Topic: Pathophysiology of Renal Tubular Acidosis: Core Curriculum 2016 pRTA Dx and Tx

Topic: Pathophysiology of Renal Tubular Acidosis: Core Curriculum 2016            pRTA Dx and Tx
整理	內容 defect HCO3 reabsorption in the proximal tubule Apical NHE3 or H-ATPase defect cytolic CA II defect Basolateral NBCE1 defect pRTA could be a component of Fanconi’s syndrome Hereditary: mutatiojn at CA II => p+d RTA (type III RTA), prominent at distal acidification defect Familial Fanconi’s synd.: cystinosis and Wilson’s dz Lowe syndr. (OCRL) Acquired: tubular injury => light chain, amyloidosis, MM, autoimmune disorder, toxin, etc. Clinical manifestation: milder Hyer-Cl +/- bicarbonturia (depends on the filtrate’s bicarbonate amount exceed or not the distal tubule reabsorption maximum; -, when serum HCO3 14~18) => Hyper-Cl + Hypo-K + variable urine pH NH4Cl test (0.1g/kg) or Furosemide test (high false positive and false negative rate)                Tx w/ HCO3 (1~2 mEq/kg/d) 2~3 wks => recheck serum HCO3 => pRTA: serum HCO3 still significantly below normal + Alkaline urine pH                         => c.f. dRTA: nearly normal reference of serum HCO3 Tx: PO HOC3 (10-15 mEq/kg/d) + K supply Hydrochlorothiazide + K supply (可能低) If Fanconi’s synd. => 要補phosphate
摘要