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Wheat starch intolerance in patients with celiac disease.

OBJECTIVE: Evaluate in patients with celiac disease the tolerance of prolonged consumption of small amounts of gliadin contained in products containing wheat starch. DESIGN: Open 1-year trial of the addition of wheat starch to a gluten-free diet in a cohort of adult patients with biopsy-proven celiac disease who had never consumed wheat starch. The control group consisted of patients with celiac disease who tolerated wheat starch. SUBJECTS: Seventeen patients with celiac disease and 14 control patients, all diagnosed according to criteria of the European Society of Pediatric Gastroenterology and Nutrition, were recruited from the Canadian Celiac Association and the Quebec Celiac Foundation. SETTING: The study was conducted in the outpatient clinic of the Gastroenterology and Nutrition Service of Ste Justine Hospital, Montreal, Quebec, Canada. INTERVENTIONS: Patients were asked to consume four to six portions daily of a wheat starch-containing product, mainly bread, for up to 1 year. MAIN OUTCOME MEASURES: The gliadin content of the wheat starch product used in this trial was quantified by enzyme-linked immunosorbent assay. Patient outcome measures included symptoms, nutritional parameters (anthropometric data, complete blood count, serum folate and iron levels), and immunologic parameters (antigliadin antibody and antiendomysium antibody titers). RESULTS: A quantifiable amount of immunoreactive gliadin (0.75 mg/100 g) was found in the wheat starch. The majority of the patients with celiac disease (11 of 17) who had never consumed wheat starch previously developed symptoms, which resolved within weeks of discontinuing the product. Relapse of skin lesions was seen in two of three patients with coexisting dermatitis herpetiformis. No weight loss or biochemical changes were observed. Despite the presence of symptoms, antigliadin antibody and antiendomysium antibody determinations were not useful to detect the clinical intolerance. APPLICATIONS: The innocuousness of the long-term ingestion of "gluten-free" products containing wheat starch is still unproven, and prolonged use of such products by patients with celiac disease cannot be recommended.

Adolescent↗

Why is celiac disease so common in Ireland?

Celiac disease (gluten sensitive enteropathy) is a condition affecting the small bowel, characterized by permanent intolerance to dietary gluten, and giving rise to varying degrees of malabsorption and diarrhea. With the advent of sensitive screening tests, the condition is being increasingly diagnosed. Celiac disease is more common in the Irish and in those of Irish descent. Simoons (1978, 1981) hypothesized that the present-day prevalence of celiac disease across Europe is related to the interaction between genetic gradients, largely determined by the advance of agriculture, and historical patterns of cereal ingestion. This essay examines Simoons' hypothesis as it relates to Ireland, reviews the ethnic and genetic mix of those living on the island of Ireland and aspects of Irish dietary history, and considers how these factors may have combined to result in a high frequency of celiac disease.

Celiac Disease↗

Celiac disease in children with autoimmune thyroid disease.

Ninety children and adolescents with autoimmune thyroid disease were screened for celiac disease. All 90 patients were typed for HLA antigen class I and II and for HLA-DQA1 and DQB1 heterodimers. Celiac disease and DQA1*0501, DQB1*02 were found in 7 (7.8%) patients. The prevalence of celiac disease was 1 of 13. Screening for celiac disease is recommended in children with autoimmune thyroid disease.

Adolescent↗

Thyroid disorders in Brazilian patients with celiac disease.

INTRODUCTION/AIM: Patients with celiac disease (CD) can develop a gluten related autoimmune disorder that affects not only the small intestine but other tissues as well. An increased prevalence of autoimmune diseases has been reported, particularly autoimmune thyroiditis. The aim of this study was to characterize thyroid disorders in patients with CD. PATIENTS/METHODS: Fifty-two patients with CD (43 female, 9 male; mean age, 41.1 years) were studied. Nine were on a gluten-free diet (GFD). They were divided into four groups: Group 1, without thyroid involvement (n=30); Groups 2A-C, with thyroid involvement (n=22); Group 2A, subclinical hypothyroidism (n=11); Group 2B, clinical hypothyroidism (n=10); and Group 2C, other thyroid disorders (n=1). CD was confirmed by serologic and histologic criteria. Thyroid involvement was detected by measurement of thyroid stimulating hormone (TSH) and anti-thyroperoxidase antibodies (anti-TPO). RESULTS: Increased levels of TSH and/or anti-TPO levels were detected in Groups 2A (21.1%) and 2B (19.2%). The patients of Group 2B presented clinical symptoms of hypothyroidism before the diagnosis of CD, and 5 of these patients were receiving levothyroxine. One woman (Group 2C; 1.92%) had a medullary carcinoma. There was statistical significance between the age when thyroid disease was diagnosed (current age) and the age of CD diagnosis between Groups 1 and 2B. Patients with thyroid involvement presented associated diseases such as diabetes mellitus (2), Down's syndrome (2), ulcerative colitis (1), and dermatitis herpetiformis (2). CONCLUSIONS: Our findings demonstrated an increased prevalence of thyroid disorders (hypothyroidism, 19.2%; and subclinical hypothyroidism, 21.2%), and other associated diseases in celiac patients, even on a GFD, increasing with the age of the patients. Screening for associated diseases is recommended for patients with CD, independent of age at diagnosis or treatment duration.

Adolescent↗

Celiac disease in India.

Celiac disease is well documented especially from northern parts of India. It should be suspected in a child having chronic diarrhoea with growth failure. There are unusual manifestations like refractory anemia, rickets, recurrent abdominal pain and short stature without other gastrointestinal manifestations. Definitive diagnosis still requires undertaking multiple intestinal biopsies, before and after wheat withdrawal from the diet. Additional difficulty in diagnosing celiac in tropical countries like India includes confounding factors of malnutrition and parasitic infestations like giardia. Serological tests like antigliadin antibodies (IgG and IgA), anti-reticulin antibodies and endomysial antibodies have shown some promise in screening and definitive evaluation of the disease. Gluten free diet gives dramatic results.

Age Distribution↗

The prevalence of manifest and latent celiac disease in type 1 diabetes mellitus.

BACKGROUND/AIMS: Celiac disease and type 1 diabetes mellitus are both autoimmune diseases which have a common genetic predisposition. The aim of this study was to determine the prevalence of manifest and latent celiac disease in type 1 diabetic patients. METHODS: Anti-endomysium IgA was tested by indirect immunofluorescence using sections of human umbilical cord for screening in 100 adult patients with type 1 diabetes mellitus and in 80 age and sex matched controls with no known disease. Distal duodenal biopsy, human leukocyte antigen typing, urinary D-xylose excretion test, stool analysis, biochemistry profile, blood counts, serum ferritin level and small intestinal radiography were performed in anti-endomysium IgA positive cases. Small bowel biopsy specimens consistent with celiac disease were defined as manifest celiac disease, while positive antiendomysium IgA and normal intestinal histology with the presence of human leukocyte antigen class II antigens consistent with the disease were defined as latent celiac disease. RESULTS: Anti-endomysium IgA was positive in eight diabetic patients, while it was negative in all controls. Celiac disease was found in a total of six (6%) patients, four with manifest and two with latent disease. Only one patient had symptoms. CONCLUSIONS: The prevalence of celiac disease is increased in patients with type 1 diabetes mellitus. Since many patients may be asymptomatic, it is suggested that all diabetic patients should be screened for this disease.

Journal Article↗

[Celiac disease and lymphoma].

Celiac disease (CD) has been acknowledge as being responsible for numerous secondary pathologies, in particular autoimmune and neoplastic diseases. Whether CD is more prevalent in patients with non-Hodgkin's lymphoma (NHL) than in the normal population is not known. Accordingly, we carried out a study of 86 patients hospitalized in the Section of Oncology, Haematology and Internal Medicine of the Department of Medical, Oncological and Radiological Sciences of the University of Modena and Reggio Emilia and who, between 1988 and 1995 had been diagnosed as affected by NHL. On diagnosis, and before the beginning of antitumour therapy, all the patients were tested for antigliadin (AGA IgA and IgG) and antiendomysium (EMA) antibodies together with total class IgA antibody levels. Our findings showed that none of the 86 patients had an IgA deficit, while one tested positive for AGA IgA (43.9% v.n. < 7.5). The same patient also tested positive for EMA. The extremely high sensitivity and specificity of the AGA IgA and EMA led us to conclude that the patient was affected by CD, although his early death precluded confirmation by biopsy. The presence of one celiac patient among 86 NHL patients examined at the onset of the disease would suggest that CD is not infrequent in NHL. The numbers involved in our study are insufficient for statistical purposes, and we are therefore awaiting the results of a SIGEP multi-centre study into the connection between CD and lymphomas.

Adolescent↗

Gluten-free diet and quality of life in patients with screen-detected celiac disease.

CONTEXT: Since the advent of serologic testing for celiac disease, most persons who receive a diagnosis of celiac disease have few or no symptoms. Although pathologic changes of celiac disease resolve on a gluten-free diet, how a gluten-free diet affects the quality of life for patients with screen-detected celiac disease is unclear. OBJECTIVE: To evaluate the effect of a gluten-free diet on the quality of life of patients with screen-detected celiac disease. DESIGN: Prospective study of patients before and 1 year after initiating a gluten-free diet. PARTICIPANTS: 19 patients with screen-detected celiac disease (found by serologically testing first-degree relatives of celiac patients) and 21 consecutive patients with symptom-detected disease. In all cases, celiac diagnosis was confirmed by finding villous atrophy and crypt hyperplasia on small-bowel biopsy. INTERVENTION: Gluten-free diet (explained during a single physician visit). MAIN OUTCOME MEASURES: Gastrointestinal Symptoms Rating Scale (GSRS), in which scores range from 0 to 6 (higher scores represent worse symptoms); and quality of life measured with the Psychological General Well-Being Questionnaire (PGWB). Scores range from 22 to 132 (higher scores mean greater well-being). RESULTS: At baseline, patients with symptom-detected celiac disease had poorer quality of life and more gastrointestinal symptoms than those with screen-detected celiac disease. Reported compliance with the gluten-free diet was good. All mucosal lesions of the small bowel had resolved at the follow-up biopsy. After 1 year of following the diet, quality of life for patients with screen-detected disease significantly improved (mean PGWB score increased from 108 to 114; P <0.01). A similar increase was noted in patients with symptom-detected disease (mean PGWB score increased from 92 to 103; P <0.01). Gastrointestinal symptoms also improved in patients with screen-detected disease and in patients with symptom-detected disease (mean GSRS scores decreased from 1.8 to 1.4 and from 2.6 to 1.9, respectively; P <0.01 for both comparisons). CONCLUSIONS: Gluten-free diet was associated with improved quality of life for patients with symptom-detected celiac disease and patients with screen-detected celiac disease. Concerns about the burden of a gluten-free diet, at least over the short term, may be unfounded.

Adult↗

Celiac disease in patients having recurrent aphthous stomatitis.

BACKGROUND/AIMS: Celiac disease is a condition related to the small intestine's intolerance to gluten. In epidemiologic studies the prevalence is highly variable. The diagnosis can be difficult due to the wide spectrum of signs and symptoms. As the risk for intestinal lymphoma is higher in these patients, early diagnosis has its privileges. The higher prevalence of recurrent aphthous stomatitis in celiac disease led us to investigate the celiac disease prevalence in patients with recurrent aphthous stomatitis, which might assist in diagnosis of asymptomatic celiac disease patients. The aim of this study was to determine the prevalence of celiac disease in patients presenting with recurrent aphthous stomatitis. METHODS: The study group consisted of patients having a history of recurrent aphthous stomatitis. The control group included patients not having aphthous stomatitis. Antibodies to gliadin IgG and IgA and antibodies to endomysium were determined from the serum samples of all patients. Biopsies were obtained from the distal part of the duodenum. RESULTS: Biopsies of two patients (4.8%) out of 41 belonging to the study group were diagnosed as celiac disease. In serum samples of both, antibodies to gliadin IgA and antibodies to endomysium were found to be positive. Antibodies to gliadin IgG antibody were positive in only one of these two patients. None of the 49 patients in the control group was diagnosed as celiac disease. CONCLUSION: Further evaluation of recurrent aphthous stomatitis patients for celiac disease must be performed. As the endoscopic procedures are invasive and costly, evaluation of recurrent aphthous stomatitis patients for celiac disease must include serologic markers at the beginning. If any positivity is determined in markers, then endoscopic procedures including biopsies of the duodenum must be considered as the second-step intervention.

Adult↗

[Clinical picture of celiac disease in children].

The celiac disease was first described over one hundred years ago. English doctor S. Gee selected a syndrome characterized by growth inhibition, diarrheas and malabsorption disorders among the group of enteropathies in children in 1888. He assumed that the large intestine was affected in such cases. That is the reason why this disease was called celiac affection. C. Herter described a similar disease in 1908 and called it intestinal infantilism. Only as early as in 1950 Dutch pediatrician W. Dicke described the inability to uptake gluten (a component found in some herbs) as an important reason for chronic intestinal disease in children for the first time. Gluten enteropathy, intestinal infantilism, gluten intolerance, idiopathic steatorrhea, non-tropical sprue and Gee-Heubner-Herter disease are synonyms for celiac affection.

Celiac Disease↗

The immunologic basis for celiac disease and related disorders.

Celiac disease is the classical food-sensitive enteropathy and is caused by an antigen-specific immunologic hypersensitivity response to gluten within the small intestinal mucosa. Antibody and T-cell hypersensitivity may play a role in vivo, but it is more likely that the primary problem is an inappropriate T-cell response to gluten. The deleterious effects of gluten in celiac patients can be completely explained by the immune-mediated alterations in upper small-intestinal mucosal shape; these alterations include the replacement of long villi and short crypts with short or absent villi and hyperplastic crypts. It is an extremely unusual disease in that it is highly linked to human leukocyte antigen (HLA)-DQ2, and CD4+ T-cell clones that recognize gluten in the context of DQ2 have been isolated from celiac mucosa lamina propria. HLA-DQ2 is commonly used in Whites, but the incidence of celiac disease varies greatly between different countries and with time, without any obvious explanation. Celiac disease is also unusual in that it has features of autoimmune disease. Patients with active disease have immunoglobulin (Ig)A antibodies in endomysium, an uncharacterized extracellular matrix protein, and the presence of these antibodies is very specific for this condition. However, because celiac disease is more common in IgA-deficient patients, the IgA antiendomysial antibodies are unlikely to be pathogenic.

CD4 Antigens↗

Bone mass and metabolism in patients with celiac disease.

BACKGROUND & AIMS: Several aspects of the pathogenesis of osteopenia in celiac disease are still unclear. Therefore, bone mass and metabolism were evaluated in adults with celiac disease in a cross-sectional study. METHODS: Bone mineral density (BMD), assessed by total body dual-photon absorptiometry, and serum indices of bone metabolism and remodeling were evaluated in 17 patients with untreated celiac disease, 14 with celiac disease on a gluten-free diet, and 24 healthy volunteers. RESULTS: BMD, expressed as a z score, was significantly lower in patients with untreated celiac disease than in patients with treated celiac disease and volunteers and lower in patients with treated celiac disease than in volunteers. Similar changes were observed in serum calcium level, whereas intact parathyroid hormone level was significantly higher in untreated than in treated patients with celiac disease and volunteers, and no difference was found between the latter two groups. 25-Vitamin D level was significantly lower and 1,25-vitamin D level significantly higher in untreated celiac disease than in treated celiac disease and volunteers. Indices of bone remodeling were significantly higher in untreated than in treated patients and volunteers and significantly and positively correlated with iPTH in untreated patients with celiac disease. CONCLUSIONS: BMD is almost invariably low in patients with untreated celiac disease. Results in treated patients suggest that gluten-free diet improves but does not normalize BMD. Untreated celiac disease is characterized by high levels of 1,25-vitamin D and by increased bone turnover, caused by the increase in intact parathyroid hormone level.

Absorptiometry, Photon↗

Celiac disease.

Timely diagnosis and treatment of celiac disease is important not only to improve the immediate quality of life of the patient but also to decrease the long-term risks of untreated celiac disease. A large Finnish study showed that the 5-year survival among patients who strictly adhered to a gluten-free diet was similar to that of the general population. Growth and development in infants and children proceed normally with continued gluten avoidance, and in adults many of the disease complications including osteopenia are avoided. However, peripheral neuropathy, ataxia, and severe osteopenia, particularly in the setting of secondary hyperparathyroidism, usually persist. Enteropathyassociated T-cell lymphoma is widely recognized as a complication of celiac disease, and gluten restriction has been shown to significantly decrease the risk of this malignancy to the level of the general population. Whether gluten restriction is beneficial or should be recommended for patients with asymptomatic disease remains controversial. However, the available evidence suggests that this treatment is always indicated in patients showing celiac enteropathy, at least to prevent the possible long-term complications of this condition. Despite a dearth of evidence presently to support population-wide screening for celiac disease, patients at high-risk for celiac disease should be screened based on symptoms, family history, and associated conditions, as morbidity from subclinical disease in young patients has been demonstrated.

Algorithms↗

Bone loss in celiac disease is related to secondary hyperparathyroidism.

Celiac disease is a major cause of intestinal malabsorption. Previous studies have demonstrated that celiac disease is associated with significant osteoporotic bone loss. These studies have suggested that successful treatment of the malabsorption is associated with amelioration of the bone loss. Such studies have failed to examine bone mass at peripheral skeletal sites which is more likely to be responsive to changes in parathyroid hormone (PTH) in response to calcium malabsorption. We have examined bone density in the lumbar spine, femoral neck, and distal forearm in 35 patients with celiac disease who had been established on gluten-free diet. In addition, the concentrations of PTH and 1,25-dihydroxyvitamin D (1,25(OH)2D) were measured. Bone density was below that expected for the subject's age and gender at all sites. This was most marked in the distal forearm where the bone density was 1.40 SD below expected (p < 0.0001). In the forearm, there was a negative relationship between bone density and PTH concentration (r = -0.49, p = 0.009). In the forearm and lumbar spine, there was a negative relationship between 1,25(OH)2D concentration and bone density. Bone mass was not related to the concentration of 25-hydroxyvitamin D at any of the skeletal sites measured. Bone density is reduced in the peripheral skeleton in celiac disease and this deficit persists despite treatment with apparent normalization at axial skeletal sites. This reduction in bone mass is related to the presence of secondary hyperparathyroidism which should be sought in all patients with treated celiac disease.

Adult↗

Prevalence and clinical characteristics of celiac disease in Downs syndrome in a US study.

Celiac disease is an autoimmune gastrointestinal disorder characterized by mucosal atrophy of the jejunum on exposure to gluten, a protein found in grains. The purpose of our study was to determine the prevalence of celiac disease in children with Downs syndrome in a U.S.-based Caucasian population. The 97 Downs syndrome children were screened for celiac disease using serum IgA-anti-endomysial antibody testing, which is highly specific and sensitive for the disorder. Children with titers greater than 1:5 (using the IgA endomysial antibody [EMA] test; EMA+) were considered affected. Ten children (10.3%) were EMA+. We examined their HLA DQA1 DQB1 genotype, karyotype, clinical characteristics, and the prevalence of celiac disease in their first-degree relatives. The nine available karyotypes were trisomy 21. Downs syndrome-specific mean height percentile was 64%+/-26% (range <5-99%) and weight percentile was 43%+/-28% (range 5-95%). Presence of diarrhea, constipation, vomiting, and abdominal pain was similar for children with and without celiac disease. Only bloating symptoms were significantly more frequent in those with celiac disease (EMA+). Seven of eight (88%) genotyped EMA+ children had the celiac disease-associated high-risk HLA DQA1*0501 DQB1*0201 genotype as compared with 13/ 80 (16%) of EMA- children. Five of 48 (10%) first-degree relatives of the celiac disease (EMA+) children were EMA+. In conclusion, celiac disease, as diagnosed by positive endomysial antibody tests, has an increased prevalence in children with Downs syndrome in the U.S. as compared with the general population (1/250). Clinical and growth characteristics do not distinguish between children with and without celiac disease. Based on these observations, it is recommended that children with Downs syndrome be screened for celiac disease.

Adolescent↗

Serological testing in screening for adult celiac disease.

Assays for celiac-related antibodies are becoming widely available, and the present review aims to clarify the use of these investigations in the diagnosis of, management of and screening for adult celiac disease. The sensitivities and specificities of various antibody tests are discussed, along with their clinical use as an adjunct to small bowel biopsy, and as a first-line investigation for patients with atypical symptoms of celiac disease or patients at high risk of developing sprue.

Adult↗

Propeptide of type I procollagen is predictive of posttreatment bone mass gain in adult celiac disease.

BACKGROUND & AIMS: Adult celiac disease is associated with osteopenia, which is not always reversible after gluten-free diet (GFD). A prospective study was conducted to evaluate whether pretreatment indices of bone and mineral metabolism are predictive of the extent of bone mass gain after diet. METHODS: Lumbar and femoral bone mineral density (z-score) and serum levels of parathyroid hormone, 1,25-dihydroxycholecalciferol, COOH-terminal propeptide of type I procollagen (PICP), and COOH-terminal telopeptide of type I collagen (ICTP) were measured in 20 celiac patients at diagnosis and after 2 years of GFD. RESULTS: All patients showed a posttreatment improvement in bone mass and in serum levels of indices of bone and mineral metabolism. Nevertheless, only in 12 of 20 patients was this improvement at least equal to half the SD of the z-score, which equals a gain of at least 5% in bone mass. Pretreatment levels of PICP strictly correlated with the increase in lumbar (r(s) = 0.92; P < 0.001) and femoral z-scores (r(s) = 0.89; P < 0.001). Only in patients with basal PICP above the normal range did the z-score increase after GFD by at least half the SD. CONCLUSIONS: In adult celiac disease, a high rate of osteosynthetic activity before treatment is predictive of the satisfactory recovery of bone mass after GFD.

Absorptiometry, Photon↗