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Patients with ITP have been reported to experience higher levels of depression and anxiety than their healthy counterparts. The limited research conducted on this subject in the pediatric age group has demonstrated that patients have psychosocial difficulties, and their quality of life is adversely affected. The correlation of depressive symptoms with disease characteristics of cITP has never been investigated. This was a cross-sectional study in patients being treated for cITP. Communication with participants was done during routine outpatient visits or by telephone or e-mail, and a survey about demographics and the Children's Depression Inventory (CDI) and the Social Anxiety Scale for Children-Revised (SAS-CR) was administered prospectively. A total of 56 children with cITP were recruited. The mean CDI score was 17 (SD: ± 9.44). Approximately half of the patients had higher CDI scores than healthy Turkish children. Older age, time since diagnosis, a number of hospitalizations (both total and within the last year) were positively correlated with CDI scores. There was no significant correlation between SAS-CR scores and disease characteristics. Depressive symptom scores were higher in children with cITP compared with healthy children in this study. Psychological needs may be overlooked in the medical management of children with cITP.
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Ansiedad , Depresión , Púrpura Trombocitopénica Idiopática , Humanos , Estudios Transversales , Niño , Depresión/etiología , Depresión/diagnóstico , Masculino , Femenino , Adolescente , Púrpura Trombocitopénica Idiopática/psicología , Púrpura Trombocitopénica Idiopática/diagnóstico , Ansiedad/etiología , Preescolar , Calidad de Vida , Enfermedad CrónicaRESUMEN
We investigated depression and anxiety levels and related psychological disorders in adolescents with transfusion-dependent thalassemia (TDT) in this study. The study was conducted in two pediatric hematology outpatient clinics and included adolescents with TDT (14.8 ± 2.4 years, n = 40) in the study and compared them with the healthy age-matched control group (14.3 ± 2.3 years, n = 62). The Turkish version of the Revised Child Anxiety and Depression Scale (RCADS) was used to determine depression, anxiety, and related psychologic disorders (obsession, panic disorder, social phobia). Depression, anxiety, obsession, panic disorder, and social phobia scores were significantly higher in the patient group compared with the control (all p < 0.05). Ferritin levels were positively correlated with total depression, general anxiety, separation anxiety, and social phobia scores, but transfusion frequency and young age were the confounding factors. Patients in early adolescence and those who require more frequent blood transfusions are at higher risk of developing psychological disorders; routine screening for mood disorders should be warranted. Serum ferritin level may be a good warning indicator for early recognition of psychologic disorders in TDT patients.
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AIM: Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. While cardiac T2* MRI is the gold standard in determining cardiac iron accumulation, it is not always feasible, which has led to the search for new biomarkers. Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major. MATERIALS AND METHOD: Forty-one patients aged 11-21 years and 41 age-, gender-, body mass index-matched healthy controls were included. Serum growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were compared between the patients and controls. Additionally, the relations of these biomarkers with cardiac and liver T2 * MRI were investigated in the patients. RESULTS: In the patients, growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were higher than healthy controls (p < 0.001, p = 0.025, p < 0.001, respectively). There were no significant correlations of growth differentiation factor-15 and N-terminal pro-B-type natriuretic peptide levels with both cardiac and liver T2 * MRI measurements. While there was no significant correlation of serum galectin-3 with cardiac T2 * MRI measurements, a negative correlation was found with liver T2 * MRI measurements (p = 0.040, rho = -0.325). CONCLUSION: All three biomarkers investigated in this study failed to predict myocardial iron accumulation in asymptomatic children with beta-thalassemia major. However, a weak relation between serum galectin-3 level and hepatic iron accumulation was demonstrated.
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Sobrecarga de Hierro , Talasemia beta , Humanos , Niño , Talasemia beta/complicaciones , Péptido Natriurético Encefálico , Galectina 3 , Sobrecarga de Hierro/diagnóstico , Sobrecarga de Hierro/etiología , Miocardio , Imagen por Resonancia Magnética , Hígado , Biomarcadores , Hierro , Factores de Diferenciación de CrecimientoRESUMEN
AIM: This study aimed to evaluate the role of real-time three-dimensional (four-dimensional) and speckle tracking echocardiography for early detection of left ventricular systolic dysfunction and also for the relationship between myocardial deformation parameters and myocardial iron load which is measured by cardiac magnetic resonance relaxation time T2* values in asymptomatic children with beta-thalassemia major. MATERIAL AND METHODS: This multicenter cross-sectional study included 40 patients (mean age 15.4 ± 2.9, 42.1% male) and 40 healthy children whose age, gender, and body mass index-matched with patients. Each participant underwent conventional echocardiography and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strains; twist; and torsion were measured by real-time three-dimensional and speckle tracking echocardiography. Cardiac magnetic resonance imaging T2* was measured in patients. RESULTS: Left ventricular global longitudinal, circumferential, and radial strains were decreased despite preserved global ventricular function in patients compared to healthy children (p = p = .029, p = p < .001, p = .003, respectively). There were no statistically significant differences between patients with T2* ≥ 20 ms and patients with T2* < 20 ms for all echocardiographic parameters. Also, there were no significant correlations between all echocardiographic parameters and T2* values in all patients, those with T2* ≥ 20 ms, and T2* < 20 ms. CONCLUSION: We found that even in asymptomatic children with beta-thalassemia major, left ventricular longitudinal, circumferential and, radial functions were impaired by real-time three-dimensional (four-dimensional) and speckle tracking echocardiography. This novel echocardiographic method might be an important tool for detecting subclinical left ventricular systolic dysfunction irrespective of T2* values.
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Ecocardiografía Tridimensional , Disfunción Ventricular Izquierda , Talasemia beta , Niño , Humanos , Masculino , Femenino , Función Ventricular Izquierda , Talasemia beta/complicaciones , Volumen Sistólico , Estudios Transversales , Ecocardiografía/métodos , Disfunción Ventricular Izquierda/complicaciones , Disfunción Ventricular Izquierda/diagnóstico por imagen , Ecocardiografía Tridimensional/métodosRESUMEN
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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Infecciones Fúngicas del Sistema Nervioso Central , Trasplante de Células Madre Hematopoyéticas , Infecciones Fúngicas Invasoras , Leucemia , Niño , Humanos , Estudios Retrospectivos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trasplante de Células Madre Hematopoyéticas/métodos , Infecciones Fúngicas Invasoras/diagnóstico , Infecciones Fúngicas Invasoras/tratamiento farmacológico , Infecciones Fúngicas Invasoras/etiología , Infecciones Fúngicas del Sistema Nervioso Central/diagnóstico , Infecciones Fúngicas del Sistema Nervioso Central/terapia , Antifúngicos/uso terapéutico , Leucemia/tratamiento farmacológicoRESUMEN
Introduction: Obesity is a gradually more important multifactorial disease in both children and adults. Obese children and adolescents are at higher risk of becoming obese in adulthood, which is associated with an increased risk of mortality and morbidity. There is subclinical systemic inflammation in obesity. The study aimed to evaluate the hematological parameters as an indicator of inflammation in obese adolescents and to show the relationship of monocyte/HDL-cholesterol ratio, having a lipid component, with other inflammatory hematological parameters. Materials and methods: We retrospectively reviewed the medical files of 60 patients, 30 obese and 30 healthy controls, aged between 11 and 16 years, who applied to the pediatric outpatient clinic. Laboratory tests, hematological parameters, gender, age, and BMI were compared between the groups. Correlations between monocyte/HDL-cholesterol ratio and other laboratory parameters in the obese group were examined. Results: BMI, Alanine aminotransferase (ALT), C-reactive protein (CRP), triglyceride, insulin, and HOMA-IR levels of the obese adolescent group were statistically significantly higher than the control group (p<0.05). There was no statistically significant difference between the obese and control groups in terms of inflammatory hematological ratios (NLR, PLR, MLR, and monocyte/HDL-cholesterol ratio) (p>0.05). There was no statistically significant relationship between monocyte/HDL-cholesterol and other inflammatory hematological rates (p>0.05). There was a positive, moderate (48.6%), and statistically significant relationship between monocyte/HDL-cholesterol and MLR (p<0.05). Conclusions: In our study, the NLR, PLR, MLR, and monocyte/HDL-cholesterol values of the obese adolescent group were similar to the control group. There was correlation between monocyte/HDL-cholesterol and monocyte/lymphocyte values. There was no correlation between other rates (AU)
Introducción: La obesidad es una enfermedad multifactorial cada vez más importante tanto en niños como en adultos. Los niños y adolescentes obesos tienen un mayor riesgo de volverse obesos en la edad adulta, lo que se asocia con un mayor riesgo de mortalidad y morbilidad. Hay inflamación sistémica subclínica en la obesidad. El estudio tuvo como objetivo evaluar los parámetros hematológicos como indicador de inflamación en adolescentes obesos y mostrar la relación de la relación monocitos/colesterol HDL, que tiene un componente lipídico, con otros parámetros hematológicos inflamatorios. Materiales y métodos: Se revisaron retrospectivamente los expedientes médicos de 60 pacientes, 30 obesos y 30 controles sanos, con edades comprendidas entre los 11 y los 16 años, que acudieron a la consulta externa de pediatría. Se compararon las pruebas de laboratorio, los parámetros hematológicos, el sexo, la edad y el IMC entre los grupos. Se examinaron las correlaciones entre la proporción de monocitos/colesterol HDL y otros parámetros de laboratorio en el grupo de obesos. Resultados: Los niveles de IMC, alanina aminotransferasa (ALT), proteína C reactiva (PCR), triglicéridos, insulina y HOMA-IR del grupo de adolescentes obesos fueron estadísticamente significativamente más altos que los del grupo de control (p<0.05). No hubo diferencias estadísticamente significativas entre los grupos de obesidad y de control en términos de proporciones hematológicas inflamatorias (NLR, PLR, MLR y proporción de monocitos/colesterol HDL) (p>0,05). No hubo una relación estadísticamente significativa entre monocitos/colesterol HDL y otras tasas hematológicas inflamatorias (p>0,05). Hubo una relación positiva, moderada (48,6%) y estadísticamente significativa entre monocitos/colesterol HDL y MLR (p <0,05). Conclusiones: En nuestro estudio, los valores de NLR, PLR, MLR y monocitos/colesterol HDL del grupo de adolescentes obesos fueron similares a los del grupo control (AU)
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Humanos , Masculino , Femenino , Niño , Adolescente , Obesidad/sangre , HDL-Colesterol/sangre , Recuento de Células Sanguíneas , Estudios Retrospectivos , Biomarcadores/sangreRESUMEN
The longer diagnostic intervals in low- and middle-income countries have been proposed among the possible causes of poorer outcomes in children with cancer. In this single-center study from Turkey, the diagnostic intervals and survival status of 138 children with solid tumors and lymphoma (excluding leukemia) were prospectively evaluated. The median total interval (from the beginning of the first cancer-related symptom to the first day of the cancer-specific therapy), the median patient interval (the time interval from the notification of the first cancer-related symptom to the first admission to a healthcare facility), and the median physician interval (the time interval between the first healthcare admission to the first pediatric oncology visit) were 65, 26, and 24 days, respectively. The estimated 5-year overall survival and event-free survival rates were 80.7% and 69.1%, respectively. The longer time intervals were correlated with age, paternal education, localization, and tumor type. Interestingly, none of the time parameters were found to be associated with survival on regression analysis. In conclusion, the diagnostic delay in children with cancer is multifactorial, and the patient- and disease-related factors are as important as the time intervals on survival.Supplemental data for this article is available online at https://doi.org/10.1080/08880018.2021.1951903.
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Linfoma , Neoplasias , Niño , Diagnóstico Tardío , Humanos , Linfoma/diagnóstico , Linfoma/terapia , Neoplasias/diagnóstico , Neoplasias/terapia , Factores de Tiempo , Turquía/epidemiologíaRESUMEN
The concepts of games and toys have a very important role in children's lives. It contributes to the development of cognitive, motor, psychosocial, emotional, and linguistic skills. It also plays a key role in raising self-confident, creative, and happy children. Therefore, attention should be paid to the concepts of games and toys, which are so important for the child to be a part of society as a healthy individual at every stage of his development. On the other hand, providing playgrounds where children can play comfortably and safely are essential in reducing the risk of accidents related to toys. All health-care components, especially pediatricians and family physicians, should take an active role in ensuring that these play processes, which are the most beautiful parts of childhood, are healthy and safe.
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Introduction: There are very few studies on the effects of regular blood transfusions on the hemodynamic organization of patients with Beta-Thalassemia Major (BTM). Ambulatory Blood Pressure Monitoring is method that evaluates fluctuations in day-night periods and life cycle changes. In this study, we aimed to investigate the effects of blood transfusion on hemodynamic parameters by the Ambulatory Blood Pressure Monitoring method on the day of transfusion in patients with a diagnosis of Beta-Thalassemia Major. Material and Methods: This study was conducted in patients who were followed up with a diagnosis of BTM between June 2020 and July 2020. The study consisted of 30 patients. The blood pressure of the patients was measured by auscultation method on the morning of the day when they received routine red blood cell transfusion treatments, and the patients were fitted with an Ambulatory Blood Pressure Monitoring device. With Ambulatory Blood Pressure Monitoring, mean systolic blood pressure, diastolic blood pressure, heart rate, mean arterial pressure, values were calculated for each patient. Statistical analysis was performed by the IBM SPSS Statistics 21 package program. The significance limit for the p-value was accepted as <0.05. Results: There was a significant difference in mean systolic blood pressure and heart rate values between pre-transfusion, transfusion, and post-transfusion periods. In our study, the rate of white coat hypertension was 8.3%, and the rate of masked hypertension was 4.1%. It was observed that 67% of the patients were non-dippers, and the blood pressure burden of one patient was more than 25%. Conclusions: Measurement of hemodynamic parameters with Ambulatory Blood Pressure Monitoring is the gold standard in terms of detection and follow-up of non-dipper patients, indicating increased cardiovascular risk. In practice, Ambulatory Blood Pressure Monitoring should be used more in the follow-up of chronic patients.(AU)
Introducción: Existen muy pocos estudios sobre los efectos de las transfusiones de sangre periódicas sobre la organización hemodinámica de los pacientes con Beta-Talasemia Mayor (BTM). La monitorización ambulatoria de la presión arterial es un método que evalúa las fluctuaciones en los períodos diurnos y nocturnos y los cambios en el ciclo de vida. En este estudio, nuestro objetivo fue investigar los efectos de la transfusión de sangre sobre los parámetros hemodinámicos mediante el método de Monitoreo Ambulatorio de la Presión Arterial el día de la transfusión en pacientes con diagnóstico de Beta-Talasemia Mayor. Material y métodos: Este estudio se realizó en pacientes que fueron seguidos con un diagnóstico de BTM entre junio de 2020 y julio de 2020. El estudio consistió en 30 pacientes. La presión arterial de los pacientes se midió mediante el método de auscultación en la mañana del día en que recibieron los tratamientos de transfusión de glóbulos rojos de manera rutinaria, y los pacientes fueron equipados con un dispositivo de monitoreo ambulatorio de la presión arterial. Con la monitorización ambulatoria de la presión arterial, se calcularon los valores de la presión arterial sistólica media, la presión arterial diastólica, la frecuencia cardíaca y la presión arterial media de cada paciente. El análisis estadístico se realizó mediante el programa de paquete IBM SPSS Statistics 21. El límite de significación para el valor p se aceptó como <0,05. Resultados: Hubo una diferencia significativa en la presión arterial sistólica media y los valores de frecuencia cardíaca entre los períodos de pretransfusión, transfusión y postransfusión. En nuestro estudio, la tasa de hipertensión de bata blanca fue del 8,3% y la tasa de hipertensión enmascarada fue del 4,1%. Se observó que el 67% de los pacientes eran no dippers y la carga de presión arterial de un paciente era superior al 25%.Conclusiones: La medición de parámetros hemodinámicos con monitorización...(AU)
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Humanos , Masculino , Femenino , Presión Arterial , Transfusión Sanguínea , Talasemia beta/diagnóstico , Monitoreo Ambulatorio de la Presión ArterialRESUMEN
PURPOSE: Different and various system complications and late effects may occur after hematopoietic stem cell transplantation (HSCT). It was aimed to obtain information about the frequency of ophthalmologic complications and their relationship with treatment. METHODS: This retrospective study includes 104 children who underwent HSCT between February 2019 and June 2020 at the Pediatric Bone Marrow Transplant Unit. Patients' ages, genders, diagnosis, transplant types, chemotherapy regimens, transplantation details, conditioning regimens, supportive cares, graft versus host disease (GvHD) prophylaxis, infection episodes, and ophthalmologic findings were evaluated. RESULTS: Of the 104 patients included in the study, 38 (36.5%) were female and 66 (63.5%) were male. Average age ± SD was 8.7 ± 4.91. Considering the diagnoses, the majority of the patients were acute lymphoblastic leukemia (46 patients-44%). Myeloablative regimen was used in 93 (89%) of the patients, and reduced intensity conditioning (RIC) was used in 11 patients (10%). While total body irradiation was applied in 16 (15%) patients, one patient was received cranial radiotherapy. Cyclosporine was used in 96 (92%) patients. CMV reactivation was detected in 54 (51%) of the patients. CMV retinitis was not seen. Ocular pathology was detected in 20 (19%) patients before HSCT and in 12 (11%) patients after HSCT. The most common pathology was dry eye. CONCLUSION: Routine ophthalmologic examinations are important in terms of early diagnosis. In addition, GvHD and CMV prophylaxis is important because of reducing the risk of ocular complications after HSCT.
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Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Niño , Ciclosporina , Femenino , Enfermedad Injerto contra Huésped/etiología , Enfermedad Injerto contra Huésped/prevención & control , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Masculino , Estudios Retrospectivos , Acondicionamiento Pretrasplante/efectos adversosRESUMEN
INTRODUCTION: Angiopoietin-like peptide 4 (ANGPTL-4) is an adipocytokine that regulates plasma lipoprotein levels by inhibiting the lipoprotein lipase enzyme. Changes in lipid profile can be seen in obese adolescents. Nonalcoholic fatty liver disease may also be a complication of obesity. Based on this information, in this study we aimed to evaluate the relationship between serum ANGPTL-4 levels and obesity and hepatosteatosis in adolescents. MATERIALS AND METHODS: A total of 85 volunteer adolescents, 55 of them were obese and 30 of them were normal weight, were included in our study. The adolescents having body mass index (BMI) 95% percentile and over according to age and sex was defined as obese. Thirty patients with grade 2-3 hepatosteatosis in abdominal ultrasound (USG) were included in 'obese adolescents with hepatosteatosis' subgroup and 25 obese cases with no hepatosteatosis in the USG were included in the 'obese adolescents without hepatosteatosis' group. Thirty patients with no hepatosteatosis in the abdominal USG and having BMI in normal percentiles according to their age and sex constituted the 'healthy control adolescents' group. Serum ANGPTL-4 levels were measured by Enzyme Linked Immunosorbent Assay. Laboratory tests, gender, age and BMI levels were compared statistically between groups. Correlations between ANGPTL-4 and other laboratory parameters were examined statistically in obese adolescent group. RESULTS: The BMI, ANGPTL-4, HbA1c, AST, ALT, total cholesterol, triglyceride, LDL-cholesterol, HOMA-IR and insulin levels of the obese adolescent group were found to be significantly higher than the healthy control group (pâ¯<â¯0.05). We found no statistically significant difference in BMI, ANGPTL-4, triglyceride, insulin and HOMA-IR levels among obese adolescents with or without hepatosteatosis (pâ¯>â¯0.05). In all obese adolescent groups and in obese adolescent group with hepatosteatosis; there was no statistically significant relationship between ANGPTL-4 and other variables (pâ¯>â¯0.05). CONCLUSIONS: We found that the levels of ANGPTL-4 increases in obesity in adolescents. However, our results make it difficult to establish a relationship between hepatosteatosis and ANGPTL-4. Targeting ANGPTL-4 may be beneficial for the pathogenesis and associated complications of obesity.
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Proteína 4 Similar a la Angiopoyetina/sangre , Hígado Graso/sangre , Obesidad/sangre , Obesidad/complicaciones , Adolescente , Índice de Masa Corporal , Niño , Correlación de Datos , Hígado Graso/complicaciones , Hígado Graso/diagnóstico por imagen , Hígado Graso/metabolismo , Femenino , Humanos , Masculino , UltrasonografíaRESUMEN
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 de Células Falciformes/complicaciones , Deferasirox/uso terapéutico , Quelantes del Hierro/uso terapéutico , Sobrecarga de Hierro/tratamiento farmacológico , Sobrecarga de Hierro/etiología , Talasemia/complicaciones , Adolescente , Anemia de Células Falciformes/terapia , Biomarcadores , Transfusión Sanguínea , Niño , Preescolar , Estudios de Cohortes , Deferasirox/administración & dosificación , Deferasirox/efectos adversos , Femenino , Ferritinas/sangre , Ferritinas/metabolismo , Humanos , Hierro/sangre , Hierro/metabolismo , Quelantes del Hierro/administración & dosificación , Quelantes del Hierro/efectos adversos , Sobrecarga de Hierro/metabolismo , Masculino , Talasemia/terapia , Resultado del Tratamiento , TurquíaRESUMEN
B-lineage acute lymphoblastic leukemia (B-ALL) is a common subtype of acute leukemia in children. PAX5 plays a central role in B-cell development and differentiation. In this study, we analyzed PAX5 expression levels, transactivation domain mutations/deletions in B-ALL patients (n=115) and healthy controls (n=10). Relative PAX5 mRNA levels were significantly increased in B-ALL patients (p<0.0001). PAX5 expression was also evaluated in three different B-ALL subgroups (pro B, Common B and Pre B ALL) and showed stage specific expression levels. Pro B (p=0.04) and pre B (p=0.04) patients showed significantly high PAX5 mRNA levels compared to stage specific controls. At least one deletion of exons 7-8 or 9 has been identified in the 41% of the patients. CD34 positivity in patients and presence of large deletions (Δ7/8/9) showed a significant correlation (p=0.05). None of our patients showed PAX5 point mutations, but two previously identified SNPs (rs3780135 and rs35469494) were detected. Our results support that PAX5 is a critical factor in B-ALL development and aberrant PAX5 expression especially at early stages may leads to leukemic transformation.
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Linfocitos B/metabolismo , Factor de Transcripción PAX5/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras B/genética , Linfocitos B/patología , Línea Celular , Transformación Celular Neoplásica/genética , Transformación Celular Neoplásica/patología , Niño , Preescolar , Estudios de Cohortes , Femenino , Regulación Leucémica de la Expresión Génica , Humanos , Lactante , Masculino , Mutación , Estadificación de Neoplasias , Factor de Transcripción PAX5/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras B/inmunología , Leucemia-Linfoma Linfoblástico de Células Precursoras B/patología , Isoformas de Proteínas/genética , Isoformas de Proteínas/metabolismoRESUMEN
OBJECTIVE: We aimed to assess the role of Magnetic Resonance Imaging (MRI) and X-Ray in the evaluation of response to radiosynovectomy (RS) in patients with hemophilic arthropathy. MATERIAL AND METHODS: Eleven patients who suffered from hemophilic arthropathy with a mean age of 11.7 (range between 7-15) were included in this study. 148-185 MBq Yttrium 90 silicate (Y-90) was administered intraarticularly to ten knee joints and one patient was treated with intraarticular 74 MBq Rhenium 186 (Re-186) injection into his ankle. Before radiosynovectomy, plain anteroposterior and lateral X-rays of the target joints were obtained by standard technique. The follow-up MRI and X-ray studies of the patients were done 6 months after RS. Pettersson hemophilic arthropathy scales were utilized to stage the condition of the joints on plain X-ray and classification of the investigated joints on MRI were done according to Denver score. The clinical assessment of the efficacy of the RS was made with the comparison of the average bleedings before and after the intervention. RESULTS: During the 6-month follow-up period after RS, an improvement in number of hemarthrosis 75% or greater compared with the prior six months occurred in six joints (54.5%). The Pettersson scores worsened in 1/11 (9%), remained unchanged in 9/11 (81.8%), and improved in 1/11 (9%) joints. At the 6-month follow-up, the MRI score worsened in one (9%) and was unchanged in 10/11 joints (90.9%). CONCLUSION: MRI is a more sensitive tool than plain radiography for evaluating and follow-up of joint disease in persons with hemophilia, but both methods don't show correlation with the therapeutic response Conflict of interest:None declared.
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Hereditary spherocytosis is a familial hemolytic anemia. Immunoglobulin A (IgA) nephropathy associated with hereditary spherocytosis has not been reported in children. Here, we report a case of a 17-year-old boy with IgA nephropathy and hereditary spherocytosis. The patient was diagnosed with hereditary spherocytosis at the age of 12 years and splenectomy was done at the age of 15 years. Later, the patient presented with macroscopic hematuria and proteinuria. Kidney biopsy of the boy was consistent with IgA nephropathy. Treatment with angiotensin-converting enzyme inhibitor was started. The patient became free of proteinuria after the 6th month of therapy.
Asunto(s)
Glomerulonefritis por IGA/complicaciones , Hematuria/etiología , Proteinuria/etiología , Esferocitosis Hereditaria/complicaciones , Adolescente , Biopsia , Glomerulonefritis por IGA/patología , Hematuria/patología , Humanos , Riñón/patología , Masculino , Proteinuria/patologíaRESUMEN
BACKGROUND: Granisetron is a safe and effective prophylaxis for nausea and vomiting associated with moderate to highly emetogenic chemotherapy. Few trials have been conducted to determine the optimal effective dose of granisetron in children with cancer. The objective of this report was to compare two doses of granisetron in patients with optic pathway tumors receiving moderately emetogenic doses of carboplatin. PATIENTS AND METHODS: In this double-blind, crossover, randomized study, antiemetic efficacy and tolerability of two dose levels (10 and 40 microg/kg) of granisetron in the prevention of acute and delayed nausea/emesis were compared in children and young adults. A total of 18 patients (13 boys) aged 1-23 years (median 7.7 years) treated with a moderately emetogenic dose of carboplatin were randomly assigned to receive either 10 or 40 microg/kg of slow granisetron intravenous (i.v.) infusions at alternating cycles of chemotherapy in a blinded fashion until the end of the study period or until their chemotherapy regimen ended. In this way, the patients acted as their own controls. RESULTS: Patients in the granisetron 10 and 40 microg/kg groups received 104 and 121 cycles of chemotherapy, respectively. There was no significant difference in antiemetic efficacy in terms of nausea and emesis between the dose groups in the first 5 days of chemotherapy. The treatment was well tolerated. CONCLUSION: We conclude that granisetron 10 and 40 microg/kg have comparable efficacy in controlling carboplatin-induced acute and delayed nausea/emesis and is well tolerated in children and young adults.
Asunto(s)
Antieméticos/administración & dosificación , Antineoplásicos/efectos adversos , Carboplatino/efectos adversos , Relación Dosis-Respuesta a Droga , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Granisetrón/administración & dosificación , Náusea/prevención & control , Vómitos/prevención & control , Adolescente , Adulto , Antieméticos/farmacología , Antineoplásicos/uso terapéutico , Carboplatino/uso terapéutico , Niño , Preescolar , Estudios Cruzados , Interacción de Doble Vínculo , Femenino , Granisetrón/farmacología , Humanos , Lactante , Masculino , Náusea/inducido químicamente , Náusea/tratamiento farmacológico , Resultado del Tratamiento , Vómitos/inducido químicamente , Vómitos/tratamiento farmacológicoRESUMEN
Helicobacter pylori infection is a common etiopathogenetic factor in children with gastrointestinal symptoms in the developing world. Although serology offers an easy noninvasive method of diagnosis, its sensitivity and specificity are reported to be low among children. In this prospective study, we investigated the frequency and endoscopical and morphological findings of H. pylori infection in 180 Turkish children who underwent upper gastrointestinal endoscopy either for peptic symptoms or on a routine basis and in asymptomatic pediatric patients who underwent endoscopy for other reasons, and then evaluated the diagnostic accuracy of serology in our population. Overall H. pylori infection was diagnosed in 77 of the 180 patients (42.7%) by histology and urease test. The sensitivity of H. pylori specific IgG antibody assay by ELISA was determined to be 100%, while the specificity was 98%, the positive predictive value 97.4%, the negative predictive value 100%. Frequency of H. pylori infection is high in Turkish pediatric patients without gastrointestinal symptoms as well as in children with gastrointestinal complaints. H. pylori specific antibody assay is a noninvasive and sensitive method for the diagnosis of H. pylori infection in the Turkish pediatric population.