The Effectiveness of High Dose Dexamethasone for the Initial Treatment of Childhood Acute Idiopathic Thrombocytopenic Purpura / 대한혈액학회지

BACKGROUND: Corticosteroids and intravenous immunoglobulin (IV-Ig) have been used asfirst line treatments for acute idiopathic thrombocytopenic purpura (AITP) in children. High dose dexamethasone (HD) has been reported to be effective for chronic refractory ITP and for the initial treatment of AITP in adults. There has been no report about HD as the initial treatment for childhood AITP. We assessed the effectiveness of HD for the initial treatment of childhood AITP, as compared to IV-Ig. METHODS: 25 Patients with newly diagnosed AITP were enrolled. We conducted a prospective, randomized study to compare the two treatment options. 11 patients were treated with IV-Ig and 14 patients were treated with HD. The platelet counts were assessed at 3, 5, 7, 14 and 21 days after the beginning of the treatment. The adverse effects were noted, and the patients were followed for more than 6 months. RESULTS: Both the IV-Ig and HD groups showed a rapid rise of the platelet counts and the platelet counts were maintained at 3, 5, 7, 14 and 21 days. The difference of platelet counts between the two groups was significant at day 5 (P<0.05). During the follow-up period, 5 patients had a recurrence: 2 in IV-Ig group and 3 in HD group. All 5 patients were re-treated with HD and they had a good response. One of the recurred patients in the IV-Ig group had chronic ITP. Some side effects were observed, but they were not severe enough to necessitate the discontinuation of treatment. CONCLUSION: We conclude that HD is as effective and safe as high dose IV-Ig for the initial treatment of childhood AITP. The choice between these treatment options can be made according to the cost-effectiveness and the therapy-related risks.

A Clinical Study of Febrile Convulsions in Children Over 5 Year Olds without Organic Problems

PURPOSE: Febrile convulsions are the most common form of childhood seizures, appearing between in children 3 months and 5 years of age and are predominantly of benign nature. Febrile convulsions occurring after 5 years of age might be estimated as epilepsy. Most data for these disorders are from mainly epilepsy populations and might not be relevant to these children, and the requirement of any specific treatment or workup is not yet established either. Thus we tried to obtain a unique perspective. METHODS: Clinical data for episodes of febrile convulsions were collected in Eulji hospital in Deajeon. These were obtained from anonymised copies of a standardised admission note, emergency, nursing, and intensive care unit notes and interviews with parents, medical, nursing, and paramedic staff. RESULTS: Finding of 259 episodes of febrile seizure in over 5 year olds. 121(46.7%) were confirmed epilepsy or afebrile seizure in under 5 year olds, 13(5.0%) were caused by infection, metabolic disorder, leukemia and so on, and 23(8.9%) had previous neurological defect. Lastly, 102(39.4%) were included to febrile convulsion in over 5 year olds. 28(27.5%) of the subjects had their initial febrile convulsions in children over 5 year olds while 74(72.5%) of the subjects were under 5 years of age. The male to female ratio of the seizure group was 2:1. In the duration and type of convulsions, generalized convulsions occurred predominantly within 15mins in both of them. 5(17.9%) of group A and 16(21.6%) of group B showed electroencephalogram abnormality. The most common causes of seizure were common colds. Most of the convulsions occurred less than 24hrs after fever but, there were no abnormal findings in lumbar puncture and brain MRI. CONCLUSION: Febrile seizure in over 5 year olds without organic problems has similar characteristics to febrile convulsion in under 5 year olds. In light of these findings, lumbar puncture and brain MRI do not require a routine checkup for the evaluation of febrile seizure in over 5 year olds. However, encephalography needs to be examined.

Effect of renin inhibition on an experimental glomerulonephritis - a preliminary report / 소아과

PURPOSE: We performed this study in order to investigate the effect of direct renin inhibition on an experimental animal model with nephrotoxic serum nephritis and tried to give useful information for clinical research and renin inhibitor treatment. METHODS: Thirty BALB/c 6-week-old male mice were divided into 4 groups: control group (CO, n=5), control-treatment group with aliskiren (CT, n=5), disease group (DO, n=10), and disease treatment group with aliskiren (DT, n=10). Nephritis was induced by an intravenous injection of 0.25 mg/g weight of rabbit anti-GBM immunoglobulin G. Model 2002 Alzet mini-osmotic pumps (Durect Corp.) for aliskiren infusion were implanted into CT and DT. Each group strain was sacrificed serially one at a time on day 14. We estimated the protein-creatinine ratio in 12-hour-collected urine (UP/Cr) and measured the mesangial matrix score in the PAS-stained kidney of each strain. RESULTS: One strain at CT and DT died on day 6 and 7, respectively. Each group strain was sacrificed serially at a time on day 10 because DO were seriously ill. The UP/Cr of each group is as follows: CO, 31.24+/-6.54 mg/mg, CT, 23.38+/-13.60 mg/mg, DO, 112.72+/-10.97 mg/mg, DT 114.07+/-32.30 mg/mg. There was no significant difference between DO and DT. The mesangial matrix score of each group was CO, 0.23+/-0.10; CT, 0.13+/-0.03; DO, 1.90+/-0.48; and DT, 1.28+/-0.41, respectively, and there was a significant difference between DO and DT in the extent of mesangial matrix expansion (P=0.008). CONCLUSION: We found that renin inhibition was able to suppress the mesangial matrix expansion in experimental mice with acute nephritis, although there were no significant differences in UP/Cr.

Clinical factors causing hyponatremia in patients with mucocutaneous lymph node syndrome / 소아과

PURPOSE: Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. METHODS: Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose) within 10 days of fever onset from January 2006 to February 2008. These patients were divided into 2 groups. Group 1 consisted of 30 (26.3%) patients with hyponatremia, and group 2 consisted of 84 (73.6%) patients without hyponatremia. Clinical manifestations, laboratory results, and echocardiographic findings were compared between the groups. RESULTS: Group 1 patients were more likely to have a coronary artery lesion (53.3% versus 20.2%, P=0.005) and suffered from diarrhea (41.3% versus 14.1%, P=0.007). There was a higher incidence of cardiovascular involvement in group 1 patients, including coronary dilatation (46.6%), valvular regurgitation (13.3%), pericardial effusion (6.7%) and medium-sized aneurysm (6.7%). There were no coronary aneurysms in group 2 patients. Serum C-reactive protein (CRP) was significantly higher in patients with hyponatremia (12.2+/-7.79 mg/ dL versus 7.3+/-4.7 mg/dL, P=0.003) and IVIG-resistant patients were more common in group 1 (13.3% versus 3.6%). CONCLUSION: These results indicate that hyponatremia in KD occurs in patients exhibiting severe inflammation and was significantly associated with the development of coronary disease. Further studies will be necessary to confirm the pathogenic mechanisms of hyponatremia in KD patients.