TY - JOUR T1 - Describing Kawasaki shock syndrome: results from a retrospective study and literature review. JF - Clin Rheumatol Y1 - 2017 A1 - Taddio, Andrea A1 - Rossi, Eleonora Dei A1 - Monasta, Lorenzo A1 - Pastore, Serena A1 - Tommasini, Alberto A1 - Lepore, Loredana A1 - Bronzetti, Gabriele A1 - Marrani, Edoardo A1 - Mottolese, Biancamaria D'Agata A1 - Simonini, Gabriele A1 - Cimaz, Rolando A1 - Ventura, Alessandro KW - C-Reactive Protein KW - Child KW - Child, Preschool KW - Echocardiography KW - Female KW - Heart Failure KW - Hemoglobins KW - Humans KW - Immunoglobulins, Intravenous KW - Male KW - Mucocutaneous Lymph Node Syndrome KW - Retrospective Studies KW - Shock KW - Syndrome AB -

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.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27230223?dopt=Abstract ER -