Servicio de Oncología y Hematología del Hospital Sant Joan de Déu / Oncology and Hematology Department of the Hospital Sant Joan de Déu

Describimos la organización y características del Servicio de Oncología y Hematología infantil del Hospital Sant Joan de Déu. El Servicio aborda de forma integral el cáncer y las enfermedades hematológicas no neoplásicas que ocurren en el niño y adolescente. El número de pacientes atendidos y los marcadores de impacto científico encabezan los del estado español. Nuestro abordaje se inicia en el diagnóstico, comprende el tratamiento y el seguimiento del superviviente hasta alcanzar la edad adulta, o los cuidados paliativos si se trata de enfermedades incurables. La atención integral relaciona todas las dimensiones imprescindibles para la atención al paciente: especialistas multidisciplinares coordinados, atención a la dimensión psicosocial e implicación en la investigación y docencia. El ámbito de actuación clínica incluye hospitalización, consultas externas, hospital de día y atención domiciliaria. La actividad de investigación engloba ensayos clínicos, incluyendo las fases precoces, así como investigación transaccional, de desarrollo propio o en colaboración con grupos internacionales (AU)

We describe the organization and characteristics of the Oncology Hematology service of the Hospital Sant Joan de Déu. The service comprehensively addresses cancer and non-neoplastic hematological diseases that occur in children and adolescents. The number of patients treated and scientific markers lead those of the Spanish state. Our approach begins in the diagnosis, includes the treatment and the follow-up of the survivor until adulthood, and the palliative care for incurable diseases. Comprehensive patient care links all dimensions: coordinated multidisciplinary specialists, attention to the psychosocial issues and active involvement in research and teaching. The clinical areas include hospitalization, external consultations, day hospital and home care. The research activity encompasses clinical trials, (including early phases), as well as own transactional research or integrated with international groups (AU)

Neuroimagen de la histiocitosis de células de Langerhans en el sistema nervioso central pediátrico / Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children

La histiocitosis de células de Langerhans (HCL) es una enfermedad rara caracterizada por la acumulación en los tejidos de células dendríticas anómalas similares a las células de Langerhans. La presentación clínica varía desde la aparición de una lesión ósea única hasta la afectación multisistémica. La implicación del sistema nervioso central (SNC), manifestada como diabetes insípida secundaria a afectación hipofisaria, es conocida desde la descripción original de la enfermedad. En la actualidad, se diferencian 2 tipos de lesiones del SNC: las lesiones seudotumorales, con infiltración por las células de Langerhans, cuya manifestación más frecuente es la infiltración hipofisaria, y otras, de más reciente descripción, las lesiones neurodegenerativas del SNC, asociadas a deterioro neurológico, que constituyen una complicación de la enfermedad de causa discutida. Nuestro objetivo es describir las manifestaciones radiológicas de la HCL en el SNC en los pacientes pediátricos (AU)

Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients (AU)

[Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children]. / Neuroimagen de la histiocitosis de células de Langerhans en el sistema nervioso central pediátrico.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients.

[Optic pathway astrocytoma: an unusual cause of failure to thrive in infants]. / Astrocitoma de vías ópticas: una causa infrecuente de retraso ponderal en el lactante.

Failure to thrive is a frequent cause of consultation in pediatric services. The main objective in these patients is the early detection of an organic cause, if present. We report a case of low-grade astrocytoma of the optic pathway in a 2-month-old child whose main symptoms at diagnosis were failure to thrive and anorexia. Unfortunately, despite therapeutic efforts, the tumor showed local and metastatic progression refractory to chemotherapy. The patient died 3 months after diagnosis. We conclude that diencephalic tumors must be considered in the differential diagnosis of failure to thrive during the first year of life, especially when, after initial investigations, a cause is not found.

Astrocitoma de vías ópticas: una causa infrecuente de retraso ponderal en el lactante / Optic pathway astrocytoma: an unusual cause of failure to thrive in infants

El retraso ponderal durante la lactancia es un motivo de consulta frecuente en los servicios de pediatría. El principal objetivo a la hora de abordar estos casos radica en distinguir aquellos casos secundarios a una causa orgánica de forma precoz. Aportamos un caso de astrocitoma de bajo grado de vías ópticas en un lactante de 2 meses de edad cuyos síntomas guía en el momento del diagnóstico fueron retraso ponderal y rechazo del alimento. Desafortunadamente, a pesar de los esfuerzos terapéuticos, el tumor presentó una progresión local y metastásica refractaria al tratamiento quimioterápico. Finalmente la paciente falleció a los 3 meses del diagnóstico. Concluimos que los tumores diencefálicos deben contemplarse en el diagnóstico diferencial del fallo de medro durante el primer año de vida; principalmente en aquellos casos en los cuales, tras un estudio inicial, no se encuentra una causa aparente

Failure to thrive is a frequent cause of consultation in pediatric services. The main objective in these patients is the early detection of an organic cause, if present. We report a case of low-grade astrocytoma of the optic pathway in a 2-month-old child whose main symptoms at diagnosis were failure to thrive and anorexia. Unfortunately, despite therapeutic efforts, the tumor showed local and metastatic progression refractory to chemotherapy. The patient died 3 months after diagnosis. We conclude that diencephalic tumors must be considered in the differential diagnosis of failure to thrive during the first year of life, especially when, after initial investigations, a cause is not found

Rendimiento de los cultivos practicados a pacientes hemato-oncológicos con enfermedades infecciosas graves / Yield of the cultures performed in hemato-oncology patients with serious infectious diseases

Introducción: Los avances en el campo de la oncología pediátrica han aumentado la supervivencia pero las complicaciones infecciosas son frecuentes y la necesidad de los Cuidados Intensivos (UCI-P) se incrementa. El objetivo del trabajo es valorar la rentabilidad de los cultivos practicados a pacientes oncológicos que precisaron ingreso en la UCI.P por una patología infecciosa. Material y Métodos. Estudio descriptivo, retrospectivo, de los pacientes oncológicos ingresados en una UCI-P por patología infecciosa en un hospital de tercer nivel. Resultados: Se incluyeron 22 pacientes que tuvieron 25 episodios de infección. Destacó la presentación en forma de insuficiencia respiratoria aguda en 14 pacientes (56%). Los síntomas infecciosos al diagnóstico previos al ingreso en la UCI-P fueron: sobreinfección respiratoria en 12 caos (48%); sospecha clínico-analítica de sepsis en 8 (16%). En un 43% de los pacientes ingresados en planta, se aisló algún germen en los cultivos realizados previos al ingreso en intensivos. Se aisló el microorganismo patógeno en 17 de los pacientes ingresados en UCI (68%), en un 82% de los casos gracias al hemocultivo. El diagnóstico final más frecuente en la UCI-P fue sepsis en 13, seguida de neumonía en 10 pacientes. Fueron exitus one pacientes (48%). Discusión: El elevado número de cultivos negativos probablemente se deba a que quedaran decapitados por la política antibiótica de amplio espectro. El conocimiento estricto de la epidemiología infecciosa de cada hospital y el estudio etiológico precoz y agresivo permitirían avanzar el tratamiento antibiótico correcto de forma empírica e incrementar la efectividad de la antibioterapia (AU)

Introduction: Recent advances in pediatric oncology have improved survival but infectious complications are frequent and the necessity of pediatric intensive care (PICU) is growing. The objective of this study is to determine the profitability of cultives in oncologic pediatric patients who required PICU admittance because of a infectious pathology. Materials and methods: Retrospective review of oncologic patients medical records, admitted to a reference pediatric intensive care unit (PICU), due to an infectious pathology. Results_ 22 patients who suffered 25 infectious processes were recruited. Acute respiratory insufficiency was the most frequent form of presentation (56%). Infectious symptoms before PICU admittance were: respiratory infection 12 cases (48%); clinical sepsis suspicion or biochemical markers compatible with sepsis in 8 patients (16%). 43% of the cultures recollected before PICU´s admittance were positive. Pathogenic microorganisms were isolated in 17 PICU´s patients (68%); in 82% of the cases, it was isolated in a blood sample. The final diagnoses were; sepsis in 13 cases, pneumonia in 10 cases. 11 patients died (48%). Discussion: The high number of negative cultures could be the result of the empiric broad-spectrum antibiotic therapy that is often used in this group of patients. The know-ledge of every hospital epidemiology and the precocious and aggressive etiologic search could improve the empiric antibiotic treatment and improve antibiotic effectiveness (AU)

Humoral immunity in pediatric patients with acute lymphoblastic leukaemia.

BACKGROUND: Cancer and its treatment are a major cause of secondary immunodeficiency in childhood. Leukaemias are the most frequent pediatric neoplastic diseases and 80 % are lymphoblastic (ALL). The objectives of this study are to describe the epidemiology of children with ALL in our hospital and to analyse the evolution of immunoglobulins' concentration at leukaemia's onset, during its treatment and after finishing it. METHODS AND RESULTS: Retrospective study of patients with ALL treated with the SHOP-LAL-94 protocol. 50 patients were studied. Patients were classified in three groups: ALL- cell line B, ALL-cell line B with relapse, and ALL-cell line T. We analysed clinical data and laboratory results (IgG, IgA and IgM concentration) at leukaemia's onset, during its treatment and until 12 months after it.1. ALL-B: 44 patients. At the onset all patients, but a newborn with congenital leukaemia, had normal immunoglobulins. During treatment there was a significant decrease in immunoglobulins'concentration, being IgM the most affected (65 % of patients), followed by IgG (53 % of patients). The mean normalization time of immunoglobulins was 12 months.2. ALL-B with relapse: 7 patients. At relapse 2 patients had an IgM deficit and 1 overall immunoglobulin deficiency. During treatment there was a decrease in all immunoglobulins, which was significant for IgG. IgG and IgM decreased in all patients during relapse's treatment. There were 5 deaths, all with IgM < 186 mg/L.3. ALL-T: 6 patients, one died 3 days after diagnosis. At the onset all patients had normal immunoglobulins. Two patients had a favourable evolution, having a decrease in immunoglobulins'concentration during treatment, significant for IgM, with normalization 6 months after treatment. The rest 3 patients relapsed and died, having a global immunoglobulins'deficit during treatment and previous to death. CONCLUSIONS: At ALL's onset immunoglobulins' concentration is normal. During treatment the majority of patients have immunoglobulins' deficiency, being IgG and IgM the most affected immunoglobulins. A persistent IgM deficit is associated in our series with a higher risk of relapse and death. In patients with a good outcome immunoglobulins normalize before one year after treatment.

Humoral immunity in pediatric patients with acute lymphoblastic leukaemia

Background: Cancer and its treatment are a major cause of secondary immunodeficiency in childhood. Leukaemias are the most frequent pediatric neoplastic diseases and 80 % are lymphoblastic (ALL). The objectives of this study are to describe the epidemiology of children with ALL in our hospital and to analyse the evolution of immunoglobulins' concentration at leukaemia's onset, during its treatment and after finishing it. Methods and results: Retrospective study of patients with ALL treated with the SHOP-LAL-94 protocol. 50 patients were studied. Patients were classified in three groups: ALL- cell line B, ALL-cell line B with relapse, and ALL-cell line T. We analysed clinical data and laboratory results (IgG, IgA and IgM concentration) at leukaemia's onset, during its treatment and until 12 months after it. 1. ALL-B: 44 patients. At the onset all patients, but a newborn with congenital leukaemia, had normal immunoglobulins. During treatment there was a significant decrease in immunoglobulins'concentration, being IgM the most affected (65 % of patients), followed by IgG (53 % of patients). The mean normalization time of immunoglobulins was 12 months. 2. ALL-B with relapse: 7 patients. At relapse 2 patients had an IgM deficit and 1 overall immunoglobulin deficiency. During treatment there was a decrease in all immunoglobulins, which was significant for IgG. IgG and IgM decreased in all patients during relapse's treatment. There were 5 deaths, all with IgM < 186 mg/L. 3. ALL-T: 6 patients, one died 3 days after diagnosis. At the onset all patients had normal immunoglobulins. Two patients had a favourable evolution, having a decrease in immunoglobulins'concentration during treatment, significant for IgM, with normalization 6 months after treatment. The rest 3 patients relapsed and died, having a global immunoglobulins'deficit during treatment and previous to death. Conclusions: At ALL's onset immunoglobulins' concentration is normal. During treatment the majority of patients have immunoglobulins' deficiency, being IgG and IgM the most affected immunoglobulins. A persistent IgM deficit is associated in our series with a higher risk of relapse and death. In patients with a good outcome immunoglobulins normalize before one year after treatment (AU)

Antecedentes: El cáncer y su tratamiento son una causa importante de inmunodeficiencia secundaria en la infancia. Las leucemias son las neoplasias pediátricas más frecuentes y un 80 por ciento son linfoblásticas (LAL). Los objetivos de este estudio son describir la epidemiología de los niños con LAL de nuestro medio y analizar la evolución de la concentración de inmunoglobulinas al comienzo de la leucemia, durante su tratamiento y al finalizarlo. Métodos y resultados: Estudio retrospectivo de pacientes con LAL tratados según el protocolo SHOP-LAL-94. Se analizaron 50 pacientes que se clasificaron en 3 grupos: LAL de línea B, LAL de línea B con recaída, y LAL de línea T. Se recogieron datos clínicos y de laboratorio (concentración de IgG, IgA e IgM) al manifestarse la enfermedad, durante su tratamiento y hasta 12 meses después.1. LAL-B: 44 pacientes. Al inicio todos los pacientes, excepto un neonato con leucemia congénita, presentaron inmunoglobulinas normales. Durante el tratamiento hubo una disminución significativa en la concentración de inmunoglobulinas, siendo la IgM la más afectada (65 por ciento de pacientes), seguida de la IgG (53 por ciento de pacientes). El tiempo medio de normalización de los valores de inmunoglobulinas fue de 12 meses.2. Recaída LAL-B: 7 pacientes. En la recaída 2 pacientes presentaron déficit de IgM y 1 déficit global de inmunoglobulinas. Durante el tratamiento disminuyó la concentración de inmunoglobulinas, resultando significativo el descenso de IgG. En todos los pacientes hubo disminución de IgG e IgM. Hubo 5 exitus, todos con IgM < 186 mg/L.3. LAL-T: 6 pacientes, uno fallecido a los 3 días del diagnóstico. Al inicio todos los pacientes presentaron inmunoglobulinas normales. Dos pacientes evolucionaron favorablemente, presentando disminución en la concentración de inmunoglobulinas durante el tratamiento, que fue significativa para IgM, con recuperación de valores normales 6 meses después del tratamiento. Los restantes 3 pacientes presentaron recaída y fallecieron, presentando déficit global de inmunoglobulinas durante su tratamiento y antes de producirse la muerte. Conclusiones: En el inicio de las LAL la concentración de inmunoglobulinas es normal. Durante el tratamiento la mayoría de pacientes presentan déficit de inmunoglobulinas, siendo la IgG y la IgM las más afectadas. El déficit persistente de IgM se asocia en nuestra serie con un mayor riesgo de recidiva y exitus. En pacientes con evolución favorable las inmunoglobulinas se normalizan antes del año de finalizar el tratamiento (AU)

[Newborn infants of diabetic mothers. Clinical study in the period 1980-1985]. / Recién nacido de madre diabética. Estudio clínico del período 1980-1985.

An statistical analysis is reported on specific problems of the infant of diabetic mother during the period 1980-1985, with a total of 287 newborns. Complications in this sample are exposed, and significant differences demonstrated in gestational age, fetal distress, hypocalcemia, polycythemia, jaundice, respiratory distress syndrome and associated problems according to clinical type of diabetes mellitus. High percentage of congenital malformations is pointed-out with a predominance of cardiac septal defects. Diabetological control was closer in insulin-dependent group, therefore, its effect has been studied separately. A lower rate of hypoglycemia was found in those under control, while infant of insulin-dependent diabetic mother showed a better compliance between weight and gestational age and a lower rate of respiratory distress syndrome.