Relationship between iron deficiency anemia and febrile convulsion in infants / 소아과

PURPOSE: The association between iron deficiency anemia and febrile convulsion in infants has been examined in several studies with conflicting results. Therefore, the authors aimed to evaluate the precise relationship involved. METHODS: In this case-control study, the authors assessed 100 children with a diagnosis of febrile convulsion, aged between 9 months and 2 years, during January 2007 to July 2009. The control group consisted of 100 febrile children without convulsion; controls were closely matched to the cases by age, gender, and underlying disease. RESULTS: The mean ages of the febrile convulsion and control group were 16.3+/-7.4 and 15.8+/-6.1 months, respectively, and the two groups had no differences in clinical features. Iron deficiency anemia (Hb <10.5 gm/dL) was more frequent in the febrile convulsion group than in the control group, although there was no statistical significance. Unexpectably, the RDW (red blood cell distribution width) was significantly lower and the MCNC (mean corpuscular hemoglobin concentration) was significantly higher among seizure cases than among the controls (P<0.05). There is no statistical difference between simple and complex febrile groups in the clinical and laboratory profiles. On multiple logistic regression analysis, iron deficiency anemia was more frequent, but the RDW was lower, among the cases with febrile convulsion, compared with the controls. Conclusions: Our study suggests that the iron deficiency anemia is associated with febrile convulsion, and screening for iron deficiency anemia should be considered in children with febrile convulsions.

Study on the Correlation between Clinical Classification according to Hemorrhagic Symptoms and Platelet Counts in Childhood Idiopathic Thrombocytopenic Purpura / 소아과

PURPOSE: Idiopathic thrombocytopenic purpura(ITP) is a relatively common hematologic disease in children. The optimal strategy for treating ITP during childhood is a matter of controversy. In general, platelet count has been the primary, if not the sole measure, on which treatment decisions have been made and outcomes determined. In this study, we tried to find out the correlation between clinical classification of Bolton-Maggs and platelet counts in childhood ITP on initial diagnosis METHODS: Seventy three patients with acute ITP in the Department of Pediatrics, Wonkwang University Hospital from June 1995 to December 2002, were enrolled. We retrospectively analyzed charts and classified our patients into four groups(no, mild, moderate, severe symptoms) according to Bolton-Maggs criteria. RESULTS: The ratio of female to male is 0.82. The peak age incidence was two to five years(41%). It was prevalent in the spring in 23 cases(31%) and winter season in 23 cases(31%). Most common clinical symptoms at presentation were petechiae and purpura in 55 cases(75.1%). According to Bolton-Maggs criteria, our patients, classified into no symptoms, mild symptoms, moderate symptoms and severe symptoms were 9 cases(12%), 22 cases(29%), 23 cases(30%) and 19 cases(25%), respectively. Among the 47 patients with platelet counts below 20,000/mm3, those with no symptoms, mild symptoms, moderate symptoms and severe symptoms were:1 cases(0.02%), 13 cases(27%), 17 cases (36%) and 16 cases(34%) respectively. We found that this classification was highly correlated with the platelet counts on initial diagnosis(P=0.005). CONCLUSION: There was a significant correlation between clinical classification of Bolton-Maggs and platelet counts. Therefore, this classification may be helpful in choosing the appropriate treatment options and evaluating the overall outcomes in childhood ITP.

Sensitization of TNF alpha and Agonistic FAS/CD95 Antibody-Induced Apoptosis by INF gamma on Neuroblastoma Cells

PURPOSE: IFN gamma sentitizes many tumor cells to TNF alpha and FASL-mediated apoptosis by enhancing the expression of TNF or FAS/CD95 receptor and modulating the activation of caspase and Bcl-2 family. It has been reported that IFN gamma and TNF alpha synergistically caused differentiation and growth inhibition of neuroblastoma cells. Even though some neuroblastoma cell express FASR/FASL on the cell surface, they could not induce apoptosis by ligation of the FAS/CD95 receptor. But the treatment of IFN gamma is reported to induce apoptosis in some neuroblastoma cell lines through the CD95/ CD95L autocrine circuit. In this study, we examined whether IFN gamma could affect TNF alpha and agonistic FAS/CD95 antibody(CH-11)-induced apoptosis against neuroblastoma cell lines that had shown diverse drug sensitivity and resistance. METHODS: CHLA-15, CHLA-90 and LA-N-2 neuroblastoma cells were cultured in IMDM and treated with recombinant IFN gamma TNF alpha and CH-11 antibody. Cell viability was measured by DIMSCAN with a fluorescent calcein-AM. Apoptosis was analyzed through flow cytometry using Annexin V- PE and 7-ADD staining and confirmed by pancaspase and caspase-8 blocking experiments. The expression of TNF RI and FAS/CD95 receptor was evaluated by flow cytometry using the corresponding antibody and PE-conjugated secondary antibody. RESULTS: IFN gamma or TNF alpha alone had no demonstrable cytotoxic effects, whereas both cytokines in combination induced apoptosis synergistically in CHLA-15 and CHLA-90 cells. Although there was no cytotoxicity with the ligation of CH-11 alone in CHLA-90 cells, pretreatment of IFNgammaincreased the sensitivity of CH-11-mediated apoptosis. The expression of TNFRI and FAS/CD95R were nonspecifically enhanced after treatment of IFN gamma without relation to sensitivity to TNF alpha and CH-11. This finding suggest up-regulation of both receptors may contribute to sensitization of TNFalphaand CH-11-mediated apoptosis by IFN gamma in only sensitive cell lines. CONCLUSION: IFN gamma induced sensitization of TNF alpha and agonistic FAS/CD95 antibody-mediated apoptosis on some neuroblastoma cells through up-regulation of TNFRI and FAS/CD95 receptor.

A Case of Late Infantile Neuronal Ceroid Lipofuscinosis that was Diagnosed by Characteristic EEG Findings / 대한간질학회지

Neuronal ceroid lipofuscinoses (NCL) are the most common childhood neurodegenerative disorders. Clinical features include seizures, blindness, psychomotor deterioration, the age of onset differ for each NCL type. Diagnosis of late infantile NCL relies on the characteristic clinical presentation, electrophysiological and neuroradiological findings, and identification of the ultrastructural abnormalities. The Photoparoxsmal response provide diagnostic clues to an atypical case of Infantile NCL in which results of extraneuronal biopsies were negative and MRI findings resembles leukodystrophy. Photic stimulation with 2 to 5 Hz activity elicited discrete spike and wave discharges in the occipital region on electroencephalogram and no sleep spindles are present. In patients with rapid neurologic deterioration, diagnosis of NCL should be considered and an EEG must be performed using photic stimulation to look for characteristic findings.