BACKGROUND AND OBJECTIVES : Anti-endothelial cell antibodies (AECA) are found in the sera of many patients with Kawasaki disease (KD). In this study, the pathogenic role of AECA in the development of coronary arterial lesions of KD was investigated. SUBJECTS AND METHODS : Serum IgM-AECA concentrations were measured in 22 KD patients. Cultured human coronary artery endothelial cells (HCAEC) were incubated with either acute or convalescent phase sera, and their expressions of intercellular adhesion molecule-1 (ICAM-1) assessed. IgM fractions of the sera were purified, and their ability to induce ICAM-1 mRNA and protein expressions evaluated. To address the signal transduction pathways involved in IgM-AECA-induced ICAM-1 expression, the blocking effect of four protein kinase inhibitors, PD98059, SB203580, dimethylaminopurine (DMAP) and parthenolide were measured. RESULTS : IgM-AECA was present in 14 out of 22 (64%) acute KD sera. ICAM-1 expression of HCAEC incubated with acute KD sera (117.1+/-46.7) and AECA-positive acute KD sera (143.3+/-37.5) were significantly higher than those of the convalescent KD sera (88.9+/-14.4, p<0.05) or AECA-negative acute KD sera (71.2+/-11.8, p<0.05), respectively. IgM-AECA from KD patients significantly induced ICAM-1 protein and mRNA expression. The upregulation of ICAM-1 expression was significantly inhibited by SB203580, DMAP and parthenolide, but not by PD98059. CONCLUSION : IgM-AECA was detected in the sera of about 2/3 of acute KD patients, which activated endothelial cells by upregulation of ICAM-1 expression, possibly via p38, JNK MAPK and NF-kappaB signal transduction pathways. Thus, IgM-AECA may play a pathogenic role in the development of coronary arterial lesions in KD patients.
Antibodies ; Coronary Vessel Anomalies ; Coronary Vessels ; Endothelial Cells ; Humans ; Immunoglobulin M ; Intercellular Adhesion Molecule-1 ; Mucocutaneous Lymph Node Syndrome* ; NF-kappa B ; Protein Kinase Inhibitors ; RNA, Messenger ; Signal Transduction ; Up-Regulation

Two case of a rare from of congenital ichthyosis in infants born to the same parents are presented. Typical features revealed of parchment like skin, ectropion of upper eyelids, and fixed semiflexion attitude of the upper limb. The skin lesion were followed by desquamation and appearance of normal looking skin from the the second day of life to a month. Diagnosis of collodion baby was established by clinical features, histopathological and laboratory studies. A brief feview of literatures were presented.
Collodion* ; Diagnosis ; Ectropion ; Eyelids ; Humans ; Ichthyosis ; Infant ; Parents ; Skin ; Upper Extremity

OBJECTIVES: The purpose of this study was to evaluate the clinical usefulness of the protein/creatinine ratio of a spot urine specimen for early detection of proteinuria in the pregnancy induced hypertension and to suggest optimum cut-off value of that. STUDY DESIGN: A spot urine specimen and 24 hour urine collection for the proteinuria were ordered for 36 women admitted to obstetric unit for pregnancy induced hypertension and ROC curve analysis was performed to evaluate the usefulness of the protein/creatinine ratio of a spot urine specimen and to suggest optimum cut-off value. RESULT: The protein/creatinine ratio of spot urine positively correlated well with 24 hour urine proteinuria. (r=0.4322, p=0.0085) and the optimum cut-off value of the protein/creatinine ratio of a spot urine specimen to maximize the diagnostic accuracy was 5.0(Youden's index=0.66). CONCLUSION: We conclude that the protein / creatinine raio of a spot urine specimen may be a simple and inexpensive method for evaluation of proteinuria in the pregnancy induced hypertension when frequent determinations are necessary. This should improve clinical care, especially when managing hypertensive pregnant women as outpatients.
Creatinine* ; Female ; Humans ; Hypertension, Pregnancy-Induced* ; Pregnancy ; Pregnancy* ; Pregnant Women ; Proteinuria ; ROC Curve ; Urine Specimen Collection