RESUMEN
BACKGROUND: Adrenocortical cancer (ACC) in childhood is a rare disease with poor prognosis. Complete surgical resection, systemic chemotherapy, and mitotane therapy are important curative treatment options for patients with advanced-stage tumors. Since 1997, pediatric ACC patients in Germany have been treated according to the non-randomized, single arm study GPOH-MET-97. PATIENTS AND METHODS: Data regarding disease course, treatment, and survival rates of 60 patients (age 0.24-17.8 years) with ACC treated according to the GPOH-MET-97 protocol were collected and analyzed to determine outcome, with a focus on examining the effectiveness of mitotane therapy. RESULTS: Among all patients, event-free survival and overall survival were found to be 43.3% and 64.8%, respectively. Chemotherapy with VCR, IFO, ADR, CARBO, and VP16 had been provided to 34 patients (56.6%) in different settings (neoadjuvant, adjuvant, and salvage) and mitotane therapy to 32 patients (53.3%). Duration of mitotane treatment longer than 6 months and mitotane levels greater than 14 mg/l were found to be associated with significantly better survival. Local relapse was found to be associated with a worse prognosis compared to distant metastasis only. CONCLUSIONS: Systemic chemotherapy and mitotane therapy are important therapeutic options in the treatment of advanced pediatric ACC patients. Neoadjuvant therapy should be considered for patients with primarily incomplete resectable or inoperable tumors, and tumor spillage is an indication for adjuvant chemo- and mitotane therapy. All pediatric ACC patients should be treated in pediatric oncological centers according to a consistent protocol in a highly interdisciplinary setting.
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Neoplasias de la Corteza Suprarrenal/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Terapia Neoadyuvante , Neoplasias de la Corteza Suprarrenal/mortalidad , Neoplasias de la Corteza Suprarrenal/patología , Neoplasias de la Corteza Suprarrenal/cirugía , Adrenalectomía , Niño , Preescolar , Terapia Combinada , Progresión de la Enfermedad , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Mitotano/administración & dosificación , Estadificación de Neoplasias , Terapia RecuperativaRESUMEN
The procedure guideline for radioiodine ((131)I) therapy and (131)I whole-body scintigraphy of differentiated thyroid cancer in paediatric patients is the counterpart to the procedure guidelines (version 3) for adult patients and specify the interdisciplinary guideline for thyroid cancer of the Deutsche Krebsgesellschaft concerning the nuclear medicine part. Characteristics of thyroid cancer in children are the higher aggressiveness of papillary thyroid cancer, the higher frequency of extrathyroidal extension and of disseminated pulmonary metastases as well as the high risk of local recurrences. Radioiodine therapy is generally recommended in children, the (131)I activity depends on the children's body weight. Radioiodine ablation in children with small papillary cancer (< or =1 cm) should be considered. TSH stimulation is reached two weeks (children) or three weeks (adolescents) after withdrawal of thyroid hormones. Anti-emetic drugs are highly recommended. CT of the chest and examination of pulmonary function are clearly indicated if there is any suspicion on metastases. 3-6 months after (131)I ablation, the (131)I whole-body scintigraphy is highly recommended as lymph node metastases are frequently detected in paediatric patients. Follow-up care should be arranged in shorter intervals than in adults to test the compliance and to adapt dosage of thyroid hormones to the children's body weight. Reference values of fT3 are higher in children than in adults. Evidence is insufficient to describe in which constellation the TSH may be kept within the low normal level. Therefore, TSH suppression is generally recommended.
Asunto(s)
Radioisótopos de Yodo , Neoplasias de la Tiroides/diagnóstico por imagen , Imagen de Cuerpo Entero/métodos , Niño , Terapia Combinada , Humanos , Radioisótopos de Yodo/efectos adversos , Radioisótopos de Yodo/normas , Guías de Práctica Clínica como Asunto , Cintigrafía , Sensibilidad y Especificidad , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/secundario , Hormonas Tiroideas/uso terapéutico , Neoplasias de la Tiroides/tratamiento farmacológico , Neoplasias de la Tiroides/patología , Imagen de Cuerpo Entero/normasRESUMEN
We describe the case of a 14-month-old boy with delayed-onset SCID due to ADA-deficiency which was masqueraded only by failure to thrive. Remarkably, the child had no serious infections and an adequate immune response. However, absolute lymphopenia, eosinophilia and absent thymus on chest x-ray were indicative for immunodeficiency.
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Adenosina Desaminasa/deficiencia , Eosinofilia/etiología , Insuficiencia de Crecimiento/etiología , Linfopenia/etiología , Inmunodeficiencia Combinada Grave/diagnóstico , Proteína C-Reactiva/análisis , Relación CD4-CD8 , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/inmunología , Insuficiencia de Crecimiento/diagnóstico , Insuficiencia de Crecimiento/inmunología , Regulación Enzimológica de la Expresión Génica/genética , Humanos , Lactante , Recuento de Linfocitos , Linfopenia/diagnóstico , Linfopenia/inmunología , Masculino , Fenotipo , Inmunodeficiencia Combinada Grave/genética , Inmunodeficiencia Combinada Grave/inmunología , Trombocitosis/diagnóstico , Trombocitosis/etiología , Trombocitosis/inmunología , Timo/anomalíasRESUMEN
The inflammatory response plays a major role in the induction of several neonatal diseases. We hypothesize that an imbalance between the pro- and anti-inflammatory response is crucial for the previously shown enhanced production of proinflammatory cytokines in term and preterm infants during infection. To test this hypothesis, we compared the capacity to produce the main anti-inflammatory cytokines IL-10 and TGF-beta in term infants, preterm infants and adults at different levels of synthesis by quantitative real time reverse-transcribed PCR, flow cytometry, as well as enzyme-linked immunoassay. Term and preterm infants showed a profoundly diminished IL-10 mRNA-expression and IL-10 production after stimulation. In addition, the amount of TGF-beta-positive lymphocytes was significantly less in neonates than adults. Furthermore, there was a considerably lower inhibition of production of IL-1alpha, IL-6, IL-8 and TNF-alpha by the use of recombinant IL-10 in term and preterm infants compared with adults. These results demonstrate not only a diminished anti-inflammatory capacity but also a reduced response to anti-inflammatory stimuli in term and preterm infants. From these data we conclude that neonates display an immature compensatory anti-inflammatory response syndrome (CARS) which may predispose preterm infants to harmful effects of proinflammatory cytokines resulting in severe organ sequelae during infection.
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Inflamación/inmunología , Interleucina-10/biosíntesis , Factor de Crecimiento Transformador beta/biosíntesis , Adulto , Envejecimiento/inmunología , Humanos , Recién Nacido , Recien Nacido Prematuro , Enfermedades del Prematuro/inmunología , Mediadores de Inflamación/antagonistas & inhibidores , Interleucina-10/genética , Interleucina-10/inmunología , Lipopolisacáridos/inmunología , ARN Mensajero/genética , Proteínas Recombinantes/farmacología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Factor de Crecimiento Transformador beta/genética , Factor de Crecimiento Transformador beta/inmunologíaRESUMEN
BACKGROUND: Excellent treatment results have been obtained for children with Hodgkin's disease (HD). Children with immunodeficiencies who present with HD do not have such a favourable prognosis. PATIENTS AND METHODS: A systematic literature search using MEDLINE and a search for immunodeficiencies in the database of the trials DAL HD78-HD90 and GPOH HD95 (n = 2263) were carried out. Age, sex, type of immunodeficiency, disease stage, treatment and outcome of all HD cases with known immunodeficiency were recorded. RESULTS: 28 published cases and 13 children in the DAL/GPOH trials were identified. 19/28 and 6/13 patients have immunodeficiencies with increased DNA breakage (24/25 ataxia teleangiectasia, 1/25 Nijmegen breakage syndrome) who present largely with stage III - IV HD. Among the published cases with increased DNA breakage there is only one child who is surviving 16 months after diagnosis, while there are 6/9 survivors in the group of immunodeficiencies without increased DNA breakage. Similarly, only 1/6 children survives in the group of children reported to the DAL/GPOH trials suffering from HD and immunodeficiency with increased DNA breakage, while the outcome in children suffering from immunodeficiency without increased DNA breakage is much better with 5/7 survivors. CONCLUSIONS: The literature review and data analysis of the DAL/GPOH studies show that treatment outcome is almost invariably fatal in children with HD and immunodeficiency with increased DNA breakage. Thus we propose to treat children with or without increased DNA breakage differently to improve the outcome of Hodgkin's disease in the subgroup of children with immunodeficiency.
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Enfermedad de Hodgkin/complicaciones , Enfermedad de Hodgkin/terapia , Síndromes de Inmunodeficiencia/complicaciones , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ataxia Telangiectasia/complicaciones , Ataxia Telangiectasia/mortalidad , Niño , Preescolar , Rotura Cromosómica , Ensayos Clínicos como Asunto , Terapia Combinada , Femenino , Enfermedad de Hodgkin/tratamiento farmacológico , Enfermedad de Hodgkin/mortalidad , Enfermedad de Hodgkin/radioterapia , Humanos , Síndromes de Inmunodeficiencia/mortalidad , Masculino , Pronóstico , Dosificación Radioterapéutica , Análisis de Supervivencia , Factores de Tiempo , Resultado del TratamientoRESUMEN
The use of long-term automated erythrocytapheresis via an arterio-venous fistula for the prevention of recurrent ischaemic stroke in a child with sickle-cell disease (SCD) has not been described previously. We report the successful use of this technique in a 13-year-old boy. A procedure was performed every 36 +/- 6 days, transfusing six units of donor packed red blood cells (RBCs) and discarding 1318 +/- 174 mL of exchanged erythrocytes (Hct 60%). After transfusion of 85 units over 17 months, there is no evidence for iron-overload, red cell alloimmunization, transfusion-transmitted infections, or other complications. Until now, no cerebrovascular ischaemia has been observed.
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Anemia de Células Falciformes/terapia , Derivación Arteriovenosa Quirúrgica , Transfusión de Eritrocitos/métodos , Adolescente , Eliminación de Componentes Sanguíneos , Humanos , Masculino , Recurrencia , Accidente Cerebrovascular/prevención & control , Resultado del TratamientoRESUMEN
BACKGROUND: Early diagnosis of Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disorder (PTLD) is required to detect a stage of disease that is more likely to respond to treatment. Elevated levels of EBV DNA were found in peripheral blood of patients at the onset of PTLD. METHODS: To compare plasma and peripheral blood mononuclear cells (PBMCs) as material for real-time quantitative polymerase chain reaction (RQ-PCR) measurement of Epstein-Barr viral load, we used two sets of primers and probes specific for the BAM HI-K or BAM HI-W region of the EBV genome. RESULTS: Patients with PTLD had a median viral load of 19,200 EBV genomes/microg DNA (n=9) or 3,225 EBV genomes/100 microl plasma (n=5), being significantly higher compared with immunosuppressed patients with primary (n=9) or reactivated (n=20) EBV infection or immunosuppressed patients without serological signs of active EBV infection (n=67) (P<0.001). Hence, a value of greater than 5,000 EBV genomes/microg PBMC DNA was considered as a diagnostic parameter for PTLD with a sensitivity and specificity of 1.00 or 0.89, respectively. When plasma was analyzed, however, a value of greater than 1,000 EBV genomes/100 microl plasma had both a sensitivity and specificity of 1.00 for the diagnosis of PTLD. During remission of PTLD, viral load was more effectively cleared in plasma compared with PBMCs. In plasma of nonimmunosuppressed individuals, even a qualitative detection of EBV-related sequences was sensitive and specific for the diagnosis of primary EBV infection, whereas for analysis of PBMC DNA a quantitative parameter had to be considered to differentiate healthy individuals (< 100 EBV genomes/microg PBMC DNA) from patients with primary EBV infection (>100 EBV genomes/microg PBMC DNA). CONCLUSION: Although both PBMCs and plasma were useful as material for EBV-specific RQ-PCR in immunosuppressed patients and nonimmunosuppressed individuals, the specificity of analysis seemed to be higher if plasma was taken for analysis.
Asunto(s)
Infecciones por Virus de Epstein-Barr/complicaciones , Trasplante de Riñón/efectos adversos , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/virología , Adolescente , Adulto , Sangre/virología , Niño , Preescolar , Sistemas de Computación , Estudios Transversales , ADN Viral/sangre , Infecciones por Virus de Epstein-Barr/sangre , Infecciones por Virus de Epstein-Barr/virología , Femenino , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/aislamiento & purificación , Humanos , Masculino , Monocitos/virología , Reacción en Cadena de la Polimerasa/métodos , Reacción en Cadena de la Polimerasa/normas , Factores de Riesgo , Sensibilidad y Especificidad , Carga ViralRESUMEN
Hodgkin's disease (HD) is commonly associated with latent Epstein-Barr virus (EBV) infection. The aim of our study was a detailed molecular analysis of the EBV status in the peripheral blood of paediatric patients with HD. Blood samples from HD patients were examined before (n=28) and after treatment (n=12). The control group consisted of 20 healthy children and 10 immunosuppressed children with primary EBV infection. EBV load in plasma and peripheral blood mononuclear cells (PBMC) were determined by real time quantitative polymerase chain reaction (RQ-PCR) as recently described. Before treatment, EBV DNA was detected in the plasma of 13/24 EBV-seropositive HD patients, whereas in plasma of healthy controls no EBV DNA was detectable (P<0.001). After treatment, no EBV genomes were found in the plasma of 6 HD patients in stable and complete remission. In contrast, 2/5 HD patients with relapse of disease were positive for EBV DNA in the plasma. In PBMCs, no differences were found in EBV load measured in HD patients before or after treatment and healthy controls. A high EBV load was found in both the plasma and PBMCs of all immunosuppressed patients with primary EBV infection. Thus, EBV DNA detection in the plasma of paediatric HD patients might be of value for non-invasive diagnostic, prognostic and follow-up tests for HD.
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ADN Viral/sangre , Herpesvirus Humano 4/aislamiento & purificación , Enfermedad de Hodgkin/virología , Adolescente , Adulto , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/inmunología , Femenino , Enfermedad de Hodgkin/terapia , Humanos , Huésped Inmunocomprometido , Leucocitos Mononucleares/virología , Masculino , Reacción en Cadena de la Polimerasa , Recurrencia , Carga ViralAsunto(s)
Bronquiolitis Viral/inmunología , Interferón gamma/sangre , Interleucina-8/sangre , Infecciones por Virus Sincitial Respiratorio/inmunología , Animales , Bronquiolitis Viral/terapia , Humanos , Recién Nacido , Ratas , Respiración Artificial , Infecciones por Virus Sincitial Respiratorio/terapiaRESUMEN
The load of Epstein-Barr virus (EBV) in peripheral blood mononuclear cells of transplant recipients represents a predictive parameter for posttransplant lymphoproliferative disorders (PTLD). The aim of our work was to develop a rapid and reliable PCR protocol for the quantification of cell-associated EBV DNA in transplant recipients. In contrast to previous studies, a protocol that facilitated quantification independent of photometric nucleic acid analysis was established. We took advantage of the real-time PCR technology which allows for single-tube coamplification of EBV and genomic C-reactive protein (CRP) DNA. EBV copy numbers were normalized by division by the amount of CRP DNA, with the quotient representing the actual amount of amplifiable genomic DNA per reaction. Coamplification of CRP DNA did not result in a diminished detection limit for EBV. By using the protocol without normalization, EBV copy numbers in 4 out of 10 PTLD patients were within the normal range determined with data for 114 transplant recipients that served as controls. After normalization, however, all of the PTLD patients had a higher viral load than the control population, indicating an increased sensitivity of the assay. Moreover, EBV copy numbers obtained for one patient by conventional quantification and suggestive of relapsing PTLD were within normal range after normalization. We conclude that normalization of PCR signals to coamplified genomic DNA allows a more accurate quantification of cell-bound EBV.
Asunto(s)
Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4/aislamiento & purificación , Trastornos Linfoproliferativos/virología , Trasplante de Órganos , Complicaciones Posoperatorias , Calibración , Línea Celular , Niño , Infecciones por Virus de Epstein-Barr/sangre , Infecciones por Virus de Epstein-Barr/etiología , Femenino , Trasplante de Corazón , Humanos , Trasplante de Riñón , Trasplante de Hígado , Trastornos Linfoproliferativos/diagnóstico , Masculino , Reacción en Cadena de la Polimerasa/métodos , Valores de Referencia , Reproducibilidad de los Resultados , Carga ViralRESUMEN
Measurement of Epstein-Barr virus (EBV) load is useful in peripheral blood for detecting primary and reactivated EBV-infections especially in immunosuppressed patients being at high risk for developing posttransplant lymphoproliferative disorder. For quantification of EBV DNA in peripheral blood of patients two real time polymerase chain reaction (RQ-PCR) assays were developed detecting sequences specific for the BAM HI-W and BAM HI-K region of EBV. In order to determine the optimal material of peripheral blood for RQ PCR analysis, DNA preparations of whole blood, peripheral blood mononuclear cells (PBMC) and B cells from 11 healthy, EBV-seropositive individuals were analysed in parallel and compared with regard to efficiency and sensitivity. While in whole blood preparations inhibitors of RQ PCR were detected influencing sensitivity, analysis of B cells being most sensitive is limited by being too labour intensive. In contrast, analysis of DNA preparations of PBMCs is sensitive enough to frequently detect EBV-specific sequences in all individuals tested and the preparation of PBMCs itself needs only a reasonable time. Thus, longitudinal monitoring of EBV load in peripheral blood of patients is possible by RQ-PCR, the optimal material for analysis being PBMCs.
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ADN Viral/sangre , Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4/aislamiento & purificación , Leucocitos Mononucleares/virología , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/prevención & control , Reacción en Cadena de la Polimerasa/métodos , Linfocitos B/virología , Enfermedad Crónica , Infecciones por Virus de Epstein-Barr/complicaciones , Herpesvirus Humano 4/genética , Humanos , Técnicas In Vitro , Trastornos Linfoproliferativos/virología , Sensibilidad y Especificidad , Carga ViralRESUMEN
The objective of this study was to investigate the maturational changes of lymphocyte surface antigens during ontogeny from fetuses to adults using the proven whole blood lysis technique. Two-color flow cytometric analysis of lymphocyte surface markers was performed on 20 fetal blood samples obtained by cordocentesis, 70 cord blood and 30 adult blood samples. The leukocyte count and all T-cell subsets were highest in neonates compared to fetuses and declined into adulthood. In contrast, the percentage of CD2+ T cells (fetal blood 57% versus adult blood 82%; p
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Envejecimiento , Antígenos CD/análisis , Sangre Fetal/citología , Linfocitos T/inmunología , Adulto , Linfocitos B , Antígenos CD2/análisis , Complejo CD3/análisis , Antígenos CD4/análisis , Antígenos CD8/análisis , Femenino , Sangre Fetal/inmunología , Humanos , Recién Nacido , Recuento de Linfocitos , Subgrupos Linfocitarios , Masculino , Caracteres SexualesRESUMEN
Antiphospholipid antibodies (APAs) are considered risk factors in patients with thromboembolic diseases. Although the incidence of such acquired coagulation disturbances in adults are well described, only few data exist for children. Therefore, in a first step to collect new data we analyzed the presence of different APAs in 202 consecutive children and compared them with two groups of adults. The children screened for APA were exclusively those who did not have any thromboembolic complications or a tendency for thrombophilia due to other underlying diseases such as systemic lupus or malignancy in their past or present medical history. Consecutive blood samples were evaluated from routine laboratory specimens. The two groups of adults comprised 200 patients after deep vein thrombosis and 200 patients without thromboembolic events that served as controls. Four lupus anticoagulant (LA) screening tests were determined: the dilute Russell's viper venom test; a lupus anticoagulant-sensitive activated partial thromboplastin time reagent; a second lupus-sensitive activated partial thromboplastin time; and the Kaolin clotting time. Furthermore, three different antiphospholipid antibodies ELISA assays against cardiolipin (ACA), beta2-glycoprotein I, and phosphatidyl-serine, were determined. The children had a much higher prevalence for LA than did the adults. On the other hand, their values for ACA were significantly lower than in adults with a history of thromboembolism. Findings in children were similar to the normal adult group. This has to be taken into account when evaluating children with thromboembolic diseases.
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Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/complicaciones , Enfermedades Autoinmunes/complicaciones , Trombofilia/inmunología , Adolescente , Adulto , Factores de Edad , Anticuerpos Anticardiolipina/sangre , Anticuerpos Anticardiolipina/inmunología , Anticuerpos Antifosfolípidos/inmunología , Especificidad de Anticuerpos , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/inmunología , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/inmunología , Pruebas de Coagulación Sanguínea , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Glicoproteínas/inmunología , Humanos , Lactante , Recién Nacido , Inhibidor de Coagulación del Lupus/sangre , Persona de Mediana Edad , Fosfatidilserinas/inmunología , Valores de Referencia , Trombofilia/etiología , Trombosis de la Vena/sangre , Trombosis de la Vena/inmunología , beta 2 Glicoproteína IRESUMEN
Cellular drug resistance is one of the main causes of the frequent ultimate failure of chemotherapy in childhood acute myeloid leukemia (AML). We here summarize the results of a literature review on in vitro drug resistance in childhood AML, focusing on studies using so-called cell culture assays. We also briefly describe some results of an ongoing collaborative study between the Research Laboratory of Pediatric Oncology in Amsterdam (University Hospital Vrije Universiteit) and the German BFM-AML Group. In general, the literature and our preliminary data on in vitro cellular drug resistance in AML are promising in terms of clinical relevance. Cell biological features and clinical response to chemotherapy are related to in vitro drug resistance. However, a large study including multivariate analysis is required to more firmly establish the clinical value of cellular drug resistance testing in childhood AML, and the collaborative study will therefore be continued. Possible applications of cell culture assays include risk-group stratification, rational improvements of current treatment protocols for subgroups of patients based on specific drug resistance profiles, individualised tailored therapy, the study of cross-resistance patterns between drugs, the study of possibilities to modulate or circumvent drug resistance, the study of drug interactions, selection of patients for clinical phase II studies and drug screening.
Asunto(s)
Antineoplásicos/farmacología , Síndrome de Down/complicaciones , Resistencia a Antineoplásicos , Leucemia Mieloide/tratamiento farmacológico , Ensayo de Tumor de Célula Madre/métodos , Enfermedad Aguda , Antibióticos Antineoplásicos/farmacología , Niño , Humanos , Leucemia Mieloide/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológicoAsunto(s)
Citocinas/sangre , Sangre Fetal/inmunología , Linfocitos/inmunología , Monocitos/inmunología , Adulto , Citometría de Flujo/métodos , Humanos , Recién Nacido , Interferón gamma/biosíntesis , Interferón gamma/sangre , Interleucina-6/biosíntesis , Interleucina-6/sangre , Reproducibilidad de los Resultados , Linfocitos T/inmunologíaRESUMEN
An 11 year old girl presented with hypertension and tachycardia. Excess urinary catecholamine excretion suggested phaeochromocytoma but imaging studies failed to demonstrate a tumour. Other symptoms included insomnia and weight loss, and she was found to have a raised concentration of mercury in blood and urine. Mercury intoxication should be considered in the differential diagnosis of hypertension with tachycardia even in patients presenting without the skin lesions typical of mercury intoxication and without a history of exposure.
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Hipertensión/inducido químicamente , Intoxicación por Mercurio/complicaciones , Taquicardia/inducido químicamente , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Niño , Femenino , Humanos , Intoxicación por Mercurio/diagnóstico , Feocromocitoma/diagnósticoRESUMEN
INTRODUCTION: There have been only a few studies on differentiated childhood thyroid cancer (DTC) in children and adolescents. METHODS: We analyzed the characteristics of DTC with respect to age, gender and histology in 114 patients under 18 years of age. In a questionnaire-based survey, data of 114 patients, aged between 3 years and 18 years, was collected from 65 clinical institutions in Germany. Characteristics of 80 females and 34 males were evaluated, and the prognostic effect of age, gender, histology, multicentric growth, tumor stage and N-status on distant metastases was tested using multivariate discriminant analysis. Between-group comparison was performed using student t-test and chi-squared test. RESULTS: The incidence of DTC in females was higher than in males with a peak of female:male ratio at puberty, which was more pronounced in children with papillary thyroid cancer, but not with follicular thyroid cancer. Papillary thyroid cancer was associated with more advanced disease (P=0.009), more lymph-node involvement (P=0.007) and more distant metastases (P=0.02) compared with follicular thyroid cancer. Multivariate analysis showed advanced tumor stage as the only significant factor (P=0.02) associating with distant metastasis. CONCLUSION: It can be concluded that in children and adolescents: 1. The incidence of papillary thyroid cancer is higher in females than males, with a peak at puberty. 2. The only significant factor associated with distant metastases is the advanced tumor stage. 3. Childhood thyroid cancer is frequently associated with lymph-node involvement, distant metastases and advanced tumor stage. 4. Papillary childhood thyroid cancer is more aggressive than follicular type.
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Neoplasias de la Tiroides/epidemiología , Neoplasias de la Tiroides/patología , Adenocarcinoma Folicular/epidemiología , Adenocarcinoma Folicular/patología , Adolescente , Factores de Edad , Carcinoma Papilar/epidemiología , Carcinoma Papilar/patología , Niño , Preescolar , Femenino , Humanos , Metástasis Linfática , Masculino , Análisis Multivariante , Metástasis de la Neoplasia , Estadificación de Neoplasias , Pronóstico , Pubertad , Factores SexualesRESUMEN
BACKGROUND: A novel Hodgkin disease (HD)-derived cell line, designated HKB-1, was established from pulmonary HD of nodular sclerosing subtype of a 14-year-old-girl. PROCEDURE AND RESULTS: Immunophenotypically, HKB-1 cells are of B cell in phenotype, being also positive for markers CD15, CD25 and CD30. Transformation of the cell line by Epstein-Barr virus (EBV) was excluded by failure to detect EBV antigens and EBV DNA in cultured cells. Chromosome studies of HKB-1 showed a pseudodiploid karyotype with complex clonal structural aberrations. The detection of a monoclonal immunoglobulin heavy chain (IgH) gene rearrangement confirmed the derivation of the HKB-1 from the B cell lineage and its monoclonality. HKB-1 produced high amounts of interleukin (IL)-6 as detected in culture supernatant whereas no secretion of IL-1 beta, IL-2, IL-4, IL-10, IL-12 or interferon (IFN)-gamma was detected. CONCLUSIONS: Our studies indicate that this cell line is of tumor origin.