A Case of Maternal Phenyletonuria.
- Author:
Hyun Kyung HONG
;
Woo Ryoung LEE
;
Dong Whan LEE
- Publication Type:Case Report
- MeSH:
Adult;
Diet;
Ductus Arteriosus, Patent;
Female;
Fetal Development;
Fetal Growth Retardation;
Fetus;
Heart Septal Defects, Ventricular;
Humans;
Infant, Low Birth Weight;
Infant, Newborn;
Intellectual Disability;
Microcephaly;
Moths;
Phenylalanine;
Pregnancy;
Pregnant Women
- From:Korean Journal of Perinatology
1999;10(3):387-390
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Maternal phenylketonuria(PKU) is a disorder which appears when a pregnant woman with PKU doesn't take low phenylalanine diet, phenylalanine level of the fetus rises and interferes with fetal development and can easily result in abortion, low birth weight, microcephaly, cardiac anomaly or mental retardation. To prevent these effects of untreated maternal PKU, low phenylalanine diet should be taken to maintain the serum phenylalanine level less than 10mg/dl throughout the pregnancy. We experienced a case of maternal PKU in a female baby born to a 26-year-old woman with PKU who didn't take low phenylalanine diet until eighth moth of pregnancy. She showed intrauterine growth retardation, ventricular septal defect and patent ductus arteriosus. We report a case of maternal PKU with a brief review.