-induced DRESS syndrome in a pediatric patient. The patient also developed nonpathogenic autoantibodies directed against BP 180 and BP 230.
- Published
- 2021
20. Vulvovaginal pyoderma gangrenosum associated with rituximab use in 2 patients with rheumatoid arthritis
- Author
-
Jason R. Sotzen and Erik J. Stratman
- Subjects
medicine.medical_specialty ,PG, pyoderma gangrenosum ,business.industry ,RA, rheumatoid arthritis ,Case Report ,Dermatology ,immunomodulatory medications ,lcsh:RL1-803 ,medicine.disease ,rituximab ,RA - Rheumatoid arthritis ,Neutrophilic dermatosis ,Rheumatoid arthritis ,neutrophilic dermatosis ,medicine ,lcsh:Dermatology ,IVIg, intravenous immunoglobulin ,Rituximab ,business ,Pyoderma gangrenosum ,medicine.drug ,pyoderma gangrenosum - Published
- 2021
21. Therapeutic antibodies – natural and pathological barriers and strategies to overcome them
- Author
-
Nicolas Joubert, Aubin Pitiot, Nathalie Heuzé-Vourc'h, Matthieu Gracia, Caroline Denevault-Sabourin, Timothée Blin, Thomas Sécher, Jean-Pierre Pouget, Juliette Lamamy, Sophie Poty, Valérie Gouilleux-Gruart, Débora Lanznaster, Yara Al Ojaimi, Imagerie et cerveau (iBrain - Inserm U1253 - UNIV Tours ), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Groupe innovation et ciblage cellulaire (GICC), EA 7501 [2018-...] (GICC EA 7501), Université de Tours (UT), Institut de Radiobiologie Cellulaire et Moléculaire (IRCM), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, Institut du Cancer de Montpellier (ICM), Le Pennec, Deborah, and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Tours (UT)
- Subjects
pI, isoelectric point ,EU, European union ,TNF, tumour necrosis factor ,US, United States ,Recombinant antibodies ,Medicine ,Pharmacology (medical) ,HER2, human epidermal growth factor receptor 2 ,Patient comfort ,Target antigen ,FcRn, neonatal Fc receptor ,biology ,GI, gastrointestinal ,VEGF, vascular endothelial growth factor ,ECM, extracellular matrix ,IFP, interstitial fluid pressure ,MMP, matrix metalloproteinase ,Therapeutic strategies ,Fab, fragment antigen-binding ,RNA, Viral ,AD, Alzheimer's disease ,Antibody ,2019-20 coronavirus outbreak ,medicine.medical_specialty ,BBB, blood-brain barrier ,IA, intra-articular ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,IgG, immunoglobulin G ,CNS, central nervous system ,ADC, antibody-drug conjugate ,PK, pharmacokinetic ,Antibodies ,Therapeutic index ,PD, pharmacodynamics ,Biological barriers ,[SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology ,IVIg, intravenous immunoglobulin ,Humans ,Immunologic Factors ,[SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology ,mAb, monoclonal antibody ,Intensive care medicine ,Pandemics ,Pharmacology ,business.industry ,SARS-CoV-2 ,IdeS, bacteria secreted proteases ,scFv, single-chain variable fragment ,Pathological barriers ,Aβ, amyloid-β ,FDA, Food and Drug Administration ,COVID-19 Drug Treatment ,Invited Article ,biology.protein ,Fc, fragment crystallizable ,business ,Site of action - Abstract
International audience; Antibody-based therapeutics have become a major class of therapeutics with over 120 recombinant antibodies approved or under review in the EU or US. This therapeutic class has experienced a remarkable expansion with an expected acceleration in 2021-2022 due to the extraordinary global response to SARS-CoV2 pandemic and the public disclosure of over a hundred anti-SARS-CoV2 antibodies. Mainly delivered intravenously, alternative delivery routes have emerged to improve antibody therapeutic index and patient comfort. A major hurdle for antibody delivery and efficacy as well as the development of alternative administration routes, is to understand the different natural and pathological barriers that antibodies face as soon as they enter the body up to the moment they bind to their target antigen. In this review, we discuss the well-known and more under-investigated extracellular and cellular barriers faced by antibodies. We also discuss some of the strategies developed in the recent years to overcome these barriers and increase antibody delivery to its site of action. A better understanding of the biological barriers that antibodies have to face will allow the optimization of antibody delivery near its target. This opens the way to the development of improved therapy with less systemic side effects and increased patients' adherence to the treatment.
- Published
- 2021
22. Vaccine induced thrombotic thrombocytopenia: The shady chapter of a success story
- Author
-
Irene Karampela, Dimitrios Tsilingiris, Maria Dalamaga, and Natalia G Vallianou
- Subjects
Coronavirus disease 2019 (COVID-19) ,Physiology ,TTS, thrombosis-thrombocytopenia-syndrome ,aPTT, activated partial thromboplastin time ,QD415-436 ,CAR, Coxsackie-adenovirus receptor ,VITT, vaccine induced thrombotic thrombocytopenia ,HIT, Heparin-induced thrombocytopenia ,Biochemistry ,Article ,Viral vector ,VCAM-1, vascular cell adhesion molecule 1 ,PF4, Platelet factor 4 ,SVT, splanchnic vein thrombosis ,ICU, Intensive Care Unit ,PT, prothrombin time ,Rare syndrome ,Medicine ,Adenovirus ,QP1-981 ,SARS-Cov-2, severe acute respiratory syndrome coronavirus 2 ,Platelet ,Adenoviral vector ,VIPIT, vaccine-induced prothrombotic immune thrombocytopenia ,CDC, Centers for Disease Control and Prevention ,ΕΜΑ, European Medicines Agency ,COVID-19, Coronavirus disease 2019 ,biology ,business.industry ,SARS-CoV-2 ,Autoantibody ,COVID-19 ,Vaccine induced thrombotic thrombocytopenia ,FDA, Food and Drug Administration ,PRAC, Pharmacovigilance Risk Assessment Committee ,LMWH, low molecular weight heparin ,Vaccination ,PLT, Platelet ,Immunology ,biology.protein ,Antibody ,Complication ,business ,CVST, cerebellar sinus thrombosis ,Vaccine ,IVIG, Intravenous immunoglobulin - Abstract
The recognition of the rare but serious and potentially lethal complication of vaccine induced thrombotic thrombocytopenia (VITT) raised concerns regarding the safety of COVID-19 vaccines and led to the reconsideration of vaccination strategies in many countries. Following the description of VITT among recipients of adenoviral vector ChAdOx1 vaccine, a review of similar cases after Ad26.COV2·S vaccination gave rise to the question whether this entity may constitute a potential class effect of all adenoviral vector vaccines. Most cases are females, typically younger than 60 years who present shortly (range: 5-30 days) following vaccination with thrombocytopenia and thrombotic manifestations, occasionally in multiple sites. Following initial incertitude, concrete recommendations to guide the diagnosis (clinical suspicion, initial laboratory screening, PF4-polyanion-antibody ELISA) and management of VITT (non-heparin anticoagulants, corticosteroids, intravenous immunoglobulin) have been issued. The mechanisms behind this rare syndrome are currently a subject of active research and include the following: 1) production of PF4-polyanion autoantibodies; 2) adenoviral vector entry in megacaryocytes and subsequent expression of spike protein on platelet surface; 3) direct platelet and endothelial cell binding and activation by the adenoviral vector; 4) activation of endothelial and inflammatory cells by the PF4-polyanion autoantibodies; 5) the presence of an inflammatory co-signal; and 6) the abundance of circulating soluble spike protein variants following vaccination. Apart from the analysis of potential underlying mechanisms, this review aims to synopsize the clinical and epidemiologic features of VITT, to present the current evidence-based recommendations on diagnostic and therapeutic work-up of VITT and to discuss new dilemmas and perspectives that emerged after the description of this entity.
- Published
- 2021
23. Summary report of seven cases of COVID-19 infection in renal transplant recipients
- Author
-
Xiaoxiong Ma, Zeya Jin, Long Zhang, Tianyu Wang, Yalong Zhang, Jiangqiao Zhou, Jilin Zou, Zhongbao Chen, Tao Qiu, and Yan Yuan
- Subjects
Male ,BP, blood pressure ,MP, methylprednisolone ,SARS-CoV2, severe acute respiratory syndrome-Coronavirus 2 ,Antibiotics ,Cr, creatine ,ESRD, end-stage renal disease ,PaO2, partial pressure of oxygen ,Immunology and Allergy ,Medicine ,DCD, donation after cardiac death ,BNP, brain natriuretic peptide ,SPO2, pulse oxygen saturation ,biology ,HR, heart rate ,eGFR, estimated glomerular filtration rate ,Acute kidney injury ,Immunoglobulins, Intravenous ,Acute Kidney Injury ,Middle Aged ,Prognosis ,ICU, intensive care unit ,PCT, procalcitonin ,Management ,CT, computed tomography ,Renal transplant ,Methylprednisolone ,CNI, calcineurin inhibitor ,COVID-19, Coronavirus Disease 2019 ,CRP, C-reactive protein ,WHO, world health organization ,Female ,Antibody ,medicine.symptom ,Immunosuppressive Agents ,medicine.drug ,WBC, white blood cell ,Adult ,medicine.medical_specialty ,Isolation (health care) ,medicine.drug_class ,Immunology ,Inflammation ,AKI, acute kidney injury ,NAT, nucleic acid testing ,Antiviral Agents ,Article ,RTRs, renal transplant recipients ,Immunocompromised Host ,Immunity ,Internal medicine ,Humans ,CRRT, continuous renal replacement therapy ,PJP, Pneumocystis jiroveci pneumonia ,COVID-19 Serotherapy ,ARDS, acute respiratory distress syndrome ,FiO2, fraction of inspiration O2 ,Transplantation ,business.industry ,SARS-CoV-2 ,Immunization, Passive ,COVID-19 ,CMV, cytomegalovirus ,medicine.disease ,Kidney Transplantation ,Transplant Recipients ,COVID-19 Drug Treatment ,Treatment ,IVIG, intravenous immunoglobulin ,biology.protein ,ADPKD, autosomal dominant polycystic kidney disease ,MMF, mycophenolate mofetil ,business ,Asymptomatic carrier - Abstract
The coronavirus disease 2019 (COVID-19) has swept the world, posing a serious threat to people's lives and health. Several cases of COVID-19 infection in renal transplant recipients (RTRs) have been reported, but the treatment and prognosis have not been fully elucidated. We followed-up with RTRs infected with SARS-CoV2 in our center and classified them as five clinical types-asymptomatic, mild, moderate, severe, and critical. The immunosuppressive agents were not adjusted in asymptomatic carriers and mild patients, the former was mainly treated by isolation, and the latter was treated by low-dose intravenous immunoglobulin (IVIG) to enhance immunity. For moderate or severe patients, the immunosuppressive agents were largely reduced or even interrupted, low-dose IVIG was adopted, and low-dose methylprednisolone (MP) was used to inhibit inflammation and rejection. Immunosuppressants were discontinued early in critical patients; IVIG, high-dose MP, and antibiotics were used. Meanwhile, all patients received at least one antiviral drugs. After aggressive treatment, three patients developed acute kidney injury, and two showed reversal, while the remaining one lost the allograft kidney; one patient died, while other patients were discharged. For different clinical types of RTRs infected with COVID-19, personalized therapies were essential, Meanwhile, patients with COVID-19 infection may have different outcomes due to their different clinical manifestations.
- Published
- 2021
24. Multisystem Inflammatory Syndrome therapies in children (MISTIC): A randomized trial.
- Author
-
Jain S, He F, Brown K, Burns JC, and Tremoulet AH
- Abstract
Background: Multisystem Inflammatory Syndrome in Children (MIS-C), which occurs 2-6 weeks after initial exposure to SARS-CoV-2, was first identified in early 2020 when patients presented with fever and significant inflammation, often requiring management in the intensive care unit. To date, there has been no clinical trial to determine the most effective treatment. This study compares anti-inflammatory treatments that were selected based on current treatments for Kawasaki disease, a coronary artery vasculitis that shares many clinical features with MIS-C., Methods: This randomized, comparative effectiveness trial of children with MIS-C uses the small N Sequential Multiple Assignment Randomized Trial (snSMART) design for rare diseases to compare multiple therapies within an individual. Study participants were treated first with intravenous immunoglobulin (IVIG), and if needed, subjects were then randomized to one of three additional treatments (steroids, anakinra, or infliximab). Participants were re-randomized to remaining treatments if they did not demonstrate clinical improvement., Conclusion: This trial continues to enroll eligible participants to determine the most effective therapies in addition to IVIG and best order in which to use them to treat MIS-C., Trial Registration: NCT04898231., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors.)
- Published
- 2023
- Full Text
- View/download PDF
25. HTLV-1-associated demyelinating neuropathy: A case report and review of the literature.
- Author
-
Tamaki K, Mishima T, Tateishi Y, Mera H, Ogura H, Tsugawa J, Fujioka S, Takamatsu Y, and Tsuboi Y
- Abstract
A 78-year-old man developed paresthesias in the extremities. He was referred to our hospital because of positive anti-human T-cell leukemia virus type 1 (HTLV-1) antibodies in the serum and the presence of abnormal lymphocytes. He was diagnosed as chronic-type adult T-cell leukemia/lymphoma. Neurological examination revealed sensory impairment in the distal parts of the extremities with loss of deep tendon reflexes. Nerve conduction study showed motor and sensory demyelinating polyneuropathy, indicating a diagnosis of HTLV-1-associated demyelinating neuropathy. Corticosteroid therapy followed by intravenous immunoglobulin therapy improved his symptoms. Since demyelinating neuropathy associated with HTLV-1 infection is not well recognized, we here report its characteristics and clinical course through our case report and literature review., Competing Interests: The authors declare no conflict of interest for this article., (© 2023 The Authors.)
- Published
- 2023
- Full Text
- View/download PDF
26. Guidelines on the treatment with integrated traditional Chinese medicine and western medicine for severe coronavirus disease 2019
- Author
-
Qiuping Liu, Hui Lan, Yue-Peng Jiang, Jianjian Wang, Shan Fang, Wu Chen, An-Ran Xi, Haichang Li, Shouyuan Wu, Yi-Ni Gao, Yaolong Chen, Wenqing Luo, Zhiyu Li, Jiao Chen, Wei-Dong Lai, Xia-Feng Zhang, Yu Lou, Lin Li, Xianghui Wen, Si-Yue Song, Ming Zhang, Ke-Er Wang, Qiangqiang Guo, Yi Zhang, Zhijun Xie, Ming-Qian Zhou, Feng-Qi Zhang, Yan Gao, Yuan-Fang He, Dian-Ming Li, Chengping Wen, Xiao-Feng Xu, and Juanjuan Zhang
- Subjects
Standardization ,Delphi Technique ,medicine.medical_treatment ,MERS, Middle East Respiratory Syndrome ,Traditional Chinese medicine ,YQYY, Yiqi Yangyin ,Guideline ,JHQG, Jinhua Qinggan ,YYQR, Yangyin Qingre ,SARS, Severe Acute Respiratory Syndrome ,Health care ,Medicine ,IOM, Institute of Medicine ,Medicine, Chinese Traditional ,Grading (education) ,HSBD, Huashi Baidu ,PICO, participants, intervention, comparison, outcome ,Infectious Disease Medicine ,Rehabilitation ,Evidence-Based Medicine ,PHEIC, Public Health Emergency of International Concern ,RIGHT, Reporting Items for Practice Guidelines in Healthcare ,TCZ, tocilizumab ,UC, usual care ,Treatment Outcome ,COVID-19, Coronavirus Disease 2019 ,Host-Pathogen Interactions ,XFBD, Xuanfei Baidu ,XBJ, Xuebijing ,medicine.medical_specialty ,Consensus ,Coronavirus disease 2019 (COVID-19) ,HXZQ, Huoxiang Zhengqi ,Antiviral Agents ,Article ,WHO, World Health Organization ,QFPD, Qingfei Paidu ,EEG, the evidence evaluation group ,Integrated Chinese and western medicine ,IVIg, intravenous immunoglobulin ,Humans ,TCM, traditional Chinese medicines ,Intensive care medicine ,GRADE, Grades of recommendations, assessment, development, and evaluation ,Pharmacology ,GDG, the guideline development group ,business.industry ,SARS-CoV-2 ,AGREE, Appraisal of Guidelines for Research and Evaluation ,Public health ,Patient Acuity ,COVID-19 ,NIV, non-invasive ventilation ,AMSTAR, Assessing the Methodological Quality of Systematic Reviews ,Lpv/R, lopinavir/ritonavir ,HCQ, hydroxychloroquine ,NHCPRC, National Health Commission of the People's Republic of China ,BFJP, Bufei Jianpi ,RCT, Randomized control trial ,COVID-19 Drug Treatment ,CPMs, Chinese patent medicines ,IMV, invasive mechanical ventilation ,FHN, femoral head necrosis ,business ,HFNC, high flow nasal cannula ,LHQW, Lianhua Qingwen ,Drugs, Chinese Herbal - Abstract
Severe Coronavirus Disease 2019 (COVID-19) is characterized by numerous complications, complex disease, and high mortality, making its treatment a top priority in the treatment of COVID-19. Integrated traditional Chinese medicine (TCM) and western medicine played an important role in the prevention, treatment, and rehabilitation of COVID-19 during the epidemic. However, currently there are no evidence-based guidelines for the integrated treatment of severe COVID-19 with TCM and western medicine. Therefore, it is important to develop an evidence-based guideline on the treatment of severe COVID-19 with integrated TCM and western medicine, in order to provide clinical guidance and decision basis for healthcare professionals, public health personnel, and scientific researchers involved in the diagnosis, treatment, and care of COVID-19 patients. We developed and completed the guideline by referring to the standardization process of the “WHO handbook for guideline development”, the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system, and the Reporting Items for Practice Guidelines in Healthcare (RIGHT)., Graphical Abstract ga1
- Published
- 2021
27. Mechanisms underlying genetic susceptibility to Multisystem Inflammatory Syndrome in Children (MIS-C)
- Author
-
David A. Williams, Leah Cheng, Janet Chou, Sabrina Weeks, Saddiq Habiballah, Megan Elkins, Faris Jaber, Mary Beth F. Son, Alan A. Nguyen, Lucinda Williams, Megan Day-Lewis, Tina Banzon, Zachary Peters, Stacy A. Kahn, Raif S. Geha, Pui Y. Lee, Lauren A. Henderson, Craig D. Platt, Mindy S. Lo, Tanya Novak, Jordan E Roberts, Abduarahman Almutairi, Adrienne G. Randolph, Olha Halyabar, Piotr Sliz, Myriam Armant, and Shira Rockowitz
- Subjects
CRP, C reactive protein ,Male ,RT-PCR, reverse transcriptase-polymerase chain reaction ,whole exome sequencing ,NOD2 ,Immunology and Allergy ,Medicine ,Prospective cohort study ,Child ,Exome sequencing ,COVID-19, coronavirus disease 2019 ,education.field_of_study ,Systemic Inflammatory Response Syndrome ,Child, Preschool ,Host-Pathogen Interactions ,XIAP, X-linked inhibitor of apoptosis ,Cytokines ,Female ,Disease Susceptibility ,Haploinsufficiency ,WES, whole exome sequencing ,NOD, nucleotide-binding oligomerization domain-containing ,Immunology ,Population ,HLH, hemophagocytic lymphohistiocytosis ,MIS-C ,SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 ,Article ,Multisystem Inflammatory Syndrome in Children ,CYBB, Cytochrome b(-245) ,EBV, Epstein Barr virus ,Genetic predisposition ,Humans ,IFN, interferon ,Genetic Predisposition to Disease ,CYBB ,education ,Hemophagocytic lymphohistiocytosis ,business.industry ,SARS-CoV-2 ,COVID-19 ,CGD, chronic granulomatous disease ,medicine.disease ,IVIG, intravenous immunoglobulin ,NGS, next-generation sequencing ,SOCS1, Suppressor of cytokine signaling 1 ,MIS-C, multisystem inflammatory syndrome in children ,business ,Biomarkers - Abstract
Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a pediatric complication of SARS-CoV-2 infection characterized by multiorgan inflammation and frequently, cardiovascular dysfunction. It occurs predominantly in otherwise healthy children. We previously reported haploinsufficiency of Suppressor of Cytokine Signaling 1 (SOCS1), a negative regulator of Type I and II interferons, as a genetic risk factor for MIS-C. Objectives We aimed to identify additional genetic mechanisms underlying susceptibility to SARS-CoV-2-associated MIS-C. Methods In a single center, prospective cohort study, whole exome sequencing was performed on patients with MIS-C. The impact of candidate variants was tested using patients’ peripheral blood mononuclear cells obtained at least seven months after recovery. Results We enrolled 18 patients with MIS-C (median age: 8 years, IQR 5 – 12.25 years), of whom 89% had no conditions other than obesity. In two boys with no significant infection history, we identified and validated hemizygous, deleterious defects in XIAP, encoding X-linked inhibitor of apoptosis, and CYBB, encoding cytochrome b-245. Including the previously reported SOCS1 haploinsufficiency, a genetic diagnosis was identified in three (17%) of 18 patients. Even after recovery, patients with defects in SOCS1, XIAP, or CYBB exhibit an inflammatory immune cell transcriptome with enrichment of differentially expressed genes in pathways downstream of IL-18, oncostatin M, and NF-κB, compared to those with mild COVID-19. Conclusions Although inflammatory disorders are rare in the general population, our cohort of patients with MIS-C was enriched for monogenic susceptibility to inflammation. Our results support the use of next-generation sequencing in previously healthy children who develop MIS-C., Graphical abstract, Capsule Summary: Hypomorphic inborn errors of immunity may predispose children to SARS-CoV-2-associated Multisystem Inflammatory Syndrome in Children (MIS-C).
- Published
- 2021
28. Longitudinally extensive transverse myelitis as a sign of multisystem inflammatory syndrome following COVID-19 infection: A pediatric case report
- Author
-
Arastoo Vossough, Seyedeh Zahra Tara, Mohammad Saadatnia, Omid Yaghini, Hamid Rahimi, Atefeh Sadeghizadeh, Niloofar Javadi, Zahra Pourmoghaddas, Rana Saleh, Alireza Zandifar, Neda Hosseini, and Mohammad-Reza Ghazavi
- Subjects
medicine.medical_specialty ,Coronavirus disease 2019 (COVID-19) ,Short Communication ,Immunology ,Transverse myelitis ,Multi-System Inflammatory Syndrome in Children, MIS-C ,Myelitis, Transverse ,SARS-CoV-2, Severe acute respiratory syndrome coronavirus-2 ,medicine ,Paralysis ,Immunology and Allergy ,Humans ,TNF-α, tumor necrosis factor alpha ,TM, Transverse myelitis ,Child ,Pediatric ,COVID-19, Coronavirus disease 2019 ,business.industry ,LETM, longitudinally extensive transverse myelitis ,COVID-19 ,CMV, cytomegalovirus ,medicine.disease ,Spinal cord ,Dermatology ,Infliximab ,Systemic Inflammatory Response Syndrome ,COVID-19 Drug Treatment ,Systemic inflammatory response syndrome ,Multi-System Inflammatory Syndrome ,IVIG, intravenous immunoglobulin ,medicine.anatomical_structure ,Neurology ,Methylprednisolone ,Antirheumatic Agents ,Female ,Neurology (clinical) ,Immunotherapy ,medicine.symptom ,business ,medicine.drug ,Pediatric population - Abstract
COVID-19 infection can cause inflammatory reactions that could involve several organs. In the pediatric population, Multi-System Inflammatory Syndrome in Children (MIS-C) has been reported as one of the consequences of COVID-19. We report a unique pediatric COVID-19 patient with MIS-C, associated with paralysis of the extremities. MRI showed abnormal signal in the cervical spinal cord compatible with transverse myelitis. Methylprednisolone and IVIG were administered, without significant symptom improvement. As a next step, Infliximab was tried for her, and she responded remarkably well to this treatment. Infliximab may be considered as a treatment option in COVID-19 patients with transverse myelitis.
- Published
- 2021
29. Cutaneous Manifestations of COVID-19 in the Inpatient Setting
- Author
-
Claire R. Stewart, Joanna Harp, and Mytrang H. Do
- Subjects
Adult ,medicine.medical_specialty ,Pityriasis Rosea ,Coronavirus disease 2019 (COVID-19) ,Urticaria ,Mucocutaneous zone ,MIS-C ,Dermatology ,Disease ,Skin Diseases, Vascular ,ACE2, angiotensin-converting enzyme 2 ,Skin Diseases ,inpatient ,SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 ,Article ,medicine ,Humans ,complement ,DIC, disseminated intravascular coagulation ,Child ,vasculopathy ,COVID-19, coronavirus disease 2019 ,Inpatients ,business.industry ,cutaneous ,COVID-19 ,EM, erythema multiforme ,Viral Exanthem ,Livedo racemosa ,Inpatient setting ,Exanthema ,KD, Kawasaki disease ,Rash ,Systemic Inflammatory Response Syndrome ,Chilblains ,IVIG, intravenous immunoglobulin ,Purpura ,medicine.symptom ,MIS-C, multisystem inflammatory syndrome in children ,business ,viral exanthem - Abstract
Adults and children hospitalized with COVID-19 display a range of mucocutaneous eruptions, the diversity of which is likely due to the varying immune response generated in response to SARS-CoV-2 infection. In the hospitalized patient, livedo racemosa and retiform purpura were associated with more severe disease course, poorer prognosis, prolonged hospitalization, and higher mortality. In contrast, most viral exanthem and inflammatory lesions, such as urticarial and vesicular eruptions, were associated with a less severe COVID-19 disease course, although were nevertheless reported more frequently in the inpatient setting. One exception, however, is the presence of an inflammatory rash associated with MIS-C in the inpatient pediatric population, which has been associated with more severe COVID-19 disease and disease sequelae. Prompt recognition of the mucocutaneous manifestations presented in this article is paramount to facilitate timely diagnosis, proper treatment, and accurate prognostication of clinical course.
- Published
- 2021
30. Juvenile Dermatomyositis triggered by SARS-CoV-2 in a Mexican Child
- Author
-
Liquidano-Perez, Eduardo, García-Romero, María Teresa, Yamazaki-Nakashimada, Marco, Maza-Morales, Mariana, Rivas-Calderón, Marian K., Bayardo-Gutierrez, Beatriz, Pardo-Díaz, Edwin, and Scheffler-Mendoza, Selma C.
- Subjects
LDH, Lactate dehydrogenase ,SARS-CoV-2 ,Juvenile Dermatomyositis ,ALT, Alanine aminotransferase ,CPK, Creatine phosphokinase ,IgG, immunoglobulin G ,COVID-19 ,JDM, Juvenile Dermatomyositis ,AST, Aspartate transaminase ,IVIG, intravenous immunoglobulin ,SARS-CoV-2, Severe Acute Respiratory Syndrome Coronavirus 2 ,pro-BNP, pro-brain natriuretic peptide ,EBV, Epstein Barr virus ,HIV, Human immunodeficiency virus ,COVID-19, Coronavirus Disease 2019 ,Correspondence ,RT-PCR, real-time reverse transcription polymerase chain reaction ,MRI, magnetic resonance imaging - Published
- 2021
31. Multisystem Inflammatory Syndrome in Adults: A Rare Sequela of SARS-CoV-2 Infection
- Author
-
Douglas R. Moore, Arslan Ahmed, Renuga Vivekanandan, Manasa Velagapudi, Faran Ahmad, Robert Plambeck, Joseph A. Nahas, Austin Loranger, Sanu Rajendraprasad, and Sonia Gupta
- Subjects
0301 basic medicine ,Pediatrics ,Case Report ,Disease ,Infectious and parasitic diseases ,RC109-216 ,law.invention ,0302 clinical medicine ,SCAD, Spontaneous coronary artery dissection ,law ,AF, Atrial fibrillation ,030212 general & internal medicine ,RT- PCR, Reverse transcriptase-polymerase chain reaction ,CRRT, Continuous renal replacement therapy ,Clinical course ,VA-ECMO, Veno-arterial extracorporeal membrane oxygenation ,VF, Ventricular fibrillation ,General Medicine ,Intensive care unit ,MMWR, Morbidity and mortality weekly report ,ICU, Intensive care unit ,Infectious Diseases ,COVID-19, Coronavirus Disease 2019 ,CRP, C-reactive protein ,SARS-CoV-2 Infection ,medicine.drug ,Microbiology (medical) ,medicine.medical_specialty ,Coronavirus disease 2019 (COVID-19) ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,030106 microbiology ,TTE, Transthoracic echocardiogram ,03 medical and health sciences ,CDC, Center for Disease Control and Prevention ,medicine ,otorhinolaryngologic diseases ,Anakinra ,LDH, Lactate Dehydrogenase ,business.industry ,MIS-A, Multisystem Inflammatory Syndrome in Adults ,Multisystem Inflammatory Syndrome in Adults (MIS-A) ,COVID-19 ,Sequela ,medicine.disease ,MIS, Multisystem inflammatory syndrome ,LVEF, Left ventricular ejection fraction ,Heart failure ,LHC, Left heart catheterization ,business ,IVIG, Intravenous immunoglobulin - Abstract
Multisystem inflammatory syndrome in adults (MIS-A) came to attention back in June 2020, when the United States Center for Disease Control and Prevention (CDC) received initial reports regarding patients who had presented delayed and multisystem involvement of the disease, with clinical course resembling multisystem inflammatory syndrome in children (MIS-C). This study introduces a case of MIS-A, where the patient presented 3 weeks after initial COVID-19 exposure. His clinical course was consistent with the working definition of MIS-A as specified by the CDC. Aggressive supportive care in the intensive care unit, utilization of advanced heart failure devices, and immunomodulatory therapeutics (high-dose steroids, anakinra, intravenous immunoglobulin) led to clinical recovery. Management of MIS-A is a topic of ongoing research and needs more studies to elaborate on treatment modalities and clinical predictors.
- Published
- 2021
32. Thymoma-associated multiorgan autoimmunity initially manifested by graft-versus-host disease–like erythroderma: Case report and possible therapeutic role of antimalarial drugs
- Author
-
Taylor Novice, Milad Eshaq, Jeanne Kochkodan, Johann E. Gudjonsson, Grace A. Hile, and May P. Chan
- Subjects
hydroxychloroquine ,Thymoma ,Erythroderma ,Case Report ,Dermatology ,GVHD, graft-versus-host disease ,medicine.disease_cause ,Autoimmunity ,IVIg, intravenous immunoglobulin ,lcsh:Dermatology ,medicine ,graft-versus-host–like disease ,GVHD - Graft-versus-host disease ,Toll-like receptor ,MGT, modified Goeckerman therapy ,business.industry ,erythroderma ,Hydroxychloroquine ,thymoma ,lcsh:RL1-803 ,medicine.disease ,TAMA, thymoma-associated multiorgan autoimmunity ,Graft-versus-host disease ,Immunology ,paraneoplastic ,business ,TLR, Toll-like receptor ,medicine.drug - Published
- 2020
33. Refractory mucositis associated with underlying follicular dendritic cell sarcoma of the thymus: Paraneoplastic pemphigus versus malignancy-exacerbated pemphigus vulgaris
- Author
-
Brittney Schultz, Laurel L. Wessman, Daniel D. Miller, Alexandra Streifel, and David R. Pearson
- Subjects
Pathology ,medicine.medical_specialty ,Thymoma ,pemphigus vulgaris ,Case Report ,Dermatology ,Malignancy ,rituximab ,medicine ,Mucositis ,business.industry ,Pemphigus vulgaris ,IIF, indirect immunofluorescence ,PNP, paraneoplastic pemphigus ,pemphigus ,BMZ, basement membrane zone ,ELISA, enzyme-linked immunosorbent assay ,thymoma ,medicine.disease ,myasthenia ,IVIG, intravenous immunoglobulin ,PV, pemphigus vulgaris ,Pemphigus ,Paraneoplastic pemphigus ,Follicular dendritic cell sarcoma ,FDCS, follicular dendritic cell sarcoma ,Rituximab ,business ,medicine.drug - Published
- 2019
34. Influenza A (H3N2) Induced Fulminant Myocarditis Requiring Mechanical Circulatory Support
- Author
-
John Hollowed and Ali Nsair
- Subjects
LVEF ,VV-ECMO ,Fulminant ,medicine.medical_treatment ,TTE, transthoracic echocardiogram ,Cardiomyopathy ,venovenous-arterial extracorporeal membranous oxygenation ,Hemodynamics ,PEA, pulseless electrical activity ,Case Report ,Cardiovascular ,hemodynamics ,creatinine kinase ,VA-ECMO, veno-arterial extracorporeal membranous oxygenation ,intravenous immunoglobulin ,LV ,LVEF, left ventricular ejection fraction ,VV-ECMO, venovenous extracorporeal membranous oxygenation ,VVA-ECMO ,VAV-ECMO, veno-arterial-venous extracorporeal membranous oxygenation ,virus diseases ,VVA-ECMO, venovenous-arterial extracorporeal membranous oxygenation ,left ventricular ejection fraction ,Heart Disease ,Infectious Diseases ,surgical procedures, operative ,CK ,Circulatory system ,Pneumonia & Influenza ,cardiovascular system ,Cardiology ,TTE ,Tamponade ,reduced ejection fraction ,ECMO ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,transthoracic echocardiogram ,medicine.medical_specialty ,Myocarditis ,left ventricle ,Vaccine Related ,ECMO, extracorporeal membranous oxygenation ,Clinical Case ,Biodefense ,Internal medicine ,VA-ECMO ,medicine ,Extracorporeal membrane oxygenation ,tamponade ,Diseases of the circulatory (Cardiovascular) system ,CK, creatinine kinase ,pulseless electrical activity ,IVIG ,veno-arterial-venous extracorporeal membranous oxygenation ,business.industry ,Prevention ,PEA ,systolic heart failure ,veno-arterial extracorporeal membranous oxygenation ,Hypoxia (medical) ,medicine.disease ,VAV-ECMO ,Influenza ,extracorporeal membranous oxygenation ,respiratory tract diseases ,IVIG, intravenous immunoglobulin ,Emerging Infectious Diseases ,LV, left ventricle ,RC666-701 ,business ,cardiac assist devices ,cardiomyopathy ,venovenous extracorporeal membranous oxygenation - Abstract
The authors report a case of fulminant myocarditis from an influenza A (H3N2) infection in a healthy individual who experienced cardiac arrest…, The authors report a case of fulminant myocarditis from an influenza A (H3N2) infection in a healthy individual who experienced cardiac arrest requiring extracorporeal membrane oxygenation (ECMO). The case highlights the management of complications arising from the use of ECMO including differential hypoxia and left ventricular overload requiring left ventricular venting. (Level of Difficulty: Beginner.), Central Illustration
- Published
- 2019
35. Flare of eosinophilic granulomatosis with polyangiitis related to pregnancy: Case report and review of the literature
- Author
-
Osamu Matsuno and Seijiro Minamoto
- Subjects
Pulmonary and Respiratory Medicine ,Pediatrics ,medicine.medical_specialty ,Case Report ,Disease ,CNS, central nervous system ,03 medical and health sciences ,0302 clinical medicine ,Pregnancy ,Eosinophilic ,medicine ,Relapse ,EGPA, eosinophilic granulomatosis with polyangiitis ,Asthma ,lcsh:RC705-779 ,Fetus ,business.industry ,Eosinophilic granulomatosis with polyangiitis ,Non-reassuring fetal status ,lcsh:Diseases of the respiratory system ,Eosinophil ,medicine.disease ,MPO-ANCA, anti-neutrophil cytoplasmic antibodies against myeloperoxidase ,IVIG, intravenous immunoglobulin ,medicine.anatomical_structure ,030228 respiratory system ,030220 oncology & carcinogenesis ,Elective caesarean section ,Complication ,Granulomatosis with polyangiitis ,business - Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by excessive eosinophil accumulation in the peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. It is strongly associated with asthma and ear-nose-throat disease. It often affects patients between the ages of 40 and 60 years. It is unknown whether pregnancy impacts the disease activity of EGPA, including initial diagnosis or relapse. Because of its rarity and age of susceptibility, there are few reported cases describing pregnancy in women with quiescent or active EGPA. Here, we describe a young woman who experienced EGPA relapse during pregnancy and subsequently underwent an elective caesarean section for non-reassuring fetal status at 37 weeks without complication. Keywords: Eosinophilic granulomatosis with polyangiitis, Pregnancy, Relapse, Elective caesarean section, Non-reassuring fetal status
- Published
- 2018
36. Cutaneous mucormycosis in a chronic lymphocytic leukemia patient on ibrutinib
- Author
-
Geoffrey C. Wall, Katherine R. Sittig, Zaheer Akhtar, Sudhir C. Kumar, and Leah Laageide
- Subjects
0301 basic medicine ,Mucorales ,Posaconazole ,medicine.medical_specialty ,CLL, Chronic Lymphocytic Leukemia ,Chronic lymphocytic leukemia ,030106 microbiology ,Case Report ,Infectious and parasitic diseases ,RC109-216 ,PAS, Periodic Acid Schiff ,14-alpha demethylase inhibitors ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Prednisone ,IVIG, Intravenous Immunoglobulin ,Amphotericin B ,medicine ,Mucormycosis ,030212 general & internal medicine ,PCR, Polymerase Chain Reaction ,Chronic ,Antifungal agents ,Leukemia ,biology ,business.industry ,Ibrutinib ,medicine.disease ,biology.organism_classification ,Dermatology ,Lymphocytic ,CNS, Central Nervous System ,Infectious Diseases ,chemistry ,business ,BTK, Bruton’s Tyrosine Kinase ,PCP, Primary Care Physician ,AFB, Acid-Fast Bacilli ,medicine.drug - Abstract
Highlights • Mucorales known to cause opportunistic infections in immunocompromised hosts. • This is the 2nd reported case of cutaneous mucormycosis in a patient on ibrutinib. • Amphotericin B is typical 1st line therapy. • We report successful completion of treatment of cutaneous mucormycosis with posaconazole., Background Mucorales is a zygomycete fungi known to cause opportunistic infections in immunosuppressed hosts. Spores may be inhaled, causing rhinocerebral or pulmonary infections, or gastrointestinal infections if swallowed. Less often, cutaneous mucormycosis develops after inoculation via broken skin. Presentation A 72-year old male on ibrutinib and prednisone for chronic lymphocytic leukemia (CLL) presented with localized, right forearm cutaneous mucormycosis at the site of a dog-scratch sustained three weeks prior. The patient failed to respond to cephalexin as an outpatient, prompting biopsy showing ribbon-like pseudo septate hyphae and possible vascular invasion suggestive of Mucorales. Treatment course included liposomal amphotericin B 5 mg/kg IV every 24 h for ten days followed by a 90-day course of posaconazole 300 mg daily after general surgery consultation was sought. Conclusion We outline the second reported case of localized cutaneous mucormycosis arising in the setting of ibrutinib use. Because the combination of immunosuppressed states, ibrutinib and skin trauma may serve as a nidus for mucormycosis, practitioners should be vigilant of thorough skin evaluations in these patients and appropriate anti-fungal treatment. Although amphotericin B has been well studied as first line therapy, oral posaconazole has been shown as an efficacious second-line treatment.
- Published
- 2021
37. Systemic Inflammation May Induce Cardiac Injury in COVID-19 Patients Including Children and adolescents without Underlying Cardiovascular Diseases: A Systematic Review
- Author
-
Arwa, Saed Aldien, Gowrii S, Ganesan, Farah, Wahbeh, Noor, Al-Nassr, Heba, Altarawneh, Lolwa, Al Theyab, Summia, Saed Aldien, Sara, Tomerak, Hiba, Naveed, Mohamed B, Elshazly, and Dalia, Zakaria
- Subjects
cardiac injury ,Adolescent ,TPA, tissue plasminogen activator ,coronavirus ,NSAIDs, non steroidal anti inflammatory drugs ,CVD, cardiovascular disease ,LA, left atrium ,Article ,CI, cardiac injury ,HFOT, high flow oxygen therapy ,IVIg, intravenous immunoglobulin ,Humans ,EF, ejection fraction ,DIC, disseminated intravascular coagulation ,Child ,Pandemics ,ECHO, echocardiogram ,HFrEF, heart failure with reduced ejection fraction ,ARDS, acute respiratory distress syndrome ,NIV, non invasive ventilation ,BNP, brain natriuretic peptide ,NT-proBNP, N-terminal (NT)-pro hormone brain natriuretic peptide ,US, ultrasound ,SARS-CoV-2 ,CMR, cardiovascular magnetic resonance imaging ,CKD, chronic kidney disease ,COVID-19 ,HCQ, hydroxychloroquine ,IL-1, interleukin 1 ,PCT, procalcitonin ,CT, computed tomography ,IV fluids, intravenous fluids ,RV, right ventricle ,IMV, invasive mechanical ventilation ,LV, left ventricle ,COPD, chronic obstructive pulmonary disease ,TNF-alpha, tumour necrosis factor-alpha ,Cardiovascular Diseases ,HTN, hypertension ,TTE, transthoracic echocardiography ,inflammation ,IL-6, Interleukin 6 ,CRP, C-reactive protein ,COVID-19/ SARS-COVID-19, Severe acute respiratory syndrome coronavirus 2 ,ECG, electrocardiogram ,MODS, multiple organ dysfunction syndrome ,myocarditis ,ECMO, extracorporeal membrane oxygenation ,MRI, magnetic resonance imaging - Abstract
Coronavirus disease 2019(COVID-19) is an ongoing global pandemic with a daily increasing number of affected individuals and a relatively high mortality rate. COVID-19 patients that develop cardiac injury are at increased risk of a worse clinical course with higher rates of mortality. Increasing amounts of evidence suggest that a system-wide inflammatory response and a cytokine storm mediated type syndrome plays a crucial role in disease progression. This systematic review investigates the possible role of hyperinflammation in inducing cardiac injury as one of the severe complications of COVID-19. A systematic literature search was performed using PubMed, Embase and Scopus databases to identify relevant clinical studies that investigated cardiovascular injury manifestations and reported inflammatory and cardiac biomarkers in COVID-19 patients. Only 29 studies met our inclusion criteria and the majority of these studies demonstrated significantly elevated inflammatory and cardiac blood markers. It was evident that underlying cardiovascular diseases may increase the risk of developing cardiac injury. However, many COVID-19 patients included in this review, developed different types of cardiac injury without having any underlying cardiovascular diseases. Furthermore, many of these patients were either children or adolescents. Therefore, age and comorbidities may not always be the two main risk factors that dictate the severity and outcome of COVID-19. Further investigations are required to understand the underlying mechanisms of pathogenicity as an urgent requirement to develop the appropriate treatment and prevention strategies. These strategies may specifically target hyperinflammation as a suspected driving factor for some of the severe complications of COVID-19.
- Published
- 2021
38. The Impact of COVID-19 Pandemic on Adult and Pediatric Allergy & Immunology Services in the UK National Health Service
- Author
-
Ravishankar Sargur, Sophie Hambleton, Andrew F. Whyte, Paul Turner, Sarah Beck, Nathan Gribbin, Claire Bethune, Mamidipudi Thirumala Krishna, and Shuaib Nasser
- Subjects
VIT, Venom Immunotherapy ,Allergy ,Service delivery framework ,QPIDS, Quality in Primary Immunodeficiency Service ,BSACI, British Society for Allergy and Clinical Immunology ,Omalizumab ,RCP, Royal College of Physicians ,Pediatrics ,AAAAI, American Academy of Asthma Allergy and Immunology ,State Medicine ,0302 clinical medicine ,Surveys and Questionnaires ,Pandemic ,Health care ,Immunology and Allergy ,service ,COVID, Coronavirus Disease ,030212 general & internal medicine ,A&I, Allergy and Immunology ,Child ,SLIT, Sublingual Immunotherapy ,OFC, Oral Food Challenge ,IGRT, Immunoglobulin Replacement Therapy ,human immunoglobulin ,impact ,CSU/A, Chronic Spontaneous Urticaria and Angioedema ,Original Article ,medicine.drug ,Adult ,Coronavirus disease 2019 (COVID-19) ,Immunology ,BAME, Black, Asian and Minority Ethnic ,Audit ,NHS, National Health Service ,EAACI, European Academy of Allergy and Clinical Immunology ,03 medical and health sciences ,Allergy and Immunology ,SCIT, Subcutaneous Injection Immunotherapy ,Hypersensitivity ,medicine ,Humans ,Pandemics ,SARS-CoV-2 ,business.industry ,COVID-19 ,National health service ,medicine.disease ,United Kingdom ,030228 respiratory system ,IQAS, Improving Quality in Allergy Services ,SCIg, Subcutaneous Immunoglobulin ,IVIg, Intravenous Immunoglobulin ,business ,immunodeficiency ,Delivery of Health Care ,UK PIN, United Kingdom Primary Immunodeficiency Network - Abstract
Background The COVID-19 pandemic imposed multiple restrictions on healthcare services. Objective To investigate the impact of the pandemic on Allergy & Immunology (A&I) services in the UK. Methods National survey of all A&I services registered with the Royal College of Physicians and/or British Society for Allergy and Clinical Immunology. The survey covered staffing, facilities, personal protective equipment, appointments & patient review, investigations, treatments and research activity. Weeks commencing 03 Feb’20 (pre-COVID), 06 April’20 and 08’May’20 were used as reference points for the dataset. Results 99 services participated. There was a reduction in nursing, medical, administrative and allied health professional staff during pandemic; 86% and 92% of A&I services continued to accept non-urgent and urgent referrals respectively during the pandemic. There were changes in immunoglobulin (Ig) dose and infusion regimen in 67% and 14% of adult and pediatric services respectively; 30% discontinued immunoglobulin replacement in some patients. There was a significant (all variables, p≤0.0001) reduction in the following: face-to-face consultations (increase in telephone consultations), initiation of venom immunotherapy, sublingual and subcutaneous injection immunotherapy, anesthetic allergy testing and hospital procedures (food challenges, immunoglobulin and omalizumab, administration); and a significant increase (p≤0.0001) in home therapy for immunoglobulin and omalizumab. Adverse clinical outcomes were reported, but none were serious. Conclusion The pandemic had significant impact on A&I services leading to multiple unplanned pragmatic amendments in service delivery. There is an urgent need for prospective audits and strategic planning in the medium and long term to achieve equitable, safe and standardised healthcare., Highlights box: 1. What is already known about this topic? There are no published data regarding the impact of COVID-19 pandemic on Allergy & Immunology services in the UK National Health Service. 2. What does this article add to our knowledge? Data showed reduction in face-to-face consultations, increase in remote consultations, reduced access to allergy testing, and increase in self-administration of omalizumab and immunoglobulin replacement therapy. 3. How does this study impact current management guidelines? These findings will shape new guidelines regarding delivery of an equitable, safe and standardized Allergy & Immunology service and governance framework in the post-pandemic recovery phase.
- Published
- 2021
- Full Text
- View/download PDF
39. The Influence of Immune Immaturity on Outcome After Virus Infections
- Author
-
Helen C. Su and Elana R. Shaw
- Subjects
Aging ,animal diseases ,pDC, Plasmacytoid dendritic cell ,Adaptive Immunity ,medicine.disease_cause ,Dengue ,0302 clinical medicine ,Chickenpox ,NK, Natural killer ,FI-RSV, Formalin-inactivated respiratory syncytial virus ,Immunology and Allergy ,030212 general & internal medicine ,Immunodeficiency ,Infectious disease ,EBV, Epstein-Barr virus ,MERS-CoV, Middle East Respiratory Syndrome coronavirus ,RSV ,Acquired immune system ,MIS-C, Multisystem inflammatory syndrome in children ,Virus Diseases ,POL III, Polymerase III ,Autoinflammation ,CRP, C-reactive protein ,RSV, Respiratory syncytial virus ,Immunomodulatory drugs ,ADE, Antibody-dependent enhancement ,IEI, Inborn errors of immunity ,chemical and pharmacologic phenomena ,Virus ,03 medical and health sciences ,Immune system ,Age ,Immunity ,MERS ,EBV ,ACE2, Angiotensin-converting enzyme 2 ,medicine ,Animals ,Humans ,Antibody-dependent enhancement ,IFN, interferon ,Review and Feature Article ,SARS ,SARS-CoV-2, Severe acute respiratory syndrome coronavirus 2 ,Innate immune system ,business.industry ,SARS-CoV-2 ,COVID-19 ,Immune dysregulation ,biochemical phenomena, metabolism, and nutrition ,medicine.disease ,VZV, Varicella-zoster virus ,HLH, Hemophagocytic lymphohistiocytosis ,Immunity, Innate ,030228 respiratory system ,Immunology ,bacteria ,DENV, Dengue virus ,business ,IVIG, Intravenous immunoglobulin ,ROS, Reactive oxygen species - Abstract
Maturation of the adaptive immune response is typically thought to improve outcome to virus infections. However, long-standing observations of natural infections with old viruses such as Epstein-Barr virus and newer observations of emerging viruses such as severe acute respiratory syndrome coronavirus 2 responsible for COVID-19 suggest that immune immaturity may be beneficial for outcome. Mechanistic studies and studies of patients with inborn errors of immunity have revealed that immune dysregulation reflecting inappropriate antibody and T-cell responses plays a crucial role in causing bystander inflammation and more severe disease. Further evidence supports a role for innate immunity in normally regulating adaptive immune responses. Thus, changes in immune responses that normally occur with age may help explain an apparent protective role of immune immaturity during virus infections.
- Published
- 2021
- Full Text
- View/download PDF
40. Multisystem Inflammatory Syndrome in Children: Survey of Protocols for Early Hospital Evaluation and Management
- Author
-
Matthew E. Oster, Shira J. Gertz, Sanmit K. Basu, Matthew Dove, Jennifer Schuette, Sophie E Katz, Sarah Parsons, Rosemary Olivero, Ted Morton, Kanokporn Mongkolrattanothai, Rupal M. Patel, Sathish Chikkabyrappa, Michael Kelleman, Wassim Ballan, Joseph D. Kuebler, Jennifer Lighter, Nadine F. Choueiter, Daniel J. Corwin, Katharine N. Clouser, Laurie E. Panesar, Mayssa Abuali, Rod Ghassemzadeh, Amy Edwards, Christina Osborne, Navjyot Vidwan, Rima J. Jarrah, M. Jay Campbell, Christine Mikesell, Preeti Jaggi, Deepika Thacker, Stacie Knutson, Natasha Nakra, Adriana H. Tremoulet, and Jocelyn Y. Ang
- Subjects
Cross-sectional study ,Anti-Inflammatory Agents ,CHOA, Children’s Healthcare of Atlanta ,Practice Patterns ,Pediatrics ,PCR, polymerase chain reaction ,0302 clinical medicine ,Clinical Protocols ,Surveys and Questionnaires ,Vasoconstrictor Agents ,030212 general & internal medicine ,Practice Patterns, Physicians' ,Child ,CDC, Centers for Disease Control and Prevention ,AHA, American Heart Association ,Pediatric ,Aspirin ,Anti-Inflammatory Agents, Non-Steroidal ,Immunoglobulins, Intravenous ,Hospitals ,Systemic Inflammatory Response Syndrome ,COVID-19, Coronavirus Disease 2019 ,Antirheumatic Agents ,Intravenous ,Non-Steroidal ,medicine.drug ,medicine.medical_specialty ,medicine.drug_class ,MEDLINE ,Low molecular weight heparin ,Immunoglobulins ,MIS-C, Multisystem Inflammatory Syndrome in Children ,SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 ,7.3 Management and decision making ,Paediatrics and Reproductive Medicine ,03 medical and health sciences ,Clinical Research ,030225 pediatrics ,medicine ,Humans ,Glucocorticoids ,Protocol (science) ,Anakinra ,Physicians' ,business.industry ,Heparin ,REDCap, Research Electronic Data Capture ,Anticoagulants ,COVID-19 ,Original Articles ,Human Movement and Sports Sciences ,medicine.disease ,United States ,IVIG, intravenous immunoglobulin ,Systemic inflammatory response syndrome ,Interleukin 1 Receptor Antagonist Protein ,Cross-Sectional Studies ,El Niño ,PIMS-TS, Pediatric Multisystem Inflammatory Syndrome - Temporally Associated with ,Pediatrics, Perinatology and Child Health ,Emergency medicine ,Management of diseases and conditions ,business ,Digestive Diseases ,MRI, magnetic resonance imaging - Abstract
Objective To describe the similarities and differences in the evaluation and treatment of multisystem inflammatory syndrome in children (MIS-C) at hospitals in the United States. Study design We conducted a cross-sectional survey from June 16 to July 16, 2020 of U.S. children’s hospitals regarding protocols for management of patients with MIS-C. Elements included characteristics of the hospital, clinical definition of MIS-C, evaluation, treatment, and follow up. We summarized key findings and compared results from centers in which >5 patients had been treated vs those in which 5 patients vs.
- Published
- 2021
41. Delayed Diagnosis and Recovery of Fulminant Immune Checkpoint Inhibitor-Associated Myocarditis on VA-ECMO Support.
- Author
-
Ramayya T, Mitchell JD, Hartupee JC, Lavine K, Ridley CH, Kotkar KD, Jimenez J, Lin CY, Alvarez-Cardona JA, Krone RK, and Campbell CM
- Abstract
Competing Interests: Dr Jimenez is supported by the National Institutes of Health (R25 HL105400). Dr. Mitchell has received modest consulting from Pfizer and BrigeBio; and research support from Pfizer, Myocardial Solutions, Abbott Laboratories, and Children's Discovery Institute. Dr Campbell is supported by the Amyloidosis Foundation. Dr Lavine is supported by grants from the National Institutes of Health (HL161185, HL150891 and HL151078), the Children’s Discovery Institute (PM-LI-2019-829), the Burroughs Welcome Fund (1014782), and the Leducq Foundation (20CVD02) and by generous gifts through Washington University and Barnes Jewish Hospital. Dr Lavine serves as a consultant for Implicit Biosciences and Medtronic; and is the recipient of sponsored research agreements with Amgen and Novartis; and has a pending patent entitled “Methods for detecting CCR2 receptors” (application number: US17/001,857). Dr Campbell has served on advisory boards for Alnylam Pharmaceuticals and Pfizer Inc; and has received research support from Alnylam Pharmaceuticals, Pfizer Inc, and Akari Therapeutics. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Published
- 2022
- Full Text
- View/download PDF
42. Intravenous immunoglobulin-refractory necrobiotic xanthogranuloma successfully treated with tofacitinib 2% cream.
- Author
-
Shahriari N, Mazori DR, and Merola JF
- Abstract
Competing Interests: Drs Mazori and Shahriari have no financial disclosures to share. Dr Merola is a consultant and/or investigator for Amgen, Bristol-Myers Squibb, Abbvie, Dermavant, Eli Lilly, Novartis, Janssen, UCB, Sanofi, Regeneron, Sun Pharma, Biogen, Pfizer, and Leo Pharma.
- Published
- 2022
- Full Text
- View/download PDF
43. Efficacy and safety of treatments in newly diagnosed adult primary immune thrombocytopenia: A systematic review and network meta-analysis.
- Author
-
Wang Y, Sheng L, Han F, Guo Q, Zhang Z, Hou Y, Feng Q, Zhou H, Ji X, Peng J, Hou M, and Xu M
- Abstract
Background: Immune thrombocytopenia is an autoimmune disease characterised by decreased platelet count. In recent years, novel therapeutic regimens have been investigated in randomised controlled trials (RCTs). We aimed to compare the efficacy and safety of different treatments in newly diagnosed adult primary immune thrombocytopenia., Methods: We did a systematic review and network meta-analysis of RCTs involving treatments for newly diagnosed primary immune thrombocytopenia. PubMed, Embase, the Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov databases were searched up to April 31, 2022. The primary outcomes were 6-month sustained response and early response. Secondary outcome was grade 3 or higher adverse events. This study is registered with PROSPERO (CRD42022296179)., Findings: Eighteen RCTs (n = 1944) were included in this study. Pairwise meta-analysis showed that the percentage of patients achieving early response was higher in the dexamethasone-containing doublet group than in the dexamethasone group (79.7% vs 68.7%, odds ratio [OR] 1.82, 95% CI 1.10-3.02). The difference was more profound for sustained response (60.5% vs 37.4%, OR 2.57, 95% CI 1.95-3.40). Network meta-analysis showed that dexamethasone plus recombinant human thrombopoietin ranked first for early response, followed by dexamethasone plus oseltamivir or tacrolimus. Rituximab plus prednisolone achieved highest sustained response, followed by dexamethasone plus all-trans retinoic acid or rituximab. Rituximab plus dexamethasone showed 15.3% of grade 3 or higher adverse events, followed by prednis(ol)one (4.8%) and all-trans retinoic acid plus dexamethasone (4.7%)., Interpretation: Our findings suggested that compared with monotherapy dexamethasone or prednis(ol)one, the combined regimens had better early and sustained responses. rhTPO plus dexamethasone ranked top in early response, while rituximab plus corticosteroids obtained the best sustained response, but with more adverse events. Adding oseltamivir, all-trans retinoic acid or tacrolimus to dexamethasone reached equally encouraging sustained response, without compromising safety profile. Although this network meta-analysis compared all the therapeutic regimens up to date, more head-to-head RCTs with larger sample size are warranted to make direct comparison among these strategies., Funding: National Natural Science Foundation of China, Major Research Plan of National Natural Science Foundation of China, Shandong Provincial Natural Science Foundation and Young Taishan Scholar Foundation of Shandong Province., Competing Interests: We declare no competing interests., (© 2022 The Author(s).)
- Published
- 2022
- Full Text
- View/download PDF
44. SARS-CoV-2/COVID-19 associated pediatric acute-onset neuropsychiatric syndrome a case report of female twin adolescents.
- Author
-
Efe A
- Abstract
"Pediatric acute-onset neuropsychiatric syndrome", or PANS, is a rare syndrome characterized by an acute onset of obsessive-compulsive disorder (OCD), and/or severely restricted food intake accompanied by a variety of neuropsychiatric symptoms. To our knowledge, this is the first case report of twin adolescents with COVID-19-associated PANS. Dizygotic twin sisters in late teens, with abrupt and acute onset of severely restrictive food intake, weight loss, OCD, anxiety with intermittent auditory and visual hallucinations, depression, attention deficit, and sleep disturbances, simultaneously accompanied by milder neurologic symptoms such as hand tremor, tinnitus, dizziness, headache, and weakness of proximal muscles, were applied to child and adolescent psychiatry clinic. The only relevant agent underlying those neuropsychiatric and somatic complaints was COVID-19, and it was validated with laboratory testing, such as positive IgG titers of SARS-CoV-2 and negative biomarkers for other possible bacterial or viral agents. Generalized epileptic anomaly and a vermian/folial atrophy in the cerebellum were detected in further evaluations. Treatment options consisted of psychotropic agents, antibiotics, antiepileptic, and intravenous immunoglobulin transfusion finely treated the neuropsychiatric symptoms. Clinicians should consider SARS-CoV-2 as a potential agent, when a child presents with abrupt onset, dramatic neuropsychiatric symptoms also consisting of PANS, even in asymptomatic patients or with mild respiratory symptoms., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. None of the authors have any conflicts of interest or financial ties to disclose, (© 2022 The Author. Published by Elsevier B.V.)
- Published
- 2022
- Full Text
- View/download PDF
45. A case of Rowell syndrome with excellent improvement following anifrolumab.
- Author
-
Shope C, Andrews L, Cunningham M, and Connett J
- Abstract
Competing Interests: None disclosed.
- Published
- 2022
- Full Text
- View/download PDF
46. Guillain–Barré syndrome as a parainfectious manifestation of SARS-CoV-2 infection: A case series
- Author
-
Matineh Heidari, Mahin Jamshidi Makiani, Marjan Zeinali, Mona Ramezani Ghamsari, Meysam Abolmaali, Zahra Mirzaasgari, and Parichehr Moghaddam
- Subjects
Male ,Pediatrics ,Neurology ,Neural Conduction ,Case Report ,Disease ,CSF, cerebrospinal fluid ,Guillain–Barré syndrome ,Fatal Outcome ,0302 clinical medicine ,PCR, polymerase chain reaction ,Active phase ,AMAN, acute motor axonal neuropathy ,Pandemic ,reproductive and urinary physiology ,COVID-19, coronavirus disease 2019 ,Aged, 80 and over ,Neurologic Examination ,GBS, Guillain-Barré Syndrome ,Guillain-Barre syndrome ,General Medicine ,Middle Aged ,ICU, intensive care unit ,CT, computed tomography ,EMG-NCS, electromyography and nerve conduction studies ,Treatment Outcome ,AIDP, acute inflammatory demyelinating polyneuropathy ,030220 oncology & carcinogenesis ,Disease Progression ,Female ,medicine.medical_specialty ,Facial diplegia ,Coronavirus disease 2019 (COVID-19) ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Facial Paralysis ,Clinical Neurology ,Guillain-Barre Syndrome ,Quadriplegia ,SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 ,WHO, World Health Organization ,03 medical and health sciences ,Physiology (medical) ,medicine ,IVIg, intravenous immunoglobulin ,Humans ,AMSAN, acute motor and sensory axonal neuropathy ,business.industry ,MERS, Middle East respiratory syndrome ,COVID-19 ,medicine.disease ,bacterial infections and mycoses ,MRC, Medical Research Council ,Coronavirus ,bacteria ,Surgery ,Neurology (clinical) ,business ,030217 neurology & neurosurgery - Abstract
Highlights • We described GBS symptoms in three infected patients with COVID-19. • The severity of GBS linked to COVID-19 seems considerable in mentioned cases. • AMSAN type of GBS was diagnosed based on electrodiagnostic findings. • GBS must be considered as a rapid progressive neurologic complication of COVID-19., The global SARS-CoV-2 pandemic posed an unprecedented challenge to almost all fields of medicine and Neurology is not an exception. Collecting information about its complications and related conditions will help clinicians to become more confident in managing this disease. Guillain-Barre Syndrome (GBS) is mostly described as a post-infectious phenomenon and its occurrence during acute phase of illness is of interest. GBS has recently been reported during the active phase of COVID-19 for the first time. Severity and fast progression of GBS associated with COVID-19 have also been shown in recent studies. Here we report three cases of GBS during the active phase of COVID-19 with severe symptoms and fast progression to quadriplegia and facial diplegia over 2 days, which led to death in one case due to severe autonomic dysfunction. We suggest SARS-CoV-2 might be associated with rather a severe, rapidly progressive and life-threatening phenotype of GBS.
- Published
- 2021
- Full Text
- View/download PDF
47. Myocarditis and myasthenia gravis by combined nivolumab and ipilimumab immunotherapy for renal cell carcinoma: A case report of successful management
- Author
-
Daigo Ueyama, Hajime Kondo, Hidetoshi Akita, Takahiro Yanase, Takahiro Yasui, and Yoshinobu Moritoki
- Subjects
Oncology ,medicine.medical_specialty ,Myocarditis ,Exacerbation ,ICI, immune checkpoint inhibitor ,Urology ,030232 urology & nephrology ,Ipilimumab ,lcsh:RC870-923 ,CPK, creatine phosphokinase ,MG, myasthenia gravis ,03 medical and health sciences ,0302 clinical medicine ,Renal cell carcinoma ,Internal medicine ,IVIg, intravenous immunoglobulin ,medicine ,Myasthenia gravis ,mPSL, methylprednisolone ,business.industry ,CAVB, complete atrioventricular block ,medicine.disease ,lcsh:Diseases of the genitourinary system. Urology ,Ipi/Nivo, ipilimumab plus nivolumab ,Nivolumab ,Methylprednisolone ,PE, plasma exchange ,030220 oncology & carcinogenesis ,Concomitant ,RCC, renal cell carcinoma ,business ,irAE, immune-related adverse event ,medicine.drug - Abstract
Ipilimumab plus nivolumab (Ipi/Nivo) has revolutionized advanced renal cell carcinoma (RCC) treatment. However, it encompassed fatal immune-related adverse events (irAEs). Myocarditis with concomitant myasthenia gravis (MG) has a mortality rate of 50%, and a high dose of methylprednisolone (mPSL) should be administered with careful attention to MG exacerbation. We present the case of a 59-year-old man with progressing lung metastasis of RCC. After one cycle of Ipi/Nivo, he experienced myocarditis and MG, managed by mPSL pulse therapy, plasma exchange, and high-dose intravenous immunoglobulin. We share the therapeutic course, aiming to contribute to the limited literature on rare but aggressive irAEs., Highlights • Ipilimumab plus nivolumab is associated with fatal immune-related adverse events. • Myocarditis concomitant with myasthenia gravis (MG) is rare, but has a high mortality rate. • In the comorbid condition, prompt intervention for myocarditis is essential for survival. • Although high-dose methylprednisolone may worsen MG, myocarditis should be prioritized.
- Published
- 2021
48. Clinical Presentation, Treatment, and Mortality Rate in Liver Transplant Recipients With Coronavirus Disease 2019: A Systematic Review and Quantitative Analysis
- Author
-
Olivia C Smibert, Jacqueline Fraser, Anoop N. Koshy, Johanna Mousley, and Adam G Testro
- Subjects
Male ,medicine.medical_treatment ,BMI, body mass index ,coronavirus ,Disease ,MERS-CoV, Middle East Respiratory Syndrome ,Risk Factors ,Case fatality rate ,Pandemic ,COVID-19, coronavirus disease 2019 ,SARS-CoV, Severe Acute Respiratory Syndrome Coronavirus 2003 ,immunosuppression ,Mortality rate ,6-MP, 6-mercaptopurine ,Immunosuppression ,Middle Aged ,Stata 13/MP, StataCorp, College Station, TX ,CT, computed tomography ,Diarrhea ,Female ,medicine.symptom ,Coronavirus Infections ,PRISMA, Preferred Reporting Items for Systematic Reviews and Meta-Analyses ,medicine.medical_specialty ,Pneumonia, Viral ,6-TG, 6-thioguianine ,Article ,Betacoronavirus ,Immunocompromised Host ,Diabetes mellitus ,Internal medicine ,medicine ,Humans ,Pandemics ,MeSH, Medical Subject Heading ,Aged ,LT, Liver transplant ,Transplantation ,business.industry ,SARS-CoV-2 ,COVID-19 ,medicine.disease ,mortality ,Transplant Recipients ,Liver Transplantation ,Pneumonia ,LFTs, Liver function tests ,Surgery ,MMF, mycophenolate mofetil ,IVIg, Intravenous Immunoglobulin ,business - Abstract
Liver transplant recipients may be at increased risk for adverse outcomes with coronavirus disease 2019 (COVID-19) infection because of chronic immunosuppression and associated comorbidities. There is a paucity of literature describing clinical presentation, treatments, and outcomes in liver transplant recipients with COVID-19. A systematic search was performed for articles published up to June 15, 2020, revealing 223 liver transplant recipients with COVID-19 in 15 studies. Patients most commonly presented with fever (66.7%), dyspnea (34.0%), and diarrhea (28.4%). Of these, 77.7% required hospitalization, 24% had mild disease, 40% had moderate disease, and 36% had severe disease. Immunosuppression was modified in 32.8% of recipients. The case fatality rate was 19.3%. Dyspnea on presentation, diabetes mellitus, and age 60 years or older were significantly associated with increased mortality (P ≤ .01) with a trend to higher mortality rate observed in those with hypertension and those receiving corticosteroids at the time of COVID-19 diagnosis. The median time from symptoms to death was 11.5 days (2-45 days). In conclusion, liver transplant recipients with severe acute respiratory syndrome coronavirus 2 are overrepresented with regard to severe disease and hospitalizations. Older liver transplant patients with diabetes mellitus or hypertension, who are on maintenance corticosteroids, with a diagnosis of COVID-19 and describing breathlessness should be aggressively monitored for signs of deterioration because of the risk for mortality.
- Published
- 2020
- Full Text
- View/download PDF
49. Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient
- Author
-
M, Della Corte, C, Delehaye, E, Savastano, M F, De Leva, P, Bernardo, and A, Varone
- Subjects
Male ,Myoclonus ,Case Report ,Neuropsychological Tests ,EEG, electroencephalogram ,IVIG, Intravenous Immunoglobulin ,VC, Verbal Comprehension ,Humans ,Immunologic Factors ,Attention ,PR, Perceptual Reasoning ,Child ,CSF, Cerebrospinal fluid ,Mental Disorders ,COVID-19 ,Immunoglobulins, Intravenous ,Syndrome ,COVID-19, Coronavirus Disease ,General Medicine ,CNS, Central Nervous System ,Neurological complications ,Paediatric ,WM, Working Memory ,PS, Processing Speed ,Surgery ,Neurology (clinical) ,MRI, magnetic resonance imaging ,Psychomotor Performance - Abstract
We report the case of a 12-years-old patient who subacutely developed a positive and negative myoclonus of limbs and face, drowsiness and memory deficits after getting infected by SARS-CoV-2. On admission, nasopharyngeal swab for SARS-CoV-2, brain and spinal MRI with and without contrast, EEG, chest X-ray and abdominal ultrasound were negative. CSF physical-chemical examination, culture, PCR testing for SARS-CoV-2 and other pathogens, and oligoclonal IgG bands were negative as well. A full panel blood test, including clotting, autoimmunity and paraneoplastic blood studies, did not show any alteration. The neuropsychological examination showed an impairment in memory, visual-motor coordination, inductive reasoning skills, attention, and concentration. The patient was first treated with clonazepam and then with intravenous methylprednisolone for five days, with poor response. For this reason, he then received a cycle of IVIG, thus reaching a gradual and complete recovery. To date, this is the first case of a COVID-19 associated myoclonus affecting a paediatric patient.
- Published
- 2022
50. Predictive monitoring and therapeutic immune biomarkers in the management of clinical complications of COVID-19
- Author
-
Amir Roudgari, Mehrnoosh Doroudchi, Golnar Sabetian, Shahram Paydar, Najmeh Rokhtabnak, Hamed Fouladseresht, and Hossein Abdolrahimzadehfard
- Subjects
ssRNA, single-stranded RNA ,CT, computerized tomography ,Complications ,Endocrinology, Diabetes and Metabolism ,RA, rheumatoid arthritis ,Ab, antibody ,LY6E, lymphocyte antigen 6 family member E ,AT1R, Ang II receptor type 1 ,MDA-5, melanoma differentiation-associated protein-5 ,Immunopathology ,GzmB, granzyme B ,SAA, serum amyloid A ,SARS, severe acute respiratory ,Medicine ,DIC, disseminated intravascular coagulation ,ORF-1ab, open reading frame 1ab ,RT-qPCR, real-time PCR-quantitative PCR ,Fio2, fraction of inspired oxygen ,Lung ,COVID-19, coronavirus disease-2019 ,LDH, lactate dehydrogenase ,AICD, activation-induced cell death ,IRF-1, IFN regulatory factor 1 ,C3 and C5, complement proteins ,RBD, receptor-binding domain ,IP-10, interferon (IFN-γ inducible protein-10 ,CXCR, chemokine (C-X-C motifligand receptor ,Acquired immune system ,Prognosis ,KL-6, Krebs von den Lungen-6 ,JUN, c-Jun N-terminal kinases ,NSP, non-structural proteins ,Cytokines ,BEC, bronchial epithelial cell ,rhIL-1ra, recombinant human IL-1 receptor ,Antibody ,Cytokine Release Syndrome ,GM-CSF, granulocyte-macrophage colony-stimulating factor ,NK, natural killer ,WBC, white blood cell ,MOF, multiple organ failure ,CP, convalescent plasma ,PLR, platelet-to-lymphocyte ratios ,FcγR, Fc gamma receptor ,Immunology ,HLH, hemophagocytic lymphohistiocytosis ,General Biochemistry, Genetics and Molecular Biology ,Article ,PKR, protein kinase R ,MERS, middle east respiratory syndrome ,nAb, neutralizing antibody ,LWR, lymphocyte-to-WBCs ratio ,PT, prothrombin time ,IVIg, intravenous immunoglobulin ,Humans ,mAb, monoclonal antibody ,APTT, activated partial thromboplastin time ,NFkB, nuclear factor kappa-light-chain-enhancer of activated B cells ,ARDS, acute respiratory distress syndrome ,Monitoring, Physiologic ,ESR, erythrocyte sedimentation rate ,rIL7, recombinant IL-7 ,Pao2, partial pressure of oxygen ,rBP-C33, Leurecombinant surfactant protein C analogue ,HLA, human leukocyte antigen ,MUC, mucin ,scFv, single-chain variable fragment ,Biomarker ,medicine.disease ,CTL, cytotoxic T lymphocyte ,Immunity, Innate ,IL, interleukin ,RAS, regulator of the renin-angiotensin system ,Clinical trial ,TNF-α, tumor necrosis factor ,APC, antigen-presenting cell ,siRNA, small interfering RNA ,CXCL, chemokine (C-X-C motifligand ,PRR, pattern recognition receptors ,NLRP3, nod-like receptor protein 3 ,TMPRSS2, transmembrane serine protease 2 ,S-protein, spike-protein ,Biomarkers ,ACEI, ACE inhibitor ,LAG-3, lymphocyte-activation gene 3 ,MIP, macrophage inflammatory proteins ,Chemokine ,NCT, clinicaltrials.gov identifier ,CCL, chemokine (C-C motifligand ,TLR, toll like receptor ,CQ, chloroquine ,NKRF, NFkB repressing factor ,Cytokine storm ,C-GAS-STING, cGAMP binds to stimulator of interferon genes ,rhACE2-Fc fusion proteins, recombinant human ACE2-Fc fusion proteins ,srhACE2, soluble recombinant human (srhACE2 ,CRP, c-reactive protein ,BALF, bronchoalveolar lavage fluid ,Immunology and Allergy ,TGF, transforming growth factor ,Respiratory Distress Syndrome ,BTK, Bruton tyrosine kinase ,biology ,ICU, admissions to intensive care units ,IFITM, interferon-induced transmembrane protein ,PD1, programmed cell death protein 1 ,CRS, cytokine release syndrome ,VEGF, vascular endothelial growth factor ,ISG, induction of IFN-stimulated gene ,PCT, procalcitonin ,S1PR, sphingosine-1-phosphate receptors ,RIG, retinoic acid-inducible gene-I ,CCR, chemokine (C-C motifligand receptor ,TIM-3, T-cell immunoglobulin mucin 3 ,Ang, angiotensin ,Biomarker (medicine) ,AAK-1, activated protein (AP2 associated kinase 1 ,Chemokines ,AEC, alveolar epithelial cells ,ACE, angiotensin-converting enzyme ,SP, surfactant ,NLR, neutrophil-to-lymphocyte ratio ,SARS-CoV, severe acute respiratory syndrome coronavirus ,ChiCTR, chinese clinical trial registry ,JAK, janus kinase ,Th, helper T cell ,Immune system ,IFN, interferon ,NTD, N terminal domain ,ComputingMethodologies_COMPUTERGRAPHICS ,HR-2, heptad repeat region-2 ,business.industry ,SARS-CoV-2 ,COVID-19 ,HCQ, hydroxychloroquine ,SPo2, arterial oxygen saturation ,MCP, monocyte chemoattractant protein ,Treg, regulatory T cells ,RLR, RIG-I-like receptors ,biology.protein ,ARB, Ang II receptor blocker ,dsRNA, double-stranded RNA ,business ,GAK, G-associated kinase ,N, nucleocapsid - Abstract
Graphical abstract, The coronavirus disease-2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), appears with a wide spectrum of mild-to-critical clinical complications. Many clinical and experimental findings suggest the role of inflammatory mechanisms in the immunopathology of COVID-19. Hence, cellular and molecular mediators of the immune system can be potential targets for predicting, monitoring, and treating the progressive complications of COVID-19. In this review, we assess the latest cellular and molecular data on the immunopathology of COVID-19 according to the pathological evidence (e.g., mucus and surfactants), dysregulations of pro- and anti-inflammatory mediators (e.g., cytokines and chemokines), and impairments of innate and acquired immune system functions (e.g., mononuclear cells, neutrophils and antibodies). Furthermore, we determine the significance of immune biomarkers for predicting, monitoring, and treating the progressive complications of COVID-19. We also discuss the clinical importance of recent immune biomarkers in COVID-19, and at the end of each section, recent clinical trials in immune biomarkers for COVID-19 are mentioned.
- Published
- 2020
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.