医药学论文-肾淀粉样变伴甲状腺淀粉样变一例及文献复习

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Ozdemir BHµÈ[9]?Ô50Àý?Ì??ÐÔϵÍ?ÐÔµí?ÛÑù?äµÄ??Õß?øÐÐÑÐ??????ÏÖÆäÖÐÓÐ38Àý?íÏÖΪ?××?ÏÙÖ×?ó??38Àý??Õß?ùÐÐÕëÎü?î×éÖ??ì?é???á?ûÏÔÊ?10ÀýΪ?××?ÏÙ?úÄÜÕý??µÄ??Ì?×ÛºÏÖ???2ÀýΪ?××?ÏÙ?úÄÜ?º?ø??2ÀýΪ?××?ÏÙ?úÄÜ?õÍË??1ÀýΪÑÇ??ÐÔ?××?ÏÙÑ×??ÆäÓà??ÕßµÄѪ???××?ÏÙ??ËØ???Ù?××?ÏÙ??ËØ?ùÕý????×?ÕßÈÏΪ?××?ÏÙµí?ÛÑù?äÒýÆðµÄ?××?ÏÙÖ×?ó?ÔÆä×ÔÉí??ÄÜ×?Ì?µÄÓ?Ïì?????ó???? Ê?ÊÇÆä×éÖ???µí?ÛÑùÎïÖÊ?ã?ºÈ??ú????Àý??ÕßΪÖÐÄêÅ?ÐÔ??ÁÙ???íÏÖΪË?Ö×??µ??×Äò??µÍ?×µ??×ѪÖ???Éö??ÄÜËðº????××?ÏÙÖ×?ó??Óë??µÀµÄµÄ??ÀýÁÙ???íÏÖ?ù??ÏàËÆ??ÈëÔººó?øÓè??ËØ??ÇïË?ÏÉ?îµÈÒ?Îï??Àíºó????ÇéµÃµ?Ò????Ì?ȵÄ?ØÖÆ??ÆäÁÆÐ?ÓëÓÐ?ØÎÄÏ×[7]??µÀÏàÀàËÆ??µ?ÕâÐ??ùΪ?ö???????ßÓÐÆÕ?éÐÔ???ü?àµÄÑ?ÕßÈÏΪ????Ä?Ç?ÉÐÎÞÌØÐ?ÁÆ????????ÕßµÄ×ÊÁÏ??Ïà?ØÎÄÏ×µÄ??µÀ?ùÌáÊ???ÔÚϵÍ?ÐÔµí?ÛÑù?äÖÐ?××?ÏÙ?ÏÉÙÊÜÀÛÇÒÊ?ÆÚ?ÏÍí????Õß×?ÔºÆÚ?äµÄ?××?ÏÙ??ÄÜÕý?????ÔÓÚµí?ÛÑù?ä??ÕßÒª×?Òâ?àϵÍ??ì?é??ÓÈÆäÊÇ?éÓÐÏà?ØϵÍ?µÄÖ?×??òÌåÕ?Ê????ñÔòÈÝÒ×Â?Õï?òÎóÕï?? ×?Õß?ºº?ÎÄÂ× ?Æ??ÐË ???Î??ÎÄÏ×?? [1]Muro K, Kobayashi M, Shimizu Y, et al. A case of systemic AA amyloidosis complicating Crohn's disease [J]. Nippon Jinzo Gakkai Shi,1998,40(4):284-289.[2]Kapadia HC, Desai RI, Desai IM, et al. Amyloid goiter-a case report[J]. Indian J Pathol Microbiol,2001,44(2):147-148.[3] Shimizu H, Ishikawa K, Kobayashi H, et al. Familial amyloidotic polyneuropathy with a transthyretin variant (Val30-->Leu) [J]. No To Shinkei,1996,48(2):175-178.[4] Kavukcu S, Turkmen M, Eroglu Y, et al. Renal, gastric and thyroidal amyloidosis due to familial Mediterranean fever[J]. Pediatr Nephrol,1997,11(2):210-212.[5] Cohan P, Hirschowitz S, Rao JY, et al. Amyloid goiter in a case of systemic amyloidosis secondary to ankylosing spondylitis[J]. J Endocrinol Invest,2000,22(11):762-764.[6] Coli A, Bigotti G, Zucchetti F, et al. Papillary carcinoma in amyloid goitre[J]. J Exp Clin Cancer Res,2000,19(3):391-394.[7] Siddiqui MA, Gertz M, Dean D. Amyloid goiter as a manifes- tation of primary systemic amyloidosis[J]. Thyroid ,2007,17(1):77-80.[8] Kallinich T, Briese S, Roesler J, et al. Two familial cases with tumor necrosis factor receptor-associated periodic syndrome caused by a non-cysteine mutation (T50M) in the TNFRSF1A gene associated with severe multiorganic amyloidosis[J]. J Rheumatol,2004,31(12):2519-2522.[9] Ozdemir BH, Uyar P, Ozdemir FN. Diagnosing amyloid goi- tre with thyroid aspiration biopsy[J]. Cytopathology,2006, 17(5):262-266.