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Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease.
Pediatr Int. 2018 Sep; 60(9):781-790.PI

Abstract

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.

Authors+Show Affiliations

Allergy and Immunology Department, Children's Hospital, Chihuahua City, Mexico.Clinical Immunology Department, National Institute of Pediatrics, Médica Sur Hospital, Mexico City, Mexico.Research Methodology Department, Children's Hospital, Chihuahua City, Mexico.Pediatric Intensive Care Unit, Médica Sur Hospital, Mexico City, Mexico.Intensive Care Unit, National Institute of Pediatrics, Médica Sur Hospital, Mexico City, Mexico.Cardiology Department, National Institute of Pediatrics, Médica Sur Hospital, Mexico City, Mexico.Clinical Immunology Department, National Institute of Pediatrics, Médica Sur Hospital, Mexico City, Mexico.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29888440

Citation

Gamez-Gonzalez, Luisa Berenise, et al. "Kawasaki Disease Shock Syndrome: Unique and Severe Subtype of Kawasaki Disease." Pediatrics International : Official Journal of the Japan Pediatric Society, vol. 60, no. 9, 2018, pp. 781-790.
Gamez-Gonzalez LB, Moribe-Quintero I, Cisneros-Castolo M, et al. Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease. Pediatr Int. 2018;60(9):781-790.
Gamez-Gonzalez, L. B., Moribe-Quintero, I., Cisneros-Castolo, M., Varela-Ortiz, J., Muñoz-Ramírez, M., Garrido-García, M., & Yamazaki-Nakashimada, M. (2018). Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease. Pediatrics International : Official Journal of the Japan Pediatric Society, 60(9), 781-790. https://doi.org/10.1111/ped.13614
Gamez-Gonzalez LB, et al. Kawasaki Disease Shock Syndrome: Unique and Severe Subtype of Kawasaki Disease. Pediatr Int. 2018;60(9):781-790. PubMed PMID: 29888440.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease. AU - Gamez-Gonzalez,Luisa Berenise, AU - Moribe-Quintero,Isabel, AU - Cisneros-Castolo,Martin, AU - Varela-Ortiz,Javier, AU - Muñoz-Ramírez,Mireya, AU - Garrido-García,Martin, AU - Yamazaki-Nakashimada,Marco, PY - 2017/04/04/received PY - 2018/01/20/revised PY - 2018/06/07/accepted PY - 2018/6/12/pubmed PY - 2018/10/12/medline PY - 2018/6/12/entrez KW - Kawasaki disease KW - Kawasaki disease shock syndrome KW - atypical Kawasaki disease KW - shock KW - toxic shock syndrome SP - 781 EP - 790 JF - Pediatrics international : official journal of the Japan Pediatric Society JO - Pediatr Int VL - 60 IS - 9 N2 - 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. SN - 1442-200X UR - https://www.unboundmedicine.com/medline/citation/29888440/Kawasaki_disease_shock_syndrome:_Unique_and_severe_subtype_of_Kawasaki_disease_ L2 - https://doi.org/10.1111/ped.13614 DB - PRIME DP - Unbound Medicine ER -