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Background The decision whether to perform endoscopy in children with suspected reflux oesophagitis is not a straightforward one. Few symptoms are specific for oesophagitis and the diagnosis is not always correlated even to visual findings on endoscopy. Aim The aim of this study was to define the role of endoscopy and especially of histology in the diagnosis of reflux oesophagitis and to examine the correlations between symptoms, endoscopic findings and histology in children with suspected gastroesophageal reflux disease. Patients and methods One hundred and thirty-six patients with a clinical diagnosis of reflux oesophagitis, aged 1–18 years (mean 8.43; standard deviation ±4.4), were enrolled from 12 Italian Paediatrics Gastroenterology Centres; symptom score, endoscopic and histologic oesophagitis scores were observed before and after therapy with proton pump inhibitors. Results Before therapy, a high correlation between the prevailing symptom score and endoscopic score was demonstrated, but not with histologic score: there was a significant tendency for histologic grade to exceed visual findings. After therapy, endoscopic score and histologic score were significantly improved. Conclusions Oesophageal biopsies increase the diagnostic accuracy of upper endoscopy. Histologic grading is often much more important than the endoscopic appearance, so that endoscopic oesophageal biopsies are very important aids in the diagnosis of oesophagitis. Appropriate clinical evaluation of symptoms must occur before endoscopic examination.

Aim: To make a prospective assessment of close family members of patients with coeliac disease (CD) by testing their endomysium (EMA) and antigliadin antibodies once a year over a period of 12 y and to investigate whether and when they would develop a positive serology for CD while on a gluten-containing diet. Methods: Since first-degree relatives of CD patients have a high prevalence of CD, we screened 92 children and adolescents, all first-degree relatives of coeliac patients, for EMA and total IgA antibodies, once a year. Results: Among 11 relatives, at the time of the first screening, 6 already had a positive serology and histology for CD, while 5 became positive only after a period of 2 to 5 y of negative testing. The jejunal mucosa biopsy of these five relatives with retarded positive serology for CD showed a flat mucosa in four of them and a partial villous atrophy in one. They were all HLA DQ2 positive and clinically silent for CD. Conclusion: CD can manifest itself after years of negative serological testing.

The integrins, a family of cell surface proteins, mediate cell adhesion and may influence within the intestinal mucosa processes such as migration and/or proliferation and differentiation of enterocytes and lymphocytes. The aim of this study was to examine the distribution pattern of integrin subunits (VLA alpha1, alpha2, alpha3, alpha4, alpha5, alpha6, beta1 chains) in normal intestinal mucosa and in that of patients with active coeliac disease (CD) and CD in remission. Immunohistochemical techniques and double immunostainings with monoclonal antibodies were used for investigation of the VLA alpha family of integrins and beta1 chain distribution. While the majority of the findings are consistent with the few data previously reported in the literature, surprising is the finding of a lack of expression of VLAalpha1 on the intraepithelial lymphocytes in the coeliac mucosa. The deficient VLA alpha1 expression on IEL in coeliac but not in normal mucosa may imply a genetic variation or a specific deficiency of gene expression during T cell differentiation and activation.

UNLABELLED Over 14 y of neonatal screening 71,675 dried blood samples were examined for the alpha-1-antitrypsin (alpha1-AT) alleles by isoelectric focusing in the Province of Bozen, Northern Italy. In infants carrying abnormal phenotypes the liver enzymes alanine aminotransferase and gamma-glutamyltransferase were determined at 2, 5 and 12 mo of age. In 17 neonates the PiMV phenotype was found, in 11 PiMF, in 11 PiMP, in 5 PiMN, in 3 PiMR, in 3 PiFZ, in 2 PiPZ and in 1 the PiMG phenotype was found. Two infants,1 carrying the PiMV and 1 the PiFZ phenotype showed at the age of 2 and 5 mo, respectively, elevated values of the liver enzyme S-ALAT[CE1]. Only the PiFZ and PiPZ carriers displayed low enough levels of alpha1-AT of 0.78 and 0.85 g l(-1) respectively, to be at moderately increased risk of pulmonary emphysema. Their early detection through the screening should discourage them from dangerous smoking habits. CONCLUSION Only a neonatal screening based on phenotyping can detect these rare carriers early in life.

The binding patterns of gliadin and selected lectins to jejunal biopsy specimens obtained from children with total villous atrophy during active celiac disease (CD; 19 patients) and in remission (16 patients) were examined by light microscopy. Three categories of carbohydrate-specific lectins were chosen for the study: those recognizing mannose/glucose residues, those recognizing N-acetyl-glucosamine/glucose (glcNAc/glc) residues, and those recognizing N-acetylgalactosamine/galactose (galNAc/gal) residues. The galNAc/gal lectins, with the exception of phaseolus vulgaris agglutinin variants, presented a typical staining of the luminal surface of the jejunal mucosa obtained from CD patients. However, these lectins displayed no reactivity to jejunal sections of CD patients in remission or control biopsies that included healthy children (25 children) and patients suffering from cow milk protein allergy (10 children). The glcNAc/glc lectin showed a strong preferential recognition of CD jejunal tissue but also bound with less intensity to specimens from patients with cow milk allergies and healthy children. Unlike other galNAc/gal lectins, phaseolus vulgaris agglutinin variants were indistinguishable in their binding patterns to the mucosa of control groups and CD patients in remission and failed to react to CD biopsies. The mannose/glc lectins were not distinctive in their binding patterns. In all cases, lectin binding was specifically inhibited by the lectins' competitive saccharides. Atypical of lectin binding patterns, gliadin reactivity was restricted to intracellular areas of enterocytes and was unique to active CD mucosa. The distinctive binding patterns of lectins and gliadin provide a diagnostic tool to distinguish patients with active CD from those in remission or patients with other intestinal disorders.(ABSTRACT TRUNCATED AT 250 WORDS)

The decision whether to perform endoscopy in children with suspected reflux oesophagitis is not a straightforward one. Few symptoms are specific for oesophagitis and the diagnosis is not always correlated even to visual findings on endoscopy.The aim of this study was to define the role of endoscopy and especially of histology in the diagnosis of reflux oesophagitis and to examine the correlations between symptoms, endoscopic findings and histology in children with suspected gastroesophageal reflux disease.One hundred and thirty-six patients with a clinical diagnosis of reflux oesophagitis, aged 1-18 years (mean 8.43; standard deviation +/-4.4), were enrolled from 12 Italian Paediatrics Gastroenterology Centres; symptom score, endoscopic and histologic oesophagitis scores were observed before and after therapy with proton pump inhibitors.Before therapy, a high correlation between the prevailing symptom score and endoscopic score was demonstrated, but not with histologic score: there was a significant tendency for histologic grade to exceed visual findings. After therapy, endoscopic score and histologic score were significantly improved.Oesophageal biopsies increase the diagnostic accuracy of upper endoscopy. Histologic grading is often much more important than the endoscopic appearance, so that endoscopic oesophageal biopsies are very important aids in the diagnosis of oesophagitis. Appropriate clinical evaluation of symptoms must occur before endoscopic examination.

Background Children allergic to cow's milk are fed a soy- or a hydrolysed cow's milk-based substitute. Neither can rule out a sensitization risk. Previous studies have shown that hydrolysed rice is tolerated by animals and children with multiple food hypersensitivities. Objective A prospective clinical assessment of tolerance to a rice-based hydrolysed formula was carried out in children allergic to cow's milk. Patients and methods One hundred children (42 girls and 58 boys, mean age 3.17+/-2.93 years, median 2.20, range 0.18-14.6 years) with a history of immediate reactions to cow's milk and confirmed at double-blind, placebo-controlled food challenge (DBPCFC) when not contraindicated were assessed for clinical tolerance to cow's milk proteins. Their allergy work-up included skin prick tests with whole milk, alpha-lactalbumin (ALA), beta-lactoglobulin (BLG) and total caseins, and specific IgE determinations using CAP technology were performed against whole milk, ALA, BLG and casein. Sensitization to rice and rice-based hydrolysed formula was similarly investigated. Patients' sera were evaluated at immunoblotting for specific IgE to cow's milk proteins, rice and rice-based hydrolysed formula. DBPCFC was carried out with increasing doses of a rice-based hydrolysed formula. Results All patients were sensitized to cow's milk and/or at least one cow's milk protein fraction. Eighty-seven out of 99 were positive to cow's milk and/or a cow's milk protein fraction at skin prick test. Positive (>0.35 kUA/L) specific IgE determinations were found for cow's milk and/or milk fractions (92/95), rice (21/91) and hydrolysed rice infant formula (4/91). At immunoblotting, sera from 96 children were positive to alpha-casein (n=54), beta-casein (n=38), ALA (n=57), BLG (n=37) and bovine serum albumin (n=61). Similarly, although patients' sera often contained specific IgE against rice proteins at CAP (21/91) and immunoblotting (70/96), only six very weakly positive responses were observed against rice-based hydrolysed formula. All DBPCFC with rice-based hydrolysed formula were negative. Conclusions Rice-based hydrolysed formula is a possible alternative not only for children with multiple allergies, but also for children with cow's milk allergy.

To make a prospective assessment of close family members of patients with coeliac disease (CD) by testing their endomysium (EMA) and antigliadin antibodies once a year over a period of 12 y and to investigate whether and when they would develop a positive serology for CD while on a gluten-containing diet.Since first-degree relatives of CD patients have a high prevalence of CD, we screened 92 children and adolescents, all first-degree relatives of coeliac patients, for EMA and total IgA antibodies, once a year.Among 11 relatives, at the time of the first screening, 6 already had a positive serology and histology for CD, while 5 became positive only after a period of 2 to 5 y of negative testing. The jejunal mucosa biopsy of these five relatives with retarded positive serology for CD showed a flat mucosa in four of them and a partial villous atrophy in one. They were all HLA DQ2 positive and clinically silent for CD.CD can manifest itself after years of negative serological testing.

Der α-1-Antitrypsin-(α1AT-)Mangel ist besonders in der kaukasischen Rasse europaischer Abstammung verbreitet und wird auch als Proteaseinhibitor- (Pi-)Krankheit bezeichnet. Dieses Defizit ist die haufigste Ursache genetisch bedingter Leberkrankheiten im Kindesalter und bei Erwachsenen Ursache der chronisch-obstruktiven Lungenkrankheit, die durch ein panacinares Emphysem gekennzeichnet ist. Das (α1-AT-Molekul ist ein ca. 55 kd schweres Glykoprotein, dessen Hauptaufgabe die Neutralisierung der destruktiven Proteasen der neutrophilen Zellen ist. Es ist ferner ein Akute-Phase-Protein, das im Serum bei Entzundungsprozessen und Gewebeschaden um das 3- bis 5fache ansteigen kann. Die hauptsachliche Bildungsstatte ist die Leberzelle, in geringer Menge wird α1-AT zusatzlich in extrahepatischen Geweben und Zellinien, wie Makrophagen, gebildet. Zwischen 20 und 53 μm/1 (150–350 mg/dl) liegen bei Gesunden die Serumspiegel, und mindestens 11 μm (ungefahr 80 mg/dl) sind notwendig, um die Lunge vor der Entwicklung eines Emphysems zu schutzen.

The ultra structural binding sites of endomysium antibodies have been studied on human umbilical cord tissue. The sensitivity and specificity of IgA endomysium antibodies were compared with recently described methods using basement membrane of smooth muscle of monkey oesophagus. Thirty adults affected by coeliac disease (10 in remission) and 75 healthy adult controls with normal intestinal mucosa (35 false antigliadin positive) were investigated. Sensitivity and correlation of endomysium antibodies with total villous atrophy in untreated coeliac disease patients were 100% on the human umbilical cord smooth muscles, and only 90% on the muscular layer of primate oesophagus. Indirect immunofluorescence was superior to peroxidase staining in detecting these IgA antibodies. The easy availability and enhanced testing sensitivity of the umbilical cord is an advance towards a better diagnostic tool for coeliac disease.

UNLABELLED: The long-term outcome of 3 PiSZ and 57 PiMZ children affected by alpha1-antitrypsin deficiency-associated liver involvement during infancy was assessed. At 5 and 10 y of age all PiSZ and PiMZ children exhibited normal levels of liver enzymes. All families, except one, were deterred from smoking following counselling. CONCLUSION: Transient liver involvement observed during early infancy is likely to be a benign self-limiting process.

Over 14 y of neonatal screening 71,675 dried blood samples were examined for the alpha-1-antitrypsin (alpha1-AT) alleles by isoelectric focusing in the Province of Bozen, Northern Italy. In infants carrying abnormal phenotypes the liver enzymes alanine aminotransferase and gamma-glutamyltransferase were determined at 2, 5 and 12 mo of age. In 17 neonates the PiMV phenotype was found, in 11 PiMF, in 11 PiMP, in 5 PiMN, in 3 PiMR, in 3 PiFZ, in 2 PiPZ and in 1 the PiMG phenotype was found. Two infants,1 carrying the PiMV and 1 the PiFZ phenotype showed at the age of 2 and 5 mo, respectively, elevated values of the liver enzyme S-ALAT[CE1]. Only the PiFZ and PiPZ carriers displayed low enough levels of alpha1-AT of 0.78 and 0.85 g l(-1) respectively, to be at moderately increased risk of pulmonary emphysema. Their early detection through the screening should discourage them from dangerous smoking habits.Only a neonatal screening based on phenotyping can detect these rare carriers early in life.

Der durch die hepatische Mikrozirkulation definierte Leberazinus ist die funktionelle Einheit der Leber. Der Austausch von gelosten Bestandteilen zwischen dem Blut und den Hepatozyten folgt einem Gradienten zwischen dem Portalfeld und der Zentralvene (Abb. 114.1). Zone 1 stellt die Hepatozyten dar, die der Zentralachse am nachsten sind, Zone 2 die intermediare Region des Azinus, und Zone 3 ist die Region, in der die Hepatozyten am weitesten von ihrer afferenten Blutversorgung entfernt sind. Die Differenzierung der Parenchymfunktionen in Zonen ist bei der Geburt nicht vollstandig. So ist bei Neugeborenen die Gallensaurenaufnahme im Azinus vermindert, so das der Konzentrationsgradient zwischen Zone 1 und 3 nicht erreicht wird. Auch die hepatische Zonierung fur den Kohlenhydratstoffwechsel tritt erst nach der Geburt ein. Die Entwicklung einer funktionellen Heterogenitat der Zonen nach der Geburt ist teilweise auf die veranderte Leberdurchblutung nach der Geburt zuruckzufuhren.

Dumping syndrome in infancy is a rare complication following gastric surgery. We describe an 11-month-old infant affected by recurrent peptic oesophagitis who underwent a combined Nissen fundoplication and pyloroplasty. Early dumping symptoms such as irritability, pallor, sweating, abdominal distension and watery diarrhoea were observed postoperatively after bolus feeding. Gastric emptying, measured after the administration of 150 ml of regular cow milk mixed with 200 microCi (8 MBq) of technetium-99m sulfur colloid (99mTc-SC), demonstrated an early rapid and massive emptying of the isotopes into the small intestine, followed by duodenogastric reflux and a second wave of emptying and reflux at 9 min. The initial pattern of gastric emptying and duodenogastric reflux was followed by a slow emptying phase with half-emptying time of 81 min. Isotope studies should be used to investigate motility disorders caused by this type of anti-reflux operation.

The integrins, a family of cell surface proteins, mediate cell adhesion and may influence within the intestinal mucosa processes such as migration and/or proliferation and differentiation of enterocytes and lymphocytes. The aim of this study was to examine the distribution pattern of integrin subunits (VLA alpha1, alpha2, alpha3, alpha4, alpha5, alpha6, beta1 chains) in normal intestinal mucosa and in that of patients with active coeliac disease (CD) and CD in remission. Immunohistochemical techniques and double immunostainings with monoclonal antibodies were used for investigation of the VLA alpha family of integrins and beta1 chain distribution. While the majority of the findings are consistent with the few data previously reported in the literature, surprising is the finding of a lack of expression of VLAalpha1 on the intraepithelial lymphocytes in the coeliac mucosa. The deficient VLA alpha1 expression on IEL in coeliac but not in normal mucosa may imply a genetic variation or a specific deficiency of gene expression during T cell differentiation and activation.

The incidence of coeliac disease is different throughout Europe. The aim of our study was to investigate its true prevalence using antigliadin and endomysial antibodies in 4615 healthy adults within two different population groups in Northern Italy: 140 were positive for antigliadin antibodies (AGA), 111 only for AGA IgG, 17 for AGA IgA and 12 for IgG and IgA. Of those positive for AGA, nine had an abnormal small-intestine mucosa and were positive for endomysium antibodies (EmA) using the umbilical cord test, a novel methodology. The prevalence, 163/100,000 in the Italian group and 216/100,000 in the German group, is much higher than what was assumed. The positive predictive value of the EmA, confirmed by biopsy, is 100%, that of AGA IgG 2%, of AGA IgA 12% and of the combined AGA IgA and IgG 33%. AGA positive but EmA negative patients displayed normal histological features and distributions of alpha/beta and gamma/delta T-cell receptors on intraepithelial lymphocytes. We can therefore conclude that antigliadin antibodies are not an ideal tool for screening purposes; the umbilical cord EmA test is superior owing to its optimal sensitivity, specificity, high positive predictive value and low cost.

A case of Munchausen syndrome by proxy in an infant presenting with recurrent vomiting and severe failure to thrive is described. Only very few cases of this syndrome have been reported in the italian literature in comparison to those described in the Anglo-Saxon countries. The factitious symptoms and signs fabricated or induced by parents lead to unnecessary medical investigation, hospital admissions and treatment. The Authors emphasize the difficulties in reaching a diagnosis and the risks of this potentially very dangerous behaviour in terms of morbidity and mortality. Since only one third of parents recover following psychotherapy, the offending parent should be sometimes separated from the child and promptly reported and closely supervised by the legal Authorities. Bizarre and otherwise peculiar, unexplained symptoms should always suggest the possibility of the Munchausen syndrome by proxy.

Low levels of alpha-1-antitrypsin can predispose deficient infants to the development of hepatitis and cirrhosis. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement during their first half year of life. One pathogenic hypothesis of liver damage is that the process seems to be mediated by the activity of toxic oxygen waste products. In the present investigation it was found that the antioxidant vitamin E was able to significantly reduce the frequency of liver involvement in PiMZ carriers at two months of age but not at five months. These findings indicate that oxidative free radicals can promote liver damage in inadequately protected young infants, such as in alpha-1-antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other anti-oxidant scavengers is discussed.

We report the case of a 13 month old boy presenting with an intestinal pseudo-obstruction. He showed the clinical and radiological features typical of a paralytic intestinal occlusion. After 48 hours of parenteral nutrition, the intestinal motility returned to normal. A jejunal biopsy showed total villous atrophy. The patient did not relapse with the same symptoms on a gluten challenge. The differential diagnosis and the similarities with obstructive events in cow's milk protein sensitive enteropathy are discussed.

A 7-year-old girl was admitted to hospital with a 2-month history of profound weakness. Anaemia, marked neutropenia and hypersegmentation of the granulocytes were the major laboratory findings. These abnormal haematological data were due to significant folate deficiency caused by active coeliac disease diagnosed by both the presence of antigliadin and endomysium antibodies and typical histological features of the small intestine. The haematological abnormalities resolved within 3 weeks following a gluten-free diet and iv supplementation of folic acid.

A Mr = 110,000 glycoprotein, GP 110, was partially purified using wheat germ agglutinin-Sepharose affinity chromatography from a bile canalicular-enriched membrane fraction denoted N2u of rat liver. This fraction was subjected to preparative sodium dodecyl sulfate-polyacrylamide gel electrophoresis and the Mr = 110,000 polypeptide was excised and used as an immunogen in rabbits. The antisera were found to specifically recognize a Mr = 110,000 polypeptide, named GP 110, in the N2u membrane fraction. In isolated hepatocytes, GP 110 was readily accessible to cell surface iodination catalyzed by lactoperoxidase at 4 degrees C and was judged by immunoprecipitation studies to contain about 2% of total radioactivity incorporated into externally oriented proteins of the cell. Immunoprecipitated GP 110 was shown by two-dimensional polyacrylamide gel electrophoresis to migrate with an approximate pI of 4.9. Indirect immunofluorescence on frozen liver sections demonstrated that GP 110 was primarily localized in the bile canaliculus. In corroborative studies employing subcellular fractionation, it was found that GP 110 was enriched nearly 19-fold in P2, a plasma membrane fraction primarily derived from the sinusoidal domain, and 44-fold in N2u. In contrast, only low levels of GP 110 were present in endoplasmic reticulum, mitochondrial, cytosolic, and nuclear-enriched fractions of liver. The physiological function of GP 110 is as yet unknown; antisera to it did not immunoprecipitate other known bile canalicular proteins of similar molecular weights. GP 110 was found to be extensively glycosylated relative to other known membrane proteins; approximately 33% of the apparent molecular weight appear to be carbohydrate. In agreement, limited removal of N-linked carbohydrate chains indicated that there are approximately eight chains/GP 110 polypeptide. Neuraminidase treatment of GP 110 resulted in a desialylated Mr = 85,000 polypeptide suggesting that the majority of carbohydrate chains on GP 110 are of the complex type.

This paper describes a case of late neonatal convulsions due to intracranial haemorrhage in a newborn with cholestatic hepatopathy due to PiZZ homozygote alpha-1-antitrypsin deficiency. The deficiency of vitamin K dependent clotting factors, responsible for the haemorrhage, seems to be due to the cholestasis and might have been aggravated by the non-administration of vitamin K at birth and by breast feeding. The response to vitamin K therapy was good.

A Mr = 110,000 glycoprotein, GP 110, was partially purified using wheat germ agglutinin-Sepharose affinity chromatography from a bile canalicular-enriched membrane fraction denoted N2u of rat liver. This fraction was subjected to preparative sodium dodecyl sulfate-polyacrylamide gel electrophoresis and the Mr = 110,000 polypeptide was excised and used as an immunogen in rabbits. The antisera were found to specifically recognize a Mr = 110,000 polypeptide, named GP 110, in the N2u membrane fraction. In isolated hepatocytes, GP 110 was readily accessible to cell surface iodination catalyzed by lactoperoxidase at 4 degrees C and was judged by immunoprecipitation studies to contain about 2% of total radioactivity incorporated into externally oriented proteins of the cell. Immunoprecipitated GP 110 was shown by two-dimensional polyacrylamide gel electrophoresis to migrate with an approximate pI of 4.9. Indirect immunofluorescence on frozen liver sections demonstrated that GP 110 was primarily localized in the bile canaliculus. In corroborative studies employing subcellular fractionation, it was found that GP 110 was enriched nearly 19-fold in P2, a plasma membrane fraction primarily derived from the sinusoidal domain, and 44-fold in N2u. In contrast, only low levels of GP 110 were present in endoplasmic reticulum, mitochondrial, cytosolic, and nuclear-enriched fractions of liver. The physiological function of GP 110 is as yet unknown; antisera to it did not immunoprecipitate other known bile canalicular proteins of similar molecular weights. GP 110 was found to be extensively glycosylated relative to other known membrane proteins; approximately 33% of the apparent molecular weight appear to be carbohydrate. In agreement, limited removal of N-linked carbohydrate chains indicated that there are approximately eight chains/GP 110 polypeptide. Neuraminidase treatment of GP 110 resulted in a desialylated Mr = 85,000 polypeptide suggesting that the majority of carbohydrate chains on GP 110 are of the complex type.

There is described a case of Gaucher's disease in its acute infantile form, interesting for the initial symptomatology marked by a hepatitic similarity, which has got manifest, already during the first days of life. A splenic and hepatic biopsy made at the age of about six months showed clearly the presence of cells with the characteristics of Gaucher's cells. A sure diagnosis was made at seven months through the determination of beta-glucosidase on the fibroblasts after the skin-biopsy. The patient died at the age of 14 months during an apnoic crisis.

Two one- and three-month-old infants were admitted with clinical and radiologic signs of acute small bowel occlusion. They had been fed since the first day of life with a formula containing cow's milk proteins. The withdrawal of the allergen alone without surgical intervention led to normalization of bowel function within one day. At a new challenge with cow's milk protein the infants had an immediate clinical and biochemical response. The precocious introduction of formula containing cow's milk proteins was responsible for the allergic reaction.

We determined the PiM, PiS and PiZ gene frequencies by isoelectric focusing in 9128 newborns from two major ethnic groups, an Italian and a German one, in the Northern part of Italy. In the Italian group the PiS allele frequency is 0.032, the PiZ = 0.015, in the German one 0.015 and 0.019 respectively. Nine ZZ, 2 SS, 10 SZ, 314 MZ and 307 MS were detected. In our two population groups the PiS reflects better than other alleles a close genetic relationship between our German population and studied groups in Austria and Germany.

During the first month of life 28 full-term newborns were breast-fed (18 males and 11 females). Thereafter 8 infants continued breast-feeding while the remainder were randomly fed on either an adapted milk formula (n=13) or a soy-formula (n=7). At five months, after an oral dose of RIT 4237 rotavirus vaccine of bovine origin was given, growth and IgM/IgG type antibodies against rotavirus were measured. Weight gain was similar in all infants. There were 2 IgM and 1 IgG responders out of 7 soy fed infants, compared with 4 out of 8 human milk fed (both IgM and IgG) and 7 out of 13 IgM and 6/12 IgG formula fed infants responding to vaccination. This observation confirms previous results obtained with polio, diphtheria tetanus and pertussis vaccines indicating that soy-protein formulas may interfere with immunization processes.

In 16 children with coeliac disease consecutively diagnosed on the basis of 3 peroral small bowel biopsies in a period of 3 years 13 (81%) had low levels of folic acid in the serum (norm. reference range: 1.8-9 ng/ml) and in erythrocytes (norm. reference range: 150-450 ng/ml cells). The determination of folic acid has a better diagnostic value as the one-hour-D-Xylosetest. The low level of folic acid is the most constant nutritional deficiency in our group of coeliac patients. The determination of folate in the serum and erythrocytes is a highly sensitive screening and at the time it will indicate the adequate therapy.

A chronic diarrhea accompanied by vomiting and weight loss was the major symptom in a one-year-old infant during a fulminant course of histiocytosis X. The diagnosis was suggested by the radiologic evidence of alternating dilated and stenotic segments in the small and large bowel as well by a massive intestinal protein loss and the presence of histiocytes in the mucosa and submucosa of the rectum. The histological picture of enlarged mesenteric lymph nodes, obtained during a diagnostic laparotomy, confirmed the diagnosis.

Superoxidase dismutase (SOD) is the main scavenger of superoxidase radicals in the mammalian body. The liver contains high levels of two SOD enzymes; Cu-Zn (SOD1) localized in cytosol and Mn SOD (SOD2) localized in mitochondria. The aim of the present study was to investigate the levels of SOD1 and SOD2 during development using enzymatic and immunological methodologies. Monospecific antibodies were prepared in rabbit against purified rat SOD1 and SOD2 polypeptides. Homogenates of fresh rat livers obtained from newborn, young, and adult rats were assessed for levels of SOD1 and SOD2 activity. It was found that the levels of SOD1 activity increased greater than ten-fold after birth, reaching adult levels after weaning. In contrast, the SOD2 level increased only four-fold after birth and reached essentially adult levels at weaning. SOD1 and SOD2 in newborn and young rat livers were not different in their molecular weight or charge from adult liver. Immunoblot quantitation indicated that changes in activity during ontogeny levels reflected the number of SOD1 and SOD2 molecules. These results show that SOD1 and SOD2 are differentially and independently regulated during ontogeny. It may be suggested that cytosolic and mitochrondrial SOD may play different roles in superoxide scavaging during liver development.

VITAMIN E AND PREVENTION OF LIVER DAMAGE IN HETEROZYCOTES WITH ALPHA-1-ANTITRYPS IN DEFICIENCY