Incidence of coeliac disease and transient gluten intolerance in children in a Swedish urban community.

The incidence of coeliac disease in children in the city of Malm?, South Sweden, was 1 : 982 during 1966 to 1975. The diagnostic criteria were: flat intestinal mucosa on gluten-containing diet, free of symptoms, and improvement in mucosal morphology on gluten-free diet, and morphological and/or evident clinical relapse (three times) on gluten challenge. 6 (12%) of 49 children with initially a flat mucosa still had a normal mucosa on a gluten-containing diet for two years or longer, having so-called transient gluten intolerance.     

Disaccharidases in coeliac disease

In 30 children presenting with complaints characteristic of malabsorption in whom congenital enzyme deficiency could be excluded, determination of the enzymes lactase, saccharase and maltase was performed in the tissue sample obtained by jejunal biopsy; histology was also carried out in all cases. In 23 cases the diagnosis of coeliac disease could subsequently be confirmed, in the other 7 cases the diagnosis could neither be rejected nor established with certainty. All three enzymes had a decreased activity in cases displaying subtotal or total villous atrophy, the most sensitive among them being lactase: in 69% of cases no lactase activity could be shown while saccharase and maltase were absent in 29 respectively 4% of the cases. No close correlation exists between the light-microscopic findings and the activity of enzymes since total absence of enzyme activity may be associated with only moderate villous atrophy. Lack of disaccharidase activity in the upper section of the small bowel does not necessarily mean disaccharide malabsorption exhibiting clinical symptoms, it only indicates a reduced capacity of disaccharide splitting. It has been concluded that routine determination of disaccharidase activities is not justified within the diagnostic procedure of coeliac disease.     

Giardiasis and coeliac disease

The incidence of Giardia lamblia infestation, as shown by examination of the stools, is the same in normal and coeliac children. 93 patients who were suspected of having coeliac disease had upper intestinal biopsies examined for giardia infestation. These patients usually had at least one stool and/or duodenal juice sample examined for G. lamblia. 26 patients (14 coeliac, 12 noncoeliac) had giardiasis. Only 13 patients would have been diagnosed if stools alone had been examined. Patients with giardiasis were usually male, younger than the other patients, more likely to have concurrent bacterial infection in the bowel, and more likely to come from a lower socioeconomic group. When the jejunal biopsies of 57 coeliac patients, including those with and without giardiasis, were compared, G. lamblia were more commonly found in those patients with less severe histological changes.     

Non-malignant complications of coeliac disease

Patients with coeliac disease are at increased risk of developing complications which increase morbidity and mortality. Emphasis on malignant complications has often overshadowed the non-malignant risks, which have received relatively little attention, although some of these can be very troublesome and even life-threatening. This article points out that a large population of unidentified or neglected coeliac patients is at potential risk. The challenge is to identify this group by case-finding or screening programmes in selected populations, so that they can be offered a gluten-free diet and other treatments which will not only improve general health but may also prevent or reduce the development of health problems. The non-malignant risks are outlined and bone and neuropsychiatric disturbances considered in more detail because of recent developments in these areas.     

Endocrine aspects of coeliac disease

Coeliac disease (CD) is a permanent intolerance to gluten that results in damage of the small intestinal mucosa, and it is one of the common causes of chronic malabsorption in children. It is well known that patients with CD are at great risk of malignant complications, but in patients with CD many other disorders have been recognized. Autoimmunity diseases, such as type 1 diabetes mellitus, thyroid diseases, and autoimmune polyglandular syndromes are known to be associated with CD, and they seem to be related to gluten exposure. Growth, bone metabolism, and fertility can be affected in patients with CD, especially if they are not on a gluten-free diet. We review the literature on endocrine aspects of CD, because patients with CD are at great risk of developing endocrine disorders.     

Latent coeliac disease in Italy

The term latent coeliac disease applies to patients who have a normal jejunal biopsy while taking a normal diet and, at some other time, before or since, have had a flat jejunal biopsy recovering on a gluten-free diet. Nineteen such patients were identified after a multicentre search conducted under the auspices of the Italian Society for Paediatric Gastroenterology and Hepatology (SIGEP). Serological data and histological material suitable for morphometric analysis were available from 10 and 8 patients, respectively, while they were on a gluten-containing diet. Neither gliadin antibodies nor increased density of intraepithelial lymphocytes are obligate markers of latent coeliac disease; endomysial antibodies are likely to be best predictors of evolution towards villous atrophy. Prospective studies are needed to allow a more precise definition of latent coeliac patient features.     

Latent and potential coeliac disease

Under the umbrella of coeliac disease (CD), or gluten-sensitive enteropathy, the concepts of silent, latent and potential CD have recently been introduced. While silent CD is marked by severe damage to the jejunal mucosa in the absence of clinical symptoms, both latent and potential CD are characterized by a jejunal mucosa that would be reported as normal by most clinical pathologists in an individual on a gluten-containing diet. As opposed to potential coeliac patients, latent subjects sometime in their life have had a flat jejunal biopsy which recovered on a gluten-free diet. Latent coeliac patients are often symptomatic; neither high titres of gliadin antibodies nor mucosal changes (including raised intraepithelial lymphocyte counts) are obligate features of latent CD, although the presence of elevated endomysial antibodies is probably the best predictor of progression towards villous atrophy. The term potential CD has been proposed for those subjects who do not have, and have never had, a jejunal biopsy consistent with overt CD, and yet have immunological abnormalities similar to those found in coeliac patients. Good markers of potential CD include the presence of serum endomysial antibodies, a high count of intraepithelial lymphocytes and subtle pathological alterations such as increased density of intraepithelial lymphocytes expressing γδ T cell receptor, signs of activated mucosal cell-mediated immunity, coeliac-like intestinal antibody pattern, and positive rectal gluten challenge.     

Thrombocytopenic purpura and coeliac disease

In a child with immune thrombocytopenic purpura (ITP) the findings of circulating reticulin antibodies and IgA and IgG gliadin antibodies suggested the diagnosis of coeliac disease. This was verified by small intestinal biopsy. In spite of a gluten-free diet the thrombocytopenia persisted. The association of ITP and coeliac disease was previously described in adults but to our knowledge this is the first report of the coexistence of ITP and coeliac disease in a child.