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)

Seventy-five patients with congenital nephrotic syndrome of Finnish type were identified in Finland in the period 1965-1973, giving an incidence of 12-2/10(5). A large placenta and proteinuria from birth are the hallmarks of the disease. About one-quarter of the patients had oedema and/or abdominal distension at birth and in all cases the full nephrotic syndrome was documented before 2 months. More than half of the patients died before 6 months and none lived longer than 2 years 3 months. A slight rise in blood urea nitrogen or serum creatinine levels occurred in 14 cases, but in none of these did a frank uraemia develop before death. Infection appeared to be the immediate cause of death in 31% of the cases; in 43% no cause of death other than congenital nephrotic syndrome could be shown. Thrombi in large vessels were found in 11 out of 58 necropsies.
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PMID:Congenital nephrotic syndrome of Finnish type. Study of 75 patients. 93 78

Many patients with idiopathic membranous nephropathy are elderly, but little is known about the natural or treated history of these patients. We have studied a cohort of 155 patients with membranous nephropathy who were recruited and followed-up over a 20 year period. We have compared the clinical features and outcome of the older (>60 years) and younger age groups. There was a higher incidence of an identifiable cause for the nephropathy in older patients. At presentation with idiopathic disease, older patients were more often hypertensive and had worse renal impairment than the younger cohort, but had a similar levels of proteinuria, hypoalbuminemia and hematuria. Thrombotic complications and minor rheumatological complaints were more common in the older patients. Prognosis for life and renal survival was worse in the older onset patients. Treatment was well tolerated in selected older patients and was associated with a better outcome in those selected for treatment.
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PMID:Characteristics and outcome of membranous nephropathy in older patients. 1209 Mar 24

Intravenous injection of Type 1 streptococcal M protein into mice and rats produced lesions confined to renal glomeruli. Thrombi of eosinophilic amorphous material, seen to occlude glomerular capillaries, were shown to contain M protein and fibrinogen. Gradual regression of the morphological lesions was observed during the 3 weeks following injection. Initial abnormal proteinuria and azotemia returned to control levels by the end of the 1st week; a second rise in urinary protein excretion and urea retention was demonstrated in some rats coincident with appearance of anti-M antibodies. The mechanism of renal localization of streptococcal M protein by means of a complex with fibrinogen was suggested, which may comprise an initial phase in the pathogenesis of acute poststreptococcal glomerulonephritis.
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PMID:FIBROGEN PRECIPITATION BY STREPTOCOCCAL M PROTEIN. II. RENAL LESIONS INDUCED BY INTRAVENOUS INJECTION OF M PROTEIN INTO MICE AND RATS. 1427 35

A 15-yr-old girl with end-stage kidney disease caused by primary focal segmental glomerulosclerosis (FSGS) underwent a living-related donor kidney transplantation. The allograft functioned well immediately after reperfusion, but massive proteinuria exceeding 50 g/d appeared on day 3. Treatment with rituximab and plasma exchange (PE) successfully decreased the proteinuria to 10 g/d. A biopsy specimen on day 30 showed no segmental glomerulosclerosis but partial interstitial infiltration of inflammatory cells. An increased number of podocytes showed intracytoplasmic vacuolization, and an electron micrograph showed diffuse mild subendothelial edema and foot process effacement. The podocytes were hypertrophied but were not detached from the basement membrane. As the therapies used to reduce the patient's proteinuria were having a limited effect, intravenous steroid pulse therapy followed by low-density lipoprotein apheresis was performed. A biopsy specimen taken on day 120 showed no segmental glomerulosclerosis. Thrombus formation in one glomerulus and packed lymphocytes in the capillary loop of another glomerulus were detected. The patient's clinical course was compatible with FSGS recurrence. Although the early pathological changes were not typical of FSGS, they might be indicative of the primary lesion that subsequently progresses to typical FSGS.
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PMID:A case of recurrent focal segmental glomerulosclerosis (FSGS) involving massive proteinuria (>50 g/day) immediately after renal transplantation. 2162 16

Nephrotic syndrome (NS) is characterized by heavy proteinuria, edema, hypoalbuminemia, and hyperlipidemia and the most frequent causes are glomerular diseases. An uncommon presentation is iatrogenic NS, an adverse effect of some drugs administration. In the clinical course of NS, a typical feature is dysregulated coagulation state, promoted by the breakdown of permselectivity barrier of the glomerular capillary wall, resulting in the leakage of high-molecular-mass proteins, at least the size of albumin. This hypercoagulable condition is supported by several factors, such as abnormalities in platelet activation and an imbalance between anticoagulation/antithrombosis and procoagulant/prothrombotic mechanisms. Thus, NS and the risk of developing thromboses are strictly related. Thrombotic events affect the venous system rather than arterial vessels with different features and frequencies. Deep venous system of the lower extremities and renal veins are the most frequent source of pulmonary embolism, the most dangerous NS complication. Prophylactic anticoagulation and thrombosis treatment are not clearly established because large randomized trials and guidelines are lacking. The management of NS and the decision of when and how to anticoagulate the patient represent a teamwork challenge for physicians.
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PMID:Thrombosis in nephrotic syndrome. 2362 54

We describe a case of nephrotic syndrome that manifested as a retropharyngeal pseudoabscess. The patient was a 32-year-old man who presented with a short history of throat discomfort and a choking sensation. Laryngoscopy identified bulging of the posterior pharyngeal wall that partially occluded the laryngeal inlet. A lateral neck x-ray revealed that the prevertebral space was widened, and computed tomography detected fluid in the retropharyngeal and parapharyngeal spaces. Neck exploration revealed that the edema had been caused by nonsuppurative fluid. Biochemical analyses revealed marked hypoalbuminemia and heavy proteinuria suggestive of nephrotic syndrome. Following surgery, the patient's symptoms resolved. Aseptic effusion into the retropharyngeal space is rare; reported etiologies include internal jugular vein thrombosis, neoplasia, radiation therapy, trauma, acute calcific tendinitis, hereditary angioedema, and myxedema of hypothyroidism. To the best of our knowledge, this is the first case of nephrotic syndrome initially manifesting as retropharyngeal pseudoabscess to be reported in the literature. Thrombotic occlusion of the pharyngeal venous plexus secondary to hypercoagulability is a plausible explanation for such isolated retropharyngeal effusion.
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PMID:Retropharyngeal pseudoabscess manifesting in nephrotic syndrome. 2481 38

Neonatal renal vein thrombosis (RVT) is associated with potentially serious morbidities. Almost 80% of cases of RVT present within the first postnatal month. The most common risk factors for RVT are birth asphyxia/ in utero fetal distress, being the infant of a diabetic mother, volume contraction and coagulation abnormalities. Thrombus formation may be initiated by vascular injury, diminished vascular flow, increased blood viscosity, hyperosmolality or underlying thrombophilia. The classic triad of RVT includes gross hematuria, flank mass (unilateral or bilateral enlargement of kidneys) and thrombocytopenia. Laboratory tests may reveal hematuria, proteinuria, polycythemia, hemolytic anemia, thrombocytopenia and possibly acute kidney injury. The etiology for a hypercoagulable state should be investigated. Renal ultrasound with Doppler may show increased size of the affected kidney, increased echogenicity and loss of corticomedullary differentiation. Renal venography remains the gold standard for the diagnosis of RVT. Other causes of renal enlargement must be considered. Supportive treatment includes correction of fluid and electrolyte disturbances and treatment of infection and underlying pathophysiologic abnormalities. Use of unfractionated heparin (UFH) or low molecular weight heparin (LMWH) should be considered if there is evidence of disseminated intravascular coagulation. Conventional anticoagulants may attenuate hypercoagulability and decrease the risk for thrombus progression and embolism. Surgery is rarely indicated unless there is bilateral involvement with involvement of the IVC. RVT carries the risk of hypertension and chronic kidney disease.
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PMID:Renal venous thrombosis in neonates. 2508 63

Thrombotic disease, a major life-threatening complication of nephrotic syndrome, has been associated with proteinuria and hypoalbuminemia severity. However, it is not fully understood how disease severity correlates with severity of the acquired hypercoagulopathy of nephrotic syndrome. Without this knowledge, the utility of proteinuria and/or hypoalbuminemia as biomarkers of thrombotic risk remains limited. Here, we show that two well established ex vivo hypercoagulopathy assays, thrombin generation and rotational thromboelastometry, are highly correlated with proteinuria and hypoalbuminemia in the puromycin aminonucleoside and adriamycin rat models of nephrotic syndrome. Notably, in the puromycin aminonucleoside model, hyperfibrinogenemia and antithrombin deficiency were also correlated with proteinuria severity, consistent with reports in human nephrotic syndrome. Importantly, although coagulation was not spontaneously activated in vivo with increasing proteinuria, vascular injury induced a more robust thrombotic response in nephrotic animals. In conclusion, hypercoagulopathy is highly correlated with nephrotic disease severity, but overt thrombosis may require an initiating insult, such as vascular injury. Our results suggest that proteinuria and/or hypoalbuminemia could be developed as clinically meaningful surrogate biomarkers of hypercoagulopathy to identify patients with nephrotic syndrome at highest risk for thrombotic disease and potentially target them for anticoagulant pharmacoprophylaxis.
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PMID:Disease Severity Correlates with Thrombotic Capacity in Experimental Nephrotic Syndrome. 2585 74

Introduction Pulmonary embolism is a potentially life-threatening complication of nephrotic syndrome. Syncope is rarely reported as an initial presentation of pulmonary embolism in nephrotic patients. Case presentation We describe a 35-year-old man who was taking steroids and diuretics for relapse of minimal change disease who presented after a syncopal event. The patient was hypotensive and had distended neck veins. The major laboratory findings were hypoalbuminemia with mild proteinuria. The findings on electrocardiography, chest radiography, and echocardiography and the elevated plasma D-dimer level raised suspicion of pulmonary embolism. Thrombi in the bilateral main pulmonary arteries on chest computed tomography together with compromised hemodynamics were consistent with the diagnosis of massive pulmonary embolism. He received anticoagulant treatment and the disease resolved. Conclusion Pulmonary embolism should be considered as a cause of syncope in patients with nephrotic syndrome, despite the absence of severe hypoalbuminemia and proteinuria, especially in patients taking concurrent steroid and diuretic therapy.
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PMID:Syncope in a patient with minimal change disease without nephrotic-range proteinuria. 2867 34

Thrombotic microangiopathies (TMAs) are associated with microangiopathic hemolytic anemia and thrombocytopenia, resulting in microvascular thrombosis and end-organ damage. In pregnancy, this may be the result of pregnancy-related TMAs such as preeclampsia; hemolysis, elevated liver enzymes, and low platelets; or pregnancy-associated TMAs, specifically thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (CM HUS). TTP and CM HUS are rare disorders, and their diagnosis may be missed, no less because features at presentation may be misdiagnosed as a pregnancy-related TMA, such as hypertension, proteinuria, fetal growth restriction, or in utero fetal death. The mainstay of treatment for pregnancy-associated TMAs is plasma exchange. Presentation is likely in the third trimester for TTP and postpartum for CM HUS. However, both conditions can present in any trimester, unlike pregnancy-related TMAs which rarely present before the second trimester, commonly in the third trimester. Delivery is the mainstay of treatment for pregnancy-related TMAs. More recently, it has become clear that pregnancy may be a trigger for late-onset congenital TTP, as well as immune-mediated TTP, diagnosed by ADAMTS13 analysis. Complement inhibitor therapy is the treatment of choice for CM HUS cases. However, their diagnosis is by exclusion, but complement inhibitor therapy reduces the risk of end-stage renal failure. Subsequent pregnancies can be supported for TTP and CM HUS.
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PMID:Microangiopathic Hemolytic Anemia in Pregnancy. 3017 29


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