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BRIEF REPORT
Ahead of print publication  

Clinical manifestations and outcomes of Kawasaki Disease: A retrospective hospital-based data from Eastern India


 Department of Paediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Date of Submission09-Jul-2021
Date of Acceptance03-Aug-2021
Date of Web Publication28-Oct-2021

Correspondence Address:
Sanjay Kumar Sahu,
Department of Paediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar - 751 024, Odisha
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_150_21

  Abstract 


Background: Kawasaki disease (KD) is a medium-vessel vasculitis having coronary predilection, usually affecting under-5 children presenting as acute febrile illness. Despite being a disease with long-term grievous outcome, only few published literature are available from India, even lesser from its eastern region.
Methods: From January 2016 to December 2020, 30 case records of children with a discharge diagnosis of KD were enrolled in this study. Demographic profile, clinical manifestations, laboratory data, echocardiographic findings, and treatment done were extracted from the case records. Laboratory investigations were done at admission and repeated after 24 h of intravenous immunoglobulin administration as per the American Heart Association Guidelines 2004. Echocardiography was carried out at diagnosis, at 2 weeks, and at 6 weeks.
Results: Out of 30 children diagnosed with KD, majority belong to 1–5 years of age group (72%) with male predominance. Complete KD was seen in 77% of children. The most common presentation was fever >5 days (100%) followed by oral changes in 26 (87%), conjunctivitis in 25 (83%), extremity changes in 23 (77%), and rash in 21 (70%) children. Desquamation of perineum and reactivation of bacillus Calmette–Guérin scar were seen in 10%. No children with complete KD and three children with incomplete KD developed coronary artery Abnormalities (CAA).
Conclusion: Infants with incomplete KD have a higher incidence of CAA. Aggressive management results in better outcome.

Keywords: Bacillus Calmette–Guérin reactivation, coronary artery abnormality, Kawasaki disease, perianal desquamation



How to cite this URL:
Behera JR, Rup AR, Dash AK, Jain MK, Sahu SK, Swain N, Polei R. Clinical manifestations and outcomes of Kawasaki Disease: A retrospective hospital-based data from Eastern India. Indian J Rheumatol [Epub ahead of print] [cited 2021 Dec 9]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=329486




  Introduction Top


Kawasaki disease (KD) is an idiopathic, acute febrile illness usually affecting under-5 children.[1] It is a medium-vessel systemic vasculitis commonly affecting the coronaries. Although self-limiting, 25% of children usually develop cardiac complications in the form of coronary artery abnormalities (CAAs), myocardial infarction, sudden death, or ischemic heart disease, unless early diagnosis and prompt treatment are made.[2] Uncertainty still prevails regarding the incidence of KD in India, but more cases are being diagnosed due to increasing awareness among clinicians. Complete variety of KD presents with unremitting fever, mucocutaneous features, and lymphadenopathy. It is usually diagnosed by either KD Research Committee. Guidelines (Japanese Guidelines 2002) or American Heart Association (AHA) Guidelines 2004. Incomplete KD is difficult to diagnose due to non-availability of any confirmatory laboratory test. It cause more coronary abnormalities than the complete variety.[3] High index of suspicion and availability of modified diagnostic algorithm for incomplete KD published in 2017 AHA guidelines have made the diagnosis little easier.[4] Prompt treatment with intravenous immunoglobulin (IVIG), which is the definitive treatment for KD, has reduced the incidence of CAA to 4%.[4] Recent studies on additional therapies such as aspirin have not shown any extra benefit by reducing residual cardiac risk.[4] Not much literature is available on KD cases from Eastern India at present. This study intends to find out demographic, clinical, and laboratory parameters, complications, and treatment response of KD from a tertiary care hospital in Eastern India.


  Methods Top


This study was a case record analysis, done in a tertiary care hospital in Odisha over a period of 5 years from January 2016 to December 2020. All patients under age 14 years with discharge diagnosis of KD were included in this study. Criteria for complete KD and incomplete KD were taken according to the guidelines given by AHA and American Academy of Pediatrics.[4] Information on the demographic profile, clinical profile, laboratory data, echocardiographic findings, and detailed treatment list was extracted from hospital records and collated on a Microsoft excel sheet after prior approval of the Institutional Ethics Committee of KIMS, KIIT University, Bhubaneswar. In all children, investigations such as complete blood count (CBC), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were done at admission. Echocardiography was carried out at diagnosis, at 2 weeks, and at 6 weeks. Abnormalities (CAA) was defined as per criteria given by the Ministry of Health and Welfare of the Japanese government.[5] All children were treated with a single dose of IVIG infusion at 2 g/kg administered over 12–24 h along with aspirin 30–50 mg/kg in 3–4 divided doses. Investigations (CBC, CRP, and ESR) were repeated after 24 h of initial dose of IVIG. Aspirin dose was reduced to 3–5 mg/kg after the child was afebrile and continued for 6–8 weeks. Repeat dose of IVIG was given to children having persistent fever after 36 h of end of the first dose of IVIG infusion, which is known as resistant KD.[4] Antithrombotics and thrombolytics were added in children who had coronary abnormality. Aspirin was stopped in children who had no CAA after 6 weeks.


  Results Top


Case records of children discharged with diagnosis of KD were analyzed and 30 children were included in the study. Mean age was 30 months with interquartile range 14–36 months. Most of the children (72%) were aged between 1 and 5 years. There was a male predominance with ratio of 4:1. The incidence was distributed throughout the year, but the peak was seen during the months of February to May.

Complete KD was seen in 23 (77%) children. Most common clinical presentation was fever >5 days, being present in all cases. Oral changes were present in 26 (87%), conjunctivitis in 25 (83%), extremity changes in 23 (77%), rash in 21 (70%), and cervical lymphadenopathy in 19 (63%) children. Gastrointestinal (GI) symptoms such as diarrhea, vomiting, and pain abdomen were seen in 6 (20%). Central nervous system (CNS) symptoms such as irritability, altered sensorium, and seizure were observed in 6 (20%) children. Arthralgia, perianal desquamation, and bacillus Calmette–Guérin (BCG) scar reactivation were seen in 4 (13%), 3 (10%), and 3 (10%) cases, respectively. Comparison between the parameters of complete and incomplete KD is depicted in [Table 1]. Sterile pyuria was seen in 17 (57%), hypoalbuminemia (serum albumin <3 g/dl) in 12 (40%), high alanine aminotransferase in 13 (43%).
Table 1: Comparison between complete Kawasaki disease and incomplete Kawasaki disease

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In all children, echocardiography was done at diagnosis. No children with complete KD and three children with incomplete KD developed coronary abnormalities. Echocardiography was followed up at 2 and 6 weeks. Two patients had mild coronary arterial dilation of the left main coronary artery (LMCA) which stabilized during follow-up at 6 weeks. The third child had multiple coronary abnormalities with dimensions of LMCA 5.8 mm (Z score 12.58), proximal left circumfle × 4.84 mm (Z score 11.30), LAD 2.90 (Z score 5.36), and right coronary artery (RCA) 2.46 mm (Z score 3.22). On follow-up at 6 weeks, the dimensions of coronaries have stabilized with no further increase in diameter. During the last follow-up, the LMCA was 4 mm (Z score 7.19) and the RCA was 2 mm (Z score 2.54).

All children were treated with a single dose of IVIG infusion, but 2 (7%) children required repeat dose of IVIG due to persistent of fever after 36 h of end of the first dose of IVIG infusion. Both the children responded to the second dose of IVIG and became afebrile within 48 h of initiation.


  Discussion Top


Although the exact etiology of KD is not known, the possible role of an infectious agents in a genetically predisposed individual is proposed. As compared to Japan where the incidence is 265 per 1 lakh children under the age of 5 years, India has an incidence of 4.5 per 1 lakh under 15 children.[6],[7] However, no data from Eastern India are available. Data from Japan, North America, and Europe show that majority of affected children are below 5 years.[7] A study from Chandigarh revealed that almost half of the children with KD were above 5 years.[6] Demographic profile in our study is similar to most of the previous studies but in contrast to Singh et al.[6],[7] Our study reveals peak in spring and early summer in contrast to extratropical northern hemisphere having seasonal peaks in winter.[8]

In our study, fever was found in all cases. Oral mucosal changes in the form of strawberry tongue and cracked lips were the most frequent finding whereas unilateral cervical lymphadenopathy was the least clinical finding. In contrast, previous studies found conjunctivitis being more common.[9],[10] Desquamation of the perianal area and reactivation of the BCG scar were observed fewer children (10%) but are key pointers toward diagnosis in acute stage, when other typical features are not present as suggested by Singh et al.[3] Frequency of musculoskeletal, GI, and CNS symptoms was less, which is consistent with findings of others.[11],[12]

The total leukocyte count, ESR, and CRP are regarded as the severity markers. Between complete and incomplete KD groups Behmadi et al. found that there is no significant difference of above-said parameters, which is similar to our study.[11] Anemia and thrombocytosis are frequent findings, in our study, though not statistically significant when equated between complete and incomplete KD. Comparable findings were also observed by Behmadi et al. and Manlhiot et al.[11],[13] Higher incidence of sterile pyuria, hypoalbuminemia, and transaminitis was found as compared to Iranian study.[11]

On comparing complete and incomplete KD groups, our study revealed significant difference in the development of CAA between two groups. Similar findings were witnessed by various researchers recently.[13] However, a study from Greece revealed 10 times fewer incidence of CAA in incomplete KD, which might be due to initiation of IVIG at an earlier stage or over diagnosis of KD.[14]

Nonresponse rate to first dose of IVIG of the present study was similar to the observations made by Kim et al (11.6%). However, Giannouli et al documented a higher percentage of non-ssresponders to IVIg (25.6%).[14],[15]

Smaller sample size and retrospective nature are the major drawbacks of this study. Prospective multicenter study with a larger sample size is required to determine the magnitude and risk factors of KD in our population.


  Conclusion Top


Infants with incomplete KD are at increased risk of developing coronary artery aneurysm. Increased awareness about this entity and prompt treatment improves the outcome significantly. The epidemiological variability of KD between India and Japan warrants detailed research on genetic and environmental factors. Although awareness about KD has increased in recent times, a pan India registry is highly desired which may unveil many unknown aspects.

Ethical statement

The study was approved by Institutional ethics committee of KIMS vide letter no KIIT/KIMS/IEC/657/2021.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shenoy B, Singh S, Ahmed MZ, Pal P, Balan S, Viswanathan V, et al. Indian academy of pediatrics position paper on Kawasaki disease. Indian Pediatr 2020;57:1040-8.  Back to cited text no. 1
    
2.
Singh S, Sharma A, Jiao F. Kawasaki disease: Issues in diagnosis and treatment--a developing country perspective. Indian J Pediatr 2016;83:140-5.  Back to cited text no. 2
    
3.
Singh S, Jindal AK, Pilania RK. Diagnosis of Kawasaki disease. Int J Rheum Dis 2018;21:36-44.  Back to cited text no. 3
    
4.
McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A scientific statement for health professionals from the American Heart Association. Circulation 2017;135:e927-99.  Back to cited text no. 4
    
5.
Ministry of Health and Welfare, Research Committee on Kawasaki Disease. Report of the Subcommittee on Standardization of Diagnostic Criteria and Reporting of Coronary Artery Lesions in Kawasaki Disease. Tokyo, Japan: Ministry of Health and Welfare; 1984.  Back to cited text no. 5
    
6.
Singh S, Aulakh R, Bhalla AK, Suri D, Manojkumar R, Narula N, et al. Is Kawasaki disease incidence rising in Chandigarh, North India? Arch Dis Child 2011;96:137-40.  Back to cited text no. 6
    
7.
Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. 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. Circulation 2004;110:2747-71.  Back to cited text no. 7
    
8.
Burns JC, Herzog L, Fabri O, Tremoulet AH, Rodó X, Uehara R, et al. Kawasaki Disease Global Climate Consortium. Seasonality of Kawasaki disease: a global perspective. PLoS One. 2013;8:e74529.  Back to cited text no. 8
    
9.
Maggio MC, Corsello G, Prinzi E, Cimaz R. Kawasaki disease in Sicily: Clinical description and markers of disease severity. Ital J Pediatr 2016;42:92.  Back to cited text no. 9
    
10.
Sakina S, Owais SS, Khan EA, Sheikh AM. Kawasaki disease: Clinico-laboratory spectrum and outcome in a cohort of children treated at a tertiary care hospital in Islamabad, Pakistan. Pak J Med Sci 2020;36:260.  Back to cited text no. 10
    
11.
Behmadi M, Alizadeh B, Malek A. Comparison of clinical symptoms and cardiac lesions in children with typical and atypical Kawasaki disease. Med Sci (Basel) 2019;7:E63.  Back to cited text no. 11
    
12.
Zulian F, Falcini F, Zancan L, Martini G, Secchieri S, Luzzatto C, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr 2003;142:731-5.  Back to cited text no. 12
    
13.
Manlhiot C, Christie E, McCrindle BW, Rosenberg H, Chahal N, Yeung RS. Complete and incomplete Kawasaki disease: Two sides of the same coin. Eur J Pediatr 2012;171:657-62.  Back to cited text no. 13
    
14.
Giannouli G, Tzoumaka-Bakoula C, Kopsidas I, Papadogeorgou P, Chrousos GP, Michos A. Epidemiology and risk factors for coronary artery abnormalities in children with complete and incomplete Kawasaki disease during a 10-year period. Pediatr Cardiol 2013;34:1476-81.  Back to cited text no. 14
    
15.
Kim GB, Han JW, Park YW, Song MS, Hong YM, Cha SH, et al. Epidemiologic features of Kawasaki disease in South Korea: Data from nationwide survey, 2009-2011. Pediatr Infect Dis J 2014;33:24-7.  Back to cited text no. 15
    



 
 
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