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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic non hereditary subepidermal blistering diseases are in childhood and present mainly problems on its clinical and histological diagnosis. Immunopathologic, immunogenetic and ultrastructural studies play a definitive role in the classification of this group of entities. In this paper we report and comment seven children affected of dermatitis herpetiformis. The clinical diagnosis was assessed by direct immunofluorescence studies of the skin, which showed granular Ig A deposits at the MBZ. In five out of our seven patients we were able to demonstrate by clinic and laboratory malabsorption syndrome. A bibliographic review of the subject is carried out, stressing specially the differential aspects with other subepidermal blistering disease of the childhood (i. e. bullous pemphigoid and chronic benign blistering disease of the childhood) as well as some other acute and chronic infantile blistering entities.
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PMID:[Juvenile Duhring's disease. Apropos of 7 cases]. 676 39

An 18-year-old-woman, with dermatitis herpetiformis, acute glomerulonephritis, malabsorption and villous atrophy due to massive infiltration of IgA producing plasma cells was studied. By light microscopy, her renal biopsy specimen showed heavy immunofluorescence for IgA, a mixed proliferative and membranous lesion with occasional crescents and focal sclerosis. Electron microscopic examination revealed three main lesions: (1) swelling and bleb formation in endothelial cells, (2) extensive fusion of foot processes of podocytes, and (3) a dense quasi-linear, continuous deposition of immune complexes, encompassing several loops; they were characteristically located within the basement membrane at the boundary between the lamina densa and the lamina rara interna. At occasional points where the immune complexes had reached the outer aspects of the basement membrane, there was damage to the podocyte cytoplasm. This electron microscopic aspect supports the interpretation that these immune complexes, although non-complement fixing, exhibit a high damaging potential, leading to relentless disease progression.
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PMID:IgA pseudo-linear deposits in glomerular basement membranes in dermatitis herpetiformis. 705 9

It has been established that considerable blast-transformation and mitotic activity occurs among epithelial lymphocytes of untreated coeliac mucosa. This paper is concerned solely with the proliferative activity of epithelial lymphocytes (expressed as percentage "mitotic index") in the prospective diagnosis of coeliac disease, in comparison with other conditions such as lymphoma. Crohn's disease and immunodeficiency which are often associated with malabsorption and flattening of jejunal mucosa. The results demonstrate that a high mitotic index (greater than 0.2%) clearly distinguishes, and hence predicts, gluten-associated enteropathies (including dermatitis herpetiformis and malignant histiocytosis) from others in which gluten plays no aetiological role and where the mitotic index differs insignificantly from normal control mucosae (much less than 0.2%). Furthermore, it has been demonstrated that the mitotic index is raised in so-called "non-responsive coeliacs," thus suggesting that such patients may also be gluten-sensitive despite their subsequent failure to respond morphologically to dietary gluten restriction.
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PMID:Studies of intestinal lymphoid tissue. IV--The predictive value of raised mitotic indices among jejunal epithelial lymphocytes in the diagnosis of gluten-sensitive enteropathy. 708 95

The clinical heterogeneity of celiac disease is well recognized, with great variability in malabsorption and in gastrointestinal symptoms of patients with active celiac disease, and the fairly common occurrence of silent celiac disease (ie, an apparently healthy individual with a flat small bowel mucosa). Research in experimental animals has shown that expression of cell-mediated immunity in the gut mucosa occurs in a spectrum of pathologies ranging from minimal enteropathy with a high count of intra-epithelial lymphocytes to the celiac-like lesion with short villi and long crypts. A similar range of pathology is seen in celiac patients in some circumstances, with low grade enteropathy occurring in the early stages of gluten challenge, in patients taking a low gluten diet, and also in some patients with dermatitis herpetiformis. In so-called latent celiac disease, sophisticated immunologic studies may reveal a high intra-epithelial lymphocyte count, an increased gamma delta intra-epithelial lymphocyte count, and a celiac-like intestinal antibody abnormality with overexpression of mucosal IgM responses. If further work confirms that one or more of these features may be the only manifestation of gluten-sensitive disease in some newly presenting patients, the current definition of celiac disease (a flat mucosa, gluten-sensitive) will need to be revised.
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PMID:Spectrum of expression of intestinal cellular immunity: proposal for a change in diagnostic criteria of celiac disease. 832 9

The clinical spectrum of celiac disease has widened over the past decades. The condition is no longer a severe malabsorption syndrome. Instead, a typical celiac disease patient today has merely mild abdominal symptoms. Malabsorption can be subclinical or absent, and there is usually only moderate, if any, loss of weight. Simultaneously, the current prevalence has increased from 1:1,000 to 1:300 inhabitants, or even higher. Clinically silent celiac disease cases are being detected in increasing numbers since the introduction and widespread use of serologic screening tests. Symptoms of celiac disease can appear outside the intestine, a typical example being dermatitis herpetiformis. Gluten intolerance is no longer limited to overt villous atrophy. Inflammation without villous damage may be observed in genetically susceptible individuals. The term latent celiac disease is applied in situations where the patient has normal villous architecture while on a gluten-containing diet, but later develops small bowel villous atrophy compatible with celiac disease.
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PMID:Clinical features of celiac disease today. 1054 15

Dermatitis herpetiformis is a gluten-sensitive skin disease with intestinal lesions and malabsorption symptoms less severe than those found in celiac disease. While several studies have shown the occurrence of osteopenia in celiac disease, bone mass and metabolism have never before been evaluated in dermatitis herpetiformis. Therefore, in 16 untreated patients, 16 sex- and age-matched untreated celiac patients, and 16 sex- and age-matched healthy volunteers, lumbar and femoral bone mineral density were measured and bone and mineral metabolism and nutritional status were evaluated. All these parameters were significantly altered in the two groups of patients and although the degree of these alterations was milder in patients with dermatitis herpetiformis than in celiac patients, the presence of subtotal villous atrophy in patients with dermatitis herpetiformis was associated with the presence of more severe alterations. Bone mineral density was significantly correlated with nutritional status, and patients showing bone loss were characterized by a body mass index lower than 20. Alterations of bone mass and mineral metabolism complicate dermatitis herpetiformis when severe intestinal lesions coexist. A low nutritional status may be predictive of the presence of bone loss.
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PMID:Bone mass and metabolism in dermatitis herpetiformis. 1054 69

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

Life-long gluten-free diet is the established therapy for coeliac disease and dermatitis herpetiformis. Diet therapy allows the intestinal mucosa to recover, improves nutrient malabsorption, osteoporosis and a weakened general condition. A gluten-free diet is without wheat, rye and barley and related products. Oats tested free of contamination by other cereals has recently been allowed for adult coeliac patients, but concern still remains regarding its general safety. Gluten-free flour mixes contain more starch and less proteins, vitamins, minerals and fibre compared to regular flour. Recently some questions have been raised as to the nutritional quality of the gluten-free diet. Successful therapy with gluten-free diet requires motivation and dietary counselling, including knowledge of the nutritional value of foods, labelling of prepackaged foods and practical training in cooking. The local chapters of the Norwegian Coeliac Society are active partners, as are the clinical nutritionists that work in most major hospitals. The physician making the diagnosis has a duty to see to it that all coeliac patients get adequate dietary counselling and management.
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PMID:[Dietary treatment of coeliac disease and dermatitis herpetiformis]. 1471 19

Celiac disease or celiac sprue is predominantly a disease of the small intestine characterized by chronic malabsorption in genetically susceptible individuals who ingest grains containing gluten, such as wheat, barley, and rye. Although previously believed to be uncommon, celiac disease may be present in up to 1% of the general population. Celiac disease is associated frequently with iron deficiency anemia, dermatitis herpetiformis, selective IgA deficiency, thyroid disorders, diabetes mellitus, and various connective tissue disorders but is rarely associated with cardiomyopathy. We describe a patient with celiac disease associated with cardiomyopathy whose cardiac function improved substantially after treatment with a gluten-free diet. Cardiomyopathy associated with celiac disease is a serious and potentially lethal condition. However, with early diagnosis and treatment with a gluten-free diet, cardiomyopathy in patients with celiac disease may be completely reversible.
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PMID:Cardiomyopathy associated with celiac disease. 1588 37

Celiac disease, or gluten-sensitive enteropathy, is an immune-mediated disease of the small bowel that results in malabsorption. It classically presents with gastrointestinal symptoms including chronic diarrhea, weight loss, abdominal bloating and anorexia. It is becoming more frequently identified in asymptomatic patients with a diagnosis of deficiencies related to malabsorption of iron, folic acid, vitamin B12 and vitamin D. It is increasingly identified as a cause for early or refractory osteoporosis. Occasionally, celiac disease presents with cutaneous manifestations alone. Dermatitis herpetiformis is a well-recognized cutaneous manifestation of celiac disease. Other cutaneous manifestations include alopecia, angular stomatitis and aphthous ulcerations. Described here is a case of a 24-year-old woman who presented with intermittent urticaria and gastrointestinal complaints. She was found to have celiac disease on small-bowel biopsy. Both her gastrointestinal symptoms and urticaria resolved when she was put on a gluten-free diet, suggesting that her urticaria was a cutaneous manifestation of celiac disease.
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PMID:Chronic urticaria: a cutaneous manifestation of celiac disease. 1660 61


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