. Author manuscript; available in PMC: 2022 Mar 2.

Published in final edited form as: J Inherit Metab Dis. 2013 Jun 20;37(1):39–42. doi: 10.1007/s10545-013-9627-x

Table 2.

Frequency of offspring with congenital heart defects (CHD) in maternal phenylketonuria (MPKU) pregnancies: classified by blood phenylalanine (Phe) and protein intake. Number and percent of MPKU pregnancies with CHD in offspring classified by mean blood Phe during 4–8 weeks of gestation and mean first-trimester protein intake compared by Fisher’s exact test within blood Phe strata. Higher protein intake with lower Phe intake indicates use of PKU formula

Blood Phe	Total protein intake (natural+medical food)	Number	Phe intake^* (mg)	CHD	Percent	P value
Low (≤910 μmol/L)	High (>50 g)	134	543(401–845)	1	0.8%	0.006
	Low (≤ 50 g)	33	662(465–963)	4	12.1%
High (>910 μmol/L)	High (>50 g)	41	523(392–615)	3	7.3%	0.02
	Low (≤ 50 g)	10	1044(541–1333)	4	40.0%

CHD not including patent ductus arteriosus, IQR interquartile range

median (IQR)