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一位常染色体显性遗传高免疫球蛋白 E(job's)综合征的年轻女性,以急性腹痛为表现:病例报告。

A Young Woman with Autosomal Dominant Hyper Immunoglobulin E (job's) Syndrome Presenting with Acute Abdomen: a Case Report.

机构信息

Surgical Department, University Hospital of Patras, Patras, Greece.

Radiology Department, General Hospital of Patras, Patras, Greece.

出版信息

Med Arch. 2022 Dec;76(6):476-479. doi: 10.5455/medarh.2022.76.476-479.

Abstract

BACKGROUND

Autosomal dominant hyper immunoglobulin IgE syndrome is a rare inherited condition that causes immune suppression.

OBJECTIVE

This case report describes a severe case of liver abscess, caused by a cavity-forming infection, secondary to Job's syndrome.

CASE PRESENTATION

A 25-year-old female patient was emergently referred to the surgical department, for the evaluation of acute, right-sided, upper abdominal pain, fever, and chills. The patient reported a past history of recurrent pulmonary infections as well as a prior diagnosis of Job's syndrome. An abdominal CT scan revealed a large intrahepatic cystic mass, consistent with a hepatic abscess on the right liver lobe. The patient was started on a course of antibiotics and was admitted to the surgical ward for further treatment. After much deliberation, a multidisciplinary team comprised of general surgeons, gastroenterologists, and interventional radiologists, decided upon the guided drainage of the abscess. Two pigtail catheters were used to drain the cavities. Antibiotic use was de-escalated, the patient gradually recovered, and the reported abscesses were greatly reduced in size. After 14 days of treatment, the patient was successfully released home.

CONCLUSION

In patients with a known history of the autosomal dominant hyper-IgE syndrome, presenting with acute abdominal pain, the liver abscess must be on the top of the differential diagnosis list.

摘要

背景

常染色体显性遗传高免疫球蛋白 E 综合征是一种罕见的遗传性疾病,可导致免疫抑制。

目的

本病例报告描述了一例由乔氏综合征继发的腔隙形成感染引起的严重肝脓肿病例。

病例介绍

一名 25 岁女性患者因突发右季肋部疼痛、发热和寒战,被紧急转至外科就诊。患者自述有反复肺部感染病史,曾被诊断为乔氏综合征。腹部 CT 扫描显示肝右叶有一个大的肝内囊性肿块,符合肝脓肿的表现。患者开始接受抗生素治疗,并被收入外科病房进行进一步治疗。经过多学科团队(包括普通外科医生、胃肠病学家和介入放射科医生)的讨论,决定对脓肿进行引导引流。使用两条猪尾导管引流脓肿。抗生素的使用逐渐减少,患者逐渐康复,报告的脓肿明显缩小。经过 14 天的治疗,患者成功出院。

结论

对于有常染色体显性遗传高免疫球蛋白 E 综合征病史、出现急性腹痛的患者,肝脓肿必须作为鉴别诊断的首要考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91fe/10019861/1a3500b280e1/medarch-76-476-g001.jpg

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