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Biomedical subjects

D Schuler

Publications and source records attributed to D Schuler.

At least 55 records · Page 3Linked to original sources
PubMed1993

[Rare congenital forms of bone marrow deficiency].

Cases of rare congenital forms of aplastic anaemia are presented. Fanconi anaemia is most frequently diagnosed, while reticular dysgenesis, amegakaryocytic thrombocytopenia and dyskeratosis congenita occur exceptionally rarely. A presumably new entity of congenital aplastic anaemia called "RAC" syndrome (retinopathy--aplastic anaemia--central nervous system abnormalities) is presented too. A short summary of the pathomechanism of congenital aplastic anaemias and present therapeutic attempts, including the possible therapeutic use of the newly discovered stem cell factor are described.

Brain Diseases↗
PubMed1993

The treatment of malignant scala posterior tumors in children: II. Preliminary result of the pre- and postoperative adjuvant chemotherapy of scala posterior tumors.

Ten children with posterior scala tumor infiltrating the surrounding brain substance and/or the brain stem entered in the present study with preoperative chemotherapy. In 8 of the 10 cases regression and necrosis of the tumor were seen by CT examination after the preoperative therapy. The diameter of the tumor decreased on the average by 35.6% (14.0-74.3%). The main side effect was granulocytopenia. According to our observation, the preoperative therapy enables a more radical surgery in some cases of medulloblastoma and ependymoma. Further observations are necessary to confirm these preliminary results.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Proteinuria due to suboptimal hydration with high-dose methotrexate therapy.

One of the major complications after high-dose methotrexate (HDMTX) infusions is renal damage. We investigated the occurrence of proteinuria after HDMTX administration in children with pediatric malignancies (acute lymphoid leukaemia, osteosarcoma Burkitt's lymphoma). In the period 1989-1990 we gave 52 HDMTX courses to 24 children. During this period, prehydration and extra urinary alkalisation were performed only if the urinary specific gravity was over 1010 or if the urinary pH fell below 7. Using this schedule the mean values obtained for protein extraction were: before the therapy, 0.12 +/- 0.03 g/m2; on day 1 after MTX treatment, 0.38 +/- 0.06 g/m2; and on day 2 after the MTX infusion, 0.39 +/- 0.11 g/m2 (P < 0.01). A significant increase in proteinuria (> 0.2 g/m2 post- vs pretreatment) was detectable in 54% of the patients. In the period 1991-1992 we modified the hydration-alkalisation schedule to include i.v. prehydration for 18-24 h at 3 l/m2/day with a 0.45% NaCl-5% glucose solution along with sodium bicarbonate and posthydration for 72 h with the same solution. On this protocol the mean values determined for the urinary protein content were all in the normal range (pretreatment, 0.03 g/m2/day; day 1, 0.05 g/m2/day; and day 2, 0.08 g/m2/day). These findings were significantly different from the previous results (P < 0.05).

Adolescent↗
PubMed1992

Treatment of malignant scala posterior brain tumors in children: the chemotherapy of relapsed medulloblastoma with a dibromdulcitol containing drug regime and pharmacokinetic studies of dibromdulcitol in children.

Dibromdulcitol (Elobromol) has favorable pharmacokinetic parameters for the treatment of brain tumors: high spinal fluid/plasma ratio and long half-life in spinal fluid. Oral application makes its administration easy. The drug combination vincristine, procarbazine, and dibromdulcitol proved to be effective in a pilot trial on relapsed medulloblastomas: 8 complete and 4 partial remissions were achieved from 16 cases. The main side effect was granulocytopenia, which was in some cases severe. However, in the dose-schedule we used it did not delay the treatment longer than 1 week.

Adolescent↗
PubMed1992

Paediatric care in Hungary.

In this overview, I have attempted to highlight the problems of medical care, particularly paediatric care, in Hungary and to describe the changing nature of its organisation and financing. We hope that through our efforts we can surmount these difficulties, improve the quality of medical care in our country, and create a more rational economic and scientific basis for future medical development and advances.

Child↗
PubMed1992

Immunoglobulin prophylaxis during intensive treatment of acute lymphoblastic leukemia in children.

60 children with acute lymphoblastic leukemia were sequentially randomized at the time of diagnosis: Immunoglobulin (Endobulin, Immuno) was administered intravenously to 30 patients at a dose 100 mg/kg/week during the first 3 months, followed by 2 x 200 mg/kg/month immunoglobulin during the 4., 5., 6. months. No immunoglobulin was administered to the control patients. We studied the effect of immunoglobulin prophylaxis on the number of days with fever, number of cases with bacteriologically proved infections, length and frequency of antibiotic therapy. Our data confirm the efficacy of immunoglobulin prophylaxis during the intensive phase of leukemia therapy in children.

Child↗
PubMed1991

Value of brainstem acoustic evoked potentials in posterior fossa tumours in childhood.

Brainstem acoustic evoked potentials (BAEPs) were measured in 14 children with different type of posterior fossa tumours several times during the clinical course, in order to assess the value of this simple and non-invasive method in the diagnosis and follow-up of posterior fossa tumours in childhood. Eight children had midline medulloblastoma, three children had lateral astrocytoma, three had intrinsic brainstem glioma. Different BAEP patterns could be detected in different tumour's type: bilateral symmetrical or slightly asymmetrical I-V. IPL prolongation in midline medulloblastomas, unilateral or markedly asymmetrical I.-V. IPL prolongation or wave V. depression on the contralateral side in lateral astrocytomas, and severely distorted asymmetrical waveform in intrinsic brainstem gliomas. The BAEPs were abnormal earlier than CT scan in a case of craniospinal astrocytoma. BAEPs were useful in the follow-up: the effect of the preoperative chemotherapy or the progression of the inoperable tumours could be as well documented by this method, as by the CT scan. BAEPs proved effective in the assessment of postoperative neurological complications: bilateral symmetrical IPL prolongation and wave V. depression with clinical signs of increased intracranial pressure occurred in a case of postoperative occlusive hydrocephalus, unilateral IPL prolongation occurred during irradiation or chemotherapy after medulloblastoma removal as signs of cerebral oedema.

Adolescent↗
PubMed1990

Neuropsychologic and CT examinations in leukemic patients surviving 10 or more years.

Long-term survivors of childhood leukemia were studied to determine their neuropsychologic status. All had had cranial irradiation 10 or more years before. The results were 1. The IQ and academic performance of 56 cured leukemic patients show no significant difference from the normal population. 2. More exact neuropsychological tests (attention, reaction time, visual-motor coordination, memory) reveal important deficits. 3. The frequency of CT aberrations in 33 study patients was 45% and showed no correlation with either the IQ or other neuropsychological results.

Adolescent↗
PubMed1990

Molecular genetics of PKU in eastern Europe: a nonsense mutation associated with haplotype 4 of the phenylalanine hydroxylase gene.

Phenylketonuria (PKU) is a genetic disorder secondary to a deficiency of hepatic phenylalanine hydroxylase (PAH). Several mutations in the PAH gene have recently been reported, and linkage disequilibrium was observed between RFLP haplotypes and specific mutations. A new molecular lesion has been identified in exon 7 of the PAH gene in a Hungarian PKU patient by direct sequencing of PCR-amplified DNA. The C-to-T transition causes the substitution of Arg243 to a termination codon, and the mutant allele is associated with haplotype 4 of the PAH gene. The mutation is present in two of nine mutant haplotype 4 alleles among Eastern Europeans and is not present among Western Europeans and Asians. The rarity of this mutant allele and its restricted geographic distribution suggest that the mutational event occurred recently on a normal haplotype 4 background in Eastern Europe.

Alleles↗
PubMed1990

Consumer evaluation of the quality of hospital services from an economics of information perspective.

Consumer evaluations of the quality of hospital services have become increasingly important as the patient and the patient's family have become more involved in hospital selection. The authors investigate the appropriateness of the search, experience, and credence typology of economics of information theory as a framework for analyzing how consumers judge the quality of specific hospital services. Advertising strategies are recommended for each category for hospitals seeking to create images of quality and excellence.

Advertising↗
PubMed1990

[The significance of the determination of catecholamines and their metabolites in neuroblastomas].

A method for the determination of catecholamines and their metabolites in the urine was established. The specimens were previously cleaned by ion exchangers and the components were separated by a 'reversed phase' ionic pair chromatography, determined by an electrochemical 'wall-jet'-detector. This method was applied in order to find a diagnostic marker in case of neuroblastoma diseases. In 85 per cent of the patients (n = 13) we stated an increased ejection of dopamine.

Biomarkers, Tumor↗
PubMed1989

[Neurologic tests in children having recovered from leukemia].

56 long-term survivors of childhood ALL have been investigated with CT and neuro-psychological methods. At the CT examination we found that 45.4% of the cured patients had alterations. Short-term memory, attention, visual-motor coordination and IQ were found to be slightly impaired in this group. Nevertheless they didn't influence the social integration and educational achievement of long-term survivors of ALL.

Adolescent↗
PubMed1989

Polymorphic DNA haplotypes at the phenylalanine hydroxylase (PAH) locus in European families with phenylketonuria (PKU).

DNA haplotype data from the phenylalanine hydroxylase (PAH) locus are available from a number of European populations as a result of RFLP testing for genetic counseling in families with phenylketonuria (PKU). We have analyzed data from Hungary and Czechoslovakia together with published data from five additional countries--Denmark, Switzerland, Scotland, Germany, and France--representing a broad geographic and ethnographic range. The data include 686 complete chromosomal haplotypes for eight RFLP sites assayed in 202 unrelated Caucasian families with PKU. Forty-six distinct RFLP haplotypes have been observed to date, 10 unique to PKU-bearing chromosomes, 12 unique to non-PKU chromosomes, and the remainder found in association with both types. Despite the large number of haplotypes observed (still much less than the theoretical maximum of 384), five haplotypes alone account for more than 76% of normal European chromosomes and four haplotypes alone account for more than 80% of PKU-bearing chromosomes. We evaluated the distribution of haplotypes and alleles within these populations and calculated pairwise disequilibrium values between RFLP sites and between these sites and a hypothetical PKU "locus." These are statistically significant differences between European populations in the frequencies of non-PKU chromosomal haplotypes (P = .025) and PKU chromosomal haplotypes (P much less than .001). Haplotype frequencies of the PKU and non-PKU chromosomes also differ significantly (P much less than .001. Disequilibrium values are consistent with the PAH physical map and support the molecular evidence for multiple, independent PKU mutations in Caucasians. However, the data do not support a single geographic origin for these mutations.(ABSTRACT TRUNCATED AT 250 WORDS)

Alleles↗