N-acetylneuraminic acid analogues. II. The action of N-acetylneuraminic acid aldolase on 8-carbon and 7-carbon analogues.
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Amino Sugars↗
Biomedical subjects
C Urban
Publications and source records attributed to C Urban.
[Bone pain after long distance flight as first manifestation of HbSC disease].
A 7-year old Ghanese boy was admitted with repeated bone pain. Multiple bone marrow alterations were seen as the causal factor. HbSC disease was diagnosed by hemoglobin electrophorics. The onset of pain attacks appears to be linked to the long distance flight from Ghana to Austria. Due to severe bone marrow affection, beside symptomatic therapy a long-term treatment with hydroxyurea was started, leading to an HbF increase from 5.1 % to 19.3 %. After a follow-up of 8 months the patient has remained free of complaints.
Aircraft↗
[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. epinephrine but also in the withdrawal of this drug. Despite of this firm recommendation we have shown in 7 patients with E. coli L-asparaginase induced anaphylaxis, that further application of the drug is possible in the presence of a continuous epinephrine infusion (0.01-0.02 mg/kg) started one hour before and finished one hour after the concomittant infusion of the E.-coli L-asparaginase. In none of the patients there was a second event of anaphylaxis even though most of the patients still had to continue on E.-coli L-asparaginase with an average of more than 6 infusion.
Anaphylaxis↗
[Aseptic bone necroses as a late complication following successful treatment of leukemias and severe aplastic anemia].
Aseptic bone necrosis is a well known complication after corticosteroid treatment in adults and several hundred cases have been reported. Alterations in fat metabolism with vascular occlusion due to fat embolization, as well as microtraumata and osteoporosis are discussed as etiologic factors. In contrast, aseptic bone necrosis in relation to corticosteroid treatment is rare in children and adolescents. We therefore report 3 patients, aged from 10 to 18 years, suffering from severe aplastic anemia, meningeal relapse after acute lymphocytic leukemia and acute myelocytic leukemia respectively, who developed aseptic bone necrosis 6, 11, and 20 months following the onset of corticoid therapy. The patients survive from 28+ to 50+ months after diagnosis of their initial hematologic disease, as it can be expected today for increasing numbers of patients. We therefore believe, that aseptic bone necrosis may represent a serious therapy related complication and suggest that, diagnostic examination in patients with suspicious complaints of the hip, shoulder or knee should also exclude the possibility of a bone necrosis after leucemic relapse has been ruled out. Since radiological changes only develop several weeks to months after the onset of the clinical symptoms and because of the disabling consequences for patients, misdiagnosed at the beginning, a 99 technetium bone scan should be done as early as possible. Corticosteroids, despite their serious side effects are still being considered as a important part of hematologic therapy and are not being omitted in the near future, so that the earliest possible diagnosis of bone necrosis will remain of great importance.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Prevention of CNS recurrence in childhood ALL: results with reduced radiotherapy combined with CNS-directed chemotherapy in four consecutive ALL-BFM trials.
BACKGROUND: The introduction of cranial radiotherapy (CRT) has provided efficient control of overt or subclinical meningeosis in acute lymphoblastic leukemia (ALL). Especially due to the long-term toxicity of CRT, reduction or elimination of radiotherapy appeared mandatory after cure rates of more than 70% had been achieved in ALL. The Berlin-Frankfurt-Münster (BFM) Study Group initiated several attempts in certain ALL subgroups to omit or reduce CRT while using more CNS-directed chemotherapy but without extended intrathecal treatment during maintenance therapy. This analysis summarizes the essential results that are in particular relevant because irradiation of the central nervous system (CNS) has been further reduced in the most recent trial ALL-BFM 95. PATIENTS AND METHODS: More than 4000 patients enrolled between 1981 and 1995 in one of the last four ALL-BFM trials have been analyzed to demonstrate the efficiency of intensive systemic and intrathecal chemotherapy with or without reduced CRT in the prevention of CNS relapses. RESULTS: In trial ALL-BFM81, it was shown that only in low-risk (LR) patients preventive radiotherapy can be replaced safely by intermediate dose (0.5 g/m2) methotrexate (MHD-MTX). In intermediate risk (IR) patients this attempt failed: IR pts had 8 times more CNS relapses if treated by MHD-MTX without CRT. In the subsequent trial ALL-BFM 83, all pts received MHD-MTX. IR pts were randomly treated with 12 or 18 Gy of preventive CRT which did not result in a significantly different outcome. The results from the subsequent trial ALL-BFM 86, using a more intensive consolidation with high-dose methotrexate (HD-MTX), demonstrated that the elimination of CRT in low-risk ALL, the reduced CRT of 12 Gy for IR, 18 Gy for medium (MR), and the reduced CRT with 18 Gy for high risk (HR) ALL, respectively, was justified: the incidence of relapses with CNS involvement was reduced to less than 5% (Reiter et al. 1994, Blood 84: 3122). When even less intensive preventive CRT (12 Gy for all medium and high risk patients) was used in trial ALL-BFM 90, the rate of CNS-related relapses was again below 5%. HR patients now treated with more CNS-directed chemotherapy had the lowest rate of CNS-related relapses observed so far in the BFM trials, even though CRT was also reduced to 12 Gy. Patients with T-cell ALL were shown to be protected from CNS recurrence by the combination of CRT (12 Gy) and HD-MTX more effectively than by HD-MTX in consolidation and TIT therapy during maintenance, especially if they presented with high WBC as shown in a joint AIEOP/BFM analysis (Conter et al. 1997, JCO 15: 2786). Patients with overt meningeosis which are characterized by a high leukemic cell load at diagnosis had a rate of recurrences that was comparable to that of patients with high WBC but no CNS disease. CONCLUSION: Low-risk ALL patients can be efficiently prevented from CNS relapse by intensive systemic and intrathecal chemotherapy without CRT. Patients with intermediate or medium risk ALL, including T-cell ALL, did not suffer from more CNS or systemic relapses when CRT was reduced to only 12 Gy. Patients with inadequate response to therapy are at particularly high risk for relapse with CNS involvement. Therefore, more CNS-directed systemic and intrathecal chemotherapy was applied in trial ALL-BFM 90, combined with only 12 Gy cranial irradiation, and improved the control of CNS recurrence. It seems likely that larger subsets of B-precursor ALL can be protected from CNS-related relapse by intensive chemotherapy without extended IT treatment and without CRT. This is being investigated in the ongoing trial ALL-BFM 95.
Adolescent↗
The influence of maximum supportive care on dose compliance and survival. Single-center analysis of childhood acute lymphoblastic leukemia and non-Hodgkin's-lymphoma treated within 1984-1993.
BACKGROUND: The administration of intensive, risk-adapted multiagent chemotherapy has markedly improved the event-free survival in childhood acute lymphoblastic leukemia and Non-Hodgkin's lymphoma. Current treatment protocols may achieve complete remission rates of more than 95% and event-free survival rates of approximately 70% in non-B-acute lymphoblastic leukemia/non-B-Non-Hodgkin's lymphoma patients. Due to a higher proliferation rate and aggressiveness of B-cells treatment of B-acute lymphoblastic leukemia/B-Non-Hodgkin's lymphoma must be more intensive, but can be shorter to attain similar event-free survival rates. METHODS: Between 1984 and 1993 79 primarily admitted patients up to 18 years of age (range 0.6-17.9 years) received therapy according to the treatment protocols ALL A 84, ALL 86, NHL 86, ALL 90 and NHL 90 of the Berlin-Frankfurt-Münster Study Group. Intensive phases of treatment were given on an inpatient basis with maximum supportive care in order to achieve the prescribed doses. RESULTS: Median follow-up time from diagnosis is now 8 years (range 40 to 157 months) with a probability of event-free survival of 92% for the entire group. CONCLUSION: Multiagent chemotherapy tailored to the individual risk profile of each patient but given in the prescribed dose is currently the mainstay to achieve high complete remission rates with the aim to cure most acute lymphoblastic leukemia/Non-Hodgkin's lymphoma patients. The administration of intensive supportive care is important for the achievement of complete drug dosage and for the reduction of therapy-related toxicity.
Adolescent↗
[Value of prognostic factors in the Austrian A-NB87 Neuroblastoma Study].
AIMS: A multivariate analysis was performed to evaluate the impact of various prospectively evaluated risk factors. PATIENTS AND METHODS: From January 1987 to December 1993, 120 patients were registered in the study. 108/120 patients were eligible. There were 49 girls and 59 boys with a median age at diagnosis of 14 months (range, 0 to 224 months). Patients were classified according to the Evans classification system. LDH, NSE, Ferritin, N-myc amplification, 1p-deletion and ploidy were evaluated at diagnosis. Treatment intensity was based on the results of primary surgery: surgery only for 22 (20%) stage I and IIA patients (macroscopic residue without lymph node involvement), the 9 IIB patients (8,5%) (macroscopic residue with lymph node involvement) had mild chemotherapy in addition (6 x VCR/CYC) and elective radiation (Rx). Stage III patients were divided into 2 groups: IIIA patients (n = 17/16%) had to have ferritin levels under 300 micrograms/ml, NSE lower than 100 ng/ml and age below 2 years at diagnosis and received 6 alternating cycles of DAMO/ MVDOC. If one of these three criteria was not fulfilled, patients were assigned to the more intensive treatment arm stage IIIB (n = 12/11%), i.e. 9 alternating cycles of DAMO/MVDOC/IPE. Stage IV pts (n = 35/32.5%) received 8 MVDOC/IPE cycles and 20 patients received megatherapy followed by stem cell reinfusion in addition. 13 stage IVs patients (12%) were registered and had elective VCR/CYC and/or liver radiation in case of poor clinical condition. The median observation time is 4.2 years (range, 1 to 7.5). RESULTS: The survival rate at 3 years was excellent for localized disease and stage IVs with survival rates of 100% for stage I/IIA and 92% for stage IVs. Stage IIIA patients had an EFS rate of 81% whereas stage IIB patients achieved only 69%. Stage IV patients reached 51%, however outcome was especially poor for stage IIIB patients (20%) due to treatment related toxicities. The toxic death rate in the study was 13% (2 surgical deaths, 8 infections, 4 multiple organ failures). Univariate analysis demonstrated the following significant unfavorable risk factors: age over 1 year at diagnosis (58/108 pts, p = 0.006), NSE > 100 ng/ml (26/95 pts, p < 0.0001), Ferritin > 300 ng/ml (19/98 pts, p = 0.007), LDH > 300 (400) U/L, 51/87 pts, p = 0.004), presence of N-myc amplification (17/59 pts, p = 0.001), deletion of the short arm of chromosome 1 (19/74 pts, p < 0.0001) and di/tetraploidy (32/72 pts, p = 0.008). The power of these factors was even stronger in patients with localized disease whereas no significant prediction was observed for stage IV patients. Furthermore a significant correlation of the serological (NSE, ferritin, LDH) and biological factors (N-myc, deletion 1p, di/tetraploidy) was detected in this study. Only NSE was identified as an independent prognostic factor (p = 0.018) whereas no independent factor could be identified within the 3 biological parameters due to their high correlations (Kendall's tau for N-myc and deletion 1p:0.7). However, N-myc (p = 0.005) as well as deletion 1p (p = 0.01) were found significantly more important than di/tetraploidy. CONCLUSIONS: Biological classification of neuroblastomas should be mandatory and be the prerequisite for any risk adapted treatment. One serological and 2 biological factors could be a good standard evaluation to identify neuroblastoma patients at risk.
Adolescent↗
[Minimal metastatic and minimal residual disease in patients with Ewing tumors].
At least 95% of Ewing tumors (ET) are characterized by an EWS-gene rearrangement with either FLI-1 or ERG and by extraordinary high MIC2/CD99 expression. Both features can be used for the specific identification of tumor cells, which might also be present in blood and bone marrow in minimal metastatic or minimal residual disease. We report the establishment of sensitive ET cell detection methods based on these characteristics. Preliminary results of 14 patients are given and two cases are reported in detail. 6/14 patients showed RT-PCR positivity in the bone marrow whereas in 8/14 patients ET cells could be detected by immunofluorescence. Only in 2 patients, one at diagnosis and one at relapse, tumor cells were detected in blood samples by RT-PCR. Peripheral blood stem cells of 5 patients showed no contamination with ET cells. The versatility of the immunocytochemical approach as compared to the RT-PCR analysis is discussed in order to explain the discrepancies between the results obtained with the two detection methods.
Adolescent↗
Treatment of children with anagrelide for thrombocythemia.
PURPOSE: Essential thrombocythemia (ET) is a rare myeloproliferative disease characterized by hyperproliferation of megakaryocytes and persistent elevation of platelets. Major complications are thrombosis, bleeding, and microvascular occlusive symptoms, sometimes resulting in serious morbidity. In patients with platelet counts > 1000 x 10(9)/l, cytoreduction is important to reduce the risk for thrombotic or hemorrhagic events. However, the frequent side effects and the carcinogenic potential of many cytoreductive agents limit their use, especially in younger patients. Anagrelide, a noncarcinogenic agent with moderate toxicity, has been successfully used in adult patients with ET, but experience with anagrelide in pediatric patients with ET is limited. PATIENTS AND METHODS: Three children who received anagrelide (Induction dose 1 mg/day, maintenance dose 1 to 2.5 mg/day) as therapy for ET are described. RESULTS: All children had a rapid and marked decline of their platelet counts, and only one child developed mild and transient abdominal side effects. CONCLUSIONS: Treatment with anagrelide seems to be a promising approach for children with ET. It should be considered as first-line therapy because of its selective activity against platelet production, tolerable toxicity, suspected negligible cancerigenic effect, and the possibility of oral administration.
Adolescent↗
Naturalistic course of obsessive compulsive disorder and comorbid depression. Longitudinal results of a prospective follow-up study of 74 actively treated patients.
Seventy-four patients who met DSM-III-R criteria for obsessive compulsive disorder (OCD) were studied in a prospective follow-up study in order to investigate course and prognosis of OCD with or without comorbid depressive symptomatology. Subjects were examined three times: at admission (baseline), 6 months later (follow-up 1) and 12 months after follow-up 1 (follow-up 2). At admission, 51 (72.9%) OCD patients were assessed as depressive by the Hamilton Depression Scale score. Between admission and follow-up 1, all patients received behavior therapy and a serotonin reuptake inhibitor, between follow-up 1 and follow-up 2 they received different kinds of treatment in order to maximize therapeutic effects. A 25% Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) score reduction from admission to follow-up 2 and in addition, a total Y-BOCS score of below 16 at follow-up 2 was defined as 'good prognosis course'. The results obtained showed that OCD patients who followed a good prognosis course, showed no significant depressive symptomatology at follow-up 2 (p = 0.001). These results imply that patients with a diagnosis of OCD may present depression at admission and/or follow-up 1; however, if OC symptomatology decreases longitudinally, depressive symptoms disappear too. We may assume that OCD is dominant over depression, and it seems that a comorbid depression does not have any major influence on the prognosis of OCD.
Adolescent↗
Memory deficits in patients with DSM-IV obsessive-compulsive disorder.
Neuropsychological testing provides increasing evidence that certain memory deficits might play an essential role in the emergence of doubts and, as a result, in perpetuating checkers' rituals. Another account of doubting implicates meta-cognitive factors, such as confidence in memory. The present study examined mnestic functioning and self-perception of memory ability in a group of 27 nondepressed patients with obsessive-compulsive disorder (OCD) and 27 normal controls. All patients met DSM-IV and ICD-10 criteria for OCD, displayed prominent behavioral checking rituals and had to show a score on the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) of at least 16. Significant deficits in intermediate (Lern- und Gedächtnistest; LGT-3) and immediate (Corsi Block-Tapping Test) nonverbal memory were identified in the patients with OCD compared to normal controls. Contrary to predictions, OCD patients also showed a significant deficit in general memory and verbal memory (LGT-3). With respect to meta-cognition, OCD patients reported less confidence in their memories than controls. These findings suggest that obsessional doubt reflects a deficit in memory as well as a deficit in memory confidence. Depending on which dysfunction predominates, different therapeutic procedures seem to be required.
Adolescent↗
Construction and characterization of a bovine BAC library with four genome-equivalent coverage.
A bovine artificial chromosome (BAC) library of 105 984 clones has been constructed in the vector pBeloBAC11 and organized in 3-dimension pools and high density membranes for screening by PCR and hybridization. The average insert size, determined after analysis of 388 clones, was estimated at 120 kb corresponding to a four genome coverage. Given the fact that a male was used to construct the library, the probability of finding any given autosomal and X or Y locus is respectively 0.98 and 0.86. The library was screened for 164 microsatellite markers and an average of 3.9 superpools was positive for each PCR system. None of the 50 or so BAC clones analysed by FISH was chimeric. This BAC library increases the international genome coverage for cattle to around 28 genome equivalents and extends the coverage of the ruminant genomes available at the Inra resource center to 15 genome equivalents.
Animals↗
[Ethical implications of Do-Not-Resuscitate orders].
UNLABELLED: Do-Not-Resuscitate (DNR) orders are those orders that direct non-initiation of cardiopulmonary resuscitation in the event of cardiopulmonary arrest. PURPOSE: To evaluate ethical implications of DNR orders in the clinical pratice. METHODS: The authors reviewed critically the major ethical issues associated with DNR orders, mainly focusing their risks and benefits, and the application of the Personalistic's view in this orders. RESULTS: Reaction to the widespread use of this orders is not universally positive, and concerns about its possible overuse and abuse have been raised. DNR orders alone can not resolve the complex problem of the terminally ill, patients and the therapeutic futility. CONCLUSION: From the Personalistic's point of view, in all instances, the physician must remain aware that her primary duty is to hold the patient's dignity.
Bioethics↗
Percutaneous insertion of Broviac catheters by pediatricians on the oncology ward.
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Adolescent↗
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. The patient has remained in complete remission without evidence of disease for 12 months after donor PBMC transfusions. The other child showed disappearance of BCR-ABL gene transcripts by PCR analysis only in peripheral blood cells, but PCR positivity persisted in bone marrow samples. These results indicate that adoptive immunotherapy may be a further alternative in children with relapse of CML after allogeneic BMT as previously described for adult patients.
Adolescent↗
MR findings in methotrexate-induced CNS abnormalities.
MR of the brain was performed in eight patients (mean age, 14.9 years) with osteogenic sarcoma during or after IV treatment with high-dose methotrexate. MR detected brain abnormalities in four patients, three of whom had concomitant neurologic dysfunction. Pathologic findings demonstrated on MR were (1) chronic brain edema, demonstrable over a period of 3-14 months (proved by autopsy in one patient); (2) multifocal white matter necrosis; and (3) deep brain atrophy. MR appears to be valuable in the detection of abnormalities induced by treatment with high-dose methotrexate.
Adolescent↗
Implementation of critical care standards.
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American Nurses' Association↗
Neuroblastoma screening: labeling of HVA and VMA for stable isotope dilution gas chromatography-mass spectrometry.
Neuroblastoma is a curable tumor if detected early enough. Therefore, a screening test is performed in Austrian infants by analyzing urine samples collected and dried on filter strips. Screening is performed by measuring homovanillic acid (HVA) and vanillylmandelic acid (VMA) using the enzymatic immunoassay chemistry (EIA) method and reexamining the elevated values by high-performance liquid chromatography. Both methods, however, give a relatively high number of false-positive results. Therefore, the positives are reexamined by stable isotope dilution (SID) gas chromatography-mass spectrometry (GC-MS), which is the most sensitive and precise method in clinical chemistry. The labeled substance is added as an internal standard to the sample; it has nearly identical chemical and physical properties as the natural analogue, undergoes the entire preparation step, and is detected simultaneously with the natural analogue by the same detector, namely the mass spectrometry, given by a difference in molecular weight. All loss is compensated in this way by the final calculation. At present, HVA but not VMA can be purchased labeled with deuterium, so we started with our method using deuterated HVA as the internal standard for both HVA and VMA. Because the coefficient of variation is not sufficient for VMA, we try to label VMA via different labeling procedures; for example, 1) exchange of the aromatic H against D by incubation with DCI or in alkaline R-OD or LiOD or 2) exchange of phenolic 16OH against 18OH by reaction with H2(18)O in an acidic environment and at high temperatures. Carboxylic 16O can be exchanged against 18O.
Austria↗