PMID- 29888440 OWN - NLM STAT- MEDLINE DCOM- 20181011 LR - 20220408 IS - 1442-200X (Electronic) IS - 1328-8067 (Linking) VI - 60 IP - 9 DP - 2018 Sep TI - Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease. PG - 781-790 LID - 10.1111/ped.13614 [doi] AB - 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. CI - (c) 2018 Japan Pediatric Society. FAU - Gamez-Gonzalez, Luisa Berenise AU - Gamez-Gonzalez LB AD - Allergy and Immunology Department, Children's Hospital, Chihuahua City, Mexico. FAU - Moribe-Quintero, Isabel AU - Moribe-Quintero I AD - Clinical Immunology Department, National Institute of Pediatrics, Medica Sur Hospital, Mexico City, Mexico. FAU - Cisneros-Castolo, Martin AU - Cisneros-Castolo M AD - Research Methodology Department, Children's Hospital, Chihuahua City, Mexico. FAU - Varela-Ortiz, Javier AU - Varela-Ortiz J AD - Pediatric Intensive Care Unit, Medica Sur Hospital, Mexico City, Mexico. FAU - Munoz-Ramirez, Mireya AU - Munoz-Ramirez M AD - Intensive Care Unit, National Institute of Pediatrics, Medica Sur Hospital, Mexico City, Mexico. FAU - Garrido-Garcia, Martin AU - Garrido-Garcia M AD - Cardiology Department, National Institute of Pediatrics, Medica Sur Hospital, Mexico City, Mexico. FAU - Yamazaki-Nakashimada, Marco AU - Yamazaki-Nakashimada M AUID- ORCID: 0000-0002-7609-3923 AD - Clinical Immunology Department, National Institute of Pediatrics, Medica Sur Hospital, Mexico City, Mexico. LA - eng PT - Journal Article PL - Australia TA - Pediatr Int JT - Pediatrics international : official journal of the Japan Pediatric Society JID - 100886002 RN - 0 (Biomarkers) RN - 0 (Glucocorticoids) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adolescent MH - Biomarkers/blood MH - Child MH - Child, Preschool MH - Diagnosis, Differential MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Infant MH - Male MH - Mucocutaneous Lymph Node Syndrome/*complications/mortality/therapy MH - Retrospective Studies MH - Shock/diagnosis/epidemiology/*etiology MH - Survival Rate OTO - NOTNLM OT - Kawasaki disease OT - Kawasaki disease shock syndrome OT - atypical Kawasaki disease OT - shock OT - toxic shock syndrome EDAT- 2018/06/12 06:00 MHDA- 2018/10/12 06:00 CRDT- 2018/06/12 06:00 PHST- 2017/04/04 00:00 [received] PHST- 2018/01/20 00:00 [revised] PHST- 2018/06/07 00:00 [accepted] PHST- 2018/06/12 06:00 [pubmed] PHST- 2018/10/12 06:00 [medline] PHST- 2018/06/12 06:00 [entrez] AID - 10.1111/ped.13614 [doi] PST - ppublish SO - Pediatr Int. 2018 Sep;60(9):781-790. doi: 10.1111/ped.13614.