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)

Sickle cell anemia and the related hemoglobinopathies are associated with a large spectrum of renal abnormalities. The patients have impaired urinary concentrating ability, defects in urinary acidification and potassium excretion, and supranormal proximal tubular function. The latter is manifest by increased secretion of creatinine and by reabsorption of phosphorus and beta(2)-microglobulin. Young patients with sickle cell disease (SCD) have supranormal renal hemodynamics with elevations in both effective renal plasma flow (ERPF) and glomerular filtration rate (GFR). These parameters decrease with age as well as following the administration of prostaglandin inhibitors. Proteinuria, a common finding in adults with sickle cell disease, may progress to the nephrotic syndrome. Proteinuria, hypertension, and increasing anemia predict end-stage renal disease (ESRD). While ESRD can be managed by dialysis and/or renal transplantation, there may be an increased rate of complications in renal transplant recipients with SCD. Hematuria is seen in individuals with all of the SCDs as well as with sickle cell trait. In most cases the etiology of the hematuria turns out to be benign. However, there does appear to be an increased association between SCD and renal medullary carcinoma. Therefore, those SCD patients who present with hematuria should initially undergo a thorough evaluation in order to exclude this aggressive neoplasm. Papillary necrosis may occur due to medullary ischemia and infarction. Erythropoietin levels are usually lower than expected for their degree of anemia and decrease further as renal function deteriorates. An abnormal balance of renal prostaglandins may be responsible for some of the changes in sickle cell nephropathy. Acute renal failure is a component of the acute multiorgan failure syndrome (MOFS). Finally, progression of sickle cell nephropathy to ESRD may be slowed by adequate control of hypertension and proteinuria. However, the prevention of the renal complications of SCD will require a cure for this genetic disorder.
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PMID:Renal abnormalities in sickle cell disease. 1142 1

Four patients who had ingested large amounts of phenacetin-salicylate medications were studied during a 12-month period. Renal failure had progressed slowly over a number of years. All patients took the drug because of psychogenic headache. Considerable skill was required to elicit the history of drug habituation. The major features of the nephropathy were multiple episodes of metabolic acidosis, minimal proteinuria, pyuria but no bacteriuria, and polyuria and polydipsia early in the course of drug ingestion. Papillary necrosis was not a prominent clinical feature of this series. Discontinuation of drug ingestion by one patient was associated with recovery of a considerable degree of renal function. Preliminary experimental evidence obtained in the dog suggests that salicylate impaired the efficiency of the counter-current multiplier by decreasing sodium transport in the ascending limb of Henle, and decreased the permeability to water of the distal convoluted and collecting tubule; phenacetin had no such effect.
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PMID:PHENACETIN NEPHROPATHY. 1422 36

A 20-year-old African American male presented with a history of left flank pain and passing of light pink urine. Medical history included sickle cell trait. Urine analysis was positive for protein and blood. Metabolic profile, autoantibody screen, and complement levels were normal. Hemoglobin electrophoresis revealed an 41.8% HbS diagnostic of sickle cell trait. Creatinine clearance was normal and proteinuria was nonnephrotic. A noncontrast computed tomography (CT) scan showed left proximal hydronephrosis. Urology follow-up was arranged and the differential included renal papillary necrosis, or renal cyst rupture. He presented 3 months later with sudden onset left flank pain and gross hematuria. Serum creatinine was 1.8 mg/dL. Computed tomography scan with contrast revealed innumerable lung lesions, an enlarged heterogenously enhancing left kidney, and retroperitoneal adenopathy. Ultrasound revealed an obstructed left collecting system and a 14-cm enlarged left kidney with no discrete mass. Testicular markers/ultrasound, upper/lower endoscopies were normal. Lung biopsy revealed poorly differentiated adenocarcinoma positive for cytokeratin 7. Renal, sarcoma, and gastrointestinal markers were negative. By exclusion, it appeared that the patient had a carcinoma of unknown primary. However, with the clinical and personal history, a diagnosis of renal medullary carcinoma (RMC) was made. RMC is a rare and highly malignant tumor that should always be included in the differential of a patient with sickle cell disorder and hematuria. Renal biopsy typically fails to sample the renal medulla and radiologic findings might not raise the suspicion of a renal tumor. Thus, clinical suspicion must always be high in order to preserve the patient's only chance of prolonged survival.
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PMID:Adenocarcinoma of unknown primary in a 20-year-old African American male. 1969 25

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management.
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PMID:[Spectrum of renal manifestations in sickle cell disease]. 2411 2

Sickle cell disease (SCD) is a major health problem in many countries. Sickle cell nephropathy (SCN) is now a well-characterized entity with specific manifestations, risk factors and prognosis. The presence of sickled erythrocytes in the renal medullary vessels is the hallmark of the disease with a variety of renal complications. Renal manifestations of SCD include renal ischemia, microinfarcts, renal papillary necrosis and renal tubular abnormalities with variable clinical presentations. Proximal tubule dysfunction generally impairs urinary concentration, while more distal tubule dysfunction may impair potassium excretion, leading to hyperkalemia. Glomerular disease with proteinuria may develop due to ischemia and results in a compensatory increase in the renal blood flow and glomerular filtration rate; such hyperfiltration, combined with glomerular hypertrophy, probably contributes to glomerulosclerosis. Acute and chronic kidney disease are the expected outcomes of the disease. Both dialysis and kidney transplantation are effective renal replacement therapies for end-stage renal disease due to SCN, with a higher advantage for transplantation. Whether bone marrow transplantation in the early stage of the disease can halt the progression of SCN is unknown and awaits clinical studies.
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PMID:An update on sickle cell nephropathy. 2462 90


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