CharlotteM. Anderson

ONE-HOUR BLOOD-XYLOSE SCREENING-TEST FOR CŒLIAC DISEASE IN INFANTS AND YOUNG CHILDREN

Abstract A single blood-xylose estimation one hour after ingestion of a standard 5 g. dose of D-xylose has proved a reliable indicator of malabsorption in children under 30 kg. with untreated cœliac disease. The test was performed on 117 children in whom the diagnosis of cœliac disease was considered; all but 1 of the 53, subsequently shown to have cœliac disease by small-bowel biopsy, had a one-hour blood-xylose level below 20 mg. per 100 ml. Of the remaining 64 with normal small-bowel-biopsy, all had levels of 20 mg. per 100 ml. or greater, in a range comparable with that of 75 controls without gastrointestinal disease and 40 treated cœliac patients. For comparison, the test was performed on 22 patients with cystic fibrosis, who also had one-hour blood-xylose results within the normal range. A normal one-hour blood-xylose level virtually excludes the diagnosis of cœliac disease in children below 30 kg. A result of 20 mg. per 100 ml. or less, if confirmed by a repeat test, is an indication for small-bowel biopsy.

CŒLIAC DISEASE IN CHILDREN OF ASIAN IMMIGRANTS

Abstract Seventeen cases of cœliac disease have been found in children of Asian immigrants. Their parents all originated from the Punjab or West Pakistan, and it is possible that these communities may have a frequency of childhood cœliac disease comparable to that found in White children in Britain. Six of the seventeen children (35%) had active rickets; it is suggested that cœliac disease may be an aetiological factor in some cases of rickets in immigrant children.

DOES THAT CHILD REALLY HAVE CYSTIC FIBROSIS

A diagnosis of cystic fibrosis was incorrectly made after false-positive sweat tests in 14 children. 13 of these children had been tested at hospitals where it seems likely that sweat tests were not done very often. All the children had normal sweat-electrolyte values when the test was repeated at a regional paediatric centre where approximately 250 sweat tests are done each year. In 5 cases, detailed testing of pancreatic function was normal. None of the children had typical chest disease and only 2 had gastrointestinal symptoms. In the absence of the typical clinical features of the disease a diagnosis of cystic fibrosis should be made with extreme caution and only after meticulous testing of both sweat electrolytes and pancreatic function.

CYSTIC FIBROSIS OF THE PANCREAS

Abstract From current knowledge of the normal secretory mechanisms in exocrine glands and their abnormal activity in patients with cystic fibrosis of the pancreas, a hypothesis has been developed which relates the disturbance in mucous and serous glands in the disease to a single biochemical abnormality—namely, inhibition of the movement of water and ions through the secretory cells in the glands affected. It is further suggested that this disturbance resides in the extracellular space, and is related to the structure or porosity of mucopolysaccharide-rich regions, either in the connective tissue proper or in a mucoid layer immediately adjacent to the cell plasma-membrane. This concept is illustrated in the accompanying figure. The primary genetic abnormality is still unidentified; however, the wide spectrum of changes observed in different patients may mean that the basic defect is a number of metabolic steps away from the final pathological manifestations of the disease. Mechanisms, possibly hormonal in nature, which control or can modify the movement of water and electrolytes in the extracellular regions of exocrine glands throughout the body should therefore be considered.

A NEW EXPERIMENTAL SYSTEM FOR THE STUDY OF THE PATHOGENESIS OF CŒLIAC DISEASE

A method is described for the differential removal of epithelial cells from human intestinal-tissue specimens. Epithelial cells can be freed of contaminating cells such as erythrocytes, and on the basis of microscopic appearance, dye exclusion tests, and short-term survival in defined media are viable. The experimental system can be used to investigate the differential cytotoxic effects,if any, of specific gluten components upon cells from normal subjects and coeliac patients.