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Pregnancy and lactation outcomes in a Turkish patient with lysinuric protein intolerance

Ozlem Unal1, Turgay Coşkun, Diclehan Orhan

  • 1Division of Metabolism, Department of Pediatrics, Hacettepe University, İhsan Doğramacı Children's Hospital, Ankara, Turkey, unalozlem@gmail.com.

JIMD Reports
|October 22, 2013
PubMed

Maternal lysinuric protein intolerance (LPI) is associated with increased risk of anemia, toxemia, and retarded growth in fetus during pregnancy, and bleeding complications during delivery. There has been limited number of reports about pregnancy and outcomes of lactation in LPI. Here we present pregnancy and lactation outcomes in a Turkish patient with LPI. In the pregnancy and delivery period, her metabolic status was stable with protein-restricted diet and citrulline. Pathological examination of the placenta revealed multifocal placental infarcts. A successful outcome was achieved with well-controlled anemia, thrombocytopenia despite hemophagocytosis in bone marrow, and placental infarcts during pregnancy. The baby was exclusively breastfed for 6 months. His growth and development was normal. Mild proteinuria started at the fourth month of the delivery. Our case report showed the importance of follow-up of these patients in terms of placental pathologies during pregnancy and for other complications during lactation period.

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