Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021831 (enteropathy)
 4,403 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To expand the clinical spectrum of ulcerative colitis-associated lung disease, we describe a patient with panbronchiolitis associated with ulcerative colitis. In addition to his having a distinctive pulmonary manifestation of this bowel disease, other noteworthy aspects of this patient's course include the onset of pulmonary symptoms several years prior to bowel manifestations and the partial resolution of his pulmonary symptoms following colectomy.
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PMID:Diffuse panbronchiolitis preceding ulcerative colitis. 272 Dec 73

Neoplasms constitute the major complication of coeliac disease, and high-grade T-cell lymphoma of the small intestine (enteropathy-associated T-cell lymphoma) is the most common neoplasm in this category. HLA genotyping indicates that in patients with enteropathy-associated T-cell lymphoma have the coeliac disease associated DQA1*0501, DQB1*0201 phenotype, although additional HLA-DR/DQ alleles may represent risk factors for lymphoma development. Molecular biological and immunohistochemical studies have shown that the intestinal mucosa distant from the tumour contains clonal populations of small T cells, often of the same clone as the high-grade T-cell lymphoma. These findings suggest that enteropathy-associated T-cell lymphoma arises in the setting of coeliac disease and evolves from reactive intraepithelial lymphocytes through a low-grade lymphocytic neoplasm to a high-grade tumour, which is usually the cause of the presenting symptoms. Most cases of chronic ulcerative enteropathy (ulcerative jejunitis) are probably part of the same disease process. If the ulceration occurs at a time when the neoplastic T-cells are of a low grade, morphological recognition of tumour cells in the ulcers may be impossible. Carcinoma of the pharynx and oesophagus, and adenocarcinoma of the small intestine, are increased in frequency in patients with coeliac disease. The increased risk of carcinoma of the oesophagus may be related to vitamin A deficiency. A number of reports have indicated an increased prevalence of various types of chronic hepatitis in patients with coeliac disease, but no coherent view of the cause of this association has emerged. Similarly, patients with coeliac disease have been reported to have various forms of fibrosing lung disease of uncertain causation. In recent years, there have been several reports, mainly from Italy, of a syndrome of epilepsy and bilateral brain calcification occurring in coeliac patients. The pathogenesis of this condition is not known and its prevalence in other communities is uncertain. Splenic atrophy occurs frequently in patients with coeliac disease and is related to the severity of the disease and degree of dietary control. Splenic atrophy predisposes to infection with capsulated bacteria, although mortality studies indicate that infection with these organisms is not a major cause of death in patients with coeliac disease.
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PMID:The major complications of coeliac disease. 754 31

The pulmonary associations of inflammatory bowel disease (IBD) are poorly characterized. The clinical, physiological and high-resolution computed tomographic thorax characteristics of the lung disease in patients with IBD presenting with respiratory symptoms are described. Detailed clinical information was obtained and standard pulmonary physiological tests and thorax high-resolution computed tomography performed on 14 patients with ulcerative colitis (UC) and three with Crohn's disease (CD), 10 male, aged 38-83 yrs. Respiratory symptoms had been present for 2-50 yrs and extraintestinal manifestations were present in three (17.6%). Normal pulmonary physiology (six patients) was associated with the high resolution computed tomographic changes of bronchiectasis, mosaic perfusion and air trapping suggestive of obliterative bronchiolitis and a pattern of centrilobular nodules and branching linear opacities ("tree in bud" appearance) suggestive of either cellular bronchiolitis or bronchiolectasis with mucoid secretions. Bronchiectasis was found in 13 patients (11 UC, 2 CD), 11 had air trapping and five had a "tree in bud" appearance on computed tomography. One patient had a predominantly peripheral reticular pattern at the lung bases similar to that found in cryptogenic fibrosing alveolitis and one patient had a mixed reticular and ground-glass pattern in the midzones with a patchy distribution in the central and peripheral portions of the lungs with air trapping. Eleven patients (three with alveolitis) exhibited a clinical and/or physiological response to steroids. Pulmonary abnormalities in ulcerative colitis and Crohn's disease can present years after the onset of the bowel disease and can affect any part of the lungs. Early recognition is important as they can be strikingly steroid-responsive.
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PMID:Clinical and radiological characteristics of lung disease in inflammatory bowel disease. 1067 19

Ulcerative colitis and Crohn's disease are associated with a variety of systemic manifestations. Pulmonary disease has been described much less frequently than other organ systems, yet more than 400 cases have been reported. Lung and gastrointestinal system are originated from primitive gut and they have same pathogenetic changes in these patients. Major patterns of pulmonary disease associated with inflammatory bowel disease (IBD) are pleuritis, airway disease, interstitial lung disease, necrobiotic nodules, pulmonary eosinophilia, thromboembolic disease, vasculitis, granulomatous lung disease, etc. Colectomy may aggravate respiratory symptoms. Drug induced disease must be kept in mind in patients taking sulfasalazine, mesalamine, methotrexate, and anti-TNF-alpha. Latent pulmonary abnormalities are evident either at pulmonary function tests or induced sputum or bronchoalveolar lavage, have been also reported in patients with inflammatory bowel disease in the absence of clinical evidence of airway disease. The treatment of IBD related respiratory involvement depends on the specific pattern of involvement, if left untreated, especially in airway disease, puts the patient at risk of developing irreversible destruction of the air passage. A high degree of suspicion is necessary to detect early the respiratory disease in association with any form of bowel disease.
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PMID:[Inflammatory bowel disease and lung]. 1700 50

Celiac disease is the most common severe food intolerance in the Western world and is due to gluten ingestion in genetically susceptible children and adults. The key treatment in these patients is a gluten-free diet, because most complications are more common when dietary compliance is poor. The most serious complication of celiac disease is the development of neoplasms (the most common of which is enteropathy-associated T-cell lymphoma). However, a number of reports have indicated an increased prevalence of ulcerative jejunitis and extraintestinal manifestations, including chronic hepatitis, fibrosing lung disease, and epilepsy syndromes. We report the case of a 53-year-old-man with long-standing diarrhea; because celiac disease was not suspected, the patient developed celiac-associated T-cell lymphoma and mesenteric panniculitis.
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PMID:[Intestinal lymphoma and mesenteric panniculitis: complications of undiagnosed celiac disease]. 1840 87

Alpha-MSH is a tridecapeptide derived from proopiomelanocortin. Many studies over the last few years have provided evidence that alpha-MSH has potent protective and antiinflammatory effects. These effects can be elicited via centrally expressed melanocortin receptors that orchestrate descending neurogenic antiinflammatory pathways. alpha-MSH can also exert antiinflammatory and protective effects on cells of the immune system and on peripheral nonimmune cell types expressing melanocortin receptors. At the molecular level, alpha-MSH affects various pathways implicated in regulation of inflammation and protection, i.e., nuclear factor-kappaB activation, expression of adhesion molecules and chemokine receptors, production of proinflammatory cytokines and mediators, IL-10 synthesis, T cell proliferation and activity, inflammatory cell migration, expression of antioxidative enzymes, and apoptosis. The antiinflammatory effects of alpha-MSH have been validated in animal models of experimentally induced fever; irritant and allergic contact dermatitis, vasculitis, and fibrosis; ocular, gastrointestinal, brain, and allergic airway inflammation; and arthritis, but also in models of organ injury. One obstacle limiting the use of alpha-MSH in inflammatory disorders is its pigmentary effect. Due to its preserved antiinflammatory effect but lack of pigmentary action, the C-terminal tripeptide of alpha-MSH, KPV, has been delineated as an alternative for antiinflammatory therapy. KdPT, a derivative of KPV corresponding to amino acids 193-195 of IL-1beta, is also emerging as a tripeptide with antiinflammatory effects. The physiochemical properties and expected low costs of production render both agents suitable for the future treatment of immune-mediated inflammatory skin and bowel disease, fibrosis, allergic and inflammatory lung disease, ocular inflammation, and arthritis.
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PMID:Alpha-melanocyte-stimulating hormone and related tripeptides: biochemistry, antiinflammatory and protective effects in vitro and in vivo, and future perspectives for the treatment of immune-mediated inflammatory diseases. 1861 39

A 5-years multicenter prospective study on 201 patients with common variable immunodeficiencies and 101 patients with X-linked agammaglobulinemia over a cumulative follow-up period of 1,365 patient-years was conducted to identify prognostic markers and risk factors for associated clinical co-morbidities, the effects of long-term immunoglobulin treatment and the IgG trough level to be maintained over time required to minimise infection risk. Overall, 21% of the patients with common variable immunodeficiencies and 24% of patients with X-linked agammaglobulinemia remained infection free during the study. A reduction of pneumonia episodes has been observed after initiation of Ig replacement. During the observation time, pneumonia incidence remained low and constant over time. Patients with pneumonia did not have significant lower IgG trough levels than patients without pneumonia, with the exception of patients whose IgG trough levels were persistently <400 mg/dL. In X-linked agammaglobulinemia, the only co-morbidity risk factor identified for pneumonia by the final multivariable model was the presence of bronchiectasis. In common variable immunodeficiencies, our data allowed us to identify a clinical phenotype characterised by a high pneumonia risk: patients with low IgG and IgA levels at diagnosis; patients who had IgA level <7 mg/dL and who had bronchiectasis. The effect of therapy with immunoglobulins at replacement dosage for non-infectious co-morbidities (autoimmunity, lymphocytic hyperplasia and enteropathy) remains to be established. A unique general protective trough IgG level in antibody deficiency patients will remain undefined because of the major role played by the progression of lung disease in X-linked agammaglobulinemia and in a subset of patients with common variable immunodeficiencies.
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PMID:Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study. 2164 92

Common variable immunodeficiency (CVID) represents one of the most frequently diagnosed disorders of the immune system. Though several causative and associated genes have been identified, the origins of most cases remain unknown. Diagnostic delay is common due to the gradual evolution and wide spectrum of phenotypes, which can include autoimmune disease, enteropathy, and lung disease. A recent genome wide array identified novel gene associations with CVID, and also showed that identification of a genetic signature via a Support Vector Machine algorithm may be a powerful diagnostic tool. Studies utilizing whole genome or exome sequencings have also met with success in identifying new causes of CVID in subgroups of patients.
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PMID:Chipping away at a mountain: genomic studies in common variable immunodeficiency. 2320 19

Pulmonary involvement in the course of inflammatory bowel disease has been a subject of interest to clinicians for long time, but despite this, its epidemiology and potential pathomechanisms remain obscured. Equally unclear is the role of medications used for bowel disease treatment in lung disease development. We present three patients with ulcerative colitis, all treated with mesalazine, in whom unexplained lung disease developed. Due to different clinical and radiological presentation, different conditions were initially placed on the top of the differential list in each of them. The outcome was favourable in all patients despite differences in management. We compared our patients with similar cases from literature. We show the level of difficulty and complexity in the issue of lung disease in patients with inflammatory bowel disease.
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PMID:Pulmonary pathology in patients with ulcerative colitis treated with mesalazine--a challenging and complex diagnostic problem. Case series and literature review. 2496 41

Fecal calprotectin (FC) is used to asses the presence of intestinal inflammation also in patients with Cystic Fibrosis (CF) and recent studies showed a correlation between bowel and lung disease in these patients. The aim of this study was to analyze the levels of FC in CF and correlate them with different phenotypes of disease. We enrolled a cohort of 54 CF patients and 50 healthy controls. In these patients, calprotectin has been assayed on a stools sample using an ELISA kit. In all patients we analyzed, FC levels were elevated above the cut-off value and significantly higher than in healthy controls. Among CF patients, FC was significantly higher in patients older than 18 years, with pancreatic insufficiency, underweight status, Pseudomonas Aeruginosa airways colonization, CF-related diabetes mellitus, reduced lung function, or high number of pulmonary exacerbations. These results suggest that in patients with CF, FC levels are not only influenced by the CF enteropathy but also by the severity of the genetic disease. Since we found higher FC levels in patients with a severe phenotype (P. Aeruginosa airways colonization, FEV1<50% of predicted, pancreatic insufficiency, underweight status,) we suggest that this marker could be useful to monitor longitudinally a clinical worsening.
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PMID:Severe disease in Cystic Fibrosis and fecal calprotectin levels. 2801 84


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