CNS late effects after ALL therapy in childhood. Part I: Neuroradiological findings in long-term survivors of childhood ALL--an evaluation of the interferences between morphology and neuropsychological performance. The German Late Effects Working Group.

The effect of cranial irradiation on possible therapy-induced morphological central nervous system (CNS) side effects of children cured from acute lymphoblastic leukemia (ALL) is controversially discussed. In a retrospective multicenter study, 118 former ALL patients in first continuous remission were investigated using cranial computerised tomography (CCT) or magnetic resonance imaging (MRI) scans to evaluate CNS related impairments. Corresponding to the different kinds of CNS prophylaxis, the patient sample was divided: group A (n = 39) receiving intrathecal methotrexate (ITMTX) and systemical medium-high-dose methotrexate (SMHDMTX), group B (n = 41) cranial irradiated (in mean 16.

Screening for neuroblastoma in late infancy by use of EIA (enzyme-linked immunoassay) method: 115000 screened infants in Austria.

The aim of this study was to investigate the feasibility of a neuroblastoma screening programme for children in late infancy, based on collaboration of general paediatricians and practitioners in Austria, using the technique of enzyme-linked immunoassay (EIA) for biochemical analyses. Analysis of catecholamine metabolites in spot urine samples by EIA with high performance liquid chromatography as a backup was undertaken. Austrian infants (median age 8.

Connatal localized neuroblastoma. The case to delay treatment.

Background: Spontaneous regression is well documented for a subset of widespread neuroblastomas (Stage 4S) and for localized residual tumors after incomplete resection. Possible spontaneous regression of untreated localized neuroblastoma in infants is frequently discussed, but has very rarely been demonstrated clinically.

Methods: We report four patients with localized neuroblastomas detected early; all were tumors of the adrenal gland.

[5 years neuroblastoma screening in Austria: rate of participation, results and a comparison with other screening areas].

In late 1990 a screening program for the early detection of neuroblastoma in infants was introduced in Austria. The program is performed on a voluntary basis in collaboration with general pediatricians and practitioners. Filter strips for urine collection are distributed to parents of infants aged seven to nine months on the occasion of a routine check up.

Partial nephrectomy in well-responding stage I Wilms' tumors: report of three cases.

We report on 3 of 21 stage I Wilms' tumor patients with tumor volumes of 190, 890, and 1300 mL, respectively, who responded dramatically to preoperative chemotherapy according to the Austrian/Hungarian Wilms' Tumor Study 89 by tumor volume reductions to 20%, 23%, and 13%, respectively. Radical resection of the tumors with renal preservation was achieved in all patients. Postoperative studies did not show any functional deficit in the preserved kidney.

Adoptive immunotransfer with viable donor mononuclear cells for recurrent chronic myelogenous leukemia after allogeneic bone marrow transplantation in two children.

Two children with Ph+ chronic myelogenous leukemia (CML) relapsed in the chronic phase after allogeneic bone marrow transplantation (BMT). They were treated with transfusions of peripheral blood mononuclear cells (PBMC) obtained from the former bone marrow donors in combination with interferon alfa-2. In one child, CML was successfully controlled as shown by disappearance of Ph+ metaphases as well as negativity for BCR-ABL fusion gene transcripts demonstrated by polymerase chain reaction (PCR) analysis.

Ocular sequelae of multimodal therapy of hematologic malignancies in children.

We retrospectively analyzed the ocular findings after polychemotherapy including intrathecal methotrexate, systemic corticosteroids, and prophylactic cranial irradiation in children with acute lymphoblastic leukemia (n = 16) and non-Hodgkin's lymphoma (n = 2). After a median surveillance time of 4.1 years, asymptomatic ocular abnormalities were observed in 83% of the patients: 7/18 had a decreased tear formation, 5/17 had an opacity of the vitreous body, and 13/18 had an opacity of the lens.

Liposomal amphotericin-B (AmBisome) for treatment of cutaneous widespread candidosis in an infant with methylmalonic acidaemia.

In a 10-week-old infant with vitamin B12-unresponsive methylmalonic acidaemia, cutaneous candidosis (Candida albicans) progressed rapidly despite topical antifungal treatment. After 1 week of intravenous therapy with liposomal amphotericin-B (AmBisome) the dermatitis disappeared completely and blood cultures were sterile. No side-effects were observed.

Treatment of aplastic anemia with a monoclonal antibody directed against the interleukin-2 receptor.

Severe aplastic anemia (SAA) has been related in most cases to underlying autoimmune conditions. Various immunosuppressive regimens have been recommended in the absence of an HLA-identical bone marrow donor. Prednisolone, antithymocyte globulin, and cyclosporin A have been shown to be effective.

The role of sonography in the evaluation of gastro-oesophageal reflux--correlation to pH-metry.

Sonography was compared to pH-metry and/or oesophagomanometry to evaluate the accuracy of sonography in the early diagnosis of gastro-oesophageal reflux. Thirty children with a mean age of 72 days (21-252 days) were studied. The results showed that specificity of sonographic diagnosis was 87.

[Clinical experiences with adrenaline as therapy and prevention of E. coli-L-asparaginase-induced anaphylaxis].

Management of E.-coli L-asparaginase induced anaphylaxis mainly consists of application of i.v.

[The effect of high-dose peroral zidovudine treatment in a 4-year-old child with HIV encephalopathy].

We report a four-year-old boy with HIV encephalopathy after vertical HIV infection. On the first admission to our hospital he showed ataxia, loose of expressive language and interstitial pneumonia. After treatment of the pneumonia the patient was started on oral zidovudine with 6 x 6 mg/kg bw/day, because of persisting neurologic symptoms and deep white matter lesions in the MR tomogram of the brain.

[Infections in the neutropenic phase following bone marrow transplantation: comparison of laminar airflow isolation with conventional isolation].

From May 1978 to September 1989 45 patients underwent 25 allogeneic and 21 autologous bone marrow transplantations (BMT) and 1 peripheral stem cell transplantation for the following indications: severe aplastic anemia (n = 4), hematological malignancies (n = 28), malignant solid tumours (n = 12) and sideroblastic anemia (n = 1). The first group of 20 patients was isolated in a conventional hospital room, while management of the aplastic phase in the second group of 25 patients was performed in a laminar air flow (LAF) unit. All patients received total decontamination.

[Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].

Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured.

[Teratoma of the stomach in a 4-day-old newborn infant].

Teratoma of the stomach is a very rare tumor. Nearly all previously reported patients were males. We report a 4 day-old male infant, who presented with a palpable abdominal mass.

Busulfan/cyclophosphamide plus bone marrow transplantation is not sufficient to eradicate the malignant clone in juvenile chronic myelogenous leukemia.

Bone marrow transplantation plays an essential role in the successful treatment of both juvenile and adult chronic myelogenous leukemia. Recently, it has been reported that conditioning with high doses of busulfan can successfully replace total body irradiation (TBI), in patients with acute myelogenous leukemia as well as adult chronic myelogenous leukemia. We report here the case of a 29-month-old boy with juvenile chronic myelogenous leukemia (JCML) transplanted with HLA-identical bone marrow after conditioning with busulfan, etoposide and cyclophosphamide.

Imipenem-antibiotic monotherapy in juvenile cancer patients with neutropenia.

From 1984 to 1987 two consecutive groups of juvenile cancer patients (n = 45) with fever and neutropenia corresponding in all criteria were examined. In half of the total of 90 febrile episodes and septicemias, a conventional antibiotic combination therapy (Pseudomonas-active penicillin/cephalosporin of the third generation/aminoglycoside) was instituted. In the remaining half imipenem was used as an antibiotic monoagent.

[Primary and secondary inoperable teratoid hepatoblastoma--multidisciplinary management].

Hepatoblastomas are the most frequent malignant liver tumors of childhood. Prognosis mostly depends on their resectability. Unresectable tumors are almost invariably fatal, unless their size can be decreased by chemotherapy and/or irradiation to permit surgical removal.

Successful treatment of CMV retinitis with ganciclovir after allogeneic marrow transplantation.

Cytomegalovirus (CMV) infection of the retina is a well recognized complication in patients with the acquired immune deficiency syndrome but is rarely seen after bone marrow transplantation (BMT). Among a variety of drugs ganciclovir so far appears to be the most effective therapy for CMV retinitis, but in previous studies relapses occurred in all patients in whom ganciclovir was interrupted. We report the clinical findings in a 22-year-old BMT recipient who developed bilateral exudative CMV retinitis 64 days after BMT despite prophylactic treatment with high-titer CMV-immunoglobulins and transfusions of CMV-negative blood products and donor bone marrow.