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A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
- Source :
- Clinical Kidney Journal
- Publication Year :
- 2019
-
Abstract
- Autoimmune disorders such as rheumatoid arthritis or Sjögren’s syndrome can be associated with impaired renal acid excretion. Only few cases of patients with primary biliary cirrhosis (PBC) and distal renal tubular acidosis (dRTA) have been described. Here, we present the case of a 60-year-old woman with PBC and dRTA. Her kidney biopsy showed an absence of markers of acid-secretory Type A intercalated cells (A-ICs) and expression of aquaporin-2, a marker of principal cells, in all cells lining the collecting duct. Moreover, the serum of the patient contained antibodies directed against a subset of cells of the collecting duct. Thus, PBC-related autoantibodies may target acid-secretory A-ICs and thereby impair urinary acidification.
- Subjects :
- medicine.medical_specialty
2747 Transplantation
autoantibodies
030232 urology & nephrology
610 Medicine & health
Exceptional Cases
Gastroenterology
10052 Institute of Physiology
Renal tubular acidosis
03 medical and health sciences
0302 clinical medicine
Primary biliary cirrhosis
Distal renal tubular acidosis
Internal medicine
Biopsy
medicine
10035 Clinic for Nephrology
Transplantation
Kidney
2727 Nephrology
medicine.diagnostic_test
business.industry
dRTA
Metabolic acidosis
medicine.disease
primary biliary cirrhosis
medicine.anatomical_structure
Nephrology
intercalated cells
570 Life sciences
biology
Renal biopsy
acidosis
business
Kidney disease
Subjects
Details
- ISSN :
- 20488505
- Volume :
- 13
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Clinical kidney journal
- Accession number :
- edsair.doi.dedup.....e62d0d2b80251497afd5e7d70e2e259b