Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between 1980 and 1986, 44 children with acute lymphoblastic leukemia (ALL) or Stage IV neuroblastoma (NB) underwent allogeneic or autologous bone marrow transplantation (BMT). Twenty-nine of these patients were alive and in remission 3 months post BMT and were evaluable for this analysis of whom eleven have developed renal dysfunction. Six of 17 (35%) evaluable ALL patients developed renal dysfunction (3.5 to 6 months post BMT). This group was transplanted for CALLA positive ALL and received an autologous transplant. Preparation included tenopiside (VM 26) cytosine arabinoside, and cyclophosphamide followed by total body irradiation (TBI). One patient received 850 cGy in a single fraction, while all other patients received fractionated TBI (1200-1400 cGy in 6-8 fractions over 3-4 days). Five of 7 (71%) evaluable patients who received a BMT for NB have developed late renal problems (4-7 months after BMT). The preparation for NB patients included VM 26, cis-platinum, melphalan, cyclophosphamide, and fractionated TBI (1200-1296 cGy). All seven NB patients had received cis-platinum as induction treatment prior to transplantation. All patients presented with anemia, hematuria, and elevations of BUN and creatinine. Two patients underwent renal biopsies which were consistent with radiation nephropathy or hemolytic uremic syndrome. In conclusion, a high incidence of renal dysfunction has occurred 3 to 7 months after BMT for children with NB and ALL. The clinical and laboratory features are consistent with either acute radiation nephropathy or hemolytic-uremic syndrome. These patients were prepared for BMT with multiple chemotherapeutic agents as well as TBI. The relatively young age of these patients and conditioning with intensive multi-agent chemotherapy may decrease the tolerance of the kidney to radiation injury.
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PMID:Late onset of renal dysfunction in survivors of bone marrow transplantation. 296 67

Encouraging results are reported with high-dose chemotherapy and total body irradiation followed by autologous bone marrow transplantation in the treatment of advanced neuroblastoma. However, relapse remains a significant problem. We used high-dose chemotherapy, surgery, intraoperative radiation and an autologous bone marrow transplant treated in vitro to remove tumor cells followed by 13-cis-retinoic acid to treat 36 children with advanced neuroblastoma. This comprehensive treatment appears to improve the survival rate of patients with advanced neuroblastoma, including those with N-myc amplification and bony involvement. The disease-free survival rate was 66% (95% confidence interval, 49-84%) at 3 years. All patients who received 13-cis-retinoic acid developed cheilitis, but no bone marrow depression occurred in these patients. Five patients developed hemolytic uremic syndrome (HUS) post-transplant. This may have been related to the procedure used for total body irradiation. Patients who had their kidneys shielded during this procedure did not develop this syndrome. Patients who received local irradiation at the primary site showed no evidence of relapse in this region, indicating that such therapy may help to prevent a relapse. These data suggest a high rate of 3 year disease-free survival with this treatment strategy. The nonrandomized nature of the study and use of multiple modalities precludes analysis of the specific contribution of each.
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PMID:Comprehensive treatment of advanced neuroblastoma involving autologous bone marrow transplant. 757 51

Of 193 children who underwent hematopoietic stem cell transplantation (HSCT) for various malignancies, 10 developed hemolytic uremic syndrome (HUS) 1 1/2-5 months later. All 10 had microangiopathic hemolytic anemia, thrombocytopenia and impaired renal function. Six of 10 presented with pericardial effusion, while three presented with hypertension. No child required plasma exchange, and all patients have survived without life-threatening long-term sequelae. By univariate analysis, the underlying diagnosis of neuroblastoma and a history of cisplatin (CDDP) administration were significantly associated with the development of HUS (P < 0.0001). By multivariate analysis using logistic regression, neuroblastoma and use of total body irradiation (TBI) as a conditioning regimen were significant contributing factors for HUS (P = 0.0001 and 0.036, respectively). Although CDDP administration could not be evaluated because of its strong correlation with the underlying diagnosis, we speculate that CDDP may enhance the nephrotoxicity of TBI, leading to a high incidence of HUS in patients with neuroblastoma.
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PMID:Hemolytic uremic syndrome after allogeneic or autologous hematopoietic stem cell transplantation for childhood malignancies. 948 51