PDF(Pubmed)
Abstract：
BACKGROUND If a young patient presents with fever, abdominal pain, jaundice and significant imaging abnormalities, especially dilation of the biliary system, it is usually due to obstruction from stones or strictures. However, on very rare occasions, it can be due to complications of congenital cystic dilatation of the biliary system, known as Caroli disease. We present such a patient and discuss the differential diagnosis and implications for long-term management. CASE REPORT A 14-year-old boy presented to the Emergency Department with a sudden onset of high-grade fever and abdominal pain for 2 weeks, accompanied by vomiting of blood. The patient had no relevant medical history. He was malnourished and had moderate pallor, jaundice, and right upper quadrant pain. Imaging revealed cystic dilatation of intrahepatic ducts and a central dot sign. There were no features suggesting advanced liver disease otherwise, and no tumors or cysts in the kidneys. A diagnosis of Caroli disease was made. The symptoms were ascribed to acute cholangitis and improved with antibiotics. He was discharged home 1 week later. No further blood loss was observed. CONCLUSIONS This case study describes a patient with ascending cholangitis, a complication of Caroli disease. This diagnosis should be considered in the differential diagnosis when a child or young adult presents with features of cholangitis, abnormal biliary imaging, and/or upper gastrointestinal bleeding, or portal hypertension. No prior cases of this disease have been encountered, documented, or published in Kenya. This case can increase awareness among primary care clinicians, including pediatricians.
摘要：
背景技术如果年轻患者出现发烧,腹痛,黄疸和明显的影像学异常，尤其是胆道系统的扩张，它通常是由于石头或狭窄的阻碍。然而,在非常罕见的情况下，这可能是由于先天性胆道系统囊性扩张的并发症，被称为Caroli病.我们介绍了这样的患者，并讨论了鉴别诊断和对长期管理的影响。个案报告一名14岁男童因高烧及腹痛突然发作2周,前往急诊科就诊,伴随着血液的呕吐。患者无相关病史。他营养不良，脸色苍白，黄疸,右上腹疼痛。影像学显示肝内导管囊性扩张和中央点征象。没有其他特征表明晚期肝病，肾脏没有肿瘤或囊肿.做出了Caroli病的诊断。症状归因于急性胆管炎，并用抗生素改善。一周后他出院回家。没有观察到进一步的失血。结论本案例研究描述了一名上行性胆管炎患者，Caroli病的并发症.当儿童或年轻人表现出胆管炎的特征时，应在鉴别诊断中考虑该诊断。胆道成像异常,和/或上消化道出血，或者门静脉高压症。以前没有遇到过这种疾病的病例，记录在案,或在肯尼亚出版。这种情况可以提高初级保健临床医生的认识，包括儿科医生.
