Enterocolitis in infantile common variable immunodeficiency. A case report and review of the literature.
Enterocolitis in infantile common variable immunodeficiency. A case report and review of the literature.
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婴儿常见变异性免疫缺陷中的小肠结肠炎。
DOI：
10.1007/bf02282351
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发表时间：
1996
影响因子：
3.1
通讯作者：
Mulberg,AE
中科院分区：
文献类型：
--
作者：
John,HA;Sullivan,KE;Smith,C;Mulberg,AE
LH是一名32岁妊娠2帕拉1白色女性通过剖腹产分娩的31周妊娠的前977 g产物，其妊娠并发胎动减少，但无宫内感染证据。在5个月大时，体重和身高明显低于第5百分位数，并注意到双侧中耳炎和鹅口疮。八个月大时的实验室数据显示IgG< t0 mg/dl（正常241-613 mg/dl），IgM < 7 mg/dl（正常26-60 mg/dl）和伊加< 8 mg/dl（正常10-46 mg/dl）; B细胞不存在，淋巴细胞的促分裂原反应被抑制（下表3）。根据免疫学标准诊断为CVID，并开始静脉注射免疫球蛋白（1VIG）400 mg/kg/week治疗。结肠镜检查显示距离肛门边缘5 cm处有直肠狭窄。上消化道造影显示小肠多处扩张，钡灌肠证实直肠乙状结肠狭窄。存在艰难梭菌毒素，口服万古霉素治疗感染10天。建立了结肠袢造口术和直肠粘液瘘。初次手术后4周，食管胃活检正常;结肠活检显示结肠粘膜发炎伴隐窝炎和隐窝脓肿，无肉芽肿。开始静脉注射甲泼尼龙、柳氮磺吡啶、美沙拉嗪灌肠和全肠外营养治疗，但无效。常规肠道培养阴性。
LH is the former 977-g product of a 31-week gestation born by Cesarean section to a 32-year-old gravida 2 para 1 white female whose gestation was complicated by decreased fetal movement but no evidence of intrauterine infection. At five months of age, weight and height were markedly below the 5th percentile and bilateral otitis media and oral thrush were noted. Laboratory data at eight months of age revealed IgG< t0 mg/dl (normal 241-613 mg/dl), lgM< 7 mg/dl (normal 26-60 m~ dl) and IgA< 8 mg/dl (normal 10-46 mg/dl); B cells were absent, and mitogen response of lymphocytes was depressed (Table 3 below). CVID was diagnosed based on immunological criteria, and treatment with intravenous immune globulin (1VIG) 400 mg/kg/week was initiated.Colonoscopy revealed a rectal stricture at 5 cm from the anal verge. Upper gastrointestinal series demonstrated multiple dilated small bowel loops and barium enema confirmed a rectosigmoid stricture. Closttqdium difficile toxin was present, and the infection was treated with oral vancomycin for 10 days. A loop colostomy and a rectal mucus fistula were established. Four weeks after initial surgery, esophagogastroduodenoscopy with biopsies was normal; colonic biopsies revealed inflamed colonic mucosa with cryptitis and crypt abscess without granuloma. Treatment with intravenous methylprednisolone, sulfasalazine, mesalamine enemas, and total parenteral nutrition was started but were ineffective. Routine intestinal cultures were negative.