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因干燥综合征导致的以雾视伴人工晶状体屈光不正为表现的蛋白丢失性胃肠病 1 例。

A case of protein-losing gastroenteropathy due to Sjögren's syndrome detected by foggy vision with refractive error of the intraocular lens.

机构信息

Department of Gastroenterology, Kanto Rosai Hospital, 1-1 Kizukisumiyoshi-cho, Nakahara-ku, Kawasaki, Kanagawa, 211-8510, Japan.

Division of Gastroenterology and Hepatology, Department of Internal Medicine (Omori), School of Medicine, Faculty of Medicine, Toho University, 6-11-1 Omorinishi, Ota-ku, Tokyo, 143-8541, Japan.

出版信息

Clin J Gastroenterol. 2024 Dec;17(6):1039-1046. doi: 10.1007/s12328-024-02038-2. Epub 2024 Sep 19.

DOI:10.1007/s12328-024-02038-2
PMID:39297920
Abstract

A woman in her forties visited an ophthalmologist for rapidly progressive foggy vision. Naked visual acuity had decreased to 0.15, and although her eyes showed no abnormalities, internal disease was suspected and albumin 2.6 g/dL was found. Protein leakage from the intestinal tract was suspected since there was no urinary protein excretion. Technetium-labeled albumin D scintigraphy showed protein leakage from the intestinal tract. A stool α1-antitrypsin clearance test showed an increase to 56.3 mL/day, leading to a diagnosis of protein-losing gastroenteropathy. Blood biochemistry revealed abnormally high levels of anti-SS-A and anti-SS-B antibodies (≥ 1200 U/mL and ≥ 1000 U/mL, respectively). A lip salivary gland biopsy revealed lymphocytic infiltrate at least 1 focus per 2 mm × 2 mm > 50 lymphocytes per conduit). The Schirmer test result was 5 mm/5 min or less, which led to the diagnosis of Sjögren's syndrome. The serum albumin level increased with intravenous administration of methylprednisolone 50 mg (1 mg/kg), and the patient is currently on oral prednisolone at a gradually decreasing dose. After administration of prednisolone, visual acuity recovered to 1.2 with recovery of albumin.

摘要

一位 40 多岁的女性因视力迅速模糊而就诊于眼科医生。裸眼视力已降至 0.15,尽管她的眼睛没有异常,但怀疑有内部疾病,并发现白蛋白为 2.6 g/dL。由于没有尿蛋白排泄,怀疑肠道有蛋白质渗漏。锝标记白蛋白 D 闪烁扫描显示蛋白质从肠道渗漏。粪便α1-抗胰蛋白酶清除试验显示增加到 56.3 mL/天,导致诊断为蛋白丢失性胃肠病。血液生化检查显示抗 SS-A 和抗 SS-B 抗体水平异常升高(分别≥1200 U/mL 和≥1000 U/mL)。唇唾液腺活检显示淋巴细胞浸润,每 2mm×2mm 至少有 1 个焦点>50 个导管内淋巴细胞)。Schirmer 测试结果为 5mm/5min 或更短,这导致干燥综合征的诊断。静脉注射甲基强的松龙 50mg(1mg/kg)后血清白蛋白水平升高,目前患者正在口服泼尼松龙,剂量逐渐减少。泼尼松龙给药后,视力恢复到 1.2,白蛋白恢复。

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Clin Case Rep. 2024 Apr 16;12(4):e8779. doi: 10.1002/ccr3.8779. eCollection 2024 Apr.
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Protein-Losing Enteropathy.蛋白丢失性肠病
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Protein-Losing Enteropathy in Crohn's Disease: Two Unusual Cases.克罗恩病中的蛋白丢失性肠病:两例罕见病例
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Protein-losing gastroenteropathy with severe hypoalbuminemia associated with Sjögren's syndrome: A case report and review of the literature.与干燥综合征相关的伴有严重低白蛋白血症的蛋白丢失性胃肠病:一例报告并文献复习
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