Fanconi syndrome secondary to primary biliary cholangitis: a case report
10.3760/cma.j.cn441217-20220929-00943
- VernacularTitle:原发性胆汁性胆管炎继发范科尼综合征1例
- Author:
Fei TENG
1
;
Wei YE
;
Yubing WEN
;
Xuemei LI
;
Ke ZHENG
Author Information
1. 中国医学科学院北京协和医院内科，北京 100730
- Keywords:
Liver cirrhosis, biliary;
Nephritis, interstitial;
Fanconi syndrome;
Renal tubular acidosis
- From:
Chinese Journal of Nephrology
2023;39(6):468-470
- CountryChina
- Language:Chinese
-
Abstract:
Primary biliary cirrhosis/cholangitis is an autoimmune disease. Renal tubular acidosis is a common form in PBC cases, but Fanconi syndrome is rarely reported. The paper reported a 66-year-old female patient with fatigue, renal insufficiency and elevated bile duct enzymes. The patient presented with type 2 proximal renal tubular acidosis and complete Fanconi syndrome. Laboratory examinations showed high-titer-positive anti-mitochondrial antibodies, elevated serum IgM, and type 3 cryoglobulinemia. Renal biopsy revealed interstitial nephritis, and electron micrographs showed abnormal mitochondria in proximal tubular epithelial cells. The patient's renal function ameliorated, and acid-base imbalance and electrolyte disturbances were corrected after high-dose glucocorticoid treatment.
