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)

During a prospective screening for proteinuria in diabetic patients, isolated Bence-Jones proteinuria was detected in 2 cases. The first patient, a 52-year-old black female, was seen for evaluation of a slow but progressive weight loss which was attributed to poor adjustment of insulin therapy. The patient gained weight after an increase of the daily insulin administration. She had plasmocytosis in a bone marrow aspirate, but no other evidence of myelomatosis. The second patient, a 59-year-old black male who was seen for routine evaluation of his diabetes, had no clinical or laboratory evidence of myelomatosis. Although precise definition of these cases as "benign" or "idiopathic" Bence-Jones proteinuria is impossible without prolonged follow-up, at the time of presentation they appeared to fit this classification. This observation is one further example that isolated Bence-Jones proteinuria may be seen without any evidence of malignant B-cell dyscrasia.
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PMID:"Idiopathic" Bence-Jones proteinuria. 10 Oct 13

From a review of 17 cases of Fanconi syndrome with Bence Jones proteinuria and myeloma or amyloidosis, including three new cases reported here in detail, there emerges a well defined set of characteristics. In most cases, the diagnosis of Fanconi syndrome preceded the development of myeloma or amyloidosis. Myeloma preceding the development of Fanconi syndrome has not been reported. All the patients had Bence Jones proteinuria, but in some it could be detected only by electrophoresis or immunoelectrophoresis, In the seven cases in which the Bence Jones protein was typed, it was of kappa type. There were no serum protein monoclonal abnormalities. In the bone marrow and renal samples of half of the patients, crystalline cytoplasmic inclusion bodies were present in lymphoplasmacytic elements and renal tubular cells. It is proposed that patients with Fanconi syndrome and Bence Jones proteinuria have a distinct type of plasma cell disorder or variant of the monoclonal gammopathies, characterized by a slow progression of the tumor and by an early phase dominated by the metabolic complications of the renal proximal tubular dysfunction. Adult patients with Fanconi syndrome should be carefully investigated for the presence of Bence Jones protein and a plasmacytic dyscrasia should be excluded.
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PMID:Fanconi syndrome in adults. A manifestation of a latent form of myeloma. 16 83

A patient with a single functioning kidney presented with the nephrotic syndrome. On the basis of highly selective proteinuria, a diagnosis was made of lipoid nephrosis. Steroid therapy over a 2-year period did not control the disease. The patient eventually developed end-stage renal failure and malignant hypertension. Nephrectomy was performed to control the hypertension. Histological examination showed congenital dysplasia in one kidney and sclerosing glomerulonephritis, malignant nephrosclerosis, as well as dysplastic changes in the other.
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PMID:Sclerosing glomerulonephritis and malignant hypertension in a patient with congenital renal dysplasia: A case report. 55 Apr 41

Six cases of leukemic myelomatosis (plasma cell leukemia), are reported. A spectrum of leukemic plasmacellular proliferation was observed in this group: in two cases the plasmacytic leukemia was a terminal event of multiple myeloma, in one of a chronic lymphocytic leukemia, and in the remaining three cases the peripheral blood plasmacytosis was a presenting manifestation of an acute immunocytic dyscrasia. The clinical features, frequencies of extraosseous plasmacytic infiltrations, hypercalcemia and Bence Jones proteinuria, and cytomorphologic difficulties in differentiating plasmacytic from chronic lymphocytic leukemia are discussed.
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PMID:Leukemic myelomatosis (plasma cell leukemia). 70 28

In two patients with terminal renal failure, manifestations of disease developed in multiple organ systems. One had a previous diagnosis of multiple myeloma with kappa light chain proteinemia and proteinuria. The other had idiopathic lobular glomerulonephritis. Hepatic and neurologic abnormalities developed in both; in the latter gastrointestinal, cardiac and endocrine disease developed as well. Clinical and pathologic correlations suggest that the retention and tissue deposition of light chains produced the organ dysfunction, inasmuch as free kappa light chain determinants were demonstrated histologically in the clinically affected organs. The deposition in these patients may be an extreme example of a common but previously unrecognized form of plasma cell dyscrasia.
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PMID:Manifestations of systemic light chain deposition. 81 12

Sixty-two of 1242 patients with M components were found to have lymphoma. There were 33 patients with immunoglobulin M(IgM), 20 WITH IgG, 5 with IgA, and one patient with Bence Jones protein M components. Three patients had biclonal gammopathy. The types of lymphoma were: lymphocytic, 31; histiocytic, 12; mixed cell, 4; stem cell, 2; Burkitt's, 1; Hodgkin's disease, 9; and unclassified, 3. All patients were in stages III or IV of lymphoma, and the average duration of disease was 29.3 months when M components were detected. Anemia, abnormal peripheral blood lymphocytes, and lymphomatous involvement of the bone marrow were especially common among patients with IgM M components. Osteolytic lesions were found in 12 patients and osteosclerotic lesions in one. A second malignancy occurred in eight patients. The level of M component was below 1.0 gm/dl in 55 per cent of patients. Significant suppression of normal immunoglobulin levels in the serum was noted in 4 and 16 patients with IgG and IgM components, respectively. Bence Jones proteinuria was found in 19 per cent, cryoglobulinemia in 11 per cent, and cold agglutinins, all of anti-i specificity, in 10 per cent of the patients. Most of the M components decreased during therapy. Only two M components gradually increased. The mean survival of 39 patients who died was 10.4 months. The living patients have been followed for a mean period of 21.2 months. The presence of M components in lymphoma may suggest B cell origin of the tumor but the coexistence of plasma cell dyscrasia cannot be ruled out.
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PMID:M components associated with lymphoma: a review of 62 cases. 82 10

A 12-year-old boy suffered from recurrent respiratory infections, an immune deficiency characterized by the complete absence of IgG, IgA, IgD, and the salivary "secretory component" associated with a plasma cell dyscrasia. Rectal and kidney biopsy specimens showed amyloid deposits. Amyloidosis has been extensively studied by several groups of investigators in patients with plasma cell dyscrasia in general, and in patients with lambda-Bence Jones proteinuria in particular, but the finding of a monocional serum IgM-lambda component in an agammaglobulinemic child with Bence Jones proteinuria and amyloidosis represents a puzzling clinical syndrome. Searching for monoclonal components in patients with amyloidosis, even if they are agammaglobulinemic, is important. We also discuss here the "mixed type" of amyloid deposition and its relation to the recurrent infections and the plasma cell dyscrasia in this patient.
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PMID:Agammaglobulinemia, plasma cell dyscrasia, and amyloidosis in a 12-year-old child. 86 21

During a 40 month interval, 35 patients were seen with a plasma cell dyscrasia in which the only detectable monoclonal immunoglobulin abnormality consisted of excess production of either type kappa or type lambda light chains (Bence Jones protein). This group constituted 17.3 per cent of the total number of patients with plasma cell dyscrasias and 25.7 per cent of the patients with myeloma identified during the same period. Variable initial clinical presentation, the absence of a typical monoclonal serum spike and the unreliability of commonly used urine protein tests made recognition of the disorder difficult in many patients. Sulfosalicylic acid and p-toluene sulfonic acid proved valuable in screening for ueine protein. Definition of "proteinuria" by quantitative, electrophoretic and immunochemical studies was essential for diagnosis. Bence Jones proteinemia was present in 80 per cent of the patients; panhypogammaglobulinemia and lytic bone lesions were demonstrable in more than 60 per cent. Although no specific morphologic abnormality of marrow plasma cells was evident by light microscopy, the absence of rouleau on peripheral blood smear was a helpful diagnostic clue. Although patients with lambda light chains presented with more Bence Jones proteinuria, this did not correlate with the severity of initial renal functional impairment or with survival when compared to patients with kappa light chains. No other clinical or laboratory observation differentiated the groups with kappa light chains from those with lambda light chains. Amyloid was identified in seven patients. Their course was dominated by the features of primary systemic amyloidosis instead of the usual findings of classic myeloma. Patients with amyloidosis had lower initial serum albumin levels, fewer lytic bone lesions and reduced survival compared to patients without amyloidosis.
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PMID:The clinical spectrum of light chain myeloma. A study of 35 patients with special reference to the occurrence of amyloidosis. 113 Apr 19

We studied three patients with late onset, chronic sensorimotor and autonomic neuropathy in course of plasma cell dyscrasia with Bence Jones proteinuria. Histopathological findings of nerve biopsies consisted in diffuse loss of myelinated and unmyelinated fibers associated with perivascular deposits of amorphous material with physico-chemical and ultrastructural features of amyloid. By immunohistochemistry, light chains of the same type as Bence Jones protein, components of the classic and lytic pathways of the complement and vitronectin were detected at the level of amyloid nodules. The colocalization of complement neoantigen and vitronectin suggests that this complex derives from the circulation. The elucidation of the chemical composition of amyloid might shed some light in the pathogenesis of these disorders.
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PMID:Complement neoantigen and vitronectin are components of plaques in amyloid AL neuropathy. 138 57

We describe a 39-year-old man who developed kappa light chain nodular glomerulosclerosis with superimposed conspicuous crescent formation and extensive tubulointerstitial injury. The clinical picture was characterized by nephrotic syndrome and rapidly progressive glomerulonephritis. Incessantly progressive loss of renal function culminated in irreversible renal failure 7 weeks after initial manifestations of renal insufficiency. The patient has since been maintained on thrice weekly hemodialysis with chemotherapy for five years. At the time of pathologic diagnosis by renal biopsy, there was no evidence of multiple myeloma, and no serum M-component or Bence-Jones proteinuria was detected. An initial bone marrow aspirate revealed the presence of 0.6% atypical lymphocytes as the sole abnormality, although these were later identified as atypical plasma cells. These cells had also infiltrated the renal interstitium. Crescentic kappa light chain nodular glomerulosclerosis lacking evidence of plasma cell dyscrasia should be included in the differential diagnosis of rapidly progressive glomerulonephritis.
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PMID:Kappa light chain nodular glomerulosclerosis with conspicuous crescent formation and tubulointerstitial injury. Report of a case. 150 4


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