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 51-yr-old man presented with renal failure, proteinuria, hematuria, and hypertension. The serum contained two monoclonal protein spikes, an IgG4 lambda and free lambda light chains. Free gamma heavy chains were absent from serum. A bone marrow biopsy did not show evidence of a plasmalymphocytic dyscrasia. Renal biopsy showed a nodular glomerulopathy with linear deposits of gamma 4 heavy chains alone along glomerular, tubular, and vascular basement membranes and in mesangial regions. No light chains were detected in the kidney despite staining with antisera directed against both free and bound light chains and the F(ab')2 fragment of IgG. The term heavy-chain deposition disease is appropriate in view of the above features.
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PMID:IgG heavy-chain deposition disease. 783 42

A 73-year-old male was admitted to the renal division of our hospital because of hypertension, proteinuria and bilateral pretibial edema. Eight years previously, he was diagnosed as being afflicted with interstitial pneumonia on the basis of a chest X-ray examination. Laboratory tests conducted during the current admission showed normocytic normochromic anemia, renal dysfunction and mild proteinuria. Total IgG was normal, but a high proportion of IgG2 was observed. M-protein in the serum was positive for both IgG lambda and Bence Jones protein (lambda type). A bone marrow biopsy showed the proportion of plasma cells to be 10.6%, but atypical cells were not found. We diagnosed the patient's condition as plasma cell dyscrasia. Light microscopy examination of a renal biopsy specimen showed moderate mesangial proliferation with a deposition of PAS-positive and Congo red negative materials in the mesangial area: nodular gomerulonephritis was seen in some glomeruli. Immunofluorescence revealed IgG and lambda light chains, strong linear staining along the glomerular basement membrane and tubular basement membrane and positivity in the mesangial area. Results of staining for IgA, IgM, fibrinogen and C3 were weakly positive in the mesangium area, while those for C4, Clq and free kappa were negative. Positive staining of IgG2 was seen by immunoperoxidase study, but the tissue was negative for IgG1, IgG3, IgG4. Electron microscopy demonstrated a dense granular deposition in the mesangial, subendothelial and peritubular area and a microfibrillar structure in the mesangial area. The diameter of the microfibrillar structure was 14 nm on the average.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of light and heavy chain deposition disease (IgG2 lambda)]. 796 78

We report a 63 years old female patient presenting with progressive edema, alopecia and pallor. Laboratory showed a proteinuria of 1.9 g/24 h, microhematuria, a serum creatinine of 3.1 mg/dl, erythrocyte sedimentation rate of 133 mm/h and antinuclear antibodies of 1/40 with a homogeneous pattern. No extrarenal disease was demonstrated and a kidney biopsy, performed 18 months later, showed a fibrillary glomerulonephritis, nodular sclerosis variety, with 20 nm phi fibrillae, immune and kappa and lambda light chain deposits and negative Congo red stain. The patient died 30 months later due to a respiratory infection. Fibrillary glomerulonephritis is an infrequent form of mesangiocapillary glomerulonephritis not associated to plasma cell dyscrasia. It leads to terminal renal failure and it recurs in transplanted kidneys.
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PMID:[Fibrillary glomerulonephritis of the nodular glomerulosclerosis variety]. 808 85

We report on a 16-year-old girl with spondyloepiphyseal dysplasia, nephrotic syndrome, lymphopenia, and signs of defective cellular immunity. The manifestations are very similar to those reported by Spranger et al. [1991: J. Pediatr 119: 64-72] as Schimke immunoosseous dysplasia, except for age of onset. In Schimke immunoosseous dysplasia, growth retardations as an initial symptom is noted in early childhood and about 1 year after onset of progressive proteinuria. In our case the skeletal abnormality was noted at age 10 years as dislocation of the hip joints and the diagnosis of nephrotic syndrome was made at age 16 years. The findings strongly suggest that our patient has a juvenile variant of Schimke immunoosseous dysplasia.
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PMID:Juvenile variant of Schimke immunoosseous dysplasia. 820 83

Renal dysplasia and agenesis may be a familial disorder. We report the familial occurrence of unilateral renal agenesis and proteinuria that, at least in one case, was related to focal glomerulosclerosis. Whether these abnormalities are related to an intrinsic abnormality in the remaining kidney, hyperfiltration injury, systemic hypertension, or some other poorly defined factor is unclear at present. However, this report, along with previous case reports of familial renal agenesis, suggests that ultrasonographic screening of first-degree relatives of patients with renal agenesis is appropriate. Whether treatments such as dietary protein restriction, use of angiotensin-converting enzyme inhibitors, or other therapeutic interventions will have a beneficial effect in asymptomatic individuals with unilateral renal agenesis remains to be determined.
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PMID:Familial unilateral renal agenesis and focal and segmental glomerulosclerosis. 850 22

The morbidity and lethality of AL amyloidosis is caused by the deposition of lg light chains as fibrillar amyloid protein in vital organs, disrupting their function, and not by the generally low burden of clonal plasma cells that produce the paraproteins. Survival of patients with AL amyloidosis is no more than 1 to 2 years from the time of diagnosis with current management approaches. Clearly, more effective therapies are needed for this rapidly lethal disease. Five patients were treated with dose-intensive melphalan and blood stem cell support and followed for a period of 1 year. Patients were diagnosed with AL amyloidosis by tissue biopsy and categorized by performance status and organ involvement. Their plasma cell dyscrasias were evaluated with immunofixation electrophoresis of serum and urine specimens, quantitative serum lgs, and immunohistochemical staining of bone marrow biopsy specimens. After treatment with dose-intensive intravenous melphalan followed by infusion of autologous growth-factor-mobilized blood stem cells, clinical evaluations and plasma cell studies were repeated at 3 and 12 months. Three men and 2 women aged 38 to 53 years were treated. Median performance status (SWOG) was 2 (1 to 3), and clinical presentations included nephrotic syndrome (n = 1), symptomatic cardiomyopathy (n = 1), gastrointestinal involvement with polyneuropathy (n = 2), and hepatomegaly (n = 1). With a median follow-up of 13 months (12 to 17 months), all five patients are well and have shown stable or improved performance status and clinical remission of organ-related dysfunction, including a 50% reduction in daily proteinuria with no change in creatinine, reversal of symptoms of cardiomyopathy and reductions of posterior wall and septal thickening, reversal of polyneuropathy and gastric atony, and resolution of hepatomegaly by computed tomographic scan. In 3 of the 5 patients (60%) at 12 months after treatment, plasma cell dyscrasias could not be detected. Dose-intensive chemotherapy with intravenous melphalan and growth-factor-mobilized blood stem cell support is feasible therapy for patients with AL amyloidosis, even when there is clinical evidence of cardiac involvement. At least some patients with AL amyloidosis achieve complete remission of their plasma cell dyscrasia, improvement in performance status, and clinical remission of organ-specific disease after this form of treatment.
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PMID:Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: one-year follow-up in five patients. 883 79

A six-month-old male Golden Retriever with a three-month history of polyuria and polydipsia was examined. Hematological examinations revealed nonregenerative anemia, azotemia, high serum creatinine level, hypercalcemia, hyperphosphatemia, hypercholesterolemia, hyperamylasemia, and low level of total serum protein. Urinalysis indicated mild proteinuria, and low specific gravity. Radiographic and ultrasonographic examinations revealed bilateral small sized kidneys. Histological examination by renal biopsy confirmed the diagnosis of renal dysplasia. Treatment with a dietary protein restriction, oral adsorbents, and dried aluminum hydroxide gel have been performed in this dog, and then, azotemia, high serum creatinine level, hypercalcemia, and hyperphosphatemia were improved. During 10 months after the initiation of treatments, no significant clinical change except polydipsia and polyuria has been observed.
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PMID:A control of a golden retriever with renal dysplasia. 936 46

AL amyloidosis is an infiltrative disorder characterized by the extracellular deposition of insoluble fibrillar immunoglobulin light chains whose production results from a plasma cell dyscrasia. Treatment with melphalan has resulted in an improvement in a few patients. Recently, intensive chemotherapy followed by autologous or syngeneic stem cell support has been shown to offer potential benefit. Allogeneic stem cell support after intensive therapy would retain the benefits of autologous transplantation, with the additional advantages of a tumor-free graft and of a possible graft-versus-tumor effect. We report a patient with AL amyloidosis and significant proteinuria. She improved after an allogeneic bone marrow transplantation.
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PMID:Allogeneic bone marrow transplantation for AL amyloidosis. 940 36

We report the case of a middle-aged woman presenting epigastric discomfort, hepatomegaly, biochemical signs of cholestasis, bone marrow plasmocytosis and Bence Jones proteinuria. Percutaneous liver biopsy disclosed kappa light chain deposition disease of the liver and fine needle aspiration of abdominal fat showed amyloid substance. Renal blood chemistries and urinalysis were repeatedly normal. To our knowledge, this is the first reported case of kappa light chain deposition disease of the liver and concomitant amyloidosis without renal involvement as the first manifestation of plasma cell dyscrasia. This condition should be considered in the differential diagnosis of intrahepatic cholestatic liver disease.
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PMID:Kappa light chain deposition disease of the liver. 961 91

In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal lambda light chains were identified in serum and urine. A low percentage of monoclonal plasma cells was detected in the bone marrow. The patient received four cycles of VAD and subsequent high-dose chemotherapy (HDCT) with melphalan (200 mg/m2) followed by autologous peripheral blood stem cell transplantation. Proteinuria rapidly diminished during chemotherapy. Three months after HDCT, the patient has no edema, and no signs of plasma cell dyscrasia are currently detectable. Using VAD before starting HDCT may improve the condition of patients with amyloidosis and reduce transplantation-related morbidity and mortality.
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PMID:Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support. 1033 56


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