Barnaby Clark

To the Editor, Neonatal liver failure, defined as acute liver failure in the first 28 days of life, is a rare condition with a high mortality rate. The most common causes include gestational alloimmune liver disease (GALD), and the overlapping neonatal haemochromatosis (GALD-NH), viral infections, metabolic diseases, and primary hemophagocyic lymphohistiocytosis [1]. When the cause of liver failure is not easily identifiable, management can be far more challenging.

Abstract Background Erythrocyte pyruvate kinase is expressed under the control of the PKLR gene located on chromosome 1q21. Pyruvate kinase catalyzes the final steps of the glycolytic pathway and creates 50% of the red cell total adenosine triphosphate. Pyruvate kinase deficiency is the commonest glycolytic defect causing congenital non-spherocytic hemolytic anemia inherited in an autosomal recessive trait in which homozygotes and compound heterozygotes are common.