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⇱ Describing Kawasaki shock syndrome: results from a retrospective study and literature review | Clinical Rheumatology | Springer Nature Link

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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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References

  1. Kanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, Watson VE, Best BM, Burns JC (2009) Recognition of a Kawasaki disease shock syndrome. Pediatrics 123:e783–e789. doi:10.1542/peds.2008-1871

    Article  PubMed  PubMed Central  Google Scholar 

  2. Thabet F, Bafaqih H, Al-Mohaimeed S, Al-Hilali M, Al-Sewari W, Chehab M (2011) Shock: an unusual presentation of Kawasaki disease. Eur J Pediatr 170:941–943. doi:10.1007/s00431-011-1426-5

    Article  PubMed  Google Scholar 

  3. Wang W, Zhu WH, Qi YQ, Fu SL, Gong FQ (2012) Cardiogenic shock: do not forget the possibility of Kawasaki disease. Turk J Pediatr 54:86–89

    PubMed  Google Scholar 

  4. Dominguez SR, Friedman K, Seewald R, Anderson MS, Willis L, Glode MP (2008) Kawasaki disease in a pediatric intensive care unit: a case-control study. Pediatrics 122:e786–e790. doi:10.1542/peds.2008-1275

    Article  PubMed  Google Scholar 

  5. Druey KM, Greipp PR (2010) Narrative review: the systemic capillary leak syndrome. Ann Intern Med 153:90–98. doi:10.7326/0003-4819-153-2-201007200-00005

    Article  PubMed  PubMed Central  Google Scholar 

  6. Natterer J, Perez MH, Di Bernardo S (2012) Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction. Cardiol Young 22:349–352. doi:10.1017/S1047951111001314

    Article  PubMed  Google Scholar 

  7. Fuse S, Tomita H, Ohara T, Iida K, Takamuro M (2003) Severely damaged aortic valve and cardiogenic shock in an infant with Kawasaki disease. Pediatr Int 45:110–113. doi:10.1046/j.1442-200X.2003.01666.x

    Article  PubMed  Google Scholar 

  8. Shin JI, Lee JS (2009) Beneficial effect of intravenous immunoglobulins on systemic capillary leak syndrome in patients with monoclonal gammopathy. Crit Care Med 37:795. doi:10.1097/CCM.0b013e3181959c3d

    Article  PubMed  Google Scholar 

  9. Terai M, Honda T, Yasukawa K, Higashi K, Hamada H, Kohno Y (2003) Prognostic impact of vascular leakage in acute Kawasaki disease. Circulation 108:325–330. doi:10.1161/01.CIR.0000079166.93475.5F

    Article  PubMed  Google Scholar 

  10. Gámez-González LB (2012) Clinical manifestations associated with Kawasaki disease shock syndrome in Mexican children. Eur J Pediatr 172:337–342. doi:10.1007/s00431-012-1879-1

    Article  PubMed  Google Scholar 

  11. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, Shulman ST, Bolger AF, Ferrieri P, Baltimore RS, Wilson WR, Baddour LM, Levison ME, Pallasch TJ, Falace DA, Taubert KA (2004) Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 114:1708–1733. doi:10.1542/peds.2004-2182

    Article  PubMed  Google Scholar 

  12. Dieckmann RA (2007) Pediatric assessment. In: Gausche-Hill M, Fuchs S, Yamamoto L (eds) APLS: the pediatric emergency medicine resource, 4th edn. Jones and Bartlett, Sudbury, pp 20–51

    Google Scholar 

  13. Haque IU, Zaritsky AL (2007) Analysis of the evidence for the lower limit of systolic and mean arterial pressure in children. Pediatr Crit Care Med 8:138–144. doi:10.1097/01.PCC.0000257039.32593.DC

    Article  PubMed  Google Scholar 

  14. Fitzmaurice L, Gerardi JM (2007) Cardiovascular system. In: Gausche-Hill M, Fuchs S, Yamamoto L (eds) APLS: the pediatric emergency medicine resource, 4th edn. Jones and Bartlett, Sudbury, pp 106–145

    Google Scholar 

  15. McCrindle BW, Li JS, Minich LL, Colan SD, Atz AM, Takahashi M, Vetter VL, Gersony WM, Mitchell PD, Newburger JW (2007) Coronary artery involvement in children with Kawasaki disease: risk factors from analysis of serial normalized measurements. Circulation 116:174–179. doi:10.1161/CIRCULATIONAHA.107.690875

    Article  PubMed  Google Scholar 

  16. Cimaz R, Sundel R (2009) Atypical and incomplete Kawasaki disease. Best Pract Res Clin Rheumatol 23:689–697. doi:10.1016/j.berh.2009.08.010

    Article  CAS  PubMed  Google Scholar 

  17. Bresson V, Bonello B, Rousset-Rouvière C, Serratrice J (2011) Kawasaki disease in older children and young adults: 10 years of experience in Marseille, France. Arch Pediatr 18:731–736. doi:10.1016/j.arcped.2011.04.014

    Article  CAS  PubMed  Google Scholar 

  18. Gatterre P, Oualha M, Dupic L, Iserin F, Bodemer C, Lesage F, Hubert P (2012) Kawasaki disease: an unexpected etiology of shock and multiple organ dysfunction syndrome. Intensive Care Med 38:872–878. doi:10.1007/s00134-012-2473-8

    Article  PubMed  Google Scholar 

  19. Zulian F, Falcini F, Zancan L, Martini G, Seccheri S, Luzzatto C, Zacchello F (2003) Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr 142:731–735, http://dx.doi.org/10.1067/mpd.2003.232

    Article  PubMed  Google Scholar 

  20. Tremoulet A, Best B, Song BS, Wang S, Corinaldesi E, Eichenfield JR, Martin DD, Newburger JW, Burns JC (2008) Resistance to intravenous immunoglobulin in children with Kawasaki disease. J Pediatr 153:117–121. doi:10.1016/j.jpeds.2007.12.021

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Davies S, Sutton N, Blackstock S, Gormley S, Hoggart CJ, Levin M, Herberg JA (2015) Predicting IVIG resistance in UK Kawasaki disease. Arch Dis Child 100:366–368. doi:10.1136/archdischild-2014-307397

    Article  PubMed  Google Scholar 

  22. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, Atz AM, Li JS, Takahashi M, Baker AL, Colan SD, Mitchell PD, Klein GL, Sundel RP (2007) Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Eng J Med 356:663–675. doi:10.1056/NEJMoa061235

    Article  CAS  Google Scholar 

  23. Kobayashi T, Kobayashi T, Morikawa A, Ikeda K, Seki M, Shimoyama S, Ishii Y, Suzuki T, Nakajima K, Sakamoto N, Arakawa H (2013) Efficacy of intravenous immunoglobulin combined with prednisolone following resistance to initial intravenous immunoglobulin treatment of acute Kawasaki disease. J Pediatr 163:521–526. doi:10.1016/j.jpeds.2013.01.022

    Article  CAS  PubMed  Google Scholar 

  24. Chen S, Dong Y, Yin Y, Krucoff MW (2013) Intravenous immunoglobulin plus corticosteroids to prevent coronary artery abnormalities in Kawasaki disease: a meta analysis. Heart 99:76–82. doi:10.1136/heartjnl-2012-302126

    Article  CAS  PubMed  Google Scholar 

  25. Kobayashi T, Saji T, Otani T, Takeuchi K, Nakamura T, Arakawa H, Kato T, Hara T, Hamaoka K, Ogawa S, Miura M, Nomura Y, Fuse S, Ichida F, Seki M, Fukazawa R, Ogawa C, Furuno K, Tokunaga H, Takatsuki S, Hara S, Morikawa A (2012) Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial. Lancet 379:1613–1620. doi:10.1016/S0140-6736(11)61930-2

    Article  CAS  PubMed  Google Scholar 

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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

Authors
  1. Andrea Taddio
  2. Eleonora Dei Rossi
  3. Lorenzo Monasta
  4. Serena Pastore
  5. Alberto Tommasini
  6. Loredana Lepore
  7. Gabriele Bronzetti
  8. Edoardo Marrani
  9. Biancamaria D’Agata Mottolese
  10. Gabriele Simonini
  11. Rolando Cimaz
  12. Alessandro Ventura

Corresponding author

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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