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)

Renal disease has not been considered a major late complication of bone marrow transplantation. Of 31 evaluable pediatric patients undergoing allogeneic or autologous bone marrow transplantation for neuroblastoma or acute lymphoblastic leukemia, 14 developed a hemolytic anemia, microscopic hematuria, and renal insufficiency at a median of 5 months (range, 3 to 7 months) posttransplant. Renal biopsies were performed in two patients at the onset of kidney disease and showed mesangiolysis with intraglomerular capillary aneurysm formation, mesangial proliferation, and focal thickening and splitting of the glomerular basement membranes. The clinical presentation, time to onset of renal disease, and biopsy material are consistent with a diagnosis of radiation nephritis, a previously uncommon finding in this patient group. The high incidence of this syndrome in the current report may have been due to the combination of intensive chemotherapy and total-body irradiation in the conditioning regimens.
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PMID:Intravascular hemolysis and renal insufficiency after bone marrow transplantation. 304 40

To document the incidence and severity of symptomatic hepatomegaly in patients with Stage IV-S neuroblastoma, we reviewed the charts of children with neuroblastoma seen at Children's Hospital of Pittsburgh between 1951 and 1985. Sixteen met the criteria for IV-S disease, and 11 of these (69%) had massive hepatomegaly. Five children had symptoms referable to their liver size including respiratory distress, gastroesophageal reflux, or decreased urine output. Liver function appeared to be normal or only mildly abnormal in the 9 patients where data were available. Because therapy was so variable, it was not possible to correlate treatment regimen with outcome. However, 3 symptomatic patients who received less than or equal to 600 rad without chemotherapy had prompt subjective responses. Follow-up was available on 10 children 6 months-18 years (median 18 years) from diagnosis. Eight were alive with resolved or resolving hepatomegaly. High dose (greater than or equal to 3,300 rad) radiation-related side effects included multiple rib chondromas, chest- and pelvic-wall hypoplasia in one patient, and radiation nephritis with hepatic fibrosis resulting in death of a second patient. Our results support prior recommendations that for symptomatic hepatomegaly, low doses of radiation be considered.
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PMID:Results of therapy in stage IV-S neuroblastoma with massive hepatomegaly. 362 25

The records of 207 neuroblastoma patients seen at the Children's Hospital of Philadelphia between 1944 and 1977 were reviewed to study some of the features associated with the unusually good prognosis found in patients with Stage IV-S neuroblastoma. Initially, 22 patients appeared to fit the criteria of small primary tumor and distant disease in liver, skin, and/or marrow without evidence of bone metastases; 5 patients were subsequently rejected as being incorrectly staged. The remaining 17 patients had abdominal primary tumors and hepatic disease; in 12 of the 17, an enlarged liver was the presenting sign. Six patients had skin lesions, 4 had disease in the marrow on routine smear, and additional sites of spread were pancreas and bowel serosa. The treatment given was not systematic, and it was not possible to correlate any specific form of therapy with a satisfactory outcome. Eleven of 17 patients survived; 6 of 11 survivors had spontaneous regression of all or part of their diseases, 5 of 6 who died received irradiation, chemotherapy, or both. Death usually occurred in the first month as a complication of the local disease; 1 patient succumbed to radiation nephritis. This study establishes that the special pattern of widespread neuroblastoma termed Stage IV-S does exist, and that is associated with a good prognosis. Careful consideration should be given before selecting treatment for the Stage IV-S child because spontaneous regression is likely to occur in most of them. In patients with rapidly enlarging livers, renal or pulmonary complications may develop because of liver bulk or coagulopathies. Treatment should be directed to the liver in these cases because distant metastases seldom supervene. Low-dose irradiation, mild chemotherapy, and possibly surgical release of intraabdominal pressure using a silastic patch have all been effective. Unfortunately, patients occasionally succumb to local disease in spite of these and more aggressive measures.
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PMID:A review of 17 IV-S neuroblastoma patients at the children's hospital of philadelphia. 726 Aug 34

Bone marrow transplant (BMT) nephropathy is characterized by the acute onset of nephritis more than 100 days after BMT. The renal lesion in BMT nephropathy is similar to radiation nephritis, but BMT nephropathy occurs earlier and with lower radiation doses than radiation nephritis. The combined effects of chemotherapeutic agents and nephrotoxic drugs given before and after BMT appear to sensitize or unmask radiation nephritis. Reporting of drugs that may contribute to BMT nephropathy is critical for the development of optimal treatment regimens. Herein, we report two cases of BMT nephropathy that developed coincident with retinoic acid therapy. Both patients received autologous BMT for neuroblastoma after preparative therapy with total body irradiation/melphalan/carboplatin/etoposide. They were randomized to receive cis-retinoic acid as part of a clinical trial. Both patients developed acute nephritis during their second 2-week course of retinoic acid on post-BMT days 105 and day 139. The nephritis was associated with hypertension, anemia, thrombocytopenia, azotemia, hematuria, and proteinuria. Clinical features, laboratory evaluation, and renal biopsy indicated that these two patients developed radiation-induced BMT nephropathy. The fact that both patients developed nephritis concurrent with retinoic acid therapy raises a concern that retinoic acid may have unmasked radiation injury and triggered BMT nephropathy.
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PMID:Possible association of retinoic acid with bone marrow transplant nephropathy. 1060 14

Three children with renal hypertension are described. Two had histories of neuroblastoma treated by surgical resection and chemotherapy. They both presented later with unilateral atrophic kidney and marked hypertension. Only the child with severe cardiac failure demonstrated high plasma brain natriuretic peptide (BNP) concentrations. The remaining patient had a history of chronic nephritis treated with continuous ambulatory peritoneal dialysis. She also had chronic hypertension and severe cardiac failure. This child demonstrated high plasma BNP levels. The endogenous secretion of BNP is not triggered by hypertension alone, even though exogenous BNP has the pharmacological effect of reducing renin activity.
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PMID:Plasma brain natriuretic peptides and renal hypertension. 1095 34

Bone marrow transplant nephropathy (BMTN) classically presents more than 100 days after transplantation as an acute nephritis with hypertension, azotaemia and anemia that usually results in end stage renal failure (ESRF). The risk of developing BMTN may be greater with the use of more intensive chemotherapy and higher total body and tumor bed irradiation. Cis-retinoic acid (RA) may further increase the risk of developing BMTN. Here, we report the cases of two children who developed typical clinical and biochemical features of BMTN. They were both treated for stage IV neuroblastoma with chemotherapy, bone marrow transplant (BMT) conditioning that included total body irradiation and RA therapy after BMT, although the patient in case 1 had established renal insufficiency prior to the commencement of RA. Renal biopsy of these children showed classical BMTN histology, and the renal manifestations progressed quickly; the patient in case 1 became dialysis dependent by 1 year post-bone marrow transplant. Recently, RA has been added to the post-BMT therapy in children with stage IV neuroblastoma. The occurrence of BMTN in two children treated with RA in our unit is unlikely to be coincidental. Although RA has been shown to confer a significant survival advantage in this disease, animal studies and a previous case report have suggested it could increase the toxic effects of chemotherapy and renal irradiation. It is likely that RA contributed to the deterioration in renal function in these patients.
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PMID:Retinoic acid may increase the risk of bone marrow transplant nephropathy. 1571 54