[Advocacy for pediatric care]. / Politische Kindermedizin: Für eine bessere Zukunft aller Kinder.

Advocacy for pediatric care ("Politische Kindermedizin") is an Austrian association of engaged pediatricians, aiming at identifying, analyzing and evaluating pressing issues of pediatric care and to suggest improvements to the responsible politicians. The aim was and is to achieve an improvement of pediatric care for children and adolescents in the sense of patient advocacy. The history and concerns of the initiative "Politische Kindermedizin" from 1997 until the present are presented.

[Intracerebral calcifications associated with intrathecal methotrexate therapy in acute lymphocytic leukemia (author's transl)]. / Methotrexatbedingte intrazerebrale Verkalkung bei Leukämie.

A case of a child with acute lymphocytic Leukemia is reported. The roentgenograms of the skull show intracerebral "railroad-track-like" calcifications mimicking those seen in Sturge-Weber syndrome. These calcifications are described and their etiology is discussed. The frequency of such observations in cases of acute lymphocytic leukemia with CNS-treatment is increasing.

[Antibodies against the Epstein-Barr Virus nuclear-antigen in children with malignant disease (author's transl)]. / Antikörper gegen das Epstein-Barr-Virus-Nuclear-Antigen bei kindlichen Malignomen

Sera of 25 patients and 50 health children were tested for the presence of antibodies against the Epstein-Barr Virus Nuclear-Antigen (EBNA). We examined 15 children with acute lymphocytic leukemia, who showed a mean arithmatic titer of 1 : 5.33, 1 child with acute myeloblastic leukemia and 1 child with the chronic myeloblastic form showing no reactivity. Two children with lymphogranuloma had titers 1 : 16 and 1 : 32, respectively. 1 patient with lymphoepithelial carcinoma and 5 with lymphosarcoma showed a mean arithmetic titer of 1 : 170.66. Control persons had antibodies against EBNA in the amount of 1 : 2.56. We think that the humoral reactivity against this regularly viral-genome-associated antigen is an additional indicator for the causative role of the Epstein-Barr Virus.

[Cerebellar vincristine toxicity (author's transl)]. / Zerebelläre Vincristin-Toxizität

The diagnosis of Wilms tumor stage IV was established in a girl of 5 1/2 years of age in July 1972. Nephrektomy was followed by radiotherapy of the tumorbed and the lung metastasis. Thereafter the child was treated with Vincristine (VCR) and Actinomycine D (AMD) for two years. The child has been off treatment for the last 14 months and is free of disease. An intention tremor of the right hand was noted 4 weeks after the 32nd application of VCR and 2 weeks after a viral infection of unknown etiology. The writing proved to be hypermetric. There was no loss of proprioception and thus the symptoms were localized into the right cerebellum. Having excluded other causes (hemorrhage, metastasis) as far as possible and considering unilateral cerebellar encephalitis as very unlikely the cerebellar symptoms were thought to be VCR toxicity.

[Investigations on the immune response against meningococcal polysaccharide in children (author's transl)]. / Untersuchungen über die durch Meningokokken-Polysaccharid ausgelöste Immunantwort bei Kindern.

Children between the 2nd and 5th, respectively between the 7th and 10th year were immunized with meningococcal vaccine A + C in comparison to adults. The vaccines contained 50 micrograms of meningococcal polysaccharide A and C and fulfilled the requirements of the WHO. Antibodies were determined before and 4 weeks after vaccination by means of the bactericidal microassay. All age groups showed a distinct immune response to polysaccharide A and polysaccharide C. The mean titer increase was between 4.5 and 9.3 titer steps, the conversion rate ranged from 71% to 100%. In the youngest age group a positive correlation between age and mean titer increase was found in prevaccinal seronegative children as well as in prevaccinal seropositve ones. In all age classes prevaccinal seronegative children showed a significant higher titer increase than prevaccinal seropositive ones. A negative correlation was found between prevaccinal titers and titer increase in all age groups with polysaccharide A and polysaccharide C.

[Sonographic demonstration of an unusual bladder wall enlargement in a child with non-Hodgkin lymphoma]. / Sonographische Darstellung einer ungewöhnlichen Blasenwandverbreiterung bei einem Kind mit Non-Hodgkin-Lymphom.

An enlargement of the wall of the urinary bladder up to 1.5 cm is described within the course of a Non Hodgkin Lymphoma of a 15 years old girl. The development of a spastic neurogenic bladder is most probable due to tumour infiltration into the spinal cord. An additional factor for the enlargement of the bladder wall is a massive fungus cystitis. A cystotatica induced (Vindesin) neurogenic bladder as another cause for the development of this bladder change cannot excluded completely, but this is very unprobable. The cystography is the method of choice in the morphological evaluation of the bladder mucosa, whereas the sonography can visualize the bladder wall directly. This method has a high value in the diagnosis and follow up of pathologic changes of the urinary bladder in children.

[Vaccination of infants and schoolchildren with an influenza subunit vaccine (author's transl)]. / Impfung von Klein- und Schulkindern mit einer Influenza Subunit Vakzine.

A new influenza subunit vaccine which contains only hemagglutinin and neuraminidase antigens was investigated for reactogenicity and immunogenicity in children aged between three and 15 years. Children under six years of age received either 500 IU or 1000 IU of the commercial vaccine, those aged from six to 15 years either 1000 IU or 2000 IU. The vaccines contained the virus strains recommended by the World Health Organisation for the vaccination season 1976/77. In a double blind study the vaccinees were allocated at random to the different dosage groups. The children were examined for reactions by the vaccinating physician 24 hours after vaccination. Serum hemagglutination inhibiting antibody titers were determined before vaccination and four weeks after vaccination. In the younger age-group additional antibody determination was made two weeks after a booster injection. A very low rate of side-reactions was observed in all dosage groups. The increase of the antigen content was not associated with a higher rate of side reactions. After the first vaccination a significant rise of antibody titers could be observed in all children. After the booster injection a further increase of these antibody titers was observed. The response of the younger age group to the dosages 500 and 100 IU did not different significantly. In contrast, in the older age group the increase of the dosage from 1000 to 2000 IU was connected with a better immune response. This was especially marked in the antibody titers against the influenza B-strain virus.

[Systemic lupus erythematosus in twins (author's transl)]. / Zum Lupus erythematodes visceralis anhand einer Beobachtung bei Zwillingen.

A report is given of systemic lupus erythematosus (SLE) in monozygotic twins. One sister developed coombs-positive haemolytic anaemia at the age of 11 years and suffered from five haemolytic crises over a period of 8 months. SLE could not be proven at that time, but following a 3-year symptom-free period, characteristic clinical and immunological evidence of SLE became manifest. The second sister developed classical SLE at the age of 15 years. Clinical, immunological, etiological and therapeutical aspects of SLE are being discussed.

[Concept for the total care of families of children with leukemia and tumors based on conversations with the parents]. / Konzept zur Ganzheitsbetreuung von Familien leukämie- und tumour- kranker Kinder, basierend auf Gesprächen mit den Eltern

In order to create a foundation for prophylactic and therapeutic measures the problems of children with leucaemia and other malignancies and of their families were assessed in detail with parents of these children in single or group discussions. The necessity for integrated therapy including families of children with leucaemia or tumours became apparent. Care should include extensive talks with all family members and also with teachers of patients and of healthy siblings, the use of aids such a painting or puppet theatre for recognition of problems and needs, particularly in small children, home visits to explore problems of health siblings, involvement of social workers, aftercare for the whole family after the death of a child, and, finally the help for overcoming problems of the treating team. Care to such an extent has been found to be necessary and requires an increase in posts in oncological units. Lack of time for the patients as human beings is unacceptable even from an economical point of view. A psychotherapeutically versed medical doctor or a clinical psychologist and a social worker should be permanent members of any oncological team.

[Oculocerebrovertebral syndrome]. / Okulozerebrovertebrales Syndrom

After description of the oculovertebral syndrome Weyers-Thier a personal observation is reported. It was characterized by severe cerebral malformations (agenesis of the olfactory lobes, incomplete agenesis of corpus callosum with large sagittal arachnoidal cyst) associated with polycystic brain damage. The relationship of this "oculovertebral syndrome" to the "oculoauriculovertebral syndrome" is discussed. The combination of various malformations in our observation indicates an embryonic lesion between the 4th and 11th week. Additional polycystic brain damage was due to perinatal anoxy. The causal factor of the malformative syndrome remains unknown, but similar lesions were experimentally induced by fetal hypoxia.

Coexisting suppressor cells for myeloid colony formation in the bone marrow of patients with aplastic anaemia.

Suppressor cells for colony forming cells (CFUC) were found in the bone marrow of 1 of 6 patients with aplastic anaemia. The assay applied was based on the fact that coexisting suppressor cells are more sensitive to dilution than CFUC, as long as adequate stimulation is provided by a source of colony stimulating activity. In the patient with suppressor cells, a 4 d trial of 1000 mg methylprednisolone per d failed to improve the blood cell production and had to be discontinued due to gastric irritation.

[Results of neuroblastoma treatment in Austria (1979-1984)]. / Ergebnisse der Neuroblastombehandlung in Osterreich (1979-1984).

During the period from 1979 until 1984 55 children were registered: 4 in Evan-stage I, 13 in stage II, 16 in stage III, 18 in stage IV and 4 in stage IV-S. 39 of these 55 patients have been followed for a minimum of two years. The current two year survival rate is 10/10 in stages I and II, 6/13 in stage III, 3/14 in stage IV and 2/2 in stage IV-S. These results demonstrate but a slight improvement in patients with stage II since the last Austrian statistic 1974. Despite modern chemotherapy the chance for survival of children with stage III or IV neuroblastoma is poor and better treatment modalities should be searched for.

[Axis deviation of lumbar spine due to abdominal expanding lesions in children (author's transl)]. / Anderungen der Lendenlordose bei abdominellen Raumforderungen im Kindesalter.

The deviation of the physiologic lordosis of the lumbar spine is described in 40 cases of abdominal expanding lesions. One can find a lumbar kyphosis between L2 and L4 in retroperitoneal and a straightening between Th12 and L2 in intraperitoneal lesions. The evidence of a deviation of the lumbar axis is an indirect sign for further localisation of the tumor.

[Diagnostic and therapeutic problems of nasopharyngeal carcinoma in childhood (author's transl)]. / Diagnostische und therapeutische Probleme des Nasopharynxkarzinoms im Kindesalter anhand von drei Patienten.

Between 1974 and 1978 three children with a nasopharyngeal carcinoma (stages: T3N3MO, T4N2MO, T4N1MO) were treated with primary radiotherapy (tumor dose: 45-67 Gy). Local control was achieved in two children, one of these (T4 primarily) had a local relapse five months after diagnosis. Only one of the three children had radiotherapy to the neck region. This child developed lung and mediastinal metastases with hypertrophic osteoarthropathy twelve months after diagnosis. In the other two children lung metastases were observed two and seven weeks after the diagnosis respectively. In these two radiotherapy of metastases and chemotherapy were considered more important than radiotherapy of the neck region. All three children showed disappearance or considerable reduction of lung metastases due to radio- and chemotherapy. Yet, all three died of progressive disease within a few months. Early diagnosis of nasopharyngeal carcinoma in children will only be possible when pediatricians are more aware of this disease, also in our regions. Radiotherapy should be done only with megavoltage equipment using extensive radiation fields for the primary tumor area and elective radiation of the neck region. The use of additional primary chemotherapy is suggested for stages T3/T4. This mode of management may help to prevent distant metastases.

[Protein malnutrition in pediatric patients with oncologic diseases]. / Protein-Malnutrition bei pädiatrischen Patienten mit onkologischen Erkrankungen.

To study the frequency of protein-caloric malnutrition in children (n = 16) with malignant diseases, we measured serum-albumin, -transferrin, -prealbumin and -retinol-binding protein (Radial immunodiffusion) before treatment was started. In 9 children at least 3 of the 4 rapid-turnover proteins were below the age-specific reference values. 7 patients (44%) with low rapid turnover proteins were free from infection, liver and renal dysfunction and thus, diagnosis of protein-caloric malnutrition was established. Anthropometric parameters as weight for age and the weight/height ratio did not predict protein-caloric malnutrition. Nutritional therapy (enteral, parenteral) during a period of 2 weeks resulted in significant increase of albumin (p less than 0.05), prealbumin (p less than 0.01) and retinol-binding protein (p less than 0.01). Rapid turnover proteins can be easily measured in each laboratory and are useful in diagnosis and treatment of protein-caloric malnutrition of children with malignant diseases as long as the limitations of this method are not neglected.

Results of LSA2-L2 therapy in 26 children with non-Hodgkin's lymphoma.

Twenty-six children with non-Hodgkin's lymphoma (NHL), 17 boys and nine girls, were treated with the LSA2-L2 protocol. Seven children had stage I or II, 16 Stage III and three Stage IV according to Murphy's staging system. Eight children had their primaries in peripheral lymph nodes, eight in the abdomen, six in the mediastinum and four in other sites. All tumors were classified histologically according to four different classifications. Overall disease-free actuarial survival is 53.6%. Complete responders show a disease-free survival of 77.8%. Fourteen children survived for 9-56 months. Included are all seven children with Stage I or II who survive irrespective of histologic type of the tumor. Of the remaining 12 children in Stage III or IV three children died in remission and nine of progressive disease. Eight of these nine patients did not attain complete remission. Whereas four of five children with the convoluted type of NHL survive, four of five patients with the Burkitt's type (small noncleaved follicle center cell lymphomas) died of progressive disease. According to Rappaport's classification, four of six children with diffuse undifferentiated lymphoma (DUL) are dead due to tumour progression. Considering the classification of lymphoblastic lymphomas introduced by Nathwani et al., 23 five of seven children suffering from lymphoblastic lymphomas but only two of eight children with nonlymphoblastic lymphomas belong to the survivors. Therefore histologic findings do hold prognostic significance in our series of children with NHL.

Hepatitis B vaccination and immune response in children with malignant diseases.

Fifty children with malignant diseases were vaccinated against hepatitis B. Twenty-nine children suffered from leukaemia or non-Hodgkin's lymphoma; 14 of these were on intensive chemotherapy (group I) and 15 were without intensive therapy (group II). The other 21 children had various forms of solid tumours, 14 of them were on intensive therapy (group III) and 7 were without intensive therapy (group IV). To evaluate the immune response, we determined antibody titres over a period of more than 14 weeks after the first vaccination. As 22 out of 50 patients had received passive immunisation together with either the first or the first and second vaccination, antibody titres at the 14th and 18th week (i.e. more than 10 weeks after passive immunisation) were used to evaluate the vaccination results. An antibody titre of greater than or equal to 10 mIU/ml was considered to be a positive response. All patients of group IV, but only 4 out of 14 in group III, 4 out of 15 in group II, and 0 out of 14 in group I produced antibody titres higher than 50 mIU/ml. In contrast to the full response in group IV, two-thirds of all other patients had no immune response (less than 10 mIU/ml). Based on our experience we recommend vaccinating patients suffering from solid tumours and receiving no intensive therapy (group IV) against hepatitis B and protecting all the other children with malignant diseases by passive immunisation, if necessary.

[Treatment of Wilms' tumor (author's transl)]. / Therapie des Wilms-Tumors.

Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.