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)

Glomerulonephritis (GN) is a group of conditions characterised by inflammation in the filtering units of the kidney which may be 'primary'; secondary to drugs, infections or tumours; or the presenting feature of systemic disease. GN is treatable, causes significant morbidity and mortality, and is a potentially preventable cause of renal failure and cardiovascular risk. It can only be precisely identified and characterised by renal biopsy which is usually undertaken in specialist nephrology centres. The role of the non-specialist is to know when and how urgently a patient should be referred to such a centre. This review aims to provide guidance on when to suspect GN, how to investigate this possibility and when to refer for further investigation. Clinically urgent situations are highlighted. The importance of urinary abnormalities, particularly proteinuria (even if aysmptomatic and only detected on routine screening) is emphasised. Earlier recognition of GN will improve patient outcomes.
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PMID:Glomerulonephritis: is it worth worrying about? 1601 Dec 19

Anderson-Fabry disease (AFD) is a rare X-linked lipid storage disorder due to a deficient lysosomal a-galactosidase A (a-Gal) activity. In males with the classic form of the disease the enzymatic defect leads to progressive accumulation of glycosphingolipids (GL) in different organs, mainly in the kidney, heart, and brain, causing severe multisystem failure. AFD is usually mild in heterozygous females, but severe cerebrovascular, renal, and cardiac manifestations have been rarely described. The aim of this study is to describe renal involvement of mild symptomatic female carriers by ultrastructural analysis focusing to microvascular lesions, considered to be one of the major causes of systemic disease in AFD. Resin-embedded renal biopsies from 2 sisters with isolated mild proteinuria and belonging to a family group with AFD were observed by light and electron microscopy. In spite of the mild clinical symptoms, diffuse GL storages were demonstrated in all types of glomerular cells and in interstitial endothelial cells. Moreover, platelets were frequently observed in glomerular vassels, a feature coherent with a possible role of prothrombotic state, and platelet activation, in early glomerular lesions.
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PMID:Ultrastructural study of renal involvement in two females with Anderson-Fabry disease. 1603 75

Ischemic nephropathy is recognized as a distinct cause of renal insufficiency and it is defined as a significant reduction in glomerular filtration rate in patients with hemodynamically significant renovascular occlusive disease. We argue the epidemiologic and clinical manifestations of atherosclerotic renovascular disease, and we evaluate the pronostic agents. Published studies of the outcome of revascularization for renal-artery stenosis have been excellent, offering a durable patency and functional improvement but they have had numerous limitations. The atherosclerosis is a systemic disease and it provides the general prognosis of patients. We conclude that ischemic renal disease is a nephropathy of smoker men, with proteinuria excretion similar to nephropathy with unilateral stenosis. The age of patients is the clinical feature that decide the treatment: surgery, angioplasty/stent or medical management. Comparative analysis of percutaneous transluminal angioplasty and operation for renal revascularization and medically treated patients have proved that the advanced chronic renal insufficiency is associated with an unfavourable response of treatment of the ischemic nephropathy. But, in this nephropathy the revascularization can be the better therapy for selected patients. The revascularization with angioplasty/stent for patients with unilateral renal stenosis and chronic renal insufficiency has a doubtful effectiveness, as the chronic renal failure is result of nephroangiosclerosis.
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PMID:[Ischemic renal disease: revascularization or conservative treatment?]. 1605 7

Collagenofibrotic glomerulopathy is an idiopathic glomerular disease characterized by massive accumulation of atypical type III collagen fibrils within the mesangial matrix and subendothelial space and marked increase in serum type III procollagen peptide levels. The disease is extremely rare, with most cases reported in Japan. The cause and pathogenesis are entirely elusive. Some cases were described in families; hence, a genetic mode of transmission, mostly by an autosomal recessive trait, has been assumed. Controversy exists about whether the glomerulopathy is a primary renal disease or manifestation of systemic disease. Proteinuria is a cardinal manifestation of this disease. Clinically, patients present with edema and hypertension and often progress to end-stage renal disease. A definite diagnosis can be established when typical histological findings are supported by immunohistochemistry for specific collagen types and electron microscopy with special staining methods. No specific treatment is available.
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PMID:Collagenofibrotic glomerulopathy: clinicopathologic overview of a rare glomerular disease. 1738 17

Chronic kidney disease complicates an increasing number of pregnancies, and at least 4% of childbearing-aged women are afflicted by this condition. Although diabetic nephropathy is the most common type of chronic kidney disease found in pregnant women, a variety of other primary and systemic kidney diseases also commonly occur. In the setting of mild maternal primary chronic kidney disease (serum creatinine <1.3 mg/dL) without poorly controlled hypertension, most pregnancies result in live births and maternal kidney function is unaffected. In cases of more moderate and severe maternal primary chronic kidney disease, the incidence of fetal prematurity, low birth weight, and death increase substantially, and the risk of accelerated irreversible decline in maternal kidney function, proteinuria, and hypertensive complications rise dramatically. In addition to kidney function, maternal hypertension and proteinuria portend negative outcomes and are important factors to consider when risk stratifying for fetal and maternal complications. In the setting of diabetic nephropathy and lupus nephropathy, other systemic disease features such as disease activity, the presence of antiphospholipid antibodies, and glycemic control play important roles in determining pregnancy outcomes. Concomitant with advances in obstetrical management and kidney disease treatments, it appears that the historically dismal maternal and fetal outcomes have greatly improved.
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PMID:Chronic kidney disease and pregnancy: maternal and fetal outcomes. 1739 16

Pregnancy has generally been regarded as very high risk in women with chronic renal insufficiency. In this review, we describe the physiologic changes in systemic and renal haemodynamics during pregnancy, as well as the nature and severity of possible maternal and foetal complications in the setting of underlying renal disease. The risks are proportional to the degree of functional renal impairment, the presence or not of proteinuria and/or arterial hypertension at the time of conception, and are related to the type of underlying nephropathy or systemic disease in the mother. Furthermore, if the renal disease has been diagnosed before pregnancy, a better planning of the moment of conception, as well as a tight follow-up, allow for a better maternal and obstetrical outcome.
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PMID:[Pregnancy in patients with underlying renal disease]. 1743 97

Sarcoidosis is a systemic disease characterized by chronic granulomatous inflammation. Chronic kidney disease (CKD) was reported in less than 1% of patients of sarcoidosis. The prevalence of tubulo-interstitial nephritis (TIN) in sarcoidosis varies from 7 to 27%. In this retrospective study, we present 15 patients with interstitial or glomerular renal involvement secondary to sarcoidosis diagnosed in our center from 1975 to 2006. Patients were 13 (96.6%) females and two males with a mean age of 56.5 years. CKD was present in 14(93.3%) patients, proteinuria in 13(96.6%), and nephrotic syndrome in one. Pulmonary involvement was present in 10 (66.6%) patients. Renal biopsy performed in 12 (80%) patients revealed TIN lesions in 10 (66.6%) patients, extracapillary proliferative glomerulonephritis (GN) in one, and membranous GN type 2 in another. Corticosteroid therapy using prednisolone 0.5 to 1 mg/kg per day was used in 12(80%) patients. Ten (66.6%) patients were followed up for a mean period of 25 months (ranged from 2 to 48 months). The outcome was favorable with 7 (46.6%) patients improved their renal function, 6 (40%) remained with a moderate CKD, one normalized his renal function, and one died suddenly after 2 months of initiating the treatment corticosteroids. We conclude that corticosteroid treatment is efficient in TIN and variably efficient in GN. Patients with sarcoidosis may cause advanced renal failure, which renders it a serious nephrological condition.
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PMID:Interstitial and glomerular renal involvement in sarcoidosis. 1808 26

Microscopic polyangiitis (MPA) is a rare systemic disease that usually presents as a pulmonary-renal syndrome. We describe 35-year-old men who presented with hemoptysis and bilateral alveolar opacities of the upper part of both lungs. The CT scan showed alveolar and round-glass opacities with a "mosaic-like" pattern. Bronchoalveolar lavage confirmed pulmonary hemorrhage. Renal biopsy was indicated because proteinuria revealed extracapillary glomerulonephritis. Laboratory tests showed a high level of serum antimyeloperoxidase-antineutrophil cytoplasmic antibody. We made a diagnosis of MPA. Cyclophosphamide and corticosteroid therapy was instituted and remission achieved. Through this case report, we discuss the diversity of the radio-clinical features of MPA.
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PMID:[A case of recurrent bilateral pneumonia with fever. Diagnosis: microscopic polyangiitis]. 1816 45

This report describes a woman with progressive renal failure without proteinuria, urinary obstruction or overt systemic disease. The progressive renal disease without pelvicalyceal deformities in the left kidney was not consistent with the vesicoureteric reflux nephropathy. A needle biopsy of the left kidney showed interstitial caseating granulomata. The patient did not have clinical, radiological or urine culture evidence of renal tuberculosis. She improved after treatment with antituberculous therapy. This case report demonstrates the value of kidney biopsy in establishing the diagnosis of such common and treatable disease even if clinically silent and urine culture negative.
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PMID:A Case of Progressive Tubulo-Interstitial Nephritis due to Culture-negative Renal Tuberculosis. 1820 99

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited systemic disease characterized by a prolonged subclinical course of gradual renal cyst expansion, resulting in massively enlarged kidneys and renal failure by the fifth to sixth decade. Renal cyst expansion results in intrarenal ischemia and activation of the renin-angiotensin-aldosterone system (RAAS) and relates to the development and maintenance of hypertension in ADPKD. Hypertension relates to disease progression in ADPKD with regard to renal volume, proteinuria, cardiovascular complications, and progression to end-stage renal disease. Novel magnetic resonance imaging methods developed in the Consortium for Radiologic Imaging for the Study of Polycystic Kidney Disease (CRISP) provide accurate estimates of change in renal volume over a short period of time in ADPKD patients with intact renal function. In CRISP an increase in renal volume of 63.4 ml/yr was found. PKD1 status, male gender, hypertension, reduced renal blood flow, and proteinuria are associated with increased renal volume and change in renal volume over time. HALT-Polycystic Kidney Disease (HALT-PKD) is designed to test whether blockade of RAAS and/or rigorous blood pressure control play a role in slowing renal progression during early (using magnetic resonance imaging methods developed in CRISP) and during late (using measures, including composite of time to doubling of serum creatinine, onset of end-stage renal disease, or death) phases in ADPKD. Findings from CRISP and the rationale for interventions in ADPKD are described, and the design of the HALT-PKD clinical trial is outlined.
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PMID:Approaches to testing new treatments in autosomal dominant polycystic kidney disease: insights from the CRISP and HALT-PKD studies. 1857 74


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