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)

We report on a Japanese girl with Upshaw-Schulman syndrome, a congenital disorder characterized by recurrent thrombocytopenia, microangiopathic hemolytic anemia, proteinuria and hematuria that can be transiently improved by the transfusion of plasma or various plasma components. Unusually Large von Willebrand Factor (ULvWF) multimers were found during both relapse and remission phases. Serial plasma levels of 6-keto-prostaglandin F1 alpha (PGF1 alpha), the stable metabolite of prostacyclin (PGI2), were low at relapse. When the patient was treated with continuous PGI2 infusion, the microangiopathic hemolytic process gradually subsided within 10 days. These results suggest that PGI2 may be partly involved in the pathogenesis of this congenital disorder.
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PMID:Partial clinical improvement in Upshaw-Schulman syndrome following prostacyclin infusion. 775 78

We described a patient with a POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal grammapathy and Skin changes) who was found to have renal involvement with peculiar renal pathological findings. Hitherto, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe HTA, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour evoke by light microscopy a MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits are found. The finding of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial space could evoke TMA. But there is neither thrombi, nor arteriolar changes. We are inclined to presume that microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. An increased production of IL-6 could support the efficacity of corticosteroid therapy, particularly in acute clinical situations.
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PMID:[Renal involvement in POEMS syndrome]. 786 28

The hemolytic-uremic syndrome (HUS) is an acute disorder, characterized by the triad of microangiopathic hemolytic anemia, nephropathy and thrombocytopenia. The great majority of patients are children, usually under 4 years of age, although adults can be affected. The onset is abrupt and usually follows gastroenteritis or upper respiratory infection. Later, clinical manifestations based on the triad, such as pallor, jaundice, edema, hypertension and purpura soon develop. The urinary output is reduced and the urine may appear dark yellow or tea-colored. Laboratory tests of peripheral blood show severe hemolytic anemia associated with fragmented red blood cells and thrombocytopenia, usually below 50,000/microliters. The blood urea nitrogen, serum creatinine and lactate dehydrogenase concentrations are elevated. Proteinuria and microscopic hematuria, which are indicative of active glomerular damage are also seen. Profound understanding of these manifestations is sufficient to permit an early diagnosis of HUS.
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PMID:[Diagnosis and clinical features of hemolytic uremic syndrome]. 843 21

A 50-year-old male was admitted to our hospital because of proteinuria, thrombocytopenia and moderate renal dysfunction. On admission, he had massive ascites, which was transudatory. During his clinical course, renal function deteriorated and the urine volume was decreased. Hemolytic uremic syndrome with massive ascites was diagnosed based on the finding of thrombocytopenia, acute renal failure and hemolytic anemia. Methylpredonisolone pulse therapy was not effective, but plasma exchange given 22 times in total combined with vincristine sulfate, PGI2 analogue and vitamin E administration was very effective for thrombocytopenia, renal dysfunction and hemolytic anemia. Massive ascites disappeared at the same time. After complete recovery of renal function, renal biopsy was performed, revealing the reticulation of mesangial matrix and mesangiolysis, which correspond to hemolytic uremic syndrome.
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PMID:[A case of hemolytic uremic syndrome accompanied by massive proteinuria and ascites]. 871 12

In 1982 Weinstein coined the term HELLP syndrome to describe a special group of pre-eclamptic women who had evidence of hemolysis, elevated liver enzymes and low platelets. The question of whether the HELLP syndrome exists as a distinct entity or is a part of a spectrum of pregnancy complications has long been a source of speculation and debate among obstetricians and internists. A review of the literature indicates that the syndrome occurs in post-partum from 20% to 30% of the cases; maternal mortality from 1% to 4% and perinatal mortality from 5% to 40%. Hypertension and proteinuria are frequently associated with HELLP syndrome, but may also be absent. The syndrome is a group of clinical and pathological manifestations resulting from an insult that leads to intravascular platelet activation (thrombocytopenia) and micro-angiopathic hemolytic anemia (elevated total bilirubin and LDH). Endothelial damage and vasospasm are responsible of hepatic hypoperfusion that results in liver damage, as indicated by a rise in circulating liver enzymes. Since natural evolution of HELLP syndrome is the disseminated intravascular coagulation (CID), the interruption of pregnancy is mandatory and irrespective of the gestational age. This is the reason of an high incidence of cesarean section. Visser and Wallemburg (1995) treated 128 pre-eclamptic patients with HELLP syndrome with volume expansion and pharmacologic vasodilatation under invasive haemodynamic monitoring; they tried to delay delivery with the aim of enhancing fetal maturity. A complete reversal of HELLP occurred in 43% of patients. Further investigations will be necessary to confirm this interesting experience.
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PMID:[The HELLP syndrome. Notes on its pathogenesis and treatment]. 876 57

Dapsone, a synthetic sulfone with chemical similarities to sulfapyridine, has been used for a number of years to treat leprosy and dermatitis herpetiformis. Recently, a number of prospective, randomized, double-blind trials have shown their success in the management of rheumatoid arthritis, with dapsone being superior to placebo and comparable to chloroquine and hydroxychloroquine. Its mode of anti-inflammatory actions in rheumatoid arthritis is not clearly understood, but modulation of neutrophil activity or inhibition of neutrophil inflammatory product formation or release appear to play a role. The major limiting side effect is hemolytic anemia, which may be mitigated through careful patient selection, conservative drug dosing, close monitoring, and possibly, concurrent administration of antioxidants or cytochrome P450 inhibitors. Methemoglobinemia is another common finding among patients receiving dapsone therapy, but rarely does it result in prominent symptoms other than transient pallor. Less common adverse events to dapsone include the idiosyncratic reactions of leukopenia and agranulocytosis, cutaneous eruptions, peripheral neuropathy, psychosis, toxic hepatitis, cholestatic jaundice, nephrotic syndrome, renal papillary necrosis, severe hypoalbuminemia without proteinuria, an infectious mononucleosis-like syndrome, and minor neurological and gastrointestinal complaints. In this report, two patients with advanced rheumatoid arthritis, who were safely and effectively treated with dapsone after failure with other second-line agents, are described and the literature is reviewed. We suggest that dapsone is an effective second-line agent in the treatment of rheumatoid arthritis.
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PMID:Dapsone in rheumatoid arthritis. 879 11

We report a case of "crack" cocaine abuse in a pregnant patient associated with haematuria, proteinuria, haemolytic anaemia, renal impairment, thrombocytopenia and pulmonary oedema. The case illustrates the problems for clinicians where unrecognized cocaine abuse interferes with the diagnosis and management of a complicated pregnancy. In addition, we discuss the principles for the safe conduct of anaesthesia in the pregnant cocaine abuser.
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PMID:Unrecognized "crack" cocaine abuse in pregnancy. 894 48

The early detection of HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) is the basic condition for immediate therapeutic management, which mainly leads to prompt delivery. The classical symptoms despite the typical laboratory evaluation (hemolysis, elevated liver enzymes, low platelets) are epigastric or right upper quadrant pain and nausea and vomiting; the classical signs of preeclampsia (proteinuria and hypertension) may be absent in 20%. The differential diagnostic problems of HELLP syndrome arise in relation to the mimicry-symptomatic: upper abdomen pain can imitate gastroenterologic diseases (e.g. cholelithiasis, appendicitis), the elevated liver enzymes combined with hyperbilirubinemia liver diseases (e.g. viral hepatitis) and thrombocytopenia in combination with hemolytic anemia, neurological symptoms and renal failure other similar pathogenetic disorders due to the category of thrombotic microangiopathies. Regarding the common symptoms thrombocytopenia, hemolysis as well as signs of preeclampsia with or without renal failure the differentiation from various autoimmune diseases also can be difficult in special cases. Rare first manifestations and serious simultaneous diseases which can overlay the typical signs of HELLP syndrome show the variety of HELLP syndrome. Interdisciplinary detours and delay are the consequences of this differential diagnostic problems, which could imply deleterious effects on the mother and the fetus, until the final diagnosis is clear. Therefore all pregnant women with upper abdomen pain irrespective of symptoms of preeclampsia should be considered to have HELLP syndrome and immediate laboratory evaluation has to be done. If there is any doubt a interdisciplinary consultation is required!
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PMID:[Differential HELLP syndrome diagnosis]. 896 90

A 71-year-old woman was treated for a relapsing pulmonary tuberculosis with reinstitution of rifampicin after a medication-free interval of 2 years. After ingestion of the second dose, she developed severe hemolytic anemia and acute renal failure (ARF) necessitating dialysis. We demonstrated the presence in the patient's serum of rifampicin-dependent immunoglobulin G (IgG) and IgM antibodies, which caused red blood cell lysis through interaction with the I antigen on the erythrocyte surface. A review of the literature yielded 48 cases of rifampicin-associated renal failure. A subgroup of 37 patients could be distinguished, which, analogous to our case, suddenly developed ARF and frequently also developed hemolytic anemia and/or thrombocytopenia during intermittent or interrupted treatment. Regarding the pathogenesis of the ARF, renal biopsy consistently revealed tubular lesions. Although intravascular hemolysis with hemoglobinuria may play a role, it is not uniformly present. Our demonstration of an antibody with anti-I specificity provides a possible explanation. The I antigen is also expressed on tubular epithelium and may, therefore, be the target structure through which rifampicin-antibody complexes lead to tubular cell destruction. The other cases of rifampicin-associated ARF were unrelated to this subgroup: two cases of rapidly progressive glomerulonephritis, five cases of acute interstitial nephritis, and four cases of light chain proteinuria were recorded.
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PMID:Rifampicin-associated acute renal failure: pathophysiologic, immunologic, and clinical features. 974 Jan 77

The objective was to study the relationship between the levels of interleukin-1 receptor antagonist (IL-1Ra) and disease activity and the acute-phase response in SLE patients with and without renal involvement. Twenty SLE patients who had distinct active clinical manifestations (eight glomerulonephritis, four systemic vasculitis without kidney involvement, nine skin rash, 12 arthritis, five serositis, four neuropsychiatric manifestations, three thrombocytopenia, one myositis and one haemolytic anaemia) were studied during a period of 8-12 months. Serum and plasma samples were taken at intervals of 6 weeks-4 months and tested for IL-1Ra, IL-1 beta, IL-6, IgG and anti-dsDNA, Clq, C3, C4 and C-reactive protein (CRP). IL-1Ra serum concentrations were increased in most SLE patients with active disease when compared to normal blood donors. However, at the time of flare, significantly higher levels of IL-1Ra were observed in patients with extra-renal disease as compared to other patients (median [range]: 363 [202-3041] and 4847 [268-27180] pg/ml for patients with and without renal involvement, respectively). This difference was not due to proteinuria. IL-1Ra levels did not correlate with SLEDAI score during flares, but they were elevated during flares in patients with extra-renal manifestations. When disease activity was at its highest, IL-1Ra concentrations correlated with IL-1 beta (r = 0.76; P < 0.001), IL-6 (r = 0.60; P < 0.01) and CRP (r = 0.61; P < 0.01), but not with C1q, C3, C4 and anti-dsDNA levels. The study showed that the pattern of inflammatory cytokines in active SLE varies in a manner that is dependent on which organs are involved. A relative absence of IL-1Ra response appears to be a feature characteristic of kidney involvement. IL-1Ra elevation clearly correlates with flares involving other organs.
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PMID:Low levels of interleukin-1 receptor antagonist coincide with kidney involvement in systemic lupus erythematosus. 944 89


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