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)

One to three-month-old A-strain mice, inoculated subcutaneously with 2 x 10(6) viable syngeneic C1300 neuroblastoma cells (clone NB9R) developed a palpable tumor within 9-12 days and died within 28-30 days. A transient glomerulopathy developed after 16-24 days. Despite a normal histologic appearance, the nephropathy was clearly demonstrated by electron microscopy and was classified as a focal mesangiopathic glomerulonephritis. Deposits of host 7S-G immunoglobulins and C3 complement fragments were detected in these same kidneys by immunofluorescence. Radioimmunoprecipitin determinations on sera obtained from mice at different intervals from tumor cell inoculation, revealed that untreated mice contained circulating antibodies capable of reacting with 125I-labeled gp69-71 glycoprotein from Gross murine leukemia virus (MuLV). Antibodies to p30 MuLV antigen and to crude membrane antigen (s) (CMA) solubilized from NB9R cells were found in sera only after tumor cell inoculation. Circulating immune complexes formed by host 7S-G immunoglobulins were clearly detected from day 16 to 22. Antibodies eluted from kidneys with nephropathy were shown to react with NB9R cells in vitro and to react specifically with CMA and the p30 MuLV antigen.
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PMID:Antibody formation and transient immune complex glomerulopathy in A-strain mice with C1300 neuroblastoma tumors. 14 55

Improving survival rates following pediatric bone marrow transplantation (BMT) will likely result in greater numbers of children progressing to end-stage renal disease (ESRD) because of prior chemotherapy, irradiation, sepsis, and exposure to nephrotoxic agents. Renal transplantation remains the treatment of choice for ESRD; however, the safety of renal transplantation in this unique population is not well established. We report our experience with living related renal transplantation in three pediatric patients with ESRD following prior BMT. Two patients with neuroblastoma and ESRD because of BMT nephropathy, and one patient with Schimke immuno-osseous dysplasia and ESRD because of immune complex mediated glomerulonephritis and nephrotic syndrome. Age at time of BMT ranged from 2 to 7 yr. All patients had stable bone marrow function prior to renal transplantation. Age at renal transplant ranged from 8 to 14 yr. All three patients have been managed with conventional immunosuppression, as no patient received a kidney and BMT from the same donor source. These patients are currently 7 months to 6 yr status post-living related transplant. All have functioning bone marrow and kidney transplants, with serum creatinine levels ranging 0.6-1.2 mg/dL. There have been no episodes of rejection. One patient with a history of grade III skin and grade IV gastrointestinal-graft-vs.-host disease (GI-GVHD) prior to transplantation, had a mild flare of GI-GVHD (grade I) post-renal transplant and is currently asymptomatic. The incidence of opportunistic infection has been comparable with our pediatric renal transplant population without prior BMT. One patient was treated for basal cell carcinoma via wide local excision. Renal transplantation is an excellent option for the treatment of pediatric patients with ESRD following BMT. Short-term results in this small population show promising patient and graft survival, however long-term follow-up is needed. Pre-existing immune system impairment and bone marrow function should be taken into consideration when weighing different immunosuppressive agents for renal transplantation. Patients who have undergone renal transplantation following BMT are at high risk for opportunistic infections and malignancy, and need life-long medical surveillance.
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PMID:Successful renal transplantation following prior bone marrow transplantation in pediatric patients. 1536 89

The glial cell line-derived neurotrophic factor (GDNF)-RET signaling pathway plays an important role in kidney development. We have previously identified a novel zinc finger protein, glial cell line-derived neurotrophic factor-inducible zinc finger protein 1 (GZF1), whose expression was induced in the human neuroblastoma cell line TGW expressing RET by GDNF stimulation and was also detected in mouse metanephric kidney. In the present study, we examined the immunohistochemical expression of GZF1 in normal human kidney and various kidney diseases including chronic kidney disease, acute kidney injury, and cancers, and assessed the clinical significance of GZF1 expression. In the normal kidney, GZF1 was highly expressed only in the proximal tubular epithelial cells that were also positive for angiotensin-converting enzyme. We also evaluated GZF1 expression in various kidney diseases including membranous nephropathy, minimal change nephrotic syndrome with or without acute kidney injury, immunoglobulin A nephropathy, diabetic nephropathy, acute tubular necrosis, and antineutrophil cytoplasmic antibody-related glomerulonephritis. We found that decreased expression of GZF1 was associated with an increase in tubulointerstitial damage and serum creatinine levels. In addition, GZF1 expression was undetectable or very low in most cases of renal cell carcinomas and Wilms tumors. These findings suggest that GZF1 represents a new marker for renal proximal tubules and that there is an inverse correlation between the expression level of GZF1 and tubular function.
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PMID:Analysis of glial cell line-derived neurotrophic factor-inducible zinc finger protein 1 expression in human diseased kidney. 2129 9