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.

Efficacy and safety of liposomal cytarabine in children with primary CNS tumours with leptomeningeal involvement.

PURPOSE: To assess the efficacy and safety of liposomal cytarabine in the treatment of de novo and relapsed leptomeningeal involvement in children with primary CNS tumours. METHODS: Data from clinical charts were entered into a database for consecutive unselected patients (n=20) from nine Spanish centres. Diagnosis of leptomeningeal involvement was confirmed by cytology, MRI and/or CT scan. The dose of liposomal cytarabine used varied from 20 to 50 mg, by age. RESULTS: There were 8 females and 12 males, mean age 7.3 years (range 8 months to 18 years). The tumours were: 10 medulloblastomas, 4 ependymomas, 3 primitive neuroectodermal tumours and 3 other tumours. Fourteen had undergone previous chemotherapy and 12 radiotherapy. Nine received concurrent chemotherapy and 2 concurrent radiotherapy. Median follow-up was 244.5 days (range 12- 869). Patients received a median of 5 doses (range 1-9) of liposomal cytarabine. A neurological response (complete or partial) was seen in 11/19 (58%) and a cytological response in 7/10 (64%). Median time to neurological progression exceeded 180 days (range 12-869). Adverse effects were reported in 11/20 patients, but none was grade IV. DISCUSSION: Liposomal cytarabine was well tolerated and efficacious in this patient group, but prospective randomised trials are needed.

Efficacy and safety of liposomal cytarabine in children with primary CNS tumours with leptomeningeal involvement

PURPOSE: To assess the efficacy and safety of liposomal cytarabine in the treatment of de novo and relapsed leptomeningeal involvement in children with primary CNS tumours. METHODS: Data from clinical charts were entered into a database for consecutive unselected patients (n=20) from nine Spanish centres. Diagnosis of leptomeningeal involvement was confirmed by cytology, MRI and/or CT scan. The dose of liposomal cytarabine used varied from 20 to 50 mg, by age. RESULTS: There were 8 females and 12 males, mean age 7.3 years (range 8 months to 18 years). The tumours were: 10 medulloblastomas, 4 ependymomas, 3 primitive neuroectodermal tumours and 3 other tumours. Fourteen had undergone previous chemotherapy and 12 radiotherapy. Nine received concurrent chemotherapy and 2 concurrent radiotherapy. Median follow-up was 244.5 days (range 12- 869). Patients received a median of 5 doses (range 1-9) of liposomal cytarabine. A neurological response (complete or partial) was seen in 11/19 (58%) and a cytological response in 7/10 (64%). Median time to neurological progression exceeded 180 days (range 12-869). Adverse effects were reported in 11/20 patients, but none was grade IV. DISCUSSION: Liposomal cytarabine was well tolerated and efficacious in this patient group, but prospective randomised trials are needed (AU)

Forma atípica de enfermedad por arañazo de gato en escolar inmunocompetente / Atypical cat scratch disease in an immunocompetent school-aged child

No disponible

Fluorescence in situ hybridization study of TEL/AML1 fusion and other abnormalities involving TEL and AML1 genes. Correlation with cytogenetic findings and prognostic value in children with acute lymphocytic leukemia.

BACKGROUND AND OBJECTIVES: The TEL/AML1 fusion is the most common genetic abnormality found in childhood acute lymphoblastic leukemias (ALL). Although it is very difficult to identify by conventional cytogenetic techniques it can be readily detected using fluorescence in situ hybridization (FISH). We carried out cytogenetic and FISH studies on 42 children with ALL in order to know the frequency of this translocation in our population, the incidence of TEL and/or AML1 gene alterations, and their correlation with clinical evolution and prognosis. In addition, we performed reverse transcription polymerase chain reaction (RT-PCR) in some cases, confirming the feasibility of FISH techniques in the detection of this translocation. DESIGN AND METHODS: Bone marrow samples were obtained from 42 childhood ALL patients. The copy number of AML1 and TEL genes were studied using fluorescent in situ hybridization with a dual color DNA probe specific for the AML1 and TEL genes. RESULTS: We found a frequency of TEL/AML1 fusion of 17% in our sample. Double TEL/AML1 fusion, lack of TEL signal and extra AML1 signals were frequent additional FISH abnormalities. Duplication of a chromosomal complement, deletion of chromosome 12p arm, and polysomies of chromosome 21 are plausible explanations for these additional FISH findings. However, a relatively high proportion of our cases (9.5%) presented specific amplification of AML1. A statistically significant difference in prognosis was found between patients with and without these additional AML1 or TEL FISH alterations (p<0.02), which could be related to the presence of specific karyotypes. INTERPRETATIONS AND CONCLUSIONS: The frequency of TEL/AML1 fusion is similar to that found in other populations (17%). We found that FISH analysis of AML and TEL is related to the evolution of the disease. The absence of alterations in these genes revealed by FISH could be indicative of bad prognosis, while the presence of alterations is related to a good evolution. Our results suggest that interphase FISH analysis to search for alterations in AML and TEL genes could be extremely useful for complementing cytogenetic studies and for providing additional information about the possible outcome of the disease in patients with ALL.

Intron and intronless transcription of the chicken polyubiquitin gene UbII.

We have previously reported that the chicken polyubiquitin gene UbII is preferentially expressed during spermatogenesis and we show here that UbII is the predominant polyubiquitin gene expressed in early embryogenesis. Two main initiation sites were detected. Transcription from the initiation site used in early embryos results in the presence of an intron in the 5'-untranslated region of the transcripts as has been reported for other polyubiquitin messages. In mature testis, however, the use of a different initiation site, located within the intron, produces intronless transcripts. Distinct promoter sequences, present in each initiation site, may regulate the differential expression observed in this gene.

Gonadal failure following busulfan therapy in an adolescent girl.

A girl with Philadelphia chromosome-positive CML (Ph1-positive CML) was treated with busulfan from the age of 10 years 11 months to 16 years. Before treatment she had no evidence of pubertal development and no sexual development has occurred over the ensuing 5 years. Endocrine evaluation revealed that the child had ovarian failure. The pubescent female appears to be prone to develop gonadal failure with resultant lack of sexual development when treated with busulfan during this period in her life.

Neuroblastoma.

Neuroblastoma originates in the adrenal medulla or anywhere in the body that sympathetic tissue normally is present. It may present with a variety of symptoms due to primary tumor, metastatic disease, or unusual signs and symptoms such as opsoclonus-myoclonus or severe diarrhea. Despite the fact that this neoplasm responds to a variety of therapeutic modalities, it remains one of the most frustrating and difficult childhood tumors to treat and cure.

[Development of intracranial pressure in the neonatal period]. / Evolución de la presión intracraneal en el período neonatal.

Intracranial pressure monitoring in preterm infants is very important because central nervous disease is frequent in this age. Intracranial pressure (ICP) was determined in a population of preterm infants by a non invasive monitor. Normal values of ICP during perinatal period has been established.

[Treatment of juvenile rheumatoid arthritis (author's transl)]. / Tratamiento de la artritis reumatoide juvenil.

A revision of the treatment of the juvenile chronic arthritis (JCA) is made, Salicylates, still in use, require control of the salicylate level in order to obtain a higher efficiency and to prevent toxicity. New drugs appeared in the last years (by-products of propionic acid, tolmetin acid, fenclofenic...), are useful as an alternative for salicylates and with very little toxicity. Steroid therapy has to be reserved for serious systemic illness only, and slow acting drugs as antimalarials, gold and pencillamine, were only used in those cases with severe persistant activity and in cases of corticosteroid dependents. Use of the immunosuppressive therapy, is justified only in exceptional cases. Immunostimulants (transfer factor, Levamisol) are still in experimental phase. Presentation of the last five years' experience of the Pediatric Department is given. It concerns 25 cases of JCA, 5 systemic forms, 9 polyarticular and 11 forms of pauci-articular. Therapy is based on the predominant use of aspirin and on steroid therapy for the system forms. The efficiency of the treatment is not easy to evaluate regarding consideration of the unpredictable evaluation of the illness.