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Biomedical subjects

G Korányi

Publications and source records attributed to G Korányi.

At least 19 recordsLinked to original sources

[Liver changes suggesting alpha-1-antitrypsin deficiency (a neglected disease)].

Liver needle biopsies of one hundred infants and children were examined. In fifteen of them the liver was normal. Of the 85 patients with liver disease three had liver changes due to severe alpha-1-antitrypsin (AAT) deficiency. In two cases fibrosis and inflammation of the portal fields could be seen. In the third case in addition to portal fibrosis and moderate periportal inflammation paucity of the intrahepatic bile ducts was found. Characteristic PAS-positive diastase resistant hepatocellular globules occurred only in one case but, using immunoperoxydase method, periportal hepatocytes showed AAT positivity in all three infants. Of 830 adult patients with liver cirrhosis 8 had PAS-positive diastase resistant AAT immunoreactive globules in the periseptal hepatocytes suggesting AAT deficiency, however, the serum AAT level and the phenotype of them were unknown. Investigation for AAT deficiency should be carried out in children and young adults with a history of neonatal liver disease and possibly in all patients with liver disease of uncertain etiology, especially in those with PAS-positive, AAT immunoreactive hepatocellular globules.

Biopsy↗

[Alpha-1-antitrypsin deficiency and liver diseases].

Authors studied liver biopsy of hundred infants and young children. 15 livers were normal and three of 85 patients suffering from liver disease had liver changes associated with serious alpha--1-antitrypsin (AAT) deficiency (in two cases with portal fibrosis associated with inflammation, in one case with portal fibrosis associated with moderate active inflammation of reduction of intrahepatic biliary ducts). Typical positive, diastase resistant globules were in the hepatocytes of only one patient, but AAT could be demonstrated in periportal liver cells of all three patients by AAT specific immuneperoxidase method. Of 830 hepatic cirrhosises in adult age, in eight cases PAS positive, diastase-resistant AAT immunreactive globules occurred in periportal hepatocytes, suggesting AAT deficiency; however, the AAT level and fenotype of these patients were not known. Examination of possibility of AAT deficiency should be performed in every case, where the cause of liver disease is unsolved; this examination is especially indicated by the presence of typical PAS positive, diastase-resistant, AAT immunreactive globules in hepatocytes.

Child↗

[Enzyme inductive effect of zixoryn (3-trifluoromethyl-alpha-ethyl-benzhydrol) in neonatal jaundice].

Zixoryn, (3-trifluoromethyl-alfa-aethyl-benzhydrole) is a new product of the Hungarian Chemical Works of Gedeon Richter Ltd. It induces the mixed function oxydase enzyme system of the endoplasmic reticulum of the liver and has no other pharmacological effects. We have studied the effect of Zixoryn on early hyperbilirubin-aemia. 42 neonates were studied, 21 of them were randomly assigned to be treated and the others served as control group Zixoryn treatment consisted of drops containing 10 mg Zixoryn per ml in a single 20 mg/kg body weight dose through a gastric tube. Results are summarized in Fig. 2. It shows the mean se bi levels during the first six days of life. It is remarkable that the decline of se bi level was much faster in the treated than in the control group. On the third day the difference between the two groups was significant. We may conclude that after Zixoryn administration the se bi level of otherwise healthy newborns decreased significantly faster than that of untreated neonates. No side-effects what so ever were observed. The administration is easy, a single oral dose has a satisfactory effect.

Benzhydryl Compounds↗

Diagnostic value of C-reactive protein in premature babies weighing less than 1500 g.

Quantitative estimation of C-reactive protein was carried out in 34 premature infants weighing less than 1500 g. An increased value proved to be a sensitive indicator of infection. Higher values seemed to be more reliable than low ones. The clinical diagnosis showed a good correlation with CRP positivity and its quantitative value.

Bacterial Infections↗

A semiquantitative score system for epidemiologic studies of fetal alcohol syndrome.

A total of 464 children of 323 women registered for alcoholism treatment in Budapest, Hungary, were studied in 1977-1979. A complex epidemiologic investigation was carried out using medical, psychological and anthropological data. The data were evaluated on the basis of a semiquantitative diagnostic scoring system for fetal alcohol syndrome. The score distribution curve for 301 children whose mothers imbibed during pregnancy was statistically significantly different from the score distribution curve for 163 children born to alcoholic mothers who remained abstinent during pregnancy and from the curve for a matched control group of 464 children. A significant difference was also found between the score distribution curves for the latter two groups. Twenty-five children of 301 drinkers (8.3%) scored below -30 points and were said to show typical manifestations of the syndrome. All of the mothers in this group imbibed large amounts of alcohol during pregnancy. A further 205 children of 464 alcoholic mothers (44.2%) scored between -30 and -10 points and were diagnosed as having an atypical form of the syndrome. Among the 205 children, 168 were the offspring of 301 drinkers (55.8%) and 37 were the offspring of 163 alcoholic females who were abstinent during pregnancy (22.7%). A stepwise discriminant analysis showed the best discriminating variables--in order of entry into the discriminant functions--to be current weight, nose-upper lip distance, behavioral disturbance (irritability), root of the nose, intelligence quotient, and palpebral fissure. The most important cause of fetal alcohol syndrome is the direct toxic effect of alcohol on the fetus.

Adult↗