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The essential effects of Tocopherol are based on its antioxidative capacity. Tocopherol, however, is just one in a group of antioxidants, which are important for the organism. Established indication for therapeutical application of vitamin E in infancy is only vitamin-E-malabsorption in connection with chronic cholestasis, pancreatic insufficiency (cystic fibrosis) and short bowel syndrome. In emergency therapy vitamin E is suggested with high dosage in case of shock lung and haemolytic-uraemic syndrome. Positive effects of daily vitamin E application in connection with prophylaxis of retinopathy prematurity, bronchopulmonary dysplasia and intraventricular encephalorrhagia of premature infants of severe underweight are not established. Very questionable therapeutic or prophylactic efficiency is opposed to the risk of higher incidence of severe complications in caring for premature infants of severe underweight, such as enterocolitis necroticans and neonatal septicaemia.

About 15% of children with alpha-1-antitrypsin-deficiency with proteinase inhibitor type ZZ develop hepatopathy, uninfluenceable in its course. These children already show symptoms of severe cholestatic hepatitis in early infancy as became obvious from data of 13 children being patients in the authors care and suffering from hepatic cirrhosis with alpha-1-antitrypsin-deficiency. At present liver transplantation is the only causal possibility of therapy. Even without highly specialized laboratory the non-laboratory assistant will recognize at least the homozygous alpha-1-antitrypsin-deficiency (PI-ZZ). The therapeutic approach must be directed on treating the patients in such a way that liver transplantation will be possible at a favourable moment and under good conditions. Since PI-ZZ family members suffer similar course of hepatopathy, genetic counsel is of special significance.

Kinder mit Alpha1-Antitrypsinmangel-Hepatopathien, Autoimmun-hepatitiden und mit extrahepatischer Gallengangsatresie werden unter der Zielstellung betreut, ihnen im Falle der Leberinsuffizienz eine Lebertransplantation zu ermöglichen. Unter den Laborparametern, die die Leberzellinsuffizienz signalisieren, haben der Anstieg des indirekten Bilirubin sowie der Abfall der Cholinesteraseaktivität und des Quickwertes besondere Bedeutung. Es gelang bisher nicht, die an longitudinal untersuchten Patienten gewonnenen Daten im Sinne einer Verallgemeinerungsfähigkeit zur Vorhersage der Prognose schwerer Hepatopathien zusammenzufassen.

Von 1980 bis 1984 wurden 48 Kinder mit einer portalen Hypertension stationar behandelt, davon hatten 37 einen prahepatischen Block. Bei 19 Kindern erfolgte die Varicensklerosierung. Zu Rezidivblutungen kam es 7mal und 2mal trat eine Oesophagusstenose auf. Der distale splenorenale Shunt (Warren) wurde 16mal angelegt. Alle Kinder uberlebten, postoperativ trat eine Pneumonie und eine leichte Pankreatitis auf. Viermal wurde bei der Nachuntersuchung ein Shuntverschlus gefunden. Blutungsrezidive traten nicht auf.

Kinetics of propranolol after oral administration were investigated in 9 children aged 5--11 years with intact liver function who had undergone porto-systemic anastomosis surgery for pylethrombosis and in a 16-year-old boy with a Warren shunt. Bioavailability of the drug had increased by twice the values measured in physiologic liver blood flow; elimination half-life had doubled. These alterations must be taken into consideration in medication, e.g. extreme care must be exercised in the oral administration of "first pass" drugs in porto-systemic shunt patients and it is recommended that the initial dose should be at the level of intravenous administration. The reduced rate of elimination should be taken into account in the case of drugs whose elimination depends on liver blood flow.

31 children aged 2,5 to 14 years suffering from portal hypertension underwent shunting operations (cavosplenal and cavomesenteric anastomoses). The follow-up period covers 2 months to 8 years. Patent anastomoses could be demonstrated in 26 patients.

From 1980 to 1984 fortyeight children with portal hypertension were treated, 37 out of these had a prehepatic bloc. In 19 children a sclerotherapy was performed. Seven times a bleeding recurrency occurred and an esophageal stenosis was seen in 2 cases. The distal splenorenal shunt (Warren) was performed 16 times. All children survived, one pneumonia and one slight pancreatitis were observed post-operatively. Four times a shunt obstruction was found at follow-up examinations. Bleeding recurrencies did not appear.

When clinical evidence provides grounds for suspecting inborn errors of metabolism it is urgent to perform the necessary, relevant, specific laboratory investigations in good time and with a view to quality. Normally, the realization depends on individual initiatives and the use of laboratories mainly designed for pediatrics and human genetics. Consequently the results are equally a matter of chance. Nothing in this situation can be changed in principle by using the catalogue of services of the Society for Human Genetics of the GDR. Central administrative provisions are necessary to improve the present unsatisfactory situation. Proposals for regulations, division of responsibility and a graduated programme of parameters are discussed here with a view to establishing uniform procedures.

On the basis of the occurrence of myopathy in a clofibric acid treated child with diabetes insipidus, three other children with the same diseases were examined in order to find indications for a myopathic side-effect of the drug. When the children were found to have increased creatine phosphokinase activity and EMG changes, two of the authors took clofibric acid themselves. In both test persons subclinical myopathy was produced. After stopping the drug, transitional hypertriglyceridaemia occurred. These side-effects should serve as a warning of an uncritical application of clofibric acid and its esters.

In Europe more than 70 per cent of children with portal hypertension suffer from a prehepatic block. Most of them underwent an umbilical vein catheter treatment in postnatal anamnesis. Constructing a selective distal splenorenal shunt (Warren-shunt) is a definitive therapy. This operation is possible for children having a patent splenic vein with a diameter of at least 7 mm measured by splenoportoseriography. Sclerosing of the oesophageal wall is a good palliative treatment for small children with a diameter of the splenic vein of less than 7 mm. Children with prehepatic block and patent Warren-shunt have a good prognosis.

Die Entfernung der Milz ist prinzipiell mit dem Leben vereinbar. Die Patienten sind jedoch lebenslang mit einer erhohten Infektionsmorbiditat belastet, da der Verlust der milzspezifischen immunologischen Funktion (Tabelle 1) durch das ubrige RHS nur partiell kompensiert werden kann. Durch zahlreiche Untersuchungen sind die Veranderungen im Immunstatus nach Splenektomie belegt: Serumimmunglobuline [1, 4, 8], Fibronectinspiegel [9], Veranderungen in der Lymphozytensubpopulation [4, 10], in-vitro-Stimulierbarkeit der Lymphozyten durch Mitogene [3, 11]. Die Uneinheitlichkeit, teilweise widerspruchlichen Ergebnisse dieser Untersuchungen betonen den Einflus der zur Splenektomie fuhrenden Grunderkrankung und der Zeitspanne zwischen Operation und Erhebung des Immunstatus. Das Risiko fur infektios-septische Komplikationen nach Splenektomie ist bei Kleinkindern am grosten. Fatale Ausgange einer postoperativen Pneumokokkensepsis sind in dieser Altersgruppe haufiger anzutreffen als im Schulkind- bzw. Adoleszentenalter. Diese bekannte Tatsache wird durch eigene Beobachtungen einer „overwhehning post-splenectomy infection“ (OPSI) bei zwei Kleinkindern mit portaler Hypertension unterstutzt. Nach derzeitigem Kenntnisstand bietet allein die orthotope Praservation von Milzgewebe einen sicheren Schutz vor septischen Pneumokokkeninfektionen [3].

The aim of this paper was to demonstrate the efficiency of partial splenic resection in treatment of chronic idiopathic thrombocytepenie purpura (Werlhof-Disease). In all but one case remissions of thrombocyte counts over 100 Gpt/l could be achieved. Immunological studies demonstrate the existence of a suppressor-state after this operation, which could be responsible for success of this procedure. In contrast to general considerations the partial-splenectomized child must be considered as an immunological deficient organism for about 6 to 12 months. Restoration of immunological-means whole splenic functions-parameters is to expect always after this time.

Children suffering from alpha 1-antitrypsin deficiency hepatopathies, autoimmune hepatitis and from extrahepatic biliary atresia are cared for with the aim of rendering possible a liver transplant in case of liver insufficiency. Among the laboratory parameters signalling hepatic insufficiency the increase in the indirect bilirubin as well as the decrease of cholinesterase activity and the Quick are of great importance. So far it has not been possible to generalize from the data obtained from the longitudinal examination of patients in order to predict the prognosis of severe hepatopathies.