Exercises in AcidBase Disorders在酸碱紊乱练习.ppt

* * * * * * * * * * * * * Gynecol Obstet Invest 2007;63:39–44 * Gynecol Obstet Invest 2007;63:39–44 * * * * * * * Both sulfate and furosemide enhance H+ and K+ secretion in the cortical collecting tubule by increasing distal Na+ delivery and generating a high luminal electronegativity in the distal nephron thus permitting the simultaneous evaluation of distal H+ and K+ secretion capacities. * * * * * * Case 1: Proximal RTA Minimum Urine pH 5.5 Plasma potassium? Low-normal Renal stones/NC No Prox. Tubular dysfunction Glycosuria, Phosphate, AA, Urate FEHCO3 15-20% Daily bicarbonate replacement needs 4 mmol/kg FEHCO3 Intravenous infusion of sodium bicarbonate at a rate of 0.5 to 1.0 meq/kg per hour ???????????????????? UHCO3?? x?? PCr ?FEHCO3????=????———————————????x????100 ???????????????????????????? PHCO3?? x?? UCr Proximal RTA: FE HCO315-20% Clinical Features of Proximal RTA Urine pH depends on plasma [HCO3-] Fractional HCO3- excretion high (15-20%) at nl plasma [HCO3-] Plasma [K+] reduced, worsens with HCO3- therapy Dose of daily HCO3- required: 10-15 mEq/kg/d Non-renal: rickets or osteomalacia Causes of Proximal RTA Primary isolated proximal RTA hereditary (persistent)?????? a. autosomal dominant?????? b. autosomal recessive associated with mental retardation and ocular abnormalities???? Sporadic (transient in infancy) Secondary proximal RTA??? in the context of Fanconi syndrome (cystinosis, galactosemia, fructose intolerance, tyrosinemia, Wilson disease, Lowe syndrome, metachromatic leukodystrophy, multiple myeloma, light chain disease)????? drugs and toxins (acetazolamide, outdated tetracycline, aminoglycoside antibiotics, valproate, 6-mercaptopurine, streptozotocin, iphosphamide, lead, cadmium, mercury)????? other clinical entities (vitamin D deficiency, hyperparathyroidism, chronic hypocapnia, Leigh syndrome, cyanotic congenital heart disease, medullary cystic disease, Alport syndrome, corticoresistant nephrotic syndrome, renal transplantation,