Kawasaki Disease: Is Atypical More Common than Typical
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《印第安小儿科》
Institute of Child Health,11, Dr Biresh Guha Street,Kolkata 700 017, India.
Kawasaki Disease (KD) is a vasculitic disorder and has replaced rheumatic fever as the commonest cause of acquired heart disease in children(1,2). In India, KD perhaps is more common than it has been diagnosed and reported.
The profile of KD patients admitted in the Institute of Child Health, Kolkata in the period from May 2003 to June 2004 was taken up for this study. A total of six patients of either typical or atypical KD were included in the study. Detailed clinical evaluation and echo-cardiography was done in all the patients. Age varied between 3 and 6 years. Two of them (Case II & V) presented with fever of 10 and 11 days respectively. The remaining had fever of less than 10 days duration. Three of the cases (Case I, II, III) had less than 4 of the classical clinical features of typical KD and were diagnosed only after echocardiography showing characteristic coronary artery findings. These three cases were labeled as Atypical KD. One case (Case V) fulfilled all the clinical criteria of KD, but did not develop any coronary artery abnormalities. The echocardiographic findings are described in Table I. LMCA diameter above 2.5 mm is considered dilated, any value above 2 mm for RCA is considered dilated and any value above 2.3 mm for LAD is considered dilated(3). Repeat echocardioghraphy at 2 months of cases I, II and V showed normal coronary arteries with minimal luminal irregularity. Case VI at 3 months showed still dilated LMCA (3.1 mm).
LMCA = Left Main Coronary Artery, RCA = Right Coronary Artery,
LAD = Left Anterior Descending Artery, N = Normal
Atypical or incomplete KD cases are those who develop fever with fewer than 4 of the classical clinical features of the disease, but develop coronary artery disease. In this study 3 of the 6 cases were atypical KD patients.
The diagnosis of KD or atypical KD is largely clinical and a high degree of suspicion needs to be borne in mind. Since KD is not being diagnosed frequently in our country(4,5) majority of the affected patients are at present being left untreated rendering them liable to coronary complications, contributing to the total load of coronary artery disease in young adults who have no other risk factor. Routine use of echocardiography, in patients with features suggestive of KD, though not fulfilling all the criteria to be labeled KD, might go a long way in early detection of this potentially devastating cardiac complication and initiation of early prophylaxis might prevent some of the unexplained myocardial infarctions of early life.
References
1. Taubert KA, Rowley AH, Shulman ST. A 10 year (1984-1993) United States hospital survey of Kawasaki disease. In: Kato H. ed. Kawasaki disease. 1st edn. Amsterdam: Elsevier Science; 1995; p. 34-38.
2. Sundel RP, Petty RE. Kawasaki Disease. In: Cassidy JT, Petty RE. eds. Textbook of Pediatric Rheumatology, 5th edn. Philadelphia: WB Saunders; 2005; p.521-538.
3. Fukushege J, Takashashi N, Ueda K, Hiju T, Igarashi H, Ohshima A. Longterm outcome of coronary abnormalities in patients after Kawasaki disease. Pediatr Cardiol 1996; 17: 71-76.
4. Singh S. Kawasaki Disease: A clinical dilemma. Indian Pediatr 1999; 36: 871- 875.
5. Singh S, Kumar L. Kawasaki Disease: Treatment with intravenous immunoglobulin during the acute stage. Indian Pediatr 1996; 33: 689-692.(Kirtisudha Mishra,Jaydeep)
Kawasaki Disease (KD) is a vasculitic disorder and has replaced rheumatic fever as the commonest cause of acquired heart disease in children(1,2). In India, KD perhaps is more common than it has been diagnosed and reported.
The profile of KD patients admitted in the Institute of Child Health, Kolkata in the period from May 2003 to June 2004 was taken up for this study. A total of six patients of either typical or atypical KD were included in the study. Detailed clinical evaluation and echo-cardiography was done in all the patients. Age varied between 3 and 6 years. Two of them (Case II & V) presented with fever of 10 and 11 days respectively. The remaining had fever of less than 10 days duration. Three of the cases (Case I, II, III) had less than 4 of the classical clinical features of typical KD and were diagnosed only after echocardiography showing characteristic coronary artery findings. These three cases were labeled as Atypical KD. One case (Case V) fulfilled all the clinical criteria of KD, but did not develop any coronary artery abnormalities. The echocardiographic findings are described in Table I. LMCA diameter above 2.5 mm is considered dilated, any value above 2 mm for RCA is considered dilated and any value above 2.3 mm for LAD is considered dilated(3). Repeat echocardioghraphy at 2 months of cases I, II and V showed normal coronary arteries with minimal luminal irregularity. Case VI at 3 months showed still dilated LMCA (3.1 mm).
LMCA = Left Main Coronary Artery, RCA = Right Coronary Artery,
LAD = Left Anterior Descending Artery, N = Normal
Atypical or incomplete KD cases are those who develop fever with fewer than 4 of the classical clinical features of the disease, but develop coronary artery disease. In this study 3 of the 6 cases were atypical KD patients.
The diagnosis of KD or atypical KD is largely clinical and a high degree of suspicion needs to be borne in mind. Since KD is not being diagnosed frequently in our country(4,5) majority of the affected patients are at present being left untreated rendering them liable to coronary complications, contributing to the total load of coronary artery disease in young adults who have no other risk factor. Routine use of echocardiography, in patients with features suggestive of KD, though not fulfilling all the criteria to be labeled KD, might go a long way in early detection of this potentially devastating cardiac complication and initiation of early prophylaxis might prevent some of the unexplained myocardial infarctions of early life.
References
1. Taubert KA, Rowley AH, Shulman ST. A 10 year (1984-1993) United States hospital survey of Kawasaki disease. In: Kato H. ed. Kawasaki disease. 1st edn. Amsterdam: Elsevier Science; 1995; p. 34-38.
2. Sundel RP, Petty RE. Kawasaki Disease. In: Cassidy JT, Petty RE. eds. Textbook of Pediatric Rheumatology, 5th edn. Philadelphia: WB Saunders; 2005; p.521-538.
3. Fukushege J, Takashashi N, Ueda K, Hiju T, Igarashi H, Ohshima A. Longterm outcome of coronary abnormalities in patients after Kawasaki disease. Pediatr Cardiol 1996; 17: 71-76.
4. Singh S. Kawasaki Disease: A clinical dilemma. Indian Pediatr 1999; 36: 871- 875.
5. Singh S, Kumar L. Kawasaki Disease: Treatment with intravenous immunoglobulin during the acute stage. Indian Pediatr 1996; 33: 689-692.(Kirtisudha Mishra,Jaydeep)