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      Clinical Analysis of Kawasaki Disease Shock Syndrome

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          Abstract

          Kawasaki disease shock syndrome (KDSS) refers to Kawasaki disease (KD) patients who present more than 20% decrease in systolic blood pressure compared to healthy individuals of the same age, or to those patients who show peripheral blood circulation perfusion disorder. KDSS may have varied clinical courses and could be easily ignored and misdiagnosed. Yet, this syndrome could be deleterious, especially when combined with coronary artery injury or multiple organ dysfunctions. Therefore, early diagnosis of KDSS through specific clinical manifestations is critical. This manuscript summarizes the clinical features and treatment methods of 11 children with KDSS who were able to fully recover in our hospital. The results shed lights on understanding the early identification of KDSS and could assist in reducing the complications of KD and improving the quality of life in KDSS patients. All patients met the criteria for KD according to the standard of American Academy of Pediatrics and Cardiology Society,[1] and they also met the requirements for concurrent shock of KD proposed by Kanegaye et al. in 2009.[2] We retrospectively reviewed the medical records of 11 children diagnosed with KDSS in our hospital from February 2012 to February 2017. Among these 11 patients, 7 were male and 4 were female. Ages ranged from 1 month to 9 years. Clinical manifestations of shock occurred in the first 3–10 days during the disease course with a mean time of 6.1 days. Five of the 11 patients experienced shock within 5 days of KD onset. These data suggested that patients already exhibited shock even before KD diagnosis could be made. All the 11 cases showed persistent high fever, lymph node enlargement, and cardiac insufficiency. More than 50% of patients had multiple organ dysfunctions as listed in Table 1. Table 1 Clinical presentations of children with Kawasaki disease shock syndrome (n = 11) Clinical presentations Cases (n) Physical examination  Lymphadenectasis 11  Rash 10  Conjunctival hyperemia 10  Joint swelling of the palms or feet 9  Chapped lips 6 Clinical manifestations  Coronary artery dilatation 9  Shock within 5 days 5  Pneumonitis 7 Auxiliary examinations  Hypoalbuminemia 10  Hepatic insufficiency 8  Anemia 8  Hyponatremia 6  Electrocardiogram abnormalities 8  Urine routine abnormalities 5  Hypokalemia 3 Intravenous immunoglobulin (IVIG) and oral administration of aspirin were given in all KDSS patients, and re-treatment with IVIG was done in five patients who presented with IVIG resistance. In addition, all patients received fluid volume resuscitation, vasoactive drugs, and nutrition support treatments. Six patients received additional treatments including respiratory support and methylprednisolone. KDSS is considered a rare disease around the world, yet in the recent years, more interests have focused on the early diagnosis of KDSS. Clinical manifestations of KDSS are atypical.[3 4] It can rapidly develop into shock, and often with strong inflammatory responses which could lead to coronary artery disease and multiple organ dysfunctions. Therefore, early identification is particularly important. Through literature review and examination of our 11 patients, we have summarized the following characteristics for KDSS: (1) It is more common in males; (2) symptoms include lymphadenectasis, hypoalbuminemia, hyponatremia, hepatic insufficiency, anemia, and electrocardiogram abnormalities; the incidence of coronary artery dilatation is high, and some children may have severe gastrointestinal symptoms; (3) inflammatory indicators are significantly increased; (4) patients often present with IVIG resistance; (5) 50% of patients need hormone therapy; and (6) shock appears at an early stage. For the treatment of KDSS, IVIG combined with aspirin and vasoactive drugs is the current standard of care. Recent studies[5] have shown that glucocorticoids combined with a large dose of gamma globulin treatment can reduce the incidence of coronary artery disease in patients with severe KD. In this study, 11 patients were given anti-shock therapy, such as fluid resuscitation, vasoactive drugs, and respiratory support therapy. Further, six children underwent hormone therapy due to inflammatory reactions. In our study, a high dosage of methylprednisolone was given to one patient in the initial treatment. Disease progression was controlled, yet heart rate declined afterward. Arrhythmia occurred with the junctional escape rhythm and the onset of cyanosis followed. Vasoactive drugs could not maintain a normal range of blood pressure; therefore, a temporary pacemaker was implanted. For the other patients in our cohort, vital signs and blood and inflammation indicators returned to normal after shock through interventions. In summary, the cases in our study shared similar clinical manifestations with previously reported cases. However, in addition to standard care, respiratory support and hormone therapy were used in our patients, and temporary pacemakers were implanted when vasoactive drugs could not maintain a normal range of blood pressure. Our study indicated that early diagnosis and comprehensive therapeutic methods based on the disease progression are important in treating KDSS. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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          Most cited references6

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          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.

          Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in approximately 15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for > or =5 days and < or =4 classic criteria should undergo echocardiography [correction], receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-alpha antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.
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            Recognition of a Kawasaki disease shock syndrome.

            We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease. We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of > or =20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease. Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients [31%] vs 2 of 86 patients [4%]), mitral regurgitation (5 of 13 patients [39%] vs 2 of 83 patients [2%]), coronary artery abnormalities (8 of 13 patients [62%] vs 20 of 86 patients [23%]), and intravenous immunoglobulin resistance (6 of 13 patients [46%] vs 32 of 174 patients [18%]). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances. Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery abnormalities, mitral regurgitation, and prolonged myocardial dysfunction. These patients may be resistant to immunoglobulin therapy and require additional antiinflammatory treatment.
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              Management of Kawasaki disease

              Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.
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                Author and article information

                Journal
                Chin Med J (Engl)
                Chin. Med. J
                CMJ
                Chinese Medical Journal
                Medknow Publications & Media Pvt Ltd (India )
                0366-6999
                05 December 2017
                : 130
                : 23
                : 2891-2892
                Affiliations
                [1]Department of Cardiology, Children's Hospital Affiliated to the Capital Institute of Pediatrics, Beijing 100020, China
                Author notes
                Address for correspondence: Dr. Lin Shi, Department of Cardiology, Children's Hospital Affiliated to the Capital Institute of Pediatrics, Beijing 100020, China E-Mail: shilin9789@ 123456126.com
                Article
                CMJ-130-2891
                10.4103/0366-6999.219151
                5717875
                29176153
                f71af902-58bb-41c7-844f-bc12559c41b6
                Copyright: © 2017 Chinese Medical Journal

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                : 19 June 2017
                Categories
                Clinical Observation

                diagnosis and treatment,kawasaki disease,shock
                diagnosis and treatment, kawasaki disease, shock

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