Dong Kai-Hui, Fang Yi-Na, Wen Xiao-Yu, Jin Qing-Long
Department of Hepatology, The First Hospital of Jilin University, Changchun, China.
Medicine (Baltimore). 2018 Nov;97(48):e13172. doi: 10.1097/MD.0000000000013172.
Renal tubular acidosis (RTA) represents a class of metabolic disorders characterized by metabolic acidosis with a normal plasma anion gap. As a rare complication of primary biliary cirrhosis (PBC), RTA is easily overlooked, likely leading to misdiagnosis.
A 32-year-old woman who had been diagnosed with PBC at our hospital was found to have hypokalemia due to repeated fatigue for 2 years, and the etiology was unknown.
Due to the laboratory test results, radiographic findings, and pathologic results, she was diagnosed with PBC associated with RTA.
She was then treated with ursodeoxycholic acid, potassium citrate, and calcium supplements together with activated vitamin D.
Thus far, the patient showed a good response to ursodeoxycholic acid, and the clinical symptoms and liver function were significantly improved.
Physicians that encounter refractory hypokalemia in a patient with PBC should be aware of the presence of RTA. The early diagnosis and treatment of such patients are of paramount importance to alleviate clinical symptoms and delay disease progression.
肾小管酸中毒(RTA)是一类以代谢性酸中毒伴正常血浆阴离子间隙为特征的代谢紊乱疾病。作为原发性胆汁性肝硬化(PBC)的一种罕见并发症,RTA很容易被忽视,可能导致误诊。
一名32岁女性,在我院被诊断为PBC,因反复疲劳2年被发现存在低钾血症,病因不明。
根据实验室检查结果、影像学表现及病理结果,她被诊断为PBC合并RTA。
随后她接受了熊去氧胆酸、枸橼酸钾、钙剂及活性维生素D联合治疗。
迄今为止,患者对熊去氧胆酸反应良好,临床症状及肝功能显著改善。
遇到PBC患者出现难治性低钾血症的医生应意识到RTA的存在。对此类患者进行早期诊断和治疗对于缓解临床症状及延缓疾病进展至关重要。