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Background: Alpha-thalassemia is a common genetic disorder in Thailand and is caused by either deletion or non-deletional mutation of one or both α-globin genes. Inactivation of three α-globin genes causes Hb H disease and interaction of Hb H disease with heterozygous Hb E results in AEBart’s disease., Objective: The present study aimed to characterize the genotype of α-globin gene in 81 pediatric patients with Hb H and AEBart’s diseases in Phramongkutklao Hospital, a tertiary care center for thalassemic patients in central Thailand., Material and Method: Eighty one unrelated pediatric patients including 60 patients with Hb H disease and 21 patients with AEBart’s disease were enrolled in our study. Mutation analysis was performed by multiplex gap-PCR, multiplex-ARMS and direct DNA sequencing of both HBA1 and HBA2 genes, respectively., Results: A total of 81 pediatric patients with Hb H and AEBart’s diseases who mainly lived in central Thailand were included in the present study. Eight different α-thalassemia mutations interacting to produce seven genotypes of α-globin gene in both Hb H and AEBart’s diseases were identified. The number of patients in the non-deletional form was higher than in the deletional form for both Hb H (51.6% VS 48.4%) and AEBart’s diseases (52.4% VS 47.6%). The SEA deletion (--SEA) was the most common (98.8%) α-thalassemia 1 mutation. While 3.7-kb deletion (-α3.7) was the most common (90%) α-thalassemia 2 deletion, Hb CS was the most common (90%) non-deletional a-thalassemia 2. Uncommon non-deletional α-thalassemia 2 mutation identified in our study were Hb QS, Hb PS and initiation codon mutation, respectively., Conclusion: All of the α-thalassemia mutation in our pediatric patients with Hb H and AEBart’s diseases have been characterized by the combination of molecular techniques including multiplex gap-PCR, multiplex-ARMS and DNA sequencing of HBA1 and HBA2 genes.

More than 80% of acute lymphoblastic leukemia (ALL) in pediatric population is curable by using combinations of chemotherapy. However, 20% of the cases still suffer from disease relapse. The most common site of relapse is bone marrow. Relapse of childhood ALL involving the eyeball is rare. However, it occurs in 2.2% of relapsing children. The authors described a 10-year-old Thai boy with underlying ALL on therapy, presented with a one-month history of progressive visual loss of his right eye. The clinical and imaging studies strongly suggested the diagnosis of isolated ocular relapse. In this report, the authors presented the findings from successfully specific treatment consisting of systemic chemotherapy and radiation therapy on the affected eye. From other studies, the outcome was more favorable in cases of ocular relapse off therapy. In our study, one case of isolated ocular relapse ALL was reported.

Background: Children often require relief of pain and anxiety when undergoing painful procedures., Objective: To determine the differences by comparing fentanyl and ketamine used in cancer-diagnosed children undergoing painful procedures., Material and Method: A randomized, double-blinded, crossover trial was conducted with 55 children undergoing painful procedures (intrathecal chemotherapy and/or bone marrow aspiration/biopsy). Patients were randomly assigned in a double-blinded fashion to receive either intravenous fentanyl or ketamine at 1 mcg/kg/dose and 1 mg/kg/dose, respectively. The result in effectiveness of the drug was measured using three parameters, 1) satisfaction score ranging from 0 to 10, 2) perception of procedural pain using FLACC scale, Wong-Baker FACES Pain Rating Scale and Visual Analog Scale, and 3) the frequency of vomiting nausea score., Results: The satisfaction amongpatients receiving fentanyl was significantly greater than ketamine (p = 0.007). In addition, both painful and nausea/vomiting were significantly decreased in the patients receiving fentanyl (p = 0.002 and p < 0.001, respectively). No serious complications were observed, Conclusion: This study demonstrated that intravenous fentanyl generated a superior clinical effect in satisfaction, decreased pain and nausea/vomiting, and showed no significant side-effects over ketamine. Fentanyl may also be recommended as a reasonable option before undergoing oncology procedures in children with cancer.

Background: Leukemia is the most common malignancy in children. Multiple prognostic factors have been used in order to assist the clinician to decide appropriate risk-adjusted treatment for each patient; the current clinical outcomes of those patients have been significantly improved over the past decades., Objective: The purpose of this study was to examine survival outcome in children who were diagnosed with acute leukemia and treated in the Department of Pediatrics, Phramongkutklao Hospital during January 1, 2000 and July 31, 2013., Material and Method: The authors retrospectively reviewed the patients who were diagnosed with acute leukemia and treated at Phramongkutklao Hospital. Their clinical data were collected and analyzed based on clinicalfeatures inchluding age, initial WBC count at diagnosis, sex, immnunophenotype and cytogenetic abnormalities., Results: Total 152 patients with acute leukemia, 123 patients were diagnosed with acute lymnphoblastic leukemia (ALL) and 29 patients were diagnosed with acute myeloid leukemia (AML). The 5-year survival rates of ALL and AML patients were 72.63% and 30.30%, respectively. In addition, we found a correlation between the ALL patients' clinical outcomes and several prognostic factors including initial white blood cell count, CNS status at diagnosis and ploidy. However, there was no correlation between those factors and clinical outcomes in AML patients., Conclusion: Our treatment outcomes on patients with acute leukemia were similar to the reports from other countries. The several prognostic factors especially initial WBC at diagnosis can assist the clinician to select appropriate treatment option for each patient.

Background: The pain and its complication during sedation with ketamine remain a significant problem for children with hematologic malignancy., Objective: The purpose of the present study was to evaluate further the parental satisfaction for procedural sedation and analgesia during pediatric hematology/oncology procedures perfonned by pediatrician in the Department of Pediatrics, Phramongkutklao Hospital., Material and Method: The authors prospectively evaluated our experience using intravenous ketamine 1 mg/kg for oncology patients undergoing procedures at Department ofPediatrics, Phramongkutklao Hospital. The procedure was assessed by way ofaphysician-completed form and by evaluation of questionnaires given to parents to estimate levels of pain by using a 0 to 10 mm visual analog scale (VAS) at 2 hours after procedures and results in any adverse events with respect to age, gende, procedures performed, ketamine dosage and recovery time., Results: Total of46 children aged 6 months to 15 years with 46procedures were observed at pediatric unit post-procedure. The indicationsfor procedural sedation and analgesia included lumbar puncture and intrathecal chemotherapy (50%), bone marrow aspiration or biopsy (21.7%), and both plrocedures (28.3%). The median VAS scale during oncology procedures was 3, which were expressed by all the parents/guardians of the children treated. Adverse effects were observed in all children including nausea (30.4%), hypersalivation (26.1%), vomiting (21.7%), hallucinlation (4.2%). No child required admission to hospital and there were no serious complications., Conclusion: hntravenous ketamine 1 mg/kg is effective for invasive procedures in children with malignancy. The use of intravenous ketamine may produce psychedelic effects in children. These adverse effects may alter the child's comfort and parental satisfaction especially in the young children.

Background: Though attention deficit, hyperactivity disorder ADHD is a common problem in childhood. Thai teachers' knowledge regarding the disease has never been assessed., Objective: To identify knowledge of Thai teachers regardingADHD and its influencingfactors., Material and Method: Cross-sectional study was operated in three primary schools in Ayutthaya, Thailand. Standardized questionnaires comprised ofdemographic data, ADHD experiences and the Knowledge of Attention Deficit Disorder Scale, KADDS, were distributed to participating teachers. Results were reported using frequency, percent, mean, and standard deviation. Association between demographic and ADHD experiences and the KADDS score was identified by logistic regression analysis., Results: Lack ofknowledge of ADHD among teachers was apparent. Only 19.4% of them passed the total scale of KADDS. Teachers under 31-years-old were more likely to pass general information and signs, symptoms & diagnosis subscales and total scale. In addition, familiarity with ADHD patients was associated with passing scores of general information subscale and total scale., Conclusion: Despite public awareness of ADHD, Thai teachers lacked knowledge concerning the disease. Young teachers were more acquainted with ADHD. Direct experience with ADHD patient might help teachers develop their knowledge on ADHD.

Kasabach-Merrit phenomenon (KMP) is a rare disorder of pediatric hematological malignancies which is previously referred to Kasabach-Merrit syndrome (KMS). The disorder is characterized by prominent vascular mass resulting from abnormal proliferation of blood vessels, consumption coagulopathy and thrombocytopenia. The diagnosis is based upon three basic findings as above. The authors describe a 6-month-old girl with a huge ecchymotic mass at left buttock who is found to have thrombocytopenia and consumption coagulopathy. The clinical and imaging studies strongly suggested the diagnosis of KMP. Vincristine was administered after a trial of corticosteroids was failed to show clinical and laboratory improvement. After 2 weeks, the patient showed that the platelet count and fibrinogen level become to be normal without blood transfusion and gradually decreased in tumor size without any surgical procedure in 4 weeks and disappeared in 5 months without any complications. In the present report, the authors present the findings from successfully specific treatment with vincristine alone and supportive treatment for life-threatening hemorrhage with platelet concentrate and cryoprecipitate should be the best management in our situation.