胃と腸40巻8号

2005年07月発行

今月の主題 免疫異常と消化管病変 主題症例

慢性大腸炎を合併した糖原病Ib型の1例

著者： 江崎幹宏¹ 松本主之¹ 中村昌太郎¹ 山縣元¹ 井原健二² 八尾隆史³ 飯田三雄¹

所属機関： ¹九州大学大学院医学研究院病態機能内科学 ²九州大学大学院医学研究院成長発達医学 ³九州大学大学院医学研究院形態機能病理学

ページ範囲：P.1190 - P.1196

要旨　症例は17歳，男性．生後3か月時に糖原病Ib型と診断され，10歳時より好中球減少症による易感染性に対してG-CSFの投与を受けていた．1999年1月より1日4～5行の水様性下痢が出現したため当科を受診した．注腸X線検査では全大腸に及ぶ鉛管状ないしは萎縮瘢痕帯様の所見がみられ，横行結腸と下行結腸には輪状傾向を有する不整形潰瘍が認められた．内視鏡検査では，不整形潰瘍に加えて介在粘膜の血管透見像の乱れが観察された．以後6年間の経過観察中，不整形潰瘍が散発性に出没し，腸管長軸方向への短縮がわずかに進行したが，腸管狭小化や瘻孔出現はなかった．糖原病Ib型では慢性炎症性腸病変を合併することが知られているが，自験例ではその進行は緩徐であった．

参考文献

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