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)

The syndrome of hereditary thrombocytopenia, deafness, and renal disease was manifest in at least eight members in three generations of a family. They had a lifelong history of bleeding, usually as epistaxis, bilateral sensorineural deafness starting in late childhood or the teenage years, and persistent proteinuria with varying degrees of renal dysfunction. Two members died at a young age, one from central nervous system hemorrhage, the other from chronic renal failure. Splenectomy and steroid therapy have been of transient benefit. There was dominant inheritance of the syndrome. Hematologic studies showed thrombocytopenia, large platelets, and megakaryocytic hyperplasia of the bone marrow. In contrast to a previous report, our studies showed that affected members had normal in-vitro platelet function and normal ultrastructural platelet morphology. At autopsy, histologic changes in the kidney of one affected family member were indistinguishable from those reported in classic hereditary nephritis with nerve deafness (Alport's syndrome).
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PMID:Hereditary thrombocytopenia, deafness, and renal disease. 113 59

In a 53-year-old woman an acute allergic thrombocytopenia (acute Werlhf's disease) occurred after treatment with D-penicillamine for one month which led to death. In two further patients transitory platelet deficiencies were observed after six and two months which regressed completely in six months and four weeks, respectively. One of the patients had nephrotic syndrome and a retrobulbar neuritis at the same time. Both symptoms were equally transitory and could not be demonstrated five weeks after cessation of therapy. In a further case a lethal Lyell syndrome developed three weeks after therapy was started. These observations show that during D-penicillamine treatment weekly, and later fornightly, blood counts should be performed. In the occurence of thrombocytopenia, leucopenia or anaemia treatments should be stopped; Signs of drug intolerance together with exanthemata should also led to a critical review of the indications and the dosage. D-Penicillamine should not be used when hypersensitivity to penicillin exists or when cell deficiencies have occurred after anti-reheumatic medication. The development of proteinuria should also result in withdrawal of the drug. Therapy with D-penicillamine requires conscientious follow-up urinalyses and blood counts as well as attention to allergic rashes.
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PMID:[Severe side-effects of treatment with D-penicillamine (author's transl)]. 117 64

The objective of this study was to characterize the hemostatic defect in dogs with infectious canine hepatitis (ICH), a naturally occurring viral disease of dogs. Five littermate dogs were inoculated with 10(3) TCID50 of ICH virus intravenously. Two littermates were controls. The clinicopathologic manifestations of ICH were fever, depression, anorexia, hematemesis, melena, widespread mucocutaneous petechiae, prolonged bleeding from venipunctures, faceial edema, leukopenia, and proteinuria. The hemostatic defect of ICH was characterized by thrombocytopenia, abnormal platelet function, prolonged one-stage prothrombin time and activated partial thromboplastin time, normal thrombin times, depressed factor VIII activity, and increased fibrin-fibrinogen degradation products. These findings suggested that the central pathologic mechanism of the abnormal hemostasis in ICH was disseminated intravascular coagulation (DIC). ICH is an example of DIC induced by viral infection. This disease is a suitable model for investigation of the detection, pathogenesis, and therapy of DIC.
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PMID:Infectious canine hepatitis: animal model for viral-induced disseminated intravascular coagulation. 124 23

Hantavirus infection was confirmed by history, symptoms and biochemical changes, as well as immunofluorescence test in 29 patients (24 men, 5 women; mean age 36.9 +/- 11.5 years) with nontraumatic renal failure (ANF), retrospectively in 15 patients. Cardinal symptoms were acute onset (n = 29), fever (n = 27), pain in the flanks, abdomen or head (n = 27), reduced glomerular filtration rate (n = 29), proteinuria (n = 25) and thrombocytopenia (n = 16). Normal renal function was restored in all patients. Follow-up examination of 15 patients 6-7 years after the acute illness revealed normal blood pressure, normal serum creatinine, absent proteinuria and normal inulin clearance in all, thus confirming the favourable prognosis of the infection in Western Europe. Nonetheless, because Hantavirus infection is by no means rare, it should be included in the differential diagnosis of acute renal failure.
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PMID:[Hantavirus infection with acute kidney failure]. 135 86

581 early cases of typical hemorrhagic fever with renal syndrome (HFRS) were dynamically studied on the clinical manifestations and laboratory findings from 1986 to 1989. All the patients were treated with various methods. The results showed: (1) The manifestations of microvascular damage, proteinuria and thrombocytopenia can be found at the first day of the onset in 80.0%, 72.2% and 33.3% respectively. In a word, the characteristic features of HFRS appear at the onset of the disease. (2) In 446 early cases the initial severity of the disease corresponded with the final severity at a rate of 89.2%; it indicated that the damage of HFRS may result from the first attack. (3) The time of the onset, peak and persistence of all the characteristic features were similar; it is suggested that the course of HFRS may be self-limited. (4) Based on the clinical understanding and the effective results in the 446 early cases treated with fluid therapy alone, we consider that the effective treatment of HFRS is early and reasonable fluid therapy.
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PMID:[A study on pathogenesis and therapy of hemorrhagic fever with renal syndrome]. 135 14

The distribution of HLA-D region antigens was studied in three groups (I, IIa, and IIb) of patients with rheumatoid arthritis (RA): group I comprised 43 patients with mild, non-progressive RA, controlled by non-steroidal anti-inflammatory drugs without progression or erosions; group II comprised 94 patients with severe disease, who had earlier been treated with non-steroidal anti-inflammatory drugs and all had incomplete response requiring treatment with gold (sodium aurothiomalate). Of these, 46 patients (group IIa) responded to gold and the disease was well controlled, and the remaining 48 patients (group IIb) did not respond to gold and developed gold induced toxic reactions, including thrombocytopenia or proteinuria, or both. HLA-D region antigens were defined by serological and molecular (Southern blot analysis and oligonucleotide typing) techniques. The results show that DR4 was significantly increased in all three groups of patients. The prevalence of DR1, or DR1 in DR4 negative patients, and DR3 and DR4 associated DQw7 specificities, however, showed differences in these three groups of patients. The prevalence of DR1 and of DR1 in DR4 negative patients was increased only in patients with mild (group I) RA, but not in patients with severe (groups IIa and IIb) disease. On the other hand, the prevalence of DR4 associated DQw7 was significantly increased in patients with severe disease, but not in patients with mild RA. In addition, DR3 was significantly increased only in patients with severe disease who developed gold induced toxic reactions (group IIb). These data suggest that the HLA-D region genes which cause susceptibility to mild RA may be different from those causing susceptibility to severe RA. The results suggest that both DR and DQ (A, B) genes may be important in conferring susceptibility to RA: DR in mild disease and DQ in severe RA.
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PMID:HLA-D region genes and rheumatoid arthritis (RA): importance of DR and DQ genes in conferring susceptibility to RA. 141 14

MIV-7 is a human monoclonal antibody that binds to DNA and carries a pathogenic anti-DNA idiotype 16/6. The antibody was generated by fusing peripheral blood lymphocytes of a healthy donor which were stimulated with an anti-idiotypic antibody to B11 (a human mAb anti-mouse mammary tumor virus-MMTV). The MIV-7, in addition to being an anti-DNA antibody, also binds to MMTV glycoproteins. Following immunization into the footpad of naive BALB/c mice with MIV-7, the mice developed anti-phospholipid syndrome (APLS) and SLE. The APLS was characterized by thrombocytopenia, the presence of anticardiolipin antibodies, lupus anticoagulant (prolonged APTT), high resorption rate of fetuses and lower mean weights of the placentae and fetuses. The SLE was characterized by serological markers (e.g. anti-DNA), laboratory (increased sedimentation rate and proteinuria) and histological findings (deposition of immune complexes in the glomeruli). Active immunization of mice with mouse monoclonal anti-cardiolipin antibodies led to the induction of primary APLS without SLE. The results add to our previous passive transfer model in which mouse monoclonal anti-cardiolipin antibody generated from immunized mice (CAM) was infused into the tail vein and also resulted in induction of pure APLS [11]. Our results demonstrate the ability to induce secondary APLS to SLE following immunization with a pathogenic idiotype of anti-DNA antibodies and to induce primary APLS with anti-cardiolipin mAb. The existence of these experimental models may permit controlled studies of novel therapeutic models.
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PMID:Induction of experimental anti-phospholipid syndrome associated with SLE following immunization with human monoclonal pathogenic anti-DNA idiotype. 141 90

Circulating lupus anticoagulant (LA) is associated with thrombosis in large and small vessels. To determine how often the presence of LA is associated with thrombosis within the renal microcirculation, 33 patients with systemic lupus erythematosus (SLE), renal dysfunction, and LA were identified over a 25-year period (LA group) and 32 patients with renal SLE but with normal gross coagulation screen were matched for age, sex, and biopsy timing (C group). Prevalences of serositis, neuropsychiatric illness, leukopenia, thrombocytopenia, hemolysis, anti-DS-DNA elevation, and complement reduction were similar. Arthritis was less and biologic false-positive (BFP) syphilis serology more common in LA. More LA patients had thrombotic events (LA 39% v C 13%; P = 0.014); bleeding episodes, including postbiopsy, were similar. At biopsy, hypertension (LA 55%, C 41%), serum creatinine (mean +/- SD: LA 186 +/- 168 mumol/L [2.1 +/- 1.9 mg/dL] v C 150 +/- 168 mumol/L [1.7 +/- 1.9 mg/dL]) and proteinuria (LA 2.6 +/- 3.1 g/24 h v C 3.1 +/- 2.7) were similar. Lesions by World Health Organization (WHO) class, activity, and chronicity indices, as well as immunofluorescence (IF) and electron microscopy (EM) findings, were not significantly different. Occlusive glomerular, arteriolar, and arterial fibrin thrombi, along with varying degrees of renal thrombotic microangiopathy, were seen in five of 33 patients with LA, but zero of 32 C patients (P = 0.053); three of these five patients died soon after biopsy. Overall, mortality was not different between LA and C. We conclude that the majority of patients with SLE, renal dysfunction, and LA exhibit renal morphologic findings indistinguishable from patients without LA. However, a significant minority of LA patients have thrombotic microangiopathy in their biopsy, which is accompanied by a worse prognosis.
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PMID:Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study. 144 58

In the F1 hybrid of phenotypically normal NZW (H-2z) and systemic lupus erythematosus (SLE)-prone BXSB mice (H-2b), features of the disease became more severe than those seen in the BXSB mice, regardless of the presence or absence of the Yaa (Y-chromosome-linked autoimmune acceleration) mutant gene. To determine whether the gene(s) linked to the major histocompatibility complex (MHC) of NZW mice is involved in this event, we developed the H-2-congenic NZW.H-2d strain and compared the severity of autoimmune disease between (NZW x BXSB) F1 (H-2z/b) and (NZW.H-2d x BXSB) F1 mice (H-2d/b). The H-2z/b, but not H-2d/b, heterozygous F1 mice of both sexes showed an accelerated, higher incidence of proteinuria and a more severe thrombocytopenia than did the BXSB mice. In NZW x (NZW x BXSB) F1 backcross mice, the H-2z/b heterozygous progeny showed more severe disease than did the H-2z/z homozygotes. Thus, disease-accelerating events in (NZW x BXSB) F1 mice are linked to the H-2z/b heterozygosity. Because H-2d/z heterozygosity plays a crucial role for SLE in (NZB x NZW) F1 mice, in which SLE features differ from those in (NZW x BXSB) F1 mice, the present observations may imply that the different but related MHC heterozygosity acts as a predisposing genetic element in these different SLE syndromes.
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PMID:Heterozygosity of the major histocompatibility complex controls the autoimmune disease in (NZW x BXSB) F1 mice. 145 34

Preeclampsia has traditionally been viewed as one of several forms of hypertension complicating pregnancy. More recently, the multisystem nature of this unique gestational disorder has been emphasized. Pathophysiologic events, including abnormal placentation and heightened vascular reactivity, may occur weeks or months prior to clinical recognition of the disease. Although most frequently presenting as hypertension and proteinuria, hepatic (abdominal pain and elevation of transaminases) and hematologic (intravascular hemolysis and thrombocytopenia) involvement may be important features of the disease. Current theories suggest that multiorgan dysfunction may be caused by widespread vascular endothelial dysfunction, vasospasm, and variable activation of coagulation mechanisms. Pending delivery, which is the only definitive therapy for preeclampsia, maternal complications of intracerebral hemorrhage and eclampsia may be prevented with judicious use of antihypertensive medication (e.g., hydralazine) and magnesium sulfate, respectively. Finally, data from a number of small trials suggest that low-dose aspirin (60-100 mg/d) may reduce the incidence of preeclampsia in patients at high risk without adversely affecting the fetus or newborn; however, it is recommended that aspirin not be used as a routine prophylactic intervention until publication of results of several ongoing large multicenter trials, which will help to more fully clarify the benefits and risks of this approach.
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PMID:The syndrome of preeclampsia. 147 40


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