An experimental renal acidification defect in patients with hereditary fructose intolerance: II. Its distinction from classic renal tubular acidosis; its resemblance to the renal acidification defect associated with the fanconi syndrome of children with cystinosis
AUTOR(ES)
Morris, R. Curtis
RESUMO
In adult patients with hereditary fructose intolerance (HFI) fructose induces a renal acidification defect characterized by (a) a 20-30% reduction in tubular reabsorption of bicarbonate (T HCO3-) at plasma bicarbonate concentrations ranging from 21-31 mEq/liter, (b) a maximal tubular reabsorption of bicarbonate (Tm HCO3-) of approximately 1.9 mEq/100 ml of glomerular filtrate, (c) disappearance of bicarbonaturia at plasma bicarbonate concentrations less than 15 mEq/liter, and (d) during moderately severe degrees of acidosis, a sustained capacity to maintain urinary pH at normal minima and to excrete acid at normal rates. In physiologic distinction from this defect, the renal acidification defect of patients with classic renal tubular acidosis is characterized by (a) just less than complete tubular reabsorption of bicarbonate at plasma bicarbonate concentrations of 26 mEq/liter or less, (b) a normal Tm HCO3- of approximately 2.8 mEq/100 ml of glomerular filtrate, and (c) during acidosis of an even severe degree, a quantitatively trivial bicarbonaturia, as well as (d) a urinary pH of greater than 6.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=297322Documentos Relacionados
- An experimental renal acidification defect in patients with hereditary fructose intolerance: I. Its resemblance to renal tubular acidosis
- Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.
- On the mechanism of renal potassium wasting in renal tubular acidosis associated with the Fanconi syndrome (type 2 RTA)
- Renal Tubular Acidosis in Infants: the Several Kinds, Including Bicarbonate-Wasting, Classic Renal Tubular Acidosis
- Attainment and Maintenance of Normal Stature with Alkali Therapy in Infants and Children with Classic Renal Tubular Acidosis