以肾小管酸中毒、肾结石、肾源性尿崩症为表现的原发性胆汁性肝硬化伴肾小管间质性肾炎1例

Primary Biliary Cirrhosis with Tubulointerstitial Nephritis Manifested as Renal Tubular Acidosis, Nephrolithiasis, and Nephrogenic Diabetes Insipidus: A Case Report

摘要: 原发性胆汁性肝硬化(PBC)的患者有时会发生肾小管间质性肾炎(TIN)，但很容易被忽视。本研究回顾性报道1位55岁的女性因持续加重口干、多饮和多尿而住院，她住院前3年被诊断为PBC。本次住院期间，被诊断为肾小管酸中毒、肾结石、肾积水、尿崩症和慢性肾病，考虑PBC相关的肾损伤。患者服用熊去氧胆酸、氢氯噻嗪、吲哚美辛和枸橼酸钾治疗后症状明显改善。由此来提高人们对肾损伤的PBC的认识。该病例报道已获得病人的知情同意。

Abstract: Tubulointerstitial nephritis (TIN) is sometimes found in patients with Primary biliary cirrhosis (PBC), but it can easily be overlooked. In our manuscript, a 55-year-old woman was hospitalized with complaints of thirst, polydipsia, and polyuria. She had been diagnosed with PBC 3 years prior. During this hospitalization, she was diagnosed with renal tubular acidosis, nephrolithiasis, hydronephrosis, diabetes insipidus, and chronic kidney disease. Kidney damage associated with PBC should be considered. The patient showed a good response to ursodeoxycholic acid, hydrochlorothiazide, indomethacin, and potassium citrate. We report a case of PBC with severe renal impairment to raise awareness of PBC with kidney involvement. The case report has obtained the informed consent of the patient.