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OBJECTIVE: Preoperative coagulation tests have not been shown to be effective in predicting bleeding complications. The Pediatric Bleeding Questionnaire (PBQ) is a proven and sensitive tool for diagnosing children with a predisposition to bleeding. The aim of this study was to evaluate the usefulness of PBQ as a preoperative screening tool for the prediction of bleeding after minor surgical interventions. METHODS: Preoperative coagulation tests and PBQ were performed in all patients who underwent minor surgery. The postoperative bleeding status was evaluated and then compared with the coagulation tests and PBQ of the patients. RESULTS: Evaluation was made of a total of 706 patients, comprising 91.2% males and 8.8% females, with a mean age of 4.8 years (median: 4 y, QR: 1 to 7 y). Prolongation in coagulation tests was observed in 131 (18.5%) patients. Repeated tests in 116 patients were within the normal range, and 5 patients received treatment. Postoperative bleeding occurred in 4 (0.5%) patients. The relationship between coagulation tests and postoperative bleeding was not significant. PBQ was found to be ≥2 in 14 patients, but none of these patients had postoperative bleeding. No significant relationship was found between postoperative bleeding status and PBQ ( p :0.77). The sensitivity, specificity, positive predictive, and negative predictive values of PBQ were 0%, 98%, 0%, and 97.4%, respectively. CONCLUSIONS: The results of this study demonstrated that neither coagulation tests nor PBQ will be sufficient to predict bleeding after minor surgery, that prolongation in coagulation tests does not always indicate a bleeding tendency, and that bleeding history should also be recorded in detail.
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Hemorragia Pós-Operatória , Humanos , Feminino , Masculino , Criança , Pré-Escolar , Inquéritos e Questionários , Hemorragia Pós-Operatória/diagnóstico , Hemorragia Pós-Operatória/etiologia , Lactente , Testes de Coagulação Sanguínea , Valor Preditivo dos Testes , Adolescente , Sensibilidade e EspecificidadeRESUMO
The number of studies evaluating teicoplanin lock therapy in coagulase-negative staphylococcus-associated catheter infection in pediatric malignancies is limited. The aim of this study was to evaluate the efficacy of teicoplanin lock therapy in pediatric cancer cases. Twenty-two patients with coagulase-negative staphylococcus-associated totally implantable venous access device infection, who had undergone teicoplanin closure treatment, were included in the study. Demographic data, number of lock treatment days, and treatment success data were obtained from the medical files of the patients. Fourteen of the patients (63.6%) had acute lymphocytic leukemia, 3 (13.6%) had acute myelocytic leukemia, and 5 (22.7%) had solid cancer. The median neutrophil count was 240×10 3 /µL (interquartile range: 0 to 1195×10 3 /µL). Between patients with and without catheter removal, no statistically significant difference was found in terms of baseline C-reactive protein, absolute neutrophil count, and the day of starting systemic teicoplanin treatment ( P >0.05). The overall port survival rate of teicoplanin lock therapy was 72.7%. Within an average of 4 days, negative cultures of 16 (72.7%) patients whose catheters had not been removed were obtained. In conclusion, we suggest that teicoplanin lock therapy is an effective and safe treatment for catheter-related infections, caused by methicillin-resistant coagulase-negative staphylococcus.
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Bacteriemia , Infecções Estafilocócicas , Criança , Humanos , Teicoplanina/uso terapêutico , Antibacterianos/uso terapêutico , Coagulase , Staphylococcus , Bacteriemia/etiologia , Bacteriemia/complicações , Infecções Estafilocócicas/tratamento farmacológicoRESUMO
BACKGROUND: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. MATERIALS AND METHODS: In this multicenter retrospective study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ). RESULTS: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae. CONCLUSION: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.
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Infecções Fúngicas do Sistema Nervoso Central , Transplante de Células-Tronco Hematopoéticas , Infecções Fúngicas Invasivas , Leucemia , Criança , Humanos , Estudos Retrospectivos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Infecções Fúngicas Invasivas/diagnóstico , Infecções Fúngicas Invasivas/tratamento farmacológico , Infecções Fúngicas Invasivas/etiologia , Infecções Fúngicas do Sistema Nervoso Central/diagnóstico , Infecções Fúngicas do Sistema Nervoso Central/terapia , Antifúngicos/uso terapêutico , Leucemia/tratamento farmacológicoRESUMO
The aim of this study was to evaluate the diagnostic utility of serum galactomannan (GM) positivity for invasive aspergillosis (IA) in children. Positive GM results between January 2015 and August 2017 were reviewed retrospectively in children with hematologic malignancies. Single and consecutive positive GM results were evaluated according to the different galactomannan index (GMI) (>0.5, >0.7, >1.0 and >1.5) values. There were 104 positive GM results of 70 patients. IA was identified in 29 patients (41.4%) (2 proven and 27 probable). For a single positive GMI of >0.5, >0.7, >1.0, and >1.5, the numbers were 104, 76, 57, and 32 and the positive predictive values (PPVs) were 39.4%, 43.2%, 47.2%, and 50.0%, respectively. The single GM positivity at different thresholds showed no difference between the IA and non-IA group (P>0.05). For 2 consecutive positive GMI values of >0.5, >0.7, >1.0, and >1.5, the numbers were 34, 20, 13, and 4, and the PPVs were 58.8%, 65.0%, 84.6%, and 100.0%, respectively. In the IA group, positivity was higher at all thresholds (P<0.05). According to our findings, consecutive GM positivity has higher PPVs independently from the cutoff value chosen. In pediatric patients with high risk, consecutive sampling should be preferred.
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Aspergilose/diagnóstico , Aspergillus/isolamento & purificação , Biomarcadores/sangue , Neoplasias Hematológicas/complicações , Mananas/sangue , Adolescente , Aspergilose/sangue , Aspergilose/epidemiologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Seguimentos , Galactose/análogos & derivados , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Centros de Atenção Terciária , Turquia/epidemiologiaRESUMO
BACKGROUND: Central line bundle programs were found to be effective in decreasing central line-associated bloodstream infection rates in pediatric cancer patients with ports. However, cost-effectiveness studies of central line bundle programs in pediatric cancer patients are limited, and most available data are from intensive care unit or adult studies. METHODS: In this cross-sectional study spanning 6 years, comprehensive assessment of total health care costs attributable to CLABSI's associated with ports between two periods. RESULTS: This cross-sectional study was carried out in the pediatric hematology-oncology ward of Dr. Behçet Uz Children's Hospital from 1 August November 2011 to 31 July 2017. The CLABSI rates decreased significantly from 8.31 CLABSIs to 3.04 per 1000 central line days (p < 0.001). In the pre-bundle period, total attributable costs spent for of patients with CLABSI were $130,661, and in the bundle period, total attributable costs spent for patients with CLABSI were $116,579. Within bundle implantation, 71 potential CLABSI were prevented, which saved an additional $208,977. CONCLUSION: Our study shows that central line bundles decreases not only the CLABSI rate but also decreases attributable costs due to CLABSI. Expenses spent for bundle elements, were covered by savings by preventing CLABSI with higher costs.
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Infecções Relacionadas a Cateter/prevenção & controle , Cateterismo Venoso Central/instrumentação , Cateteres Venosos Centrais/microbiologia , Infecção Hospitalar/prevenção & controle , Seringas , Adulto , Criança , Análise Custo-Benefício , Estudos Transversais , Feminino , Custos de Cuidados de Saúde , Recursos em Saúde , Hospitais Pediátricos , Humanos , Unidades de Terapia Intensiva , Masculino , NeoplasiasRESUMO
An amendment to this paper has been published and can be accessed via the original article.
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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.
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Anemia Falciforme/complicações , Deferasirox/uso terapêutico , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Talassemia/complicações , Adolescente , Anemia Falciforme/terapia , Biomarcadores , Transfusão de Sangue , Criança , Pré-Escolar , Estudos de Coortes , Deferasirox/administração & dosagem , Deferasirox/efeitos adversos , Feminino , Ferritinas/sangue , Ferritinas/metabolismo , Humanos , Ferro/sangue , Ferro/metabolismo , Quelantes de Ferro/administração & dosagem , Quelantes de Ferro/efeitos adversos , Sobrecarga de Ferro/metabolismo , Masculino , Talassemia/terapia , Resultado do Tratamento , TurquiaRESUMO
BACKGROUND: Acute viral respiratory infections are common causes of febrile episodes in children. There are still limited data about distribution of acute viral respiratory infections in children with cancer. OBJECTIVE: The first aim of this study was to evaluate the viral etiology and seasonality of acute viral respiratory infection in pediatric patients with cancer in a 3-year study. Our second aim was to evaluate the impact of viral infections on delaying the patients' chemotherapy or radiotherapy. MATERIALS AND METHODS: This cross-sectional study was conducted from January 2014 to July 2017. Nasopharyngeal aspirates were analyzed in patients younger than 21 years with acute respiratory infections. Patients were treated in the Pediatric Hematology and Oncology Department of Dr. Behçet Uz Children's Hospital with real-time multiplex polymerase chain reaction. Data were analyzed to determine the frequency and seasonality of infections. The χ or the Fisher exact tests were used. RESULTS: A total of 219 samples of nasopharyngeal aspirates and blood were analyzed. The mean patient age was 76.8±59.3 months, with 46.3% female and 53.7% male children in a total of 108 patients. Of this total, 55% (60/108 cases) had multiple acute respiratory infections. Acute lymphoblastic leukemia (48.1%) was the most prevalent disease. The 3 most prevalent viruses were human rhinovirus (HRV) (33.1%), parainfluenza (PI) (18.7%), and coronavirus (CoV) (14.8%). In terms of the seasonal distribution of viruses, PI was most common in winter 2014, HRV in spring 2014, HRV in fall 2014, PI in winter 2015 and summer 2015, CoV in spring 2015, HRV in fall 2015, both influenza and HRV in winter 2016, both human metapneumovirus and bocavirus in spring 2016, HRV in summer 2016, both HRV and PI in fall 2016, both respiratory syncytial virus and influenza in winter 2017, HRV in spring 2017, and both HRV and adenovirus in summer 2017. The mean duration of neutropenia for patients with viral respiratory infection was 17.1±13.8 (range: 2 to 90) days. The mean duration of symptoms of viral respiratory infection was 6.8±4.2 (range: 2 to 31) days. A delay in chemotherapy treatment owing to viral respiratory infection was detected in 73 (33.3%) patients. The mean duration of delay in chemotherapy treatment was 9.6±5.4 (range: 3 to 31) days. CONCLUSIONS: In conclusion, we report our 3-year experience about the frequency and seasonality of respiratory viruses in children with cancer.
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Neoplasias/complicações , Infecções Respiratórias/complicações , Viroses/complicações , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Neoplasias/tratamento farmacológico , Prevalência , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/virologia , Estudos Retrospectivos , Estações do Ano , Viroses/epidemiologia , Viroses/virologiaRESUMO
Leukocyte adhesion deficiency type II (LAD II) is a rare, autosomal, recessive inherited immunodeficiency disease that induces frequent and recurrent infections, persistent leukocytosis, severe mental and growth retardation, and impaired wound healing. The Bombay blood group is a rare blood group phenotype that is characterised by the deficiency of H, A, and B antigens on the surface of red cells. LAD II and the Bombay blood group are always seen together, because both of them are associated with a global defect in the common pathway of fucose metabolism. Here we report the case of an 11-year-old boy with LAD II, who presented with the Bombay blood group. Agglutination with strength of 4+ was detected in all cross-matching due to erythrocyte transfusions for our patient. Therefore, the Bombay blood group was incidentally determined due to deficient expression of the CD15 adhesion molecules on the surface of the leukocytes according to the results of flow cytometry. Upon detecting the Bombay blood type, LAD II was then diagnosed as a result of flow cytometry and the clinical findings of mental retardation and history of recurrent infections such as abscesses.
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Congenital dyserythropoietic anemias (CDAs) are rare hereditary blood disorders characterized by ineffective erythropoiesis, hemolysis, and erythroblast morphologic abnormalities in the bone marrow. The 3 main types of CDA, I to III, and variant types of CDA, IV-VIII, have been described. The causative genes have been identified as CDAN1, C15ORF41, SEC23B, KIF23, KLF1, and GATA1. CDA type II is the most frequent form. Typical symptoms are jaundice, hepatosplenomegaly, mild-to-severe normocytic anemia, and inadequate reticulocyte response. We report an 18-year-old boy who had chronic mild congenital anemia, jaundice, and splenomegaly mimicking nonautoimmune hemolytic anemia since 18 months of age. Compound heterozygous mutations in SEC23B gene were detected by the use of a gene-targeted next-generation sequencing panel: the already reported missense mutation c.40C>T (p.Arg14Trp), and a new frameshift deletion (c.489_489delG, p.Val164Trpfs*3), confirming the diagnosis of CDA type II. The study underlines the molecular heterogeneity of CDA II and the importance of a precise diagnosis in rare congenital diseases such as CDA II. In consequence, it can be difficult to diagnose because of limited resources, financial constraint, and rarity of disease in the developing country. Advanced laboratories and new molecular approaches may help in diagnosing rare anemias.
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Anemia Diseritropoética Congênita/genética , Anemia Hemolítica Congênita/genética , Mutação , Proteínas de Transporte Vesicular/genética , Adolescente , Anemia Hemolítica Congênita/diagnóstico , Doença Crônica , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Técnicas de Diagnóstico MolecularRESUMO
Thymomas are the most common masses located in the anterior mediastinum, and they are often associated with autoimmune disorders including myasthenia gravis, polymyositis, and aplastic anemia (AA). Autoreactive T-cell clones generated by the thymoma may lead to autoimmune disorders. We report the case of a 14-year-old boy who was examined for AA, and the underlying cause was determined to be an immune-mediated complication of thymoma. He had no matched sibling donors. He underwent thymectomy, and 3 months later he was treated with immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A. The duration of the IST was determined to be a period of 12 months. He has recently been in complete response condition for 6 months since IST stopped. IST is a successful treatment choice for thymomas associated with AA in childhood.
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Anemia Aplástica/imunologia , Imunossupressores/administração & dosagem , Timoma/complicações , Adolescente , Anemia Aplástica/etiologia , Soro Antilinfocitário/uso terapêutico , Ciclosporina/uso terapêutico , Humanos , Masculino , Timoma/tratamento farmacológico , Resultado do TratamentoRESUMO
Non-typhoidal Salmonella (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by Salmonella in paediatric leukaemia patients, which has been reported for the first time in both Europe and the US. According to our research, NTS might be a cause of serious infections in paediatric haematology-oncology patients. Following a low bacterial diet and increasing the hygiene of both the children and their surroundings would be beneficial in preventing these infections.
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The genetic basis of haemophagocytic lymphohistiocytosis (HLH) has not been elucidated in 10% of affected patients. In this study, we report four HLH episodes in three patients with HAX1 gene mutations. We screened the mutations associated with congenital neutropenia (CN) because the neutropenia persisted following HLH treatment. There were homozygous HAX1 mutations detected in all patients. This is the first case series of patients with CN caused by HAX1 mutation who presented with HLH. We hypothesize that severe neutropenia persists after an HLH episode in children without HLH mutations (especially infants) because these patients have CN caused by HAX1 mutations.
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Proteínas Adaptadoras de Transdução de Sinal/genética , Linfo-Histiocitose Hemofagocítica/genética , Mutação/genética , Síndrome Congênita de Insuficiência da Medula Óssea , Feminino , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Hematínicos/uso terapêutico , Humanos , Lactente , Masculino , Neutropenia/congênito , Neutropenia/genéticaRESUMO
Chronic neutropenia (CN) is defined as neutropenia that persists for >3 months; it is caused by a heterogeneous group of diseases in children. The aim of the present study was to evaluate the significance and clinical manifestations of CN in children at a single children's hospital. Between October 2004 and April 2014, CN patient data were evaluated retrospectively. Thirty-one patients were assessed in this study. Thirteen of them (41.9%) were younger than 12 months of age at initial diagnosis. There was no difference in the absolute number of neutrophils at diagnosis between the children aged younger than 12 months and those aged 12 months and older at CN onset. Twenty-two of the patients (70.9%) were diagnosed during treatment for acute infections. A total of 11 patients (35.5%) were hospitalized because of recurrent infections. Most of the recurrent infections occurred in the lungs (81.8%). Congenital neutropenia (CoN) was identified in 14 patients (45.1%). Eight of 14 patients (57.1%) required granulocyte-colony stimulating factor treatment, and none of them experienced adverse effects from this treatment. Fifteen patients (48.3%) were diagnosed with idiopathic neutropenia. Comparison between the idiopathic and CoN groups revealed no differences in age, the absolute number of neutrophils, or the presence of infection at diagnosis; however, differences were detected in sex and the rate of spontaneous recovery from neutropenia. Ten of the patients (32.2%) experienced spontaneous recovery from neutropenia during a follow-up period of 7 to 52 months. In current study, we found a higher CoN ratio in the CN patients compared with previous reports, which may be due to the high rate of consanguineous marriages in our country. However, the finding of CN requires several laboratory investigations, prolonged follow-up, and advanced molecular analysis, and its etiology can remain idiopathic.
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Neutropenia/diagnóstico , Neutropenia/epidemiologia , Neutropenia/etiologia , Adolescente , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
INTRODUCTION: Hemophilia is a genetic disorder in which recurrent joint bleeding causes arthropathy. Inflammation and degeneration play roles in the pathogenesis of hemophilic arthropathy. Patients with juvenile idiopathic arthritis (JIA) experience a similar inflammatory degenerative joint disease. A comparison of different patients with common pathogenetic features may identify unique features helpful in terms of the follow-up. AIM: We compared the quality of life (QoL) of patients with hemophilia and JIA, and healthy controls, using a generic QoL scale, Kidscreen and Disabkids Questionnaires (KINDL). Differences among groups were evaluated in terms of sociodemographic characteristics and clinical parameters affecting the QoL. METHODS: We included 33 hemophilia patients, 19 JIA patients, and 32 healthy individuals aged 4 to 18 years. Sociodemographic characteristics (the age, the maternal educational status, the place of residence, the size of the household, the household income, divorced parents) were noted, and the KINDL was administered to all participants. Clinical parameters associated with arthropathy (the functional independence score [FISH], the hemophilia joint health score [HJHS], the arthropathic joint count, and the painful joint count) were documented. Differences in frequencies and medians among the groups were evaluated using the χ, the Mann-Whitney U, and the Kruskal-Wallis tests. RESULTS: All KINDL dimensions were above 50, reflecting "good conditions" in the 2 patient groups. No difference between patients with hemophilia and JIA was evident in terms of the clinical parameters of FISH, the HJHS, or the arthropathic or painful joint counts (P>0.05). Sociodemographically, only the frequency of literate mothers was lower in patients with hemophilia than in those with JIA and healthy controls (P=0.03). Patients with JIA scored more higher on the KINDL dimension of chronic illness than those with hemophilia (P=0.02). The FISH score correlated with the total QoL score in both patients with hemophilia and JIA (r=0.39, P=0.03 and r=0.48, P=0.04, respectively). CONCLUSIONS: Although no difference was evident between the patient groups in terms of clinical parameters associated with arthropathy, JIA patients coped better with illness than those with hemophilia. JIA patients had a higher proportion of literate mothers than hemophilia patients; this may affect a patient's ability to cope with issues relating to chronic illness. Implementation of an educational program for mothers of hemophilia patients, during follow-up, may improve the patient's QoL. Also, hemophilia patients should be assisted to improve their QoL in the dimensions of self-esteem and schooling. Lastly, the evaluation of functional disability by FISH in hemophilia patients is important because the FISH score correlated with the total QoL score, as revealed by KINDL. In JIA patients also, functional disabilities caused by arthropathy affected the QoL.
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Artrite Juvenil/psicologia , Hemofilia A/psicologia , Artropatias/psicologia , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Feminino , Hemartrose/etiologia , Hemartrose/psicologia , Hemofilia A/complicações , Hemofilia B/complicações , Hemofilia B/psicologia , Humanos , Vida Independente , Artropatias/etiologia , Masculino , Índice de Gravidade de Doença , Fatores Socioeconômicos , Inquéritos e Questionários , Sinovite/etiologia , Sinovite/psicologiaRESUMO
The granulocyte transfusion (GTX) has been used for a long time due to uncontrolled neutropenic fever with antimicrobial agents. In some cases, the product needs to be splitted for using in the next 12 hours. The aim of this study is to evaluate the efficacy of splitted product and clinical response to GTX. In this study, 15 patients with malignancy with 19 neutropenic fever, who had received 56 GTX, were included. Seventeen of 56 GTX were splitted and used in maximum 12 hours during infections which did not respond to antibacterial and antifungal therapy in 7 days. The patients were divided in to response groups as a complete, partial and progressive. The predictive factors for response group were evaluated. GTX were well tolerated in all patients. The median granulocyte dose was 1.26 (0.38-5.22) × 10(9)/kg. Total response rate was 89.5%. The infection-related mortality rate was 10.5%. Although the granulocyte doses are the same in both of the product groups, an hour later ANC increment of primer product was higher than that of splitted product (p = 0.001). Among the products, 48.7% of primer product and 17.6% of splitted product had induced ≥ 1000/mm(3) ANC increment after an hour (p = 0.039). Granulocyte transfusion is safe and effective in controlling the febrile neutropenia attack. GTX should be applied in a short time to provide effective ANC increment. For now, main granulocyte product instead of splitted product should be preferred in case of uncontrolled neutropenic fever with antibacterial/antifungal agents.
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Febre/terapia , Granulócitos/transplante , Infecções/terapia , Transfusão de Leucócitos , Neutropenia/terapia , Adolescente , Antibacterianos/administração & dosagem , Antifúngicos/administração & dosagem , Criança , Pré-Escolar , Feminino , Febre/sangue , Febre/mortalidade , Humanos , Lactente , Infecções/sangue , Infecções/mortalidade , Masculino , Neoplasias/sangue , Neoplasias/mortalidade , Neoplasias/terapia , Neutropenia/sangue , Neutropenia/mortalidade , Estudos RetrospectivosRESUMO
ß-Thalassemia (ß-thal) is the most common monogenic disorder in Turkey. The aim of this study was to investigate the spectrum of ß-thal mutations in the Aegean region of Turkey. The data was derived from 1171 unrelated ß-thal subjects, detected in a regional reference hospital between November 2004 and December 2013. Screening for the 22 common mutations was performed using the polymerase chain reaction (PCR)-reverse dot-blot method, and direct automated DNA sequencing for the unknown samples. Thirty-one different ß-thal alleles were identified. Seven mutations, namely IVS-I-110 (G > A) (41.7%), IVS-I-1 (G > A) (8.9%), IVS-II-745 (C > G) (8.6%), codon 8 (-AA) (7.7%), IVS-II-1 (G > A) (7.2%), IVS-I-6 (T > C) (6.6%), codon 39 (C > T) (4.6%) accounted for 85.3% of the mutated alleles. Frequencies of the remaining 24 ß-thal mutations were less than 2.2%; these included one novel mutation [HBB: c.206_212del (p.Leu69Profs*19)], and four others [-56 (G > C), codon 16 (-C), IVS-I (-3) (C > T) (codon 29), codon 76 (-C)] found in Turkey for the first time. The results will help to prevent severe ß-thal through genetic counseling and prenatal diagnosis (PND) in the Aegean region of Turkey.
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Deleção de Sequência , Globinas beta/genética , Talassemia beta/genética , Alelos , Análise Mutacional de DNA , Índices de Eritrócitos , Frequência do Gene , Genótipo , Geografia , Humanos , Turquia , Talassemia beta/sangue , Talassemia beta/diagnósticoRESUMO
Half-and-half nail, characterized by a reddish brown distal band with a sharply demarcated white proximal band, is a specific manifestation of chronic kidney disease, but it is unusual to occur after chemotherapy. We report a seven-year-old girl who developed half-and-half nails in her fingers one month after treatment with modified Berlin-Frankfurt-Munster protocol followed by maintenance therapy with oral methotrexate and 6-mercaptopurine for pre-B acute lymphoblastic leukemia.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Doenças da Unha/induzido quimicamente , Unhas/efeitos dos fármacos , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Asparaginase/administração & dosagem , Asparaginase/efeitos adversos , Asparaginase/uso terapêutico , Criança , Daunorrubicina/administração & dosagem , Daunorrubicina/efeitos adversos , Daunorrubicina/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Doenças da Unha/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamento farmacológico , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Prednisona/uso terapêutico , Vincristina/administração & dosagem , Vincristina/efeitos adversos , Vincristina/uso terapêuticoRESUMO
Pediatric cancer patients have an increased risk of potentially life-threatening fungal infections such as Candida parapsilosis, associated with long-term CVADs. The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related bloodstream infections recommend systemic antifungal therapy and catheter removal. In this study, we focused on our experience with antifungal failure due to totally implanted catheter-associated C. parapsilosis bloodstream infections. We investigated cases leading to port removal in pediatric malignancy patients and the associated patient outcomes. In the first phase of the study, a retrospective chart review was performed to collect patient information, including primary disease; time from hospitalization to port-related candidemia; antifungal drug choice; and the time at which port removal occurred. During the second phase, antifungal susceptibility tests for C. parapsilosis were performed in our microbiology laboratory. All patients had fevers and were neutropenic at the time of candidemia diagnosis. The mean duration between the first isolation of Candida parapsilosis from the port samples to the port removal was 9.75 ± 5.29 days for 11 patients. Patient fevers lasted for a mean time of 16.22 ± 6.51 days. The median recovery duration from fever after CVC removal was four days (range 2-12 days). The median duration for achieving negative blood cultures, following antifungal treatment was 18 days (range 10-27 days). Our data favored the removal of catheters in the presence of ongoing fever, as suggested by the guidelines, independent of the chosen antifungal treatment. Future studies with large samples are needed to evaluate the effects of catheter removal on mortality rates and patient outcomes.
Assuntos
Antifúngicos/administração & dosagem , Proteínas Sanguíneas , Candidíase/tratamento farmacológico , Cateteres Venosos Centrais/efeitos adversos , Tomada de Decisões , Fungemia/tratamento farmacológico , Adolescente , Candidíase/etiologia , Criança , Pré-Escolar , Feminino , Febre/tratamento farmacológico , Fungemia/etiologia , Humanos , Lactente , Masculino , Neoplasias/tratamento farmacológico , Neutropenia/tratamento farmacológicoRESUMO
The aim of the study is to the determine the profiles of cell cycle genes and a new candidate oncogene of URG4/URGCP which play role in leukemia, establishing the association between the early prognosis of cancer and the quantitation of genetic changes, and bringing a molecular approach to definite diagnosis. In this study, 36 newly diagnosed patients' with ALL-AML in the range of 0-18 years and six control group patients' bone marrow samples were included. Total RNA was isolated from samples and then complementary DNA synthesis was performed. The obtained cDNAs have been installed 96 well plates after prepared appropriate mixtures and assessed with LightCycler(®) 480 Real-Time PCR quantitatively. CHEK1, URG4/URGCP, CCNG1, CCNC, CDC16, KRAS, CDKN2D genes in the T-ALL group; CCND2, ATM, CDK8, CHEK1, TP53, CHEK2, CCNG2, CDK4, CDKN2A, E2F4, CCNC, KRAS genes in the precursor B-ALL group and CCND2, CDK6 genes in the AML group have shown significant increase in mRNA expression level. In the featured role of acute leukemia the regulating signaling pathways of leukemogenesis partially defined, although identification of new genetic markers in acute leukemia subgroups, will allow the development of early diagnostic and new treatment protocols.