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Andrea Hunold MD Gerhard Alzen MD Stefan A. Wudy MD Renate Bluetters‐Sawatzki MD Eva Landmann MD Alfred Reiter MD Hans‐Joachim Wagner MD 《Pediatric blood & cancer》2009,52(5):677-679
We report a 12‐year‐old female presenting with an abdominal tumor. Diagnostic workup revealed giant bilateral ovarian cysts, severe hypothyroidism as well as an elevation of CA 125. We refrained from ovariectomy, which would be necessary for a malignant tumor, in view of an evident Van Wyk and Grumbach syndrome. The patient promptly responded to L ‐thyroxine with complete regression of all symptoms. Hypothyroidism should be considered in the evaluation of ovarian cysts. Although the Van Wyk and Grumbach syndrome is rare, it is crucial to rule it out in order to avoid unnecessary ovarian surgery when thyroid replacement is completely sufficient. Pediatr Blood Cancer 2009;52:677–679. © 2009 Wiley‐Liss, Inc. 相似文献
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Sebastian Müller Ingo Marenholz Young-Ae Lee Claudia Sengler Simona Eva Zitnik Rupino W. Griffioen Paolo Meglio Ulrich Wahn Renate Nickel 《Pediatric allergy and immunology》2009,20(4):358-361
Many candidate gene studies for atopic dermatitis (AD) and associated phenotypes have been conducted so far, but replication of significant results has been a major problem. Two loss of function polymorphisms FLG R501X- and 2282del4, in the Filaggrin ( FLG ) gene encoding for an epidermal barrier protein were recently identified. They were reported to be predisposing factors for AD and concomitant asthma. Several groups confirmed the initial results in independent populations. The aim of this study is to further investigate the importance of these FLG variants in the development of AD and subsequent asthma symptoms in pre-school children, we investigated children and parents of the Early Treatment of the Atopic Child (ETAC)-trial. We genotyped 496 children and 488 parents of the ETAC population for the two FLG variants, evaluating an association by family based analysis (transmission disequilibrium test). We found a highly significant association of the FLG null variants R501X- and 2282del4 with AD (combined genotype p < 0.0001) and asthma (combined genotype p < 0.0001). The replication and its statistical significance underlines the importance of the FLG polymorphisms and the importance of the skin barrier function in the development of AD and subsequent asthma. 相似文献
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Congenital and acquired nystagmus, particularly pendular and jerk nystagmus, see-saw nystagmus and spasmus nutans, may be the presenting sign of a suprasellar mass lesion.(1) The large variety of different suprasellar mass lesions requiring quite different therapeutic measures necessitates exact histological diagnosis for optimal therapeutic strategy planning.(2) Stereotactic tumor biopsy has become a well-established diagnostic approach, combining minimal surgical trauma with a high degree of diagnostic safety. Particularly in the two most frequent suprasellar mass lesions - craniopharyngiomas and pilocytic astrocytomas - accurately planned stereotactic drainage of tumor cysts combined with radiotherapy and/or stereotactic radiosurgery allows successful decompression and tumor control as well as maximum preservation of visual and endocrinological functions when compared with conventional surgical procedures.(2-11) 相似文献
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Viestenz A Küchle M Seitz B Langenbucher A Viestenz A Ferreira de Souza R Naumann GO 《Cornea》2002,21(5):501-504
PURPOSE: To assess thermal effects of Q-switched Er:YAG laser trephination to corneal epithelium and superficial stroma using different mask types and materials for experimental penetrating keratoplasty. METHODS: Laser trephination was performed in 20 freshly-enucleated porcine eyes (repetition rate 5 Hz, pulse energy 65 mJ, spot size 0.7 mm). We used flat, open-metal and ceramic masks for donor and recipient trephination placed directly onto the corneal surface. Main outcome measures as assessed by light microscopy after PAS staining of 8-microm paraffin sections included: extension of tissue thermal damage at the cut edge in the superficial and basal epithelial layers, the basement membrane and subepithelial stroma, and depth and width of epithelial/stromal involvement in the area of the donor mask contact. RESULTS: The thermal damage in the superficial epithelium was more pronounced in donor (mean extension 61.6 +/- 15.6 microm) than in recipient (29.4 +/- 24.9 microm, p= 0.05) trephination. In donor trephination, thermal damage zone of the superficial epithelial layer was significantly smaller with ceramic than with metal masks (21.0 +/- 23.0 versus 61.6 +/- 15.6 microm, p= 0.014). In contrast, differences at basal epithelial layer (p= 0.44), basement membrane (p= 0.79), and subepithelial stroma (p= 0.2) were not statistically significant. Superficial donor involvement of the cornea adjacent to the paracentral donor mask contact zone was seen neither with ceramic nor with metal masks. CONCLUSION: Superficial corneal alterations adjacent to the mask-cornea contact zone may be minimized by using the Er:YAG laser in a Q-switched mode. Ceramic masks, in contrast to metal masks, further reduce superficial thermal alterations at the cut edge. 相似文献
99.
Effect of oral application of an immunomodulating plant extract on Influenza virus type A infection in mice 总被引:5,自引:0,他引:5
Bodinet C Mentel R Wegner U Lindequist U Teuscher E Freudenstein J 《Planta medica》2002,68(10):896-900
The influence of the oral administration of an aqueous-ethanolic extract of a mixture of Thujae occidentalis herba, Baptisiae tinctoriae radix, Echinaceae purpureae radix and Echinaceae pallidae radix, on the course of Influenza A virus infection in Balb/c mice was investigated. The extract was administered to mice via the drinking water for 14 days starting 6 days before intranasal infection with Influenza A virus. The progress of infection was recorded during a time range of 21 days. Parameters for the evaluation of antiviral activity were survival rate and mean day to death. In a further set of experiments infected mice were sacrificed on defined days. Determination of consolidation score and virus titer were performed for each lung. The data show that the oral treatment with the extract induced a statistically significant increase in the survival rate, prolonged the mean survival time and reduced lung consolidation and virus titer. The experiments demonstrate that the plant immunomodulator given 6 days before exposure is a potent inhibitor of Influenza A virus pathology in vivo. 相似文献
100.
Thiele A Bang R Gütschow M Rossol M Loos S Eger K Tiegs G Hauschildt S 《European journal of pharmacology》2002,453(2-3):325-334
Thalidomide has been shown to reduce the production of tumor necrosis factor-alpha (TNF-alpha), a cytokine with deleterious pathophysiologic effects in various diseases. In search of thalidomide analogues with improved TNF-alpha inhibiting properties, 5-ethyl-1-phenyl-5-(3,4,5,6-tetrafluorophthalimido)barbituric acid (TFBA) was found to be superior to thalidomide. Besides TNF-alpha, TFBA also suppressed interleukin-6 and interleukin-10 production of isolated monocytes. The possibility that TFBA exerts its action by increasing levels of cAMP via inhibition of phosphodiesterase-4 activity was excluded. TFBA had no influence on T cell proliferation; neither did it inhibit TNF-alpha production in peripheral blood mononuclear cells stimulated by anti-CD3 monoclonal antibody. When applied to mice treated with D-galactosamine and lipopolysaccharide, TFBA prevented a rise in serum TNF-alpha, had no effect on interleukin-6 levels and led to an increase in interleukin-10 production. The changes in cytokine production observed in vitro and in vivo were reflected by similar changes in the mRNA expression. Moreover, TFBA significantly reduced liver transaminase levels in D-galactosamine/lipopolysaccharide-treated mice and thus efficiently protected the animals from liver injury. Thus, according to its properties, TFBA has the potential of modulating an immune response by acting as an anti-inflammatory agent. 相似文献