Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A report of the occurrence of membranous nephropathy and peripheral neuropathy in a patient with systemic mastocytosis is presented. Previous reviews of patients with systemic mastocytosis have not noted this association. During cyclical therapy with prednisone and chlorambucil in this case, nephrotic-range proteinuria remitted. Peripheral neuropathy resolved 10 months after discontinuation of therapy. Pathophysiological mechanisms resulting in this clinical presentation may be immunologically mediated.
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PMID:Systemic mastocytosis associated with membranous nephropathy and peripheral neuropathy. 284 13

We report here the interesting case of a 76-year-old man with severe proteinuria who was diagnosed with systemic mastocytosis accompanied by a clonal non-mast-cell lineage haematological disorder (a non-secretory plasma cell dyscrasia). This is a unique report of systemic mastocytosis with a non-secretory plasma cell dyscrasia and nephrotic syndrome. The pathophysiological relevance between these entities along with the probability of occult amyloidosis is discussed.
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PMID:Systemic mastocytosis accompanied by a non-secretory plasma cell dyscrasia and nephrotic syndrome-level proteinuria in a 76-year-old patient. 2408 Nov 51

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