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Biomedical subjects

K Pittschieler

Publications and source records attributed to K Pittschieler.

35 records · Page 2Linked to original sources
PubMed1991

Liver disease and heterozygous alpha-1-antitrypsin deficiency.

From 1985 to 1988 14,938 newborns were screened during the first days of life to determine their protease inhibitor phenotype (Pi) and 467 PiMZ and 456 PiMS were identified. Of these 101 PiMZ and 135 PiMS were followed-up and their clinical, biochemical and, in selected cases, histological data were recorded at two, five and twelve months of age. Nineteen out of 101 PiMZ infants showed hepatic dysfunction at two months, eight at five, and one at twelve months of age, respectively. In 20 of 135 PiMS infants, liver function tests were abnormal at two months, in ten at five and in none at twelve months. It appears that PiMZ and PiMS phenotypes can be associated with hepatic dysfunction during the first six months of life. The marked variability of serum levels of alpha-1-antitrypsin in heterozygotes, make Pi-typing in all cases of neonatal hepatitis advisable. This should also be done for screening purpose.

Biopsy↗
PubMed1991

Oxidative radicals and liver involvement of infants with alpha-1-antitrypsin deficiency.

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.

Free Radicals↗
PubMed1990

Cow's milk protein-induced colitis in the breast-fed infant.

A 3-month-old boy is described who developed an inflammatory proctocolitis during exclusive breast feeding. The major symptom was bloody diarrhea. No growth failure or constitutional symptoms were present. Bacterial, viral, and parasitic infection was excluded. The colonoscopic findings of focal ulceration, edema, and increased friability and the histologic picture with a marked eosinophilic infiltration were indicative of an allergic intestinal reaction. The infant responded favorably to the withdrawal of the mother's milk and the initiation of hydrolyzed casein formula. The challenge with cow's milk led to a clinical relapse resembling the initial manifestation.

Animals↗
PubMed1989

Response to RIT 4237 oral rotavirus vaccine in human milk, adapted-and soy-formula fed infants.

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.

Administration, Oral↗
PubMed1989

[Chronic diarrhea as the main symptom of histiocytosis X].

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.

Biopsy↗
PubMed1988

Alpha 1 antitrypsin deficiency in two population groups in north Italy.

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.

Alleles↗
PubMed1987

Isolation and characterization of a Mr = 110,000 glycoprotein localized to the hepatocyte bile canaliculus.

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.

Animals↗
PubMed1986

[Folic acid concentration in the serum and erythrocytes of patients with celiac disease].

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.

Celiac Disease↗
PubMed1986

[Cow's milk protein intolerance as a cause of small bowel occlusion].

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.

Acute Disease↗
PubMed1985

[Neonatal spasms caused by cerebral hemorrhage in alpha 1-anti-trypsin deficiency].

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.

Cerebral Hemorrhage↗
PubMedDate not supplied

[Celiac disease as a cause for occlusion of the small intestine].

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.

Biopsy↗
PubMedDate not supplied

Celiac disease in two different population groups of South Tirol.

The expression of celiac disease is assumed to be influenced by both genetic and environmental factors. Our study investigated the prevalence and HLA type in two different ethnic groups: a German group and an Italian group living in South Tirol. Intermarriages are rare between these population groups. The live birth rate during 1973-1982 (10 years) was 42,739 for the German group and 14,874 for the Italian group. Fifty people with celiac disease, born during this period, were diagnosed according to the ESPGAN criteria: 45 were German and five were Italian. The incidence in the German group was 105 per 100,000, and 33 per 100,000 in the Italian group. HLA typing was performed in 40 patients, and in 50 German and 50 Italian controls. Of the patients with celiac disease, 43% were positive for HLA B8, 85% for DR3, and 66% for DR7. Although lower, no difference was found in the expression of HLA B8, DR3, and DR7 in the Italian and German controls. The data presented here strongly suggest that there is little association between the incidence of the disease and HLA type in the two ethnic populations. However, from preliminary data, it is postulated that the age of the infant when gluten is added to its diet will affect the incidence of celiac disease.

Celiac Disease↗
PubMedDate not supplied

[Neonatal hepatitis in a case of Gaucher's disease in its acute infantile form].

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.

Biopsy↗
PubMedDate not supplied

[A case of Munchausen syndrome by proxy with digestive symptoms and severe growth retardation].

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.

Child Abuse↗