Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A soluble glomerular basement membrane (GBM) antigen was detected in the urines of patients with various glomerular diseases including chronic glomerulonephritis, nephrotic syndrome, chronic renal insufficiency, and lupus nephropathy. The urinary GBM antigen (u-GBM) was immunochemically distinct from other renal antigens and other serum components, but it was cross-reactive with trypsinized human GBM antigen (t-GBM). The molecular size of u-GBM was approximately the same as human serum albumin as estimated by elution patterns on Sephadex G-200. The concentration of u-GBM was estimated quantitatively by a single radial radioimmunodiffusion. Although differed from case to case, a rough correlation with the type and/or stage of nephrotic syndrom existed. It was also demonstrated that the amounts of u-GBM decreased in response to steroid therapy of nephrotic syndrome. It was further shown that in a case of membranoproliferative glomerulonephritis, anti-GBM antibody could be eluted from the kidney removed from the patient. These findings imply that the GBM antigen plays an important role in the pathogenesis of human renal diseases. The pathophysiological significance of urinary GBM excretion in renal diseases is also discussed here.
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PMID:Immunochemical characterization and quantitation of the human glomerular basement membrane antigen from the urine of patients with glomerular diseases. 40 15

In five patients suffering from recurrent thrombosis and/or fetal death, a lupus anticoagulant was associated with a renal vasculopathy. Ischaemic episodes also involved the skin, heart, eyes and/or central nervous system. All patients were hypertensive. Two had renal insufficiency, two had non-nephrotic proteinuria, and in the last patient renal cortical ischaemia was detected by a tomographic scan in the absence of proteinuria. Renal biopsy showed thrombosis and/or intimal fibrosis of intrarenal vessels, and normal or ischaemic glomeruli without proliferative lesions. High-titres of anticardiolipin antibodies were found in 3 of 3 cases, and persisted after steroid therapy even if the circulating anticoagulant factor disappeared. All patients received corticosteroid therapy, alone or in combination with immunosuppressive drugs; two patients had prolonged oral anticoagulation, but thrombotic episodes recurred after stopping the drug. One patient died; the remaining four survived 18 months to 11 years after diagnosis, with stable chronic renal insufficiency in one of them. These results show that a lupus anticoagulant may be associated with prominent renal vascular disease, in the absence of proliferative glomerular lesions, and suggest that continuous anticoagulation may be beneficial in these patients.
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PMID:Recurrent thrombosis and renal vascular disease in patients with a lupus anticoagulant. 251 88

The aim of this work was to determine the activity of helix-destabilizing protein (HD-protein), which is indispensable in basic genetic processes such as DNA replication, genetic recombination and transcription, in lymphocytes of patients with different morphological types of glomerulonephritis and with chronic renal insufficiency, treated conservatively and by maintenance hemodialysis. The estimation was done on 44 patients with glomerulonephritis and on 20 patients with chronic renal insufficiency. The control group consisted of 20 healthy individuals. The investigations prove the fact that in human lymphocytes helix-destabilizing protein occurs, and its quantity enables us to determine its activity in the diseased and healthy state. Measurement of HD-protein activity seems to be a good marker of the altered lymphocyte function. Especially high HD-protein activity was noticed in cases of acute glomerulonephritis, lupus glomerulonephritis and in the active forms of chronic glomerulopathies. Generally, the tested parameter showed a positive correlation with the activity of the glomerulonephritic process. In lymphocytes of patients with chronic renal insufficiency treated conservatively, a greater activity of helix-destabilizing protein occurred than in the control group. Lymphocytes from patients with chronic renal insufficiency, treated with repetitive maintenance hemodialysis, did not reveal significant differences in HD-protein activity, determined directly before hemodialysis and after its completion, in comparison to the control group of healthy individuals.
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PMID:Activity of helix: destabilizing protein in lymphocytes of patients with glomerulopathies and with chronic renal insufficiency. 298 9

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.
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PMID:Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature. 360 12

Over a period of nine years we observed 52 children with acute neurological symptoms which were caused by a cerebrovascular disease. Fourteen patients had congenital vascular malformations, most frequently AV-angiomas (9 patients). A Sturge-Weber-Syndrome and a venous angioma were found in two cases and one patient had an aneurysm of the middle cerebral artery. Thirty-eight patients had acquired cerebrovascular diseases such as ischaemic infarctions (22), intracranial haemorrhages without vascular malformations (14) and thromboses of the dural sinus (2). The cerebral infraction was a complication of a congenital heart disease in 8 children, two others suffered from chronic renal insufficiency and were on haemodialysis. Two children had a trauma of the internal carotid artery and in one patient a large haemorrhagic infarct was caused by hypernatremic dehydration. In 9 patients (6 females, 3 males) no obvious aetiology of the infarct could be found. However, in most of these cases a nonspecific febrile illness preceded the neurological manifestations. The thrombosis of the dural sinus occurred in a 6-week old previously healthy infant and in a 3-year old boy as a complication of a nephrotic syndrome. Intracranial haemorrhages (without cerebrovascular malformations) occurred in 14 patients, mainly as a complication of haematological diseases (acute lymphatic leukaemia, severe aplastic anaemia, haemophilia A, lupus erythematodes). Four children had spontaneous intracerebral haemorrhages without obvious causes. The prognosis for survival was good in children with infarcts, but persisting neurological deficits were more severe than in children with haemorrhages. At the acute stage the lethality was higher in children with intracranial haemorrhages.
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PMID:[Cerebrovascular diseases in childhood--etiology, clinical aspects and prognosis]. 395 16

The renal effects of aspirin and acetaminophen are minor. With a major overdose of acetaminophen, uncommonly renal failure may occur that cannot be ascribed to hepatic failure; its mechanism is unknown. Aspirin may cause a transient shedding of renal tubular cells, alterations in urate excretion, inhibition of spironolactone action, and, in certain clinical settings, a reversible decline in renal function manifested as a fall in glomerular filtration that may be accompanied by mild water, sodium, and potassium retention. Active systemic lupus erythematosus, advanced cirrhosis, and chronic renal insufficiency seem to predispose patients to the effects on renal function, and there is direct or indirect evidence in those conditions that prostaglandin synthesis is an important part of the body's attempt to preserve renal blood flow. Study of these effects has provided new insight into the way in which the kidneys may use prostaglandins to preserve renal function when it is threatened.
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PMID:Acute effects of aspirin and acetaminophen on renal function. 700 35

The results from the long-term combined anticoagulant, immunesuppressive and corticosteroid treatment of 36 patients with various histological forms of idiopathic and lupus glomerulonephritis are reported. Twenty five of them are with preserved or slightly disturbed renal function, and 11 - with chronic renal insufficiency. The treatment was carried out with heparin, acenocoumarol, azathioprin and to the patients with lupus glomerulonephritis - corticosteroids in the course of 4 to 50 months. The results were "very good", "good", with "no change" and "deterioration" according to clinical and laboratory indices. Very good and good effect was obtained in 18 patients (72%) of the group with preserved or slightly disturbed renal function (25 patients). Five patients were with no change (20%) and 2 patients died of complications in connection with corticosteroid treatment. Very good results was obtained in 2 patients with rapid progressive glomerulonephritis from the group with chronic renal insufficiency (11 patients) and the rest were without effect. The adverse effects are not frequent and are mainly due to corticosteroids. The combined anticoagulant and immunosuppressive treatment is concluded to be indicated in patients, with idiopathic and lupus nephritis with preserved or slightly disturbed renal function, in patients with rapidly-progressive glomerulonephritis but not in case of manifested chronic renal insufficiency. The considerable duration and the systematic follow-up control are of essential importance for the treatment success.
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PMID:[Current trends in the treatment of chronic glomerulonephritis]. 732 54

The antiphospholipid antibodies (AAFL) are acquired antibodies of different types whose common characteristic is that they are directed against the negatively and neutrally charged phospholipids of the cell membranes. They were first described in 1952 in patients with systemic lupus erythematosus. They are also found in many pathological conditions such as: infections, solid and haematological neoplasms, chronic renal insufficiency, primary immunological deficiencies and other collagenoses, hepatobiliary diseases, etc. They are also found in other circumstances: due to the use of various drugs (which in general may induce lupus), in elderly patients and in apparently healthy people. The great interest shown in the past decade, as a result of their association with and possible etiopathogenic role in a series of thrombotic phenomena, neuropsychiatric disorders, thrombocytopenia and abortion-fetal death, has made them a major challenge to the clinician. This interest is increased by the controversial and very doubtful relationship that may exist with a series of disorders which have been described in detail. Their role as a probable risk factor for thrombosis in the general population is also discussed. For these reasons, we decided to review the basic biology of the AAFL and the aspects related to neurological disorders.
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PMID:[Antiphospholipid antibodies and neurological disorders. Types and mechanisms]. 898 18

We describe a 12-year-old girl with systemic lupus erythematosus (SLE) who first presented with an atypical hemolytic uremic syndrome (HUS) associated with hypocomplementemia, and compare the clinical manifestations and prognosis between SLE patients with HUS and thrombotic thrombocytopenic purpura in the reported literature. Diagnoses were based on renal failure, hemolytic anemia, and thrombocytonemia, including the observation of fragmented red blood cells, hypocomplementemia and on the American College of Rheumatology criteria for SLE. Cocktail therapy may have been effective against the pathological condition of SLE. In 4 patients with SLE and HUS, prednisolone and immunosuppressive drugs were administered, and none of the patients suffered from chronic renal insufficiency. The prognosis for SLE patients with HUS is good. These findings suggest that SLE should be suspected in any HUS patient presenting with hemolytic anemia, thrombocytopenia, acute renal failure and hypocomplementemia, and the therapeutic response and prognosis for SLE with HUS are good.
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PMID:A 12-year-old girl with hemolytic uremic syndrome as initial symptom of systemic lupus erythematosus and a literature review. 1238 63

The study population comprised all 20 patients followed since 1990 through December 2004 at the Le Bonheur Children's Medical Center with diagnosis of C1q nephropathy (55% boys; 60% African Americans). All were aged under 18 years at biopsy (mean 11.2 years, 65% aged 11 or over); the youngest presented at age 10 months and progressed to end-stage renal disease at 14 months. None had clinical or laboratory features of systemic lupus erythematosis or membranoproliferative glomerulonephritis. Clinical features assessed at diagnosis were age, gender, blood pressure, history of macroscopic hematuria, urinary protein to creatinine ratio, serum creatinine, estimated glomerular filtration rate, renal histology, and pattern for immunofluorescent reactants. At the time of biopsy 40% had nephrotic syndrome and 30% nephrotic range proteinuria without nephrotic syndrome. Three patients with nephrotic syndrome also had chronic renal insufficiency at diagnosis. The most common histological feature was focal segmental glomerulosclerosis in 40%, but 30% had minimal change lesion. Four patients, all with nephrotic syndrome at diagnosis, progressed to end-stage renal disease. Of the 12 patients not presenting with nephrotic syndrome, none had chronic renal insufficiency at last follow-up. Kidney survival was 94% and 78% at 1 and 5 years, respectively, in all patients and 88% and 49% in those presenting with nephrotic syndrome.
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PMID:C1q nephropathy: features at presentation and outcome. 1613 42


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