La Unidad de Oncología Pediátrica del Hospital La Fe en 2013 / The Pediatric Oncology Unit of the Hospital la Fe in 2013

Tras un breve recorrido histórico y tras señalar la misión, visión y valores de la Unidad de Oncología Pediátrica, se describe la estructura de la unidad, recursos estructurales y humanos y su actividad asistencial, docente y de investigación. En referencia a la labor asistencial cabe destacar su atención universal en régimen ambulatorio (consulta externa y hospitalización a domicilio), de hospitalización y de urgencia, incluyendo todos los tratamientos disponibles en Oncología Pediátrica en todas las fases de la enfermedad (desde el diagnóstico hasta la terminal en su caso o en la prevención o rehabilitación de efectos adversos a largo plazo. Su labor en docencia e investigación es también destacable (AU)

After a short history and to point out objectives and care level of Pediatric Oncology Unit, we describe structural and human resources of the Unit and its clinical, investigational and teaching activity. Concerning clinical, activity, we would like to highline the global dedication in outpatient and impatient regimen including home care assistance. We also remark out disposition to offer all types of treatments with known efficacy in children with cancer, including every phase of the disease and late effects (AU)

Reacción de fototoxicidad por metotrexato / Photoxicity reaction due to methotrexate

No disponible

Observational prospective study of viral infections in children undergoing allogeneic hematopoietic cell transplantation: a 3-year GETMON experience.

We studied surveillance, incidence and outcome of viral infections in children undergoing allogeneic hematopoietic cell transplantation (HCT) in the main pediatric transplant units in Spain. We prospectively collected data from first year post-HCT in every consecutive allogeneic HCT performed during 3 years (N = 215): first HCT = 188 and second HCT = 27; median age = 6.6 years (0.1-20.7). Most patients had acute leukemia (N = 137) and 135 recipients (63%) were CMV seropositive. A total of 46 patients underwent cord blood transplant, 133 patients underwent HCT from alternative donors (62%) and 101 patients received anti-thymocyte globulin. Observational time was completed in 137 patients, whereas the remaining 78 died after a median survival time of 99 days (3-352). CMV was monitored in all patients; adenovirus (ADV) and human herpesvirus 6 (HHV-6) were monitored in 101 and 33 patients, respectively. We found 145 viral infections in 103 patients: CMV (n = 42), ADV (n = 32), HHV-6 (n = 7), polyomavirus (n = 20), EBV (n = 6), VZV (n=17) and others (n = 8). CMV infection was significantly higher in seropositive patients (25 vs 7%) (P = 0.02). Extensive chronic GVHD (cGVHD) was significantly associated with an increased rate of viral infections (12 of 16 patients with cGVHD had infections vs 91 of 199 without GVHD) (P = 0.035). In total, 10 patients (4.6%) died of viral infections (CMV = 5, ADV = 3, respiratory = 2). We found a high incidence of viral infection, but mortality was low.

Evaluation of chimerism by quantitative PCR analysis of DNA polymorphism after allogeneic hematopoietic stem cell transplantation in a pediatric population with malignancies.

Relapse remains the major pitfall to success for Allo-HSCT in children with malignancies. Ninety-one patients undergoing Allo-HSCT were retrospectively reviewed. Chimerism status was evaluated at days +30, +60, and +100 in PB. VNTR-PCR and STR-PCR were used for this purpose. Thirty-one patients recurred (34%) and none survived. Thirty-two remain alive in CR (35%). Patients who achieved a CC at those days had a significant higher RFS and OS than patients who did not. Twelve patients showing PMC had an increased risk of recurrence (p=0.02. OR 7.7). In the univariate analysis, the probability of death was higher in patients who were not in first CR before transplant (p=0.008.OR 2.09) and in those receiving cells not from PB (p=0.002.OR 2.03). In the multivariate analysis, the absence of CC at day +100 was associated with a higher probability of relapse (p=0.004. OR 10.8) and death (p=0.016. OR 9.3). Serial chimerism PCR-based analyses of polymorphic DNA markers can predict relapse. Patients with PMC are at the highest risk of recurrence. Patients receiving an Allo-HSCT in first CR from PB who achieve a CC at day +100 have a better outcome.

Study of broadband near-infrared emission in Tm3+-Er3+ codoped TeO2-WO3-PbO glasses.

In this work, we report the near-infrared emission properties of Tm(3+)-Er(3+) codoped tellurite TeO(2)-WO(3)-PbO glasses under 794 nm excitation. A broad emission from 1350 to 1750 nm corresponding to the Tm(3+) and Er(3+) emissions is observed. The full width at half-maximum of this broadband increases with increasing [Tm]/[Er] concentration ratio up to a value of ~ 160 nm. The energy transfer between Tm(3+) and Er(3+) ions is evidenced by both the temporal behavior of the near-infrared luminescence and the effect of Tm3+ codoping on the visible upconversion of Er(3+) ions.

Diagnosis of paediatric anaplastic large-cell lymphoma: a historical perspective from a single institution.

INTRODUCTION: Anaplastic large-cell lymphoma (ALCL) is an infrequent childhood malignancy whose diagnosis and treatment have largely evolved since its initial description in 1985. PATIENTS AND METHODS: We retrospectively reviewed our experience in the field, and report here a single institution experience focusing on diagnostic and therapeutic milestones achieved as novel tools have been developed. This is a series of 9 children diagnosed from 1987 to 2007. RESULTS: Our first patient was diagnosed shortly alter this entity was described based on morphology and Ki-1 positivity, while the diagnostic work-up for the last two children included accurate molecular diagnosis for ALK-NPM rearrangement. Despite a wide variety of multimodal therapies used over time, only one patient died of toxicity during progression and another child relapsed and survived alter an autograft. After 156 months of median follow-up (range 4-245), 8 out of 9 children are alive, free of disease. CONCLUSIONS: Our series exemplifies the long journey travelled from the definition of a new entity only 20 years ago to the molecular characterization not only with diagnostic but also therapeutic purposes. Besides this, significant efforts are being made to recruit all European patients into a multinational collaborative trial in order to start drawing major evidence-based conclusions.

Diagnosis of paediatric anaplastic large-cell lymphoma: a historical perspective from a single institution

INTRODUCTION: Anaplastic large-cell lymphoma (ALCL) is an infrequent childhood malignancy whose diagnosis and treatment have largely evolved since its initial description in 1985. PATIENTS AND METHODS: We retrospectively reviewed our experience in the field, and report here a single institution experience focusing on diagnostic and therapeutic milestones achieved as novel tools have been developed. This is a series of 9 children diagnosed from 1987 to 2007. RESULTS: Our first patient was diagnosed shortly alter this entity was described based on morphology and Ki-1 positivity, while the diagnostic work-up for the last two children included accurate molecular diagnosis for ALK-NPM rearrangement. Despite a wide variety of multimodal therapies used over time, only one patient died of toxicity during progression and another child relapsed and survived alter an autograft. After 156 months of median follow-up (range 4-245), 8 out of 9 children are alive, free of disease. CONCLUSIONS: Our series exemplifies the long journey travelled from the definition of a new entity only 20 years ago to the molecular characterization not only with diagnostic but also therapeutic purposes. Besides this, significant efforts are being made to recruit all European patients into a multinational collaborative trial in order to start drawing major evidence-based conclusions (AU)

Spectroscopic properties of the 1.4 microm emission of Tm3+ ions in TeO2-WO3-PbO glasses.

In this work, we report the spectroscopic properties of the infrared 3H4-->3F4 emission of Tm3+ ions in two different compositions of glasses based on TeO2, WO3, and PbO for three Tm2O3 concentrations (0.1,0.5, and 1 wt%). Judd-Ofelt intensity parameters have been determined and used to calculate the radiative transition probabilities and radiative lifetimes. The infrared emission at around 1490 nm corresponding to the 3H4-->F4 transition has two noticeable features if compared to fluoride glasses used for S-band amplifiers. On one hand, it is broader by nearly 30 nm, and on the other, the stimulated emission cross section is twice the value for fluoride glasses. Both the relative intensity ratio of the 1490 nm emission to 1820 nm and the measured lifetime of the 3H4 level decrease as concentration increases, due to the existence of energy transfer via cross-relaxation among Tm3+ ions. The analysis of the decays from the 3H4 level with increasing concentration indicates the presence of a dipole-dipole quenching process assisted by energy migration.

Tumores renales en niños menores de un año / Renal tumors in infants aged less than 1 year

Objetivo: Conocer la frecuencia y distribución de los tumores renales diagnosticados en una unidad de oncología pediátrica en niños menores de un año, sus características clínicas, anatomopatológicas, tratamientos utilizados y evolución. Material y métodos: Estudio retrospectivo de tumores renales primarios diagnosticados en lactantes en un hospital pediátrico, desde 1972 hasta febrero de 2003. Resultados: Obtuvimos 25 niños menores de un año (18,2 %), de un total de 137 tumores renales registrados. Diecisiete niños y 8 niñas. El más frecuente fue el nefroblastoma (15/25), seguido del nefroma mesoblástico (9/25), y uno fue un tumor rabdoide. La media de edad al diagnóstico fue de 4,8 meses (rango, 1 día-11 meses), la mediana de 5,03 meses; para los nefromas mesoblásticos la mediana fue de 1 día (rango, 1 día-3 meses). La forma de presentación fue como masa abdominal en 20 de ellos, en cuatro hematuria y un recién nacido empezó con obstrucción intestinal (nefroma mesoblástico). Presentaron hipertensión arterial 12/25 niños; 9/15 casos de nefroblastomas eran estadio I; uno, estadio II; uno, estadio III; dos, estadio IV; uno, estadio V, y uno falleció antes de la cirugía. La supervivencia global de los nefroblastomas a los 5 años es de 0,67 (error estándar [EE]: 0,12); y del nefroma mesoblástico de 0,89 (EE: 0,1), con un tiempo de supervivencia media de 290 meses. Conclusiones: En los niños menores de 6 meses el nefroma mesoblástico es más frecuente que el tumor de Wilms, siendo el tratamiento inicial de elección la cirugía, ya que este tipo de tumor es poco quimiosensible y la quimioterapia es peor tolerada en los lactantes

Objective: To determine the frequency and distribution of primary renal tumors diagnosed in a pediatric oncology unit in children younger than 1 year and identify their clinical and histopathological characteristics, the treatment used, and outcomes. Material and methods: We retrospectively reviewed the medical records of infants with primary tumors of the kidney diagnosed between January 1972 and February 2003. Results: A total of 137 tumors were diagnosed in our unit during the period studied. Of these, 25 (18.2 %) occurred in infants aged less than 1 year. There were 17 boys and 8 girls. The most prevalent tumor in this age group was Wilms' tumor (WT) in 15 patients, followed by mesoblastic nephroma (MN) in 9 patients and rhabdoid tumor in 1 patient. The mean age at diagnosis of WT was 4.8 months (range: 1 day-11 months), with a median of 5.03 months. The median age at diagnosis of MN was 1 day (range: 1 day-3 months). Presenting symptoms consisted of abdominal mass in 20 patients, hematuria in 4 patients and intestinal pseudo-occlusion (MN) in 1 patient. High blood pressure was found in 12 of the 25 patients. Among the 15 WT, 9 were stage I, 1 was stage II, one was stage III, 2 were stage IV, and 1 was stage V. One patient died before surgery. Overall survival at 5 years was 0.67 (SE 0.12) for WT and 0.89 (SE 0.1) for MN, respectively, with a mean follow-up of 290 months. Conclusions: MN was more frequent than WT in infants aged less than 6 months. The first-line therapy in these patients is surgery since this type of tumor shows little chemosensitivity and chemotherapy is poorly tolerated in infants

Relación de la Unidad de Oncología Pediátrica con Atención Primaria: el papel de la Unidad de Hospital d Domicilio / Relation of the Pediatric Oncology Unit with Primary Care: the role of the home care unit

Las unidades de Oncología Pediátrica cubren habitualmente una extensa área geográfica. Las modalidades de alternativas de hospitalización, como el hospital a domicilio (HaD), requieren resolver el problema de la dispersión geográfica para ofrecer cobertura asistencial a todos los pacientes, con independencia de su lugar de domicilio. Gracias al amplio desarrollo de la Atención Primaria Pediátrica en nuestra Comunidad y a la implantación de un sistema de comunicación fluida bidireccional (Atención Primaria Unidad de Oncología) a través de la unidad de HaD, hemos establecido un cauce de actuación clínica, delegado en los equipos de Atención Primaria, cuando la distancia impide al personal de la Unidad de Oncología realizar la asistencia de forma directa. Así, la asistencia del niño con cáncer se efectúa en algunos puntos del proceso terapéutico por el pediatra de cabecera, contando con el respaldo del servicio de Oncología en cualquier momento (equipo de guardia localizada). Este aspecto es importante en todas las etapas de la enfermedad, pero especialmente trascendental y significativo en la fase terminal

In our country, Pediatric Oncology Units are reference centers for a wide geographic area. Alternatives to traditional hospitalization, as home care, must sort out the problem of the geographical dispersion of the patients in order to give medical attention to all of them independently from the distance home - hospital Primary care in Pediatrics is very well developed in our community. The creation of a bidirectional channel of communication between primary care teams and the hospital, through the home care unit from the hospital, allows to establish a network able to bring medical attention to patients living far from the hospital. Primary care team members directly carry on the clinical control of the patient at home, supported by the on call members of the home care team. This aspect is very important in every phase of the disease and specially relevant in the field of palliative care

[Evaluation of a home care program for children with cancer]. / Evaluación de un programa de hospitalización a domicilio para niños con cáncer.

The home care team dependent from the pediatric oncology unit in our institution started working in April, 1997. We evaluate in this paper the medical activities accomplished in seventeen month experience. The team is constituted by a pediatric oncologist, two pediatric nurses and a clinical assistant with experience in the specialty. The geographic area we cover is la Communidad Valenciana. We directly attend children living in Valencia city and its metropolitan area. For the rest of patients, we coordinate the interventions of the local primary care teams and local hospitals. 127 patients have been admitted in the home care unit in 433 occasions. The immediate reasons for the admission were: early discharge from the hospital (61%), followed by the administration of antibiotics (18%) and chemotherapy (12%) at home. We attended 17 children in the terminal phase of their diseases. Five of them required opioid treatment for pain control. Six out of eight patients living in the area of direct intervention of the home care team died at home. The most common cause of discharge (73%) was the achievement of the goals planned when the patient was included in the program. Only in two cases (0.5%) we did not found enough cooperation from the parents and the treatment was completed in the hospital. This program has been well accepted by our patients and their parents and permits to shorten the stay in the hospital.

Evaluación de un programa de hospitalización a domicilio para niños con cáncer / Evaluation of a home care program for children with cancer

La hospitalización a domicilio para niños con cáncer empezó a funcionar en la Unidad de Oncología Pediátrica (UOP) del Hospital Infantil La Fe en abril de 1997. Recogemos en este trabajo la asistencia realizada en los primeros 17 meses de experiencia. El equipo responsable, integrado en la UOP, está constituido por un oncólogo pediatra, dos enfermeras y una auxiliar con amplia experiencia en la especialidad. El área de cobertura geográfica abarca la totalidad de la Comunidad Valenciana. Atendemos de forma directa a los pacientes residentes en el área metropolitana de la ciudad de Valencia; en el resto, la asistencia es delegada, en coordinación con los equipos de atención primaria y hospitales comarcales. Un total de 127 pacientes han sido ingresados en hospitalización a domicilio en 433 ocasiones. El motivo de ingreso más frecuente fue el alta hospitalaria precoz (61 por ciento), seguido por la administración intravenosa de antibióticos (18 por ciento) o quimioterapia (12 por ciento) en el domicilio. Diecisiete pacientes fueron atendidos en sus casas durante la fase terminal de la enfermedad, para tratamiento paliativo. Cinco de ellos precisaron tratamiento con opioides para controlar adecuadamente el dolor. Seis de los 8 pacientes residentes en el área metropolitana de Valencia fallecieron en sus domicilios. La causa más frecuente de alta del programa fue el logro de las metas programadas al ingreso (73 por ciento). Sólo en 2 casos (0,5 por ciento) no hubo cooperación por parte de los padres y el tratamiento se completó en el hospital. Este programa es bien aceptado por nuestros pacientes y sus familias y permite acortar la estancia en el hospital (AU)

[Central nervous system tumors in children less than three years of age]. / Tumores de sistema nervioso central en niños menores de 3 años.

OBJECTIVE: Central nervous system (CNS) tumors are the most frequent solid tumors in children. Twelve to twenty percent are diagnosed in patients younger than two years of age and these patient present more morbidity and mortality due to the illness and the treatment itself. PATIENTS AND METHODS: A retrospective study of CNS tumors in children younger than three years of age diagnosed in our hospital between 1985 and 1995 was carried out. RESULTS: We treated 21 patients between 1985 and 1995. There were 10 male and 11 females. The mean age was 20.3 months (range: 0-32 months). The mean time between symptoms and treatment was 2.4 months (range: 0-18 months). The most common symptoms included ataxia, nausea and vomits. The most common locations of the tumor were: infratentorial (57.1%) and supratentorial (38.1%). Complete surgery was performed in 3 patients, subtotal in 10, partial in 5, and a biopsy in 2. The anatomical-pathological diagnosis was: astrocytoma (6), ependinoma (5), meduloblastoma (4), ganglioglioma (1), neuroblastoma (1), and primitive neuroectodermic tumor (1). We could not document the histology in 3 patients. Ten patients received chemotherapy that was well tolerated and 14 received radiotherapy whose sequels were updated. The mean follow-up period was 44.42 months (range: 0-136 months). Overall survival was 42.86%. There were no statistically significant differences in survival between those who were irradiated and those who were not, nor between those with supra-or infratentorial tumors. CONCLUSIONS: CNS tumors in children younger than three years of age have a worse prognosis than in older children. New therapeutic schedule with chemotherapy are being tested to avoid radiotherapy side-effects.

[Acute lymphoblastic leukemia: clinical data and results of intensive chemotherapy]. / Leucemia no linfoblástica aguda: datos clínicos y resultados de una quimioterapia intensiva.

Twenty-one children, between eight months and twelve years old, diagnosed as having acute nonlymphoblastic leukemia (ANLL), were treated with an intensive chemotherapy based on a modified VAPA protocol. Complete remission rate was 80%, and the 5 year-survival rate is 46%. One chloroma, one central nervous system, and four bone marrow relapses were observed; all except the first having an unfavourable outcome. Significant risk prognostic factors in this series proved to be hepatomegaly (greater than 5 cm) and delay in obtaining remission. No fatal complications were observed.

[Systemic candidiasis in children with cancer]. / Candidiasis sistémicas en niños con cáncer.

Systemic fungal infections have long been recognized in terminally ill patients with cancer. Systemic candidiasis is the most common, the incidence having increased in the last few years. Eight children diagnosed as having systemic candidiasis during a two-year period (1987-1988) are presented. Three had an isolated fungal pneumonitis, two an hepatosplenic candidiasis, one a multisystemic involvement (hepatosplenic, pulmonary and nodular skin lesions) and the remaining two patients had a Candida sepsis with no visceral lesions having been documented. All patients had neutropenia and prolonged fever no responsive to broad spectrum antibiotics. We would like to underline the importance of an early and prolonged antifungal therapy, especially in hepatosplenic candidiasis, in order to obtain the cure.