2014-18 Proximal RTA: hypokalemia Causes of proximal rta Increased distal Na+ delivery(NaHCO3) Inherited pRTA: NBCe1/SLC4A4)mutation Increased aldosterone levels(dehydration accompanied by ocular abnormalities such as because of loss of Na in the urine) cataracts, glaucoma. Treatment of acidosis with HCO3 improves the Carbonic anhydrase inhibitor: acetazolamide acidosis but worsens the degree of Fanconi syndrome: inherited and acquired Adult with Fanconi: dysproteinemic condition drTa(type 1) Systemic acidosis in drTa tends to be more severe han in patients with a proximal RTA (serum HCO3- can reach as low as 10 mmol/l vs 16 to 18 mmol/l) Hypokelemia can also be severe: musculoskeletal Nephrolithiasis and nephrocalcinosis dRTA: kidney stone dRTA Urinary calcium excretion is high Primary: idiopathic or inherited(SLC4A1 Acidosis induced bone mineral dissolution mutation) Low intraluminal concentration of Hco3- because Urinary citrate levels are low- citrate serve High urine ph decrease the solubility of m phosphate complexes.2014-1-8 6 Proximal RTA: hypokalemia • Increased distal Na+ delivery (NaHCO3) • Increased aldosterone levels (dehydration because of loss of Na in the urine). • Treatment of acidosis with HCO3 improves the acidosis but worsens the degree of hypokalemia. Causes of Proximal RTA • Inherited pRTA: NBCe1/SLC4A4) mutation, accompanied by ocular abnormalities such as cataracts, glaucoma. • Carbonic anhydrase inhibitor: acetazolamide • Fanconi syndrome: inherited and acquired • Adult with Fanconi: dysproteinemic condition such as multiple myeloma dRTA (type 1) • Systemic acidosis in dRTA tends to be more severe than in patients with a proximal RTA (serum HCO3- can reach as low as 10 mmol/l vs 16 to 18 mmol/l) • Hypokelemia can also be severe: musculoskeletal weakness • Nephrolithiasis and nephrocalcinosis HCO3 HCO3 HCO3 dRTA: kidney stone • Urinary calcium excretion is high – Acidosis induced bone mineral dissolution – Low intraluminal concentration of HCO3- because of acidosis • Urinary citrate levels are low – citrate serve as the major Ca++ chelator in the urine • High urine pH decrease the solubility of calcium phosphate complexes. dRTA • Primary: idiopathic or inherited (SLC4A1 mutation) • Systemic disease: Sjogren syndrome