A Case of Trisomy 9 Syndrome

Since Feingold and his collegues first described the trisomy 9 syndrome in 1973, approximately 30 patients with trisomy of the chromsome 9 have been described. Trisomy 9 has been reported as either partial or complete. Complete trisomy is rare and incompatible with a long life. Before this report, this syndrome has not been reported in Korea. A neonate was diagnosed trisomy 9 syndrome by clinical feature and chromosomal study. He had multiple anomalies such as broad-based nose, partially cleft lip, ambiguous genitalia, hyperconvex nails, overriding of fingers, and ventricular septal defect. The patient died at home on the 113th day of life.

Clinical Trial of 2g/kg/day of Intravenous Immunoglobulin Single Dose as Compared with 400mg/kg/day of Intravenous Immunoglobulin for 5days in Kawasaki Disease

PURPOSE: Since the effect of intravenous immunoglobulin (IVIG) therapy in Kawasaki disease was reported in 1984, the combined therapy of IVIG & oral aspirin has been popular. In early period, the protocol of 400mg/kg/day for 3-5 days of IVIG had been used, but rescently, the protocol of 2g/kg/day single dose has been preferred. So authers performed a clinical study to compare th efficacies & side effects between 400mg/ kg/day for 5 days & 2g/kg/day single dose of IVIG plus oral aspirin (100mg/kg/ day). METHODS: Seventy five patients who admitted to Eulji medical college hospital from January 1990 to July 1996 were evaluated retrospectively. Twenty nine patients (Group A) were treated with 400mg/kg/day for 5 days of IVIG plus aspirin (100mg/kg/day) and 46 patients (Group B) were treated with 2g/kg/day single dose of IVIG plus oral aspirin (100mg/kg/day). RESULTS: 1) The duration of fever after treatment was not significant difference between two groups (Group A, 25.5+/-30.3 days : Group B, 29.7+/-44.5 days P=0.7440) 2) The total admission days were significant difference between two groups (Group A, 9.9+/-0.4; Group B, 8.2+/-3.0, P=0.0308). 3) The incidence of side effects was not significant difference between two groups (Group A, 62%; Group B, 80%, P=0799). 4) The attack rate of coronary artery involvement was not significant difference between two groups (Group A, 34.5%; Group B, 26%, P=0.1198) 5) The case of re-treatment of IVIG due to relapse was not in Group A, but 5 in Group B (P=0.0661). 6) The mean platelet counts at admission were not difference between two groups (Group A, 434.4+/-17.2x103/mm3; Group B, 374.0+/-13.3x103/mm3, P=0.1449), but on the 7th hospital day, platelet counts were significant differnece between two groups (Group A, 531.9+/-16.5x103/mm3; Group B, 419.8+/-19.0x103/mm3, P=0.0066). 7) There was no significant difference in laboratory findings on admission and in the rate of coronary artery involvement between recurrent and non-recurrent cases of Group B. Conculusions : We conculuded that the protocol of 2mg/kg/day single dose of IVIG in Kawasaki disease may be have some benefits of shorter admission days and less coronary artery involvement, but the incidence of side effects and relapse rate might be less in the protocol of 400mg/kg/day of IVIG for 5 days.