scholarly journals COVID-19 Associated Multisystem Inflammatory Syndrome in Children (MIS-C): A New Pediatric Alert

2021 ◽  
Vol 39 (4) ◽  
pp. 249-260
Author(s):  
Shamima Sharmin Shova ◽  
Mohammad Imnul Islam
Keyword(s):  
Kawasaki Disease ◽  
Early Recognition ◽  
Myocardial Dysfunction ◽  
Hemodynamic Instability ◽  
Respiratory Compromise ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Life Threatening ◽  
Inflammatory Syndrome

Multisystem inflammatory syndrome in children (MIS-C), also termed pediatric multisystem inflammatory syndrome (PMIS) temporally associated with coronavirus disease-2019 (COVID-19) is rare but an emerging alarming condition. Though the exact pathogenesis is unknown, COVID-19 can trigger the condition directly or indirectly via immune complex mediated or antibody-dependent enhancement. Patients with MIS-C can present with persistent fever and a constellation of symptoms including hypotension, multiorgan involvement and elevated inflammatory markers. Presentations of MIS-C have overlapping features of Kawasaki disease (KD), toxic shock syndrome (TSS) and Kawasaki disease shock syndrome. Age of presentation, features of shock and more predilections for myocardial dysfunction can distinguish MIS-C from Kawasaki disease. Early recognition is essential, followed by prompt admission to the hospital for specialist attention. Admission to a pediatric intensive unit is mandatory for children with hemodynamic instability (shock, arrhythmia), significant respiratory compromise, or other potentially life-threatening complications. It is also necessary to maintain an appropriate follow-up schedule to observe the long-term outcome. The prognosis of PMIS/ MIS-C is uncertain, given that it is a new clinical entity and understanding of the disease is still evolving. J Bangladesh Coll Phys Surg 2021; 39: 249-260

scholarly journals Multisystem Inflammatory Syndrome in Children and Adolescents Associated with COVID-19: Review

2020 ◽  
Vol 07 (04) ◽  
pp. 13-15 ◽  
Author(s):  
BS Mahapatra ◽  
Keyword(s):  
Early Recognition ◽  
Artery Aneurysm ◽  
Multidisciplinary Care ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Immune Mediated ◽  
Life Threatening ◽  
Adolescent And Young Adults ◽  
Inflammatory Syndrome

Multisystem Inflammatory Syndrome in Children (MIS-C) is a newly recognized multiorgan disease seen in children, adolescent and young adults presumed to be a delayed immune mediated complication of Corona virus 2 (SARS-CoV-2) infection leading to severe acute respiratory syndrome. MIS-C can be associated with life threatening organ dysfunction requiring complex multidisciplinary care. Early recognition is important in order to prevent complication and serious sequalae. Because it is a post infective complication, in most of the cases RT-PCR comes negative though antibodies to COVID-19 are positive. Although SARS-CoV-2 in children are generally mild and nonfatal, there is increasing evidence of MIS-C. Clinical and laboratory features of MIS-C are similar to those of Kawasaki disease like syndrome and Toxic Shock Syndrome. Pathophysiology of MIS-C is still unclear and mainly due to formation of autoantibody and immune complex which activates inflammation. Most of the MIS-C associated with COVID-19, need treatment with ionotropic agents and anticoagulants. The long-term outcome of MIS-C like coronary artery aneurysm formation remain unknown and needs close follow up.

scholarly journals Long-term Outcome of Patients with Kawasaki Disease

2003 ◽  
Vol 53 (1) ◽  
pp. 185-185
Author(s):  
Kenji Waki ◽  
Kiyoshi Baba ◽  
Yoshio Arakaki ◽  
Shin-Ichiro Yoshimura
Keyword(s):  
Kawasaki Disease ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Long-term Outcome of Coronary Abnormalities in Kawasaki Disease

2003 ◽  
Vol 53 (1) ◽  
pp. 186-186
Author(s):  
Hiroshi Nishikawa ◽  
Masaki Matsushima ◽  
Naoki Oohashi ◽  
Namiko Kojima ◽  
Masami Nagashima
Keyword(s):  
Kawasaki Disease ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Coronary Abnormalities

scholarly journals A Five-Year Followup of Human Seminal Plasma Allergy in an 18-Year-Old Woman

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Ole D. Wolthers
Keyword(s):  
Seminal Plasma ◽  
Case Reports ◽  
Nasal Congestion ◽  
Rare Condition ◽  
Human Seminal Plasma ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Life Threatening ◽  
Low Prevalence

Case reports of women with the rare condition of human seminal plasma allergy have indicated that the condition may be associated with life-threatening anaphylactic reactions in relation to coitus. Few observations, if any, of long-term outcome of the condition are available. The aim of this paper was to present a case diagnosed in an 18-year-old girl who presented with generalized urticaria, nasal congestion and secretion, conjunctivitis, and periorbital and labial oedema 6–8 hours after coitus. During five years of followup the condition improved clinically significantly. Due to intimacy concerns and the low prevalence of the condition robust long term data on the natural course of the condition are difficult to obtain. The present case suggests that in some patients the condition may improve over time.

X-linked thrombocytopenia (XLT) due to WAS mutations: clinical characteristics, long-term outcome, and treatment options

Blood ◽  
2010 ◽  
Vol 115 (16) ◽  
pp. 3231-3238 ◽  
Author(s):  
Michael H. Albert ◽  
Tanja C. Bittner ◽  
Shigeaki Nonoyama ◽  
Lucia Dora Notarangelo ◽  
Siobhan Burns ◽  
...  
Keyword(s):  
Severe Disease ◽  
Treatment Options ◽  
Gene Mutations ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Increased Risk ◽  
Life Threatening ◽  
Severe Infections

Abstract A large proportion of patients with mutations in the Wiskott-Aldrich syndrome (WAS) protein gene exhibit the milder phenotype termed X-linked thrombocytopenia (XLT). Whereas stem cell transplantation at an early age is the treatment of choice for patients with WAS, therapeutic options for patients with XLT are controversial. In a retrospective multicenter study we defined the clinical phenotype of XLT and determined the probability of severe disease-related complications in patients older than 2 years with documented WAS gene mutations and mild-to-moderate eczema or mild, infrequent infections. Enrolled were 173 patients (median age, 11.5 years) from 12 countries spanning 2830 patient-years. Serious bleeding episodes occurred in 13.9%, life-threatening infections in 6.9%, autoimmunity in 12.1%, and malignancy in 5.2% of patients. Overall and event-free survival probabilities were not significantly influenced by the type of mutation or intravenous immunoglobulin or antibiotic prophylaxis. Splenectomy resulted in increased risk of severe infections. This analysis of the clinical outcome and molecular basis of patients with XLT shows excellent long-term survival but also a high probability of severe disease-related complications. These observations will allow better decision making when considering treatment options for individual patients with XLT.

Long-Term Outcome of Coronary Abnormalities in Patients After Kawasaki Disease

1996 ◽  
Vol 17 (2) ◽  
pp. 71-76
Author(s):  
J. Fukushige ◽  
N. Takahashi ◽  
K. Ueda ◽  
T. Hijii ◽  
H. Igarashi ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Coronary Abnormalities

scholarly journals Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia

2020 ◽  
Vol 9 (4) ◽  
pp. 903
Author(s):  
Michael Veith ◽  
Ibrahim El-Battrawy ◽  
Gretje Roterberg ◽  
Laura Raschwitz ◽  
Siegfried Lang ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blockers ◽  
Polymorphic Ventricular Tachycardia ◽  
Cardiac Events ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Follow Up ◽  
Life Threatening

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective: The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods: In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results: We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 ± 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion: Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.

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