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A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis.

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.

SUBMITTER: Elitok S

PROVIDER: S-EPMC7367120 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis.

Elitok Saban S Sidler Marius M Bieringer Markus M Mohebbi Nilufar N Schneider Wolfgang W Wagner Carsten A CA

Clinical kidney journal 20190621 3

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 t ...[more]

PMID: 32699627

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Project description:BackgroundCorrection of metabolic acidosis (MA) with nutritional therapy or bicarbonate administration is widely used in chronic kidney disease (CKD) patients. However, it is unknown whether these interventions reduce insulin resistance (IR) in diabetic patients with CKD. We sought to evaluate the effect of MA correction on endogenous insulin action in diabetic type 2 (DM2) CKD patients.MethodsA total of 145 CKD subjects (83 men e 62 women) with DM2 treated with oral antidiabetic drugs were included in the study and followed up to 1 year. All patients were randomly assigned 1:1 to either open-label (A) oral bicarbonate to achieve serum bicarbonate levels of 24-28 mmol/L (treatment group) or (B) no treatment (control group). The Homeostatic model assessment (HOMA) index was used to evaluate IR at study inception and conclusion. Parametric and non-parametric tests as well as linear regression were used.ResultsAt baseline no differences in demographic and clinical characteristics between the two groups was observed. Average dose of bicarbonate in the treatment group was 0.7 ± 0.2 mmol/kg. Treated patients showed a better metabolic control as confirmed by lower insulin levels (13.4 ± 5.2 vs 19.9 ± 6.3; for treated and control subjects respectively; p < 0.001), Homa-IR (5.9[5.0-7.0] vs 6.3[5.3-8.2]; p = 0.01) and need for oral antidiabetic drugs. The serum bicarbonate and HOMA-IR relationship was non-linear and the largest HOMA-IR reduction was noted for serum bicarbonate levels between 24 and 28 mmol/l. Adjustment for confounders, suggests that serum bicarbonate rather than treatment drives the effect on HOMA-IR.ConclusionsSerum bicarbonate is related to IR and the largest HOMA-IR reduction is noted for serum bicarbonate between 24 and 28 mmol/l. Treatment with bicarbonate influences IR. However, changes in serum bicarbonate explains the effect of treatment on HOMA index. Future efforts are required to validate these results in diabetic and non-diabetic CKD patients.Trial registrationThe trial was registered at www.clinicaltrial.gov (Use of Bicarbonate in Chronic Renal Insufficiency (UBI) study - NCT01640119 ).

Project description:Primary biliary cirrhosis (PBC) is an immune-mediated chronic cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis and cirrhosis of the liver and may need liver transplantation in the late stage of disease. PBC primarily affects women (female preponderance 9-10:1) with a prevalence of up to 1 in 1,000 women over 40 years of age. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The diagnosis is based on sustained elevation of serum markers of cholestasis, i.e., alkaline phosphatase and gamma-glutamyl transferase, and the presence of serum antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex. Histologically, PBC is characterized by florid bile duct lesions with damage to biliary epithelial cells, an often dense portal inflammatory infiltrate and progressive loss of small intrahepatic bile ducts. Although the insight into pathogenetic aspects of PBC has grown enormously during the recent decade and numerous genetic, environmental, and infectious factors have been disclosed which may contribute to the development of PBC, the precise pathogenesis remains enigmatic. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at adequate doses of 13-15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. The mode of action of UDCA is still under discussion, but stimulation of impaired hepatocellular and cholangiocellular secretion, detoxification of bile, and antiapoptotic effects may represent key mechanisms. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarizes current knowledge on the clinical, diagnostic, pathogenetic, and therapeutic aspects of PBC.

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A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis.

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A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis.

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