Masaki Matsushima

High-dose gammaglobulin therapy for Kawasaki disease

To evaluate the effectiveness of gammaglobulin in decreasing the incidence of coronary artery lesions in Kawasaki disease, a randomized controlled study in 136 patients was conducted using high doses of gammaglobulin 400 mg/kg/d for 3 days plus aspirin 30 mg/kg/d (gammaglobulin group) and aspirin alone at the same dosage (aspirin group). The total febrile period and the duration of fever after treatment were significantly shorter in the gammaglobulin group than in the aspirin group (P less than 0.001). The incidence of coronary artery lesions and of coronary artery aneurysms was significantly lower in the gammaglobulin group than in the aspirin group up to 30 days after the onset of Kawasaki disease (P less than 0.01 and P less than 0.05, respectively). In 16 of 69 patients given gammaglobulin, fever persisted for longer than 3 days, and there was a higher incidence of coronary artery lesions among them. The effectiveness of high doses of gammaglobulin in preventing coronary artery lesions has been demonstrated, but the indications and the optimal dose of gammaglobulin remain to be determined.

Cardiac arrhythmias in healthy children revealed by 24-hour ambulatory ECG monitoring

SummaryAmbulatory electrocardiographic monitoring was performed on 360 healthy children, from newborn infants to junior high school students. They were divided into five groups by age: group A, 63 newborn infants on the first day of life; group B, 50 infants aged 1–11 months; group C, 53 kindergarten pupils aged 4–6 years; group D, 97 primary school pupils aged 9–12 years; and group E, 97 junior high school students aged 13–15 years.The maximal and minimal heart rates were significantly greater in infants than in older children. Sinus arrhythmia was recorded in every child. One boy in group E had an episode of sinus arrest for three seconds without any symptoms. First-degree and Wenckebach type second-degree atrioventricular blocks were not detected in group A and group B, but were most frequent in group E, especially during sleep. Supraventricular premature contractions (SVPCs) were the most common type of arrhythmia detected in this study. More than half of the children had at least one SVPC per 24-h monitoring period, and there were many children with frequent SVPCs in group E. The incidence of ventricular premature contractions (VPCs) in children of groups A and E was rather higher than in the other groups. Ventricular tachycardia was not recorded in any child except one newborn infant who had a couplet of VPCs without symptoms.Each group had different types and incidences of arrhythmias. There was a rising incidence of arrhythmias with advancing age, except in the neonatal period.

Advantages of Oxygen Inhalation Therapy for Postoperative Pulmonary Hypertension

Total correction was performed in a case of complete transposition of the great arteries (TGA) with severe pulmonary vascular obstructive disease (PVOD). Although severe pulmonary hypertension remained after surgery, oxygenation was continued for 15 months, which included a shift to at-home oxygen inhalation therapy (HOT). Cardiac catheterization 15 months after surgery demonstrated that pulmonary hypertension was greatly improved. For patients in whom the palliative Mustard operation is considered due to severe PVOD on the basis of lung biopsy diagnosis, total correction of TGA is possible by employing HOT after surgery.

Clinical features of adult patients with Eisenmenger's syndrome in Japan and Korea

BACKGROUND There are few articles on mortality and morbidity of adult patients with Eisenmengers syndrome (ES) in the current era when disease targeting therapy (DTT) has been available. METHODS AND RESULTS 198 patients (a median age 35 years, 64% female) with ES who visited the 16 participating institutes in Japan and Korea from 1998 to 2009 were enrolled. Clinical data during adulthood were collected from each institutional chart and analyzed centrally. During a median follow-up of 8 years, 30 patients died including 14 sudden deaths. 89 patients took oral medication of DTT and clinical improvement was observed in 54 of them. However, survival rate in patients taking DTT was not different from those without (87% vs 84%, p=0.55). When the clinical data in between first and last clinic visits were compared in 85 patients, the patients with NYHA >/=III increased from 24% to 48% (p<0.001), SpO2 decreased from 89% to 85% (p=0.008) and hematocrit increased from 51.4% to 52.9% (p=0.04). Non-survivors had poorer NYHA function class, lower body weight (BW), lower body mass index (BMI), and higher serum level of Cr at the first visits than survivors. CONCLUSIONS Long term survival and clinical status of adult patients with ES remains unsatisfactory even in the current era of DTT. Poor NYHA functional class, low BW, low BMI and high serum level of Cr were related to mortality. DTT therapy improved clinical status in many patients with Eisenmengers syndrome, but no significant impact on survival could be shown.

Low humoral responses to hepatitis C virus among pediatric renal transplant recipients.

Immunosuppressed patients infected with hepatitis C virus (HCV) are known to have a low prevalence of antibodies to the virus. We examined 12 HCV‐infected renal transplant recipients and 18 immunocompetent patients in terms of viral markers. The renal patients had a lower prevalence of anti‐C100‐3 antibodies and lower titers of anti‐p22 than the immunocompetents. Low titers of the antibodies seemed to be characteristic of HCV, because titers of anti‐mumps and anti‐cytomegalovirus antibodies were the same or even increased. Quantitation of HCV RNA showed approximately 200‐fold more copies of mean HCV RNA in the sera of the kidney patients than in the immunocompetents. This indicates that the low humoral responsiveness to HCV is not due to the lower concentration of the virus. Immunosuppressive conditions seemed to suppress anti‐HCV production with greater magnitude than other viral antibodies.

Long-term Outcome of Coronary Abnormalities in Kawasaki Disease

OBJECT To analyze long-term outcome of coronary abnormalities in patients with Kawasaki disease (KD). SUBJECTS The subjects of this study are 46 patients who underwent coronary angiography (CAG) twice or more times in our hospital for 20 years since 1981. The age of the first CAG was 5.2±3.3 years. Sixteen patients were referred from the affiliated hospitals. RESULTS The dilated lesions were revealed on the first CAG in 34 patients; large-sized aneurysms (ANl) in 8 patients, moderate-sized aneurysms (ANm) in 12 and dilation lesions (Dil) in 14, while the stenotic lesions were found in 11 patients; occlusion (OC) in 2 patients, segmental stenosis (SS) in 4 and local stenosis(LS) in 5. Coronary abnormalities improved in 7 patients, and were aggravated in 18. The smaller dilated lesions had a tendency to improve during follow-up period, compared with moderate-sized aneurysms on the initial CAG. Four among 14 the dilated lesions with diameter of 4-5mm were already aggravated on the second CAGs. ANl changed to OC and LS at the high rate(63%), while LS has a trend to be aggravated to further stenosis as well as obstruction(80%). Most of SS could be found 1 to 3 years after the onset of KD(75%), while OC and LS could be found in any time. Some of them were found over 10 years after the onset. During the follow-up period, one patient died due to ischemic heart disease. Four patients underwent bypass-grafting, and one had PTCA with a good result. Two patients are waiting for interventional treatment. Most of ANl appeared in acute phase. However, distal Dil in a patient among the subjects changed to LS 4 years later. CONCLUSION Even though coronary dilatation was revealed on the initial CAG and no treatment was required, the patients should be followed up carefully for a long time.