[The use of vitamin E in childhood]. / Zum Einsatz von vitamin E im Kindesalter.

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.

[Liver diseases in alpha 1 antitrypsin deficiency syndrome in children]. / Lebererkrankungen bei Alpha-1-Antitrypsinmangel-Syndrom im Kindesalter.

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.

[A staged plan for laboratory diagnosis of hereditary metabolic diseases]. / Stufenplan zur Labordiagnostik hereditärer Stoffwechselerkrankungen.

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.

[Variations in the composition of breast milk in Wilson's disease]. / Abweichungen in der Zusammensetzung der Muttermilch bei Morbus Wilson.

Wilson's disease is a rare genetic disorder of copper metabolism with autosomal recessive inheritance. It occurs between the 6th and 45th year of life. An early and reliable diagnosis, if possible in the preclinical stage, is the prerequisite for starting therapy in time. By the treatment the quality of life and the expectation of life are raised considerably. If consistent treatment is given, there will be no objections to pregnancy in Wilson's disease. It should be interrupted only in case of marked portal hypertension and in the presence of oesophageal varices. The examination of the breast milk of a patient suffering from Wilson's disease showed a reduction in the trace elements copper and zinc. It may be necessary to think of copper and zinc substitution in children fed with breast milk only.

[Management of children with fatal liver diseases with reference to eventual liver transplantation]. / Die Betreuung von Kindern mit schicksalhaft verlaufenden Leberkrankheiten unter dem Aspekt der eventuellen späteren Lebertransplantation.

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.

[Nutrition in pregnancy]. / Ernährung in der Schwangerschaft.

Review about during pregnancy. Nutrition of a pregnant woman has to cover the maternal need of energy and additionally the stuff for the fetus as preconditions for its growth. In the trimester an extra intake of 15 grams protein is necessary, but no one of fat and carbohydrates. An increase of 400-500 mg Calcium, 10 mg fernum and vitamins A, B1, B2 and C is very important. Smoking, alcohol and drug abuse may be injurious to the fetus. Passive smoking of the mothers, therefore automatically of the fetus, too, is harmful for the fetus. Our recommendations for nutrition of pregnant women demonstrate, that feeding during pregnancy is simple.

Partial splenectomy for treatment of chronic idiopathic thrombocytopenia in childhood.

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.

[Long-term prognosis of portal hypertension in childhood]. / Die Langzeitprognose der portalen Hypertension im Kindesalter.

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.

[Therapy of portal hypertension in childhood]. / Die Therapie der portalen Hypertension im Kindesalter.

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.