AAP Papers
Fetal MRI lung volumes are predictive of perinatal outcomes in fetuses with congenital lung masses
The purpose of this study was to evaluate fetal magnetic resonance imaging (MRI) as a modality for predicting perinatal outcomes and lung-related morbidity in fetuses with congenital lung masses (CLM).Slide tracheoplasty for the treatment of tracheoesophogeal fistulas
The purpose of this study is to determine the surgical outcome of slide tracheoplasty for the treatment of tracheoesophageal (TE) fistula in pediatric patients.Postoperative opioid analgesic use after Nuss versus Ravitch pectus excavatum repair
Anticipated postoperative pain may affect procedure choice in patients with pectus excavatum. This study aims to compare postoperative pain in patients undergoing Nuss and Ravitch procedures.Is early delivery beneficial in gastroschisis?
Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37 weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD).Application of prostaglandin E2 improves ileal blood flow in NEC
Indomethacin, a nonselective prostaglandin inhibitor used to treat patent ductus arteriosus, is associated with intestinal perforation inducing an NEC-like illness. We sought to define the contribution of prostaglandin E2 (PG E2) and its receptor EP4 to intestinal blood flow regulation in premature neonates with NEC.Predictors of early lung function in patients with congenital diaphragmatic hernia
Long-term pulmonary outcomes of congenital diaphragmatic hernia (CDH) have demonstrated airflow obstruction in later childhood. We examined pulmonary function data to assess what factors predict lung function in the first three years of life in children with CDH.Musculoskeletal deformities following repair of large congenital diaphragmatic hernias
Large congenital diaphragmatic hernias (CDH) can be repaired with either a muscle flap or prosthetic patch. The purpose of this study was to assess the frequency and severity of scoliosis, chest wall, and abdominal wall deformities following these repairs.Sensorineural hearing loss in congenital diaphragmatic hernia survivors is associated with postnatal management and not defect size
We determined the incidence of sensorineural hearing loss (SNHL; >20 dB at any frequency) in a contemporary cohort of congenital diaphragmatic hernia (CDH) survivors at a single tertiary care center and identified potential risk factors for SNHL.Increased uptake of dietary retinoids at the maternal-fetal barrier in the nitrofen model of congenital diaphragmatic hernia
The retinol signaling pathway is disrupted in congenital diaphragmatic hernia (CDH). Since there is no fetal retinol synthesis, maternal retinol has to cross the placenta. Nitrofen interferes with the retinol-binding protein (RBP) transfer pathway in CDH. However, in RBP knockout mice, retinol has been shown to be present. In this model, increased uptake of maternal dietary retinyl ester (RE) bounded in low-dense-lipoprotein (LDL) through low-density-lipoprotein-receptor 1 (LRP1) and increased activity of RE hydrolysis by lipoprotein-lipase (LPL) have been found.Segmental resection for the treatment of congenital pulmonary malformations
The purpose of this study was to compare clinical outcomes of segmental resection to lobectomy as increasing antenatal diagnosis of congenital pulmonary malformations has led to a shift in surgical management.Prenatal retinoic acid increases lipofibroblast expression in hypoplastic rat lungs with experimental congenital diaphragmatic hernia
Prenatal administration of all-trans retinoic acid (ATRA) has been shown to stimulate alveolarization in nitrofen-induced pulmonary hypoplasia (PH) associated with congenital diaphragmatic hernia (CDH). Lipid-containing interstitial lipofibroblasts (LIFs), characterized by adipocyte differentiation-related protein (ADRP), play a critical role in alveolar development by coordinating lipid homeostasis. Previous studies have demonstrated that ATRA positively affects LIF expression in developing lungs.A comparative analysis of human mesenchymal stem cell response to hypoxia in vitro: Implications to translational strategies
Mesenchymal stem cells (MSCs) are particularly valuable for structural tissue replacement. We compared the response to hypoxia among human MSCs derived from four different clinically relevant sources as an adjunct to translational developments.Evaluation of the treatment of pectus carinatum with compressive orthotic bracing using three dimensional body scans
The purpose of this study is to measure the effectiveness of compressive orthotic brace therapy for the treatment of pectus carinatum using an adjusted Haller Index (HI) measurement calculated from 3D body scan (BS) images.Amniotic fluid derived mesenchymal stromal cells augment fetal lung growth in a nitrofen explant model
Recent experimental work suggests the therapeutic role of mesenchymal stromal cells (MSCs) during lung morphogenesis. The purpose of this study was to investigate the potential paracrine effects of amniotic fluid-derived MSCs (AF-MSCs) on fetal lung growth in a nitrofen explant model.Upregulation of serotonin-receptor-2a and serotonin transporter expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia
Congenital diaphragmatic hernia (CDH) is attributed to severe pulmonary hypoplasia and pulmonary hypertension (PH). PH is characterized by structural changes resulting in vascular remodeling. Serotonin, a potent vasoconstrictor, plays a central role in the development of PH. It exerts its constricting effects on the vessels via Serotonin receptor 2A (5-HT2A) and induces pulmonary smooth muscle cell proliferation via the serotonin transporter (5-HTT). This study was designed to investigate expressions of 5-HT2A and 5-HTT in the pulmonary vasculature of rats with nitrofen-induced CDH.Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia
The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.Outcomes of fetal intervention for primary hydrothorax
Primary hydrothorax is a rare congenital anomaly with outcomes ranging from spontaneous resolution to fetal demise. We reviewed our experience with fetuses diagnosed with primary hydrothorax to evaluate prenatal management strategies.Heterotaxy syndrome and malrotation: Does isomerism influence risk and decision to treat
Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management.HB-EGF augments the ability of mesenchymal stem cells to attenuate intestinal injury
We have previously demonstrated that heparin-binding EGF-like growth factor (HB-EGF) and mesenchymal stem cell (MSC) administration protect the intestines from ischemia/reperfusion (I/R) injury in vivo, with amniotic fluid-derived MSC (AF-MSC) being more efficacious than bone marrow-derived MSC (BM-MSC). The goal of the current study was to determine whether the protective effects of HB-EGF were from direct effects on MSC or via alternative mechanisms.
