Classical Celiac Disease Research Articles

Prospective study of body mass index in patients with coeliac disease

Population screening suggests that coeliac disease is much commoner than previously supposed. The prevalence of biopsy proved classic coeliac disease, with subtotal or total villous atrophy of the small bowel,...

Identification of tissue transglutaminase as the autoantigen of celiac disease.

Celiac disease is characterized by small intestinal damage with loss of absorptive villi and hyperplasia of the crypts, typically leading to malabsorption. In addition to nutrient deficiencies, prolonged celiac disease is associated with an increased risk for malignancy, especially intestinal T-cell lymphoma. Celiac disease is precipitated by ingestion of the protein gliadin, a component of wheat gluten, and usually resolves on its withdrawal. Gliadin initiates mucosal damage which involves an immunological process in individuals with a genetic predisposition. However, the mechanism responsible for the small intestinal damage characteristic of celiac disease is still under debate. Small intestinal biopsy with the demonstration of a flat mucosa which is reversed on a gluten-free diet is considered the main approach for diagnosis of classical celiac disease. In addition, IgA antibodies against gliadin and endomysium, a structure of the smooth muscle connective tissue, are valuable tools for the detection of patients with celiac disease and for therapy control. Incidence rates of childhood celiac disease range from 1:300 in Western Ireland to 1:4700 in other European countries, and subclinical cases detected by serological screening revealed prevalences of 3.3 and 4 per 1000 in Italy and the USA, respectively. IgA antibodies to endomysium are particularly specific indicators of celiac disease, suggesting that this structure contains one or more target autoantigens that play a role in the pathogenesis of the disease. However, the identification of the endomysial autoantigen(s) has remained elusive. We identified tissue transglutaminase as the unknown endomysial autoantigen. Interestingly, gliadin is a preferred substrate for this enzyme, giving rise to novel antigenic epitopes.

Celiac Disease Assocaited with Primary Biliary Cirrhosis in a Coast Salish Native

A 41-year-old Coast Salish female was initially diagnosed with typical features of classical adult celiac disease. Clinical and pathological features of primary biliary cirrhosis were also present, along with a familial history of insulin-dependent diabetes. Later, childhood celiac disease was detected in a male first-degree relative with diabetes. These patients are the first reported natives in Canada with celiac disease, a disorder believed to be genetically based but dependent on environmental factors for its clinical expression. The recognition of a ‘new’ disease in the setting of an aboriginal population may reflect geographical and climatic factors that permitted subsistence of this culturally complex food-gathering society up until most recent historical times, followed by adaptation of this society to European-based agricultural methods, particularly wheat cultivation.

What will be the adult height of coeliac patients?

We studied three groups of patients with coeliac disease: group 1 = 95 adult patients with gastro-intestinal symptoms diagnosed after reaching adult height, 41 of whom had had symptoms during childhood; group 2 = 23 adult patients with classic coeliac disease treated before or during puberty; group 3 = 11 coeliac children with short stature and no gastro-intestinal symptoms diagnosed and treated before or during puberty. We evaluated the adult height in groups 1 and 2 and the growth during the first years of diet in group 3. Our study leads us to the following conclusions. Dieting leads to a modest increase (on average not more than 3 cm) of the final height of coeliac patients. Subjects with gastro-intestinal symptoms who have been treated before adulthood reach a mean height similar to the normal population and have a slightly better adult height than non-treated subjects. This difference seems to exist only in men and this might be related to puberty evolving more rapidly in women receiving treatment. Subjects without symptoms during childhood reach a normal final height even without treatment. In our patients, early treatment seemed to have no great effect on adult height.

Wanderings in the small intestine.

T HE small intestine is a fascinating = o r g a n through which to wander. It combines digestive, absorptive, metabolic and endocrine functions. Areas that are morphological ly similar are functfonally quite different for example, bile acids and vitamin B,= are selectively absorbed in the terminal few feet of the ileum, but not from the structurally similar mid-ileum. To review the funcbons of the smal l intestine [s not the purpose of this article, but some concentra6on on aspects of work on the small intestine of particular interest to the group working on disorders of this organ in University Colrege, Galway is attempted. The clinical effects of cys5nuria are due to the formation of renal calculi because of a defective renal tubular reabsorption of cystine. Ornithine, lysine and arginine are also poorly re-absorbed in the renal tubules. Milne et al. (1961) first demonstrated that malabsorption of some of these amino acids was present in cystinurir patients. In 1964 it was possible to demonstrate that the active transport system of these amine acids in the small intestine of two cystinuric patients was absent (McCarthy et al., 1984). Mult iple intestinal biopsies from these patients were incubated with dilute solutions of amino acids and at the end of a period of incubation the level of the amino acids in the intestinal tissue water was no higher than that in the incubation fluid. No histological abnormality was present ~n the cystinuric mucosa Intestinal tissue from non-cystinuric patients concentrated the amino acids six to eight times. Major structural differences do not exist between the proximal and minor differences are apparent and in some instances correlate with functional dissimilarities. The villi of the proximal small intestine of the golden hamster are long and slender compared to the shorter villi of the distal intestine, and the proximal intestine is more effective to the esterification of fat. Comparison of fatty acid esterification by vill i of different shape in the hamster, rat, guinea pig and pigeon also show this correlation of function and structure (McCarthy and Tyor, 1964). Coeliac disease is a common disorder of the small intestine. Described by Gee in 1585, it was not unti l more than 60 years later that Paulley (1954) demonstrated that the malabsorption in the disease is due to gross damage to the mucosa. This description was some years after Dicke's (1950) major breakthrough on the role of gluten in the disease. For the practical management of patients, this disorder may be described as a condition in which the small intestinar rnucosa is significantly abnormal and in which improvement occurs when gluten is excluded from the diet. Difficulties occur when this definition is applied to indNidual patients. A patient with an initial response to a gluten free diet and a return towards normal of the intestinal mucosal morphology wil l certainly be considered as having classical coeliac disease. When such patient is given gluten with resultant deterioration in mucosal morphology, and who then fails to improve again when g~uten is removed from the diet is difficult to classify. Initially classification as a coe[iac is cerdistal parts of the small intestine, but tainly correct but what of later on ? What