Clinical practice update of antifungal prophylaxis in immunocompromised children.

Due to the rise in the number and types of immunosuppressed patients, invasive fungal infections (IFI) are an increasing and major cause of morbidity and mortality in immunocompromised adults and children. There is a broad group of pediatric patients at risk for IFI in whom primary and/or secondary antifungal prophylaxis (AFP) should be considered despite scant evidence. Pediatric groups at risk for IFI includes extremely premature infants in some settings, while in high-risk children with cancer receiving chemotherapy or undergoing haematopoietic stem cell transplantation (HCT), AFP against yeast and moulds is usually recommended. For solid organ transplanted, children, prophylaxis depends on the type of transplant and associated risk factors. In children with primary or acquired immunodeficiency such as HIV or long-term immunosuppressive treatment, AFP depends on the type of immunodeficiency and the degree of immunosuppression. Chronic granulomatous disease is associated with a particular high-risk of IFI and anti-mould prophylaxis is always indicated. In contrast, AFP is not generally recommended in children with long stay in intensive care units. The choice of AFP is limited by the approval of antifungal agents in different age groups and by their pharmacokinetics characteristics. This document aims to review current available information on AFP in children and to provide a comprehensive proposal for each type of patient.

Sección de Oncología y Hematología Pediátricas del Hospital General Universitario Gregorio Marañón / Pediatric Oncology and Hematology Area of the Hospital General Universitario Gregorio Marañón

La Unidad de Oncología y Hematología Infantil del Hospital Gregorio Marañón comenzó su andadura en los años 70, presentando desde entonces un crecimiento progresivo y una modernización acorde con la evolución de la propia especialidad. En esta monografía se describe la organización de la sección, así como los recursos estructurales, las características del trabajo asistencial, la actividad docente e investigadora y la participación en diversos grupos de trabajo colaborativos o multidisciplinares. Se destaca la capacidad de abordaje integral de este tipo de patologías en todas las fases de las mismas, desde el diagnóstico al tratamiento, sin Olvidar el aspecto psicosocial o la atención paliativa en su fase terminal, si fuera necesario. En conjunto, se dibuja un cuadro que es una obra coral de muchos profesionales sanitarios (personal médico, psicooncología, enfermería, auxiliares...) y no sanitarios, pero cuyo tema principal es proporcionar la mejor asistencia posible al niño y a su familia (AU)

The Pediatric Oncology and Hematology Unit at the Gregorio Marañón Children's Hospital began its activity in the 705, presenting since then a progressive growth and modernization in accordance with the evolution of the specialty itself… In this paper we describe the organization of the section, our structural resources, the characteristics of care work, teaching and research activities and our participation in various collaborative or multidisciplinary work groups. It is remarkable the ability to comprehensively address this type of pathologies in its different phases, from diagnosis to treatment, without forgetting to mention the psychosocial aspect or palliative care in its terminal phase, if necessary. Altogether, a choral picture is drawn with the work of many health professionals (medical, psycho-oncology, nursing, assistants …) and non—health, but the main theme is to provide the best care for the child and his family (AU)

Evaluación de la densidad mineral ósea en pacientes con enfermedad de células falciformes / Evaluation of bone mineral density in children with sickle cell disease

OBJETIVO: Evaluar la densidad mineral ósea (DMO) en niños con enfermedad de células falciformes (ECF) de la Comunidad de Madrid. MATERIAL Y MÉTODOS: Se valora la DMO en un total de 40 niños con ECF y rango de edad entre 3-16 años, mediante densitometría (DEXA) siguiendo las recomendaciones de la Sociedad Internacional de Densitometría Clínica (ISCD). RESULTADOS:La edad media en el momento del estudio fue de 7,97 ± 3,95 años; el valor medio de la DEXA expresado en Z-score es de -0,91 ± 1,46 con un rango de valores mínimo de -5,30 y máximo de 2,30. Un 57,5% de los niños tiene DMO normal (Z > -1), un 25% tienen DMO baja (Z entre -1 y -2) y un 17,5% presentan Z-score patológico con valores de osteoporosis (Z-score<-2). Los estudios de correlación solo encuentran una correlación lineal de Pearson significativa estadísticamente entre valor de Z-score y valor de Hb (r = 0,368, p = 0,019), no encontrando correlación con los niveles de 25 (OH) D. CONCLUSIÓN: Se necesitan estudios prospectivos, con mayor número de enfermos para conocer las implicaciones futuras de la densitometría alterada y los factores de riesgo asociados

OBJECTIVE: To evaluate bone mineral density (BMD) in children with sickle cell disease (SCD) in the Community of Madrid. MATERIAL AND METHODS: The BMD was estimated in 40 children with SCD, and with an age range between 3 and 16 years, using densitometry (DXA), as recommended by the International Society for Clinical Densitometry (ISCD). RESULTS: The mean age at the time of the study was 7.97 ± 3.95 years, the mean value of the DXA expressed in Z -score was -0.91 ± 1.46 with a range of minimum values - 5.30 and 2.30 maximum. More than half (57.5%) of all the children had normal BMD (Z > -1), 25% had low BMD (Z between -1 and -2), and 17.5% showed an abnormal Z -score values of osteoporosis (Z -score < -2). The Pearson linear correlation was statistically significant between Z -score value and the haemoglobin level (r = 0.368, p = .019), finding no correlation with the levels of 25 (OH) vitamin D. CONCLUSION: Prospective studies are needed with a larger number of patients to understand the future implications of bone densitometry changes and associated risk factors

[Evaluation of bone mineral density in children with sickle cell disease]. / Evaluación de la densidad mineral ósea en pacientes con enfermedad de células falciformes.

OBJECTIVE: To evaluate bone mineral density (BMD) in children with sickle cell disease (SCD) in the Community of Madrid. MATERIAL AND METHODS: The BMD was estimated in 40 children with SCD, and with an age range between 3 and 16 years, using densitometry (DXA), as recommended by the International Society for Clinical Densitometry (ISCD). RESULTS: The mean age at the time of the study was 7.97±3.95 years, the mean value of the DXA expressed in Z -score was -0.91±1.46 with a range of minimum values - 5.30 and 2.30 maximum. More than half (57.5%) of all the children had normal BMD (Z>-1), 25% had low BMD (Z between -1 and -2), and 17.5% showed an abnormal Z -score values of osteoporosis (Z -score<-2). The Pearson linear correlation was statistically significant between Z -score value and the haemoglobin level (r=0.368, p=.019), finding no correlation with the levels of 25 (OH) vitamin D. CONCLUSION: Prospective studies are needed with a larger number of patients to understand the future implications of bone densitometry changes and associated risk factors.

Avances en el conocimiento y manejo del síndrome linfoproliferativo autoinmune / Advances in the knowledge and management of autoimmune lymphoproliferative syndrome

Esta revisión tiene como objetivo discutir los recientes avances en el conocimiento, diagnóstico y manejo del síndrome linfoproliferativo autoinmune (ALPS). El ALPS consiste en una alteración en la homeostasis de los linfocitos debida a un fallo en la apoptosis. Las manifestaciones clínicas en la infancia son las citopenias recurrentes, la linfoproliferación crónica no maligna y complicaciones autoinmunes. Los recientes descubrimientos en la biología y genética de esta afección permiten un mejor conocimiento de la misma, que se traduce en cambios en los algoritmos diagnósticos y de tratamiento, con la aparición de nuevos inmunosupresores que han demostrado una mejoría en la calidad de vida de muchos pacientes

Autoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis. ALPS often manifest in childhood with cytopenias, chronic non-malignant lymphoproliferation and autoimmune complications. A number of new insights have improved the understanding of the genetics and biology of ALPS. The treatment of the disease has changed and mycophenolate mofetil and sirolimus have been demonstrated to have marked activity against the disease, improving quality of life for many patients. These will be discussed in this review

[Advances in the knowledge and management of autoimmune lymphoproliferative syndrome]. / Avances en el conocimiento y manejo del síndrome linfoproliferativo autoinmune.

Autoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis. ALPS often manifest in childhood with cytopenias, chronic non-malignant lymphoproliferation and autoimmune complications. A number of new insights have improved the understanding of the genetics and biology of ALPS. The treatment of the disease has changed and mycophenolate mofetil and sirolimus have been demonstrated to have marked activity against the disease, improving quality of life for many patients. These will be discussed in this review.

Situación actual de los adolescentes con cáncer en las unidades de hemato-oncología pediátrica espa˜nolas / The current situation of adolescents with cancer in pediatric hematology-oncology units in Spain. Results of a national survey

En España, hasta el año 2010 se ha prestado poca atención a los adolescentes con cáncer. En el año 2011 se crea el Comité de adolescentes con cáncer dentro de la Sociedad Española de Onco-Hematología Pediátrica (SEHOP), con el objetivo de cubrir las demandas y necesidades de estos pacientes. Con esta encuesta nacional se pretende obtener una fotografía de la situación actual de los adolescentes con cáncer en las unidades de hemato-oncología pediátrica españolas. Se ha realizado una encuesta, enviada vía email a 41 unidades de hemato-oncología pediátrica españolas. Se incluyen preguntas acerca de la epidemiología, el tratamiento, el abordaje psicosocial, las instalaciones y el seguimiento de estos pacientes. Un total de 40 unidades respondieron a la encuesta (98%). El 56% de las unidades trata a pacientes por encima de los 14 años, pero solo el 36% hasta los 18 años. Solo el 25,5% de las unidades trata más de 40 casos nuevos cada año. El porcentaje de los pacientes entre 14 y 18 años del total es menor del 10% en la mayoría de las unidades (77%).El 30,8 y el 48,7% de las unidades pediátricas tratan los adolescentes con hemopatías malignas y con tumores sólidos, respectivamente. El resto de los adolescentes con estas afecciones son tratados por los servicios de adultos. Solo existe una unidad en España que tenga un médico dedicado a la enfermedad oncológica del adolescente y solo hay 2 unidades que tengan una sala específica para adolescentes. Esta encuesta demuestra que la mayoría de los adolescentes con cáncer en España son tratados por especialistas de adultos, a pesar de que la supervivencia de estos pacientes es mayor cuando se utilizan protocolos pediátricos para su tratamiento. La mayoría de las unidades no tienen instalaciones ni personal especialmente dedicados a este grupo de pacientes. La SEHOP está aunando esfuerzos para mejorar tanto la supervivencia como la calidad del tratamiento de estos pacientes(AU)

Little attention was paid to adolescents with Cancer in Spain up to 2010. In 2011 an “Adolescents with Cancer Committee” was established by the Spanish Society of Pediatric Hemato-Oncology (SEHOP) to care for the needs of these patients. The aim of this national survey was to outline the present situation of adolescents with cancer in Spanish Pediatric Hemato-Oncology units. A web based survey assessed institutional management of adolescents with cancer. The survey was personally sent to one member of the staff of each Pediatric Hemato-Oncology unit in Spain. It included questions about epidemiology, management, psycho-social coverage, specific facilities, and follow up of these patients. A total of 40 institutions out of 41 responded to the survey (overall response rate 98%). Fifty-six percent of the institutions had patients over 14, but only 36% of the institutions treated patients up to 18 years old. Only 25.6% of the units have more than 40 new pediatric cases every year. The percentage of patients between 14 and 18 years of age is below 10% in most of the units (77%).The survey shows that most adolescents with cancer in Spain between 14 and 18 years of age are treated by adult oncologists. Most pediatric institutions still do not have specific facilities and psychosocial support for adolescents. The SEHOP is working hard in order to improve the quality of cancer care, and the quality of survival of this population. In 30.8% and 48.7% of the institutions, pediatric hemato-oncologists treat adolescents with hematological and solid tumors, respectively. The rest of the patients are seen by adult oncologists. There is only one institution that has a physician specifically dedicated to adolescent patients, and only two units have a “teenager's room”. Only 2 units have a psychologist specifically trained to treat adolescents with cancer(AU)

[The current situation of adolescents with cancer in pediatric hematology-oncology units in Spain. Results of a national survey]. / Situación actual de los adolescentes con cáncer en las unidades de hemato-oncología pediátrica españolas.

Little attention was paid to adolescents with Cancer in Spain up to 2010. In 2011 an "Adolescents with Cancer Committee" was established by the Spanish Society of Pediatric Hemato-Oncology (SEHOP) to care for the needs of these patients. The aim of this national survey was to outline the present situation of adolescents with cancer in Spanish Pediatric Hemato-Oncology units. A web based survey assessed institutional management of adolescents with cancer. The survey was personally sent to one member of the staff of each Pediatric Hemato-Oncology unit in Spain. It included questions about epidemiology, management, psycho-social coverage, specific facilities, and follow up of these patients. A total of 40 institutions out of 41 responded to the survey (overall response rate 98%). Fifty-six percent of the institutions had patients over 14, but only 36% of the institutions treated patients up to 18 years old. Only 25.6% of the units have more than 40 new pediatric cases every year. The percentage of patients between 14 and 18 years of age is below 10% in most of the units (77%). In 30.8% and 48.7% of the institutions, pediatric hemato-oncologists treat adolescents with hematological and solid tumors, respectively. The rest of the patients are seen by adult oncologists. There is only one institution that has a physician specifically dedicated to adolescent patients, and only two units have a "teenager's room". Only 2 units have a psychologist specifically trained to treat adolescents with cancer. The survey shows that most adolescents with cancer in Spain between 14 and 18 years of age are treated by adult oncologists. Most pediatric institutions still do not have specific facilities and psychosocial support for adolescents. The SEHOP is working hard in order to improve the quality of cancer care, and the quality of survival of this population.

Tratamiento con anakinra en artritis idiopática juvenil de comienzo sistémico refractaria a otras terapias / Anakinra treatment in refractory systemic onset juvenile arthritis

No disponible

Estado actual de la investigación con células madre / Current concepts in stem cell research

En los últimos años, los avances en la investigación con células madre han abierto un campo de esperanza para la curación de enfermedades y la medicina regenerativa. No es raro encontrar noticias en la prensa diaria o en otros medios de comunicación sobre este tema. En este artículo se pretenden aclarar algunos conceptos básicos en el campo de la investigación para entender la literatura médica referente a células madre, y aportar los datos y la bibliografía necesarios para una puesta al día sobre uno de los temas que más publicaciones generan en los últimos tiempos (AU)

[Current concepts in stem cell research]. / Estado actual de la investigación con células madre.

In the last few years, advances in stem cell research have opened up new horizons in the treatment of human diseases and in regenerative medicine. It is not unusual to find news on stem cell research in newspapers and other media. This review describes some basic concepts in research needed to understand the medical literature on stem cells and to provide the information and bibliography necessary to be up to date in one of the subjects that has generated the greatest number of publications in the last few years.

Pediculosis infantil ¿un problema emergente? / No disponible

La pediculosis infantil (en especial los piojos de la cabeza) es un problema universal y, aunque no produce enfermedades graves, es causa de perdida de un gran número de días de escolarización en los niños, y soporta en ocasiones un estigma social negativo. La aplicación de “remedios caseros” en ocasiones peligrosos para el niño debe ser desaconsejada, ya que existen preparados tópicos eficaces, aunque en los últimos años se ha constatado un aumento en el número de resistencias a estos tratamientos que pueden llevar a un incremento en la prevalencia de esta parasitación. En este artículo se revisan la etiología, clínica , diagnóstico y tratamiento de esta infestación y se hacen unas recomendaciones prácticas para su manejo (AU)

Child pediculosis (specially head lice) is an universal problem, although it is no cause of severe diseases, it causes a lost of great number of school days and produce a negative social stigma. The application of “home therapy” often dangerous for the child must be disallowed, because there are efficacious topical treatments, although in last years there is a raising number of resistance for these treatments that may increase the prevalence of this parasitation. We review in this report the etiology, clinica setting, diagnosis, and treatment of this infestation, and we make a practice guideline to its management (AU)

[Osteosarcoma and brain metastasis]. / Osteosarcoma y metástasis cerebral.

INTRODUCTION: Since the advent of multimodal therapy, survival among patients with osteosarcoma in general and among those with aggressive tumors has improved. Consequently, the pattern of relapse is also changing. Brain metastasis is considered to be a rare event in osteosarcoma, although recent reports suggest that the incidence of this complication may be increasing. CASE REPORT: We report two girls with osteoblastic osteosarcoma of the femur with poor response to preoperative chemotherapy. Both patients developed brain metastasis concurrent with or after the development of lung metastasis. Clinical manifestations of brain metastasis were symptoms of intracranial hypertension in one patient, and a complex partial seizure in the other. DISCUSSION: We advocate periodic neurologic examination and neuroradiologic screening for the early detection of brain involvement in patients whose disease recurs within 1 year of diagnosis, in those with metastasis at diagnosis and in those with a poor histologic response to preoperative chemotherapy.

Osteosarcoma y metástasis cerebral / Osteosarcoma and brain metastasis

La introducción del tratamiento multimodal ha supuesto una prolongación de la supervivencia, tanto de los pacientes con osteosarcoma globalmente como del subgrupo con tumores más agresivos, lo cual se ha asociado a un cambio en los patrones de metástasis. Tradicionalmente, las metástasis cerebrales constituían una rareza, aunque en los últimos años se ha observado un incremento en su incidencia. Se describen dos niñas con osteosarcoma osteoblástico de fémur cuya respuesta a la quimioterapia preoperatoria había sido escasa. Ambas presentaron metástasis cerebrales a la vez o tras el desarrollo de metástasis pulmonares. Clínicamente se manifestaron con síntomas de hipertensión intracraneal en una paciente; con una crisis parcial compleja en el otro caso. Consideramos que los pacientes con metástasis al diagnóstico, los que recidiven durante el primer año del diagnóstico y aquellos con una respuesta histológica escasa a la quimioterapia prequirúrgica deben ser examinados de forma periódica clínica y radiológicamente para descartar afectación cerebral (AU)

Infiltración de la cámara anterior ocular en una paciente con leucemia no linfoblástica aguda / Ocular infiltration in the anterior chamber in a female infant with acute non-lymphoblastic leukemia

La infiltración ocular diferente del sarcoma mieloide en la leucemia no linfoblástica aguda (LNLA) es un hallazgo inusual. Se revisa el caso de una niña de 9 meses de edad con LNLA M5 e infiltración del sistema nervioso central (SNC), que presentó afectación de la cámara anterior del ojo intratratamiento, coincidiendo con la remisión completa del SNC. Se trató con esteroides tópicos, quimioterapia sistémica y radioterapia ocular bilateral (dosis total de 1.000 cGy) con buena respuesta, aunque falleció a los 2 meses al no conseguirse la remisión hematológica de su enfermedad. Cualquier manifestación ocular en niños con leucemia debe ser evaluada y tratada de forma precoz. La radioterapia tiene un papel importante en el tratamiento de la infiltración de la cámara anterior del ojo. La infiltración ocular junto a la medular y del SNC es un signo de mal pronóstico en niños afectados de leucemia (AU)

[Ocular infiltration in the anterior chamber in a female infant with acute non-lymphoblastic leukemia]. / Infiltración de la cámara anterior ocular en una paciente con leucemia no linfoblástica aguda.

Unlike myeloid sarcoma, ocular involvement is unusual in acute non-lymphoblastic leukemia.A 9-month-old female infant with acute non-lymphoblastic leukemia M5 and evidence of active central nervous system (CNS) disease showed infiltration of the anterior chamber during therapy. At that time, the CNS disease was in completed remission. She was treated with topical corticosteroids, chemotherapy and bilateral ocular radiotherapy (total dose 1,000 cGy). The ocular manifestations responded well to treatment but hematologic response was poor. The patient died a few months later. Any ophthalmic manifestation in children with leukemia should be detected and treated early. Radiotherapy is warranted in infiltration of the anterior chamber of the eye. The presence of ocular, central CNS or bone marrow involvement indicates poor prognosis in acute childhood leukemia.

[Aggressive fibromatosis in childhood: retrospective analysis of four cases]. / Fibromatosis agresiva en la infancia: análisis retrospectivo de 4 casos.

OBJECTIVE: Aggressive fibromatosis is a rare illness in children. We analyzed the records of four patients, taken from a total of 505, all of which had different types of tumours, over a period of ten years. In the four cases, three were male and one female. Their ages ranged from 0-7 years old. We also did a review of this pathology. RESULTS: The illness may present itself a a painless mass found particularly in the pelvic area, knee, buttock and anterior mediastinum, respectively. Two of these cases presented lysis of osseous tissue shown in radiologic assays. The diagnosis was histological in every case. The only treatment was surgical in all of them. Two of the cases required only one intervention to achieve remission and up to now there has been no record of further illness. At the time of writing this paper the patients are alive after a follow-up period of thirty months. CONCLUSIONS: Up to now, no other alternative forms of treatment have been convincing. Adjuvant radiation and chemotherapy are probably beneficial, but the precise indication for its use is not well defined.