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)

IGA nephropathy (IGAN) is the most common glomerular disease worldwide. Patients may present with hematuria and non-nephrotic (NNRP) or uncommonly nephrotic range proteinuria (NRP). To the authors' knowledge, correlation of podocyte foot process effacement (FPE) with subclasses of IGAN and proteinuria (PT) has not been studied. Retrospectively, 161 cases of IGAN with light, immunofluorescence, and electron microscopy (EM) were reviewed and classified according to Haas classification. EM was available in 110 out of 161 (67%) cases. FPE was evaluated as mild, <30%; moderate, 30–70%; and severe, >70% and was correlated with class and the level of PT. Out of 161 cases, 101 were males and 60 were females with M:F ratio of 1.71:1. In 72 cases, race was known as follows: white, 63 (88%); black, 6 (8%); Hispanic, 2 (3%); Asian, 1 (1%). Clinical history was available in 94 cases: PT 39 cases (42%), PT+hematuria 33 cases (35%), hematuria 15 cases (16%), and renal failure in 7 cases (7%). In 88 cases with FPE, PT was nephrotic in 21 and non-nephrotic in 29 cases. FPE is common in IGAN. No correlation between FPE and IGAN subclass (p=.42) or proteinuria group and IGAN subclass (p=.10) is present. Whether FPE is simply a reflection of other pathologic mechanisms and its significance in the pathophysiology of IGAN requires further investigation.
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PMID:The significance of foot process effacement in immunoglobulin a nephropathy: clinicopathologic study of 161 cases with light, immunofluorescence and electron microscopic studies. 2056 84

Nephrotic syndrome is a rare presentation of IgA nephropathy. The degree of proteinuria in IgA nephropathy predicts poor prognosis. We herein report a teenager with IGA nephropathy, the nephrotic syndrome and segmental glomerular scars who after developing complications from high dose corticosteroid therapy was successfully treated with tacrolimus and low dose prednisone.
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PMID:Nephrotic syndrome is a rare manifestation of IGA nephropathy. 2761 69

Multiple myelomas (MM) of the immunoglobulin D (IgD) subtype is rare amongst plasma cell malignancies. It can present a diagnostic challenge because of the low amount of immunoglobulin in the serum. The amount of monoclonal (M)-protein is often undetectable on electrophoresis. Historically, survival in these patients was typically shorter compared to the immunoglobulin A (IgA) and immunoglobulin G (IgG) subtypes due to advanced disease upon presentation. With the advent of better diagnostic techniques, the prognosis of this disease is changing. We describe a case of an extramedullary testicular plasmacytoma (EMP) of the IgD subtype as the primary feature of MM, which responded well to novel therapy. A 72-year-old White male presented to the emergency room with a right testicular mass for three months. He subsequently underwent right radical orchiectomy. Pathology of the specimen revealed plasmacytoid cells positive for cluster of differentiation (CD79a), lambda free light chain, IgD, and BCL-1 (Cyclin D1) on immunochemical stains. Urine and serum immunofixation were positive for monoclonal IgD with lambda light chain specificity and Bence Jones proteinuria. Bone marrow biopsy showed large sheets of plasma cells with greater than 90% cellularity. Flow cytometry displayed atypical plasma cells expressing cluster of differentiation (CD38, CD20, and CD56) with cytoplasm and lambda light chain, approximately 20%, consistent with a plasma cell dyscrasia. Stage 3 IgD lambda multiple myeloma was diagnosed. He received novel treatment with Bortezomib and dexamethasone for three months, followed by Lenalidomide. His performance status and lab data improved significantly. He had progression-free survival (PFS) of approximately three years and remained in complete remission low-dose dose of Lenalidomide daily. IgD myeloma was considered a diagnostic challenge due to undetectable M-protein levels on serum protein electrophoresis (SPEP). With the advent of serum free light chain assay and serum and cytologic examinations, diagnostic accuracy has significantly improved. The IgD subtype is commonly associated with poor clinical outcomes. However, the use of novel agents and autologous transplant has changed the prognosis of this disease.
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PMID:An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma. 2884 77

Herein, we describe the first case of renal intravascular large B cell lymphoma in Korea occurring in a 66-year-old female. She presented with mild fever and dyspnea. On physical and laboratory evaluations, hemophagocytic lymphohistiocytosis was suspected, but the bone marrow biopsy results were unremarkable. During the work-up, massive proteinuria developed, which led to a renal biopsy. The renal architecture was relatively well-preserved, but the glomeruli were hypercellular with the infiltration of atypical, large lymphoid cells with increased nucleus-cytoplasm ratio and clumped chromatin. Similar cells were also present in the peritubular capillaries. The tumor cells exhibited membranous staining for CD20 and CD79a. After the diagnosis of intravascular large B cell lymphoma, the patient received rituximab-based chemotherapy under close follow-up.
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PMID:Renal intravascular large B cell lymphoma: the first case report in Korea and a review of the literature. 3278 26