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  • 1
    Electronic Resource
    Electronic Resource
    Adenosine Deaminase and Thymocyte Maturation (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 33 (1991), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The congenital absence of adenosine deaminase in humans results in severe combined immunodeficiency. To clarity the process whereby thymocytes are destroyed in the absence of adenosine deaminase activity, we induced a parallel condition in mice through the injection of an inhibitor of adenosine deaminase, deoxycoformycin. We have observed that deoxycoformycin, in addition to maintaining high levels of dATP in thymocytes, blocks the progression of thymocyte differentiation at two points. As a result of the first block, the corlex is depleted of immature cortical thymocyles while CD4+ CD8+ thymocytes with functionally rearranged T-cell receptors survive. As a result of the second block, the CD4+ CD8− thymocytes are prevented from further differentiation lo mature CD4− CD8+ or CD4− CD8+ T lymphocytes and accumulate at the corticomedullar junction and in the medulla. These observations suggest that the maintenance of dNTP pools by adenosine deaminase is critical to at least two stages of thymocyte differentiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1991.tb01788.x
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  • 2
    Electronic Resource
    Electronic Resource
    The Pro-Peptide of the Proβ-Polypeptide Chain of Human β-Hexosaminidase Is Necessary for Proper Protein Folding and Exit from the Endoplasmic Reticulum
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 204 (1994), S. 135-141 
    Details
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1006/bbrc.1994.2436
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Cutaneous telangiectasias, sparse hair, and type I membranoproliferative glomerulonephritis (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 129-131 
    Details
    ISSN: 1432-198X
    Keywords: Key words Membranoproliferative glomerulonephritis ; Cutaneous telangiectasias ; Sparse hair ; Atrial septal defect ; Ectodermal dysplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report the unusual association of normocomplementemic type I membranoproliferative glomerulonephritis in a 10-year-old girl with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasias, and an atrial septal defect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050578
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  • 4
    Electronic Resource
    Electronic Resource
    Acute plasmacytic interstitial nephritis in a child with Down syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 333-335 
    Details
    ISSN: 1432-198X
    Keywords: Key words Down syndrome ; Acute interstitial nephritis ; Polyclonal plasma cells ; Acute renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A 7.5-year-old boy with Down syndrome presented in acute renal failure (ARF) needing dialysis. When 1.5 years old he had a neuroblastoma, was treated for 1 year with chemotherapy and radiotherapy, and off chemotherapy had since been in remission. Renal biopsy revealed an interstitial inflammation, principally of plasma cells with some lymphocytes and eosinophils. Immunofluorescence showed no deposition of immunoglobulins or complement (C3). The plasma cells were a mixture of kappa and lambda light chain-producing cells. The patient spontaneously improved a week after admission. Initial ultrasonography showed enlarged kidneys with loss of corticomedullary differentiation. We are unaware of a report of ARF in a child, resulting primarily from a polyclonal plasmacytic interstitial nephritis. The etiology remains unclear.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050620
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  • 5
    Electronic Resource
    Electronic Resource
    MRI of fibromatosis: With pathologic correlation (1992)
    Springer
    Pediatric radiology 22 (1992), S. 587-589 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fibromatosis refers to group of benign but sometimes locally aggressive proliferative lesions of myofibroblasts. These are characterized by infiltrative growth, and hence may mimic a malignant lesion. These lesions reveal a low signal intensity on T1-weighted pulse sequences, but may show low or high signal intensity on the T2-weighted sequences. Histologic correlation reveals that the lesion showing low signal intensity on T2-weighted sequences has a larger collagenous component, and reduced cellularity comoared with the lesion showing high signal intensity on T2-weighted sequences.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02015358
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  • 6
    Electronic Resource
    Electronic Resource
    Sonographic appearance of Sertoli cell tumour: With pathologic correlation (1993)
    Springer
    Pediatric radiology 23 (1993), S. 127-128 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sertoli cell tumor is a rare tumor of the testis, with a good prognosis following orchiectomy in the pediatric patient. Sonography of such a tumor in a 5-year-old boy revealed diffuse inhomogeneous increase in echogenicity in an enlarged left testicle. The increased echogenicity of the testicle reflected the dense collagenous matrix of the lesion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02012404
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  • 7
    Electronic Resource
    Electronic Resource
    Perforation during attempted intussusception reduction in children —a comparison of perforation with barium and air (1995)
    Springer
    Pediatric radiology 25 (1995), S. 81-88 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This paper compares the effects on patients of perforation with barium and with air during attempted intussusception reduction by reviewing the clinical, radiological, surgical and pathological findings and sequelae in seven children who received barium and seven who received air. In both groups perforation occurred in infants under 6 months of age (with one exception) with a long duration of symptoms. All patients with barium enema required resection of bowel whereas only four with air enema required resection. Anesthetic times were longer in those patients with barium perforation in whom the intussusception did not move and there was a large leak. The patients with perforation due to air had a shorter hospital stay with decreased morbidity compared to those with perforation due to barium. Perforation occurred through areas of transmural necrosis in a minority of patients in each group. Perforations through normal bowel and shear injury (with air enema) indicate that increased pressure during the examination is an important factor in some patients. Because perforation with air is so much easier to deal with surgically and the children do better clinically, there is a tendency for some to consider perforation with air an inconsequential situation. However, a potential rare complication with this technique is tension pneumoperitoneum. Keeping this in mind, we continue to use air as the contrast of choice because the procedure in our hands is a safe, quicker and easier technique and we have achieved a substantial improvement in reduction rates. Overall reported perforation rates with air enema compare favourably with those due to barium enema.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02010311
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