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

Primary systemic amyloidosis (AL), a disease involving the deposition of immunoglobulin light chains in tissue, is caused by a plasma cell dyscrasia. In the case of amyloidosis reported here, no monoclonal component was seen upon routine protein electrophoresis of serum or urine nor was a bone marrow analysis positive for AL. Immunofixation electrophoresis did not show a typical paraprotein band but did show, in the gamma region, two large diffuse bands and a lower concentration of oligoclonal-type bands, all of which stained for free lambda but not for free kappa chain. The ratio of kappa to lambda chains in urine was 0.178, much less than the ratio in serum (1.3). Six other urine samples from a group of patients with documented Bence Jones proteinuria also exhibited kappa/lambda ratios that differed manyfold from the ratios in their corresponding serum samples. On the other hand, the kappa/lambda ratios from seven controls (seven patients with generalized proteinuria unrelated to plasma cell dyscrasia) were similar in serum and urine. This difference between the kappa/lambda ratios from serum and urine can be expressed as a kappa/lambda index. The index was significantly different (P less than 0.01) between the two patient groups compared here, and was useful in confirming the presence of Bence Jones protein in this case with a difficult-to-interpret electrophoretic pattern. Although the kappa/lambda ratio has been widely used for confirmation and identification of monoclonal components in serum, its use in clinical laboratories has not been widely extended to urine. Comparison of serum and urine kappa/lambda ratios as a kappa/lambda index may help reduce the need for more complex immunoelectrophoresis techniques in identifying free light chains in urine.
 ...
PMID:kappa/lambda index for confirming urinary free light chain in amyloidosis AL and other plasma cell dyscrasias. 190 99

The detection of Bence Jones protein, an important part of the investigation of suspected myeloma, is most commonly done by agarose or cellulose nitrate electrophoresis followed by immunofixation. Bence Jones protein is recognized as single or multiple bands of one type of light chain. Unfortunately, improvements in sensitivity of these techniques (use of high-affinity antisera and higher resolution electrophoresis) frequently allow detection of multiple light chain bands in the urine of patients who do not have a B-cell dyscrasia. The bands are usually kappa, although they may be accompanied by lambda bands. This pattern may lead to the misdiagnosis of Bence Jones protein and oligoclonal light chain production in patients. Here we show that this pattern is produced by polyclonal light chains; it is present in the urine of all patients with a tubular proteinuria of any etiology and may be induced in healthy individuals by blocking their renal tubular protein reabsorption. Polyclonal light chains separate into monomers and dimers on sodium dodecyl sulfate-polyacrylamide gel electrophoresis and into four major bands with many minor bands by isoelectric focusing. This difference in charge and possibly size results in the banding pattern seen on good-quality electrophoresis and immunofixation.
 ...
PMID:Restricted electrophoretic heterogeneity of immunoglobulin light chains in urine: a cause for confusion with Bence Jones protein. 190 42

A 19-year-old female patient with renovascular hypertension accompanied by focal segmental glomerulosclerosis like lesion in the contralateral kidney is described. She was admitted to our hospital for uncontrollable hypertension which was first pointed out at check-up when she was 18 years old. On admission physical examination revealed remarkable hypertension and a bruit around the umbilical region. Laboratory findings disclosed renal dysfunction and slight proteinuria (0.8-1.1 g/day). Arteriography revealed severe narrowing of the right renal artery suggesting fibromuscular dysplasia. Taken together, renovascular hypertension was diagnosed. She underwent an operation to reconstruct the artery with autotransplantation of the ipsilateral kidney in the pelvic cavity, and sections were taken from both kidney for histological evaluation during the procedure. After the operation her blood pressure normalized and proteinuria decreased. And interestingly, the kidney specimens revealed that the left side consisted of focal segmental glomerulosclerosis like lesion whereas the right was only of ischemic change. Hyperfiltration theory has recently been highlighted experimentally. And the etiology of the renal findings in this case may be based on such hemodynamic alteration as has been described in animal models. We present the case so that it may be of some help to understand how focal segmental glomerulosclerosis is brought about in humans.
 ...
PMID:[A case of renovascular hypertension accompanied by focal segmental glomerulosclerosis like lesion in the contralateral kidney]. 205 44

Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease. The nonamyloid forms were found in 13 patients who had evidence of plasmacytic dyscrasia by the immunohistochemical detection of immunoglobulin light chains of kappa or lambda class (with or without staining for a single heavy chain isotype) and by the absence of amyloid P component in tissue sections that did not show the birefringence characteristic of amyloid after Congo Red staining. All but two of the patients presented with proteinuria with or without azotemia. Clinical syndromes involving other organ systems were less common but occasionally severe. Four patients had overt multiple myeloma. Three others had hypercalcemia and mild bone marrow plasmacytosis but no lytic lesions. Analyses of immunoglobulin synthesis in bone marrow cells from seven patients showed excess light chains in all and incomplete light chains or heavy chain fragments in six, regardless of whether an intact monoclonal protein or related subunit was in the serum or urine. The fibrillar (amyloidotic) and nonfibrillar forms of monoclonal immunoglobulin deposition occur either in overt multiple myeloma or in the course of less neoplastically aggressive plasmacytic dyscrasias. Bone marrow cells from patients with either type produce immunoglobulin fragments that are related to those deposited in the affected tissues.
 ...
PMID:Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis. 210 17

The authors deal with the clinicopathology of the renal, alterations in light-chain disease in connection with 6 cases. The disease was recognized by the monotype (in 5 cases kappa, in 1 case lambda) immunoreactivity of the light-chain paraprotein deposited in the basal membranes of the renal tissue. Electron microscopic examinations proved the fine-granulated, electrodense character of the paraprotein. Multiple myeloma was found in 3 cases and plasma cell dyscrasia of non-tumorous characteristic in 3 cases in the background of the deposition. The renal involvement appeared clinically in the picture of proteinuria without nephrosis syndrome and in progressing azotemia. Chronic renal insufficiency developed during some months in 5 patients. Morphologically renal impairment manifested in interstitial fibrosis, tubular atrophy and ateriolar hyalinosis was seen. These were associated with different glomerular alterations, for instance in 3 cases with nodular glomerulosclerosis. In 1 patient with plasma cell dyscrasia of non-tumorous characteristic nodular glomerulosclerosis and semilunar formation was observed in 56% of the glomeruli. In an other patient with myeloma the simultaneous existence of cylinder nephropathy and light-chain nephropathy was demonstrated. Both observations are unusual phenomena in plasma cell dyscrasia.
 ...
PMID:[Light-chain nephropathy]. 211 26

Bence Jones proteins (monoclonal free light chains of immunoglobulins) are the earliest known biological markers of malignant cell dyscrasia; Bence Jones proteinuria is also present in many types of B cell-related neoplasms. Sometimes, it may also occur in Hodgkin's disease. In some cases, benign monoclonal gammapathy was found to be associated nontumorous diseases as well. The type of monoclonal light chain, the degree of polymerization, and the isoelectric point of the molecule may affect the course of the disease. Urine samples from 637 patients with true or suspected lymphoproliferative diseases were investigated over a 2-yr period by different immunochemical methods. Bence Jones proteinuria was identified in 71 cases by isoelectric focusing combined with immunofixation, while the pathological protein was detected only in 63 cases by conventional methods. Bence Jones proteins can be detected by this new method at a level below the sensitivity of conventional procedures. Bence Jones proteins in the urine may signal a malignant tumor or malignant transformation of an earlier disease. The early detection of monoclonal immunoglobulin light chains in the urine may be important in clinical diagnosis, therapy, and follow-up.
 ...
PMID:Monoclonal free light chains in urine and their significance in clinical diagnostics: are they really tumor markers? 228 64

Bence Jones proteins (monoclonal free light-chains of immunoglobulins) are the earliest known biologic markers of malignant plasma cell dyscrasia, but Bence Jones proteinuria is also found in many types of B-cell related neoplasms. In some cases it may occur in Hodgkin disease. In some cases benign monoclonal gammopathy related to non-tumor conditions was found. The type of the monoclonal light-chain, the polymerisation and the isoelectric point of the molecules are considerable factors in the development of the disease. Urine specimens from 637 patients with (or suspected) lymphoproliferative diseases were investigated in a two year period using different immunochemical methods. Bence Jones proteinuria was found in 71 cases with the new method (isoelectric focusing combined with immunofixation) and 63 cases by the conventional ones. Bence Jones proteins can be detected by this new method at level below the sensitivity of conventional procedures. Bence Jones proteins in the urine may call the attention to the malignant tumor or the malignant transformation of the disease. The early detection of the monoclonal light-chain excretion may be important in the respect of clinical diagnosis, therapy and the follow-up the clinical status of the patient.
 ...
PMID:[The significance of analysis and detection of Bence Jones protein in the urine in clinical laboratory diagnosis]. 250 96

There are indications that there is an increased risk of chronic renal failure (CRF) in the Negroid race, yet few studies have been carried out in the native 'black' environment. A clinico-pathological study of 100 consecutive Nigerian subjects with CRF, seen over a 3-year period, is presented. Primary chronic glomerulonephritis (CGN) accounted for 50, accelerated hypertension for 25, and various aetiological entities for a further nine; these included, chronic pyelonephritis (two), diabetic nephropathy (two), calculous nephropathy (one), toxaemia of pregnancy (one), renal dysplasia (one), tuberculosis (one) and polycystic disease in the ninth subject. In 16 cases, no definitive aetiological diagnosis could be made. Combinations of the following features, protracted hypertension, proteinuria, significant analgesic intake and gouty arthritis, were observed. CGN and accelerated hypertension still remain the leading causes of CRF, while diseases such as diabetes mellitus and chronic pyelonephritis do not contribute significantly to CRF in Nigerians. Recognition of the early features and the causes of CRF would considerably reduce the prevalence of this condition.
 ...
PMID:Diseases causing chronic renal failure in Nigerians--a prospective study of 100 cases. 254 87

Acrorenal syndrome is characterized by central longitudinal axis defects of the limbs, ie, split hand and/or foot. Associated renal lesions described so far comprise agenesis, bilateral hypoplasia (originally diagnosed as oligomeganephronia), and duplication abnormalities. The case of a 29-year-old patient with split hand resulting from bilateral aplasia of the third phalanges associated with dysplasia of the third and fourth metacarpals is reported. In addition, the following lesions were noted: hypoplasia of the middle phalanx of the fifth toe, arched palate, pectus excavatum, hypoplastic mammilae, scoliosis, and congenital hip dislocation. The patient presented with hypertension, modest reduction of glomerular filtration, proteinuria, microhematuria, cylindruria, and moderate harmonic hypoplasia of the right kidney on angiography. Glomeruli had no immune deposits on immunohistology. Light microscopy showed widening of the mesangial axis, focal segmental glomerular sclerosis, and renal interstitial fibrosis with occasional foam cells. This case shows that the spectrum of renal abnormalities in the acrorenal syndrome is wider than previously noted.
 ...
PMID:Acrorenal syndrome in an adult--presentation with proteinuria, hypertension, and glomerular lesions. 277 25

A patient with serum monoclonal gammopathy, Bence-Jones proteinuria, and bone marrow plasmacytosis underwent fiberoptic bronchoscopic study for evaluation of interstitial lung disease. Bronchoalveolar lavage fluid contained 47 percent plasma cells, which were monoclonal by immunoperoxidase staining. This is the first time BAL plasmacytosis has been demonstrated in a patient with a plasma cell dyscrasia.
 ...
PMID:Bronchoalveolar lavage plasmacytosis in a patient with a plasma cell dyscrasia. 290 39


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>