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 27-year-old woman with proteinuria and normal serum creatinine for 10 years developed chronic lymphocytic leukaemia (CLL) and after further 2 years during treatment with prednisone and chlorambucil a malignant histiocytoma appeared. Free mu-chains were demonstrated in serum, but it is possible that mu-chain disease (mu-CD) and Bence-Jones proteinuria had persisted unrecognized for 12 years, since vacuolated plasma cells were found when the primary bone marrow smear was re-evaluated and no glomerular disorders were found. The clinical findings from the now 11 known cases of mu-CD are reveiwed and the immunochemical findings are reported ain part II (Axelsen et al 1976). The patient's mother died of acute leukaemia at the age of 24. The mother's sister has mammary carcinoma and several members of the mother's line apparently have increased tendency to infections. Of 27 family members, none exhibited immunoglobulin defects, but 10 members of the mother's line had B- as well as T-lymphocytosis and 5 members had leukocytosis.
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PMID:mu-chain disease in a case of chronic lymphocytic leukaemia and malignant histiocytoma. I. Clinical aspects. 17 30

There is considerable circumstantial evidence relating neoplasia to glomerular injury. Recently, more convincing evidence has been derived from the demonstration of tumor-associated antigen or antibody to such antigen, in relation to glomerular basement membranes in four patients with glomerular injury and cancer. The most common form of glomerulopathy reported in patients with carcinoma has been membranous glomerulonephritis. However, increased mesangial cells and matrix have also been found in some patients with hematuria and progressive renal failure. In contrast, most patients with Hodgkin's disease and glomerulopathy have had the minimal lesion-type nephrotic syndrome, which has usually responded to successful treatment of the Hodgkin's disease. Glomerular abnormalities have also been reported with chronic lymphocytic leukemia, lymphosarcoma, Waldenstrom's macroglobulinemia, and benign tumors. When there is no apparent cause, proteinuria with or without hematuria or impaired renal function should suggest the possibility of associated neoplasia, particularly in elderly patients.
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PMID:Glomerular injury in patients with neoplasia. 18 Aug 69

Chronic administration of a potent oral carcinogen N-Methyl-N-Nitroso guanidine (M.N.N.G.) failed to produce gastric carcinoma in CFHB Wistar rats sensitised to 2.4 dinitrochlorobenzene (D.N.C.B.). In addition animals receiving both D.N.C.B. alone and D.N.C.B. and M.N.N.G. developed a severe nephropathy characterised by heavy proteinuria and extensive renal cortical damage. The histological features include proliferation of parietal epithelial cells and mesangial sclerosis. An association between the nephropathy and long term D.N.C.B. administration is suggested and on the basis of investigation to date direct toxicity rather than immune complex disease seems more likely.
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PMID:A nephropathy occuring in rats treated with dinitrochlorobenzene and N-methyl-N-nitroso guanidine. 118 Aug 8

The incidence of intestinal adenocarcinoma in Crohn's ileal disease is reported in the literature to be higher than in the normal population. The authors report two cases of adenocarcinoma observed during the course of Crohn's ileitis and, by comparing these findings with earlier published data, highlight their characteristics. Two patients, a 53-year-old woman and a 62-year-old man, are reported as suffering from Crohn's ileitis. The woman underwent resection of the right colon with ileo-transversostomy due to stenosis twenty years earlier, whereas diagnosis in the male patient dates back to an appendicectomy 11 years earlier. Surgery, which was performed in both due to subocclusion which failed to respond to steroid therapy, showed the typical alterations of Crohn's disease. An adenocarcinoma was also found in both patients in the stenotic zone with highly dysplasic foci. The man presented a monoclonal gammopathy of an IgA-type with light lambda chains and Bence-Jones' proteinuria. Both patients are living and enjoy apparently good health respectively 2 and 6 years after surgery. The authors emphasize that the association between Crohn's ileal disease and intestinal adenocarcinoma cannot be considered casual. In the literature the relative risk is reported to be between 6 and 320 times higher, with approximately 1 out of 350 patients suffering from both diseases contemporaneously. In the series reported earlier, 2 cases of carcinoma were diagnosed in 38 Crohn's patients studied over a period of 12 years out of a total population of 114000 inhabitants, with a frequency of carcinoma of 5.13% compared to 0.3% reported in the literature. The incidence of Crohn's disease was 2.84/100000 per year.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Ileal adenocarcinoma in Crohn's disease. A report of 2 cases]. 129 79

YK-176 is a newly isolated 2'-deoxycoformycin (DCF), a potent inhibitor of adenosine deaminase, produced by Aspergillus nidulans. In a cooperative phase I study, YK-176 was administered to 22 patients, comprising 18 with adult T-cell leukemia-lymphoma (ATL), two with cutaneous T-cell lymphoma (CTCL), one with lymphoblastic lymphoma of T-cell type and one with carcinoma of the uterine cervix. Doses of YK-176 ranged from 3.0 to 9.0 mg/m2 and were given intravenously for three consecutive days. General malaise, anorexia, nausea, vomiting and low grade fever were frequently encountered, but were transient and not dose-related. At all dose levels hematological toxicities were mild. Two of seven patients receiving 7.0 mg/m2 for three consecutive days developed hepatocellular enzyme elevations (grade 2) and one patient, proteinuria (grade 2). One of two patients given 9.0 mg/m2 for three consecutive days manifested a life-threatening (grade 4) disturbance of consciousness and dyspnea, presumably ascribable to the drug-related toxicity of YK-176. The results suggest that 7.0 mg/m2 i.v. for three consecutive days is the maximum acceptable dose of YK-176. Central nervous system, pulmonary and possibly renal toxicities appeared to be dose-limiting. Out of the 20 patients evaluable for therapeutic response, partial remissions were observed in four, three with ATL and one with CTCL, who received less than 7.0 mg/m2 for three consecutive days. We conclude that YK-176 is an active agent against ATL at doses that may not be associated with prohibitive toxicity. A starting dose of 5.0 mg/m2 for three consecutive days is recommended for further phase II studies on ATL.
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PMID:Phase I study of YK-176 (2'-deoxycoformycin) in patients with adult T-cell leukemia-lymphoma. The DCF Study Group. 151 64

Diaziquone (AZQ), a synthetic quinone with demonstrated activity against acute nonlymphocytic leukemia (ANLL), primary CNS tumors, and non-Hodgkin's lymphoma (NHL), is virtually devoid of nonhematopoietic toxicity at conventional doses. As a prelude to its inclusion into bone marrow transplant (BMT) preparative regimens, a phase I study of high-dose AZQ with autologous BMT (ABMT) was performed. Patients with refractory solid tumors and lymphomas were treated with a single 24-hour infusion of AZQ at 50 to 355 mg/m2 in dose escalations of 20%. Fifty-six patients received 69 courses. Those receiving greater than 60 mg/m2 had nadir granulocyte and platelet counts less than 500/microL and 20,000/microL, respectively. Nausea, vomiting, stomatitis, and diarrhea were mild, transient, and not dose-related. Transient minimal elevations of liver function tests were seen in five patients and were also not dose-related. The maximally tolerated dose (MTD) of high-dose AZQ was found to be 245 mg/m2, with nephrotoxicity being dose-limiting. Significant azotemia was seen in four of 12 patients treated at 295 and 355 mg/m2, including fatal anuric renal failure in three of these patients. Reversible proteinuria also occurred in 24 of 26 courses above 150 mg/m2, including nephrotic range proteinuria in eight courses, all at doses of 205 to 355 mg/m2. The proteinuria was also associated with multiple proximal tubular defects including generalized aminoaciduria and proximal renal tubular acidosis. There were six early deaths including two of early renal failure (295 and 355 mg/m2), two of sepsis (205 and 245 mg/m2), one of a pulmonary embolus (85 mg/m2), and one of progressive disease (60 mg/m2). Of 50 patients who were assessable for response, there were seven responses including two of 10 with primary CNS tumors, one of 12 with malignant melanoma, one of five with non-small-cell lung carcinoma, two of two with breast carcinoma, and one of one with ovarian carcinoma. Because of its activity in ANLL and NHL and its unique toxicity spectrum, high-dose AZQ may improve the efficacy of current BMT preparative regimens without significantly increasing their nonhematopoietic toxicity.
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PMID:A phase I trial of high-dose diaziquone and autologous bone marrow transplantation: an Illinois Cancer Council study. 207 48

Evaluation of proteinuria in a 66-year-old white female detected the presence of two distinct anomalous proteins: a gamma heavy chain devoid of light chains and an intact IgM-lambda paraprotein. The gamma heavy chain protein was incomplete, antigenically corresponding to the Fc portion of the molecule. Subsequent diagnostic studies of the patient revealed the simultaneous presence of an adenocarcinoma of the colon and an extranodal well-differentiated lymphocytic lymphoma involving the lungs, pulmonary hilum, and upper gastrointestinal tract. Levels of the two paraproteins were unaltered by surgical excision of the colonic carcinoma but were reduced by chemotherapy directed at the lymphoma.
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PMID:Lymphocytic lymphoma with associated gamma heavy chain and IgM-lambda paraproteins. An unusual biclonal gammopathy. 210 38

A 57 year old man was referred to us with multiple long-standing, recurring and refractory mouth ulcers involving the palate, the intermaxillary folds, the inner side of the cheeks, the frenum of the tongue, a tonsil, the pharynx, the gums and a lower lip. Multiple biopsies had excluded a lymphoma, a carcinoma and infection. A new biopsy showed a polymorphous granuloma with up to 40% of atypical lymphoid cells. No angiitis was present, but an angiocentric pattern was present in the granuloma. The atypical cells stained positively with OKT3 and OKT4 antisera. The patient also had cardiac rhythm disturbances with defects on the myocardial scan and a proteinuria. Thus, we suspected the diagnosis of lymphomatoid granulomatosis, although there were no pulmonary nodules. Cyclophosphamide and prednisone were given with immediate success. As the doses were gradually decreased, a small relapse occurred. It was controlled by increasing the cyclophosphamide dosage for six months. The patient is now in complete remission more than four years after having discontinued the treatment. Thus, lymphomatoid granulomatosis can be diagnosed on the basis of a biopsy of buccal ulcers and in the absence of the classical pulmonary nodules. We found no evidence that the atypical cells were lymphomatous; rather, the very good response to the treatment points to a curable granulomatosis.
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PMID:[Buccal lymphomatoid granulomatosis]. 232 57

Two patients, one with myeloma (Patient 1) and the other with probable chronic lymphocytic leukemia (Patient 2), had reduced renal tubular phosphate reabsorption in the absence of hyperparathyroidism together with other features of the Fanconi syndrome, as consequences of the nephropathy associated with light-chain proteinuria. Both patients had hypophosphatemic osteomalacia, demonstrated for the first time in this condition by iliac bone histomorphometry after in vivo double tetracycline labeling, despite absence of bone pain or Looser zones. Neither patient was vitamin D-depleted, but plasma calcitriol level was normal in Patient 1 and low in Patient 2; only the latter patient had severe muscle weakness. Complete histologic correction of osteomalacia was achieved by treatment in accordance with the biochemical defects--oral phosphate therapy alone in Patient 1 and combined with calcitriol in Patient 2. Both patients are now symptom-free, five and three years after the initial diagnosis of bone disease and hematogenous malignancy. Thirteen previous instances of the same form of osteomalacia were reviewed; in most cases, the Fanconi syndrome developed before its probable cause became apparent. The Fanconi syndrome has also been reported in two cases of osteomalacia due to mesenchymal tumor, but not in osteomalacia associated with prostatic carcinoma. Light-chain nephropathy and consequent renal tubular dysfunction appears to be a third form of oncogenous osteomalacia.
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PMID:Hypophosphatemic osteomalacia and adult Fanconi syndrome due to light-chain nephropathy. Another form of oncogenous osteomalacia. 310 97

To elucidate the natural course of the nephropathy associated with penicillamine and thereby facilitate its clinical management 33 patients with rheumatoid arthritis who developed proteinuria during treatment with oral penicillamine were studied in detail throughout their renal illness. Renal biopsies were performed, and creatinine clearance and proteinuria were measured serially for 74 months (range 16-148 months). Fourteen patients developed proteinuria within six months after the start of treatment and 27 within 12 months. When treatment was stopped the proteinuria reached a median peak of 4.2 g/24 h (range 0.3-15.0 g/24 h) at one month (range 0-7 months) before resolving spontaneously by six months (12 patients), 12 months (21), or 18 months (29). In all patients but one, who developed carcinoma of the renal pelvis, proteinuria resolved by 21 months and its median duration was eight months. The median first and last measurements of creatinine clearance showed no appreciable change (80 ml/min and 78 ml/min), and no patient died from or needed treatment for renal failure. The HLA-B8 or HLA-DR3 alloantigen, or both, were identified in 10 patients. Renal biopsy specimens showed membranous glomerulonephritis in 29 patients, minimal change nephropathy in two, and electron dense deposits in the mesangial regions in two. In all the patients whose nephropathy was due solely to treatment with penicillamine the proteinuria resolved completely when the drug was withdrawn; renal function did not deteriorate, and corticosteroids were unnecessary.
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PMID:Natural course of penicillamine nephropathy: a long term study of 33 patients. 313 18


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