RESUMEN
We have examined the imprinting of the insulin-like growth factor II gene (IGF2) in ten normal kidney samples from children with renal embryonal neoplasms. In kidney samples from nine children with normal growth profiles, IGF2 mRNA was transcribed monoallelically, consistent with normal imprinting of the gene. But in one child who had generalized somatic overgrowth, IGF2 was transcribed from both alleles in her kidney, peripheral blood leukocytes and Wilms' tumour. These findings suggest that a defect in genomic imprinting can occur constitutionally, leading to growth abnormalities and predisposition to Wilms' tumour.
Asunto(s)
Genes del Tumor de Wilms , Gigantismo/genética , Factor II del Crecimiento Similar a la Insulina/metabolismo , Neoplasias Renales/genética , Alelos , Humanos , Factor II del Crecimiento Similar a la Insulina/genética , Reacción en Cadena de la Polimerasa , Polimorfismo Genético , ARN Mensajero/metabolismoRESUMEN
AIMS: To report the survival and event-free survival of children with acute lymphoblastic leukaemia treated in Auckland between 1985-1991, using an international protocol, ANZCCSG ALL Study V. METHODS: The data were collected prospectively as part of the study. The analysis was carried out using standard survival methods. RESULTS: At a median follow up of 7.5 years, the overall survival is 75% and event-free survival 60%. CONCLUSIONS: For an unselected series, these outcomes are comparable to overseas series. The survival for children diagnosed in the 1990's is likely to be similar.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Irradiación Craneana , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Adolescente , Asparaginasa/administración & dosificación , Niño , Preescolar , Ciclofosfamida/administración & dosificación , Citarabina/administración & dosificación , Daunorrubicina/administración & dosificación , Humanos , Lactante , Mercaptopurina/administración & dosificación , Metotrexato/administración & dosificación , Nueva Zelanda , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/etiología , Prednisona/administración & dosificación , Modelos de Riesgos Proporcionales , Estudios Prospectivos , Radioterapia Adyuvante , Análisis de Supervivencia , Resultado del Tratamiento , Vincristina/administración & dosificaciónRESUMEN
A caucasian female infant with acute lymphoblastic leukaemia in second remission received a bone marrow transplantation. Engraftment was confirmed at 14 days following infusion of bone marrow from a sex-matched, ABO and HLA compatible sibling. 171 days posttransplantation the patient is clinically well and in haematological remission. A mild transient skin rash and hepatocellular disturbance, the only manifestations of graft versus host disease, responded successfully to high dose prednisone.
Asunto(s)
Trasplante de Médula Ósea , Leucemia Linfoide/terapia , Preescolar , Femenino , Reacción Injerto-Huésped/efectos de los fármacos , Humanos , Prednisona/uso terapéutico , Pronóstico , Trasplante HomólogoAsunto(s)
Meningitis Viral/diagnóstico , Infecciones por Parvoviridae/diagnóstico , Parvovirus B19 Humano/aislamiento & purificación , Preescolar , Enfermedad Crónica , ADN Viral/análisis , Femenino , Humanos , Meningitis Viral/complicaciones , Infecciones por Parvoviridae/complicaciones , Reacción en Cadena de la Polimerasa , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicacionesAsunto(s)
Trastornos de la Coagulación Sanguínea/etiología , Infecciones Meningocócicas/sangre , Sepsis/sangre , Enfermedad Aguda , Factores de Coagulación Sanguínea/análisis , Pruebas de Coagulación Sanguínea , Preescolar , Coagulación Intravascular Diseminada/etiología , Humanos , Lactante , Infecciones Meningocócicas/complicaciones , Sepsis/complicacionesRESUMEN
A large congenital mesoblastic nephroma (CMN) of combined classical and and cellular histological structure was removed from a 1-month-old female infant. The tumor extended extrarenally and may have been incompletely excised. Tumor tissue showed a mosaic hyperdiploidy with 54 chromosomes in the hyperdiploid line. No other antitumor therapy was given and there has been no recurrence after 4 years. Genomic imprinting normally prevents transcription of the maternal gene for insulin-like growth factor 2 (IGF2). Relaxation of IGF2 imprinting leading to abnormal transcription of the maternal gene is found in a majority of Wilms' tumors and in other malignant neoplasms. The biallelic transcription of IGF2 demonstrated in the CMN from this case is consistent with abnormal transcription of the maternal allele. Relaxation of imprinting of the maternal IGF2 gene or abnormal expression of the gene through other mechanisms may have a role in the genesis of CMN or the cellular subtype.
Asunto(s)
Diploidia , Impresión Genómica , Factor II del Crecimiento Similar a la Insulina/genética , Neoplasias Renales/genética , Neoplasias Renales/patología , Mesonefroma/genética , Mesonefroma/patología , Femenino , Humanos , Recién Nacido , Cariotipificación , Neoplasias Renales/cirugía , Mesonefroma/cirugía , PronósticoRESUMEN
From June 1981 through June 1989, 95 Polynesian children were seen for initial care of malignancy at the Princess Mary Hospital for Children (PMHC). The incidence of malignancy in the Polynesian populations served, the histology of the malignancies, and the outcome of therapy were reviewed and compared with 185 non-Polynesian (non-P) patients seen during the same period. Incidence figures for Polynesians and non-P were similar, but histologic patterns differed, showing an increased occurrence of leukemia, particularly nonlymphoblastic leukemia, an increased occurrence of bone tumors, and a decreased incidence of central nervous system tumors for Polynesians. Survival for Polynesian children with acute lymphoblastic leukemia was worse than for non-P. Survival in all other disease categories was similar.