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Indian Pediatr 2009;46: 607-609 |
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Coronary Artery Dilatation in Incomplete
Kawasaki Disease |
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AP Vijayan, KB Dinesh and KR Divia Nath
From Department of Pediatrics, Malabar Institute of
Medical Sciences, Calicut, Kerala, India.
Correspondence to: Dr A P Vijayan, Consultant
Pediatrician, Malabar Institute of Medical Sciences, Mini Baypass Road,
Govindapuram PO, Calicut 673 016, Kerala, India.
E-mail: [email protected]
Manuscript received: June 5, 2008;
Initial review : January 29, 2009;
Accepted: April 13, 2009.
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Abstract
We conducted this study to compare the incidence of
coronary artery dilatation in children with Incomplete and Classical
Kawasaki disease, diagnosed as per AHA criteria. Subjects were included
on a retrospective review of records (2002-2007); those with a discharge
diagnosis of Kawasaki disease were enrolled. A total of 29 patients were
identified (3.1 per 1000 pediatric admissions), out of which 22 were
boys (median age: 4.8 years; range: 4 months-11 years). Seventeen
(58.6%) had Classical KD and twelve (41.4%) children had Incomplete KD.
All children received IVIG and underwent echocardiography. Coronary
involvement was more in Incomplete KD (11/12 = 91.6 %) as compared to
Classical KD (7/17= 41.1%). The sensitivity, specificity and predictive
value of AHA criteria to detect coronary artery dilatation was 39%, 9%,
and 41%, respectively. We conclude that children presenting with
Incomplete Kawasaki disease are at a higher risk of coronary artery
abnormalities.
Key words: AHA criteria, Coronary artery abnormalities,
Diagnosis, Kawasaki disease.
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Coronary artery dilatations or aneurysms
develop in approximately 25% of untreated children with Kawasaki disease
and may lead to myocardial infarction (MI), sudden death, or ischemic
heart disease(1). Early diagnosis and treatment with intravenous immune
globulin (IVIG) can reduce the incidence of coronary artery abnormalities
(CAA) to <5%(2-4).
The diagnosis of Kawasaki disease is based on clinical
criteria summarized by the American Heart Association (AHA) in 1993(2).
These include fever for 5 or more days; four of the other five findings
i.e. a polymorphous exanthem, nonpurulent conjunctivitis, changes in the
lips or oral cavity, redness and edema with later desquamation of the
extremities, and at least one cervical lymph node that is >1.5 cm in
diameter and no evidence of another disease with similar clinical
features. Incomplete Kawasaki disease is being increasingly diagnosed.
According to AHA, incomplete Kawasaki disease should be considered
in all children with unexplained
fever for 5 days associated with 2 or 3 of the principal clinical features
of Kawasaki disease. We sought to deter-mine the incidence of cases
of Incomplete Kawasaki disease; and whether there is a significant
difference in the incidence of coronary artery abnormalities between
Classical and Incomplete Kawasaki disease.
Methods
The study was conducted at Malabar Institute of Medical
Sciences, a tertiary referral center. The records
of all children between 2002 and 2007 with a discharge diagnosis of
Kawasaki disease were reviewed retrospectively. The diagnosis of Classical
or Incomplete Kawasaki disease was based on American Heart Association
criteria(2). Hemoglobin, blood count, ESR, platelet count and routine
urine examination were done in all patients. Children in Classical and
Incomplete Kawasaki disease groups were analyzed with respect to duration
of fever, presence of the other AHA criteria, interval from symptom onset
to initiation of treatment with IVIG, presence of coronary artery
dilatation determined by echocardiography, and the results of all
laboratory tests. Characteristics of the two groups were compared by
Chi-square test.
All patients received IVIG in a dose of 2 g/kg
bodyweight on day 1 of admission. One child failed to respond despite 2
doses of IVIG and needed steroids, in view of persisting fever and other
symptoms.
Results
During the 5-year study period, we had 29 patients
diagnosed with KD (3 cases per 1000 pediatric admissions). Of the 29
cases, 22 (76%) were males and 70 % of the cases were <5 years.
Table I compares the characteristics of
children with classical and Incomplete Kawasaki Disease. One child
with Classical KD had sensorineural hearing loss. One patient in the
Incomplete KD group was initially diagnosed with dengue shock syndrome.
Majority of children presented late (average 10 days after onset of
fever). Of 18 (62%) children with coronary involvement, 11 (61.1%) did not
meet the AHA criteria.
TABLE I
Comparative Features of Classical and Incomplete Kawasaki Disease
| Features |
Classical KD |
Incomplete KD |
P value |
| Total cases |
17 |
12 |
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| Males |
10 |
11 |
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| Age of presentation (y) Mean (SD) |
4.43 (2.54) |
5.25 (3.62) |
>0.05 |
| Duration of fever (d) Mean (SD) |
9.76 (6.4) |
11.17 (7.1) |
>0.05 |
| Rash |
13 (76.5%) |
7 (58.3 %) |
>0.05 |
| Lymphadenopathy |
15 (88.2%) |
6 (50%) |
<0.05 |
| Conjunctival congestion |
13 (76.4%) |
5 (41.7%) |
<0.05 |
| Oral mucosal changes |
16 (94.1%) |
8 (66.7%) |
<0.05 |
| Peeling |
10 (58.8%) |
2 (16.7%) |
<0.05 |
| Arthritis |
6 (35.3%) |
– |
|
| Coronary dilatation |
7 (41.1%) |
11 (91.7%) |
< 0.001 |
| Thrombocytosis |
14 (82.3%) |
6 (50%) |
<0.05 |
| Pyuria |
8 (47 %) |
3 (25%) |
>0.05 |
| Jaundice |
1 |
– |
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| Giant peripheral aneurysm |
1 |
– |
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Coronary involvement was seen in 7 of the 8 cases (87%)
who presented late in the Incomplete group, while all the 4 children who
had an early presentation of Incomplete KD also had coronary dilatation.
In the Classical group, coronary involvement was seen only in 2 of the 8
cases who had fever >10 days (25%) while 5 of the remaining 9 patients
(55%) had coronary abnormalities.
There was no significant difference in the mean age of
those with or without coronary involvement. Giant axillary and coronary
aneurysm was noted in one infant who had fever for more than one month.
Discussion
Our series data shows a high number of cases not
satisfying AHA criteria. This might probably be due to increased awareness
among the clinicians of a hitherto unknown disease with cardiac
complications affecting otherwise normal children, and the fact that early
treatment may prevent the risk of developing coronary artery
abnormalities. Literature review suggests that early treatment with IVIG
within the first 10 days of onset of symptoms is effective in
decreasing the incidence of coronary artery dilatation(1,2,4). We noted
that coronary artery dilatation was more in patients who did not meet AHA
criteria.
Is it probably because coronary artery dilatation may
be developing slightly earlier in incomplete cases as compared to
classical KD where we have the opportunity to start treatment early? We
have not been able to find published reviews corroborating this and number
of our cases is too small to demonstrate statistical significance. Or is
it because the incomplete KD is basically a virulent subgroup of Kawasaki
disease with more predilections for coronary arteries? In our series
children with fever and single criteria like oral mucosal changes are not
included. Do they have the risk of developing coronary artery dilatations
as incomplete or complete KD? We have not studied their coronaries.
And if so, are we missing out a major proportion of cases, which could be
potential KD with dilated or normal coronaries?
This study points out the need for better diagnostic
modalities to detect children susceptible for developing CAA. We agree
that this study has limitations as it is a retrospective review. A
prospective study with a large sample size could address some
of the issues, clinicians will continue to face difficult situations until
sensitive and specific diagnostic tests are developed for Kawasaki
disease.
Our study has shown that the incidence of Incomplete
Kawasaki disease is high, and the incidence of coronary artery dilatation
is more in these children. We recommend that clinicians continue to
maintain high levels of suspicion, even in the absence of the complete
clinical picture of KD.
Acknowledgments
Management of Malabar Institute of Medical Science.
Contributors: APV conceived and designed the study
and managed the cases. KBD conducted the literature search and
collected all data pertaining to the study. KRD collected data and
analyzed results. All authors contributed to preparation of the
manuscripts.
Funding: None.
Competing interests: None stated.
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What This Study Adds?
• Incidence of coronary artery dilatation is
more in incomplete Kawasaki disease, as compared to classical KD. |
References
1. Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R,
Michie C, et al. Kawasaki disease: an evidence based
approach to diagnosis, treatment, and proposals for future research. Arch
Dis Child 2002; 86: 286-290.
2. Newburger J , Takahashi M, Gerber MA, Gewitz MH,
Tani LY, 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: 2747-2751.
3. Maconochie IK. Kawasaki disease. Arch Dis
Child Educ Pract Ed 2004 ; 89: ep3-8.
4. Oates-Whitehead RM, Baumer JH, Haines L, Love S,
Maconochie IK, Gupta A, et al. Intravenous immunoglobulin
for the treatment of Kawasaki disease in children. Cochrane Database
System Rev 2003; 4: CD 004000.
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