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PubMedID: 30300449 Objectives: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (?100 mL/kg of pRBC or a serum ferritin [SF] level >1000 µg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 µg/L (P

Thalassemia major (TM) results in hemolytic anemia, an increase in intestinal iron absorption, and occurrence of iron loading due to erythrocyte transfusion; the disease is characterized by oxidative damage in major organs. Oxidative stress leads to vascular endothelial damage and forms the basis for serious cardiovascular diseases. Pentraxin-3 (PTX-3) is one of the markers of vascular endothelial damage that increases in response to the oxidative stress, which can be used as an early diagnostic marker for inflammation. This study's purpose is to define the relation between PTX-3 and the vascular endothelial damage that increases with oxidative stress in thalassemia patients. Our study included 35 TM patients, 30 β-thalassemia minor patients, and 30 healthy children. As a result of our study, in TM patients, a positive relation was detected between the PTX-3 levels and the total oxidative stress, triglyceride, and very low-density lipoprotein values, whereas a negative relation was detected with the total antioxidant capacity and high-density lipoprotein values. This result shows that as oxidant stress increases, PTX-3 levels also increase; very low-density lipoprotein and triglyceride contribute to the endothelial damage occurring with oxidative stress. As a result, it was concluded that vascular endothelial damage in thalassemia patients can be evaluated through the serum PTX-3 level. © Copyright 2015 Wolters Kluwer Health, Inc. All rights reserved.

BACKGROUND: The purpose of this study was to determine the epidemiological features of cases that were registered for burns and treated at a hospital after the Van earthquake to compare burn cases from the previous year and to determine the factors that influenced mortality. METHODS: Patients who were admitted to the Van Region Training and Research Hospital within the 3-month period after the earthquake were categorized as group 1; patients who were admitted within the same time interval in the previous year were categorized as group 2. RESULTS: There were 121 patients in Group 1 and 89 patients in Group 2. It was determined that there were 36% more burn cases in Group 1. Flame burns were observed 4.8 times more often in Group 1 compared to Group 1 (p=0.002). Exitus was observed in 25.4% of cases in Group 1 and in 7% of cases in Group 2 (p=0.0069). CONCLUSION: It was determined that the number of burn cases registered after the earthquake, the number of flame burns, the percentage of burns and the rate of mortality were higher than the data before the earthquake. Language: en

Leishmaniasis is a reticuloendothelial system disease that mostly observed before the age of 5. Visceral infection causes long-standing fever, weight loss, weakness, pancytopenia, and hepatosplenomegaly. Leishmania infantum is responsible for visceral leishmaniasis (VL) in Turkey. We present a case of hemophagocytic syndrome due to Leishmania infection diagnosed with an immunofluorescence antibody test (IFAT). Leishmania amastigotes were not observed on bone marrow aspiration. We consider that IFAT is very important for parasite detection in the diagnosis of VL in children, particularly when amastigotes are not obtained on bone marrow aspiration.

Although cleft palate anomaly is frequent, the criterion standards in surgical treatment have not been determined yet. There are a few techniques described for cleft palate repair owing to the limited tissue in the palatal mucosa, the rigid structure of the palatal mucosa, and the limited vascularity of the hard palate. In this study, a novel cleft palate repair technique based on separating the soft palate from the hard palate as a musculomucosal flap and using it as a rotation flap has been described. The operation is evaluated individually for each anomaly because variations occur in the surgical technique according to the extension of the cleft toward the teeth in the palate. This operation was performed on a total of 28 patients (17 girls and 11 boys) aged between 1.5 and 16 years and presented to our clinic. Patients were assessed for speech analysis outcomes, tympanogram values, hearing functions, magnitude of palatal lengthening during the operation, and rate of fistulae. Statistically significant differences in values of the speech analysis and the audiometric assessment were determined between before and 6 months after surgery. Complete recovery of otitis was observed 1 month after surgery without another treatment in 9 (42.8%) of 21 patients who were detected to have serous otitis media preoperatively. Tension-free closure, lower risk of fistula, good restoration of velopharyngeal functions, ability to be performed on all types of cleft palate, ability to provide a good intraoperative exposure, and being a single stage seem to be the most important advantages of this technique.

The major aims of correction of hypospadias include construction of a neourethra, removal of the chordee, and reduction of the probability of postoperative fistulization. Here, we describe a new technique that can be easily used in cases with subcoronal distal penile hypospadias (even those with chordee) for the construction of the urethra using meatus-based transverse flaps. This new technique was used in 10 patients (mean age, 4.6 years). There were no complications such as fistula formation, chordee recurrence, hematoma formation, or flap necrosis during the follow-up period (mean follow-up period, 13.4 months). We believe that this surgical technique can be used in cases with chordee, carries a low risk of fistula formation, has the advantage of the use of richly vascular flaps for construction of the urethra, and yields a better cosmetic result. Hence, it can safely be used for surgical correction of distal hypospadias.

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. Blood Coagul Fibrinolysis 19:519-524 © 2008 Wolters Kluwer Health Lippincott Williams & Wilkins.

Introduction: We evaluated that response to parenteral iron therapy could be helpful in distinguishing the types of iron deficiency anemia. Patients and Methods: This study analyzed responses to IV iron sucrose therapy of 15 children with unexplained refractory iron deficiency anemia (URIDA). We compared the results at diagnosis, 6 weeks and 6 months after the therapy. Results were compared with responses of 11 patients' results with iron-refractory iron deficiency anemia (IRIDA) from our previous study. Result: Six weeks after the start of treatment, ferritin, MCV, MCH and Hb values were in normal range in 10 patients. The increase in Hb, MCH, MCV, and ferritin values ranged 2.6-3.5 g/dL, 1.7-4.2 pg, 2-9 fL, and 13-25 ng/mL, respectively. In five patients, Hb, MCH, and MCV mean (range) values [11.2 g/dL (11-12.2), 24.5 pg (24-25.6), and 67 fL (65-70)] were nearly normal but ferritin mean (range) values [9.8 ng/mL (8-11)] were below normal. Six weeks after the start of treatment, Hb, MCH, MCV and ferritin values of patients with IRIDA were increased. The increase in Hb, MCH, MCV, and ferritin values ranged 0.8-2.7 g/dL, 1.7-4.2 pg, 2-9 fL, and 13-25 ng/mL, respectively. IRIDA is only partially responsive to parenteral iron supplementation. In conclusion, this study demonstrated that the response to intravenous iron therapy for the URIDA cases improved blood parameters more effectively than hereditary IRIDA. Response to parenteral iron therapy would be helpful to distinguish unexplained refractory IDA from hereditary IRIDA for clinicians who do not have access to hepcidin or TMPRS6 mutation analysis. © 2016 John Wiley & Sons Ltd.

Background: Subclinical hypothyroidism (SH) is defined as elevated serum thyroid-stimulating hormone (TSH) concentration associated with normal serum-free thyroxine levels. Effects of hypothyroidism on hemorheology had widely attracted the attention of researchers during the last decade. Objective: The purpose of this study is to determine alterations in hemorheological parameters and carotid intima-media thickness (CIMT) in children with SH. Methods: Fifty-three SH children and 31 healthy controls were enrolled. Erythrocyte deformability and aggregation were determined by an ektacytometer and plasma viscosity (PV) by a cone-plate rotational viscometer. CIMT was evaluated sonographically. Results: Erythrocyte deformability of the SH group measured at 0.53 and 1.69-30 Pa was lower than that of the control group. The erythrocyte aggregation index, aggregation half time and PV were not different between the groups. However, the aggregation amplitude and mean corpuscular hemoglobin concentration were significantly higher in SH compared to the control group. There was a negative correlation between TSH and deformability values measured at 5.33-30.0 Pa. CIMT in patients with SH was significantly higher than in the control group (p = 0.001; SH = 0.48 ± 0.04 mm, control group = 0.43 ± 0.03 mm). Conclusion: Impaired hemorheology and increased CIMT are well-known risk factors for developing cardiovascular pathologies. The results of the current study suggest the treatment of children with SH in order to avoid early circulatory problems.

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.

It is well-known that β-thalassemia is associated with changes in plasma lipids and lipoproteins [1,2,3]. To our knowledge, no data are available on lipid profiles in Turkish β-thalassemia major (TM) and β-thalassemia trait (TT) patients together. The aim of this study was to evaluate lipid profiles in two groups of patients with β-TM and β-TT and to compare them with healthy controls. The study included a total of 311 subjects. Group 1 included 131 β-TM patients (mean age: 16.3±7.58 years). Group 2 included 68 β-TT patients (mean age: 7.25±4.43 years). Group 3 consisted of 112 age- and sex-matched healthy controls (mean age: 9±4.7 years). Serum ferritin level was 2487±1103 (range: 661-5745) ng/mL in Group 1. In comparing the correlation between ferritin and lipid parameters, while a significantly negative relationship was detected between ferritin and highdensity lipoprotein cholesterol (HDL-C) (p=0.000, r=-0.602), a significantly positive relationship was detected between ferritin and triglyceride (TG) levels (p=0.02) in TM patients. Serum lipid profiles of the 3 groups are shown in Table 1.

SummaryBackground and objective: Low flow desflurane and sevoflurane anaesthesia were administered to children and compared for haemodynamic response, renal and hepatic function, recovery time and postoperative nausea and vomiting. Methods: Eighty ASA I–II patients aged 5–15 yr were included in the study. Midazolam was given for premedication. Anaesthesia induction was performed with fentanyl, propofol and atracurium. After intubation, the first group received desflurane, oxygen and nitrous oxide at 6 L min−1 and the second sevoflurane, oxygen and nitrous oxide at 6L min−1. Ten minutes after induction the flow was decreased to 1L min−1 in both groups. Haemodynamic parameters, preoperative and postoperative renal and hepatic function, the times of operation and anaesthesia, and early recovery data were recorded. Modified Aldrete scores were noted at the 10th and 30th minutes postoperatively and postoperative nausea, and vomiting were assessed. Results: There were no significant differences in haemodynamic parameters, renal and hepatic functions, postoperative recovery and postoperative nausea and vomiting between groups. The recovery time was shorter in the desflurane group compared to the sevoflurane group. Conclusion: Low flow desflurane and sevoflurane anaesthesia do not adversely affect haemodynamic parameters, hepatic and renal function in children. Desflurane may be preferred when early recovery from anaesthesia is warranted.

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Journal of Neuroradiology - In Press.Proof corrected by the author Available online since samedi 8 novembre 2014

We aimed to investigate the effects of vitamin B12 deficiency on hemorheological parameters, and the changes in these parameters following vitamin B12 treatment. 33 patients (mean-age:7±5.7 years) diagnosed as nutritional vitamin B12 deficiency, and 31 age and sex matched controls (mean-age:7.1±5.2 years) were enrolled. Erythrocyte deformability and aggregation were determined by an ectacytometer, plasma and whole blood viscosities by a cone-plate rotational viscometer. The differences between patients and controls were compared. Hemorheological parameters were repeated in the patient group following vitamin B12 treatment, and the results were compared with the initial results. In vitamin B12 deficiency, erythrocyte deformability and whole blood viscosity were found to be significantly decreased, eythrocyte aggregation was found to be significantly increased compared with the controls. Plasma viscosity was found to be decreased in deficiency but this decrease was not statistically significant. In patient group, erythrocyte deformability, whole blood and plasma viscosities were found to be significantly increased and erythrocyte aggregation was significantly decreased, after treatment. This study indicates that vitamin B12 deficiency has important effects on hemorheological parameters and adequate treatment of deficiency not only corrects the hematological parameters, but also by helping to normalize the hemorheological parameters, may contribute to the regulation of microvascular perfusion. © 2015 - IOS Press and the authors. All rights reserved.

Background: Although there are many available data about renal involvement in patients with beta thalassemia major (TM), the changes in renal functions of other types, such as thalassemia intermedia (TI) and thalassemia minor (TMin), were reported less. Therefore, we aimed to evaluate renal tubular and glomerular functions in patients with three types of beta thalassemia. Methods: This prospective case-control study was conducted on 118 beta-thalassemia patients (49 in TM, 18 in TI and 51 TMin) and 51 healthy controls. Glomerular functions [estimated glomerular filtration rate (GFR), serum cystatin C and urinary protein creatinine ratio] and tubular functions [fractioned sodium excretion (FENa), tubular reabsorption of phosphorus, urinary excretion of uric acid, levels of retinol-binding protein, alpha-1 macroglobulin (alpha-1M), and beta-2 microglobulin, calcium creatinine ratio] were assessed in all patients and controls. Results: The mean ages of the groups and controls at presentation were similar. Although GFR was similar in all patients and control groups, serum levels of cystatin C in patients with TM and TI were significantly higher compared to TMin and controls. Alpha-1M, FENa, urinary excretion of uric acid, and urine protein/creatinine ratio in TM and TI groups were significantly higher than the others. Mean cystatin C level was also higher in patients with TMin compared the controls. However, there were no significant differences according to all tubular and other glomerular functions between TMin and control groups. Conclusions: Although all types of beta thalassemia patients should be closely monitored to prevent further decrease in renal functions, the patients with TI should be considered to have a higher risk of glomerular and tubular deterioration as well as TM. © 2015 © 2015 Taylor & Francis.

OBJECTIVE:We aimed to investigate the effects of iron deficiency anemia (IDA) and vitamin B12 deficiency coexisting with IDA which is called as mixed anemia (MA) on hemorheological parameters, to compare them with each other and healthy controls, and to assess the changes in hemorheological parameters after treatment. MATERIALS AND METHODS: 32 IDA patients (mean age:6.3±5.3 years), 30 MA patients (mean age:7.2±5.4 years), and 31 healthy controls (mean age:7.1±5.2 years) were enrolled. Erythrocyte deformability and aggregation were determined by an ectacytometer, plasma and whole blood viscosities by a cone-plate rotational viscometer. Differences between IDA and MA, and healthy controls were compared. Hemorheological parameters were repeated in the patient groups after treatment and compared with the initial results. RESULTS: In both of the patient groups, erythrocyte deformability, whole blood and plasma viscosities were found to be significantly decreased before treatment, compared with the controls. After treatment these parameters were found to be increased significantly. There were no significant differences in these parameters between the IDA and MA group. Additionally, no statistically significant alteration was found in erythrocyte aggregation measurements of both patient groups. CONCLUSION: This study indicates that IDA andMAhave similar effects on hemorheological parameters. When vitamin B12 deficiency accompanies IDA which is called as MA, no further alterations occur in hemorheological parameters. The adequate treatment of these anemias not only corrects the hematological parameters, but also by helping to normalize the hemorheological parameters, may contribute to the regulation of microvascular perfusion. © 2015 - IOS Press and the authors.

Background. Beta-thalassemia is an inherited blood disorder. It results from the impaired production of ß-globin chains, leading to a relative excess of alpha-globin chains. Clinical severity separates this disease into three main subtypes: ß- thalassemia major, ß-thalassemia intermedia and ß-thalassemia minor, the former two being clinically more significant. Inflammatory processes may play an important role in some of the complications of thalassemia. Adipose tissue is one of the most important endocrine and secretory organs that release adipocytokines like adiponectin, resistin and visfatin. Aim. The aim of our study was to analyze adipocytokine concentrations (adiponectin, resistin and visfatin) in different types of ß-thalassemia patients and determine any possible correlations with disease severity. Methods. We recruited 29 patients who were transfusion-dependent ß-thalassemia- major patients, 17 patients with ß-thalassemia intermedia, 30 ß-thalassemia minor patients. The control group consisted of 30 healthy children. Anthropometric measurements, complete blood count, biochemical parameters, serum concentrations of adiponectin, resistin, visfatin were performed for all subjects. Results. Resistin and visfatin concentrations were significantly higher in ß-thalassemia minor patients than in controls. Adiponectin, resistin and visfatin concentrations were significantly higher in both ß-thalassemia intermedia and major patients than in controls. The concentrations of adiponectin, resistin and visfatin were significantly higher in both ß-thalassemia intermedia and major patients than in ß-thalassemia minor patients. There was no significant difference between ß-thalassemia intermedia and ß-thalassemia major patients for adipocytokines concentrations. Conclusion. We speculate that these adipocytokines may play a role in the development of complications in ß-thalassaemia. © 2014 Informa Healthcare.

Bernard-Soulier syndrome is an autosomal recessive coagulopathy characterized by thrombocytopenia, prolonged bleeding time and large platelets. Because of the rarity of this disease, standard perioperative treatment protocols have not been developed and there are not enough available data for the management of surgical procedures. In this case, we successfully performed adenoidectomy and tonsillectomy and circumcision surgery concurrently under the preventive and intermittent transfusion of platelets. During hospitalization, no intraoperative or postoperative bleeding complications occurred. © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins.

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 ß-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 ß-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. © 2011 Informa Healthcare USA, Inc.

WOS: 000296823500011 PubMed ID: 21718764 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 30 mg/kg/day for 3 days and 20 mg/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 238 bp to T/T genotype, 172 bp and 60 bp fragments to the C/C genotype, and 238 bp, 172 bp and 60 bp 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 LIP and the control group (chi(2)=0.84 p = 0.65, chi(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.

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.

Objective.To investigate pubertal and menstrual problems and evaluate pelvic sonographic findings in patients with β-thalassaemia major. Material and Methods.Twenty-five female patients followed for thalassaemia major constituted the study population. Sexual maturation and hormonal status were assessed. Pubertal and menstrual problems were investigated. Results.There was one patient with delayed puberty and five patients with arrested puberty. Mean ferritin level in this group of patients was slightly but not significantly higher than patients with normal pubertal maturation (2620±994ng/ml vs. 2409±1348ng/ml, p>0.05). There were 10 patients with primary amenorrhoea, three with secondary amenorrhoea, five with oligomenorrhoea and irregular menstruation and one with regular menstruation. Compared to menstruating patients, the mean uterine size was smaller (4.1±3.5cm 3 vs. 52.8±14.5cm3) in all patients with delayed and arrested puberty (p

Conjunctival melanoma (CM) is a highly malignant tumor that derives from melanocytes and is rarely seen in children. This report describes a 6-year-old female diagnosed with CM. Pediatr Blood Cancer 2008;50:384-386. (c) 2006 Wiley-Liss, Inc.

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. Pediatr Blood Cancer 2008;50:1065–1067. © 2007 Wiley-Liss, Inc.

Fanconi aplastic anemia (FAA), an inherited form of bone marrow failure syndrome, is characterized by progressive bone marrow failure and high risk of developing a malignancy. One or more congenital malformations may be detected in at least 90% of the affected individuals. Despite many forms of congenital malformations in FAA, there are no reports of coexisting FAA and diaphragmatic mesothelial cyst. Here, we report a 7-year-old female patient with FAA is associated with diaphragmatic mesothelial cyst.