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Cytosine arabinoside (ARA-C) is a pyrimidine analog that may cause keratoconjunctivitis when used in high doses. The underlying mechanism may be the increased amounts of reactive oxygen radicals that may damage the DNA synthesis of corneal and conjunctival epithelial cells. Topical corticosteroids are one of the prophylactic treatments for keratoconjunctivitis induced by ARA-C. Forty Wistar-type albino rats were included in this study the rats were divided into four groups. The first group (Group 1) received only ARA-C, the second group (Group 2) received ARA-C and N-acetylcysteine (NAC), the third group (Group 3) received only NAC and the fourth group (Group 4) was the control group. The total oxidant status (TOS), the total antioxidant capacity and the oxidative stress index (OSI) measurements of the cornea and the conjunctiva were evaluated in these four groups. The mean TOS and OSI value was the highest in Group 1 and the lowest in Group 3. The differences in TOS and OSI values were statistically significant between Group 1 and Group 2. There are decreases in TOS and OSI values in rats which received ARA-C with NAC administration. NAC may have a protective effect on ARA-C-induced keratoconjunctivitis.

Adenovirus is an infectious viral agent that causes variety of clinical presentations such as respiratory disease, conjunctivitis, and gastroenteritis. Hepatitis, pancreatitis, myocarditis, encephalitis, and disseminated infection are primarily seen in immunocompromised patients. Rarely, adenovirus infection can present with pertussis-like syndrome. Described here is case of pertussis-like syndrome associated with adenovirus presenting with hyperleukocytosis., Competing Interests: Conflicts of Interest: None declared.

Background: Reticulocytes are the youngest erythrocytes released from the bone marrow into the blood and they circulate for 1-2 days before becoming mature erythrocytes. In literature, there were studies about reticulocyte parameters that could help in differentiation of iron deficiency anemia (IDA) from vitamin B12 deficiency anemia. However, in those studies there were no data about differentiation of mixed anemia (vitamin B12 deficiency and IDA). The purpose of this study is to explore a response to 'could reticulocyte parameters help in differential diagnosis of mixed anemia?' in 6-12 years old children., Methods: The study enrolled 26 patients with IDA, 22 patients with mixed anemia, 32 patients with vitamin B12 deficiency, and 32 age and gender matched healthy controls. Blood for hematological parameters such as complete blood count, reticulocyte count, CHr, MCVr, CHCMr were collected into standard tubes containing EDTA., Results: There is a statistically significant difference of both MCV (mean corpuscular volume)/MCVr (Reticulocyte mean corpuscular volume) ratio and MCVr between IDA and controls; in controls and vitamin B12 deficiency anemia; in controls and mixed anemia; in IDA and vitamin B12 deficiency anemia; in IDA and mixed anemia. Also in terms of both CHr and CHCMr (Corpuscular mean hemoglobin concentration of reticulocyte), there is a statistically significant difference between controls and IDA; controls and mixed anemia; IDA and mixed anemia; IDA and vitamin B12 deficiency anemia., Conclusions: In mixed anemia, MCV could be normal or decreased, and in peripheral blood smear erythrocytes cells could be morphologically normal. For this reason diagnosis of mixed anemia is not easy and needs additional laboratory investigations. Our results suggest that in a differential diagnosis of mixed anemia from vitamin B12 deficiency, IDA, and healthy controls, CHr, CHCMr, and MCVr (together with MCV and individually) could be useful. So, with a simple and cheap laboratory parameter, differentiation of mixed anemia could be done.

Roberts syndrome, which is inherited as an autosomal recessive group of disorders, is a rare syndrome characterized with symmetrical extremity defects, craniofacial abnormalities, and prenatal and postnatal growth retardation. Here, we present a case of Roberts Syndrome brought to the clinic with diarrhoea and multiple abnormalities, that had tetra phocomelia, growth and developmental retardation, abnormality of complete cleft lip-palate accompanied with Aortic stenosis and PDA, and in which cytogenetic analysis identified premature centromere separation. Mutation analysis of ESCO2 revealed a splice site mutation [c.1131+1G>A] in intron 6 in homozygous status in the patient and heterozygous status in the parents. Our case is the first Robert- Syndrome with valvular aortic stenosis in the literature, to the best of our knowledge.

Unlabelled: The current study was conducted to assess 3435C>T multidrug resistance 1 gene polymorphism and the efficacy of high dose methylprednisolone (HDMP) in childhood acute idiopathic thrombocytopenic purpura patients., Methods: A total of 31 childhood acute Idiopathic thrombocytopenic purpura patients (17 females, 14 males) between the ages of 2 and 16 years of age were included in the study. High-dose methylprednisolone was given at a dose of 30mg/kg/day for 3 days and 20mg/kg/day for 4 days, consecutively and intravenously. Polymerase chain reaction-restriction fragment length polymorphism was used for the detection of C3435T single nucleotide polymorphism. Fragments obtained were 238bp to T/T genotype, 172bp and 60bp fragments to the C/C genotype, and 238bp, 172bp and 60bp to the C/T genotype., Results: The distribution of CC, CT, and TT genotypes were 19.0%, 61.3%, and 19.4%, respectively. Both allele frequencies of C and T were the same - 50%. There was no significant difference in genotype and allele distribution between the patients with ITP and the control group (χ(2)=0.84 p=0.65, χ(2)=0.2 p=0.63, respectively). There were no significant differences in age, gender, and pre- and post-treatment platelet counts between CC, CT, and TT genotypes of the MDR gene. Response to treatment shows no significant difference between genotype and allele groups., Conclusion: In our study, there was no difference in the HDMP treatment response between MDR1 gene genotypes. However, it should be noted that this study includes a small group of patients. Our data should therefore be considered preliminary, awaiting further confirmatory studies on an expanded patient base., (Copyright © 2011 Elsevier B.V. All rights reserved.)

In this retrospective study, cyclosporine levels at the second hour (C2 levels) were measured during oral cyclosporine intake in 28 pediatric hematopoietic stem cell transplant patients, and the relations between cyclosporine dosage and C0, C2 levels, C2/C0 ratio, and cyclosporine-related adverse effects were investigated. Cyclosporine levels at the second hour levels were found to be significantly lower in children younger than 7 years old, suggesting age-related differences in absorption and metabolism of the drug. There were statistically significant correlations of both C0 and C2 levels with blood creatinine values. In addition, a statistically significant negative relation was found between C0 and C2 levels and serum potassium levels; this unexpected finding was attributed to multiple drug effects in the early posttransplant period. The common adverse effects of cyclosporine (gingival overgrowth, gynecomastia, and hypertrichosis) were also evaluated in this study, and no correlation was found between those adverse effects and C0, C2 levels, C2/C0 ratio, and cyclosporine dosage. In the present study, despite the highly significant correlation of C2 levels with renal and metabolic effects, in pediatric hematopoietic stem cell transplant patients, measurement of C2 levels as a standard practice did not provide an advantage over C0 monitoring. However, the preliminary results suggest that C2 level monitoring could be useful in selected patients with increased risk of renal toxicity or in states where a better estimation of gastrointestinal absorption is needed.

Cardiac complications are the leading cause of death in β-thalassemia major (TM) patients. The aim of this study was to investigate the impact of iron overload on ventricular functions using conventional and tissue Doppler imaging (TDI) in patients with TM and compare them with children with thalassemia trait (TT) and healthy controls. This prospective study includes 3 groups: group 1: 29 patients with β-TM; group 2: 28 patients with TT; group 3: 29 healthy controls. Peak late relaxation velocity determined by conventional echocardiography for the right ventricle was significantly higher and the E/A ratio for the right ventricle and left ventricle were significantly lower in TM patients than the other groups (P < .05). Peak late relaxation velocity determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly higher in TM patients than the TT subjects and controls (P < .001). The E/A ratio determined by TDI for the left ventricle, interventricular septum, and right ventricle were significantly lower in group 1 than the other 2 groups (P < .001). There was a negative correlation between the ferritin level and E/A ratio for the left ventricle, interventricular septum, and right ventricle using TDI (P < .05). Conventional echocardiographic techniques have failed to distinguish ventricular functions of asymptomatic patients with TM from the subjects with TT and from normal controls when global functions were examined. The present study indicates that TDI should be used for screening of TM and TT subjects' cardiac functions.

Glanzmann thrombasthenia (GT) is a rare inherited qualitative platelet disorder due to the deficiency or defect of platelet membrane glycoprotein (GP) IIb/IIIa complex. Symptoms include purpura, petechiae, bruising, gingival bleeding, epistaxis, and menorrhagia. Platelet transfusion is considered the standard therapy for securing hemostasis in patients with GT when local measures and antifibrinolytic agents are inadequate. However, repeated platelet transfusions may result in GP IIb/IIIa and/or human leukocyte antigen (HLA) immunization and development of platelet refractoriness. Recombinant factor VIIa (rFVIIa) has been introduced as therapeutic alternative and has been suggested to be effective. Recombinant factor VIIa is indicated in Europe for the treatment of GT refractory of platelet transfusion. In previous studies, rFVIIa has been used in the prophylactic treatment of bleeding in patients with GT undergoing pelvic surgery, cesarean section, and vaginal delivery. In this article, we present a case of intensive menstrual bleeding refractory to previous antifibrinolytic agents and platelet transfusions but which responded well to treatment with rFVIIa. To our knowledge, there is no study or reported case in the literature reporting successful use of rFVIIa in a patient with excessive menstrual bleeding due to GT.

In this study, the authors aimed to evaluate the frequency of pulmonary hypertension (PHT) in asymptomatic thalassemia major (TM) patients, and to investigate the impact of pulmonary function test (PFT) and CO diffusion results on the evaluation of pulmonary hypertension. Data from 50 asymptomatic patients with TM over age 10 were evaluated. Pulmonary hypertension was diagnosed in 10 patients (20%). High tricuspid regurgitant jet velocity was found in 14% of adults and in 25% of children. Pulmonary function test including CO diffusion testing results were not different between the TM patients with or without pulmonary hypertension. In conclusion, PHT was frequent among TM patients even they were asymptomatic and although PFT results has shown lack of association, it should be investigated in larger population.

Fludarabine, cytarabine, granulocyte colony-stimulating factor (G-CSF), and idarubicin (FLAG-IDA) regimen has been proven to be a potentially useful chemotherapy regimen for relapsed or poor-prognosis childhood leukemia. The aim of the study was to evaluate complete remission (CR) rate, toxicity, and overall survival of children with poor-prognosis acute leukemia who received the FLAG-IDA regimen. Furthermore, the authors investigated the children who achieved CR following FLAG-IDA treatment regarding their eligibility for allogeneic hematopoietic stem cell transplantation (HSCT). Between January 2002 and April 2007, 25 children with poor-prognosis acute leukemia were treated with FLAG-IDA regimen in our center. Of the 25 children (16 AML, 9 ALL) with poor-prognosis acute leukemia, 7 (28.0%) received 1 cycle, 17 (68.0%) received 2 cycles, and 1 (4%) received 3 cycles of FLAG or FLAG-IDA regimen. After 44 cycles of FLAG-IDA or FLAG regimen, 10/25 (40%) children were nonresponders, 15/25 (60.0%) showed CR. Five (20%) of these patients in CR who underwent allogeneic HSCT are still in remission. The remaining 20 (80.0%) children were lost due to infection or relapse of the primary diseases. The overall survival of patients who are still alive and underwent allogeneic HSCT (mean: 40.6 ± 4.7, median: 40, range: 34-46 months) was longer than that of patients (mean: 5.5 ± 4.3, median: 4, range: 1-15 months) who did not undergo allogeneic HSCT. The CR rate was quite high in the present study using the FLAG-IDA regimen, and the authors believe this regimen is a possible option prior to allogeneic HSCT in children with poor-prognosis acute leukemia.

Of the 488 pediatric patients with thrombosis in a single center, 50 (10.2%) had nonstroke arterial thrombosis. Half of the nonstroke arterial thrombosis patients had a preceding arterial catheterization history. Infection was a common contributing underlying disorder among both catheterized and overall groups (28 and 42%, respectively). The third most common underlying risk factor for development of arterial thrombosis was associated cardiac disease, and 70% of these patients had congenital heart disease and 30% had dilated cardiomyopathy. The most common site of arterial thrombosis was the heart, and 22% of the patients developed arterial thrombosis following cardiac surgery for congenital heart diseases. The prothrombotic risk factors were also analyzed in the study group and factor V G1691A and PT G20210A heterozygous mutations were detected to be higher (16 and 4%, respectively) than those in healthy controls (7.4 and 2.3%, respectively). In addition to factor V G1691A and PT G20210A mutations, other prothrombotic risk factors including protein C and protein S deficiencies and elevated factor VIII levels were commonly associated with pediatric nonstroke arterial thrombosis cases in the present study. This finding indicates the importance of these factors in arterial thrombosis development, similar to venous thrombosis. Another striking factor of the study is the higher number of prothrombotic risk factors (more than three) in patients who had persistent thrombosis, indicating the need for more intensive treatment in these patients.

WAS is a rare X-linked recessive disorder characterized by primary progressive T cell immunodeficiency, impaired antipolysaccharide antibody response, thrombocytopenia with small platelet, and eczematoid dermatitis. Untreated patients with typical WAS have poor prognosis with the major causes of death being infection, bleeding, lymphoproliferative disorders, and malignancy. Due to the increased risk of infectious and hemorrhagic episodes the best results with HSCT are achieved in patients less than five yr of age and are recommended as early as possible. Here, we report a three-yr-old boy with WAS who underwent UCB and BMT from his genotypically identical brother with Klinefelter syndrome.

Hematopoetic stem cell transplantation, even from an HLA 6/6 identical family member is associated with an increased frequency of complication in fanconi anemia (FA). The increased susceptibility for chromosomal breaks has been suggested as a contributory factor for increased risk of toxicity, graft versus host disease (GVHD) and increased incidence of post-transplant solid tumors. Therefore, non-irradiation based preparative regimens usually containing fludarabine and T-cell depletion of HLA geno-identical bone marrow cells have increasingly been used in patients with FA. Here, we report three children with FA who underwent CD-34 selected HSCT from HLA-identical family donors with reduced intensity fludarabine-based regimen., ((c) 2008 Wiley-Liss, Inc.)

The authors report on a 4-year-old child with the diagnosis of tetralogy of Fallot (TOF) and infective endocarditis. Methicillin-resistant Staphylococcus aureus (MRSA) was isolated from the blood culture of the patient. While receiving imipenem, amikacin, and linezolid therapies, the boy's general condition improved, acute phase reactants decreased, and his blood culture became negative for MRSA. On his follow-up echocardiography, the vegetation had also disappeared. However, he developed progressive bicytopenia following linezolid therapy for 5 weeks. During linezolid therapy, his hemoglobin level decreased from 12.1 to 5.3 g/dL and his platelet count from 242 x 10(9) to 14 x 10(9)/L. His white blood cell count (WBC) did not decrease during linezolid therapy. Six days following termination of linezolid therapy, his hemoglobin had increased to 8.2 g/dL and platelet count to 192 x 10(9)/L. Thus, it should be kept in mind that linezolid may induce cytopenias in children. If these side effects of linezolid are known, unnecessary laboratory investigations may be prevented and cessation of the drug may be sufficient for reversal of the cytopenias.