167 - INFANTILE KAWASAKI DISEASE: A DISTINCT ENTITY

Background:

Infants with Kawasaki disease (KD) mostly present with fewer signs and symptoms, leading to delayed diagnosis and subsequent late initiation of intravenous immunoglobulin (IVIG). This in turn increases the incidence of coronary artery abnormalities (CAA). 

AIMS AND OBJECTIES:

To assess the epidemiology, clinical features, laboratory investigations, treatment given and outcome in infants with KD. to compare the epidemiology and laboratory investigations in those up to 1 year of age with those more than 1 year of age.

METHODS

It is a retrospective, observational study of data on children with Kawasaki Disease (KD) aged less than or equal  to one year, admitted  at Institute of Child Health, Kolkata, India,  from January 2020 to August 2023. 

RESULTS

103  KD patients were included of whom 43 (42%)  were infants. Median age of the infants was 7 months, the youngest being 6 weeks old.  Off the infants 7 (16%) were 0 - 3months, 14 (33%) more 4 - 6months and 22 (51%)  were > 6months old.  30 (70%) were males. 28 (65%) presented as incomplete KD; The median duration of illness at admission was 7 days and median duration of illness when first dose of intravenous immunoglobulin (IVIG) was administered was 8 days. (table 1)

Mucocutaneous manifestations were most common.

IVIG was given to all. 7 (16%) were resistant to IVIG and were given infliximab (IFX) of whom had 1 developed IVIG resistance inspite of receiving steroids along with IVIG due to high Kobayashi score.

9 had small coronary artery aneurysm (CAA) , of whom 1 received Infliximab (IFX) and rest received steroids. All small CAA regressed

8 had medium CAA.  Of these 6 received IFX.  1 had normal echo findings at diagnosis, had received steroids for high Kobayashi, yet developed medium CAA on follow up requiring IFX, one had rising CAA inspite of IVIG and IFX  with thrombus formation requiring additional cyclosporine. All medium CAA patients received anticoagulation. In & patients CAA has regressed. In 1 patient aneurysms are still persisting.  

 1 patient with late presentation at 12 days of illness had Giant CAA requiring additional  steroids, cyclosporin,  IFX and anticoagulation. CAA have regressed to small aneurysm.  

Investigations are tabulated in Table 2.

Conclusion: infants have a higher incidence of incomplete manifestations IVIG resistance and  aneurysms. In our cohort, day of illness at diagnosis was comparable. High index of suspicion is required to diagnose KD in infants with incomplete manifestations.