Statistical Analysis of 1,000 Cases of Kawasaki Disease Patients Diagnosed at a Single Institute / 소아과

PURPOSE: To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. METHODS: We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed and non-relapsed groups, and as to the relapsed group, we also compared variables between patients in their first and second attack states. Finally, factors associated with longer-fever duration from disease onset were evaluated. RESULTS: Longer fever durations before and after IVIG treatment, male sex, lower Hgb and Hct level, higher WBC count and segmented WBC proportion, and higher CRP and Harada's score were related with coronary artery lesions. Non-responsiveness was related to higher WBC count, segmented WBC proportion, CRP, SGPT, Harada's score, and pyuria. Moderate-to-severe coronary artery dilatations and recurrences were more commonly seen among the non-responder group. No significant predictive factors for recurrence were found. In the relapsed group, lower WBC count, CRP, and shorter fever duration from disease onset were observed in their second attack state. Fever duration from disease onset showed positive correlation with WBC count, CRP, and Harada's score and negative correlation with Hgb levels. CONCLUSION: Higher WBC count, CRP, and higher Harada's score were related to both higher incidences of coronary artery lesions and non-responsiveness to IVIG treatment, and these factors were also related with longer fever duration. Non-responders to IVIG treatment showed higher recurrence rate and more moderate-to-severe coronary artery dilatations than responders.

Expression of Toll-like Receptor-2 on the Peripheral Blood Monocytes in Kawasaki Disease Patients / 소아과

OBJECTIVE: Toll like receptor(TLR) is known to be involved in innate immunity. Many microbial antigens stimulate TLR, and as a result of intracellular signal transduction, they activate nuclear factor-kB which produces diverse inflammtory cytokines. Until now, many research topics in Kawasaki disease focused on cytokine increasement. In this study, we aim to reveal TLR increasement which might be associated with initiation of inflammatory response. METHODS: We obtained the peripheral blood of ten patients who were diagnosed with Kawasaki disease in Yonsei University College of Medicine from March 2003 to August 2003, as well as those of a febrile control group and the same number of a normal control group. Flow cytometry was done in all samples for quantification of TLR-2 expression in CD14 positive monocyte. And we also extracted total RNA of periphral monocyte and quantificated expression of TLR-2 mRNA by RT- PCR. RESULTS: The expression of TLR-2 in Kawasaki disease increased significantly compared with the normal control group but not when compared with the febrile control group. And the expression decreased slightly in the subacute phase of Kawasaki disease compared with the acute phase, but this was statistically insignificant. mRNA expression of TLR-2 in peripheral blood monocyte also increased in the acute phase of Kawasaki disease. CONCLUSION: Expression of TLR-2 in Kawasaki disease increased when compared with the normal control group, which means that innate immunity is associated with the pathogenesis of Kawasaki disease.

Effect of a Flavon Extracted from Artemisia absinthium on Collagen Induced Arthritis in Mice / 소아과

PURPOSE: In this study, a possible suppressive effect of a flavon extracted from Artemisia absinthium on a mouse collagen-induced arthritis (CIA) model was investigated. METHODS: DBA/1 mice were injected intradermally with emulsified chicken type II collagen. Three weeks after immunization, a flavon was introduced p.o. everyday. Clinical incidences of arthritis and arthritis index were measured. Measurement of anti-collagen antibodies and a stimulation index of the splenocytes of the mice were measured. IL-10 and TNF-alpha in the supernatants of the mice sera were measured by ELISA. mRNA expression for IL-10 and TNF-alpha in the splenocytes were tested. RESULTS: Flavon extracted from Artemisia absinthium appears to be an effective suppressor of CIA in mice. The serum anti-collagen antibody level and stimulation index of the cultured splenocytes showed no significant differences among the three experimental groups. Also serum IL-10 and TNF-alpha levels did not show any significant differences among the three experimental groups. An increased expression of mRNA for IL-10 was observed in the splenocytes treated with flavon. CONCLUSION: With these results, flavon extracted from Artemisia absinthium appears to have a suppressive effect of CIA. The mechanism of the suppressive effect of flavon extracted from Artemisia absinthium may be from a stimulation of IL-10 production.

A Case of Primary Intestinal Lymphangiectasia / 대한소아소화기영양학회지

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

Clinical Manifestations of Ureteral Duplication in Children

PURPOSE: Ureteral duplication is a common urologic anomaly and early diagnosis may prevent irreversible renal damage. We thus aimed to evaluate the benefits of prenatal sonography in early detection. METHODS: We retrospectively studied 55 children with duplicated systems who were admitted to Severance Hospital, Yonsei University, Seoul, Korea from January 1998 to July 2003. RESULTS: The mean age at diagnosis was 2.3 years old. 89% of the children had complete duplicated ureter. 47% of the children visited the hospital with an initial complaint of abnormal prenatal sonographic findings. Among these patients, 70.8% had DMSA defects. The mean differential renal function(DRF) in the DMSA scan was 48.2% in the group diagnosed prenatally. In the postnatally-diagnosed group, DMSA defects were found in 67.7% patients and the mean DRF was 45.5%. CONCLUSION: In comparison with the past studies, the mean age at diagnosis is becoming younger, and the proportion of abnormal prenatal sonography as an initial complaint larger. Other clinical manifestations were similar. The difference of the renal damage between the prenatally diagnosed group and the postnatally diagnosed group was not statistically significant. A multi-center study may help to prove the importance of prenatal sonography in early diagnosis and treatment of ureteral duplication.

Clinical Manifestations of Ureteral Duplication in Children

PURPOSE: Ureteral duplication is a common urologic anomaly and early diagnosis may prevent irreversible renal damage. We thus aimed to evaluate the benefits of prenatal sonography in early detection. METHODS: We retrospectively studied 55 children with duplicated systems who were admitted to Severance Hospital, Yonsei University, Seoul, Korea from January 1998 to July 2003. RESULTS: The mean age at diagnosis was 2.3 years old. 89% of the children had complete duplicated ureter. 47% of the children visited the hospital with an initial complaint of abnormal prenatal sonographic findings. Among these patients, 70.8% had DMSA defects. The mean differential renal function(DRF) in the DMSA scan was 48.2% in the group diagnosed prenatally. In the postnatally-diagnosed group, DMSA defects were found in 67.7% patients and the mean DRF was 45.5%. CONCLUSION: In comparison with the past studies, the mean age at diagnosis is becoming younger, and the proportion of abnormal prenatal sonography as an initial complaint larger. Other clinical manifestations were similar. The difference of the renal damage between the prenatally diagnosed group and the postnatally diagnosed group was not statistically significant. A multi-center study may help to prove the importance of prenatal sonography in early diagnosis and treatment of ureteral duplication.