SEVERE DIARRHEA CONTROLLED BY GAMMA GLOBULIN IN A PATIENT WITH AGAMMAGLOBULINEMIA AMYLOIDOSIS AND THYMOMA
SEVERE DIARRHEA CONTROLLED BY GAMMA GLOBULIN IN A PATIENT WITH AGAMMAGLOBULINEMIA AMYLOIDOSIS AND THYMOMA
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DOI：
10.7326/0003-4819-65-3-528
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发表时间：
1966-01-01
影响因子：
39.2
通讯作者：
QUINTILIANI, R
中科院分区：
文献类型：
--
作者：
CONN, HO;QUINTILIANI, R
本报告描述了一位老年患者，他发展为获得性无球蛋白血症，伴有反复呼吸道感染和严重腹泻。与吸收不良无关的腹泻对丙种球蛋白治疗有显著的反复反应。在尸检中发现淀粉样蛋白存在于小肠粘膜和血管，以及脾脏、肾上腺和甲状腺。去世前一年十二指肠活检未检出淀粉样蛋白。回顾了38例报告的无球蛋白血症与腹泻的关系。其中一些患者的腹泻对抗生素或丙种球蛋白治疗的反应表明，肠道细菌是导致腹泻的原因。无球蛋白血症中对细菌感染的防御能力受损似乎可能是由于正常情况下非致病性细菌群过度生长或侵入肠壁而导致腹泻。该患者肠道淀粉样蛋白沉积的意义和机制尚不清楚。无大球蛋白血症和甲状腺瘤的关联，仅报道与获得性无大球蛋白血症，伴脾肿大，白细胞减少和贫血。虽然胸腺瘤通常比复发性感染早几年发生，但胸腺切除术并不能改变病情。
This report describes an elderly patient who developed acquired agammaglobulinemia with recurrent respiratory infections and severe diarrhea. The diarrhea, which was not associated with malabsorptlon, responded dramatically and repeatedly to gamma globulin therapy. At autopsy amyloid was found in the mucosa and blood vessels of the small intestine, as well as in the spleen, adrenals and thyroid. No amyloid was detected on biopsy of the duodenum a yr. before his death. The association of agammaglobulinemia and diarrhea, reported in 38 patients, was reviewed. The response of the diarrhea in some of these patients to antibiotic or gamma globulin therapy suggests that intestinal bacteria was responsible for the diarrhea. It seems probable that impaired defenses against bacterial infection in agammaglobulinemia cause diarrhea by permitting overgrowth or invasion of the intestinal wall by normally nonpathogenic bacterial flora. The significance and the mechanism of the intestinal amyloid deposition in this patient are unknown. The association of agammaglobulinemia and thvmoma, which was reported only with acquired agammaglobulinemia, is accompanied by splenomegaly, leukopenia and anemia. Although the thymoma often preceded the recurrent infections by some years, thymectomy did not alter the disease.