[Zinc in the therapy of acrodermatitis enteropathica].
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Biomedical subjects
Publications and source records attributed to J Jodl.
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In a group of 26 child patients with coeliac sprue verified by clinical tests and biopsy, we observed a significant change in the proportion of the intestinal alkaline phosphatase isoenzyme in the serum in correlation to a gluten-free therapeutic diet. In untreated patients, in the active phase of the disease, intestinal AP isoenzyme activity rises in the serum and falls in the intestinal mucosa. In our experience, the change in activity is reversible within 3--5 days of instituting adequate treatment, i.e. a gluten-free diet. No such activity changes were observed in children with other forms of malabsorption syndrome (hepatogenic, pancreatogenic) or in the children in the control group. This test was found to be a suitable and sensitive method for indirect determination of the response of the intestinal mucosa to treatment in children with coeliac sprue.
The clinical features consisting mainly of enophthalmos, beaked nose, narrow palpebral fissures, receding chin, long fingers and toes, typical for chromsomal syndrome or partial trisomy 9q, were confirmed in a new case.
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Pancreatic enzyme activity in low birth weight (LBW) infants during the first postnatal week has been evaluated by analysing the chymotrypsin content of 198 stool specimens from 42 LBW infants with birth weights ranging between 750 and 2570 g. A wide variation in chymotryptic activity yet with a tendency to initially low values with a peak on the third day after birth was found. Small-for-gestational age (SGA) infants had significantly lower values than appropriate-for-gestational age (AGA) infants. This is considered due to intra-uterine malnutrition with secondary pancreatic dysfunction in SGA infants. In screening program for cystic fibrosis or other defects of exocrine pancreatic activity, low stool chymotrypsin values cannot be considered pathological until after the fourth day of life.
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Thirty-eight specimens obtained by jejunal biopsy from 22 children suffering from mucoviscidosis were examined by histochemical techniques. In 27% of the patients the findings were within normal limits. In 18% of cases, associated coeliacal sprue was disclosed. The remaining cases displayed slight morphological abnormalities associated with trehalase and/or lactase deficiency, and in 41% there was hypersecretion of viscous mucus filling up dilated crypts and adhering to the surface of villi. The findings as observed in enterobiopsis are not pathognostic of mucoviscidosis. They however, should make one to think of it, particularly if trehalase and/or lactase deficiency is found associated with hypersecretion of viscous mucus and an almost normal morphological appearance. Neither normal findings nor that of coeliacal sprue exclude the diagnosis of mucoviscidosis. It appears that malabsorption in mucoviscidosis is not only pancreatogenic; the intestinal mucosa may be contributory to a various degree as well.
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