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  • 1
    ISSN: 1573-6776
    Keywords: fucosyltransferase ; N-acetylglucosaminyltransferase ; P-selectin ; PSGL-1 ; tricistronic vectors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Process Engineering, Biotechnology, Nutrition Technology
    Notes: Abstract Expression of recombinant glycoproteins carrying the sialyl-LewisX epitope requires host cells equipped with appropriate glycosyltransferases. Chinese hamster ovary cells transfected with tricistronic vectors and encoding the resistance marker gene, neoR, in the third cistron and core 2 N-acetylglucosaminyltransferase or α1,3-fucosyltransferase III or IV in either the first or second cistron, respectively, produced both enzyme activities in a constant ratio. A representative clone was transfected with PSGL-1 (P-selectin glycoprotien ligand 1) and conferred P-selectin-binding activity to PSGL-1.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 10 (2000), S. 1675-1677 
    ISSN: 1432-1084
    Keywords: Key words: Osteogenesis imperfecta – MR imaging – Temporal bone – Otosclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Osteogenesis imperfecta (OI) is an inherited generalized disorder of type-I collagen synthesis often associated with hearing loss. We present a case of OI type I in which hearing loss led to examination of the temporal bone with MRI. In the osseous otic capsule MRI demonstrated pericochlear lesions with soft tissue signal intensity and contrast enhancement. Changes similar to otosclerosis have been described in the temporal bone of OI patients when applying CT, but reports on MRI findings do not yet exist.
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  • 3
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. X-linked congenital stationary night blindness (CSNB) is a nonprogressive retinal disorder characterized by impaired night vision, variably involving high myopia, nystagmus, decreased visual acuity, and strabismus. Linkage studies have identified two distinct loci for X-linked CSNB1 and CSNB2 on the short arm of chromosome X. The gene mutated in families displaying the "incomplete phenotype" of CSNB (i.e., CSNB2) has recently been identified. To identify novel candidate genes for the "complete form" of CSNB (i.e., CSNB1) we screened the physically vast region Xp11.3-Xp11.4 for cDNA sequences. This led us to identify and map the G protein coupled receptor (GPCR) gene GPR34 to Xp11.4 within 650 kb of the marker DXS993. Deletion screening via Southern blotting and direct sequencing of GPR34 revealed no mutations in 19 unrelated men with CSNB1, excluding a causal role in the disease. However, because of its expression in retinal and neural tissue and the involvement of GPCRs in transmembrane signal transduction, GPR34 remains a putative candidate gene for a number of ocular diseases which also map to the Xp11.4 region
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  • 4
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. Congenital disorders of glycosylation (CDG), formerly known as carbohydrate-deficient glycoprotein syndrome, represent a family of genetic diseases with variable clinical presentations. Common to all types of CDG characterized to date is a defective Asn-linked glycosylation caused by enzymatic defects of N-glycan synthesis. Previously, we have identified a mutation in the ALG6 α1,3 glucosyltransferase gene as the cause of CDG-Ic in four related patients. Here, we present the identification of seven additional cases of CDG-Ic among a group of 35 untyped CDG patients. Analysis of lipid-linked oligosaccharides in fibroblasts confirmed the accumulation of dolichyl pyrophosphate-Man9GlcNAc2 in the CDG-Ic patients. The genomic organization of the human ALG6 gene was determined, revealing 14 exons spread over 55 kb. By polymerase chain reaction amplification and sequencing of ALG6 exons, three mutations, in addition to the previously described A333 V substitution, were detected in CDG-Ic patients. The detrimental effect of these mutations on ALG6 activity was confirmed by complementation of alg6 yeast mutants. Haplotype analysis of CDG-Ic patients revealed a founder effect for the ALG6 allele bearing the A333 V mutation. Although more than 80% of CDG are type Ia, CDG-Ic may be the second most common form of the disease.
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  • 5
    ISSN: 1432-1076
    Keywords: Key wordsCandida albicans ; Premature ; Renal fungal balls ; Liposomal amphotericin B ; Fluconazole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract At the age of 8 weeks, an extremely low birth weight infant (gestational age 26 0/7 weeks, birth weight 740 g) had non-obstructing bilateral renal fungal balls. Urine cultures had repeatedly grown Candida albicans. Combination therapy with liposomal amphotericin B intravenously and fluconazole orally was administered for 6 weeks. Monotherapy with fluconazole was then continued until complete resolution of the renal fungal balls. Conclusion Combination therapy with liposomal amphotericin B and fluconazole was successful in eliminating non-obstructing bilateral renal fungal balls and obviated the need for surgical intervention.
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  • 6
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Our previous study demonstrated that soluble CD14 (sCD14) modulates the biologic activity of circulating endotoxin, which appears after surgery. In this study, we examined the behavior of endotoxin-binding proteins, such as sCD14, lipopolysaccharide-binding protein (LBP), and bactericidal/permeability-increasing protein (BPI), in patients' plasma after major abdominal surgery and the phagocytic secretion of sCD14 from peripheral blood mononuclear cells (PBMCs) throughout the observation period. In a prospective study, 15 patients undergoing major abdominal surgery (gastrectomy, n= 3; pancreatectomy, n= 10; colectomy, n= 2) were involved in this study. The endotoxin-binding proteins were perioperatively (preoperatively; postoperative hour 6; days 1, 2, 3, 4, 5, 7, and 10) measured by an enzyme-linked immunosorbent assay (ELISA). To exclude the hemodilution effect of samples, each parameter was corrected by dividing the respective value by the albumin concentration. The phagocytic activity at each time point was tested as an ex vivo sCD14 secretion from PBMCs in the presence and absence of exogenously added endotoxin, Escherichia coli 055B5 (1 ng/ml). Significant endotoxemia (0.35 ± 0.13 EU/ml; p 〈 0.05) was observed 6 hours after the beginning of surgery. The sCD14/albumin value rapidly increased at 6 hours after surgery, peaked on day 1, and sequentially declined, whereas the BPI/albumin and LBP/albumin ratios increased more gradually and peaked on day 2. The secretion of sCD14 from 2 × 106 PBMCs was significantly enhanced from 6 hours after operation. The increased plasma level of sCD14 may be explained by the parallel-enhanced sCD14 PBMC production. Activated secretion of these endotoxin-binding proteins may play a role in regulating the biologic activity of circulating endotoxin.
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  • 8
    ISSN: 1433-0350
    Keywords: Key words External ventricular drainage ; Preterm infant ; Intraventricular-periventricular hemorrhage ; Posthemorrhagic hydrocephalus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We previously reported on a series of 27 newborn infants treated for posthemorrhagic hydrocephalus with external ventricular drainage during 1984 to 1989. In the present study we continued to evaluate this technique during the subsequent 8 years (37 patients; mean birthweight 1251±478 g; mean gestational age 29±2.9 weeks; 51 drains), and we now report on the long-term experience with this method, complications, and neurodevelopmental outcome of the survivors. The mean age at drain insertion was 21 days, and the mean duration of drainage 23 days. Eight infants died of causes unrelated to external ventricular drainage. Eleven of the survivors did not require a permanent shunt. Two patients experienced ventriculitis, resulting in an infection rate of 5.4% per patient and 3.9% per drain. The neurodevelopmental outcome was mainly dependent on the extent of the pre-existing parenchymal injury. We conclude that external ventricular drainage is a safe and effective technique for the management of preterm infants with posthemorrhagic hydrocephalus.
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  • 9
    ISSN: 1433-0385
    Keywords: Keywords: Burn injury ; Artificial skin ; Neodermis ; Scar. ; Schlüsselwörter: Verbrennung ; Kunsthaut ; Neodermis ; Narbe.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung. Von Februar 1996 bis April 1999 wurden in unserer Klinik 9 Patienten mit primären Verbrennungswunden und 13 Patienten mit sekundären instabilen Narben mit Integra – artificial skin – gedeckt. Dieser künstliche Hautersatz, bestehend aus einer Kollagen/Glykosaminoglykanmatrix und einer Silikonschicht wurde 1980 erstmalig in den USA eingesetzt. Durch seine mechanischen und biologischen Eigenschaften dient er in der Akutphase nach Verbrennungen dem Schutz vor Flüssigkeitsverlust und Bakterieninvasion. Langfristig wird durch integra-induzierte körpereigene Produktion einer Neodermis eine in funktioneller und ästhetischer Hinsicht bessere Narbenbildung erreicht. Wir berichten über unsere klinischen Erfahrungen mit dem Einsatz von Integra in Akut- und Langzeitverlauf und über Langzeitbeobachtungen.
    Notes: Abstract. We report on our experience with Integra application in 22 patients treated from February 1996 through April 1999. Nine patients suffered a primary burn injury and 13 patients had secondary unstable scars. Integra is artificial skin that consists of a collagen/glycosaminoglycane matrix and a silicon layer. It was first used by Burke and Yannas in 1980 in the USA. The mechanical and biological features of Integra protect the tissue from fluid loss and bacterial invasion in the acutely burned patient. The integra-induced formation of a neodermis leads to functionally and aesthetically highly acceptable scar formation. We present our clinical experience with Integra in long-term follow-up.
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Der Gynäkologe 33 (2000), S. 494-502 
    ISSN: 1433-0393
    Keywords: Schlüsselwörter Mammakarzinom ; Zervixkarzinom ; Korpuskarzinom ; Ovarialkarzinom ; Rehabilitation ; Keywords Oncology ; Rehabilitation ; Gynaecological carcinomas ; Physiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract In Germany about 41% of malignant neoplasies of women are carcinomas of mamma, cervix, Corpus uteri and ovary. Therapy of these malignomas consists of surgery, radio-, chemo- or hormonotherapy. Apart from therapeutic effects primary therapy may have negative effects in different physical, psychical and social aspects. These side effects are the concern of stationary oncological rehabilitation. Interdisciplinary cooperation makes it possible to consider the individual requirements of the patient.
    Notes: Zusammenfassung In Deutschland machen das Mamma-, Zervix-, Korpus- und Ovarialkarzinom ca. 41% der malignen Neoplasien bei der Frau aus. Die Behandlung dieser Malignome beinhaltet Operation, Strahlen-, Chemo- oder Hormontherapie. Neben der therapeutischen Wirkung kann es bei den Patientinnen durch die Primärbehandlung auch zu unterschiedlich ausgeprägten Beeinträchtigungen im physischen, psychischen oder sozialen Bereich kommen. An dieser Stelle greift die stationäre onkologische Rehabilitation an, die in interdisziplinärer Zusammenarbeit die individuellen Bedürfnisse der Patientinnen berücksichtigt.
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