Follow-Up Method in Patients with Kawasaki Disease who had No Coronary Artery Abnormalities in the Convalescent Period / 소아과

PURPOSE: We performed the study to evaluate the value of the follow-up echocardiogram performed 6 months to 1 year after the onset of Kawasaki disease(KD), as recommended by American Heart Association(AHA) guidelines, when echocardiograms in the convalescent period were normal. METHODS: Patients were selected from 147 cases diagnosed with KD at Pusan Paik hospital from January 2000 to October 2003. A total of 45 KD patients belonged to AHA risk level I and II were performed follow-up echocardiography. The patient's medical records and echocardiogram were reviewed. Additionally, we sought the opinion of pediatric cardiologists on the subject by means of a multiple-choice survey. RESULTS: 37 children were belonged to AHA risk level I and the remaining 8 patients were belonged to risk level II. Of these 45 children, none were noted to have abnormalities on later follow-up echocardiogram. In the results of questionnaire, 37 percent of the participants advocate no follow-up after convalescent period for risk level I, and 33.3 percent favor periodic follow-up with echocardiography for risk level II up to one year. But there were no consensus about the diagnostic criteria of coronary abnormalities and how to follow-up these patients. CONCLUSION: All children with KD should have an echocardiogram at present and a follow-up study 6 to 8 weeks after the onset of fever. However, additional echocardiographies are not justified if the 6- to 8-week follow-up echocardiogram is normal. We would recommend that the more reasonable diagnostic criteria for coronary abnormalities and the Korean guidelines for long-term cardiovascular management and follow-up of KD need to be established.

Angiotensinogen M235T Polymorphism in Children with Henoch-Schonlein Purpura Nephritis

PURPOSE: Henoch-Schonlein purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angiotensinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. METHODS: The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. RESULTS: The M235T polymorphism of AGT gene frequency was MM:75%, MT:25%, TT:0% in HSP and MM:64%, MT:36%, TT:0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 mg/m2/day) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. CONCLUSION: We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

A Case of Repeated Ventricular Tachycardia Caused by Cardiac Rhabdomyomas in an Infant with Tuberous Sclerosis

Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained arrythmia in spite of intravenous amiodarone therapy. The pathologic examination confirmed the diagnosis of rhabdomyoma. The patient had no more arrythmia during the 14 month follow up period. Although cardiac rhabdomyomas may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrythmia.