Kawasaki disease shock syndrome in the COVID-19 pandemic.

Multisystem inflammatory syndrome in children is a new entity in association with SARS-CoV2. Clinical features of Kawasaki disease were noted from the first reported cases of MIS-C. Before the COVID-19 pandemic, Kawasaki disease shock syndrome was considered to be a distinct and unique form of KD. We present a representative case that prove the current difficulty in clearly distinguishing MIS-C from pre-COVID-19-KDSS and emphasie the overlap of the diagnostic criteria.

[Giant coronary aneurysms in infant with Kawasaki disease shock syndrome]. / Aneurismas coronarios gigantes en lactante con síndrome de choque por enfermedad de Kawasaki.

BACKGROUND: Kawasaki disease shock syndrome is a rare presentation of Kawasaki disease, in which cardiovascular manifestations associated with elevated inflammation biomarkers that develop hypotension are observed. It is preceded by gastrointestinal and neurological manifestations, with an increased risk of coronary lesions and resistance to intravenous immunoglobulin. CASE REPORT: A 5-month-old male patient with a fever that had developed in the last week, gastrointestinal and neurological symptoms with hypotensive shock, urticarial rash, BCG lymphadenitis, and edema of palms and soles. Giant coronary aneurysms were evident, so Kawasaki disease shock syndrome was diagnosed, which was treated with corticosteroid pulse and intravenous immunoglobulin. CONCLUSIONS: Clinicians must suspect Kawasaki disease shock syndrome when there is hypotensive shock, and the gastrointestinal, neurological and mucocutaneous symptoms that are characteristic of the disease, especially in infants under one year of age. The timely treatment of this disease reduces severe complications.

Antecedentes: El síndrome de choque es una presentación poco habitual de la enfermedad de Kawasaki en el que se observan manifestaciones cardiovasculares asociadas con niveles elevados de marcadores de inflamación, que llevan a hipotensión. Es precedido por manifestaciones gastrointestinales y neurológicas y existe mayor riesgo de lesiones coronarias y resistencia a inmunoglobulina intravenosa. Caso clínico: Varón de cinco meses de edad con fiebre de una semana de evolución, síntomas gastrointestinales y neurológicos con choque hipotensivo, erupciones urticariforme, linfadenitis por vacunación con bacilo de Calmette-Guérin, así como edema de manos y pies. Se evidenciaron aneurismas coronarios gigantes, por lo que se diagnosticó síndrome de choque por enfermedad de Kawasaki, el cual fue tratado con pulso de corticoesteroide e inmunoglobulina intravenosa. Conclusiones: El diagnóstico de síndrome de choque por enfermedad de Kawasaki se debe sospechar por choque hipotensivo, síntomas gastrointestinales, neurológicos y mucocutáneos propios de la enfermedad, especialmente en menores de un año. El tratamiento oportuno reduce las complicaciones graves.

Multisystemic Inflammatory Syndrome in Children Associated With SARS-CoV-2 Infection: A Case Series Report in a Pediatric Center in Mexico.

We report four cases of patients with multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, of which three patients presented characteristics of Kawasaki disease (KD). All presented fever of more than 3 days, and gastrointestinal involvement, significant increase in C-reactive protein (CRP), polymorphonuclear cells, procalcitonin, D-dimer, fibrinogen and troponin, lymphopenia and hypoalbuminemia. Myocardial involvement was observed in two patients. All were treated with fluids resuscitation and vasoactive therapy, 75% received intravenous immunoglobulin (IVIG) and systemic steroids. Two patients developed a transient acute kidney injury, one patient presented as acute appendicitis and developed a bilateral pleural effusion. One patient required a second dose of IVIG and boluses of methylprednisolone. None required mechanical ventilation and there were no deaths.

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease.

BACKGROUND: Kawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance. METHODS: A retrospective, descriptive study of children with KDSS in two hospitals was performed. Relevant articles about KD and shock were collected, and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature. RESULTS: Seventy-two patients presented with complete KD (69.9%), and 30.1% (31/103) had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS; n = 20). Sixteen of the 20 had coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 65% of the patients. The mortality rate was 6.8%. CONCLUSION: The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.