Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P10721 (c-kit)
 6,575 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 69-year-old man was admitted with a large elastic mass in the upper abdomen. Computed tomography revealed a massive tumor in contact with the liver and gastrointestinal endoscopy revealed a gastric adenocarcinoma. He developed acute renal failure with massive proteinuria and died with a marked enlargement of the tumor. Autopsy revealed a tumor located in the lesser omentum. The tumor was considered to be a Gastrointestinal Stromal Tumor (GIST) because it was positive for c-kit. In addition, crescent formations and immune complexes in glomeruli were observed. We report the first case of GIST complicated by rapidly progressive glomerulonephritis and gastric carcinoma.
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PMID:Malignant gastrointestinal stromal tumor originating in the lesser omentum, complicated by rapidly progressive glomerulonephritis and gastric carcinoma. 1500 50

Imatinib is a receptor tyrosine kinase inhibitor that blocks the activity of c-Abl, c-Kit, and PDGF receptors. We tested the protective effects of imatinib in thymic stromal lymphopoietin transgenic mice, a model of cryoglobulinemia and associated membranoproliferative glomerulonephritis (MPGN), in which some glomerular manifestations likely result from PDGF receptor activation. Surprising, administration of imatinib beginning at weaning suppressed production of cryoglobulin, attenuating both the renal injury and systemic features of cryoglobulinemia. Flow cytometry suggested that inhibition of B cell development in the bone marrow likely caused the reduction in cryoglobulin production. In addition, administration of imatinib to thymic stromal lymphopoietin transgenic mice with established MPGN also diminished cryoglobulin production and reversed the renal and systemic lesions. These data suggest that treatment with imatinib may be a novel therapeutic approach for cryoglobulinemia and MPGN in humans.
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PMID:Imatinib suppresses cryoglobulinemia and secondary membranoproliferative glomerulonephritis. 1902 5

Mastocytosis is a rare myeloproliferative disease in which mast cells abnormally accumulate in the skin, bone marrow, intestine, liver, spleen, and lymph nodes. Characterized by uncontrolled proliferation of aberrant mast cells, the disease can present either cutaneously or systemically. Mast cells facilitate the immune response and inflammation, and mastocytosis with renal involvement has been rarely reported in adults. Here, we describe a pediatric case of renal involvement in a patient with mastocytosis. A 12-year-old female with mastocytosis was admitted for edema, foamy urine, and gross hematuria. Initial laboratory findings showed azotemia, proteinuria, and hematuria. Renal biopsy findings were compatible with diffuse proliferative glomerulonephritis (DPGN). Immunofluorescence analysis of CD117 (c-Kit) staining resulted positive for rare infiltrating cells. These findings are unusual for primary glomerulonephritis (GN), and secondary GN is typically associated with mastocytosis. According to the literature, steroid treatment can be attempted in cases with renal disease associated with systemic mastocytosis. Therefore, the patient was treated with oral prednisolone, and proteinuria and hematuria disappeared after 4 months of treatment. After 5 months, prednisolone treatment was stopped, and the skin lesion improved. The renal function 22 months after prednisolone treatment was normal. This is a unique report of mastocytosis with DPGN in a child. c-Kit staining can be helpful for diagnosis, and the response to steroid treatment is favorable. Further study about the pathological relevance between mastocytosis and GN is necessary.
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PMID:Renal involvement and favorable outcome in a child with mastocytosis. 3283 Nov 56