UMLS:C0024523 - FACTA Search

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Query: UMLS:C0024523 (malabsorption)
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A 79-year-old woman developed collagenous sprue, a rare small intestinal mucosal disorder. Later, extensive T-cell lymphoma was documented, a neoplasm known to complicate celiac disease. Although the precise relationship of collagenous sprue to celiac disease has been debated and remains controversial, the findings here provide additional evidence that collagenous sprue and celiac disease are closely linked. In the past, long-term survival with collagenous sprue may have been compromised due to severe pan-malabsorption. With improved treatment measures, including modern nutritional support, it is likely that there will be an increased opportunity in future for improved appreciation of the complications of collagenous sprue, specifically, lymphoma.
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PMID:Collagenous sprue associated with an extensive T-cell lymphoma. 1254 98

Celiac disease (Celiacal sprue = gluten-sensitive enteropathy = netropic sprue) is the all-life genetically determined autoimmune disease with permanent intolerance to gluten, which damages the intestinal mucous membrane and alterates the immune system. The atrophy and typical inflammatory changes of mucous membrane results in malabsorption with diarrhea, general weakness, anemia and weight loss. The clinical picture of celiac disease is considerably heterologous. Only 20-30% of patients suffer from active-classical form of the disease. Non-diagnosed, inactive forms of the disease form 70-80% of cases of celiac disease in adult individuals. The therapy is based on diet without gluten. Application of the diet usually results in clinical improvement and signs of the disease are diminished. The relapse of celiac disease occurs after a gluten load. Celiac disease and dermatitis Duhring are considered to be two equal forms how gluten enteropathy becomes manifest. Celiac disease is often associated with other autoimmune diseases (e.g. insulin-dependent diabetes mellitus, autoimmune thyreoiditis). Untreated celiac disease still represents a serious medical risk, since it is an important precancerosis. Introduction of highly sensitive methods for the determination of antibodies against endomysium and tissue transglutaminase significantly extended possibilities of diagnosis and screening for celiac disease. It became obvious that the real incidence of celiac disease including the non-diagnosed forms of the disease in the European population is greater than 1:200 to 1:250.
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PMID:[Celiac disease--a severe disease]. 1450 71

Tropical sprue is a disease that causes progressive villus atrophy in the small intestine, similar to nontropical (celiac) sprue. The loss of intestinal villi profoundly affects intestinal absorptive function, and patients with tropical or nontropical sprue present with malabsorption. Whereas the etiology of celiac sprue has been elucidated in considerable detail, the etiology of tropical sprue remains obscure. The favored hypothesis is that the disease is either initiated or sustained by a still-undefined infection. Patients with tropical sprue typically present with macrocytic anemia due to malabsorption of folate and/or vitamin B(12). Treatment of tropical sprue with folic acid replacement was introduced more than 50 years ago and has become standard medical treatment. Vitamin B(12) replacement is usually added if there is evidence of B(12) deficiency or malabsorption. Treatment of tropical sprue with folate and B(12) cures the macrocytic anemia and the accompanying glossitis, and often results in increased appetite and weight gain. However, even prolonged treatment with these vitamins fails to restore villus atrophy, and malabsorption usually persists. The benefit of antibiotic treatment of tropical sprue was first documented during World War II, when sulfonamides were used to treat epidemics of tropical sprue in British and Italian troops in India. Antibiotic treatment has since become the standard treatment, and tetracycline has replaced sulfonamides. The recommended length of treatment with tetracycline is 6 months and it is given in combination with folate. The treatment has been shown to normalize mucosal structure in the small intestine and resolve malabsorption in most patients with tropical sprue. However, there is a substantial relapse rate in treated patients who return to, or remain in, endemic areas in the tropics.
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PMID:Tropical Sprue. 1472 33

Celiac disease is a small bowel disorder characterized by flattened villi and crypt hyperplasia, often with malabsorption. Improvement occurs with a gluten-free diet. Sensitive and specific assays (eg, immunoglobulin A antibodies to tissue transglutaminase [tTG]) that can be quantified appear to be valuable tools for population screening studies. In addition, their use is expanding widely in the clinical practice arena, being employed as a method of case finding. In this evaluation, clinical use of a commercially available test kit was explored. Of 1330 samples submitted to our hospital laboratory by physicians in British Columbia, Alberta and the Yukon Territory (from 1999 to 2003, inclusive), 96 patients (7%) had increased values (normal range greater than 20 units) and markedly increased levels greater than 100 units were detected in 36 patients (3%). Of these, 14 patients (almost 40%) were referred to gastroenterologists in our hospital and all 14 had small intestinal biopsies. Of these, three patients (more than 20%) did not have celiac disease. Two had normal small bowel biopsies and one had unclassified sprue or 'sprue-like' intestinal disease that failed to respond to a gluten-free diet. The other 11 had biopsy-defined celiac disease. While the tTG assay may be a useful predictor of celiac disease, small intestinal biopsy is still required to confirm the diagnosis. In clinical practice, even strongly positive tTG results are not specific in individual patients, do not necessarily correlate with the degree of severity of biopsy change and, as a result, are also unlikely to be useful for monitoring diet compliance.
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PMID:Strongly positive tissue transglutaminase antibody assays without celiac disease. 1476 Apr 28

Immunodeficient patients can develop malabsorption, mimicking celiac disease clinically and histologically. Such individuals may also occasionally require immunosuppressive therapy for autoimmune disorders. We have identified a patient with common variable immunodeficiency (CVID)-associated sprue complicated by duodenal cytomegalovirus (CMV) infection following corticosteroid and ancillary immunomodulatory therapy. Ganciclovir and a modification of the immunosuppressant regimen improved both clinical symptoms and villous atrophy. To our knowledge, this is original documentation of duodenal CMV infection secondary to immunomodulatory therapy for steroid-refractory CVID-sprue.
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PMID:Multimodal immunosuppressant therapy in steroid-refractory common variable immunodeficiency sprue: a case report complicating cytomegalovirus infection. 1650 46

Because of the wide variations in the clinical presentation of celiac disease and because treatment exists that is effective in most cases, screening of the general population for celiac disease has been considered. There is still no evidence that patients who have symptom-free celiac disease are at increased risk of small intestinal lymphoma or other complications. Prevention of osteoporosis seems to be the strongest indicator for widespread screening today [22]. The major cause of failure to respond to a gluten-free diet is continuing ingestion of gluten, but other underlying diseases must be considered. Many different drugs (eg, anti-tumor necrosis factor [TNF]-alpha) have been used in patients who have RCD [23]. Steroid treatment has been reported to be effective even in patients who have underlying early EATL. Histologic recovery in patients who have celiac disease usually takes several months but can take up to 1 year, even if the patient remains on a strict gluten-free diet. Some patients report celiac-related symptoms for months after a single gluten intake. The definitions for RCD in literature vary. The authors consider the definition give by Daum and colleagues [24] suitable. They defined true RCD as villous atrophy with crypt hyperplasia and increased IELs persisting for more than 12 months in spite of a strict gluten-free diet. If a patient is not responding well to a gluten-free diet, three considerations are necessary: (1) the initial diagnosis of celiac disease must be reassessed;(2) the patient should be sent to a dietician to check for errors in diet or compliance problems, because problems with the gluten-free diet are the most important cause for persisting symptoms; (3) other reasons for persisting symptoms (eg, pancreatic insufficiency, irritable bowel syndrome, bacterial overgrowth, lymphocytic colitis, collagenous colitis, ulcerative jejunitis, protein-losing enteropathy,T-cell lymphoma, fructose intolerance, cavitating lymphadenopathy, and tropical sprue) should be considered. Other causes for villous atrophy are Crohn's disease, collagenous sprue, and autoimmune enteropathy. Abdulkarim and colleagues [25] examined 55 patients who had a diagnosis of nonresponsive celiac disease. He found that 6 did not have celiac disease, and25 still had some gluten ingestion.Tursi and colleagues [26] reported 15 patients who had celiac disease with persisting symptoms. Because histology improved in all patients after several months, RCD was excluded. Of the 15 patients, 10 had small intestinal bacterial overgrowth, 2 showed lactose malabsorption causing the described symptoms, 1 had mistakenly taken an antibiotic containing gluten, and 1 patient each had Giardia lamblia and Ascaris lumbricoides. Thus, other entities must be considered in patients who have celiac disease and ongoing symptoms. In a follow-up clinical trial, 158 patients who had celiac disease underwent follow-up small intestine biopsies within 2 years after starting a gluten-free diet. Eleven patients (7.0%) with persisting (partial) villous atrophy were considered to have RCD; 5 of them developed EATL [27].RCD type I is characterized by normal expression of T-cell antigens and polyclonal TCR gene rearrangement.RCD type II is characterized by an abnormal IEL phenotype with the expression of intracytoplasmic CD3e, surface CD103, and the lack of classic surface T-cell markers such as CD8, CD4, and TCR-alpha/beta. This clonal IEL population can be considered crypt IEL [24]. RCD II has a poor prognosis, which is a problem for therapy. Clonal TCR gene rearrangements and loss of T-cell antigens such as CD8 and TCR-beta in IELs may indicate the development of an EATL in patients who have RCD. The markers for an overt EATL are a positive stool blood test, increased lactate dehydrogenase, or beta2-microglobulin [24]. If an overt lymphoma is suspected, upper and lower endoscopy, an ear, nose, and throat work-up, CT scan, capsule endoscopy, and possibly double-balloon enteroscopy should be performed. Most reports of the difficulties in treating patients who have true RCE are casereports. Turner and colleagues [28] reported on an induction of remission by useof the anti-TNF-alpha antibody infliximab and maintenance with prednisoloneand azathioprine. Olaussen and colleagues [29] and Mandal and colleagues [30]tried a nonimmunogenic elemental diet. Gillet and colleagues [31] reported successful treatment of a patient who hadRCD using anti-TNF-alpha antibodies (infliximab) for induction and azathioprinefor maintenance. Maurino and colleagues [32] studied seven consecutive patients diagnosed ashaving refractory sprue and no response to oral or parenteral steroids. Aftertreatment with azathioprine (2 mg/kg/d) and oral prednisone (1 mg/kg/d), fivepatients had a complete clinical remission. Two patients who did not respond totreatment at any time died. Goerres and colleagues [33] described 18 patients who had RCD, 10 of whomhad type I RCD, and 8 of whom had type II RCD. Treatment consisted ofazathioprine combined with prednisone for 1 year. Consistent with reports byother investigators, the response rates in the two groups differed. Eight of the10 patients who had type I RCD had a histologic response. Seven of the eightpatients who had type II RCD died, and six of the eight developed a lymphoma. At present there is no effective treatment for type II RCD.Fig. 3 presents a proposed algorithm for monitoring patients who have ce-liac disease.
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PMID:Monitoring nonresponsive patients who have celiac disease. 1687 29

We report the case of a 35-year-old woman with a diagnosis of coeliac disease at the age of 32 due to a severe malabsorption and flat mucosa without endomysial and tissue transglutaminase antibodies. The lack of clinical and histological improvement after 1 year of a gluten-free diet led to a diagnosis of refractory sprue. She had a good clinical response to steroids that were stopped after 3 months when she became pregnant. After delivery, she again started to complain of malabsorption with arthritis. Positivity for enterocyte autoantibodies together with a flat mucosa persistence allowed to identify a condition of autoimmune enteropathy; moreover, a rheumatological assessment gave evidence of an associated rheumatoid arthritis. Treatment by steroids and methotrexate brought to the remission of intestinal and articular symptoms together with an improvement of duodenal histology. This is the first description of an autoimmune enteropathy associated with rheumatoid arthritis. Autoimmune enteropathy should be always ruled out in patients with a villous atrophy unresponsive to a gluten-free diet, autoimmune manifestations and negativity of coeliac disease markers.
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PMID:Autoimmune enteropathy and rheumatoid arthritis: a new association in the field of autoimmunity. 1692 48

Celiac disease is a gluten-dependent small intestinal mucosal disorder that causes malabsorption, often with diarrhea and weight loss. Diagnosis is based on detection of typical biopsy changes in the proximal small bowel, followed by evidence for an unequivocal response to a gluten-free diet. Refractoriness in celiac disease may be due to poor diet compliance, sometimes intentional, or consumption of ubiquitious sources of gluten. Alternatively, the original diagnosis may not be correct (eg., duodenal Crohn's disease), or a second cause for symptoms may be present (eg., collagenous colitis, functional bowel disorder). In some with recurrent symptoms, a complication may be present (eg., collagenous sprue, small bowel carcinoma, lymphoma). In some, a response to a gluten-free diet can not be unequivocally defined, and more precise historical terms have been used including "sprue-like intestinal disease" or "unclassified sprue". Although a "wastebasket diagnosis", these likely represent a heterogeneous group, and some, but not all, may develop lymphoma. Precise definition will be critical in the future as an array of new treatments, including biological agents, may emerge.
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PMID:Refractory celiac disease and sprue-like intestinal disease. 1824 Mar 39

In adults with diarrhea or suspected malabsorption, a diagnosis of celiac disease requires that two criteria be fulfilled: first, a demonstration of typical pathological changes of untreated disease in biopsies from the proximal small bowel; and second, evidence should exist that clinical (and/or pathological) changes are gluten-dependent, most often as an unequivocal response to a gluten-free diet. Pathological abnormalities of celiac disease may include severe ('flat') or variably severe (mild or moderate) small bowel mucosal architectural abnormalities that are associated with both epithelial cell and lymphoid cell changes, including intraepithelial lymphocytosis. Architectural changes tend to be most severe in the duodenum and proximal jejunum and less severe, or absent, in the ileum. These findings, while characteristic of celiac disease, are not specific because several other conditions can produce similar changes. Some serological assays (eg, tissue transglutaminase antibody assays) are very useful screening tools in clinical practice because of their high specificity and sensitivity, but these do not provide a definitive diagnosis. The most critical step in the diagnosis of celiac disease is the demonstration of its gluten-dependent nature. The clinical response to gluten restriction in celiac disease is usually reflected in the resolution of diarrhea and weight gain. Normalization of biopsy changes can be first shown in the most distal intestinal sites of involvement, and later, sometimes only after prolonged periods (months to years) in the duodenum. Rarely, recurrent (or refractory) celiac disease may occur after an initial gluten-free diet response. Finally, some with 'sprue-like intestinal disease' cannot be classified because a diet response fails to occur. This may be a heterogeneous group, although some are eventually found to have a malignant lymphoma.
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PMID:Pearls and pitfalls in the diagnosis of adult celiac disease. 1835 56

The cell population of the upper jejunal mucosa has been studied in cases of tropical sprue from the Far East and Middle East, and in similar cases arising in western Europe (;post-infective malabsorption'), and compared with cases of untreated coeliac disease and patients without small bowel disease.Infiltration of the epithelial layer of the upper jejunal mucosa by lymphocytes was found in tropical sprue to the same extent as in coeliac disease, and, to a lesser extent, in ;postinfective malabsorption'.In the lamina propria, in all forms of acute sprue there was an increased density of lymphocytes. With increasing duration and with increasing mucosal atrophy, the lymphocytes were progressively replaced by plasma cells, and the cellular infiltration in chronic sprue was indistinguishable from that of coeliac disease.The findings suggest that a humoral antibody response is a feature of sprue, and becomes more prominent as the condition becomes chronic.
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PMID:The cell population of the upper jejunal mucosa in tropical sprue and postinfective malabsorption. 1866 48

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