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Describing Kawasaki shock syndrome: results from a retrospective study and literature review

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Abstract

Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6 %) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60 % vs. 23/79, 30 %, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

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Authors and Affiliations

  1. University of Study of Trieste, Trieste, Italy

    Andrea Taddio, Eleonora Dei Rossi, Loredana Lepore & Alessandro Ventura

  2. Institute for Maternal and Child Health—IRCCS “Burlo Garofolo”, Trieste, Italy

    Andrea Taddio, Lorenzo Monasta, Serena Pastore, Alberto Tommasini, Biancamaria D’Agata Mottolese & Alessandro Ventura

  3. Cardiologia Pediatrica e dell’Età Evolutiva, Policlinico Sant’Orsola—Malpighi, Bologna, Italy

    Gabriele Bronzetti

  4. AOU Meyer, Florence, Italy

    Edoardo Marrani, Gabriele Simonini & Rolando Cimaz

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  1. Andrea Taddio
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  2. Eleonora Dei Rossi
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  3. Lorenzo Monasta
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Correspondence to Andrea Taddio.

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Taddio, A., Rossi, E.D., Monasta, L. et al. Describing Kawasaki shock syndrome: results from a retrospective study and literature review. Clin Rheumatol 36, 223–228 (2017). https://doi.org/10.1007/s10067-016-3316-8

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  • DOI: https://doi.org/10.1007/s10067-016-3316-8

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