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  • Bone marrow transplantation  (4)
  • Key words Phenylketonuria  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Natural killer cell activity against a thymoma cell line thy 121 in bone marrow transplant recipients (1986)
    Springer
    Journal of molecular medicine 64 (1986), S. 125-130 
    Details
    ISSN: 1432-1440
    Keywords: Bone marrow transplantation ; Natural killer cells ; Interferon ; Cycloporin A ; Graft-versus-host disease ; Methotrexate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Twenty-one patients with acute and chronic leukemia or severe aplastic anemia were studied for NK activity against a thymoma cell line (Thy 121) before and after allogeneic bone marrow transplantation. The means of the pretransplant and post-transplant levels did not differ from the mean of 134 NK determinations in 67 healthy donors. There was no correlation between pretransplant NK levels and the appearance of graft-versus-host disease. Three weeks following bone marrow transplantation, pretransplant NK levels were observed. The sensitivity of NK cells to interferon was the same as in normal donors both before and after bone marrow transplantation. In contrast to methotrexate, cyclosporin A inhibited NK activity in patients and controls in vitro. In vivo cyclosporin A treatment, however, did not decrease NK levels in bone marrow recipients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01732635
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Knochenmarktransplantation bei akuter Leukämie, chronisch myeloischer Leukämie, schwerer aplastischer Anämie und Neuroblastom Stadium IV. Einfluß antiviraler Prophylaxe mit Anti-CMV-Hyperimmunglobulin und Azyklovir (1986)
    Springer
    Journal of molecular medicine 64 (1986), S. 452-466 
    Details
    ISSN: 1432-1440
    Keywords: Bone marrow transplantation ; Anti-CMV hypergammaglobulin ; Azyklovir ; Conditioning
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Bone marrow transplantation was performed between IV/82 and X/85 in 64 patients with acute leukemia (n=36), chronic myelogenous leukemia (CML;n=13), severe aplastic anemia (n=12), and neuroblastoma stage IV (n=3). Of these patients 57 received allogeneic marrow from HLA-ABCDR identical, MLC-negative sibling donors. Six transplants were performed with syngenic marrow and one with autologous marrow. Of the 64 patients 48 survived 40-1,250 days after transplantation, resulting in a survival rate (SR) of 75% and a survival probability (SP) of 71%. Of the 36 patients suffering from acute leukemia (SR=64%, SP=51%), patients with acute myelogenous leukemia (AML) in first complete remission (n=11; SR=81%, SP=76%), as well as patients with acute lymphatic leukemia (ALL) in 1st to 4th complete remission at the time of transplantation (n=14; SR=81%, SP=76%) show a favorable prognosis. A poor survival rate was seen for patients with AML when transplanted in second or partial remission (1/5; SR=20%), as well as for patients suffering from ALL and transplanted during relapse or partial remission (1/6; SR=16%). Of 13 patients suffering from CML 12 survived the transplantation free of relapse (SR=93%, SP=92%), and one patient died from varicella zoster pneumonia. Of the transplanted patients with severe aplastic anemia, 12 of 13 are surviving with complete hematologic reconstitution; one patient, however, died on day 10 from a sepsis. In our patient group, the SR as well as the SP has been improved through changes in the irradiation protocol concomitant with prophylactic application of anti-CMV hypergammaglobulin, as well as through additional oral medication of Azyklovir. The 41 patients (BMT No. 7–47) with total body irradiation at one time-show an SR of 44% and an SP of 41%. The following 46 patients (BMT No. 48–93) have reached an SR of 83% and an SP of 74% under the regimen of fractionated total body irradiation, plus prophylaxis with anti-CMV hypergammaglobulin and Azyklovir. Within this group, no fatal CMV pneumonia was encountered as opposed to six patients lost from CMV pneumonia in the first group.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01713171
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Orale Prophylaxe von Herpes-Infektionen mit Acyclovir nach Knochenmarktransplantation: Eine klinische und klinisch-pharmakologische Untersuchung (1986)
    Springer
    Journal of molecular medicine 64 (1986), S. 570-574 
    Details
    ISSN: 1432-1440
    Keywords: Acyclovir, bioavailability ; Drug monitoring ; Bone marrow transplantation ; Herpes infections, prevention and control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Viral infections are one of the major complications after bone marrow transplantation, with high mortality and morbidity. Fourty-six patients between 3 and 48 years old (median 15 years) received orally 400 mg (under age 6, 200 mg) acyclovir 4 times daily from day −12 before to day 84 after BMT. All patients were isolated in laminar-airflow units for at least 23 days with total enteral decontamination. They were concomitantly treated with anti-CMV-hyperimmunoglobulin and cotrimoxazol. During acyclovir prophylaxis seven patients had herpes simplex virus infections, all of them were seropositive before BMT. Acyclovir plasma concentrations were measured by use of a new HPLC method. No acyclovir was present (detection limit 40 ng/ml) in the plasma of five out of six patients with HSV infections. Three of them had non-compliance, and a lack of acyclovir absorption developed in two patients under conditioning regimen. No drug-related side effects were observed. Laboratory tests did not show liver or renal toxicity. Take and hematologic reconstitution were unchanged. In our study, oral acyclovir reduced the incidence of herpes simplex infections after bone marrow transplantation. Herpes infections only occurred in patients with non-compliance or lack of acyclovir absorption.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01735321
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  • 4
    Electronic Resource
    Electronic Resource
    Correlation between low CSA plasma concentration and severity of acute GvHD in bone marrow transplantation (1988)
    Springer
    Annals of hematology 57 (1988), S. 139-142 
    Details
    ISSN: 1432-0584
    Keywords: Ciclosporin ; Graft-versus-host-disease ; Bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Between 1982 and 1986 51 patients were treated with ciclosporin a (CSA) to prevent graft versus host disease (GvHD) after bone marrow transplantation (BMT). Major side effects of the drug were tremor, hypertension, hepatotoxicity and nephrotoxicity. Acute GvHD 0° to II° occurred in 80% of our patients, and GvHD III° and IV° in 20% despite the use of CSA. Two to four days before the onset of GvHD, CSA serum levels were significantly lower on the average in patients who developed GvHD III° and IV° compared to the others. Our data indicate that plasma CSA concentrations higher than 250 ng/ml should be achieved to reduce the severity of GvHD after BMT.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320154
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Magnetic resonance imaging of the brain in adolescents with phenyketonuria and in one case of 6-pyruvoyl tetrahydropteridine synthase deficiency (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S69 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Phenylalanine ; Magnetic resonance ; imaging ; Evoked potentials ; Myelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract White matter abnormalities on MRI have been observed in phenylketonuria (PKU) patients with late onset neurological symptoms as well as in neurologically inconspicious patients. We investigated 14 early treated adolescents at an age between 12 and 17 years (mean age 14.3 years) with classical PKU as well as one retarded patient with atypical PKU by cranial MRI with spinecho T1-, T2- and proton density sequences. Clinical examination was normal. Visual evoked potential (VEP) examination showed a prolonged latency of peak P100 (mean 122.6 ms; control mean 115.9) and IQ testing showed a mean IQ of 101.1. To investigate the influence of plasma phenylalanine (Phe) levels three approaches were used: Phe was determined for the day of MRI, for a period of 6 months prior to MRI and for lifetime up to 12 years. MRI scans revealed areas of abnormally increased signal intensity on T2-weighted and proton density images in 12 (86%) patients, preferably involving the parieto-occipital lobes. MRI of the patient with atypical PKU was normal. MRI findings correlated most strongly to long-term dietary control up to 12 years. We found no correlation with the other parameters of biochemical control, IQ or VEP latency. The nature and prognosis of MRI abnormalities in neurologically normal PKU patients remain unclear although abnormalities in VEPs which were not associated with the degree of MRI abnormalities in our sample indicate a disturbance in myelination along the visual pathways.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014255
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    Phenylalanine hydroxylase genotypes, predicted residual enzyme activity and phenotypic parameters of diagnosis and treatment of phenylketonuria (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S11 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Genotype ; Treatment ; IQ
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The interdependence of the predicted in vitro residual enzyme activity (PRA), as deduced from the complete genotypes of 64 hyperphenylalaninaemic patients, and parameters for diagnosis of hyperphenylalaninaemic disorders, the fluctuation of the phyenlylalanine (Phe) values during treatment, long-term dietary control during treatment, and a parameter for the outcome of therapy (IQ) was investigated by correlation analysis. A highly significant correlation was found between the PRA and diagnostic parameters, as well as the fluctuation of the Phe values during treatment. Significant correlations were also observed between the parameter describing the fluctuation of the Phe values and the IQ, as well as between the quality of dietary control and IQ. The PRA is a valuable tool for the differential diagnosis of hyperphenylalaninaemic disorders and for the prediction of one aspect of the course of the disease which is related to the intellectual outcome of therapy. The quality of dietary control was independent of the genotype, indicating that the outcome of therapy can be successfully manipulated in spite of the genetic make-up.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014222
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Intelligence and professional career in young adults treated early for phenylketonuria (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S97 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Intelligence ; Education ; Professional career ; Phenylalanine ; control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The intellectual status and professional careers of 51 young adults with phenylketonuria whose treatment started before 3 months of age are described. Their mean IQ was 97 (SD = 16). Of the IQs, 4% were more than 2 SD below the norm. The distribution of types of schooling of the patients was comparable to that in the German population. The professional careers of nearly all the patients were according to their educational level. Within the sample the outcome was significantly correlated with phenylalanine (Phe) control, even when the patients’ social background was statistically taken into account. The main influence of Phe on intelligence seems to occur during the first decade of life since IQ data remain stable even after Phe levels increased during adolescence.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014262
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