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Kawasaki disease (KD) is a vasculitis
which has a penchant for the coronary arteries, with occurrence
of coronary artery aneurysms(CAA) in 25% of cases [1]. It is the
commonest acquired heart disease among children in the Western
world and recent data suggest that the same may be true for our
country also [2]. The clinical manifestations of the disease are
myriad and this coupled with the lack of a single diagnostic
investigation makes accurate diagnosis difficult. Unnecessary
delay in the diagnosis increases the risk of coronary aneurysms
and increases morbidity in a potentially self-limiting disorder.
There is a paucity of Indian literature with regard to factors
associated with delayed diagnosis of Kawasaki Disease [2,3]. We
report a series of cases of Kawasaki disease with specific
reference to factors delaying diagnosis.
Methods
This was a retrospective analysis of all
children diagnosed to have KD at a tertiary care center between
August 2009 and July 2011. Clearance was obtained from the
Institutional Ethics Committee. A single investigator obtained
the history and conducted the physical examination of all the
patients after a diagnosis of Kawasaki was made or suspected. An
echocardiogram was done as soon as Kawasaki disease was
suspected and repeated if fever did not settle after IvIg
therapy. All patients also had a follow up echocardiogram at 6
weeks. Diagnosis was established based on the AHA diagnostic
criteria for Kawasaki disease [4]. The cases were categorized as
into Early Diagnosis (ED) group, if diagnosed before 10 days and
Delayed Diagnosis (DD) group if diagnosed later. The data in
these two groups were analysed using SPSS v17 with regard to the
differences in clinical and laboratory parameters.
Results
A total of 37 children (26 boys) were
diagnosed to have Kawasaki disease during the study period. The
ages ranged from 4 months to 14 years. 20 (54%) were between 1
and 5 years while 10 (27%) were less than 1 year. 19 (51%) had
complete Kawasaki disease satisfying all criteria of the AHA
guidelines while 18 (49%) had incomplete disease. 24 (65%)
children were in the ED group and 13 (35%) were in the DD group.
Coronary artery abnormalities (CAA) on echocardiography were
found in 9 children out of which 7(77%) were in the DD group.
All patients referred from outside our
hospital were referred by pediatricians. None of the patients in
the DD group was referred as Kawasaki disease while two of 6
patients in the ED group were referred as Kawasaki disease. One
child in the ED group developed recurrence of the disease 6
months after the initial attack. 6 children had disease
recrudescence. 5 were in the ED group and one of those developed
CAA during recrudescence. 34 children were treated with IvIg and
two were treated with corticosteroids because of socio-economic
considerations. Among children with disease recrudescence, five
received a second dose of IvIg while one child received
corticosteroids.
Referrals from private pediatricians, lower
hemoglobin, higher platelet counts and increased coronary artery
abnormalities (CAA) were statistically significant factors
associated with delayed diagnosis (Table I).
Clustering of symptoms, particularly cervical lymphadenopathy
and oral lesions, was associated with early diagnosis.
TABLE I Differences Between Early and Late Diagnosis of Kawasaki Disease
|
Parameter (mean)
|
Early diagnosis |
Delayed diagnosis
|
|
(n= 24 ) |
(n=13 ) |
|
Age (y) |
|
Onset of findings(d) |
2.92 |
4.33 |
|
Fever
|
1.21 (1-3) |
1.31 (1-3) |
|
Conjunctivitis
|
4.26 (3-7) |
4.40 (4-11) |
|
Skin lesion
|
3.33 (1-8) |
3.36 (3-11) |
|
Neck swelling* |
3.53 (1-7) |
6.14 (5-11) |
|
Oral lesions* |
3.39 (3-5) |
5.89 (3-12) |
|
Hemoglobin (g %)* |
10.4 |
9.3
|
| TLC
(cells/mm3)
|
19,375 |
22,223 |
|
ESR (mm/hr) |
74.5 |
90.7 |
|
CRP (IU/L)) |
89.4 |
44.8 |
|
Platelets (in lakhs/mm3) |
4.3 |
6.4 |
|
CAA on ECHO* |
2 |
7 |
|
Incomplete KD |
10 |
8 |
|
Referral* |
6 |
10 |
CRP- C-Reactive
Protein, ECHO- Echocardiogram, ESR- Erythrocyte
sedimentation rate, KD- Kawasaki disease, TLC- Total
leukocyte count;
* P<0.05; CAA - Coronary artery abnormalities. |
Discussion
We recognized that referral from
pediatricians outside the institute and wide dispersion of
symptoms with time were features associated with delayed
diagnosis. In particular, onset of neck swelling and oral
lesions occurred later in the DD group. The DD group also had
lower hemoglobin, higher platelet counts and more CAAs.
In our study, 35% of children had delayed
diagnosis in comparison to reports from the USA (16-27%) [5-7]
and Taiwan (18%) [8]. This illustrates the need for increased
awareness among pediatricians in India to the diagnosis and
importance of early treatment. Referrals from pediatricians
outside our hospital was more in the DD group. Literature
provides conflicting data on physician delay in diagnosis.
Anderson, et al. [5] found no difference between the two
groups based on number of physician visits or speciality of
treating physician; while others suggested that increased
distance from medical center and delay in diagnosis by
physicians could be important in delaying diagnosis [6,7]. There
were no comments on referral pattern in these studies. This
could reflect difference in the style of medical practice,
institutional attachments and referral guidelines between India
and USA.
We live in an era where classical Kawasaki
disease continues to be missed frequently [9] and the prospect
of incomplete KD appears more daunting. In our study, though the
number of incomplete cases was more in the DD groups, this
association was not statistically significant. Similar
observations have been reported in literature earlier [5,6,10].
Wider dispersion of symptoms had been
reported as a risk factor for delayed diagnosis [5,8]. In our
series, wide dispersal of time of onset of cervical adenopathy
and oral lesions were significantly associated with delayed
diagnosis. Juan, et al. [8] noticed higher white blood
cell counts in DD group but no such association was found in our
study. Children in the DD group had lower hemoglobin in our
study. This association has not been reported in the literature.
However, platelet counts were higher in the DD group consistent
with earlier reports in literature [5,6,8]. CAAs were more
common in the DD group. A longer period of inflammation leads to
an increased incidence of CAAs [13]. The efficacy of IVIg in
reversing inflammation also decreases with prolonged duration of
inflammation [14,15].
This is a retrospective analysis carried out
at a single private children hospital with no fixed referral
population and hence might not be truly indicative of the
situation in the entire community. Recent data suggest that
incidence of KD is increasing in India [16]. In such a scenario,
our observations suggest that improving awareness amongst
practicing pediatricians across the community might facilitate
early diagnosis and thereby possibly prevent coronary sequelae.
Acknowledgment: CHILDS Trust Medical
Research Foundation for their support. We also thank Dr.
Gnanasambandam, pediatric cardiologist for performing all the
echocardiograms and Mr Arun, Statistician for helping with
statistical analysis.
Contributors: SBS: Designed the study,
supervised the data collections, finalised the manuscript and
stands as guarantor for the data; MRK: Collected the data,
carried literature search and prepared the manuscript; KD and
SA: supervised data collection and analysed the clinical data;
AVR: helped to design the study, prepare the manuscript and
obtaining references.
Funding: None;
Competing interests: None stated.
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What This Study Adds?
• Children presenting primarily to a
tertiary care setting in India are more likely to be
associated with early diagnosis of KD than those
initially seen by pediatricians in the community.
|
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