Proximal renal tubular acidosis with primary Fanconi syndrome

Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome

Primary Sjögren's syndrome (pSS) is characterized by lymphocytic infiltration of the exocrine glands resulting in decreased saliva and tear production. It uncommonly involves the kidneys in various forms, including tubulointerstitial nephritis, renal tubular acidosis, Fanconi syndrome, and rarely glomerulonephritis. Its clinical symptoms include muscle weakness, periodic paralysis, and bone pai...

Prevalence of distal renal tubular acidosis in primary Sjögren's syndrome.

OBJECTIVES Our objectives were to analyse the prevalence of distal renal tubular acidosis (dRTA) in primary SS (pSS) and to compare a novel urinary acidification test with furosemide and fludrocortisone (FF) with the gold standard ammonium chloride (NH4Cl) to detect dRTA. METHODS Urinary acidification was assessed in 57 pSS patients using NH4Cl and FF. A urinary acidification defect was defin...

Renal Tubular Acidosis Type 2 and 3: Proximal Renal Tubular Acidosis (pRTA) and Combined Proximal and Distal RTA in Children

Renal tubular acidosis (RTA) type 2, also called proximal renal tubular acidosis, is characterized by hyperchloremic metabolic acidosis due to impaired reabsorption of bicarbonate (HCO3) in proximal tubules. It can be due to isolated defect or part of generalized defect (Fanconi syndrome). Rickets/Osteomalacia is more common and treatment is usually difficult. RTA type 3 is designated when comb...

(pink Disease and Primary Renal Tubular Acidosis.)

Unraveling the molecular pathogenesis of isolated proximal renal tubular acidosis.

Proximal renal tubular acidosis (pRTA) results from an impairment of bicarbonate (HCO(3)(-)) reabsorption in the renal proximal tubules and is characterized by a decreased renal HCO(3)(-) threshold. Proximal RTA most commonly occurs in association with multiple defects of proximal tubular transport (renal Fanconi syndrome). Although much more rare, pRTA may occur without other functional defect...