Metabolic acidosis definition

Tubular acidosis is primary (an isolated inherited metabolic disorder) or secondary (a metabolic disorder associated with inherited or acquired lesions). It is characterized by the inability to maintain normal blood pH as a result of interference with bicarbonate reabsorption or a defect in renal hydrogen excretion. By definition, glomerular filtration is not altered in renal tubular acidosis. 

The disorders of isoleucine and valine metabolism are detected in a sequential process that begins with the evaluation of the symptoms and signs displayed by the patient. Clinical chemistry is helpful in the assessment of ketogenesis by urinary tests for ketones or quantification of 3-hydroxybuty-rate and acetoacetate in the blood. The electrolytes and pH may provide evidence of acidosis and it is important to assess the presence or absence of hyperammonemia. Amino acid analysis of the plasma and urine may be helpful. In virtually all instances the definitive diagnosis will come from organic acid analysis of the urine. 

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