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Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet.

AIM: To determine the fasting plasma carnitine ester in patients with celiac disease. METHODS: We determined the fasting plasma carnitine ester profile using ESI triple quadrupol mass spectrometry in 33 adult patients with biopsy-confirmed maturity onset celiac disease maintained on long term gluten free diet. RESULTS: The level of free carnitine did not differ as the celiac disease patients were compared with the healthy controls, whereas the acetylcarnitine level was markedly reduced (4.703 +/- 0.205 vs 10.227 +/-0.368 nmol/mL, P<0.01). The level of propionylcarnitine was 61.5%, butyrylcarnitine 56.9%, hexanoylcarnitine 75%, octanoylcarnitine 71.1%, octenoylcarnitine 52.1%, decanoylcarnitine 73.1%, cecenoylcarnitine 58.3%, lauroylcarnitine 61.5%, miristoylcarnitine 66.7%, miristoleylcarnitine 62.5% and oleylcarnitine 81.1% in the celiac disease patients compared to the control values, respectively (P<0.01). CONCLUSION: The marked decrease of circulating acetylcarnitine with 50-80 % decrease of 11 other carnitine esters shows that the carnitine ester metabolism can be influenced even in clinically asymptomatic and well being adult celiac disease patients, and gluten withdrawal alone does not necessarily normalize all elements of the disturbed carnitine homeostasis.

Adult↗

[Physico-chemical and structural changes gliadin and glutenin components of different qualities of gluten].

By gel-chromatography on Sephadex and electrophoresis in polyacrylamide gel the structure and composition of gliadin and glutenin compounds of gluten from the strong and weak varieties of Saratovskaya 29 and Akmolinskaya 1 wheats were investigated. It was shown that similar protein fractions of strong and week gluten differed in the content of certain amino acids, number of disulphide bonds, electrophoretic mobility, ratio of components made of one and several polypeptide chains, molecular weight of individual components and subunits, and the content of high molecular weight proteins.

Amino Acids↗

[Oats can be included in gluten-free diet].

In the management of coeliac disease, it has been widely accepted that oats must also be excluded from the diet, along with wheat, rye and barley. The article consists in a review of published reports, and an account of our experience of including oats in the gluten-free diets of adults. Oats were found to be safe and well tolerated by adults with coeliac disease and dermatitis herpetiformis, though the risk of wheat contamination of commercial oat products remains a cause of concern. Similar findings were reported from a study of adolescents, but no such studies have been made of small children. Thus, the inclusion of oats, known to be a fibre-rich, naturally gluten-free food, would broaden the range of foodstuffs tolerable to coeliac patients, though for safety reasons they should be used only by adults until more information is available.

Adult↗

An apparently gluten-induced photosensitivity in horses.

Primary photosensitization was observed in 3 Appaloosa mares. The skin lesions were diffuse erythema followed by edema and subsequently weeping and finally dry gangrene and ulceration. The severe photosensitivity dermatitis was apparently induced by gluten ingestion. Resolution of lesions occurred after withdrawal of the suspected dairy concentrate feed and prevention of exposure to sunlight. Neither the ponies nor donkey, which were not fed with the suspected concentrate, exhibited similar skin lesions or other clinical abnormalities. Gluten metabolites may contain photodynamic agents that cause photosensitization in horses.

Animals↗

Gluten-free diet improves iron-deficiency anaemia in patients with coeliac disease.

Two cases of newly-diagnosed asymptomatic coeliac disease with 3 years of unexplained severe iron-deficiency anaemia are presented. Oral iron supplementation had no effect on their serum iron levels and, therefore, had no influence on their anaemia. Upper gastrointestinal endoscopy confirmed normal macroscopic findings. Duodenal biopsies revealed subtotal villous atrophy of the mucosa of the small intestine. A strict gluten-free diet led to an increase in serum iron, resolution of anaemia, and restitution of normal mucosal morphology. Thus, severe iron-deficiency anaemia associated with asymptomatic coeliac disease is responsible to gluten-free diet.

Adult↗

[Influence of gluten free diet on bone mineral density (BMD) in children with celiac disease].

UNLABELLED: In children with celiac disease (CD) bone metabolism and mineralization are frequently disturbed. The present study aimed to assess the influence of gluten free diet (GFD) on bone mineral density (BMD) in 73 children with CD, mean age of 12.4 +/- 0.4 years and mean body mass index (BMI) 17.9 +/- 0.4 kg/m2 (mean +/- SEM). Diagnosis of CD was established according to ESPEGAN criteria. Compliance to the GFD was verified on the basis of interview and by estimation of antiendomysial antibodies (IgAEmA/IgGEmA) in blood serum. BMD was measured by dual energy X-ray absorptiometry (DEXA). Plasma calcium (Ca) and phosphorus (P) concentrations, alkaline phosphatase (AP) and its bone fraction (BAP) were estimated before BMD measurement. All children were divided into two groups. Group A consisted of 33 children where gluten free diet was strictly respected for 11.7 +/- 0.6 years. The second group (Group B) comprised 40 children without strictly respected GFD. Children who strictly followed GFD showed statistically higher BMI, AP-spine BMD and total body BMD in comparison with children without GFD (BMI 19 +/- 0.52 kg/m2 vs 17.3 +/- 0.4 kg/m2; p < 0.01, AP-spine BMD 0.951 +/- 0.04 g/cm2 vs 0.767 + 0.03 g/cm2; p < 0.005, Total Body BMD 1.013 +/- 0.02 g/cm2 vs 0.933 +/- 0.01 g/cm2; p < 0.05) respectively. No significant differences were found in plasma Ca, P, AP, BAP between both groups. A statistically significant positive correlation (p < 0.001) was found between duration of GFD and AP-spine BMD and total body BMD, respectively. A statistically significant positive correlation (p < 0.05) was also found between duration of GFD and BMI. CONCLUSION: Long-term GFD significantly improves BMD and BMI in children with CD.

Absorptiometry, Photon↗

Selective immunoglobulin a deficiency, ulcerative colitis, and gluten-sensitive enteropathy--a unique association.

A patient with selective immunoglobulin A deficiency, severe ulcerative colitis, and malabsorption had a flat jejunal mucosa demonstrated by peroral biopsy. Treatment at different times with a gluten-free diet for the jejunal lesion and corticosteroids for the ulcerative colitis, led to improvement of the malabsorption. A repeat jejunal biopsy demonstrated histological improvement of the jejunal mucosa, even though the colitis remained active. The occurrence of immunoglobulin A deficiency in a patient with ulcerative colitis and gluten-sensitive enteropathy is uncommon.

Adrenal Cortex Hormones↗

[Rheological properties of wheat gluten and their relation to molecular parameters].

In the light of the general results of protein chemistry and of the interrelationships up to now established, it is stated that the factors which determine the mechanical properties are complex in nature. Though the amino-acid composition (cystine content, amino acids with hydrophobic side-chains) and the ratio of the wheat gluten fractions (ratio of high molecular protein components to low-molecular ones) exert some effect on the rheological properties, the linkages and the interactions between the different fractions play the main role in the development of the mechanical properties of the gluten. Among the various linkages and interactions which occur in protein architecture, the disulphide linkages, the hydrogen bonds (amido group, primary amino group) and the hydrophobic interactions (side-chains of proline, leucine, isoleucine, glutamine, etc.) are of great importance. The complex evaluation of data about the content of disulphide linkages, the degree of amidation of the glutamic and aspartic acids and the amount of amino acids with hydrophobic side-chain gives a significant insight which might be of practical value.

Amides↗

[Reduction dynamics of S-S bonds in wheat gluten].

The new method of S-S bond determination was elaborated. It was based on the reduction of S-S bonds using 2-mercaptoethanol/ME/, the separation of the excess of ME using the Sephadex column and the application of ELLMAN's reagent for the determination of reduced SH groups. Using this method much higher contents of total S-S bonds, as well as of intermolecular ones could be observed, than by using Na2SO3 as a reducing agent. During the experiments on the reductivity of intermolecular S-S bonds in all investigated samples of gluten under the action of ME initially the increase of SH groups and then some steady state period could be noticed, in which no increase occurred, followed by further reduction. This means, that the one more type of S-S bonds, more accessible for the reducing agent of all may exist. It is suggested, that it can be the quaternary S-S bonds joining together separate molecules to form complexes able to disperse. As the observed "plateau" representing those bonds occurs at different level of the reduction percent and at the different time, after the reduction have been started, does the amount and strength of those bonds not influence the gluten quality.

Disulfides↗

[Molecular mechanisms behind the immunological reaction against gluten in patients with celiac disease].

Coeliac disease is a complex inflammatory disorder of the small intestine, induced by dietary gluten in genetically susceptible individuals. Recently, the nature of gluten-derived peptides recognized by gut-derived T cells from patients has been elucidated. It has further been established that the binding of such peptides to HLA-DQ2 and DQ8 molecules is enhanced following the modification of the peptides by the ubiquitous enzyme tissue transglutaminase. This knowledge has allowed the establishment of a molecular basis for the well-known association between coeliac disease and the expression of these HLA-molecules. This article summarizes the latest progress in coeliac disease molecular biology.

Animals↗

Collagenous enterocolitis: a manifestation of gluten-sensitive enteropathy.

We report coexistent collagenous colitis and collagenous sprue in a 62-year-old woman with diarrhea. Investigations suggested malabsorption, and small intestinal biopsies demonstrated a flattened mucosa with subepithelial collagen deposition. Colonic biopsies also showed a thickened subepithelial collagen band as well as a striking lamina propria inflammatory cell infiltrate. Symptomatic remission was induced with a gluten/lactose-free diet, oral prednisone, and sulfasalazine and has been maintained with gluten restriction alone. Repeat biopsies after 2 months demonstrated restoration of normal small intestinal and colonic collagen bands; only a chronic inflammatory cell infiltrate (consistent with microscopic/lymphocytic colitis) persisted in colonic biopsies. We propose that, in this instance, collagenous enterocolitis represented a diffuse manifestation of gluten sensitivity.

Celiac Disease↗

Gluten determination by gliadin enzyme-linked immunosorbent assay kit: interlaboratory study.

An interlaboratory study with 10 participants was performed to obtain validation and performance data for an enzyme-linked immunosorbent assay (ELISA) kit developed for quantitative gluten determination in foods. The ELISA kit used for this study is based on 2 monoclonal and 1 polyclonal antibody developed by Immunotech, a Beckman Coulter Co. This kit did not show any false positive results or cross-reactivity with oat, rice, maize, and buckwheat. The gliadin standard from the Working Group on Prolamin Analysis and Toxicity was included in the kit as reference material for calibration. All participants obtained a gliadin ELISA kit with Standard Operational Procedure and a form for recording test results. The study included 13 samples labeled as "gluten-free" and 2 samples spiked by wheat flour. Seven samples had gliadin content below the limit of quantitation (LOQ) of the method, and 1 sample exceeded the highest calibration level. Gliadin content in the range from 10 to 157 mg/kg (1st day) and from 11 to 183 mg/kg (2nd day) was found in 7 samples (including 2 spiked samples). Results of these samples were used for further statistical analysis and evaluation. The Cochran, Dixon, and Mandel statistical tests were applied for detection of outliers. The LOQ of the kit was estimated.

Avena↗

Determination of the gluten content of foods.

Coeliac disease affects 1:1,500 people in Europe. Treatment of the condition involves a gluten-free diet with avoidance of foods containing wheat, rye, barley or oats. Evidence suggests that individuals should adhere strictly to the diet as this reduces the increased incidence of malignancy observed in the condition. A large number of gluten-free products are currently available. Immunological assays have been developed to assess their suitability for patients with coeliac disease. Quantitative assays, based on both polyclonal antisera and monoclonal antibodies, methods of extraction and problems associated with interfering substances are reviewed in this paper.

Celiac Disease↗

[Hyposplenism in celiac disease. Role of a gluten-free diet].

Hyposplenism is a common complication of adult celiac disease. A study of splenic function in 17 adult patients with celiac disease was performed both before and during treatment with a gluten-free diet. Splenic hypofunction was assessed evaluating morphological changes in red cells (Howell-Jolly bodies, spherocytosis, achantocytosis). The hyposplenic changes were compared with: age of patients, duration of symptoms previous to diagnosis, severity of the clinical picture and histological response to treatment. We concluded that 82.4% of patients had hyposplenic changes and that there was a non significant tendency to improve such changes by a strict adherence to gluten-free diet.

Adolescent↗

The determination of prolamins in gluten-free food. Introductory remarks.

This paper gives the considerations on the Codex Alimentarius Standard on gluten free food and the levels that are proposed as a new standard. Background for the level of tolerance for gliadin in coeliac patients is given as well as the arguments for the choice of an enzyme immunochemical method for the determination of gliadin in gluten free food.

Dietary Proteins↗

[Celiac disease (gluten sensitive enteropathy)].

The frequency in the Austrian population of coeliac disease is approximately 1 in 1000 live births. The diagnosis is easy in most cases, if not obscured by an inconsiderate and inconsequent approach. The most frequent mistake is prescribing gluten free diet on trial before proving the diagnosis by small bowel biopsy. Failure to thrive, deficiency states and abnormal stools are the leading symptoms of coeliac disease. Careful inquiries and drawing of curves for length and weight usually give more information than expensive laboratory tests. Symptoms are frequently equivocal in relapses, in older children, and especially in adults. The basic prerequisite for the diagnosis of coeliac disease is the finding of subtotal villous atrophy in the small bowel mucosa. The only absolute contraindication against small bowel suction biopsy is a blood clotting defect. The small inconvenience from this procedure can be further reduced by experience and discipline of the examiner. Small bowel biopsies are performed in many paediatric hospitals. Thus, there is no excuse for omitting or delaying this procedure. Antigliadin antibodies and antiendomysial antibodies are of high diagnostic significance. The respective IgA antibodies show a high specificity, IgG antibodies a very high sensitivity. Thus, the decision for or against small bowel biopsy is made easier in doubtful cases, the evidence by villous atrophy for coeliac disease is approaching absolute proof. Gluten loading tests and multiple biopsies are therefore necessary only in rare cases. The very high frequency of certain HLA alleles in coeliac patients may also be used for diagnostic purposes. The pathogenesis of coeliac disease appears to involve genetic, immunologic, and environmental factors. The contribution of each of these factors may vary considerably in various patients.

Biopsy↗

[Two cases of gluten intolerance presenting as dwarfism (author's transl)].

Two patients with dwarfism were found to have intolerance to gluten. The authors describe the clinical, biological and radiological signs which enable the growth disorder to be related to its true cause, when obvious signs of a celiac syndrome are lacking. In fact, a small intestine biopsy is the essential diagnostic procedure. The hormonal changes found in such patients are discussed, and include normal HGH levels, reduced somatomedin activity which becomes normal after a gluten-free diet, and low blood insulin levels in the basal state as in insulin tolerance tests.

Adolescent↗

[Significance of anti-gluten IgA levels for detection and monitoring of celiac disease].

Diagnosis of coeliac disease rests in jejunal biopsy. This is recognised as a traumatic examination which today may perhaps be replaced by a biological test produced by Pharmacia in which the anti-gluten IgA is measured by the ELISA method. The sensitivity of the test reaches 100% and the specificity to 96%. This biological test makes it possible to follow the development of the coeliac illness under diet, since the anti-gluten IgA diminishes significantly, often to the normal level.

Adult↗