Objectives: The aim of this study was the evaluation of glycometabolic function, lipid profile, and liver function in patients with β thalassemia major (TM) and their relationship with serum iron and ferritin.Methods: Fasting serum glucose, insulin, cholesterol, triglyceride (TG), and liver enzymes were evaluated for 78 homozygous TM patients and 122 normal control subjects. Serum iron, ferritin, and insulin resistance index (IRI) were determined for them.Results: Fasting serum glucose, TG, aspartate transaminase (AST) levels, alanine transaminase (ALT) levels, and IRI were significantly higher in the homozygous TM patients than in the controls (P<0.0001 for all). Serum cholesterol was significantly lower in patients (P=0.003), and 5.1% of cases had diabetes mellitus.Conclusions: Impaired glucose and lipid metabolism, and also liver dysfunction were observed in about 18%, 30%, and 20% of the patients, respectively. Normoinsulinemia and a high IRI suggest beta cell failure. Better evaluation of iron toxicity can protect patients from complications associated with treatment.
Neuroblastoma is the most common extracranial solid tumor of children, accounting for an estimated 15% cancer-related deaths in this period. It has been hypothesized that drug resistance of cancer stem cells may be responsible for chemotherapy failure, sustained tumor growth, and recurrence in many solid tumors. In this study, we investigated the expression of Octamer-binding transcription factor 4 (Oct4) and Nanog, two stem cell markers, in 47 neuroblastic tumors by immunohistochemistry and correlated their expression by other prognostic factors especially with NMYC amplification using both fluorescent and chromogenic in situ hybridization methods. Twenty three cases (48.9%) showed Oct4 signals and eight cases (17%) showed Nanog expression. All Nanog positive tumors showed Oct4 expression. Seven cases (14.1%) had NMYC amplification. There was also no association between positive Oct4 and Nanog reactivity and tumor morphology, age, mitosis-karyorrhexis index, NMYC amplification, favorable or unfavorable histology, and risk groups (p > 0.05). Cancer stem cells hypothesis is a challenging issue and controversies exist about their significance. Although our study did not show strong association between prognostic factors and expression of stem cell markers, performing of further large-scale studies of various neuroblastic tumors with various stages is suggested.
Administration of one dosage of DTP/Td vaccine during maintenance therapy and after cessation of therapy could be recommended, while one dosage of MMR vaccine at least three months after completion of therapy could be safe and beneficial.
Background: This study aimed at comparing the therapeutic effect of anti-D immunoglobulin (anti-D IG) and Intravenous Immunoglobulin (IVIG) in children with acute immune thrombocytopenic purpura (ITP). Methods: This Randomized double-blind, controlled clinical trial was conducted on 98 children attending a Children's medical center during year 2008. The selected participants were allocated to IVIG and anti-D IG groups using the balanced-block randomization method. The platelet count, hemoglobin level, and side effects of the medications were evaluated on days 1, 3, 7, 14, and 21 after drug injection. Results: After drug injection, the platelet count increased in both groups (P < 0.001) yet there was no difference between the 2 groups (P > 0.05). The hemoglobin level also decreased after injection in both groups (P < 0.001) and the decrease was similar in both groups (P > 0.05). Regarding the ability of the drugs to increase the platelet count, 56% of the patients in the anti-D IG group and 52% of the children in the IVIG groups had platelet counts of more than 20 000 during the 24 hours after drug administration with no difference between the 2 groups (P = 0.836). The incidence of drug adverse effects, including fever and chills (4.1% in the anti-D group versus 10.4% in the IVIG group), severe hemolysis (4.5% in the anti D group versus 0% in the IVIG group) and headache (6.25% in the anti-D group versus 4.1% in the IVIG group), had no significant difference between the two groups (P > 0.05). Conclusions: Intravenous Immunoglobulin and anti-D have similar effectiveness in the treatment of children with acute ITP. Considering the fewer side effects of anti-D, it may be a suitable replacement for IVIG in ITP patients.
This is the first study to analyse the SH2D1A gene mutations in Iranian paediatric patients diagnosed with lymphoma. Although we could not demonstrate such an association in our cohort of patients, larger, multi-centre studies are required to extend and confirm our early findings.
Acute lymphoblastic leukemia (ALL) is a systemic disease that is presented with different symptoms and signs. Cardiac manifestation is rare in ALL, but it is very important and needs appropriate management. It usually presents as leukemic myocardial infiltration and in the presence of cardiac mass comprehensive evaluation for other etiologies is mandatory. We reported on a 6-year-old boy in remission phase of ALL and large cardiac mass in the right atrium with obscure early symptoms and signs, in whom infective endocarditis (IE) was diagnosed and appropriate medical treatment was performed. Because the mass was sustained, surgical resection was considered for the patient.
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