RESUMEN
PURPOSE: The aim of this study was to investigate children presenting with malignant pelvic tumors obstructing the upper urinary tract. METHODS: Seventeen children with upper urinary tract obstruction by a malignant tumor were reviewed. A nephrostomy tube or Double J (DJ) stent was inserted into each obstructed urinary system and removed after tumor shrinkage and/or hydronephrosis regression. RESULTS: There were 9 boys and 8 girls in the study; the mean age and median follow-up were 5.7 years and 2.5 years, respectively. The most common obstructing tumor was rhabdomyosarcoma. Twelve children underwent diversion by nephrostomy tubes and 3 by DJ stents; 2 patients underwent resection of the tumors with ureteroureterostomy. Complications after the insertion of the stents included febrile urinary tract infections (UTI) or pyelonephritis in 4 of the children with DJ stents. In the nephrostomy group, febrile UTI developed in 3 and the tube fell out in 1, and was blocked in another. Of the 17 children, 9 have no evidence of disease, 2 are currently under treatment, and 6 died of cancer. CONCLUSIONS: The prognosis of children with malignant pelvic tumor obstructing the upper urinary system justifies urgent and optimal upper tract diversion, enabling chemotherapy to be started immediately.
Asunto(s)
Neoplasias Pélvicas/complicaciones , Obstrucción Ureteral/etiología , Obstrucción Ureteral/cirugía , Derivación Urinaria , Preescolar , Femenino , Estudios de Seguimiento , Humanos , MasculinoRESUMEN
We studied the extent to which patient characteristics influenced outcome in childhood idiopathic thrombocytopenic purpura in a historical cohort of 289 children over a 20 year period (1968-87). Outcome was classified as acute or chronic depending on whether the platelet count had returned to normal (150 X 10(9)/l) by six months after diagnosis. Fifty three cases (18%) had chronic idiopathic thrombocytopenic purpura. The likelihood of chronic disease was determined by logistic regression analysis of five patient variables: age, sex, season of onset of symptoms, history of recent viral illness, and duration of symptoms at presentation. A history of symptoms of greater than 14 days at presentation, adjusted for the other variables, was strongly predictive of chronic idiopathic thrombocytopenic purpura; the other variables did not significantly affect outcome. At 28 days after diagnosis 138 (47%) of the study cohort had normal platelet counts. Children whose platelet counts were less than 150 X 10(9)/l had a threefold risk of progressing to chronic idiopathic thrombocytopenic purpura, which increased to fivefold if counts were less than 50 X 10(9)/l. Two thirds of patients in the chronic group, irrespective of treatment, remained thrombocytopenic two years after diagnosis. We conclude that a history of symptoms for greater than two weeks at presentation is strongly predictive of chronic idiopathic thrombocytopenic purpura. If platelet counts are subnormal 28 days after diagnosis the risk of chronic idiopathic thrombocytopenic purpura is increased with prolonged thrombocytopenia being very likely if platelet counts remain low three months after diagnosis.
Asunto(s)
Púrpura Trombocitopénica/complicaciones , Adolescente , Factores de Edad , Australia/epidemiología , Niño , Preescolar , Enfermedad Crónica , Estudios de Cohortes , Femenino , Humanos , Inmunización Pasiva , Lactante , Masculino , Oportunidad Relativa , Recuento de Plaquetas , Prednisolona/uso terapéutico , Pronóstico , Púrpura Trombocitopénica/mortalidad , Púrpura Trombocitopénica/terapia , Esplenectomía , Factores de TiempoRESUMEN
A male infant with reticular dysgenesis received a bone-marrow transplant from his HLA-identical brother. Severe graft-versus-host disease developed but he responded to high-dose methylprednisolone. 3 years after grafting, the child is thriving with full haematological reconstitution and normal cell-mediated and humoral immunity. This is the first report of the survival beyond 17 weeks of a child with reticular dysgenesis.