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K Pittschieler

Publications and source records attributed to K Pittschieler.

At least 19 recordsLinked to original sources
PubMed2006

A hydrolysed rice-based formula is tolerated by children with cow's milk allergy: a multi-centre study.

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.

Adolescent↗
PubMed2003

Onset of coeliac disease: a prospective longitudinal study.

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.

Adolescent↗
PubMed2002

Liver involvement in alpha1-antitrypsin-deficient phenotypes PiSZ and PiMZ.

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.

Humans↗
PubMed2001

Rare alpha-1-antitrypsin phenotypes and liver-test abnormalities during infancy.

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.

Alleles↗
PubMed1999

No fear of NO?

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Cell Communication↗
PubMed1998

Expression of the VLA family of integrins in coeliac intestinal mucosa.

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.

Antibodies, Monoclonal↗
PubMed1996

Coeliac disease: screened by a new strategy.

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.

Adolescent↗
PubMed1995

Neutropenia, granulocytic hypersegmentation and coeliac disease.

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.

Anemia↗
PubMed1994

Endomysium antibodies in coeliac disease: an improved method.

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.

Adolescent↗
PubMed1994

Reactivity of gliadin and lectins with celiac intestinal mucosa.

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)

Binding, Competitive↗
PubMed1993

Vitamin E and liver damage in MZ heterozygous infants with alpha 1-antitrypsin deficiency.

Low levels of alpha 1-antitrypsin can predispose affected infants to develop a wide spectrum of liver diseases. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement in the first six months of life. The liver damage appears to be mediated by the activity of toxic oxygen waste products originating from partially unchecked proteases which can cause enough damage to impair hepatic function significantly. In the present study it was found that the antioxidant properties of vitamin E were able to reduce the frequency of liver dysfunction in PiMZ carriers at two but not at five months of age. Liver damage is highly related to low levels of alpha-tocopherol in the plasma. These findings show that oxidative free radicals can promote liver damage in inadequately protected young infants, such as those affected by alpha 1-antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other antioxidant scavengers is discussed.

Alleles↗
PubMed1992

Liver involvement in infants with PiSZ phenotype of alpha 1-antitrypsin deficiency.

Between July 1985 and June 1989, 19,432 newborns were screened during the first days of life to determine their Pi (protease inhibitor) phenotype. Fourteen infants were identified to be carriers of the PiSZ phenotype. Their clinical and biochemical follow-up data were recorded at 2, 5, and 12 months of age; only one case underwent a liver biopsy due to repeated abnormal liver enzymes. Three of 14 PiSZ infants showed some hepatic dysfunction at 2 and 5 months of age, but at 12 months all patients had normal liver function tests. None of them had clinical, biochemical, or morphological signs of neonatal cholestasis. The clinical and biochemical data related to the liver involvement are similar to those of the PiMZ phenotype, though the serum levels of alpha 1-antitrypsin in PiSZ carriers match those of the PiZZ group.

Alanine Transaminase↗
PubMed1991

Adipsic hypernatremia in two sisters.

We describe two sisters with chronic hypernatremia, lack of thirst, and inappropriate osmoregulated vasopressin secretion. Only one sister, who presented with microcephaly and developmental delay, showed signs of dysplasia of the midline structures (ie, septum pellucidum and corpus callosum) and a large intracranial cyst. Neither sister showed any signs of thirst, even when osmolality exceeded 337 mmol/kg. In both patients, the vasopressin secretion did not respond to either osmotic or nonosmotic stimuli or was suppressed by a water load. Plasma osmolality values returned to normal after treatment with forced hydration and a vasopressin analogue, desamino-D-arginine vasopressin. These findings indicate a severe defect in the hypothalamic osmoreceptors that control thirst and vasopressin secretion. To our knowledge, this is the first report of such a disorder in two sisters.

Aldosterone↗
PubMed1991

Dumping syndrome after combined pyloroplasty and fundoplication.

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.

Dumping Syndrome↗
PubMed1991

Levels of Cu-Zn and Mn superoxide dismutases in rat liver during development.

Superoxide dismutase is the main scavenger of superoxide radicals in the mammalian body. The liver has high levels of two types of superoxide dismutase enzymes, cytosolic Cu-Zn and mitochondrial Mn enzymes. The aim of the present study was to investigate the content of two distinct superoxide dismutases in liver during the perinatal transition from a hypoxic to a hyperoxic environment. Both isozymes were purified to homogeneity and used as immunogens in rabbits. Antisera raised were found to recognize only polypeptides of molecular weight 16,900 or 23,400, which correspond to Cu-Zn and Mn superoxide dismutases, respectively. It was found that the level of Cu-Zn superoxide dismutase enzymatic activity and protein as assessed by immunoquantitation increased 10-fold during the postnatal period, reaching adult levels by 3 weeks. In contrast, the amount of Mn superoxide dismutase content increased only twofold to adult levels during the first week of life. Neither of the superoxide dismutases showed an alteration in specific activity or apparent molecular weight in rat livers during ontogeny. These results show that the levels of two intracellular superoxide dismutases are differentially elevated during the perinatal period. It is suggested that each dismutase plays a different physiological role for superoxide scavenging in liver as a function of the hypoxic/hyperoxic environment at birth.

Animals↗