Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM).

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 < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 µg/L), SCA (1655.5 to 1260 µg/L), and across age groups of 2-6 years (1971.5 to 1499 µg/L), 7-12 years (1688.5 to 1159.8 µg/L), and 13-18 years (1496.5 to 1107 µg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ≥30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. CONCLUSIONS: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (≥30 mg/kg/d) may be required to achieve iron balance.

N-acetylcysteine supplementation reduces oxidative stress for cytosine arabinoside in rat model.

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.

Pentraxin-3 Levels in Beta Thalassemia Major and Minor Patients and Its Relationship With Antioxidant Capacity and Total Oxidant Stress.

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 ß-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.

Fatal Pulmonary Embolism Due to Inherited Thrombophilia Factors in a Child With Wolfram Syndrome.

Wolfram syndrome-1 is a rare and severe autosomal recessive neurodegenerative disease characterized by diabetes mellitus (DM), optic atrophy, diabetes insipidus, and deafness. Poorly controlled type 1 DM increases the risk for thrombosis. However, coexistence of DM and hereditary thrombosis factors is rarely observed. Here we present the case of a 13.5-year-old, nonfollowed girl newly diagnosed with poorly controlled Wolfram syndrome on the basis of the results of clinical and laboratory examinations. On the eighth day after diabetic ketoacidosis treatment, pulmonary embolism developed in the subject. Thrombus identified in the right atrium using echocardiography was treated by emergency thrombectomy. Homozygous mutation in the methylenetetrahydrofolate reductase gene C677T, heterozygous factor-V Leiden mutation, and active protein C resistance were identified in the patient. The patient was lost because of a recurring episode of pulmonary embolism on the 86th day of hospitalization. We present this case to highlight the need for investigating hereditary thrombosis risk factors in diabetic patients in whom thromboembolism develops.

Evaluation of Reticulocyte Parameters in Iron Deficiency, Vitamin B12 Deficiency and Mixed Anemia.

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.

Glomerular and tubular functions in children with different forms of beta thalassemia.

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.

Adipocytokine concentrations in children with different types of beta-thalassemia.

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. Adiponetin, 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.

Serum vitamin B12 and folate concentrations and the effect of the Mediterranean diet on vulnerable populations.

Low vitamin B12 and folate levels in expectant mothers may lead to low stores in babies. The aim of this study was to determine the frequencies of vitamin B12 and folate deficiencies in pregnant women and neonates, and to assess the effect of maternal vitamin status on babies' vitamin levels in the Aegean region of Turkey, where the Mediterranean diet (mainly fresh fruits and vegetables) is adopted. We studied 72 pregnant women and their singleton-term babies. Venous blood samples of expectant mothers were collected 1 h before delivery and cord blood of babies were obtained at birth. The mean vitamin B12 in maternal and cord blood serum was 163.1 ± 72.0 pg/mL and 146.2 ± 102.5 pg/mL, and the mean folate, 9.8 ± 4.8 ng/mL and 15.8 ± 3.8 ng/mL, respectively. There were statistically significant correlation between maternal and cord blood serum vitamin B12 (r = 0.61, P = .04) and folate levels (r = 0.65, P < .001). 70.8% of the mothers and 83.9% of the babies were vitamin B12 deficient (<200 pg/mL). Neither group showed folate deficiency. The mean level of vitamin B12 in mothers significantly varied by the type of diet (241.6 (72.1) pg/mL versus 155.9 (68.2) pg/mL; P = .012). Vitamin B12 deficiency in pregnant women and neonates may be a public health problem in our community. The Mediterranean diet in these vulnerable groups may be an aggravating factor for vitamin B12 deficiency. Prenatal screening of all expectant mothers, prenatal supplementation of vitamin B12, and an increase in animal-source food intake may improve expectant mother's vitamin B12 level.

The Roberts syndrome: a case report of an infant with valvular aortic stenosis and mutation in ESCO2.

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.

Evaluation of forensic medical aspects of medical interventions and damages caused by unauthorized persons.

BACKGROUND: Medical interventions are procedures that are conducive to creating danger on people's life and body immunity due to their characteristics. We aimed to raise awareness and make contributions by revealing the fact that serious bodily and mental harms occur due to medical interventions by the ones who do not have the authority to perform. METHODS: We evaluated 210 cases appealled to our institution between the years of 2017 and 2022 who suffered damage after medical interventions by people who do not have any authorization or license. The damages that occurred in the cases were evaluated in terms of medical and legal aspects. Analysis of the data were done with SPSS 25.0 for Windows and the statistical alpha significance level were accepted as p < 0.05. RESULTS: 76.7% of the cases were women, 87.1% were young individuals under the age of 40 Of the medical interventions, 91.9% were for aesthetic/cosmetic purposes (laser epilation, tattoo removal, botox, dermapen, cold lipolysis), 8.1% were for therapeutic purposes (bone setter intervention, tooth extraction/prosthesis, intramuscular injection, cupping pull) was found. It was found that 87.1% of the medical interventions were performed in beauty salons, 12.9% in hairdressers, home, medical markets, pharmacies and dental clinics. CONCLUSIONS: All over the world and in our country, serious bodily and mental injuries occur in people after medical interventions by unauthorized persons. Therefore, it is necessary to see this situation as an important problem, and raise awareness in the society to make legal arrangements.

Autopsy findings of suicidal hanging in Mugla, Turkey.

Introduction: External and internal autopsy findings of suicidal hanging were evaluated retrospectively in Mugla, Turkey. The relations between macroscopic autopsy findings and age, gender, nature of the ligature, position of the knot, and the ligature marks were assessed. The aim of this study was to describe autopsy findings of the hangings and discuss the regional differences. Method: The reports of 175 hanging autopsies between 2013 to 2018 were evaluated retrospectively. Macroscopic external and internal findings were evaluated and compared statistically. Results: Among the 2534 autopsies, the cause of death was suicide by hanging in 175 cases (6.9%). The male-to-female ratio was 3:1, 76%(n = 133) of the cases were male, 24% (n = 42) were female, the mean age was 44.9(SD = 19.3) years. Suicide by hanging was most prevalent in the 25-44 age group and among men. Complete hanging was more common than incomplete hanging, and softer materials were more commonly used in incomplete hangings. Females utilized soft materials more than males. Males used atypical hanging more than females. Bilateral hemorrhage in the neck muscles was more common with typical hanging. There was a significant relationship between tongue protrusion and hemorrhage at the clavicular head of sternocleidomastoid muscle. Conclusion: In this study, which includes a large series, some regional and gender differences were observed. Careful and detailed macroscopic examination was important to diagnose antemortem vitality findings.

Increased availability of family donors for hematopoietic stem cell transplantation in a population with increased incidence of consanguinity.

The study was planned to determine the frequency of parental and non-sibling family donor transplants in our center and to investigate the rate of familial donor availability at two HLA-typing laboratories in Turkey. Among 203 patients who underwent hematopoietic stem cell transplantation (HSCT), 151 (74.4%) received stem cells from siblings, 48 (23.6%) from non-sibling family donors, two (1.0%) from unrelated cord blood, and two (1.0%) autologous transplantation. Of these 48 patients received stem cells from non-sibling family donors; donors were mothers for 26 (12.8%), fathers for 20 (9.9%), and aunts for two (1.0%). The rate of transplants from parental donors was 22.6% in this patient population with increased frequency of inherited diseases (58.1%). Among these 203 patients, there was consanguinity between parents in 60.6% of the patients. Of 833 subjects applying as donor candidates to HLA-typing laboratories, 527 (63.3%) had HLA 6/6 identical family donors. Among 527 full-matched donors, 479 (90.9%) were sibling, 21 (4.0%) were fathers, and 17 (3.2%) were mothers. The remaining 10 (1.9%) were other relatives. The results have shown that the unfavorable factor of consanguinity marriage may increase the availability of family donors for HSCT in particularly developing countries where large donor registries are lacking.

Detection of early cardiac dysfunction in patients with ß-thalassemia major and thalassemia trait by tissue doppler echocardiography.

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.

[An evaluation of dog and cat bites over a five-year period: a sample case from Eskisehir]. / Bes yillik süreçte köpek ve kedi isiriklarinin degerlendirilmesi: Eskisehir'den örnek bir çalisma.

BACKGROUND: In this study, it was aimed to evaluate the demographic, medical and forensic aspects of patients who were bitten by cats or dogs and admitted to the Eskisehir Osmangazi University, Education, Training and Research Hospital. METHODS: All the medical records of the last five years inside the automation system of the hospital were investigated. One hundred sixty-seven cases were included according to their ICD-10 diagnostic codes. On statistical evaluation, frequency and chi-square analyses were conducted and SPSS 13.0 program was used. RESULTS: One hundred sixteen patients were bitten by dogs, whereas 46 patients were bitten by cats. Most of the victims were children. 51.9% of the bites were on an upper extremity, and 29.1% were on a lower extremity. Although cat and dog bites demonstrate seasonal changes, summer months were found important, when children were on holidays. Hyperemia, scratch and laceration were the most frequent diagnoses after injury. The length of hospital stay was between 1-22 days and calculated as a mean 7.3±5.8 days. CONCLUSION: Dog and cat bites are very well known public health issues in our country. To know animals and their needs and to respect them should be the priority of families to avoid unexpected injury. Families should teach their children to treat dogs and cats with respect, to avoid direct eye contact with the animals and not to annoy them.

Gender differences in completed suicidal hangings from 2013 to 2018 in Mugla, Turkey.

BACKGROUND: Hanging is the most preferred among suicide methods. Although methods vary based on age groups and social and cultural conditions, many studies have reported it as the most common suicide method in Turkey. OBJECTIVE: Assess autopsy findings of suicidal hangings according to gender differences. DESIGN: Retrospective, cross-sectional. SETTING: Local morgue. SUBJECTS AND METHODS: The autopsy reports of hanging autopsies between 2013-2018. Data was retrospectively collected from autopsy reports. Sociodemographic features, reasons, suicide notes, the news media, and macroscopic autopsy findings were evaluated. Findings were statistically compared by gender. MAIN OUTCOME MEASURE: Gender differences in autopsy findings. SAMPLE SIZE AND CHARACTERISTICS: 175 autopsy reports of suicide by hanging. RESULTS: Among 2534 autopsies, 175 (6.9%) cases involved hanging. Most (76%, n=133) were males; the median (interquartile range) age was 42.0 (29.5), the male/female ratio was 3:1. Suicide notes were found in 42 cases; 81 (24%) were from men. The most common reason for suicide was a mental illness (n=51, 29.2%), followed by family problems (n=26, 14.9%), but the cause was unknown in 42 cases (24.0%). More than half of the events were covered in the news media (58.9%). Rope was most commonly used with women preferring a softer material. Men were more frequently single than women. Men were more frequently single and not actively working than women (P=.026 and P≤.001, respectively). The incidence of atypical hanging was higher for males than females (P<.05). CONCLUSION: The findings showed that there were some statistically significant differences in socioeconomic and mental health factors between men and women in hanging suicide. This study may serve as the basis for more comprehensive studies to investigate the causes of suicide. LIMITATIONS: Single-center, retrospective. CONFLICT OF INTEREST: None.

Gonadal dysfunction and pelvic sonographic findings in females with thalassaemia major.

OBJECTIVE: To investigate pubertal and menstrual problems and evaluate pelvic sonographic findings in patients with beta-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 +/- 994 ng/ml vs. 2409 +/- 1348 ng/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 +/- 3.5 cm(3) vs. 52.8 +/- 14.5 cm(3)) in all patients with delayed and arrested puberty (p < 0.05). Ten patients were taking hormone replacement therapy (HRT). The mean uterine size in these patients was larger than that in patients with amenorrhoea who were not taking HRT, but smaller than that in menstruating patients (9.1 +/- 15.9 cm(3), 2.7 +/- 1.3 cm(3) and 52.8 +/- 14.5 cm(3), respectively) (p < 0.05). CONCLUSION: Thalassaemia major has important side effects on the hypothaloma-pituitary-gonadal axis resulting in pubertal and menstrual abnormalities. HRT should be given to provide normal sexual maturation in these patients.

Consistency of postmortem interval estimations of physicians using only postmortem changes of putrefied dead bodies.

The postmortem interval is estimated based on physical, biochemical, and morphologic changes in dead bodies, scene investigation findings, and judicial investigation findings. Many factors affect the onset and the course of the postmortem changes. There is no established method for accurate estimation of the postmortem interval, especially with regard to putrefied dead bodies. Aiming to determine the consistency and the variation of postmortem interval estimations in cases with various putrefaction degrees by forensic medicine assistants and specialists from different centers, a prospective study was undertaken. A form containing data about the time, environment, and geographical location where the cases were found, along with their postmortem changes was mailed to 110 forensic medicine assistants and specialists from different centers, who were also asked to note their postmortem interval estimations. About half of the forms were returned. Those postmortem interval estimations made based on the information included in the forms, showed great variability between the participants, and in only 6 cases a consistency exceeding 50% was found between the participants and the autopsy teams. It is not possible to estimate the postmortem interval in a standard way by using only the postmortem changes of putrefied bodies. Therefore, especially in the cases involving putrefied bodies, in addition to the postmortem changes, factors such as environmental conditions, the scene, and judicial investigation findings should be taken into consideration.

The importance of measuring intraocular pressure using a tonometer in order to estimate the postmortem interval.

AIM: The purpose of this study is to evaluate postmortem eye changes and to investigate the relationship between these changes and time elapsed after death. MATERIAL AND METHOD: The eyes of 100 noncriminal cases who had died while being treated at Eskisehir Osmangazi University (ESOGU) hospital were evaluated for corneal turbidity and tache noire macroscopically, and also repeatedly evaluated by ophthalmoscope, pupilometer, and tonometer at intervals until removal from hospital. The postmortem time, corneal turbidity, development of tache noire, pupil size, intraocular pressure (IOP), and fundus findings were recorded. The relationship between these findings and the postmortem interval (PMI) was evaluated. RESULTS: No relationship between tache noire development and postmortem time (P > 0.05) was found. The corneal turbidity ratio increased significantly at 8 hours after decease (P < 0.01). No relationship between right-left pupil size and postmortem time (P > 0.05) was found. There was, however, a significant relationship between the fundus findings and postmortem time. Over time, the first optic disc becomes pale, then vascular clarity decreases and segmentation increases. The right and left IOP related significantly to postmortem time and decreased gradually as time passed (P < 0.05). Application of linear, exponential, and power equations showed that IOP can be used to estimate postmortem time by a 2 hour interval with a 95% probability. CONCLUSIONS: This study shows that corneal turbidity and IOP have a significant relationship with postmortem time and can be used to estimate a postmortem interval with other postmortem findings. This study provides data that would support the idea that such examination might be useful in estimating postmortem interval.

Frequency of pulmonary hypertension in asymptomatic ß-thalassemia major patients and the role of physiological parameters in evaluation.

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, and idarubicin (FLAG-IDA) for the treatment of children with poor-prognosis acute leukemia: the Hacettepe experience.

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.