Vol 4-2 Short Communication

Kawasaki Disease, Multisystem Inflammatory Syndrome in Children: Antibody-Induced Mast Cell Activation Hypothesis

Darrell O. Ricke1*, Nicole Gherlone2, Philip Fremont-Smith1, Philip Tisdall3, Maurice Fremont-Smith2

1MIT Lincoln Laboratory, USA

2Frank H. Netter MD School of Medicine – Quinnipiac University, USA

3Medical School Companion LLC, USA

Multisystem Inflammatory Syndrome in Children (MIS-C) is appearing in infants, children, and young adults in association with COVID-19 (coronavirus disease 2019) infections of SARS-CoV-2. Kawasaki Disease (KD) is one of the most common vasculitides of childhood. KD presents with similar symptoms to MIS-C especially in severe forms such as Kawasaki Disease Shock Syndrome (KDSS). The observed symptoms for MIS-C and KD are consistent with Mast Cell Activation Syndrome (MCAS) characterized by inflammatory molecules released from activated mast cells. Based on the associations of KD with multiple viral and bacterial pathogens, we put forward the hypothesis that KD and MIS-C result from antibody activation of mast cells by Fc receptor-bound pathogen antibodies causing a hyperinflammatory response upon second pathogen exposure. Within this hypothesis, MIS-C may be atypical KD or a KD-like disease associated with SARS-CoV-2. We extend the mast cell hypothesis that increased histamine levels are inducing contraction of effector cells with impeded blood flow through cardiac capillaries. In some patients, pressure from impeded blood flow, within cardiac capillaries, may result in increased coronary artery blood pressure leading to aneurysms, a well-known complication in KD.

DOI: 10.29245/2578-2940/2020/2.1157 View / Download Pdf
Vol 4-2 Mini Review

Monitoring Viscoelastic Blood Properties during Pediatric Cardiac Surgery: The Challenge of Data Driven Hemostasis

Zhe Amy Fang1, Meena Nathan2, Sirisha Emani2, Sitaram Emani2, Juan C. Ibla3*

1Department of Anesthesia and Pain Medicine, Hospital for Sick Children, Toronto, Ontario, Canada

2Department of Cardiac Surgery, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

3Division of Cardiac Anesthesia, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

Thromboelastography, a point of care graphical representation of the activation of coagulation and fibrin polymerization process, assists clinicians in making diagnostic and transfusion-related decisions in the perioperative setting. There is growing interest in applying this technology to pediatric cardiac surgery and this article reviews the currently available evidence for the use of thromboelastography (TEG) and thromboelastometry (ROTEM) in this population. A few studies exist on the use of TEG/ROTEM to accurately guide transfusion and positively impacting patient outcomes, indicating a need for additional studies to validate its utility during pediatric cardiac surgery.

DOI: 10.29245/2578-2940/2020/2.1158 View / Download Pdf
Vol 4-3 Mini Review

Clinical Importance of Family History in Recurrent Chronic Tonsillitis Pediatric Patients: Mini-Review

Fuat Bulut1*, Alev Cumbul2, Basak Ballica3

1*Department of Otorhinolaringology, Private Corlu Reyap Hospital, Rumeli University, Istanbul

2Istanbul Yeditepe University Faculty of Medicine, Istanbul

3Istanbul Bahcesehir University Faculty of Medicine, Istanbul

Introduction: In pediatric patients with a history of recurrent chronic tonsillitis in both their mother and father, they may show an excessive immune response due to genetic inheritance.

Method: Family history in immune defense mechanism predispose to tonsil disorders. Biofilm formation theory explains that the underlying factor of recurrent/ chronic tonsillitis formation is the bacterial biofilm on the surface of the tonsils.

Results: Although recurrent chronic tonsillitis is a clinical diagnosis, patient's symptoms and signs should be monitored carefully. The history of each patient and family members should be taken carefully. Since the clinical presentation of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) syndrome can be confused with pediatric patients with a history of recurrent chronic tonsillitis in both parents, must follow a multidisciplinary approach before starting a treatment.

Conclusions: Family history determines the severity of tonsillar damage with immune response in pediatric patients with a history of recurrent chronic tonsillitis in both their mother and father.

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