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)

Protein losing enteropathy is described as a diverse group of disorders associated with excessive loss of serum proteins into the gastrointestinal (GI) tract. The etiology of protein losing enteropathy is various. Increased mucosal permeability to protein as a result of cell damage, mucosal erosion, or lymphatic obstruction may develop protein losing enteropathy. Celiac disease is a common cause of protein losing enteropathy associated with small bowel villous atrophy in Europe. We experienced a case of protein losing enteropathy associated with small bowel villous atrophy of unknown origin. A 36-year-old woman was admitted due to chronic watery diarrhea and weight loss. Laboratory findings showed total protein 4.7 g/dL, albumin 2.7 g/dL, cholesterol 100 mg/dL, WBC 6,000/mm3 (lymphocyte 13.6%) with the absence of proteinuria. On esophagogastroduodenoscopic examination, duodenal ulcer scar was noted on the bulb and colonoscopic finding was nonspecific. On small bowel enteroscopy, jejunal and ileal villi was scantly noticed. Small bowel biopsy showed marked villous atrophy. Her symptoms did not improve after supportive care. Gluten free diet was tried because celiac disease could not be ruled out completely. Diarrhea ceased and body weight regained after gluten free diet.
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PMID:[A case of protein-losing enteropathy associated with small bowel villous atrophy]. 1816 31

Several studies have suggested that T cell-producing permeability factors might lead to proteinuria in minimal change nephrotic syndrome (MCNS). However, it is still unclear whether T-cell abnormalities cause MCNS. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder of the immune regulation system, which leads to severe autoimmune phenomena including autoimmune enteropathy, atopic dermatitis with high levels of serum immunoglobulin E (IgE), type 1 diabetes mellitus (T1DM), and severe infection such as sepsis, which frequently result in death within the first 2 years of life. This disease is caused by mutations in the FOXP3 gene that result in the defective development of regulatory T (Treg) cells. This report describes a 5-year-old boy with IPEX syndrome with a 3 bp deletion in the FOXP3 gene (c.748-750delAAG, p.250K.del) and a paucity of CD4(+) CD25(+) FOXP3(+) T cells. The boy's condition was complicated by MCNS in addition to many IPEX-related manifestations, such as atopic dermatitis, T1DM, enteropathy, sepsis and hemolytic anemia. This is the first report of IPEX syndrome complicated by MCNS, and our findings imply that Treg cell dysfunction may be crucial for the development of MCNS.
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PMID:Minimal change nephrotic syndrome associated with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. 1918 34

A case of immunoglobulin A nephropathy (IgAN) complicating a 10-year history of biopsy-proven Crohn's disease in a 31-year-old man is described. The patient presented with mild proteinuria and impaired renal function in the setting of an exacerbation of Crohn's disease. Renal biopsy showed IgAN. The patient responded to steroid treatment with clinical remission of the bowel disease and improvement of renal function, while proteinuria remained unchanged. IgA glomerulonephritis is rarely associated with Crohn's disease with only a few previously described cases. We briefly review these cases together with an overview of potential pathophysiological connections between these two diseases.
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PMID:IgA nephropathy in association with Crohn's disease: a case report and brief review of the literature. 2044 96

The mechanism responsible for proteinuria in non-genetic idiopathic nephrotic syndrome (iNS) is unknown. Animal models suggest an effect of free radicals on podocytes, and indirect evidence in humans confirm this implication. We determined the oxidative burst by blood CD15+ polymorphonucleates (PMN) utilizing the 5-(and-6)-carboxy-2',7'-dichlorofluorescin diacetate (DCF-DA) fluorescence assay in 38 children with iNS. Results were compared with PMN from normal subjects and patients with renal pathologies considered traditionally to be models of oxidative stress [six anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis, seven post-infectious glomerulonephritis]. Radicals of oxygen (ROS) production was finally determined in a patient with immunodeficiency, polyendocrinopathy, enteropathy X-linked (IPEX) and in seven iNS children after treatment with Rituximab. Results demonstrated a 10-fold increase of ROS production by resting PMN in iNS compared to normal PMN. When PMN were separated from other cells, ROS increased significantly in all conditions while a near-normal production was restored by adding autologous cells and/or supernatants in controls, vasculitis and post-infectious glomerulonephritis but not in iNS. Results indicated that the oxidative burst was regulated by soluble factors and that this regulatory circuit was altered in iNS. PMN obtained from a child with IPEX produced 100 times more ROS during exacerbation of clinical symptoms and restored to a near normal-level in remission. Rituximab decreased ROS production by 60%. In conclusion, our study shows that oxidant production is increased in iNS for an imbalance between PMN and other blood cells. Regulatory T cells (Tregs) and CD20 are probably involved in this regulation. Overall, our observations reinforce the concept that oxidants deriving from PMN are implicated in iNS.
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PMID:Failure of regulation results in an amplified oxidation burst by neutrophils in children with primary nephrotic syndrome. 2049 93

Protein-loosing enteropathy in patients with inflammatory bowel diseases (IBDs) is an uncommon complication, but should be considered in any patient with hypoproteinemia in whom other causes have been excluded such as concomitant hepatic disease, severe malnutrition or proteinuria. The diagnosis is based on determination of fecal alpha-1 antitripsin clearance and stool analysis. Prognosis depends upon the patient and the disease location, severity and complication. Treatment is directed at control of the underlying IBDs but also includes albumin infusion in the most severe cases of hypoalbuminemia and fluid retention, dietary modifications for recovery and maintenance of nutritional status and supportive care to prevent further complication like deep venous thrombosis. Surgery is not curative in Crohn's disease patients, but in severe protein-loosing enteropathy with severe disease, not responding to conventional therapy, it may be the best choice.
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PMID:Protein-losing enteropathy in inflammatory bowel diseases. 2644 87


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