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)

A 60-year-old man was admitted to our hospital because of fever, hemorrhagic tendency, anemia and neurological abnormality. A blood count revealed that the hemoglobin was 6.8 g/dl, the reticulocyte was 17.3 percent with 2 erythroblasts per 100 white cells, the white cell count was 7,100/microliters and the platelet count was 0.8 x 10(4)/microliters. Peripheral blood smear demonstrated marked fragmentation of red cells. Bone marrow examination disclosed the marked erythroid hyperplasia. Although the bleeding time was prolonged (14 minutes 30 seconds), the other hemostatic data were within normal limits. The serum bilirubin level was 1.57 mg/dl; LDH level, 1,437 U/l; creatinine level, 0.92 mg/dl; BUN level 14.7 mg/dl. Haptoglobin was below 10 mg/dl. Results of immunological tests were all negative except the result of PAIgG (576.6 ng/10(7) cells). The urinalysis showed proteinuria, microhematuria and trace granular and hyaline casts. A diagnosis of thrombotic thrombocytopenic purpura was made. The patient was initially treated with prednisolone (60 mg), aspirin (1,000 mg), dipyridamole (150 mg), gabexate mesilate (1.5 g), sodium oxagrel (80 mg) daily with little response. The thirty days after admission, infusion of gamma globulin (20 g, daily) was given for 3 days. The clinical state and laboratory findings became dramatically improved shortly after the administration of gamma globulin and the laboratory data came to be normalized after 1 month. After ten months of this treatment, the patient is remained asymptomatic and the hematological data are within normal range without using any drug. A trial seems justified to confirm the value of this mode of therapy.
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PMID:[Thrombotic thrombocytopenic pupura (TTP)--remission following treatment with high-dose immunoglobulin]. 177 57

Thrombotic thrombocytopenic purpura (TTP) is a syndrome that occurs mainly in adults with multiorgan microvascular thrombosis consisting of thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal involvement, and fever. The female to male ratio is 3:2, and peak incidence occurs in the 3rd decade of life. Clinical signs are the consequence of hyaline thrombosis and occlusion of capillaries and arterioles. Renal ailment manifests itself in hematuria and proteinuria with azotemia and even overt renal failure. In severe disease, azotemia is typical of hemolytic uremic syndrome (HUS). TTP was first described in 1925 by Moschcowitz. The clinical picture of TTP consists of a prodromal phase, a viruslike disease occurring in up to 40% of patients. 60% have neurologic disturbances, 90% have purpura initially, and fever occurs in all. Anemia is often severe with hemoglobin values of 7-9 gm/dl, renal involvement in 90%, and renal failure in 40-80% of patients. Clinical variants include the acute and fulminant variety mortality, the chronic form, and the relapsing form. Predisposing factors and triggering agents are autosomal recessive inherited traits in acute idiopathic TTP, systemic diseases, tumor antigens, pregnancy and puerperium, viruses (endotoxins for HUS), and possibly oral contraceptives and hypertension. Therapy includes corticosteroids (prednisone 100-400 mg/day); heparin for postpartum HUS; and antiplatelet agents (Dextran 70, aspirin, and dipyridamole in high doses). The infusion of PGI2 is controversial; splenectomy is also questionable; and vincristine, azathioprine, and cyclophosphamide have unproven efficacy. Fresh-frozen plasma exchange is the method of choice as it produces survival in 90%. Others are iv immunoglobulins, vitamin E, and dialysis and renal transplant. Platelet transfusions are contraindicated because of sudden death and decreased survival.
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PMID:Thrombotic thrombocytopenic purpura and related disorders. 210 74

A 28-year-old female, who suffered from thrombotic thrombocytopenic purpura (TTP) in the 14th week of her first pregnancy, recovered after a plasma exchange followed by an induced abortion. From six months after the abortion, she no longer required plasma infusions every 3-4 weeks to prevent a relapse of TTP manifested as thrombocytopenia, and complete remission continued until her next pregnancy. In her second pregnancy, she had an immediate relapse of TTP and responded to plasma infusion until the 24th week. However, the TTP gradually became resistant to plasma infusion, and developed into toxemia with edema, hypertension and proteinuria in the 27th week. Although the TTP was alleviated by the infusion of large amounts of plasma, the placenta failed as the result of numerous white infarcts. She delivered a 948 g live baby by cesarean section in the 33rd week. The baby had transient thrombocytopenia but did not suffer from TTP. The mother required plasma infusions every 3-4 weeks for about five months, and she has continued in remission.
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PMID:Successful delivery in a female with thrombotic thrombocytopenic purpura. 369 21

A retrospective review of the records of 15 patients with thrombotic thrombocytopenic purpura (TTP) was performed to determine the spectrum of renal involvement that occurs in this disease. All cases exhibited some evidence of renal involvement, the most common manifestation of which was an abnormal urinalysis. Twelve cases (80%) had some degree of elevation of the serum urea nitrogen (SUN) or creatinine level at some time during the course of their disease. Renal involvement could be categorized into three types depending on the severity of TTP. In those cases presenting as an acute devastating illness, renal insufficiency, when present, was severe and a dominant component of the disease. In those that pursued a more protracted course, with subsequent acute exacerbations of TTP, renal insufficiency was variable and less severe than in the first group. In those presenting with a mild form of TTP, renal involvement consisted primarily of an abnormal urine sediment and azotemia that corrected rapidly following fluid replacement. A review of the literature beginning with 1966, when renal disease was established as part of the features that characterize TTP, provided 216 cases in whom sufficient data on renal involvement were recorded. Of the 168 cases where urinalysis was reported, hematuria was noted in 78% of the cases, proteinuria in 75, pyuria in 31, and cylindriuria in 24%. Of the 181 cases where the admission SUN was reported, it was higher than 20 mg/dl in 69% and greater than 60 mg/dl in 17% of the cases. The level of SUN was a significant determinant of the final prognosis of these patients.
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PMID:Renal involvement in patients with thrombotic thrombocytopenic purpura. 370 16

Thirty-six patients received allogeneic (34) or syngeneic (two) bone marrow transplants as treatment for severe aplastic anaemia or acute leukaemia. Nineteen of the allogeneic recipients received methotrexate (MTX) and 15 received cyclosporin A (CyA) as the predominant immunosuppressive agent to minimize graft-versus-host disease (GVHD) post transplant. In the first 100 d post transplant renal dysfunction was much less frequent in the MTX recipients than in the CyA recipients who exhibited three distinct syndromes of nephrotoxicity: most commonly. CyA recipients developed asymptomatic azotaemia, proteinuria, urinary casts, impaired urinary concentrating ability and hypertension. Secondly, two CyA recipients developed acute reversible renal failure precipitated by systemic bacterial infection which required dialysis and in which the kidney was the sole target organ; thirdly, two recipients of HLA-genotypically non-identical grafts developed a rapidly progressive fatal syndrome with multiple organ involvement including lung, brain and kidney which clinically and histologically resembled thrombotic thrombocytopenic purpura.
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PMID:Cyclosporin A associated nephrotoxicity in the first 100 days after allogeneic bone marrow transplantation: three distinct syndromes. 634 55

Thrombotic thrombocytopenic purpura (TTP) is usually accompanied by renal disfunction presumable due to diffuse thrombotic occlusions in the microcirculation. Two patients with TTP and slight renal failure with proteinuria and microscopic hematuria, were treated by repeated plasma exchanges with fresh frozen plasma, associated with prednisone and cyclophosphamide in one case, and prednisone alone in the other one. Platelet count, hematocrit and lactic dehydrogenase reverted to normal values within the fourth exchange; circulating immune complexes were never detected. Plasma factor stimulating prostacyclin activity lacked in only one patient and returned to normal levels after plasma exchange without being affected during a hematologic relapse. Renal function and urinary abnormalities reverted to normal by the end of plasma exchange and nine and six months renal and hematologic follow-up is still negative. Renal abnormalities in TTP seem to take advantage of early treatment by plasma exchange, which further to replacement of missing plasma factors, can account for the removal of toxic substances to be further investigated on.
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PMID:Renal abnormalities reverted by plasma exchange in thrombotic thrombocytopenic purpura. 668 92

We describe a patient with recurrent thrombotic thrombocytopenic purpura (TTP) manifested solely by aphasia after influenza infection. The clinical diagnosis was not made during acute episodes, and during the intercurrent period the patient had features of chronic glomerular disease, including hypertension, proteinuria, RBC casts, and a nonspecific renal histological appearance. A final episode of aphasia, acute renal failure, and microangiopathic anemia and thrombocytopenia made the diagnosis of TTP apparent. Chronic glomerular disease may in rare instances be a manifestation of occult TTP or the sequel of a prior acute episode of this disorder.
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PMID:Recurrent thrombotic thrombocytopenic purpura after viral infection. Clinical and histologic simulation of chronic glomerulonephritis. 719 23

Microangiopathic hemolytic anemia and thrombocytopenia secondary to disseminated intravascular coagulation is a well-described complication of widely metastatic carcinoma. The authors report four cases of gastric carcinoma, one case of colon cancer, and one case of adenocarcinoma of unknown primary in which the patient developed a syndrome analogous to thrombotic thrombocytopenic purpura, consisting of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure without definite evidence of disseminated intravascular coagulation. In contrast to previous reports, postmortem examination in three of the cases revealed no recurrence or only microscopic foci of residual tumor. In the remaining three, there was clinical and pathologic evidence of grossly disseminated carcinoma. Also in contrast to previous cases, all patients evidenced azotemia and proteinuria at the onset of the syndrome and ultimately uremia was a contributing cause of death. Coagulation profiles showed prolonged thrombin times and elevated fibrin degradation products in four instances and did not distinguish the patients with grossly metastatic disease from those with no tumor or only microscopic residua. Circulating immune complexes containing carcinoembryonic antigen were found in the patient with metastatic colon carcinoma. The syndrome was clinically identical whether or not grossly metastatic tumor was present, and it should not be attributed to advanced disease without definite clinical or pathologic evidence of a recurrence.
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PMID:Microangiopathic hemolytic anemia, thrombocytopenia, and renal failure in patients treated for adenocarcinoma. 728 73

The (NZW x BXSB)F1 (W/BF1) mouse is known to be an animal model of systemic lupus erythematosus (SLE) and immune thrombocytopenic purpura (ITP). These mice produce not only anti-DNA antibodies but also anti-platelet antibodies, resulting in decreased platelet counts. They show a high level of proteinuria, increased white blood cell (WBC) counts, hypertension, and myocardial infarction due to the high levels of anti-cardiolipin antibodies. When W/BF1 mice (4-5 months) were lethally irradiated and then reconstituted with T cell-depleted bone marrow cells of normal BALB/c mice (8 weeks), 60% of the mice survived more than one year. The WBC and platelet counts in the mice were normalized, and the levels of anti-DNA and anti-platelet antibodies decreased. The renal dysfunction was also ameliorated as indicated by a lower level of proteinuria, lower levels of serum creatinine (S-CRTN) and blood urea nitrogen (BUN), and by improved histology. The blood pressure (BP) of the treated W/BF1 mice decreased due to the improved renal functions. In contrast to the non-treated W/BF1 mice which died of myocardial infarction or renal failure by the age of 7 months, the treated W/BF1 mice showed no evidence of myocardial infarction even one year after BMT. This was due to the lower cardiolipin levels.
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PMID:Effect of bone marrow transplantation on antiphospholipid antibody syndrome in murine lupus mice. 778 96

Clinical manifestations and laboratory findings in 40 patients with thrombotic thrombocytopenic purpura (TTP) in Japan are reviewed. The most common clinical features were central neurological sings. jaundice, fever, hemorrhagic tendency, and renal abnormalities. Laboratory tests showed anemia, thrombocytopenia, hyperbilirubinemia, high serum LDH levels, and low serum haptoglobin levels. BUN and serum creatinine levels were elevated only in about 15% of the patients, although microscopic hematuria and proteinuria were observed more frequently (about 70%). Autoantibodies, such as antinuclear antibody and rheumatoid factor, were also observed in 4% to 9% of the patients. Coagulation and fibrinolysis studies showed normal values in the majority of the patients, suggesting intravascular generation of thrombin and plasmin was minimal in TTP.
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PMID:[Clinical manifestations and laboratory findings of thrombotic thrombocytopenic purpura]. 843 12


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