@entity8 in XXXX .

@entity1 @entity4388 @entity8 @entity3884 @entity1483 @entity2397 @entity1372 @entity4418 @entity520 @entity1391 @entity701 @entity5819 @entity972 @entity916 @entity7842 @entity2753 @entity4144 @entity774
Functional indices of distal urinary acidification were assessed in two male @entity1 , aged 1 and 3 months, with @entity774 . In both cases the diagnosis was sustained by the presence of elevated plasma levels of @entity4418 , @entity2753 , @entity4144 , @entity520 and increased plasma @entity916 activity. Both @entity1 were unable to lower urinary pH below 5.9 either during acute @entity5819 -induced @entity520 or after i.v. administration of @entity1483 . One @entity1 also failed to decrease urine pH below 5.5 and to increase urinary @entity972 excretion during @entity2397 infusion. Oral @entity4388 loading was given to both @entity1 but failed to induce a significant increase in the urine minus blood PCO2 gradient. This gradient remained low also after neutral @entity1391 administration. Repeated studies after acute administration of @entity7842 in one case or after prolonged administration of @entity701 in the other resulted in complete normalization of all functional studies. We conclude that @entity774 can lead to @entity8 in early infancy. The defective renal secretion of @entity3884 ion and @entity972 is probably related to the abolishment of the negative potential difference in the cortical collecting tubule induced by the impaired reabsorption of @entity1372 .
