title: [Nephrotic syndrome as a paraneoplastic symptom of Hodgkin's disease in two children].
abstract: A 13-year-old girl and a 3-year-old boy presented with the clinical symptoms of nephrotic syndrome. Both patients developed a nodular mass in the cervical region, which appeared to be due to Hodgkin's disease. The renal disease was a paraneoplastic phenomenon of Hodgkin's disease. Chemotherapy given for Hodgkin's disease also cured the nephrotic syndrome. A few years later one of the patients experienced a recurrent episode of nephrotic syndrome in conjunction with a recurrence of Hodgkin's disease but this was once again treated successfully. Nephrotic syndrome is a known but rare paraneoplastic syndrome accompanying Hodgkin's disease. It is thought that T cell dysfunction leads to a secretion of cytokines which alters the permeability of the glomerular basement membrane. This dysfunction is not only apparent during the period of active disease and this could clarify why paraneoplastic syndrome can exist or recur outside the period in which the malignancy is overt. The clear recognition of a paraneoplastic syndrome is important, as it is often only necessary to treat the malignancy.
authors.affiliation: Academisch Medisch Centrum/Universiteit van Amsterdam, locatie Emma Kinderziekenhuis, Meibergdreef 9, 1105 AZ Amsterdam. h.vandenberg@amc.uva.nl
authors.forename: M F
authors.initials: MF
authors.lastname: Raphaël
authors.forename: J W
authors.initials: JW
authors.lastname: Groothoff
authors.forename: H
authors.initials: H
authors.lastname: van den Berg
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D005260:Female; D006689:Hodgkin Disease / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local; D009404:Nephrotic Syndrome / Q000209:etiology*; D010257:Paraneoplastic Syndromes / Q000209:etiology*