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1.
Childs Nerv Syst ; 2024 Mar 13.
Artículo en Inglés | MEDLINE | ID: mdl-38478066

RESUMEN

PURPOSE: Pediatric diffuse intrinsic pontine glioma (DIPG) is a fatal disease associated with a median survival of < 1 year despite aggressive treatments. This retrospective study analyzed the treatment outcomes of patients aged < 18 years who were diagnosed with DIPG between 2012 and 2022 and who received different chemotherapy regimens. METHODS: After radiotherapy, patients with DIPG received nimotuzumab-vinorelbine combination or temozolomide-containing therapy. When nimotuzumab was unavailable, it was replaced by vincristine, etoposide, and carboplatin/cyclophosphamide (VECC). Temozolomide was administered as a single agent or a part of the combination chemotherapy comprising temozolomide, irinotecan, and bevacizumab. Furthermore, 1- and 3-year overall survival (OS), progression-free survival (PFS), and median OS and PFS were analyzed. RESULTS: The median age of 40 patients with DIPG was 97 ± 46.93 (23-213) months; the median follow-up time was 12 months. One and 3-year OS were 35.0% and 7.5%, respectively. Median OS was 12 months in all patients (n = 40), and it was 16, 10, and 11 months in those who received first-line nimotuzumab-vinorelbine combination (n = 13), temozolomide-based (n = 14), and VECC (n = 6) chemotherapy regimens, respectively (p = 0.360). One patient who received gefitinib survived for 16 months. Conversely, patients who never received radiotherapy and any antineoplastic medicamentous therapy (n = 6) had a median OS of 4 months. CONCLUSION: Nimotuzumab-vinorelbine combination therapy prolonged OS by 6 months compared with temozolomide-containing chemotherapy, although the difference was not statistically significant.

2.
Pediatr Transplant ; 28(1): e14691, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38317340

RESUMEN

BACKGROUND: Advances in stem cell transplantation have resulted in improved outcomes. METHODS: This is a retrospective study aimed to analyze changes in patient profile, transplantation, graft characteristics, and outcome among 241 pediatric patients who received stem cell transplantation in a single center between 1993 and 2019. RESULTS: In the 2010-2019, compared with the 1993-2009 period, a significantly higher 5-year overall survival (60% vs. 44%, p = .022) and an event-free survival (53% vs. 34%, p = .025) were observed. Cumulative incidence of deaths due to relapse or progression between the 1993-2009 and 2010-2019 periods were 33% and 26% respectively (p = .66). Cumulative incidence of non-relapse mortality was significantly higher during the 1993-2009 period compared with the 2010-2019 period for malignant diseases (57.7% vs. 28.3%, p = .007). The overall survival from acute graft-versus-host disease between 1993 and 2009 was 11% versus 46% between 2010 and 2019 (p = .0001). The overall survival from infection in both eras did not show any difference (p = .41). CONCLUSIONS: Development in transplantation technology has led to a decrease in non-relapse mortality and better control of graft-versus-host disease. However, relapse and infection remained as major causes of death. Studies evaluating institutional trends in patients undergoing HSCT and analyzing their mortality profile, can improve the management of patients, leading to a reduction in transplant-related problems.


Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Humanos , Niño , Estudios Retrospectivos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedad Injerto contra Huésped/epidemiología , Enfermedad Injerto contra Huésped/etiología , Trasplante Homólogo/efectos adversos , Recurrencia
3.
Pediatr Blood Cancer ; 70(12): e30695, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37740727

RESUMEN

OBJECTIVES: Recent studies have shown that the neutrophil-to-lymphocyte ratio (NLR) is a new inflammatory marker that is effective in determining the prognosis of many solid tumors, chemotherapy responses, survival, and their recurrence rate. Therefore, we performed a retrospective study to investigate the effect of neutrophil-to-lymphocyte and platelet-to-lymphocyte ratio (PLR) on risk factors and prognosis in these patients. MATERIALS AND METHODS: In this study, 246 pediatric patients with neuroblastoma who were diagnosed, treated, and followed up during 2000-2021 in Division of Pediatric Oncology, Çukurova University Faculty of Medicine, were included. Required information of patients was obtained from archive files, Mergentech hospital program, and E-pulse system. RESULTS: Median value for NLR was found to be 1.06, for PLR it was found as 92. The relationship of NLR values with age, stage, risk group, and Shimada was found to be statistically significant with p < .001, vanillylmandelic acid (VMA) (p = .006) also depicted the significant value. Likewise, the relationship of PLR values with age (p < .001), stage (p = .022), Shimada (p = .004), and N-Myc amplification (p = .039) was found to be statistically significant as well. Survival analysis showed that no statistically significant difference was observed among the higher and lower values of NLR. Survival rates were noticed to be higher in the lower values of NLR (10-year overall survival [OS] 55% vs. 49%, 10-year event-free survival (EFS) 54% vs. 43%), albeit nonsignificant. CONCLUSION: Pretreatment evaluation of NLR and PLR values in patients with neuroblastoma may be instructive in respect of prognosis and risk group.

4.
Pediatr Int ; 65(1): e15562, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37310120

RESUMEN

BACKGROUND: The aim of this study was to analyze the characteristics of pediatric posterior reversible encephalopathy syndrome (PRES) to determine clinical and radiologic differences between younger and older age groups, and to identify risk factors for development of any neurologic sequelae. METHODS: The study cohort consisted of confirmed pediatric PRES patients in a tertiary care university hospital from January, 2015, to December, 2020. Demographic and clinical properties, radiological manifestations, and neurologic outcomes were noted. Children aged ≤6 years were compared with those older than 6 years and factors affecting neurologic outcomes were evaluated. RESULTS: The most common underlying diseases were oncological (37%) and kidney diseases (29%). Epileptic seizures were the most frequent symptoms at initial clinical presentation. The regions in the brain that were most commonly involved were the occipital region (n = 65, 96%), the parietal region (n = 52, 77%), and the frontal lobe (n = 35, 54%). Magnetic resonance imaging (MRI) findings were consistent with atypical patterns in most of the study cohort (71%). Patients with unfavorable clinical outcomes (n = 13, 19.1%) had longer initial seizure times and longer encephalopathy times, lower leucocyte and absolute neutrophil counts, and lower neutrophil to lymphocyte ratios. No relationship was found between MRI findings, involvement patterns, and neurologic outcomes. CONCLUSIONS: No clinically specific differences between two different age groups were found. Atypical imaging manifestations of pediatric PRES in our study had an incidence that was as high as those found in earlier adult studies. Multivariate logistic regression analysis showed that the initial neutrophil to lymphocyte ratio, absolute neutrophil counts, and white cell counts could not predict poor neurologic outcomes.


Asunto(s)
Síndrome de Leucoencefalopatía Posterior , Adulto , Humanos , Niño , Anciano , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/epidemiología , Radiografía , Recuento de Leucocitos , Leucocitos , Neutrófilos , Convulsiones/epidemiología , Convulsiones/etiología
5.
New Microbiol ; 46(2): 141-145, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37247234

RESUMEN

BK virus (BKV) associated with hemorrhagic cystitis (HC) is the most important complication that develops after hematopoietic stem cell transplantation (HSCT) in patients with hematological malignancies. This study aims to investigate BKV infections and HC in pediatric patients after allogeneic hematopoietic stem cell transplantation. Between November 2018 and November 2019, a total of 51 patients between the ages of 11 months and 17 years were included in the study. BKV Bosphore ® v1 quantification kit (Geneworks Anatolia, Turkey) was used for the detection of BKV DNA in urine and blood samples. Among the total of 51 patients, the incidence of BKV infection was found to be 86.3%. Allogeneic HSCT was performed in 40 patients and autologous HSCT in 11 patients. BK viruria and/or viremia were detected in 85% (44) of patients who underwent allogeneic HSCT and in 90% in the autologous group. High-level BK viruria (>107 copies/mL) was found in 41% (9) of 22 patients who were BKV positive before transplantation, while in 27.5% (8) of 29 patients who were BKV negative before transplantation; thus, BKV positivity before transplantation was considered a risk factor for high-level BK viruria. Acute GVHD developed in 6 of 40 patients in the allogeneic group. HC was prevented in 12 (67%) of 18 patients who received preemptive treatment, while HC developed in 6 (33%). HC occurred at a median of 35 days (17-49 days) post-transplant. Despite preemptive treatment, 6 (15%) patients who developed HC associated with BKV were in the allogeneic group but not in the autologous group. Of these patients with HC, 5 received a myeloablative treatment regimen, and 1 patient was given a reduced-intensity treatment regimen. The viral load in urine was found to be 107-9 copies/mL within 2 weeks before the development of HC and has been identified as a prognostic indicator. In conclusion, early diagnosis of viral infections by monitoring BKV viral load in HSCT patients will be effective in preventing the progression of complications such as BKV-associated HC by providing timely initiation of preemptive treatment.


Asunto(s)
Virus BK , Cistitis , Trasplante de Células Madre Hematopoyéticas , Infecciones por Polyomavirus , Humanos , Niño , Lactante , Cistitis/epidemiología , Cistitis/etiología , Factores de Riesgo , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Infecciones por Polyomavirus/etiología , Infecciones por Polyomavirus/complicaciones , Receptores de Trasplantes , Virus BK/genética , Hemorragia/epidemiología , Hemorragia/etiología
6.
JAMA Netw Open ; 6(5): e2312903, 2023 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-37219908

RESUMEN

Importance: Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed. Objective: To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees. Design, Setting, and Participants: This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated for cancer between January 1, 2011, and December 31, 2020, in hematology-oncology departments of 8 university hospitals in the Southern province of Turkey. Data were analyzed from May 1, 2022, to September 30, 2022. Main Outcomes and Measures: Demographic characteristics (date of birth, sex, and residence), date of first cancer-related symptom, date and place of diagnosis, disease status at first presentation, treatment modalities, date and status at last hospital visit, and date of death. The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision and International Classification of Childhood Cancers, Third Edition, were used for the classification of cancer. The Surveillance, Epidemiology, and End Results system was applied for staging. The diagnostic interval was defined as the number of days from first symptoms until the diagnosis. Treatment abandonment was documented if the patient did not attend the clinic within 4 weeks of a prescribed appointment throughout the treatment. Results: A total of 1114 Syrian adult and 421 Syrian children with cancer were included. The median age at diagnosis was 48.2 (IQR, 34.2-59.4) years for adults and 5.7 (IQR, 3.1-10.7) years for children. The median diagnostic interval was 66 (IQR, 26.5-114.3) days for adults and 28 (IQR, 14.0-69.0) days for children. Breast cancer (154 [13.8%]), leukemia and multiple myeloma (147 [13.2%]), and lymphoma (141 [12.7%]) were common among adults, and leukemias (180 [42.8%]), lymphomas (66 [15.7%]), and central nervous system neoplasms (40 [9.5%]) were common among children. The median follow-up time was 37.5 (IQR, 32.6-42.3) months for adults and 25.4 (IQR, 20.9-29.9) months for children. The 5-year survival rate was 17.5% in adults and 29.7% in children. Conclusions and Relevance: Despite universal health coverage and investment in the health care system, low survival rates were reported in this study for both adults and children with cancer. These findings suggest that cancer care in refugees requires novel planning within national cancer control programs with global cooperation.


Asunto(s)
Leucemia , Refugiados , Adulto , Niño , Humanos , Siria , Estudios Transversales , Estudios Retrospectivos , Turquía , Instituciones de Atención Ambulatoria , Hospitales Universitarios
7.
J Bone Oncol ; 35: 100435, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35664540

RESUMEN

Background: Treatment of Ewing sarcoma (ES) requires multidisciplinary approach and deficiencies in treatment adversely affect the results. This study included patients diagnosed with ES and aimed to determine the factors affecting prognosis and investigate the efficacy of replacing actinomycin-D with carboplatin in consolidation treatment. Methods: Eighty-two pediatric ES patients diagnosed at a single institution between 2005 and 2020 were retrospectively evaluated. Clinical and epidemiological features, treatment modalities, prognostic criteria, and overall survival (OS) rates of patients revieved. In consolidation treatment, 22 patients were treated with actinomycin-D and 32 patients with carboplatin (500 mg/m2/dose), 24 patients could not receive consolidation treatment. The 5- and 10-year OS rates of the patients were compared. Results: The 5- and 10-year OS rates of the 82 patients with ES were 46% and 40%, respectively. The 5-year OS rates in the group with localized disease (n = 55) and metastasis (n = 27) at diagnosis were 54% and 26%, respectively (p = 0.006). When evaluated according to the consolidation treatment administered both the 5- and 10-year OS rates of the patients receiving actinomycin-D were 50%. The 5-year OS rate was 58% in the carboplatin group, and the 5- and 10-year OS rates of patients that did not receive consolidation treatment was 20%. Conclusions: Survival was significantly worse in the group that did not receive consolidation treatment. Furthermore, our results suggested that carboplatin could be used effectively as an alternative to actinomycin-D in ES consolidation treatment.

8.
Med Princ Pract ; 31(6): 532-539, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35537400

RESUMEN

OBJECTIVE: Neuroblastoma is a common fatal tumor of childhood. Natural killer (NK) cells can exert direct cytotoxicity on tumor cells. The killer immunoglobulin-like receptor (KIR) family of NK cell receptors is involved in activation/inhibition of NK cells. In the KIR gene cluster, six of them (3DS1, 2DS1-5) encode receptors triggering activation, while seven of them (3DL1-3, 2DL1-3, 2DL5) encode receptors triggering inhibition. We aimed to assess the distribution of genetic polymorphisms of KIRs on the clinical course of neuroblastoma and provide guidance on potential therapeutic options. METHODS: Our study group included 50 neuroblastoma patients and 100 healthy children as controls. Twenty-eight patients were boys, and twenty-two were girls; median age was 36 months. Fourteen patients had stage 1, 2, 3, or 4S disease, and 36 patients had stage 4 disease. Isolated DNA from the peripheral blood was amplified for sequence-specific oligonucleotide probe analysis of 16 KIR genes. The Fisher's exact test was used to evaluate the variation of KIR gene distribution. RESULTS: All patients had a lower frequency of KIR2DS3 compared to the control group (p = 0.005). Evaluation of individual KIR genes/genotypes in patients with early stages (stage 1, 2, 3, and 4S) versus stage 4 disease revealed that the frequency of KIR2DS3 was increased in early stages (p = 0.023). Inhibitory KIR2DL3 was increased in the patient group compared to controls (p = 0.038). Furthermore, the frequency of KIR2DL3 was higher in stage 4 neuroblastoma patients compared to the patients with early stages (p = 0.023). CONCLUSION: Our data suggest a role for KIR2DS3 and KIR2DL3 in development of neuroblastoma. Thus, modulation of KIR2SD3 and/or KIR2DL3 expression or function might present a novel therapeutic strategy for neuroblastoma.


Asunto(s)
Neuroblastoma , Receptores KIR , Masculino , Femenino , Niño , Humanos , Preescolar , Receptores KIR/genética , Receptores KIR/metabolismo , Genotipo , Polimorfismo Genético/genética , Células Asesinas Naturales/metabolismo , Neuroblastoma/genética , Neuroblastoma/metabolismo , Frecuencia de los Genes , Receptores KIR2DL3/genética , Receptores KIR2DL3/metabolismo
9.
J Clin Apher ; 37(1): 19-24, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34694023

RESUMEN

BACKGROUND: Most studies investigating the impact of graft composition on transplant-related outcomes have focused on the effect of CD34+ cell dose and reported equivocal results. The aim of this study is to investigate the impact of doses of total nucleated cells (TNCs), total mononuclear cells (TMCs), CD3+, and CD34+ cells on the outcome of children receiving allogeneic hematopoietic stem cell transplantation (HSCT). METHODS: Children and adolescents who underwent allogeneic HSCT for malignant hemato-oncological diseases or nonmalignant diseases in Cukurova University Faculty of Medicine, Pediatric Bone Marrow Transplantation Center between 2010 and 2020 were enrolled in the study. RESULTS: A total of 212 patients receiving allogeneic HSCT (154 bone marrow transplantation; 58 peripheral blood stem cell transplantation) from matched related or unrelated donors were included in the study. Higher TNC doses associated with a superior 5-year event-free survival (EFS; 67.7% vs 44.7%) in the whole group (log-rank P = .027). Overall survival (OS) and EFS of bone marrow-transplanted patients differed significantly according to TNC doses (log-rank P = .041 and .027, respectively). Multivariant analysis for OS revealed a P value of .038 for TNC, Exp(B) = 1.939 (95% CI: [1.038, 3.621]). That for EFS revealed a P value of .025 for TNC, Exp(B) = 1.992 (95% CI: [1.088, 3.647]). There was no relationship between doses of CD34+ cells, CD3+ cells, TMC, TNC, and neutrophil or platelet engraftment. CONCLUSION: Our data suggest that TNC dose is a better prognostic factor for pediatric allogeneic HSCT outcomes than doses of CD34+ cells, CD3+ cells, or TMC in patients transplanted with bone marrow. Future studies analyzing cell subsets and other components in TNC could elaborate the factor(s) accompanying this observed survival advantage.


Asunto(s)
Antígenos CD34 , Trasplante de Médula Ósea , Complejo CD3 , Trasplante de Células Madre Hematopoyéticas , Adolescente , Antígenos CD34/biosíntesis , Complejo CD3/biosíntesis , Recuento de Células , Niño , Preescolar , Femenino , Humanos , Lactante , Leucocitos Mononucleares/metabolismo , Masculino , Pronóstico , Tasa de Supervivencia
10.
Turk J Pediatr ; 63(1): 11-22, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33686822

RESUMEN

BACKGROUND: Mucormycosis is a fatal invasive fungal infection seen most often in patients with compromised defense mechanisms. The aim of this article was to review the data of pediatric mucor in the South of Turkey. METHODS: Twenty pediatric cases with biopsy proven mucormycosis were reported, between January 2007 through January 2017. Data were extracted from the medical charts of patients retrospectively. RESULTS: Underlying conditions were hematological malignancy (75%), in whom 93% had acute leukemia, aplastic anemia (15%), diabetes mellitus (5%) and other malignancies (5%). The main sites of infection were sinus (85%); alone (29.4%) or with cerebral (17.6%), and orbital involvement (17.6%). Pulmonary involvement was reported in 11 patients (55%), two of them had the alone form and nine cases were associated with nasal sinus involvement. Disseminated mucormycosis was documented in 45%. Fever and pain/swelling of organs were the most commonly encountered signs and symptoms. Treatment compromised of am-photericin B monotherapy in five patients. All patients except one received liposomal formu-lations (LAmB). A combination of surgery and antifungal therapy was performed in 75%. Crude survival was 55%; among 15 cases treated with a combination of surgery and antifun-gal therapy, survival rate was 8/15 (53%). The overall mortality rate was high in patients diagnosed with disseminated infection (100%). CONCLUSIONS: Mucormycosis in pediatric cases requires a high index of suspicion and urgent evaluation of clinical samples. Surgical debridement should be considered when feasible. Initial medical therapy should include an amphotericin preparation with or step-down to posaconazole.


Asunto(s)
Mucormicosis , Anfotericina B , Antifúngicos/uso terapéutico , Niño , Humanos , Mucormicosis/diagnóstico , Mucormicosis/epidemiología , Mucormicosis/terapia , Estudios Retrospectivos , Turquía/epidemiología
11.
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
12.
J Pediatr Hematol Oncol ; 42(8): e738-e744, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32925403

RESUMEN

Febrile neutropenia (FEN) is a significant side effect after chemotherapy, and it is known that using granulocyte colony-stimulating factor (G-CSF) has positive effects on treatment results. In this study, the effects of different G-CSF doses (5 to 10 mcg/kg/day) on treatment results in patients with high-risk FEN were evaluated. A total of 124 high-risk FEN episodes of 62 patients were enrolled in the study between June 2017 and October 2018. The episodes were divided into 2 groups according to G-CSF treatment doses, they received from 5 to 10 mcg/kg/day. The clinical characteristics of the patients, the treatments they received, laboratory findings, microbiologic results, and cost analysis were recorded. No statistically significant difference was found between 2 groups in terms of the mean duration of recovery from neutropenia, duration of fever, total length of hospital stay, duration of FEN episode, duration of G-CSF use, costs, bacteremia frequency, and other treatments. In patients with solid tumors, the cost of filgrastim was significantly higher in the high-dose G-CSF group. Using different doses of G-CSF in high-risk FEN episodes did not show any different effects on clinical and treatment results. The dose of 5 mcg/kg/day would be more appropriate in FEN treatment.


Asunto(s)
Neutropenia Febril/tratamiento farmacológico , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Tiempo de Internación/estadística & datos numéricos , Neoplasias/complicaciones , Adolescente , Niño , Preescolar , Relación Dosis-Respuesta a Droga , Neutropenia Febril/etiología , Neutropenia Febril/patología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento
13.
Leuk Lymphoma ; 61(6): 1465-1474, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32037917

RESUMEN

We examined outcomes of 51 pediatric patients with relapsed acute leukemia (AL) who underwent a second allogeneic hematopoietic stem cell transplantation (alloHSCT). After a median follow-up of 941 days (range, 69-2842 days), leukemia-free survival (LFS) and overall survival (OS) at 3 years were 26.6% and 25.6%, respectively. The nonrelapse mortality rate (NMR) and cumulative incidence of relapse (CIR) were 36.4% and 42.4%, respectively. The Cox regression analysis demonstrated that the risk factors at second transplantation for predicting limited LFS were active disease (hazard ratio (HR) = 5.1), reduced intensity conditioning (RIC) (HR = 5.0), matched unrelated donor (MUD) (HR = 3.4) and performance score <80 (HR = 3.2). Pediatric patients with AL who relapsed after their first alloHSCT may survive with a second alloHSCT. Disease status, conditioning intensity, donor type, and performance score at the second transplantation are the relevant risk factors. A score based on these factors may predict the results of the second transplantation.


Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Leucemia Mieloide Aguda , Trasplante de Médula Ósea , Niño , Enfermedad Injerto contra Huésped/etiología , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Leucemia Mieloide Aguda/terapia , Recurrencia , Estudios Retrospectivos , Acondicionamiento Pretrasplante , Trasplante Homólogo , Donante no Emparentado
14.
J Pediatr Hematol Oncol ; 42(3): 204-207, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-31335823

RESUMEN

Unicentric Castleman disease (CD) is a rare lymphoproliferative disorder that is characterized by the enlargement of lymph nodes on the neck, mediastinum, and retroperitoneum. Herein, we present a 6-year-old female patient, referred to our medical center because of recurrent fever accompanied by cervical lymphadenopathy and elevated inflammatory markers since 3 years of age. Fever episodes lasting 1 day continued irregularly without any accompanying symptom. MEditerranean FeVer (MEFV) gene analysis showed no mutations; however, as inflammatory markers including serum amyloid A remained markedly high during attack-free periods, colchicines was initiated. The patient did not respond to maximally tolerated doses of colchicine; therefore, we added canakinumab and systemic methylprednisolone, subsequently. Unresponsiveness to 3 doses of bimonthly canakinumab and new-onset hepatosplenomegaly led us to investigate large-vessel vasculitis and malignancy; therefore, we performed Position emission tomography, which further revealed a hypermetabolic retroperitoneal solid mass. After performing the excisional biopsy, the patient has been diagnosed as suffering from hyaline vascular variant CD, confirmed by histopathology. In conclusion, we report a pediatric unicentric CD, which resembled autoinflammatory diseases and responded well to surgical resection, with the normalization of inflammatory markers 1 month after the procedure. CD, even the unicentric and hyaline vascular variant, should be considered in the differential diagnosis of the patients with an autoinflammatory phenotype.


Asunto(s)
Enfermedades Autoinmunes/diagnóstico , Enfermedad de Castleman/diagnóstico , Fiebre/etiología , Enfermedad de Castleman/patología , Niño , Diagnóstico Diferencial , Femenino , Humanos
15.
J Clin Res Pediatr Endocrinol ; 10(3): 198-205, 2018 07 31.
Artículo en Inglés | MEDLINE | ID: mdl-29553046

RESUMEN

OBJECTIVE: In this study, we evaluated the frequency of euthyroid sick syndrome (ESS) among patients with childhood cancer and its association with the stage of disease, nutritional parameters and cytokines levels. METHODS: Eighty newly diagnosed children were included in the study. ESS was assessed in two different ways. According to criteria 1 ESS was present if free triiodothyronine (fT3) was below the lower limit and free thyroxine was within the normal or low limits, thyroid-stimulating hormone (TSH) was in the normal range. According to criteria 2, in addition to the above, it was required that reverse triiodothyronine (rT3) be performed and was higher than normal limits. RESULTS: Three of our pediatric patients had subclinical hypothyroidism and two had subclinical hyperthyroidism. Out of 75 patients, ESS was identified in 14 (17.3%) according to criteria 1 and in eight (10.6%) according to criteria 2. Only fT3 levels were significantly different in the ESS (+) and ESS (-) groups (p<0.05) according to criteria 1. A significantly negative correlation between interleukin (IL)-6 and fT3 was found, according to both sets of criteria. tumor necrosis factor alpha was negatively correlated with fT3 levels only in the criteria 1 group. There were no correlations between IL-1ß and fT3, free thyroxine, rT3 and TSH levels. CONCLUSION: ESS may occur in childhood cancer and thyroid function testing should be performed routinely when cancer is diagnosed.


Asunto(s)
Síndromes del Eutiroideo Enfermo/complicaciones , Síndromes del Eutiroideo Enfermo/epidemiología , Neoplasias/complicaciones , Adolescente , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Incidencia , Lactante , Masculino
16.
Turk J Pediatr ; 60(6): 660-668, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-31365202

RESUMEN

Çiftçi AÇ, Küpeli S, Sezgin G, Bayram I. Evaluation of pediatric patients with an oncologic emergency: Single center experience. Turk J Pediatr 2018; 60: 660-668. In this study, we aimed to prospectively analyze the emergency diagnosis and clinical characteristics of the oncologic emergency patients admitted, as well as to evaluate the approach considerations to the cases, their treatment, patient outcomes and the prognostic factors. A total of 88 oncologic emergency patients (90 oncologic emergency hospitalizations), except for febrile neutropenia, were admitted to the pediatric oncology department between July 2014 and December 2015. A total of 30 cases of tumor lysis syndrome, 19 hyperleukocytosis, 11 convulsion, 7 spinal cord compression, 7 hemorrhagic cystitis, 9 pleural effusion, 6 vena cava superior syndrome, 4 typhlitis, 3 anuria, 4 gastrointestinal bleeding, 3 hypercalcemia, 4 acute respiratory distress syndrome, 2 acute massive hepatomegaly and 1 increased intracranial pressure were identified. Sixty-five (72.2%) of the study patients had a primary oncologic disease, whereas 25 (27.8%) had a recurring condition. The most common presenting complaint was fever (23.3%) followed by breathing difficulties (22.2%). Fifty-nine patients (65.6%) recovered and were discharged home, while 31 cases (34.4%) died. Tumor lysis syndrome was the most frequently encountered emergency condition in the pediatric oncology patient group. Oncologic emergencies need to be approached quickly and systematically in the light of current scientific knowledge.

17.
Tumori ; 104(5): 381-387, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-28315509

RESUMEN

BACKGROUND:: We aimed to establish the early diagnosis of cerebral vascular complications by using cerebral magnetic resonance angiography (MRA) in patients who were treated with cranial RT in childhood as part of their cancer treatment. PROCEDURE:: Patients who had received cranial RT before the age of 18 and had been in remission for at least 1 year were enrolled in the study. A data form including demographic and clinical characteristics and findings of cerebral MRA was filled in for each patient. RESULTS:: Cerebral MRA examination was performed between November 2013 and October 2015 in 53 patients who met the inclusion criteria. Abnormalities were found in 7 patients (13.2%). All patients were asymptomatic at the time of examination. There was a significant difference between patients in the abnormality-positive and abnormality-negative groups related to cranial radiation dose (p = 0.013) and age at the time of examination (p = 0.015) in univariate analysis. In multivariate analysis, cranial radiation dose was found to have an impact on developing cerebral vascular abnormalities (p = 0.045). CONCLUSIONS:: Cerebral MRA is a noninvasive method of follow-up for late cerebral vascular complications in surviving pediatric oncology patients who were treated with cranial RT as part of their cancer treatment.


Asunto(s)
Trastornos Cerebrovasculares/patología , Irradiación Craneana/efectos adversos , Angiografía por Resonancia Magnética/métodos , Neoplasias/complicaciones , Neoplasias/radioterapia , Adolescente , Estudios de Casos y Controles , Trastornos Cerebrovasculares/diagnóstico por imagen , Trastornos Cerebrovasculares/etiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Pronóstico , Estudios Prospectivos
18.
Mikrobiyol Bul ; 51(4): 370-377, 2017 Oct.
Artículo en Turco | MEDLINE | ID: mdl-29153067

RESUMEN

Influenza virus infections are extremely important for human health due to the occurence of seasonal epidemics and pandemics worldwide. Influenza is associated with high morbidity and may result in serious complications such as life threatening viral or bacterial pneumonia. Especially, young children, older adults, patients with chronic diseases such as heart, lung, kidney, and diabetes and immunosuppressed people are at higher risk for complications and death from influenza virus infections. The aim of this study was to determine the incidence of influenza type A and B virus infections and influenza A virus subtypes in hospitalized patients with respiratory tract infections by real-time reverse transcriptase-polymerase chain reaction (RT-PCR, Sacace, Italy), conventional RT-PCR and direct immunofluorescence antibody (DFA, Argene SA, France) tests. Nasopharyngeal swab specimens were collected from a total of 476 patients with respiratory tract symptoms by using flocked swabs (Copan Diagnostics, Italy) between 1 April 2012 and 31 December 2013. Influenza A virus was detected in 20.5% (98/476) and influenza B virus in 3.3% (16/476) of the cases by real-time RT-PCR test. During the study period, 63.3% of 98 influenza virus isolates were found as influenza A(H1N1)pdm09 and 36.7% were influenza A(H3N2) subtypes. Influenza A (H1N1) pdm09 subtype was observed in 12 cases in January 2013 and influenza A(H3N2) subtype was observed in 11 cases in December 2013 as the highest values. When the real-time RT-PCR test was regarded as the reference test, the sensitivities of DFA test for influenza A and B and conventional RT-PCR test with WHO primers (M30F2/08 and M264R3/08) for influenza A were detected as 72.4%, 75%, 96% and the specificities were detected as 99.2%, 99.5% and 100%, respectively. In conclusion, influenza A virus infection was detected rather high with a rate of 20.5% in the study group. The monitoring of influenza virus types and subtypes is required for the evaluation of influenza vaccine strains and circulating influenza viruses and for the identification of subtypes with pandemic potential. Planning for appropriate antiviral therapy using real-time RT-PCR in the early diagnosis of influenza virus infections will significantly contribute to the management of the patient's treatment. Thus, unnecessary drug use will be prevented and controlled with effective treatment of the disease at the time of infection.


Asunto(s)
Virus de la Influenza A , Virus de la Influenza B , Gripe Humana/diagnóstico , Técnica del Anticuerpo Fluorescente Directa , Humanos , Incidencia , Subtipo H1N1 del Virus de la Influenza A/genética , Subtipo H1N1 del Virus de la Influenza A/inmunología , Subtipo H1N1 del Virus de la Influenza A/aislamiento & purificación , Subtipo H3N2 del Virus de la Influenza A/genética , Subtipo H3N2 del Virus de la Influenza A/inmunología , Subtipo H3N2 del Virus de la Influenza A/aislamiento & purificación , Virus de la Influenza A/genética , Virus de la Influenza A/inmunología , Virus de la Influenza A/aislamiento & purificación , Virus de la Influenza B/genética , Virus de la Influenza B/inmunología , Virus de la Influenza B/aislamiento & purificación , Gripe Humana/epidemiología , Nasofaringe/virología , Reacción en Cadena en Tiempo Real de la Polimerasa , Estudios Retrospectivos , Estaciones del Año
19.
J Pediatr Hematol Oncol ; 39(6): e336-e337, 2017 08.
Artículo en Inglés | MEDLINE | ID: mdl-28538505

RESUMEN

Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major. To our knowledge this is the second publication reporting the association between thalassemia major and priapism in childhood.


Asunto(s)
Priapismo/etiología , Talasemia beta/complicaciones , Adolescente , Humanos , Hipodermoclisis , Ibuprofeno/uso terapéutico , Masculino , Enfermedades del Pene , Priapismo/terapia , Talasemia beta/terapia
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