关键词: Chronic granulomatous disease Gastric outlet obstruction Gastrointestinal endoscopy

来  源:   DOI:10.22037/ghfbb.v17i2.2898   PDF(Pubmed)

Abstract:
UNASSIGNED: Chronic granulomatous disease (CGD) is a rare disorder normally diagnosed in infancy.
UNASSIGNED: A 27-year-old man admitted with non-specific symptoms of CGD first underwent endoscopy, and colonoscopy procedures as primary evaluation of clinical presentation. Eighteen months after the first admission, he was referred to the emergency department for hematemesis, and critical situations, such as a severe anemic with Hgb= 2.6 mg/dl. As a result of this specific clinical presentation, urgent emergency treatment was performed, and endoscopic examination revealed ulcers and abnormalities in the duodenal bulb and jejunum. Other imaging procedures, such as sonography, and abdominal CT scans, showed splenomegaly. He underwent splenectomy, and after that, endoscopic treatment with balloon TTS dilation was scheduled, but this procedure failed. So, we decided to do a gastro-jujenostomy that alleviated the clinical symptoms. After nine months, he was referred to GOO, and endoscopic evaluation showed giant ulceration with severe stricture in the duodenum, and a polyp in the jejunostomy. Finally, Based on clinical presentation and pathologic evidence of biopsies, the patient approached CGD as the final diagnosis.
UNASSIGNED: Step-by-step, rule out of different highly suspicious diseases may result in a definite CGD diagnosis, and rapid management of these patients may increase the chance of survival.
摘要:
慢性肉芽肿病(CGD)是一种通常在婴儿期诊断的罕见疾病。
一名27岁的男性因CGD的非特异性症状而首次接受了内窥镜检查,和结肠镜检查程序作为临床表现的主要评估。第一次入院18个月后,他因呕血被转诊到急诊室,和危急情况,如Hgb=2.6mg/dl的严重贫血。由于这种特殊的临床表现,进行了紧急紧急治疗,内镜检查显示十二指肠球部和空肠溃疡和异常。其他成像程序,比如超声检查,腹部CT扫描,表现为脾肿大.他做了脾切除术,之后,计划采用球囊TTS扩张的内镜治疗,但是这个程序失败了。所以,我们决定做胃造口术以减轻临床症状。九个月后,他被称为GOO,内镜检查显示巨大溃疡伴十二指肠严重狭窄,和空肠造口术中的息肉.最后,根据活检的临床表现和病理证据,患者将CGD作为最终诊断。
循序渐进,排除不同的高度可疑疾病可能导致明确的CGD诊断,和这些患者的快速管理可能会增加生存的机会。
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