Background: There is limited information available regarding the management of IVIG-refractory Kawasaki Disease (KD). Objective: This study aimed to evaluate the safety and efficacy of a second intravenous immunoglobulin (IVIG) infusion versus intravenous methylprednisolone (IVMP) in patients with IVIG-refractory KD. Methodology: Cochrane Library, PubMed, Medline, Elsevier (Science Direct), Springer Link and BMJ databases were searched from May 1, 2020 to December 31, 2020. We included randomized controlled trials (RCTs) and high-quality prospective and retrospective studies, with population restricted to children 0 months to 18 years, with KD refractory to initial IVIG at 2g/kg, who remained febrile for 24-48 hours after completion of initial IVIG, and who received second-line monotherapy with either a second dose IVIG or IVMP. We conducted a meta-analysis using Review Manager [RevMan] 5.4.1 software. Results: A total of six studies (n=188 patients) were analyzed. The incidence of coronary artery lesions was comparable between a second dose of IVIG and IVMP (RR 0.82, 0.34-1.96, P=0.66) in patients with IVIG-refractory KD. The rate of fever resolution to a second IVIG, compared to IVMP, was not significantly different between groups (RR 0.97, 0.84-1.13, P=0.72). There was a significantly higher incidence of adverse events in the IVMP group (RR 0.42, 0.26-0.57, P=0.0002), but these were all transient and resolved without further treatment. Conclusion There is no significant difference in the incidence of coronary artery lesions and rate of fever resolution post-retreatment with a second dose of IVIG versus IVMP in IVIG-refractory KD. More adverse events were reported in the IVMP group.
Mucocutaneous Lymph Node Syndrome ; Immunosuppressive Agents ; Immunoglobulins, Intravenous ; Methylprednisolone

Background: Since the start of SARS-CoV-2 pandemic, a post-infection hyperinflammatory process in children with features similar to Kawasaki disease, termed multisystem inflammatory syndrome in children (MIS-C),1 was identified. Thousands of MIS-C cases have already been reported worldwide.2 As possible cases of MIS-C in neonates were increasingly identified, multisystem inflammatory syndrome in neonates (MIS-N) as a distinct entity was proposed as neonates may not manifest all the typical features described in older children. Case Presentation: We describe the case of a previously well term neonate with sudden signs of bowel obstruction who later had multisystem involvement (cardiac, gastrointestinal, and hematologic). The baby was born to a 23-yearold multigravida with an unremarkable prenatal history except for COVID-19 infection during her 34th week age of gestation. The mother presented with mild respiratory symptoms and resolved with supportive management. Our patient was born stable, then had sudden manifestations of feeding intolerance on the 16th day of life and upon work-up had moderate anemia, elevated inflammatory and cardiac markers, ileus, and dilatation of proximal left coronary artery. RT-PCR for SARS-CoV2 was negative. The baby was managed with intravenous immunoglobulin (IVIG) and steroids, with rapid clinical and laboratory parameters improvement thereafter. Conclusion MIS-N is still evolving as a disease entity with no clear, directed guidance yet on diagnosis and management. Management is extrapolated from treatment of MIS-C. Additional case reports and series are warranted to increase awareness and enable better understanding of the disease pathology among clinicians for timely investigation, diagnosis, and management.
SARS-CoV-2