Short stature and malnutrition in cystic fibrosis.
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Biomedical subjects
Publications and source records attributed to G Magazzù.
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The steps of the morphological relapse occurring during gluten-challenge in coeliac children were for the first time investigated by scanning electron microscopy and compared with the morphological changes observed in untreated and treated coeliac patients, in pathological and normal controls. Some peculiar morphological changes, not reported up to now, were observed in treated and relapsed coeliac patients. No relationship was found among the degrees of mucosal atrophy observed by SEM and the duration of the challenge performed by an uncontrolled ingestion of gluten.
The effects of a gluten-free diet on catch-up growth and predicted height were evaluated in 12 children with coeliac disease diagnosed after the age of 5 years and followed for 2-5.5 years. In the majority of the patients, height and bone age were retarded at the time of diagnosis. Under a gluten-free diet growth velocity, age-related height, predicted height and relative bone age increased, height for bone age slightly decreased. In four patients the predicted height remained below the target height, indicating incomplete catch-up growth.
The distribution pattern of some labelled lectins (WGA, SBA, PNA, DBA, ConA, LCA) has been investigated in small intestinal mucosa of coeliac children (119 cases) and normal (short stature) and pathological (postenteritis syndrome) controls. The oligosaccharide side chains of glycoproteins present in the cytoplasm and in the brush border of enterocytes, goblet cells and luminal crypt surface have been revealed. The most important differences in lectin binding reactivity between coeliac and controls mucosae are discussed.
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This is the first report of obesity in an untreated coeliac patient diagnosed at the age of 5.1 years according to the criteria of the European Society for Paediatric Gastroenterology. Diagnosis of coeliac disease was suspected on the basis of recurrent episodes of abdominal pain and of family history. Gluten-free diet induced evident acceleration of both length and weight velocity and increase of weight excess, apart from resolution of the abdominal symptoms. The present case report demonstrates that obesity in a child does not exclude the diagnosis of coeliac disease.
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The reliability of random fecal alpha 1-antitrypsin (FA-1-AT) concentration has been evaluated by comparing FA-1-AT values on random specimens and on concomitant 24-72 h fecal collections. In order to simplify the method, FA-1-AT data derived from lyophilized fecal samples were compared with those obtained from 37 degrees C heat-dried fecal samples. Random FA-1-AT concentration was assayed in 80 children with various gastrointestinal illnesses and 36 healthy age-matched controls. There was a close relationship between FA-1-AT values obtained from random samples and 1-day or 3-day collections. There was also a significant relationship between FA-1-AT values derived from the two different ways of drying the stools. Mean FA-1-AT values were statistically different when compared to the controls in the following groups of disorders: untreated and after-gluten-challenge celiac disease, post-enteritis syndrome, and cow's milk intolerance. The possible meanings of the abnormal FA-1-AT concentration in the various disorders are discussed. We conclude that FA-1-AT is a simple and reliable test for enteric protein loss. The simplification of the method proposed by us should reduce the cost of the test and moreover make it feasible in all laboratories.
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Using histochemical methods for mucins, we have studied duodeno-jejunal biopsies obtained from infants with proved coeliac disease (19 subjects with subtotal villous atrophy and 11 subjects with partial villous atrophy) and from controls with histologically normal mucosa. In coeliac disease, but not in controls, an increase of both weakly and strongly acidic sulphomucins is observed. These histochemical findings are discussed in relation to their significance and their possible diagnostic value in confirming the diagnosis of coeliac disease before treatment, during remission and after gluten challenge.
Neutral mucins in duodeno-jejunal biopsies of coeliac infants were studied. Subtotal villous atrophy was observed in 22 untreated patients whereas 18 patients, after a gluten-free diet for twelve months, had an histologic pattern of partial villous atrophy. The results showed a different distribution of neutral mucosubstances along the crypts and villi; in mucosae with subtotal villous atrophy, some goblet cells producing only neutral mucins were evident at various levels in deep crypts, when compared to those observed in partial villous atrophy. The possible correlation between epithelial cell differentiation and neutral mucins secretion was discussed.
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A case of likewise atrial dissociation in a twelve years old patient affected by mucovisclosis with severe respiratory failure is described. The classic criteria of the atrial dissociation were met in the ECG, in which, beside the sinus P waves, low P-like waves, whose rate was 69/m', were present: they did not conduct to the ventricles and were independent from the sinus rhythm. Such waves always coincided with the beginning of the inspiration, and disappeared during the apnoea. So they were not believed as being the results of electrical forces of the heart, but artifacts produced by respiratory movements. The diagnostic criteria of the atrial dissociation are discussed on the basis of these observations. the exclusion of artifacts, especially the respiratory ones, and the demonstration, obtained through the intracavitary ECG, that the waves are really produced by the heart, are necessary to diagnoses the arrhythmia. By the application of these diagnostic criteria only one of the cases found in the Literature has been accepted as demonstrative of atrial dissociation due to complete intraatrial block in man.
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