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[伴有突出胃肠道症状的播散性结节病罕见病例]

[Unusual case of disseminated sarcoidosis with prominent gastrointestinal symptoms].

作者信息

Klebl F H, Merger M, Hierlmeier F X, Büttner R, Palitzsch K D

机构信息

Klinik und Poliklinik für Innere Medizin I, Universität Regensburg.

出版信息

Dtsch Med Wochenschr. 1999 Jan 22;124(3):39-44. doi: 10.1055/s-2007-1024240.

DOI:10.1055/s-2007-1024240
PMID:9987484
Abstract

HISTORY AND ADMISSION FINDINGS

A 63-year-old man had for 10 months suffered from marked weight loss, night sweats, diffuse abdominal pain and increased stool frequency. He was admitted to evaluate an ultrasonically abnormal focus in the liver parenchyma and elevated liver function parameters. His sclerae were obviously icteric and he looked under-weight.

INVESTIGATIONS

He had a hypochromic microcytic anemia and abnormal liver and pancreatic function tests: total bilirubin 3.11 mg/dl, direct bilirubin 2.21 mg/dl, GOT21U/l, gamma-GT 422 U/l, alkaline phosphatase 1449 U/l, alpha-amylase 481 U/l, lipase 2827 U/l. The serum creatinine level was elevated to 1.47 mg/dl. Computed tomography revealed enlarged liver and spleen as well as an enlargement of intraabdominal lymph nodes, chest radiogram and endoscopic cholangio-pancreatography were unremarkable. Biopsies from the lower duodenum, large intestine, bone marrow and liver showed inflammatory changes with Langhans-type mononuclear granulomas. Together with these findings an increased activity of the angiotensin-converting-enzyme (ACE) indicated sarcoidosis, other causes having been excluded.

TREATMENT AND COURSE

All signs and symptoms rapidly improved under prednisolone, and 4 weeks after begin of treatment the biochemical abnormalities had clearly regressed. The raised serum levels of soluble IL-2 receptors and of neopterin, measures of sarcoidosis activity, had decreased. Activity of ACE had fallen.

CONCLUSION

Sarcoidosis can present with diverse clinical signs and symptoms. In a case of multi-system disease that cannot be readily classified, sarcoidosis should be included in the differential diagnosis.

摘要

病史及入院检查结果

一名63岁男性,10个月来体重显著减轻、盗汗、全腹疼痛且排便次数增多。因肝脏实质超声检查发现异常病灶及肝功能参数升高入院。他巩膜明显黄染,看起来体重不足。

检查

患有低色素小细胞性贫血,肝功能和胰腺功能检查异常:总胆红素3.11mg/dl,直接胆红素2.21mg/dl,谷草转氨酶21U/l,γ-谷氨酰转肽酶422U/l,碱性磷酸酶1449U/l,α-淀粉酶481U/l,脂肪酶2827U/l。血清肌酐水平升至1.47mg/dl。计算机断层扫描显示肝脏和脾脏肿大以及腹腔内淋巴结肿大,胸部X线片和内镜逆行胰胆管造影未见异常。十二指肠下段、大肠、骨髓和肝脏活检显示有炎症改变,并伴有朗汉斯型单核肉芽肿。结合这些发现,血管紧张素转换酶(ACE)活性升高提示为结节病,已排除其他病因。

治疗及病程

在泼尼松龙治疗下,所有体征和症状迅速改善,治疗开始4周后生化异常明显消退。作为结节病活动指标的血清可溶性白细胞介素-2受体和新蝶呤水平升高有所下降。ACE活性降低。

结论

结节病可表现出多种临床体征和症状。对于难以明确分类的多系统疾病病例,鉴别诊断应考虑结节病。

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