title: Describing Kawasaki shock syndrome: results from a retrospective study and literature review.
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.
authors.affiliation: University of Study of Trieste, Trieste, Italy. andrea.taddio@burlo.trieste.it.|Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy. andrea.taddio@burlo.trieste.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Taddio
authors.affiliation: University of Study of Trieste, Trieste, Italy.
authors.forename: Eleonora Dei
authors.initials: ED
authors.lastname: Rossi
authors.affiliation: Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Monasta
authors.affiliation: Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Pastore
authors.affiliation: Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Tommasini
authors.affiliation: University of Study of Trieste, Trieste, Italy.
authors.forename: Loredana
authors.initials: L
authors.lastname: Lepore
authors.affiliation: Cardiologia Pediatrica e dell'Età Evolutiva, Policlinico Sant'Orsola-Malpighi, Bologna, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Bronzetti
authors.affiliation: AOU Meyer, Florence, Italy.
authors.forename: Edoardo
authors.initials: E
authors.lastname: Marrani
authors.affiliation: Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Biancamaria D'Agata
authors.initials: BD
authors.lastname: Mottolese
authors.affiliation: AOU Meyer, Florence, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Simonini
authors.affiliation: AOU Meyer, Florence, Italy.
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.affiliation: University of Study of Trieste, Trieste, Italy.|Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ventura
mesh_terms: D002097:C-Reactive Protein / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006333:Heart Failure; D006454:Hemoglobins / Q000737:chemistry; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000175:diagnosis*; D012189:Retrospective Studies; D012769:Shock / Q000150:complications*; D013577:Syndrome
chemical_list: D006454:Hemoglobins; D016756:Immunoglobulins, Intravenous; D002097:C-Reactive Protein
keywords: Coronary artery involvement; Heart failure; Hypotension; Kawasaki disease; Kawasaki shock syndrome