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Purpose: To examine the competing risks of stillbirth versus infant death and to evaluate the optimal time of delivery in the population of small for gestational age (SGA) and non-SGA late preterm and term fetuses. Methods: This was a retrospective national cohort study of all singleton births between 34 0/7 and 42 6/7 weeks of gestation using the Korean vital statistics (n = 2,106,159). We compared the risk of infant mortality with a composite of fetal–infant mortality risk that would occur after expectant management for one additional week and evaluated the optimal time of delivery, in SGA and non-SGA pregnancies. Results: In the total population, the risk of expectant management became significantly higher than the risk of delivery, at 39 weeks and beyond, similar with non-SGA group. In the SGA group, the risk of stillbirth was significantly greater at all GAs than for non-SGA pregnancies, and the risk of infant death was significantly increased until 38 weeks (25.8 per 10,000 live births, 95% CI 20.11–32.47), and the risk of stillbirth was significantly increased at 41 weeks (11.65 per 10,000 ongoing pregnancies, 95% CI 6.95–18.09), compared to 39 weeks (12 per 10,000 live births, 95% CI 8.98–15.64 and 5.12 per 10,000 ongoing pregnancies, 95% CI 3.84–6.66, respectively). Conclusion: In Korean women, delivery between 39 and 41 weeks minimizes fetal/infant mortality, in non-SGA pregnancies. In uncomplicated SGA pregnancies, delivery between 39 and 40 weeks can be considered to decrease risk of infant death and stillbirths. [ABSTRACT FROM AUTHOR]

Congenital lipoid adrenal hyperplasia (CLAH) is caused by mutations to the steroidogenic acute regulatory protein (StAR) gene associated with the inability to synthesize all adrenal and gonadal steroids. Inadequate treatment in an infant with this condition may result in sudden death from an adrenal crisis. We report a case in which CLAH developed in Korean siblings; the second child was prenatally diagnosed because the first child was affected and low maternal serum estriol was detected in a prenatal screening test. To our knowledge, this is the first prenatal diagnosis of the Q258X StAR mutation, which is the only consistent genetic cluster identified to date in Japanese and Korean populations.