title: Tubular function and histological findings in ifosfamide-induced renal Fanconi syndrome--a report of two cases.
abstract: Two patients developed renal Fanconi syndrome (RFS) after intensive long-term chemotherapy for metastatic Ewing sarcoma and disseminated neuroblastoma. Whereas RFS was diagnosed in patient 1 before he developed osteomalacia, patient 2 experienced severe rickets and growth retardation. Renal function studies revealed slight glomerular impairment and severe tubular defects leading to increased excretion of glucose, amino acids, inorganic phosphate and low molecular weight proteins, indicating proximal tubular damage. Patient 2 additionally showed distal tubular dysfunction with acidosis and diminished concentrating capacity. Renal biopsy in patient 1 revealed marked proximal tubular defects without interstitial lymphocytic infiltration. In both patients renal damage could most likely be ascribed to previous ifosfamide (IFOS) therapy. Our patients showed no improvement in renal function after cessation of IFOS treatment, indicating a poor prognosis of once established RFS after IFOS therapy. Measurement of tubular reabsorption capacities provides exact information on the extent of tubular toxicity induced by IFOS and may be used to monitor IFOS treated patients.
authors.affiliation: Department of Haematology and Oncology, University Children's Hospital Münster, Federal Republic of Germany.
authors.forename: R
authors.initials: R
authors.lastname: Rossi
authors.forename: U
authors.initials: U
authors.lastname: Helmchen
authors.forename: G
authors.initials: G
authors.lastname: Schellong
mesh_terms: D000293:Adolescent; D002648:Child; D005198:Fanconi Syndrome / Q000139:chemically induced* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000009:adverse effects* / Q000627:therapeutic use; D007684:Kidney Tubules / Q000473:pathology / Q000503:physiopathology; D008297:Male
chemical_list: D007069:Ifosfamide