<b><i>Introduction:</i></b> This study aimed to assess the association between antenatal corticosteroid (ACS) and in-hospital outcomes of preterm singleton appropriate for gestational age (AGA) infants according to the presence of maternal histologic chorioamnionitis (HCA). <b><i>Methods:</i></b> A retrospective study was performed with singleton AGA neonates of 23⁺⁰ to 33⁺⁶ weeks’ gestation born between 2007 and 2014. We compared the clinical outcomes according to the presence of HCA and ACS use. We also divided the ACS group into 2 groups: infants who received ACS 2–7 days before birth (optimal ACS) or not (suboptimal ACS). Multivariate logistic regression with Firth’s penalized likelihood was performed. <b><i>Results:</i></b> In total, 254 neonates were eligible with 109 neonates with HCA (42.9%). In multivariate analysis adjusting for GA, sex, and cesarean section, ACS use was associated with reduced severe bronchopulmonary dysplasia (BPD) or death and hypotension within 7 postnatal days among the neonates with HCA. However, it was associated with increased patent ductus arteriosus (PDA) treatment. In the optimal ACS group, severe BPD or death (aOR 0.03, 95% CI 0.01–0.42), hypotension (aOR 0.02, 95% CI 0.01–0.26), and inhaled nitric oxide use (aOR 0.06, 95% CI 0.00–0.81) were lower, however, PDA treatment (aOR 8.14, 95% CI 1.20–55.24) and sepsis (aOR 6.85, 95% CI 1.02–46.07) were higher when compared with the no ACS group among HCA+ infants. Among HCA– infants, only PDA treatment was lower in the ACS group. <b><i>Conclusion:</i></b> In neonates with HCA, ACS treatment was associated with reduced morbidities. However, increased sepsis was associated with optimal ACS use.

The term "LADD" was first introduced by Temtamy (1974). LADD syndrome is characterized by varying degrees of hypoplasia/ aplasia of lacrimal ducts/glands, hypoplasia/aplasia of salivary glands, dental anomalies such as hypoplastic enamel, hypodontia, oligodontia, microdontia, inner and outer ear malformations, possible hearing loss, and digital anomalies

Background Severe protein C deficiency is a rare and inherited cause of thrombophilia in neonates. Protein C acts as an anticoagulant, and its deficiency results in vascular thrombosis. Herein, we report a case of protein C deficiency with a homozygous pathogenic variant in a term neonate, with good outcomes after proper treatment. Case presentation A four-day-old male newborn was transferred to the Seoul National University Hospital on account of dark red to black skin lesions. He was born full-term with an average birth weight without perinatal problems. There were no abnormal findings in the prenatal tests, including intrauterine sonography. The first skin lesion was observed on his right toes and rapidly progressed to proximal areas, such as the lower legs, left arm, and buttock. Under the impression of thromboembolism or vasculitis, we performed a coagulopathy workup, which revealed a high D-dimer level of 23.05 μg/ml. A skin biopsy showed fibrin clots in most capillaries, and his protein C activity level was below 10%, from which we diagnosed protein C deficiency. On postnatal day 6, he experienced an apnea event with desaturation and an abnormal right pupillary light reflex. Brain computed tomography showed multifocal patchy intracranial hemorrhage and intraventricular hemorrhage with an old ischemic lesion. Ophthalmic examination revealed bilateral retinal traction detachments with retinal folds. Protein C concentrate replacement therapy was added to previous treatments including steroids, prostaglandin E1, and anticoagulation. After replacement therapy, there were no new skin lesions, and the previous lesions recovered with scarring. Although there were no new brain hemorrhagic infarctions, there was ongoing ischemic tissue loss, which required further rehabilitation. Ophthalmic surgical interventions were performed to treat the bilateral retinal traction detachments with retinal folds. Molecular analysis revealed a homozygous pathogenic variant in the PROC gene. Conclusion Severe protein C deficiency can manifest as a fatal coagulopathy in any organ. Early diagnosis and proper treatment, including protein C concentrate replacement, may improve outcomes without serious sequelae.