Katsuhiko Sone

Corticosteroids in the treatment of the acute phase of Kawasaki disease

OBJECTIVES Corticosteroids are considered to be contraindicated during the acute phase of Kawasaki disease (KD) based on unfavorable results in early studies. In our hospital, however, corticosteroids have been used in some cases of KD with satisfactory results. We analyzed outcomes of patients with KD treated with or without corticosteroids. STUDY DESIGN Medical records of 299 children with KD treated with one of the 4 regimens were reviewed retrospectively. Regimen 1 consisted of aspirin, dipyridamole, and propranolol; regimen 2 was regimen 1 plus prednisolone, 2 mg/kg/d, for 1 week, followed by tapering over 2 weeks; regimen 3 was regimen 1 plus intravenous gamma-globulin (IVGG), 200 or 400 mg/kg/d, for 5 consecutive days; and regimen 4 was regimen 1 plus both prednisolone and IVGG. RESULTS Although patients treated with regimens 2 and 4 were more ill at presentation than those treated with regimens 1 and 3, respectively, the duration of fever was shorter in the former patient groups (P =.0013). Coronary aneurysms developed least frequently in patients treated with regimen 4 and less frequently with regimen 2 than with regimen 1 (P =.0730). Multiple regression analysis showed significant reductions of fever and coronary aneurysm incidence with prednisolone (P <.0001 and P =.0307, respectively). CONCLUSION Our data suggest a possible role of corticosteroids in the treatment of the acute phase of KD.

Short arm deletion of chromosome 1: del(1)(p13.3 p22.3) in a female infant with an extreme tetralogy of Fallot.

High‐resolution chromosome analysis showed the karyotype 46,XX,del(1)(p13.3 p22.3) in a female infant with an extreme tetralogy of Fallot and multiple congenital anomalies. The patient showed characteristic features: upper and lower eyelids connected to each other by a string‐like epithelium, low hairline, epicanthal folds, saddle nose with a broad, flat root, micrognathia, short neck, high‐arched palate, prominent xiphisternum, wide‐spaced nipples, bilateral pes equinovarus, fifth toes that overlapped the fourth toes bilaterally, a deep fissure between the first and second toes bilaterally, and abnormal flexions of fingers and toes. Growth and psychomotor retardation were also noted. Cardiac catheterization revealed an extreme tetralogy of Fallot complicated by a patent ductus arteriosus. Ventricular tachycardia and ventricular premature beats developed during the neonatal period and did not respond well to anti‐arrhythmic drugs. She died of the anoxia caused by closure of the patent ductus arteriosus when she was 7 months old.

Effect of dipyridamole on the blood flow in coronary aneurysms resulting from Kawasaki disease.

SummaryDipyridamole has been widely used in Japan to treat patients with a coronary aneurysm resulting from Kawasaki disease, but its effect in these patients has not been established. In the present study we assessed the effect of dipyridamole on the coronary arteries of patients with a history of Kawasaki disease by measuring the diameter of the coronary arteries and by quantifying the disappearance time of contrast medium (runoff time) on coronary angiography. Intravenous injection of dipyridamole increased the diameter of nondilated arteries by 7.9%. Its effect on the diameter of dilated coronary arteries (coronary aneurysm) was less than 3% (p<0.01). Runoff time of dilated coronary arteries was significantly (p<0.01) greater than that of nondilated coronary arteries. Dipyridamole accelerated runoff time not only in nondilated coronary arteries (p<0.01) but also in coronary arteries with various degrees of dilatation (p<0.01). We conclude that dipyridamole increases blood flow in coronary arteries without dilating the proximal aneurysm in children with a history of Kawasaki disease.

Assessment of left ventricular function in Kawasaki disease by dipyridamole-loading cineventriculography

Dipyridamole-loading cineventriculography was performed to evaluate left ventricular (LV) function in 76 patients with Kawasaki disease. Forty-five of 76 patients underwent the procedure within 6 months from the onset of illness (group 1A, normal findings on coronary angiogram; group 1B, maximal diameter of coronary lesions less than 4 mm; group 1C, maximal diameter of coronary lesions greater than 4 mm). Thirty-one of 76 patients underwent the procedure greater than 6 months after onset (group 2A, coronary lesions improved to normal or less than 4 mm; group 2B, remaining coronary lesions greater than 4 mm; group 2C, stenosis or obstruction of the coronary artery). Global LV ejection fraction increased in groups 1A, 1B and 2A after infusion of dipyridamole, but there was no significant change in groups 1C, 2B and 2C. In analyzing LV excursion, the pattern of LV wall motion with dipyridamole in groups 1B and 2A were similar to that in group 1A, but in 38% (5 of 13) of group 2A patients, abnormal regional LV wall motion after administration of dipyridamole was detected. Abnormal LV wall motion after dipyridamole infusion was detected in 42% (5 of 12) of group 1C patients, 38% (3 of 8 cases) of group 2B patients and 70% (7 of 10 cases) of group 2C patients. In conclusion, analysis of LV wall motion with dipyridamole is useful in evaluating cardiac involvement in Kawasaki disease.

Morphologic and functional assessment of coronary aneurysm after Kawasaki disease by repeated dipyridamole-loading coronary angiography

The long-term outcome of coronary aneurysm after Kawasaki disease was investigated using dipyridamole-loading angiography in 33 children with coronary aneurysms after Kawasaki disease. Morphologic regression may not accompany functional improvement in the dilated coronary arteries.