[Phenylalanine hydroxylase activity in the liver as a parameter for distinguishing various forms of hyperphenylalaninemias (author's transl)]. / Phenylalaninhydroxylase-Aktivität in der Leber als Parameter zur Unterscheidung der verschiedenen Formen der Hyperphenylalaninämien

The activity of phenylalanine hydroxylase in the liver without the cofactor 2-amino-4-hydroxy-6,7-dimethyl-5,6,7,8-tetrahydropterin is similar in cases of classical PKU (14 cases), mild hyperphenylalaninemias (2 cases), heterozygotes (1 case) and normal controls (18 cases). The addition of cofactor increases the activity 30-40-fold in normal controls and 2-5 fold in mild hyperphenylalaninemias or heterozygotes. In classical PKU the addition of cofactor is without effect or causes even a diminution of activity. Therefore, for classification of the various forms of hyperphenylalaninemias the effect of cofactor on enzyme activity is important. For decisions from this findings to be made as to whether treatment should or should not be used further investigations are needed.

[Acute and chronic viral hepatitis in childhood. A review]. / Akute und chronische Virushepatitiden im Kindesalter - Ubersicht.

The author reviews nomenclature, pathogenetic agents, immunological markers and mode of infection of hepatitis in childhood. Epidemiology, incubation period, prejaundiced phase, clinic and course in regard to different types of viral hepatitis are characterised. Especially jaundiced and nonjaundiced courses, biochemical parameters immunological diagnosis of hepatitis type A and type B respectively are emphasized together with histological and histologic-immunological criteria. Therapy and prophylaxis of A- and B-hepatitis are discussed. The author reports on the chronic, chronic-persisting and chronic-active form of the hepatitis including immunological markers, auto-antibodies and therapy.

[Current aspects of the sudden infant death syndrome]. / Aktuelle Aspekte zum Syndrom des plötzlichen Kindestodes (SIDS).

SIDS is the most frequent cause of death in infancy outside the neonatal period. The pathogenesis of the disease is not clearly understood. Risk factors associated with SIDS can be defined. At present, the hypothesis of apnoea plays a major role in the pathogenesis. Findings show a central nervous dysfunction with an interruption of brain stem regulation of respiratory and cardiac function. In an emergency, the physician must carry out appropriate measures. Prophylaxis is possible in infants at risk. The active involvement of parents plays a decisive role in the prophylaxis of SIDS.

[Recurrence of congenital mesoblastic nephroma. Case report]. / Rezidiv beim konnatalen mesoblastischen Nephrom. Fallbericht.

The congenital mesoblastic nephroma is a tumour with a low malignant potential. In this case report the clinical course as well as histological and postmortem findings of the recurrence are presented. The hitherto known recurrences of this lesion are mentioned.

[The effect of combined ALL therapy in childhood on body growth]. / Zum Einfluss der komplexen ALL-Therapie im Kindesalter auf das Körperwachstum.

Female ALL long-term survivors who received cranial telecobalt radiation under the conditions of Therapy Study III of the Study Group on "Paediatric Haematology/Oncology" showed a significant reduction in adult height. On completion of therapy a drop in growth can already be observed in female ALL patients. This limitation on growth can probably be attributed to a disturbance in the hypothalamus-hypophysis system and to reduced GH secretion as a result of radiation.

[Mitotic index in the bone marrow of children during the clinical course of acute lymphoblastic leukemia (ALL)]. / Zum Mitoseindex im Knochenmark bei Kindern im Krankheitsverlauf der akuten lymphoblastischen Leukose (ALL).

From 31 children with acute lymphoblastic leukaemia the mitosis index in the bone-marrow was determined before the onset of therapy and during the clinical progress. Initially, the mean white mitosis index lay with 3.4% below that of the normal test persons, it rose significantly in the hematologic full remission and showed a decreasing tendency with a great range of dispersion in the recidive. The most lowered mitosis index was found in the final stage. Strong shifts in the kariologic distributions make a remaining in the prophase of the mitosis evident. The influence of polychemotherapy on the mitosis index and the phases of mitosis is discussed. Correlations between the mitosis index and clinical as well as paraclinical parameters were only to be found with respect to granulocytes and lymphoblasts. The considerable ranges of fluctuations of the mitosis index and the lack of congruity with the clinical progress of ALL allow no ensured assertions to be made for the single patient. It seems to be important in eosinophilia and in leukaemoid reactions.

[Quantitative behavior of eosinophil granulocytes in the bone marrow of children with acute lymphoblastic leukemia]. / Quantitatives Verhalten der eosinophilen Granulozyten im Knochenmark von Kindern mit akuter lymphoblastischer Leukose.

Of 31 children affected with acute lymphoblastic leukaemia the quantitative behaviour of eosinophilie granulocytes was examined in the course of the disease. Nearly all patients were treated according to a chemotherapy scheme (Memphis IV). During this therapy the eosinophils greatly diminished initially increased significantly to subnormal values and to the values of healthy persons with persisting full remission. Another significant decrease occurred during the relapse and in the pre-final stage. During each following relapse a greater diminution of bone-marrow eosinophils could be observed. Simultaneously the decrease of eosinophils led to a shift in the degree of maturation. In this connection the similar behaviour of neutrophilic and eosinophilic granulocytes of the bone-marrow must be stressed. Eventually, the lbast excrescence in the bone-marrow and its therapy cannot solely be decisive for the findings made. Relations to the lymphocytic system can be referred to.

[Intracranial metastases in neuroblastomas]. / Intrakranielle Metastasierung beim Neuroblastom.

Primary extracranial neuroblastomas frequently metastasize into calvaria, orbita and dura. A direct infiltration is known as secondary metastases to the brain, if the lesion penetrates the bone and dura. Primary metastases to the brain without lesions of the calvaria or dura were observed rarely in extracranial neuroblastomas very interesting with respect to the pathway of metastases. Only a in one of our cases a secondary metastases to the brain could be seen among 21 autopsies. A primary metastases to the brain was diagnosed in a child with a presacral neuroblastoma in the true pelvin. No extracranial metastases could be found, further cases are reviewed from the literature.

Evaluation of the erythrocyte-UFA (E-UFA) mobility test for the diagnosis of multiple sclerosis.

The absolute electrophoretic mobility of red blood cells was examined before and after the addition of linoleic acid. The determinations were performed with a Zeiss cytopherometer. The blood was taken from 22 patients with multiple sclerosis in different phases of their disease. A group of 17 patients with other neurological diseases and three apparently healthy subjects served as controls. Our results show that the mobility of red blood cells was either accelerated or inhibited in the presence of linoleic acid, both in the multiple sclerosis patients as well as in the control group. The increase or inhibition of the absolute mobility was small and randomly distributed. The value of this so-called E-UFA test (erythrocyte-unsaturated fatty acid) as a specific diagnostic test for multiple sclerosis is not confirmed by our results.

[Congenital mesoblastic nephroma in infancy--survey of 74 cases with regard to recidivism and therapeutic results (author's transl)]. / Konnatales mesoblastisches Nephrom im Säuglingsalter--Ubersicht zur Rezidivhäufigkeit und zu Therapieergebnissen von 74 Fällen.

The congenital mesoblastic nephroma is clearly distinguishable from Wilms' tumour. Almost exclusively it becomes manifest in early infancy. From the literature 71 established cases were complied, 3 own cases have been added. By many authors this tumour was characterized as benign. There has been, however, a total of 7 recurrences. An epicritical review is given of the course of one own case. The complications arising from radio-and chemotherapy clearly indicate that surgery is the method of choice of semi-malignant tumour.

[Long-term survival following acute lymphoblastic leukemia (ALL) in childhood]. / Langzeitüberlebende nach akuter lymphoblastischer Leukose (ALL) des Kindesalters.

48 patients with ALL (3%) treated according to two different protocols survived 5 years or longer. The mean survival time was 13.0 +/- 5.0 years, but 29 patients lived 16.5 +/- 3.0 years. Only 18 patients (38%) were 16.9 +/- 3.0 years in them 1. CCR, the longest remission time amounted to more than 25 years. Of the patients relapsed 9 are 8.4 +/- 2.8 years in 2. CR. Late relapses after 5 years were observed in seven cases the last in the 9th year of disease. After 5 years the survival rate in both protocols was not different. As a late sequelae, there was one patient with intrahepatic block and portal hypertension and one with encephalopathy and imbecility. All patients were able to leave their professional examination, 5 patients married and 6 healthy children were born.

[Case observations on the significance of early pregnancy factor as a tumor marker]. / Kasuistische Betrachtungen zur Bedeutung des Early Pregnancy Factor (EPF) als Tumormarker.

At three patients who have been operated on because of completely different tumours (trophoblast tumour, endodermalsinus tumour of the right ovary and rhabdomyosarcoma as well as adrenal cortex carcinoma) pre-operatively the Early Pregnancy Factor (EPF) could in each case be shown in the serum with the help of the rosette inhibition test. In two cases the EPF already intra-operatively disappeared from the serum. In the third case it could be found until the second day after operation. Since that patient suffered from two histogenetically non-related malignant tumours it was not possible to trace whether the adrenal cortex carcinoma or the rhabdomyosarcoma have been the reason for the EPF-synthesis. The already intra-operative disappearance and the also during further post-operative controls unsuccessful proof of EPF in serum can indicate an opportunity for the early assessment of efficiency of the surgical treatment of tumours which develop EPF or which induce the EPF-synthesis.

[Fibrous hamartoma in the infant]. / Das fibröse Hamartom des Kleinkindes.

Fibrous hamartoma is a rare example of juvenile fibromatoses. We report a case of an 11 month old infancy with presenting of clinical picture, morphological findings and therapy. Biological behaviour, differential diagnosis and pathogenetic connections to other fibromatoses were discussed.

[Intrathecal methotrexate--overdosing in pediatric lymphoblastic leukemia]. / Intrathekale Methotrexat--Uberdosierung bei kindlicher lymphoblastischer Leukose.

Intrathecal methotrexate-overdosage in the meningosis therapy leads to CNS damages dependent on dose. It was given a own case report and a literature review with clinical symptoms and the therapy in this complication. Neuropathologic findings included demyelinisation und necrosis of the white matter.

[Prefinal clinical findings and results of autopsy in 82 children with acute lymphatic leuckemia (ALL) under various courses of therapy]. / Präfinale klinische Befunde und Autopsieergebnisse von 82 5indern mit akuter lymphatischer Leukose (ALL) bei unterschiedlichem Therapieregime

At present 80...90 % of the patients with acute lymphatic leukaemia die in the blastic crisis. About 10% will come ad finem during full remission caused by side effects of the treatment and their complication. The leukaemic terminal crisis may be accompanied or overlapped by a number of complications, the most frequent among own patients being acute bleeding in the terminal phase. First of all the source of bleeding is to be found in the gastro-intestinal tract (80%). Other authors found infections to be the most frequent final cause of death. It is only under autopsy that leukaemic infiltrates, infections and bleeding are completely recognized to their full extent. After polychemo-therapy the patients showed a significant increase of complications including pulmonary oedema and a marked insufficiency of the bone-marrow with leukocytopenia and granulocytopenia in the peripheral blood. Among the biochemical parameters only a generally significant increase of alpha2 and gamma globulins could be found in the serum. A correlation towards a form of therapy could not be ensured.

[Intrathecal radiogold prophylaxis and the cerebrospinal fluid findings in children with acute lymphoblastic leukemia]. / Intrathekale Radiogoldprophylaxe und Liquorbefunde bei Kindern mit akuter lymphoblastischer Leukose.

Intrathecal radiogold application represents an alternative to the prophylaxis of meningosis in childhood acute lymphoblastic leukemia. Its compatibility is good, there are rarely any clinical side-effects. In addition to inconstant phagocytosis, changes of the protein value and its fractions could be identified in the cerebrospinal fluid. The cumulative rates of remission and survival are less marked in these groups of patients than in those who received a prophylactic skull irradiation.