Rathi Karishma M, Pingat Priyanka, Bansode Prachi, Dongare Shaili
Department of Pharmacy Practice at Dr. D.Y. Patil Institute of Pharmaceutical Sciences and Research Pimpri, Pune, India.
Middle East J Dig Dis. 2023 Oct;15(4):289-292. doi: 10.34172/mejdd.2023.360. Epub 2023 Oct 30.
A rare congenital hepatobiliary disorder called Caroli's disease is characterized by multifocal segmental dilatation of intrahepatic bile ducts that can affect the entire liver or only specific areas of it. Coexisting conditions with Caroli's disease include autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD results in the development of cysts, which are tiny fluid-filled sacs, in the kidneys. Caroli's disease is considered a rare disorder, affecting a small number of individuals worldwide. The symptoms of Caroli's disease can vary from person to person and it also may overlap with other liver and biliary disorders. As a result, it may be challenging to diagnose and manage the condition due to limited awareness and expertise. Increased awareness, research, and specialized medical care are crucial in improving outcomes for individuals affected by this rare disorder. This study involves the case of a 60- year-old woman presented with abdominal pain, fever, weight loss, and jaundice. Her imaging test endoscopic retrograde cholangiopancreatography (ERCP) signifies Caroli's disease with pancreatic duct (PD) calculi and management involves supportive care with antibiotics. Antibiotics were prescribed to prevent or treat infections such as cholangitis and nutritional supplement was recommended in managing Caroli's disease. The patient underwent pancreatic stent placement and was discharged with regular follow-up. So, this case highlights the clinical and diagnostic aspects to improve disease understanding and the progression of Caroli's illness along with ADPKD.
一种名为卡罗利病的罕见先天性肝胆疾病，其特征是肝内胆管多灶性节段性扩张，可累及整个肝脏或仅累及肝脏的特定区域。卡罗利病的并存疾病包括常染色体显性遗传性多囊肾病（ADPKD）和常染色体隐性遗传性多囊肾病（ARPKD）。ADPKD会导致肾脏中形成囊肿，即充满液体的微小囊袋。卡罗利病被认为是一种罕见疾病，在全球影响的人数较少。卡罗利病的症状因人而异，也可能与其他肝脏和胆道疾病重叠。因此，由于认识和专业知识有限，诊断和管理这种疾病可能具有挑战性。提高认识、开展研究和提供专门的医疗护理对于改善受这种罕见疾病影响的个体的治疗效果至关重要。本研究涉及一名60岁女性，她出现腹痛、发热、体重减轻和黄疸症状。她的成像检查——内镜逆行胰胆管造影（ERCP）显示患有卡罗利病并伴有胰管结石，治疗包括使用抗生素进行支持性护理。使用抗生素预防或治疗诸如胆管炎等感染，并建议在管理卡罗利病时补充营养。该患者接受了胰管支架置入术，并在定期随访后出院。所以，这个病例突出了临床和诊断方面的问题，以增进对疾病的了解以及卡罗利病与ADPKD的病情进展。
