Liver transplantation in glycogen storage disease type I.

Glycogen storage disease type I (GSDI), an inborn error of carbohydrate metabolism, is caused by defects in the glucose-6-transporter/glucose-6-phosphatase complex, which is essential in glucose homeostasis. Two types exist, GSDIa and GSDIb, each caused by different defects in the complex. GSDIa is characterized by fasting intolerance and subsequent metabolic derangements.

A different approach to breast-feeding of the infant with phenylketonuria.

Unlabelled: We studied the possibility and safety of a new approach to breast-feeding infants with phenylketonuria (PKU). We compared a group of PKU infants being breast-fed according to our new protocol with a group of PKU infants receiving formula only. The breast-fed group consisted of nine infants born between 1994 and 1999 being breast-fed at the time of diagnosis.