1.

Patients with impaired pyruvate metabolism, caused by nonfunctional enzymes in the pyruvate
dehydrogenase complex, often show a higher than normal concentration of blood lactate. Explain
why.
Patients with abnormal concentration of blood lactate that cause by pyruvate dehydrogenase
difficiency are called as lactic acidosis.On the other hand, is associated with major metabolic
dysregulation, tissue hypoperfusion, the effects of certain drugs or toxins, and congenital
abnormalities in carbohydrate metabolism. It also occurs as a result on markedly increased
transient metabolic demand (eg, postseizure lactic acidosis). Congenital lactic acidosis is
secondary to inborn errors of metabolism, such as defects in gluconeogenesis, pyruvate
dehydrogenase, the tricarboxylic acid (TCA) cycle, or the respiratory chain. These disorders
generally reflect situations in which the disposal of pyruvate by biosynthetic or oxidative routes is
impaired.
In this case, this is a lactic acidosis type B. Type B is lactic acidosis occurring when no clinical
evidence of poor tissue perfusion or oxygenation exists. However, in many cases of type B lactic
acidosis, occult tissue hypoperfusion is now recognized to accompany the primary etiology. Type
B is divided into 3 subtypes based on underlying etiology which is in this case is a type B3 . It
associate with inborn errors of metabolism (example congenital forms of lactic acidosis with
various enzyme defects example pyruvate dehydrogenase deficiency)