Further treatment of coking wastewater treated in A2O-MBR by the nanofiltration-powder activated carbon hybrid system.

In this study, further treatment of coking wastewater treated in anoxic-oxic-membrane bioreactor (A2O-MBR) was investigated to meet the standards of the ministry by means of nanofiltration (NF) (with two different membranes and different pressures), microfiltration -powder activated carbon (MF-PAC) hybrid system and NF-PAC (with two different membranes and five different PAC concentrations) hybrid system. In addition to the parameters determined by the ministry, other parameters such as ammonium, thiocyanate (SCN-), hydrogen cyanide (HCN), dissolved organic carbon (DOC), dissolved inorganic carbon (DIC), color were also examined to evaluate the flux performance and treatment efficiency of the hybrid processes. According to the results, chemical oxygen demand (COD) in the NF process, COD and total cyanide (T-CN) in the MF-PAC process could not meet the discharge standards. As for the NF-PAC hybrid system, XN45 membrane met the discharge standards in all parameters (COD = 96±1.88 mg/L, T-CN =<0,02 mg/L, phenol =<0.05 mg/L), with a recovery rate of 78% at 0.5 g/L PAC concentration.

ST2 and Reg3α: Can they predict aGvHD, steroid refractoriness and transplant-related mortality in pediatric patients after HSCT?

BACKGROUND/AIM: There are several complications of hematopoietic stem cell transplantation. Without any doubt, most important of these is aGvHD that increases transplant-related mortality. The aim of this study is to investigate whether ST-2 and Reg3α levels measured at an early stage in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation can be individual biomarkers identifying future GvHD and predicting treatment response. MATERIALS AND METHODS: From January 2019 to January 2021, 27 patients undergoing hematopoietic stem cell transplantation for primary immunodeficiency or hematopoietic diseases formed the study group. During their follow-up, the patients were classified into two groups as those developing and those not developing aGvHD. Nineteen healthy volunteers from a similar age group who needed their blood samples drawn for other reasons and who did not have any history of chronic disease, infection or medication use formed the control group. Blood samples of patients scheduled to have allogeneic HSCT were obtained before the administration of the preparative regimen, on Day +7 post-transplant and on the day of diagnosis if they developed aGvHD. Serum samples were stored at -20ºC until the day of processing. ST2 and Reg3α levels were measured using the ELISA method. RESULTS: For patients who developed aGvHD (n = 13), ST2 levels obtained before the transplantation, on Day +7 post-transplant and on the day of aGvHD diagnosis (in patients developing GvHD) were significantly higher compared to the healthy Control Group (p-value <0.05). As regards to the samples obtained on the same days, ST2 levels did not differ significantly among patients who developed and those who did not develop GvHD (n = 14; p-value >0.05). ST2 levels of samples obtained on the days that acute skin and gastrointestinal tract GvHD developed did not differ significantly between these two groups (p-value >0.05). Reg3α levels of the pre-transplant samples, on Day +7 after the transplantation and on the day of aGvHD diagnosis did not show any difference between any of the groups (p-value >0.05). As only two patients died after transplantation, thus correlation of ST2 and Reg3α levels with transplant-related mortality could not be proven. CONCLUSION: The results of this study suggest that ST2 and Reg3α levels are neither diagnostic nor prognostic or predictive biomarkers of aGvHD, steroid resistance or transplant-related mortality in pediatric patients. This study can be regarded as a pilot study because of the small patient population; more research involving a larger patient population is required.

Evaluation of the reliability and accuracy of YouTube™ and TikTok™ contents about storage media for avulsed teeth: A cross-sectional study.

BACKGROUND/AIMS: The preservation of avulsed teeth is critical in dental trauma management, necessitating effective storage media to ensure viable tooth reimplantation. The urgent need for accurate information has led both professional and non-professional individuals to increasingly seek guidance from video-sharing platforms such as YouTube™ and TikTok™. These platforms have become key resources for advice on such dental emergencies, but the reliability and accuracy of this information have not yet been systematically evaluated. This study aimed to assess the quality, accuracy, and reliability YouTube™ and TikTok™ of videos regarding storage solutions for avulsion injuries. MATERIAL AND METHODS: A search was conducted on YouTube™ and TikTok™ on February 4th, 2023, using terms related to traumatic dental injuries, and 53 videos were included. Video demographics such as platform, duration, type, upload date, likes, and comments were recorded. Video content quality, reliability, and accuracy were measured using the Global Quality Scale (GQS), Journal of the American Medical Association (JAMA) benchmarks, and Video Information and Quality Index (VIQI). Research data were analyzed using SPSS IBM 24.0 at a 95% confidence interval. RESULTS: In total, 53 videos were examined. The average scores were JAMA: 1.70/4.00, modified DISCERN: 54.3/80.00, GQS: 3.88/5.00, and VIQI: 13.8/20.00. According to the modified DISCERN index, there were no "very poor" or "poor" quality videos, 49.05% were "moderate," 22.6% were "good," and 28.3% were "very good." No difference was found between YouTube™ and TikTok™ videos in terms of the modified DISCERN index, JAMA scores, GQS, and VIQI scoring (p > .05). CONCLUSIONS: Traumatic dental injuries are a common problem among children, and evaluating the reliability of available contents is becoming crucial for appropriate intervention. The study reveals that despite being sourced mainly from dental professionals, the overall quality of videos on avulsed tooth management was mediocre, underscoring the risks associated with misleading or incomplete information in such critical situations.

Evaluation of the genotoxicity, cytotoxicity, and bioactivity of calcium silicate-based cements.

BACKGROUND: As calcium silicate-based cements (CSCs) have found success in various vital pulp therapy applications, several new CSC products have emerged. This study aimed to assess the genotoxicity, cytotoxicity, and bioactivity of four CSCs by comparing the newly introduced materials Bio MTA+ and MTA Cem with previously studied materials, Biodentine and NeoMTA. METHODS: Genotoxicity was evaluated using the micronucleus (MN) assay in human peripheral blood lymphocyte cells, measuring MN frequency and nuclear division index (NDI). Cytotoxicity was assessed in human dental pulp stem cells through the Water-Soluble Tetrazolium Salt-1 (WST-1) colorimetric assay. Bioactivity was determined by ELISA, measuring the levels of angiogenic and odontogenic markers (BMP-2, FGF-2, VEGF, and ALP). Statistical analyses included ANOVA, Dunnet and Sidak tests, and Wald chi-square test. (p < .05). RESULTS: The MN frequency in the groups was significantly lower than that in the positive control group (tetraconazole) (p < .05). NDI values decreased with increasing concentration (p < .05). Bio MTA+ and NeoMTA showed decreased cell viability at all concentrations in 7-day cultures (p < .01). All materials increased BMP-2, FGF-2, and VEGF levels, with Biodentine and NeoMTA showing the highest levels of BMP-2 and FGF-2 on day 7. Biodentine displayed the highest VEGF levels on day 7. Biodentine and NeoMTA groups exhibited significantly higher ALP activity than the Bio MTA+ and MTA Cem groups by day 7. CONCLUSION: Bio MTA+ and MTA Cem demonstrated no genotoxic or cytotoxic effects. Moreover, this study revealed bioactive potentials of Bio MTA+ and MTA Cem by enhancing the expression of angiogenic and osteogenic growth factors.

Clinical assessment of deleterious oral habits and dental caries-periodontal parameters among Turkish twins.

Abstract Background/purpose: Twin studies are crucial to assess the relative contribution of genetic and environmental factors. This study was conducted to evaluate association between deleterious oral habits and dental caries-periodontal parameters among Turkish twins. Materials and methods: The study comprised 143 pairs of dizygotic (DZ) twins and 59 pairs of monozygotic (MZ) twins aged 3-15 years. Twins were examined for dental caries, plaque index, gingival index, bleeding on probing and deleterious oral habits. Mann Whitney U test was used to examine the data. Results: The MZ twin pairs consisted of 60 male and 58 female twin pairs, whereas the DZ twin pairs consisted of 144 male and 142 female. The mean age of the twins was 9.63 ± 3.0 in MZ twins and 9.47 ± 3.2 in DZ twins. The mean DMFS value of MZ twins with bruxism is higher than those of MZ twins without bruxism (P = 0.001). The mean DMFS value of DZ twins with pacifier sucking is lower than those without (P = 0.007). A statistically significant difference was found between MZ twins with and without nail biting in terms of bleeding on probing and dmfs values (P = 0.035; P = 0.012). The mean values of the plaque index increased due to the mouth breathing in DZ twins (P = 0.024). Regarding the bleeding on probing, there was a statistically significant difference between MZ twins with and without atypical swallowing (P = 0.016). Conclusion: These findings suggest that dental caries-periodontal parameters are similarly affected by deleterious oral habits in MZ and DZ twins.

Thalassemia patients in transfussion dependent period and after hematopoietic stem cell transplantation: how are the psychiatric status and life quality of these patients?

Although hematopoietic stem cell transplantation (HSCT) has been widely used to treat patients with beta-thalassemia major, evidence showing whether this treatment improves mental health, self esteem and health-related quality of life (HRQoL) is limited. We aimed to describe psychiatric problems, HRQoL and self-esteem scores of patients who have thalassemia and compared with patients who underwent HSCT in the current study. A total of 24 patients with thalassemia major and 13 patients who underwent HSCT at least 2 years ago aged between 7-37 years were included. We used The Children's Depression Inventory, The Spielberger State-Trait Anxiety Inventory, and Pediatric Quality of LifeTM (PedsQL™) for assesment of children and Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), World Health Organization Quality of Life Scale Brief Version (WHOQOL-BREF) for assessment of adults. We also used Piers Harris Self Concept Scale for children and adults. Psychopathologies are common in both groups (50% in Thalassemia group and 69.2% in HSCT group). Popularity scores in Piers Haris scale of patients in HSCT group were significantly higher compared to thalassemia group (p = 0.03). Additionally, HSCT group had higher scores in physical health subscales of HRQoL in both children and parents'(p = 0.02, p = 0.03 respectively). Our findings suggest improved HRQoL and self-esteem in thalassemia patients after HSCT. However, due to the high prevalence of mental disorders in both groups, we would like to emphasize that clinicians should examine not only the physical but also the psychological state of the patients with thalessemia during the their treatment and follow-up period after HSCT.

Patterns and Grading of Gastrointestinal Graft-Versus-Host Disease: A Clinicopathologic Correlation Study.

BACKGROUND: The present study investigated gastrointestinal involvement patterns of acute graft-versus-host disease and assessed the correlation of pathologic severity with clinical grading. METHODS: Pathology reports of gastrointestinal (GI) endoscopic biopsies taken from 164 post-hematopoietic stem cell transplant patients with at least 1 endoscopic gastrointestinal biopsy diagnosed as "consistent with acute graft-versus-host disease" between 2005 and 2019 were retrieved from the automated hospital database. Endoscopic, pathologic and clinical gradings were performed using Freiburg criteria, Lerner and modified Seattle-Glucksberg grading systems, respectively. RESULTS: The majority of the patients (n = 140, 85.4%) were investigated with more than one biopsy from various gastrointestinal sites with a total of 479 biopsies: 44 (9.2%) esophagus, 90 (18.8%) stomach, 91 (19.0%) duodenum, 20 (4.2%) terminal ileum, 32 (6.7%) right colon, 87 (18.2%) left colon and, 115 (23.9%) rectum. Overall, lower gastrointestinal (n = 118/126, 93.6%) and upper gastrointestinal (n = 91/97, 93.8%) involvements were similar (P = .3). While the most severely affected site was duodenum (P = .021) in upper gastrointestinal, pathologic grades were similar in lower gastrointestinal sites, though more severe than upper gastrointestinal (P = .003). Pathologic grading had a low positive correlation with both clinical (r = 0.308, P = .001) and endoscopic grading (coefficient: 0.261, P = .003). CONCLUSION: Considering the similar graft-versus-host disease frequency of upper and lower gastrointestinal tract, distal colon evaluation with rectosigmoidoscopy seems to be a practical approach in patients with suspected gastrointestinal graft-versus-host disease. As it was positively correlated with both endoscopic and clinical grade, pathologic grading should be performed in these patients to assess gastrointestinal involvement patterns.

The Determining Factors for Outcome of Pediatric Intensive Care Admitted Children After Stem Cell Transplantation.

INTRODUCTION: Requiring pediatric intensive care unit (PICU) admission relates to high mortality and morbidity in patients who received hematopoietic stem cell transplantation (HSCT). In this study, we aimed to evaluate the indications for PICU admission, treatments, and the determining risk factors for morbidity and mortality in patients who had allogeneic HSCT from various donors. MATERIALS AND METHODS: In this retrospective study, we enrolled to patients who required the PICU after receiving allogeneic HSCT at our Pediatric Bone Marrow Transplantation Unit between 2005 and 2020. We evaluated to indication to PICU admission, applications, mortality rate, and the determining factors to outcomes. RESULTS: Thirty-three (7%) patients had 47 PICU admissions and 471 patients underwent bone marrow transplantation during 16-year study period. Also, 14 repeated episodes were registered in 9 different patients. The median age of PICU admitted patients was 4 (0.3 to 18) years and 29 (62%) were male. The main reasons for PICU admission were a respiratory failure, sepsis, and neurological event in 20, 8, and 7 patients, respectively. The average length of PICU stay was 14.5 (1 to 80) days, 14 (43%) of patients survived and the mortality rate was 57%. Multiple organ failure ( P =0.001), need for respiratory support ( P =0.007), inotrope agents ( P =0.001), and renal replacement therapy ( P =0.013) were found as significant risk factors for mortality. CONCLUSIONS: Allogeneic HSCT recipients need PICU admission because of its related different life-threatening complications. But there is a good chance of survival with quality PICU care and different advanced organ support methods.

Evaluation of the predictors of oral health-related quality of life among 3-5-year-old children with dental trauma.

This study evaluated the influence of socio-demographic, clinical, and parental psychological factors on oral health-related quality of life (OHRQoL) and determined their predictors in preschool-aged children with traumatic dental injury (TDI). The study sample consisted of 324 dyads of children and their parents attending the Clinics of Pediatric Dentistry at Istanbul University. After clinical examination, the Sense of Coherence (SOC), Generalized Self-Efficacy Scale, Early Childhood Oral Health Impact Scale, and Multidimensional Health Locus of Control Scale-MHLC questionnaires were administered to the parents. The statistical analyses included Spearman correlation coefficients, Mann-Whitney U tests, Kruskal-Wallis tests, multiple linear regression, and confirmatory factor analysis. Having mixed TDI, a non-nuclear family, fewer children, and weak parental SOC were important predictors of worse overall OHRQoL and its sections of child and family. Lower internal health locus of control and dental pain due to TDI were predictors of worse overall OHRQoL and child impact, respectively. Consideration of these predictors may help oral health professionals to develop prevention and treatment programs for TDI and oral health literacy programs for families.

Pediatric Invasive Aspergillosis: a Retrospective Review of 59 Cases.

Invasive aspergillosis (IA) is a major cause of morbidity and mortality. This study aimed to present our 10-year IA experience at a single center. Fifty-nine pediatric patients with IA were included in this study. The male-to-female ratio was 42/17. The median age was 8.75 years. Hematologic malignancy was present in the majority of the patients (40/59, 68%). The mean neutropenia duration was 18.5 days. Cytosine arabinoside was the most common immunosuppressive therapy directed at T cells during IA diagnosis. IA cases were categorized as proven (27%), probable (51%), or possible (22%) according to the 2008 European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) criteria. The lungs (78%) were the most common site of IA, and nodules were the most frequent radiological findings (75.5%). In 38 patients (64.4%) receiving antifungal prophylaxis, prophylactic agents included fluconazole (30.5%), liposomal amphotericin B (23.7%), posaconazole (8.5%), and voriconazole (1.7%). Initial treatment was most commonly administered as monotherapy (69.5%). The median antifungal treatment duration was 67 days. Eleven deaths (18.6%) were due to aspergillosis. With the increased use of corticosteroids, biological agents, and intensive immunosuppressive chemotherapy, IA will most likely continue to occur frequently in pediatric patients.

Comparative Analysis of Perfusion Index and End-Tidal Carbon Dioxide in Cardiac Arrest Patients: Implications for Hemodynamic Monitoring and Resuscitation Outcomes.

BACKGROUND: During cardiopulmonary resuscitation (CPR), some parameters (e.g., intraarterial pressure measurement and end-tidal carbon dioxide (EtCO2)) indicate the quality and outcome of resuscitation. These parameters are generally based on monitoring the hemodynamic status. Perfusion index (PI) is a calculation from the photoplethysmography (PPG) signal and displays the proportion of pulsatile to non-pulsatile light absorption or reflection in the PPG signal. It helps to evaluate cardiac output and tissue perfusion in the care of a critical patient. Its most important advantages are that it can be easily measured with a pulse oximeter probe attached to the finger (non-invasive), can be objectively repeated, can be applied quickly, and is inexpensive. Normal PI values range from 0.2% to 20%. Despite being recognized as a valuable indicator of hemodynamics, there is limited information regarding its relevance in patients experiencing cardiac arrest. Although the PI is known to be a valuable parameter to indicate hemodynamics, information about its value in cardiac arrest patients is limited. This study aims to evaluate the performance of PI and EtCO2 in predicting the return of spontaneous circulation (ROSC) among cardiac arrest patients. METHODS: This was a single-center, prospective, observational clinical study including both out-of-hospital and in-hospital adult cardiac arrest patients. The study was conducted from November 1, 2018 to April 30, 2019 at the Emergency Department (ED) of the Hacettepe University Hospital, Ankara, Turkey. The EtCO2 values of the patients were recorded at the time they were intubated (t0) and every five minutes (t5, t10, t15...) during CPR. Along with these measurements, PI values were measured with the Masimo Signal Extraction Technology device (Masimo, California, United States). The study's primary outcome was PI's performance in predicting the ROSC among cardiac arrest patients. The secondary outcomes of the study were the performance of EtCO2 in predicting the ROSC among cardiac arrest patients and the association between PI and EtCO2 values. RESULTS: We included a total of 100 cases. The mean age of patients was 70.4 ± 13.4 years, and 65% were male. The ROSC was achieved in 29 patients. There was no statistical difference in PI values between the ROSC (+) and ROSC (-) groups at any minute. However, in the ROSC (+) group, EtCO2 values were observed to be high starting from the fifth minute (t5, p=0.010; t10, p<0.001; t15, p=0.014; t20, p=0.033; t25, p=0.003, respectively). There was no correlation between the PI and EtCO2 values at 0, 5, 10, 15, 20, and 25 minutes (t0, p=0.436; t5, p=0.154; t10, p=0.557; t15, p=0.740; t20 p=0.241; t25 p=0.201, respectively). CONCLUSION: Measuring PI values during resuscitation in intubated cardiac arrest patients does not help clinicians predict the outcome. In addition, no correlation was found with EtCO2 values. However, EtCO2 values remained high in patients with the ROSC from the fifth minute onward. Further larger-scale studies are needed regarding the optimal use of PI in cardiac arrest patients.

Chickenpox Associated Retinitis and Occlusive Vasculitis in a Vaccinated Child: A Case Report and Literature Review.

Either retinitis and occlusive vasculitis are rare but vision threatening ocular complications of chickenpox in children. In this case report a 13-year-old girl who developed chickenpox 2 days before complaining with visual loss in her right eye is presented. She was vaccinated one dose of varicella zoster virus (VZV) vaccine when she was 12 months old. Best corrected visual acuity was counting fingers at 1.5 m in right eye. A subtle anterior segment inflammation and mild vitritis were observed. Fundoscopic examination of right eye showed ischemia in paracentral macula and white foci of retinitis along the superotemporal branch of retinal vessels. She was hospitalized and intravenous acyclovir treatment at 3 × 10 mg/kg daily dose was started. Serum IgM and IgG for VZV were positive. Aqueous humor PCR test was also reported positive for VZV DNA. Oral methylprednisolone was added at a dose of 64 mg/day at the 3rd day acyclovir treatment. Macular edema developed at 4th week of treatment and bevacizumab was administered intravitreally. After 3 injections retinal edema subsided completely. At 6-month follow-up retinal ischemia in superotemporal periphery was observed and photocoagulation was added to treatment.

Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience.

We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for thalassemia major (TM) patients in Turkey on behalf of the Turkish Pediatric Stem Cell Transplantation Group. We retrospectively enrolled 1469 patients with TM who underwent their first HSCT between 1988 and 2020 in 25 pediatric centers in Turkey. The median follow-up duration and transplant ages were 62 months and 7 years, respectively; 113 patients had chronic graft versus host disease (cGVHD) and the cGVHD rate was 8.3% in surviving patients. Upon the last visit, 30 patients still had cGvHD (2.2%). The 5-year overall survival (OS), thalassemia-free survival (TFS) and thalassemia-GVHD-free survival (TGFS) rates were 92.3%, 82.1%, and 80.8%, respectively. cGVHD incidence was significantly lower in the mixed chimerism (MC) group compared to the complete chimerism (CC) group (p < 0.001). In survival analysis, OS, TFS, and TGFS rates were significantly higher for transplants after 2010. TFS and TGFS rates were better for patients under 7 years and at centers that had performed over 100 thalassemia transplants. Transplants from matched unrelated donors had significantly higher TFS rates. We recommend HSCT before 7 years old in thalassemia patients who have a matched donor for improved outcomes.

The role of galactomannan test results in the diagnosis of pediatric invasive aspergillosis.

BACKGROUND: Invasive aspergillosis (IA) is an important cause of morbidity and mortality in immunosuppressed children. Early detection of the infection can improve prognosis in this patient population. OBJECTIVES: To investigate the utility of Aspergillus galactomannan antigen assay (GM-EIA) as a diagnostic tool for IA in at-risk paediatric patients. PATIENTS/METHODS: For the study, 659 GM-EIA results from 59 patients diagnosed with IA and 3368 GM-EIA results from 351 subjects without evidence for IA (controls) were reviewed retrospectively. Three cut-off values (i.e. ≥0.5, ≥1, ≥1.5) were specified to determine GM-EIA positivity. RESULTS: The median age was 6.3 years for boys and 14.5 years for girls. There was a significant difference between the girls and boys in terms of age (p < 0.01). For proven/probable/possible IA patients, sensitivity of 67.8% and specificity of 59.8% were detected when the ≥0.5 cut-off value was used for GM-EIA-positivity. The specificity increased to 80% at the cut-off of ≥1 and to 88% at the cut-off of ≥1.5. False positivity rates were 9.14, 3, and 1.45% at the ≥0.5, ≥1 and ≥1.5 cut-offs respectively. In the proven/probable IA group, sensitivity and negative predictive values were 86.9 and 97.2% at the ≥0.5 cut-off, 85.7 and 97.9%, at the ≥1 cut-off and 84.2 and 98.1% at ≥1.5 cut-off respectively. The positive likelihood ratio was 7.57 and the odds ratio was 42.67 at ≥1.5 cut-off. CONCLUSION: The GM-EIA may be used for both screening and diagnostic purposes in paediatric patients using a cut-off value of ≥1.5 for GM-EIA positivity.

Evaluation of the predictors of oral health-related quality of life among 3-5-year-old children with dental trauma

Abstract This study evaluated the influence of socio-demographic, clinical, and parental psychological factors on oral health-related quality of life (OHRQoL) and determined their predictors in preschool-aged children with traumatic dental injury (TDI). The study sample consisted of 324 dyads of children and their parents attending the Clinics of Pediatric Dentistry at Istanbul University. After clinical examination, the Sense of Coherence (SOC), Generalized Self-Efficacy Scale, Early Childhood Oral Health Impact Scale, and Multidimensional Health Locus of Control Scale-MHLC questionnaires were administered to the parents. The statistical analyses included Spearman correlation coefficients, Mann-Whitney U tests, Kruskal-Wallis tests, multiple linear regression, and confirmatory factor analysis. Having mixed TDI, a non-nuclear family, fewer children, and weak parental SOC were important predictors of worse overall OHRQoL and its sections of child and family. Lower internal health locus of control and dental pain due to TDI were predictors of worse overall OHRQoL and child impact, respectively. Consideration of these predictors may help oral health professionals to develop prevention and treatment programs for TDI and oral health literacy programs for families.

Ocular Findings of Pediatric Dry Eye Related to Graft-Versus-Host Disease

Objectives: To evaluate the frequency and findings of dry eye associated with ocular graft-versus-host disease (GVHD) in pediatric hematopoietic stem cell transplantation (HSCT) patients. Materials and Methods: Retrospectively the records of pediatric patients with ocular GVHD were evaluated and ophthalmologic examination findings as well as Schirmer test results, tear film break-up time, and corneal staining grades were recorded. In severe dry eye patients topical cyclosporine-A was prescribed and the results were evaluated. Results: GVHD was detected in 51 (23.4%) of 218 HSCT patients, 4 of whom died during follow-up. Thirty (63.8%) of the remaining 47 patients had chronic ocular GVHD and 4 patients with severe dry eye were treated with topical cyclosporine-A with a median follow-up of 12.1 months. Severe dry eye symptoms and findings significantly improved in 2 patients. However, 1 patient had to stop treatment due to side effects. Conclusion: In children, chronic ocular GVHD is a common finding of GVHD after HSCT. Therefore, these patients should be examined periodically for dry eye.

Tissue Plasminogen Activator Use in Pediatric Patients: A Single Center Experience.

INTRODUCTION: Thrombosis is rare in children and antithrombolytic treatment is controversial. Most commonly used thrombolytic agent is tissue plasminogen activator (t-PA) in pediatrics. In this study, we report our experience in the use of thrombolytic treatment. METHODS: Eighteen patients who had received systemic t-PA between January 2006 and December 2013 were recorded. The response to t-PA was evaluated as complete, partial, and no. The bleeding complication during t-PA administration was graded as minor or major. RESULTS: There were 18 patients (2 mo to 12 y) who received systemic t-PA. Three patients had venous, 14 patients had arterial, and 1 patient had intracardiac thrombosis. Thrombosis was related to cardiac catheterization (61.1%), central venous catheterization (16.7%), cardiac surgery (11.1%), and arrhythmia (5.5%). In 1 patient thrombosis occurred spontaneously (5.5%). Eighteen patients received 25 courses of systemic t-PA (0.15 to 0.3 mg/kg/h). A total of 55.6% of cases had complete, 27.8% had partial, and 16.6% showed no resolution. CONCLUSION: t-PA infusion at doses of median 0.2 mg/kg/h (0.15 to 0.3) seems effective and safe. There is still no consensus on indications and dosing of antithrombolytic treatment in children but in selected patients it decreases long-term complications due to thrombosis.

A Novel PUS1 Mutation in 2 Siblings with MLASA Syndrome: A Review of the Literature.

ABSTRACT: Myopathy, lactic acidosis, and sideroblastic anemia (MLASA) is a rare mitochondrial disorder characterized by MLASA. Variable features of this condition include failure to thrive, and developmental delay or intellectual disability. Additional symptoms consist of cognitive impairment, skeletal and dental abnormalities, delayed motor milestones, cardiomyopathy, dysphagia, and respiratory insufficiency. MLASA has previously been associated with mutations in pseudouridylate synthase 1 (PUS1) and YARS2. PUS1 encodes the nuclear PUS1 enzyme, which is located in both the nucleus and the mitochondria. PUS1 converts uridine into pseudouridine in several cytosolic and mitochondrial transfer RNA positions and increases the efficiency of protein synthesis in both compartments.In the present report, we report on 2 Turkish sisters 4 and 11 of years with an MLASA plus phenotype. Both patients have sideroblastic anemia, lactic acidosis, failure to thrive, developmental delay, and chronic diarrhea; in addition, the older sister has strabismus and skeletal anomalies. The sequencing of the PUS1 gene revealed a novel homozygous p.Glu311* mutation. The phenotype of the older sibling is also unique because of the strabismus and skeletal anomalies, when compared with her sister and other previously reported patients with MLASA. The structural differences in the nuclear versus mitochondrial isoforms of PUS1 and modifier genes may be implicated in the variability of the clinical presentations in MLASA. CONCLUSION: This report adds to the growing number of mutations causing complex clinical manifestations of MLASA including lactic acidosis, sideroblastic anemia, chronic diarrhea, and myopathy.

Nosocomial pneumonia caused by water-born Legionella pneumophila in a pediatric hematopoietic stem cell transplantation recipient for thalassemia major.

BACKGROUND: Nosocomial pneumonia caused by Legionella pneumophila serogroup 2-14 occurred in a 7-year-old patient following allogeneic hematopoietic stem cell transplantation for thalassemia major. CASE: The patient was diagnosed with nosocomial Legionella pneumophila by polymerase chain reaction (PCR) examination of the bronchoalveolar lavage and culturing Legionella pneumophila serogroup 2-14 from the patient`s room faucet water. Legionella pneumophila was eradicated from our hospital`s water distribution system by superheating and chemical eradication methods (hyper-chlorination and hydrogen peroxide). We did not detect any other case after this event. CONCLUSION: Early recognition of contamination of the hospital water system with Legionella proves the importance of prevention in new cases.

Delayed Diagnosis of Cobalamin E Defect in an Adolescent Patient.

Cobalamin and its metabolites play a critical role in deoxyribonucleic acid synthesis. Disorders of cobalamin metabolism are rare and related with neurological and hematological problems. We report an adolescent patient with cobalamin E (CblE) defect presenting with megaloblastic anemia, mental retardation, cerebral atrophy, cortical visual impairment, white matter changes on brain magnetic resonance imaging, and hyperhomocysteinemia. Homozygous mutation at the c.245C>T in exon 3 of the MTRR gene was identified, which had been found to be related to CblE defect. He was treated with betaine, folic acid, vitamin B6, riboflavin, hydroxycobalamin (OH-B12), and carnitine. During treatment, homocysteine levels decreased over time.