BACKGROUND Primary distal renal tubular acidosis is a clinical disorder characterized by hyperchloremic metabolic acidosis, hypercalciuria, hypocitraturia, urinary acidification impairment, hypokalemia, metabolic bone disease, and nephrocalcinosis. Urinary acidification ability may be evaluated by an acidification test or maximum urinary pCO2 assessment with alkaline urine. The maximum urinary ...

1. Delineate features of a medical history that should raise suspicion for an inborn error of metabolism. 2. Describe common ocular findings associated with inborn errors of metabolism. 3. List the primary clinical findings of inborn errors of metabolism associated with encephalopathy without metabolic acidosis. 4. Delineate the categories of inborn errors of metabolism associated with encephal...

[11] , endothelin [10] , and aldosterone [10] . Together, these studies suggest that patients with reduced GFR but no metabolic acidosis nevertheless have acid retention that might mediate nephropathy progression. Reducing acid retention with less acid-producing diets that lower net endogenous acid production (NEAP) might slow nephropathy progression in patients with reduced GFR even without me...

M etabolic acidosis is a common acid-base disturbance in hospitalized patients. Distinguishing anion gap from non–anion gap metabolic acidosis is a very helpful exercise and enables the clinician to narrow the etiology of the particular acidosis. The use of the anion gap was popularized in an article by Emmett and Narins (1). Over the past few years, the list of conditions that can cause an ani...

The primary mechanism by which the kidneys mediate net acid excretion is through ammonia metabolism. In the current study, we examined whether chronic metabolic acidosis, which increases ammonia metabolism, alters the cell-specific and/or the subcellular expression of the ammonia transporter family member, Rhcg, in the outer medullary collecting duct in the inner stripe (OMCDi). Chronic metabol...