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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.
Cardiol Young ; 33(9): 1606-1613, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-36102124

RESUMEN

The aim of this study is to evaluate the changes in myocardial functions in children who underwent haematopoietic stem cell transplantation along with associated chemotherapy. Additionally, we evaluated the effect of baseline echocardiographic parameters on mortality. We evaluated 39 patients (mean age 7.4 years) who underwent haematopoietic stem cell transplantation owing to non-malignant disease. The control group included 39 healthy children who had normal cardiac findings. The myocardial functions were evaluated in all subjects by conventional echocardiography and tissue Doppler echocardiography before haematopoietic stem cell transplantation and in the 1st, 3rd, 6th, and 12th month after haematopoietic stem cell transplantation. All patients had normal left ventricular ejection fraction before haematopoietic stem cell transplantation, except one case. Before haematopoietic stem cell transplantation, the patient group had significantly greater mean pulmonary artery pressure and lower tricuspid valve annular plane excursion rate. Baseline E' velocities for mitral lateral annuli, septum, and tricuspid lateral annuli were lower in the patient group than the control group. The E' velocities for the left ventricle decreased in the patient group after haematopoietic stem cell transplantation, and then returned to baseline levels at the 6 months. E' and S' velocities for tricuspid lateral annuli also decreased after haematopoietic stem cell transplantation and were still depressed in the first year after haematopoietic stem cell transplantation. Baseline E' velocity for septum was significantly lower in patients who died after haematopoietic stem cell transplantation than patients who survived (p = 0.009). Subclinical impairment in both ventricular functions was observed after haematopoietic stem cell transplantation and the right ventricular functions were affected for longer periods than left ventricle after haematopoietic stem cell transplantation. The myocardial functions should be monitored after the first year of haematopoietic stem cell transplantation.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Función Ventricular Izquierda , Humanos , Niño , Volumen Sistólico , Función Ventricular Izquierda/fisiología , Ecocardiografía Doppler , Ecocardiografía , Trasplante de Células Madre Hematopoyéticas/efectos adversos
3.
J Pediatr Hematol Oncol ; 44(1): e26-e34, 2022 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-34986131

RESUMEN

BACKGROUND: Studies on the increased body iron load in patients with thalassemia major have thoroughly demonstrated the problems caused by iron overload. In patients who undergo hematopoietic stem cell transplantation (HSCT) as curative therapy, iron overload continues long after transplantation. There are few pediatric studies on chelation therapy in the posttransplant period. In this study, we present the outcomes of our patients who received posttransplant oral chelation therapy. PATIENTS AND METHODS: This retrospective observational study evaluated the outcomes of pediatric patients with thalassemia major who used oral chelation therapy after allogeneic HSCT at the Akdeniz University Pediatric Bone Marrow Unit between January 2008 and October 2019. RESULTS: Deferasirox therapy was initiated in 58 pediatric patients who underwent HSCT for thalassemia. Pretreatment mean serum ferritin was 2166±1038 ng/mL. Treatment was initiated at a mean of 12±6.7 months after transplantation and continued for a mean of 15.7±11.5 months. At treatment discontinuation, the mean serum ferritin was 693±405 ng/mL and the mean reduction was -1472.75±1121.09 ng/mL (P<0.001 vs. posttreatment). Serum ferritin was below 500 ng/mL in 52% of the patients at treatment discontinuation. Manageable side effects such as nausea, vomiting, liver enzyme elevation, and proteinuria were observed in 17% of the patients, while one patient developed ototoxicity. CONCLUSIONS: Deferasirox therapy effectively reduces iron overload in the posttransplant period. Studies evaluating the effects of early treatment on the graft may help to establish guidelines for posttransplant chelation therapy. Clear guidelines are needed regarding when to initiate and discontinue treatment.


Asunto(s)
Deferasirox/administración & dosificación , Trasplante de Células Madre Hematopoyéticas , Quelantes del Hierro/administración & dosificación , Sobrecarga de Hierro/terapia , Talasemia/terapia , Adolescente , Aloinjertos , Niño , Preescolar , Deferasirox/efectos adversos , Femenino , Ferritinas/sangre , Humanos , Quelantes del Hierro/efectos adversos , Sobrecarga de Hierro/sangre , Masculino , Estudios Retrospectivos , Talasemia/sangre
4.
Eur J Cancer Care (Engl) ; 31(6): e13698, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-36069654

RESUMEN

OBJECTIVE: The aim of this study is to evaluate the effect of 'peer-assisted video-based education programme' (VTPA) and counselling to reduce anxiety in children newly diagnosed with cancer. METHODS: A non-randomised controlled trial design with repeated measures was conducted. The sample of the study consisted of 50 children between the ages of 9 and 18 who were newly diagnosed with cancer in the hospital. Intervention and control groups were created. Children in the intervention group received both VTPA training and counselling practice 3 days a week for 2 months. Data were collected with the State-Trait Anxiety Inventory for Children. The basal assessment in the research was the first meeting with the child, with interval assessments 1 month after, 2 months after and 3 months after the education. RESULTS: In the study, it was determined that the state anxiety mean scores of the children in the PAVEP and control groups differed over time (p < 0.05). However, it was determined that there was no difference between the trait anxiety mean scores of the children (p > 0.05). CONCLUSION: VTPA with peer assistance and counselling application supported the reduction of state anxiety in children newly diagnosed with cancer over time.


Asunto(s)
Ansiedad , Neoplasias , Niño , Humanos , Adolescente , Ansiedad/etiología , Ansiedad/prevención & control , Consejo , Trastornos de Ansiedad , Grupo Paritario , Neoplasias/terapia
5.
Clin Transplant ; 35(3): e14209, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-33368539

RESUMEN

BACKGROUND: The spectrum of human adenovirus (HAdV)-related disease is broad, and the virus acts on many organs and systems in hematopoietic stem cell transplantation (HSCT) recipients. We aimed to evaluate the effect of HAdV-DNA positivity with clinical and laboratory findings 4 months after HSCT. METHODS AND RESULTS: We retrospectively investigated HAdV-DNA in 153 HSCT recipients (≤18 years) by quantitative real-time polymerase chain reaction (RealStar; Altona Diagnostics). The results of samples from January 2014 to December 2017 are included. HAdV-DNA was positive for at least one sample type in 50 (32.67%) patients. HAdV-DNA positivity rate was 8.92% (N: 145/1625), 40.25% (N: 64/159), and 25% (N: 2/8) for plasma, stool, and urine samples, respectively. HAdV-DNA was positive in the plasma of 38 (24.83%) patients at a median 16 (range: 1-58 days) days after HSCT. The mortality rate was 23.68% and 6.95% in plasma HAdV-positive and HAdV-negative patients (p = .014). Moreover, HAdV-DNA positivity had an impact on overall survival for allogeneic-HSCT (p = .013), with the cumulative effect including graft-versus-host disease state in multivariate analysis (p = .014). CONCLUSIONS: Plasma HAdV-DNA positivity is a potential influencer that decreases survival in the early post-transplant period. Due to the high mortality rates, close monitoring is required of HAdV infections after HSCT with sensitive methods, especially at the early stage.


Asunto(s)
Adenovirus Humanos , Trasplante de Células Madre Hematopoyéticas , Adenovirus Humanos/genética , Niño , ADN Viral , Estudios de Seguimiento , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Estudios Retrospectivos , Receptores de Trasplantes , Carga Viral
6.
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
7.
J Pediatr Hematol Oncol ; 42(5): e315-e320, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32427707

RESUMEN

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is a curative therapy option for hematologic malignancies. Iron overload is common in this patient group and can impact short-term and long-term nonrelapse mortality. STUDY DESIGN: Retrospective observational cohort study. AIMS: To evaluate the effect of iron load on early and late HSCT outcomes in patients with acute leukemia and myelodysplasia to assess the necessity of reducing iron load. PATIENTS AND METHODS: Sixty patients who underwent HSCT in pediatric stem cell transplantation unit between 2000 and 2012 were evaluated retrospectively. The patients were divided into those with pretransplantation serum ferritin levels above and below the median value of 1299 ng/mL. RESULTS: Forty-two (70%) of the patients were male, mean ages of the low and high ferritin groups were 85.43±9.42 and 118.56±10.04 months, respectively. Acute graft-versus-host disease (GVHD) within the first 100 days and acute liver GVHD were significantly more common in the high ferritin group (P<0.011 for both). Ferritin level was not associated with rates of engraftment syndrome, veno-occlusive disease, early/late infection, relapse, or overall and disease-free survival. CONCLUSIONS: In our study, significant result especially in terms of acute liver GVHD, was important to emphasize the need to be more careful in terms of acute liver GVHD risk in early liver pathologies in patients with high levels of ferritin after transplantation. In future large studies may be helpful to explain the relationship between acute liver GVHD and high ferritin levels.


Asunto(s)
Enfermedad Injerto contra Huésped/patología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Sobrecarga de Hierro/patología , Leucemia Mieloide Aguda/terapia , Hepatopatías/patología , Síndromes Mielodisplásicos/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Biomarcadores/sangre , Niño , Femenino , Ferritinas/sangre , Estudios de Seguimiento , Enfermedad Injerto contra Huésped/sangre , Enfermedad Injerto contra Huésped/etiología , Humanos , Sobrecarga de Hierro/sangre , Sobrecarga de Hierro/etiología , Leucemia Mieloide Aguda/patología , Hepatopatías/sangre , Hepatopatías/etiología , Masculino , Síndromes Mielodisplásicos/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Pronóstico , Estudios Retrospectivos , Trasplante Homólogo
8.
Turk J Med Sci ; 50(8): 1916-1921, 2020 12 17.
Artículo en Inglés | MEDLINE | ID: mdl-32628434

RESUMEN

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.


Asunto(s)
Cuidados Posteriores/métodos , Supervivientes de Cáncer/estadística & datos numéricos , Países en Desarrollo , Pediatría/métodos , Encuestas y Cuestionarios/estadística & datos numéricos , Niño , Estudios Transversales , Humanos , Transición a la Atención de Adultos , Turquía
9.
Biol Blood Marrow Transplant ; 25(10): 2017-2023, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31199982

RESUMEN

Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for bone marrow failure (BMF) in patients with Fanconi anemia (FA). We retrospectively analyzed the records of patients with FA who underwent HSCT with a radiation-free, reduced-intensity conditioning regimen (fludarabine, cyclophosphamide, and antithymocyte globulin) along with an unmanipulated graft infusion between 2004 and 2018. A total of 44 patients underwent HSCT during the study period. Median age at transplantation was 121 months. Regarding the donor source, 22 transplants (50%) were collected from matched related donors (MRDs), and 22 transplants (50%) were collected from alternative donors (ADs). The median infused CD34+ cell dose was 4.7 × 106/kg (range, 0.8 to 23) in bone marrow or peripheral blood stem cell recipients and 1.2 × 105/kg (range, 1.1 to 3.6) in umbilical cord blood recipients. All but 2 patients achieved primary neutrophil engraftment (95%). In a median follow-up of 36 months (range, 1 to 159), 3-year overall survival was 70.5% in the entire group and 91% in the MRD recipients. Primary causes of death were infections (n = 5), acute grade 3 to 4 graft-versus-host disease (n = 4), and hemorrhagic cystitis (n = 3). All surviving patients have full (n = 29) and acceptable mixed (n = 2) donor chimerism and good clinical status. Our results showed an excellent outcome with unmanipulated grafts using a fludarabine-based, radiation-free preparative regimen for MRD recipients. Even though primary neutrophil engraftment rates were good in AD recipients, intervening complications increased mortality in these patients. In clinics where T cell depletion is not feasible, more effort is warranted to improve outcomes for AD recipients.


Asunto(s)
Anemia de Fanconi/terapia , Trasplante de Células Madre Hematopoyéticas/métodos , Acondicionamiento Pretrasplante/métodos , Trasplante Homólogo/métodos , Anciano , Anciano de 80 o más Años , Anemia de Fanconi/mortalidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Análisis de Supervivencia , Factores de Tiempo
12.
Pediatr Hematol Oncol ; 35(1): 1-10, 2018 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-29528260

RESUMEN

AIM: In recent years, survival rates of childhood cancers have significantly increased, and occurrence of long-term adverse late effects (eg, insulin resistance, diabetes mellitus, metabolic syndrome, hypertension) has become increasingly important. Early diagnosis of obesity/hypertension in childhood is essential to avoid morbidity in the adulthood. Therefore, this study was aimed to determine the blood pressure (BP) profile by ambulatory BP monitoring (ABPM) method, and prevalence of hypertension, obesity, abdominal obesity among childhood cancer survivors. MATERIAL AND METHOD: The study was carried out with 52 cancer survivors. The ABPM measurement was performed during 24 hours. The anthropometric measurements of patients were performed using standardized protocols. The body composition analysis was performed with bioelectrical impedance analysis (BIA) method. Statistical significance was considered at p < 0.05. RESULTS: The mean age of patients was 12.84 ± 3.88 years. Time off therapy ranged 24-125 month. The prevalence of prehypertension and hypertension were 57.7% and 9.6%, respectively. There was no statistically significant relationship between diagnosis and BP status (p = 0.59). The prevalence of obesity, and abdominal obesity were 1.9% and 30.4%, respectively. There was a positive correlation between waist circumference (WC) and time off therapy (p = 0.046). The WC was found to be higher in patients who received cranial irradiation (p = 0.048). Weight/WC were higher in patients who used corticosteroids in the treatment (p = 0.019). CONCLUSION: Careful follow up of BP, weight and WC is necessary for long-term cancer survivors to prevent complications. Especially patients who receive cranial radiotherapy and use corticosteroid are at increased risk of abdominal obesity.


Asunto(s)
Monitoreo Ambulatorio de la Presión Arterial , Composición Corporal , Supervivientes de Cáncer , Hipertensión , Obesidad Abdominal , Adolescente , Adulto , Niño , Femenino , Humanos , Hipertensión/epidemiología , Hipertensión/etiología , Hipertensión/patología , Hipertensión/fisiopatología , Masculino , Neoplasias/epidemiología , Neoplasias/patología , Neoplasias/fisiopatología , Neoplasias/terapia , Obesidad Abdominal/epidemiología , Obesidad Abdominal/etiología , Obesidad Abdominal/patología , Obesidad Abdominal/fisiopatología , Prevalencia
13.
Pediatr Hematol Oncol ; 32(2): 153-63, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24498898

RESUMEN

In childhood cancer patients, early diagnosis may have an impact on survival that reduces the potential morbidity. This study aimed to identify the factors associated with delay in diagnosis in children with cancer in southeastern Turkey. The clinical records of 682 patients with childhood cancer were evaluated. Study variables were classified as factors related to the patient, their disease, and the health care system. The median parental delay, physician delay, and total delay were determined as 20, 23, and 60 days, respectively. There was a significant relationship between parental delay, physician delay, and total delay and age at diagnosis (P = .005, P = .008, and P = .004, respectively). Long parental delay was least frequent in children younger than 1 year (P = .001). Parental, physician, and total delay were longer in patients with solid tumors than in patients with leukemias (P = .007, P = .000, and P = .000, respectively). Patients with tumors of the genitalia had longer physician delay and total delay than patients with other solid tumors (P = .001 and P = .000, respectively). Patients with solid tumor and early-stage disease had longer physician delay and total delay (P = .016 and P = .013, respectively). According to the first physician contacted, long physician delay was less frequent among pediatricians (P = .003). Delayed diagnosis was associated with age, type/localization and stage of tumor, the first physician consulted, and area of residence. A sustained effort should be made to raise the level of awareness of childhood cancer among parents and to sensitize all physicians, especially those who treat pediatric patients infrequently, with regard to the warning signs of the disease.


Asunto(s)
Diagnóstico Tardío/mortalidad , Neoplasias/diagnóstico , Neoplasias/mortalidad , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estadificación de Neoplasias , Neoplasias/patología , Factores de Tiempo , Turquía/epidemiología
14.
Am J Med Genet B Neuropsychiatr Genet ; 168(7): 609-16, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26174935

RESUMEN

To investigate relationships between the polymorphisms and social functioning of children with Attention Deficit/Hyperactivity Disorder (ADHD), according to the polymorphism of three oxytocin receptor (OXTR) genes (rs53576, rs13316193, and and rs2268493). A total of 198 children-studying in the same primary and secondary school and matched in terms of age and gender (99 ADHD, 99 control)-were included in this study. The Schedule for Affective Disorders and Schizophrenia for School-Age Children-Present and Lifetime Version was administered to establish the clinical diagnosis. The Social Reciprocity Scale (SRS) was applied to evaluate social functioning. The total genomic DNA was isolated from buccal mucosa samples. No significant differences were determined between the ADHD and control groups in terms of rs2268493, rs13316193, and rs53576 genotype distribution (P = 0.078, P = 0.330, and P = 0.149, respectively). However, the control group T allele frequency in the OXTR Single Nucleotide Polymorphism (SNP) rs2268493 was significantly higher than the ADHD group (P = 0.024). Compared to the control group, the ADHD group had a higher score on the SRS scale (SRS total; Z = -21,135, P < 0.001). No significant difference existed in the SRS scale scores between the children with the T/T genotype and the C allele in the ADHD group (SRS total; Z = -0.543, P = 0.587). The allele distribution of the OXTR gene SNP rs2268493 was significantly different in the ADHD group, compared to the control group. This observation is important in understanding the underlying biological infrastructure in ADHD and developing treatment modalities.


Asunto(s)
Trastorno por Déficit de Atención con Hiperactividad/genética , Oxitocina/genética , Receptores de Oxitocina/genética , Adolescente , Trastorno por Déficit de Atención con Hiperactividad/metabolismo , Estudios de Casos y Controles , Niño , Femenino , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Humanos , Relaciones Interpersonales , Masculino , Oxitocina/metabolismo , Polimorfismo de Nucleótido Simple , Receptores de Oxitocina/metabolismo , Conducta Social , Turquía
15.
J Cancer Res Ther ; 18(6): 1823-1826, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36412455

RESUMEN

Parameningeal rhabdomyosarcomas (PM RMSs) are rarely seen childhood tumors. Their treatment might be challenging and prognosis is poor compared to other head and neck RMS. Here we report a PM RMS presenting with leptomeningeal seeding metastasis a year after diagnosis. A five-year-old girl presented with an enlarging mass protruding from the right ear and right facial paralysis. Magnetic resonance imaging (MRI) revealed a large mass extending from right external auditory canal to the temporal lobe, pterygoid fossa and nasopharynx with an intracranial component indenting the right temporal lobe and extending into the right cavernous sinus. Trucut biopsy revealed embryonal rhabdomyosarcoma. Cerebrospinal fluid (CSF) cytology was negative for malignant cells. Chemotherapy was started since it was found unresectable. At second week of chemotherapy, radiotherapy was applied to primary tumor location with intensity-modulated radiation therapy (IMRT) technique in 1.8 Gy fractions to total dose of 50.4 Gy. At week 27, MRI showed significant response. At week 36, the patient presented with vomiting and tendency to sleep. MRI was found to be compatible with meningitis and antibacterial therapy was started. At week 39, chemotherapy was stopped. But MRI performed one month later revealed linear contrast enhancements around the spinal cord compatible with leptomeningeal metastases. Chemotherapy and craniospinal irradiation were applied. But the patient did not improve and received palliative treatment. Six months after the completion of radiotherapy the patient died. Treatment of parameningeal rhabdomyosarcomas require multidisciplinary approach including surgery, radiotherapy, and chemotherapy. Prognosis is poor for patients with leptomeningeal spread.


Asunto(s)
Carcinomatosis Meníngea , Rabdomiosarcoma Embrionario , Rabdomiosarcoma , Femenino , Humanos , Niño , Preescolar , Rabdomiosarcoma/terapia , Meninges , Imagen por Resonancia Magnética
16.
Turk J Gastroenterol ; 33(12): 1069-1078, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36262102

RESUMEN

BACKGROUND: Hepatoblastoma is rare cancer that responds well to risk-based chemotherapy, and surgical treatment is needed to achieve complete remission and satisfactory survival rates in hepatoblastoma patients. In this study, we evaluated the clinical features and treatment outcomes of pediatric hepatoblastoma patients treated in our clinic. METHODS: Eighteen patients with hepatoblastoma who were treated and followed up in our center between June 1999 and June 2020 were analyzed retrospectively. All patients were evaluated by a multidisciplinary team and managed using a risk-based protocol (SIOPEL-1 and SIOPEL-3). RESULTS: The patients' mean age at diagnosis was 38.33 ± 52.34 months. Sixteen patients (89%) received neoadjuvant chemotherapy, and 2 patients (11%) who underwent complete mass excision at diagnosis received adjuvant chemotherapy. After neoadjuvant therapy, the tumor was completely resected in 8 patients (45%), while liver transplantation was performed in 6 patients (34%) because complete resection of the tumor was not possible. Two patients died before surgical treatment. One patient relapsed with lung metastasis after salvage chemotherapy. She is alive without disease at 64 months. The mean follow-up time was 59.3 ± 49.8 months; 5-year overall and disease-free survival rates were 88.9% and 80.8%, respectively. The 5-year overall survival rate was 100% for both liver transplant and resected patients, whereas 5-year disease-free survival was lower in transplant patients (75% vs 100%, P < .001). CONCLUSION: Multidisciplinary follow-up is especially important for patients who may need liver transplantation. Some patients may benefit from new treatment options such as radiofrequency ablation and cyberknife treatment.


Asunto(s)
Hepatoblastoma , Neoplasias Hepáticas , Niño , Femenino , Humanos , Lactante , Preescolar , Estudios Retrospectivos , Neoplasias Hepáticas/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Hepatoblastoma/tratamiento farmacológico , Hepatoblastoma/patología , Terapia Neoadyuvante , Quimioterapia Adyuvante , Resultado del Tratamiento
17.
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
18.
Pediatr Hematol Oncol ; 28(5): 395-402, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21699469

RESUMEN

Doxorubicin (DOX) has been used in the treatment of childhood cancers, but its usage is limited because of cardiotoxicity. There are many studies on the role of nitric oxide (NO) in several cardiac diseases. However, to the authors' knowledge, no studies have investigated the plasma levels of total nitrite, a stable product of NO, in children that received DOX. The authors determined plasma total nitrite levels in 29 children who received DOX and investigated the correlations between these and other clinical and laboratory findings. All patients were in remission during the study period. Plasma total nitrite levels and cardiac functions were determined prospectively. The mean cumulative DOX dose was 310.0 ± 90.9 mg/m(2). In echocardiographic evaluation, 3 patients (10.3%) had a pathological value for ejection fraction (EF) and/or fractional shortening (FS), whereas 11 patients (37.9%) had a threshold value. Total nitrite levels were higher in patients than in matched controls (75.24 ± 39.4 vs 43.14 ± 10.58 µmol/L, respectively, P < .001). It was also found to be higher in patients who had a pathological/threshold value of EF and FS than in patients who had a physiological value of EF and FS (92.35 ± 50.36 vs 59.26 ± 13.56 µmol/L, respectively, P = .038). A negative correlation was found between FS and plasma total nitrite level (r = -.42, P = .023). The authors speculate that increased NO may be a sign of subclinical cardiotoxicity of DOX.


Asunto(s)
Antibióticos Antineoplásicos/efectos adversos , Doxorrubicina/efectos adversos , Corazón/efectos de los fármacos , Óxido Nítrico/sangre , Adolescente , Biomarcadores/sangre , Niño , Preescolar , Femenino , Humanos , Masculino , Mediastino/efectos de la radiación , Neoplasias/tratamiento farmacológico , Nitritos/sangre , Volumen Sistólico/efectos de los fármacos
19.
J Dent Sci ; 16(1): 311-317, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33384814

RESUMEN

BACKGROUND/PURPOSE: Multivitamin syrups and effervescent tablets are commonly used for dietary support in children; however, these preparations may have detrimental effects on the surface of restorative materials. The aim of this study was to evaluate the effect of two multivitamin syrups and two effervescent tablets taken daily on the surface microhardness and roughness of two different restorative materials. MATERIALS AND METHODS: The study groups were comprised of a conventional glass ionomer cement, Ketac Molar, and a giomer, Beautifil II. A total of 140 disc-shaped specimens were prepared and randomly assigned to 5 subgroups. The samples were immersed in 4 multivitamins (Supradyne syrup, Supradyne effervescent, Sambucol syrup, Sambucol effervescent) and deionized water (control group) for 2 min once a day for 28 days. Surface microhardness and roughness measurements were recorded at baseline and on the 7th, 14th, 21st and 28th days. Surface microhardness was measured using a Vickers hardness tester and surface roughness was determined using a profilometer. RESULTS: For all subgroups; the surface microhardness values of the Ketac Molar and Beautifil II demonstrated a statistically significant decrease from the baseline to the 28th day measurements (p < 0.05), while the surface roughness displayed increased values that were found to be statistically significant (p < 0.05). The Ketac Molar surface roughness values were significantly higher than Beautifil II in all subgroups for all measurement days (p = 0.0001). CONCLUSION: The prolonged use of multivitamin syrups and effervescent tablets may have negative effects on the physical properties of restorative materials.

20.
Turk J Pediatr ; 63(5): 884-892, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34738370

RESUMEN

BACKGROUND: Previous studies have shown that the immune system plays a critical role in cancer pathogenesis. The lymphocyte monocyte ratio (LMR) and monocyte percentage (MP) have been found to be prognostic factors in various types of adult cancers. But studies about pediatric tumors are scarce and to our knowledge, there are no studies evaluating the immune system effect in pediatric neuroblastoma patients. The aim of this study was to assess whether LMR and MP at diagnosis may have an effect on prognosis in neuroblastoma patients. METHODS: We retrospectively analyzed MP and LMR at diagnosis in 71 pediatric neuroblastoma patients treated between 2002 and 2016. RESULTS: The optimal cut-off values of LMR and MP were determined using the receiver operating characteristics curves (ROC) and area under the curve (AUC). We found that a low LMR (≤3.5) and a high MP (≥7.5%) were correlated with worse overall survival and shorter event-free survival in univariate analysis. Multivariate analysis revealed that elevated LMR was an independent factor for better OS and EFS. CONCLUSIONS: In conclusion, LMR and MP might be valuable prognostic factors for predicting OS in neuroblastoma patients. Multicenter and prospective studies are warranted to confirm this hypothesis.


Asunto(s)
Monocitos , Neuroblastoma , Adulto , Niño , Humanos , Linfocitos , Neuroblastoma/diagnóstico , Pronóstico , Estudios Retrospectivos
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