Brenner and Rector's The Kidney, 8th ed.[]
- Section II - Integrated Control of Body Fluid Volume and Composition
- Chapter 14 - Disorders of Acid-Base Balance
- Metabolic Disorders
- Metabolic Acidosis
- Hyperchloremic (Normal Anion Gap) Metabolic Acidoses
- Disorders of Impaired Renal Bicarbonate Reclamation: Proximal Renal Tubular Acidosis
- Disorders of Impaired Net Acid Excretion with Hypokalemia: Classic Distal Renal Tubule Acidosis
- Disorders of Impaired Net Acid Excretion with Hyperkalemia: Generalized Distal Nephron Dysfunction (Type 4 Renal Tubular Acidosis)
- Hyperchloremic (Normal Anion Gap) Metabolic Acidoses
- Metabolic Acidosis
- Metabolic Disorders
- Chapter 14 - Disorders of Acid-Base Balance
Pathophysiology
- 遠端高血鉀性是CCT有問題或缺aldosterone
- 遠端低血鉀性則是下列異常（在type A intercalated cell in medullary collecting duct），共同特色為無法將尿液酸至pH<5.5：
- Basolateral HCO3-/Cl- exchanger
- Subunit of H+-ATPase的問題
- Abnormal leak pathway (Amphotericin B)
- H+,K+-ATPase
- Defective intracellular carbonic anhydrase (type 2)
- 正常人給大量HCO3-，會使distal H+ secretion造成尿液高的PCO2（U-B PCO2反映遠端酸化之能力）。
- 除了leak（amphotericin B，此為"gradient" defect）外，distal classic RTA皆有low U-B PCO2 gradient。
Original Articles[]
- Carboni I, Andreucci E, and Caruso MR et al. Medullary sponge kidney associated with primary distal renal tubular acidosis and mutations of the H+-ATPase genes. Nephrol Dial Transplant. 2009 Sep;24(9):2734-8. Epub 2009 Apr 13.