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1.
Pediatr Transplant ; 28(3): e14758, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38659218

RESUMEN

BACKGROUND: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. OBJECTIVES: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection. METHOD: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022. RESULTS: The median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality. CONCLUSION: While COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD.


Asunto(s)
COVID-19 , Trasplante de Células Madre Hematopoyéticas , Humanos , COVID-19/epidemiología , COVID-19/terapia , COVID-19/mortalidad , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Niño , Masculino , Femenino , Estudios Retrospectivos , Adolescente , Turquía/epidemiología , Preescolar , Factores de Riesgo , SARS-CoV-2 , Lactante , Trasplante Homólogo , Índice de Severidad de la Enfermedad
2.
Pediatr Transplant ; 26(8): e14397, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36207767

RESUMEN

BACKGROUND: Refugee or asylum seekers (RAS) children are at increased risk of physical, developmental, and behavioral health issues. The aim of this study was to evaluate clinical and psychosocial outcomes of hematopoietic stem cell transplantation (HSCT) in RAS children and compare health-related quality of life (HRQOL) to those of Turkish peers. METHODS: This retrospective study included patients who underwent HSCT aged 0-18 years and completed 100-day post-transplant. The PedsQL 4.0 Generic Core Scale was used in children over 5 years old to compare HRQOL. RESULTS: A total of 166 RAS patients (M/F: 106 /60) underwent 174 HSCTs (six patients had two, and one had three HSCT) compared to 66 Turkish patients. The mean age of the patients in the RAS group was 7.8 ± 4.9 years and similar to controls. A total of 124 patients (75%) were from Syria, and 49 (25%) were from other countries in the Middle East and Africa. The cause of migration was war in 121 (74%) RAS patients. Complications of HSCT were no different between the groups. However, the rate of neutropenic sepsis was significantly higher in the RAS group (p = 0.004). The total scores of HRQOL were not different between RAS and controls. In the RAS group, ratings of social functioning were lower in patients with consanguinity or non-malignant disease or who had match-related donors. DISCUSSION: Identifying areas of difficulty in subscales of HRQOL may help physicians to classify patients who need additional supportive care. Regular monitoring and supporting physical needs may result in better functional outcomes after HSCT.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Refugiados , Humanos , Niño , Preescolar , Calidad de Vida/psicología , Turquía , Estudios Retrospectivos , Trasplante de Células Madre Hematopoyéticas/psicología
3.
Pediatr Transplant ; 25(5): e13942, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-33320995

RESUMEN

BACKGROUND: Post-transplant relapse has a dismal prognosis in children with acute leukemia undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Data on risk factors, treatment options, and outcomes are limited. PROCEDURE: In this retrospective multicenter study in which a questionnaire was sent to all pediatric transplant centers reporting relapse after allo-HSCT for a cohort of 938 children with acute leukemia, we analyzed 255 children with relapse of acute leukemia after their first allo-HSCT. RESULTS: The median interval from transplantation to relapse was 180 days, and the median follow-up from relapse to the last follow-up was 1844 days. The 3-year overall survival (OS) rate was 12.0%. The main cause of death was disease progression or subsequent relapse (82.6%). The majority of children received salvage treatment with curative intent without a second HSCT (67.8%), 22.0% of children underwent a second allo-HSCT, and 10.2% received palliative therapy. Isolated extramedullary relapse (hazard ratio (HR): 0.607, P = .011) and relapse earlier than 365 days post-transplantation (HR: 2.101, P < .001 for 0-180 days; HR: 1.522, P = .041 for 181-365 days) were found in multivariate analysis to be significant prognostic factors for outcome. The type of salvage therapy in chemosensitive relapse was identified as a significant prognostic factor for OS. CONCLUSION: A salvage approach with curative intent may be considered for patients with post-transplant relapse, even if they relapse in the first year post-transplantation. For sustainable remission, a second allo-HSCT may be recommended for patients who achieve complete remission after reinduction treatment.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Leucemia/mortalidad , Leucemia/terapia , Enfermedad Aguda , Adolescente , Niño , Preescolar , Terapia Combinada , Femenino , Estudios de Seguimiento , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Lactante , Recién Nacido , Leucemia/diagnóstico , Masculino , Pronóstico , Recurrencia , Estudios Retrospectivos , Terapia Recuperativa , Análisis de Supervivencia , Trasplante Homólogo , Turquía/epidemiología , Adulto Joven
4.
J Clin Immunol ; 39(3): 316-323, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30924026

RESUMEN

Severe combined immunodeficiency (SCID) is the most serious PID, characterized by T cell lymphopenia and lack of antigen-specific T cell and B cell immune responses, inevitably leading to death within the first year of life if hematopoietic stem cell transplantation (HSCT) is not performed. PURPOSE AND METHODS: Since SCID is a common type of PID with an estimated incidence of 1/10.000 in Turkey, a retrospective analysis of HSCT characteristics, survival, immune recovery, and the major clinical features of SCID prior to HSCT is the aim of this multi-transplant center-based analysis. RESULTS: A total of 234 SCID patients transplanted between the years 1994 and 2014 were included in the study. Median age at diagnosis was 5 months, at transplantation, 7 months, B- phenotype and RAGs were the most common defects among others. Immune phenotype did not seem to have an effect on survival rate (p > 0.05), Immunoglobulin (Ig) requirement following HSCT did not differ between B+ and B- phenotypes (p > 0.05). Overall survival rate was 65.7% over a period of 20 years. It increased from 54% (1994-2004) to 69% (p = 0.052) during the last 10 years (2005-2014). Ten-year survival after HSCT has improved over time although the difference was not significant. Infection at the time of transplantation (p = 0.006), mismatched related donor (MMRD) (haploidentical parents), and matched unrelated donor (MUD) donor transplants p < 0.001 were the most important factors, significantly affecting the outcome. CONCLUSIONS: This is the first multicenter study with the largest data obtained from transplanted SCID patients in Turkey. Early diagnosis with newborn screening (NBS) together with emerging referrals, treatment by transplantation centers, and specialized teams are mandatory in countries with high parental consanguinity such as Turkey.


Asunto(s)
Linfocitos B/inmunología , Enfermedad Injerto contra Huésped/epidemiología , Trasplante de Células Madre Hematopoyéticas , Inmunodeficiencia Combinada Grave/terapia , Femenino , Histocompatibilidad , Humanos , Tolerancia Inmunológica , Lactante , Masculino , Factores de Riesgo , Inmunodeficiencia Combinada Grave/epidemiología , Inmunodeficiencia Combinada Grave/mortalidad , Análisis de Supervivencia , Resultado del Tratamiento , Turquía/epidemiología
5.
Med Mycol ; 57(2): 161-170, 2019 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-29608706

RESUMEN

Invasive fungal infections (IFIs) are a major cause of infection-related morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT). Data from pediatric settings are scarce. To determine the incidence, risk factors and outcomes of IFIs in a 180-day period post-transplantation, 408 pediatric patients who underwent allogeneic HSCT were retrospectively analyzed. The study included only proven and probable IFIs. The cumulative incidences of IFI were 2.7%, 5.0%, and 6.5% at 30, 100, and 180 days post-transplantation, respectively. According to the multivariate analysis, the factors associated with increased IFI risk in the 180-day period post-HSCT were previous HSCT history (hazard ratio [HR], 4.57; 95% confidence interval [CI] 1.42-14.71; P = .011), use of anti-thymocyte globulin (ATG) (HR, 2.94; 95% CI 1.27-6.80; P = .012), grade III-IV acute graft-versus-host-disease (GVHD) (HR, 2.91; 95% CI 1.24-6.80; P = .014) and late or no lymphocyte engraftment (HR, 2.71; 95% CI 1.30-5.62; P = .007). CMV reactivation was marginally associated with an increased risk of IFI development (HR, 1.91; 95% CI 0.97-3.74; P = .063). IFI-related mortality was 1.5%, and case fatality rate was 27.0%.The close monitoring of IFIs in pediatric patients with severe acute GVHD who receive ATG during conditioning is critical to reduce morbidity and mortality after allogeneic HSCT, particularly among those with prior HSCT and no or late lymphocyte engraftment.


Asunto(s)
Profilaxis Antibiótica , Fluconazol/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Infecciones Fúngicas Invasoras/epidemiología , Infecciones Fúngicas Invasoras/prevención & control , Adolescente , Profilaxis Antibiótica/normas , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Infecciones Fúngicas Invasoras/tratamiento farmacológico , Infecciones Fúngicas Invasoras/mortalidad , Masculino , Estudios Retrospectivos , Factores de Riesgo , Análisis de Supervivencia , Trasplante Homólogo , Turquía/epidemiología
6.
Biol Blood Marrow Transplant ; 23(5): 790-794, 2017 May.
Artículo en Inglés | MEDLINE | ID: mdl-28192253

RESUMEN

Preimplantation genetic diagnosis involves the diagnosis of a genetic disorder in embryos obtained through in vitro fertilization, selection of healthy embryos, and transfer of the embryos to the mother's uterus. Preimplantation genetic diagnosis has been used not only to avoid the risk of having an affected child, but it also offers, using HLA matching, preselection of potential HLA-genoidentical healthy donor progeny for an affected sibling who requires bone marrow transplantation. Here, we share the hematopoietic stem cell transplantation results of 52 patients with different benign and malign hematological or metabolic diseases or immunodeficiencies whose donors were siblings born with this technique in Turkey since 2008. The median age of the patients' at the time of the transplantation was 8 years (range, 3 to 16 years) and the median age of the donors was 2 years (range, .5 to 6 years). The most common indication for HSCT was thalassemia major (42 of all patients, 80%). The stem cell source in all of the transplantations was bone marrow. In 37 of the transplantations, umbilical cord blood of the same donor was also used. In 50 of the 52 patients, full engraftment was achieved with a mean of 4.6 × 106 CD 34+ cells per kg of recipient weight. Ninety-six percent of the patients have been cured through hematopoietic stem cell transplantation without any complication. Primary engraftment failure was seen in only 2 patients with thalassemia major. All of the donors and the patients are alive with good health status. Preimplantation genetic diagnosis with HLA matching offers a life-saving chance for patients who need transplantation but lack an HLA genoidentical donor.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas/métodos , Prueba de Histocompatibilidad/métodos , Diagnóstico Preimplantación , Talasemia beta/terapia , Adolescente , Trasplante de Médula Ósea , Niño , Preescolar , Trasplante de Células Madre de Sangre del Cordón Umbilical , Femenino , Supervivencia de Injerto , Antígenos HLA/análisis , Humanos , Embarazo , Hermanos , Donantes de Tejidos
7.
Transfus Apher Sci ; 56(4): 539-543, 2017 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-28818403

RESUMEN

OBJECTIVES: Iron is taken into enterocytes at the duodenum via apical divalent metal-ion transporter 1 protein. Besides iron, divalent metal-ion transporter 1 also transports other divalent metals. We aimed to investigate blood heavy metal levels in patients with ineffective erythropoiesis. METHODS: Blood levels of heavy metals including Pb, Al, Cd, Cr, Co, Cu, and Zn were measured in patients with thalassemia major (TM), thalassemia intermedia (TI), congenital dyserythropoietic anemia (CDA), and age- and sex-matched healthy controls. RESULTS: Blood samples were obtained from 68 patients (51 patients with TM, 8 with TI, 9 with CDA), and a control group that included 65 volunteers. Patients with TM were found to have lower Al, Pb, and Zn, and higher Cd levels compared with the control group. The patients treated with deferasirox were further analyzed and Pb and Zn levels were found lower compared with the control group. DISCUSSION: Patients with TM had tendency to have elevated levels of plasma cadmium; however, the median level was not at a toxic level. Increased metal-ion transporter 1 activity may cause heavy metal accumulation, but deferasirox chelation may be protective against heavy metals besides iron.


Asunto(s)
Anemia Diseritropoyética Congénita/sangre , Eritropoyesis , Metales Pesados/sangre , Talasemia beta/sangre , Adolescente , Adulto , Anemia Diseritropoyética Congénita/tratamiento farmacológico , Benzoatos/administración & dosificación , Niño , Preescolar , Deferasirox , Femenino , Humanos , Masculino , Triazoles/administración & dosificación , Talasemia beta/tratamiento farmacológico
8.
Helicobacter ; 21(1): 35-9, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25982543

RESUMEN

BACKGROUND: The purpose of this study was to evaluate the association between vitamin B12 levels and Helicobacter Pylori infection and to examine the clinical usefulness of holotranscobalamin (holoTC) measurement in children. MATERIALS AND METHODS: Thirty patients between 6 and 15 years of age, who were diagnosed as H. pylori infected by C(14) urea breath test, and 26 controls were enrolled in the study. Tests for complete blood count, serum vitamin B12 and folate, plasma total homocysteine, and holoTC levels were performed in each patient in the study and control groups. RESULTS: Mean plasma holoTC concentrations were significantly lower in children with H. pylori infection before treatment (median 23.7 pmol/L (12.9-37.1 pmol/L)) versus after treatment (median 38.2 pmol/L (21.2-61.4 pmol/L)) and controls (median 36.1 pmol/L (12.6-58.7 pmol/L)). CONCLUSIONS: The findings of our study suggest that H. pylori infection has a reversible negative effect on vitamin B12 status reflected in a decreased level of plasma holoTC that normalizes upon treatment of the infection, while no change is observed in total plasma vitamin B12 .


Asunto(s)
Infecciones por Helicobacter/sangre , Helicobacter pylori/fisiología , Vitamina B 12/sangre , Adolescente , Niño , Femenino , Ácido Fólico/sangre , Infecciones por Helicobacter/microbiología , Humanos , Masculino
9.
Transfus Apher Sci ; 55(3): 323-328, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27743710

RESUMEN

BACKGROUND: Platelet suspensions (PSs) are stored at room temperature. However, recent reports show that PSs stored at 4 °C possess superior hemostatic properties. We compared the viabilities and thrombin generation capacities of PSs stored either at 4 °C or 22 °C hours. MATERIALS AND METHODS: Twenty units of apheresis derived platelets (ADPs) from 20 male donors and 20 units of random platelet suspensions (RPSs) from another 20 male donors were obtained. Half of the ADPs and half of the RPSs (10 units/per group) were stored at 4 °C, the other halves of ADPs and RPSs (10 units/per group) were stored in agitators at 22 °C for 48 hours. The flow cytometric viability tests and thrombin generation tests of the PSs were assessed. RESULTS: The viabilities of both ADPs and RPSs group platelets, stored either at 4 °C or 22 °C for 48 hours, were not statistically significantly different. The ADPs and RPSs stored at 4 °C generated significantly higher peak thrombin levels than the platelets stored at 22 °C. Moreover, the ADPs group stored at 4 °C showed significantly shorter time to thrombin generation and reach peak levels. CONCLUSION: The PSs stored at 4 °C showed higher and faster thrombin generation capacities than the room temperature PSs. Given the superior hemostatic properties of refrigerated platelets, creating different storage temperature capabilities for specific transfusion purposes may be a prudent approach, especially for improving the outcome of bleeding trauma casualties.


Asunto(s)
Plaquetas/citología , Conservación de la Sangre/métodos , Antígenos CD/metabolismo , Eliminación de Componentes Sanguíneos , Supervivencia Celular , Humanos , Masculino
10.
Helicobacter ; 20(6): 418-21, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25856798

RESUMEN

BACKGROUND: Anticardiolipin (aCL) antibodies are associated with thrombosis and have an important role in the etiology of diseases such as stroke and myocardial infarction whose etiologies were based on thrombosis. H. pylori has been proposed to be responsible for the pathophysiology of some diseases including stroke, myocardial infarction, thrombosis, and autoimmune diseases. From this point of view, we hypothesized a possible relationship between H. pylori infection and aCL antibodies and initially aimed to determine the prevalence of aCL antibody positivity in children with H. pylori infection. MATERIALS AND METHODS: Anticardiolipin antibodies were studied in 84 patients before and after eradication therapy and in a control group including 40 children. RESULTS: The pretreatment aCL IgA (median 12.78 APL/mL), aCL IgM (median 21.60 MPL/mL), and aCL IgG antibody levels (median 14.22 GPL/mL) were significantly higher than those of post-treatment results (median 5.38 APL/mL, 7.02 MPL/mL, and 6.64 GPL/mL, respectively) and controls (median 5.90 APL/mL, 4.80 MPL/mL, and 4.81 GPL/mL, respectively). Anticardiolipin antibodies revealed no significant differences between the study group after therapy and the control group. CONCLUSIONS: In our particular experience, H. pylori can cause aCL antibody positivity in children and eradication of H. pylori provides the disappearance of these antibodies.


Asunto(s)
Anticuerpos Anticardiolipina/sangre , Infecciones por Helicobacter/patología , Helicobacter pylori/patogenicidad , Adolescente , Niño , Femenino , Humanos , Masculino
11.
Eur J Pediatr ; 172(11): 1501-9, 2013 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23812509

RESUMEN

UNLABELLED: The aim of this study was to investigate the changes in the peripheral blood of newborns of hypertensive mothers. The umbilical cord blood from newborns of 31 hypertensive mothers and 32 healthy mothers were examined. In all subjects, complete blood count, peripheral blood smear, reticulocyte count, vitamin B12, folate, ferritin levels and hemoglobin electrophoresis were performed. The subjects were followed up on for 1 year in terms of infections. RBC, hemoglobin, reticulocyte count and normoblast count were higher in the newborns of hypertensive mothers compared to the control group, and total leukocytes, neutrophil, lymphocyte, monocyte, eosinophil, and thrombocyte counts were lower. The number of neutropenic and thrombocytopenic subjects in newborns of hypertensive mothers was higher compared to the control group. On peripheral smears, dysplastic changes in neutrophils and erythrocytes were observed with a higher rate in newborns of hypertensive mothers compared to the control group. HbF levels were found to be higher in newborns of hypertensive mothers compared to the control group. During the follow-up period of 1 year, the number of infections in newborns of hypertensive mothers was found to be higher than the control group. CONCLUSION: Newborns of hypertensive mothers should be carefully evaluated and monitored in terms of hematologic abnormalities. Complete blood counts and peripheral blood smears can be used as significant parameters for early diagnosis of possible complications.


Asunto(s)
Sangre Fetal/metabolismo , Hipertensión Inducida en el Embarazo , Adulto , Biomarcadores/sangre , Recuento de Células Sanguíneas , Estudios de Casos y Controles , Índices de Eritrocitos , Femenino , Ferritinas/sangre , Estudios de Seguimiento , Hemoglobinas/metabolismo , Humanos , Recién Nacido , Modelos Logísticos , Masculino , Embarazo , Recuento de Reticulocitos , Vitamina B 12/sangre
12.
Turk J Haematol ; 30(3): 325-7, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24385815

RESUMEN

UNLABELLED: Herein we report an asphyctic preterm neonate with respiratory distress and prothrombotic risk factors that responded positively to rtPA treatment following 2 attacks of acute thrombosis. CONFLICT OF INTEREST: None declared.

13.
Indian J Cancer ; 60(3): 325-330, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36861687

RESUMEN

Objective: This study aims to demonstrate the prevalence of metabolic syndrome parameters and to investigate their relationship with body mass index in pediatric acute lymphoblastic leukemia survivors. Methods: The cross-sectional study was conducted between January and October 2019 at the Department of Pediatric Hematology and comprised acute lymphoblastic leukemia survivors who had been treated between 1995 and 2016 and had been off treatment for at least 2 years. The control group included 40 healthy participants who were matched for age and gender. The two groups were compared in terms of various parameters (BMI [body mass index], waist circumference, fasting plasma glucose, HOMA-IR [Homeostatic Model Assessment-Insulin Resistance], etc.). Data were analyzed using Statistical Package for the Social Sciences (SPSS) 21. Results: Of the 96 participants, 56 (58.3%) were survivors and 40 (41.6%) were controls. Among the survivors, there were 36 (64.3%) men, whereas the control group had 23 (57.5%) men. The mean age of the survivors was 16.67 ± 3.41 years, whereas the mean age of the controls was 15.51 ± 4.2 years (P > 0.05). Multinomial logistic regression analysis showed that cranial radiation therapy and female gender were associated with overweight and obesity (P < 0.05). A significant positive correlation was found between BMI and fasting insulin, in survivors (P < 0.05). Conclusion: Disorders of the metabolic parameter were found to be more common among acute lymphoblastic leukemia survivors than among healthy controls.


Asunto(s)
Síndrome Metabólico , Leucemia-Linfoma Linfoblástico de Células Precursoras , Niño , Masculino , Humanos , Femenino , Adolescente , Adulto Joven , Adulto , Síndrome Metabólico/epidemiología , Síndrome Metabólico/complicaciones , Estudios Transversales , Obesidad , Índice de Masa Corporal , Sobrevivientes , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia
14.
J Clin Res Pediatr Endocrinol ; 15(1): 55-61, 2023 02 27.
Artículo en Inglés | MEDLINE | ID: mdl-36264033

RESUMEN

Objective: Premature adrenarche (PA) has been associated with an increase in adrenal androgens, and the hyperandrogenic hormonal environment is known to lead to increased platelet (PLT) aggregation. Here, we evaluated the effects of PA on PLT aggregation in PLT-rich plasma samples from female patients. Methods: The study included 40 female patients diagnosed with PA between February, 2014 and June, 2018 and 30 healthy female individuals as a control group. Adenosine diphosphate (ADP) and collagen-induced PLT aggregation were studied via the photometric aggregometry method. Results: There were no significant differences in the PLT count or volume values between those participants with PA and the control group. Additionally, the ADP-induced maximum aggregation time, value, and slope values did not significantly differ between the patient and control groups (p>0.05). However, the collagen-induced maximum aggregation time, value, and slope values were significantly higher in the studygroup (p<0.001). Conclusion: Increased collagen-induced PLT aggregation was detected in female patients with PA. As PA is associated with a higher risk of cardiovascular events later in life, close follow-up of PA in this respect may be beneficial.


Asunto(s)
Adrenarquia , Pubertad Precoz , Humanos , Femenino , Agregación Plaquetaria , Andrógenos/farmacología , Adenosina Difosfato/farmacología , Colágeno/farmacología
15.
J Pediatr Hematol Oncol ; 34(2): e84-5, 2012 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-22258345

RESUMEN

Although childhood acute lymphoblastic leukemias are of good prognosis than leukemias of adulthood, some chromosomal abnormalities may have negative effects on their prognosis. Inverted duplication (1q) is a chromosomal abnormality with negative effect on outcome of Burkitt leukemia and lymphomas. We report a case of CD20 Burkitt leukemia with inverted duplication (1q) mutation, who had an early relapse during NHL-BFM 95 treatment. Two courses of ICE-rituximab treatment were administered after relapse and a successful HLA-full match bone marrow transplantation was carried out. He is in follow-up for 18 months without any problem after the bone marrow transplantation. We suggest the usage of ICE protocol combined with rituximab in childhood CD20 Burkitt leukemia with poor prognostic criteria such as inverted duplication (1q) mutation.


Asunto(s)
Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trasplante de Médula Ósea , Linfoma de Burkitt/terapia , Duplicación Cromosómica , Cromosomas Humanos Par 1/genética , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Linfoma de Burkitt/genética , Carboplatino/uso terapéutico , Niño , Etopósido/uso terapéutico , Humanos , Ifosfamida/uso terapéutico , Masculino , Mesna/uso terapéutico , Recurrencia Local de Neoplasia/genética , Recurrencia Local de Neoplasia/terapia , Rituximab
16.
J Pediatr Hematol Oncol ; 34(8): 630-4, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23108004

RESUMEN

OBJECTIVES: In this study, we aimed to investigate the relationship between chronic hemolysis and increased body iron burden with development of premature atherosclerosis by carotid intima-media thickness (IMT), ferritin, serum lipid profile, homocysteine, nitrate/nitrite, and chitotriosidase enzyme activity in children with ß-thalassemia major. MATERIALS AND METHODS: A total of 31 children with a diagnosis of ß-thalassemia major between the ages of 4 to 16 years constituted the study group. Control group was consisted of 36 age-matched healthy children. Complete blood count, serum glucose, lipid profile, ferritin, homocysteine, calcium, chitotriosidase, and nitrate/nitrite levels were measured and electrocardiographic and echocardiographic investigation and carotid IMT measurement were performed. RESULTS: In study group serum total cholesterol, low-density lipoprotein cholesterol and high-density lipoprotein cholesterol levels were found to be significantly reduced, and very-low-density lipoprotein cholesterol levels were found to be significantly elevated. Plasma nitrate/nitrite levels were significantly reduced; chitotroisidase enzyme activity was significantly increased and carotid IMT was significantly increased in study group. Nitrate/nitrite was found to be the only variable that was statistically significantly related to carotid IMT. CONCLUSIONS: Subclinical atherosclerosis in children with ß-thalassemia major begins early in life, and these children are at risk for development of premature atherosclerosis.


Asunto(s)
Aterosclerosis/etiología , Sobrecarga de Hierro/etiología , Talasemia beta/complicaciones , Adolescente , Edad de Inicio , Aterosclerosis/epidemiología , Aterosclerosis/patología , Biomarcadores , Glucemia/análisis , Grosor Intima-Media Carotídeo , Terapia por Quelación , Niño , Preescolar , Electrocardiografía , Femenino , Ferritinas/sangre , Hemólisis , Hexosaminidasas/sangre , Homocisteína/sangre , Humanos , Sobrecarga de Hierro/tratamiento farmacológico , Lípidos/sangre , Masculino , Nitratos/sangre , Nitritos/sangre , Reacción a la Transfusión
17.
J Pediatr Hematol Oncol ; 34(7): 541-4, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-22983416

RESUMEN

Griscelli syndrome (GS) is a rare autosomal recessive disorder associated with skin or hair hypopigmentation, hepatosplenomegaly, pancytopenia, and immunologic and central nervous system abnormalities. GS type II is caused by RAB27A mutations. We present RAB27A mutation analysis of 6 cases diagnosed as GS type II. Missense mutations (L26P and L130P) in 2 cases, deletion of 5 bases (514delCAAGC) in 2 cases, and 1 base deletion (148delA) in 2 cases were detected. This report has importance in phenotype-genotype correlation of different types of mutations including missense mutations and deletions within the RAB27A gene in GSII syndrome.


Asunto(s)
Síndromes de Inmunodeficiencia/genética , Mutación , Piebaldismo/genética , Proteínas de Unión al GTP rab/genética , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Linfohistiocitosis Hemofagocítica , Masculino , Enfermedades de Inmunodeficiencia Primaria , Proteínas rab27 de Unión a GTP
18.
Bone Marrow Transplant ; 57(5): 760-767, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35210564

RESUMEN

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.


Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Talasemia , Talasemia beta , Niño , Enfermedad Injerto contra Huésped/etiología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Estudios Retrospectivos , Talasemia/complicaciones , Talasemia/terapia , Acondicionamiento Pretrasplante/efectos adversos , Turquía/epidemiología , Talasemia beta/complicaciones , Talasemia beta/terapia
19.
Turk Arch Pediatr ; 56(5): 451-457, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35110113

RESUMEN

OBJECTIVE: This study aimed to compare the effectiveness of 2% chlorhexidine gluconate in 70% alcohol with that of 10% povidone-iodine, for dressing changes in pediatric hematology-oncology patients with port catheters, in preventing catheter-related bloodstream infection (CRBSI). METHODS: In this prospective, multicenter, observational, and cross-sectional study, 45 patients (25 patients for chlorhexidine, 20 patients for povidone-iodine) with port catheters were evaluated from January 2018 to May 2019. The sociodemographic, clinical, and port catheter-related variables were evaluated. The mean age of the patients was 6.28 ± 4.58 years, and 60% of patients were female. RESULTS: Among the patients whose dressings were changed using 2% chlorhexidine gluconate in 70% alcohol, the mean number of dressing changes was 39.52 ± 29.7 and the rates of exit-site infection and CRBSI were 20% (2.37/1000 catheter-days) and 16% (1.90/1000 catheter-days), respectively. Among the patients whose dressings were changed using 10% povidone-iodine, the mean number of dressing changes was 48.0 ± 31.48 and the rates of exit-site infection and CRBSI were 15% (1.59/1000 catheter-days) and 10% (1.06/1000 catheter-days), respectively. None of the patients developed pocket infections. The rates of CRBSI and exit-site infections were not different between the 2 antiseptic solutions. CONCLUSION: This study found no differences between the effectiveness of 2% chlorhexidine in 70% alcohol and that of 10% povidone-iodine solution in preventing CRBSI. Therefore, both solutions can be used in dressing changes.

20.
Turk J Pediatr ; 63(2): 251-256, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33929114

RESUMEN

BACKGROUND: Febrile neutropenia (FN) is a common and serious complication in patients with leukemia. Hemostasis and inflammation are two interrelated systems in response to infection. We aimed to investigate the course of thrombin formation in febrile neutropenia attack of children with acute lymphoblastic leukemia (ALL). METHODS: Thrombin generation was monitored in children treated for ALL at diagnosis of febrile neutropenia (FN) (t < sub > 0 < /sub > ), at 48 < sup > th < /sup > hour of FN (t1) and after recovery from neutropenia (t < sub > 2 < /sub > ). RESULTS: Twenty-nine patients and 50 healthy children as control were enrolled into the study. Mean endogenous thrombin potential (ETP) and mean peak value of thrombin results at t < sub > 1 < /sub > were significantly higher than at t < sub > 0 < /sub > , t < sub > 2 < /sub > and control groups, respectively. A positive but statistically nonsignificant correlation between ETP values at t < sub > 1 < /sub > and duration of neutropenia was observed. CONCLUSION: Although thrombin generation is enhanced both due to chemotherapy or malignancy itself, our results revealed that thrombin formation also increased in neutropenic infection of children with leukemia.


Asunto(s)
Neutropenia Febril , Leucemia-Linfoma Linfoblástico de Células Precursoras , Pruebas de Coagulación Sanguínea , Niño , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Trombina
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