Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia.

Aim Of The Study: Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH.

Antegrade nailing can prevent cubitus varus and valgus after pediatric supracondylar fractures with impacted columns.

Supracondylar fractures are commonly encountered in the pediatric population. An impacted radial or ulnar column may result in the deviation of the elbow axis in the frontal plane clinically seen as cubitus varus or valgus. Antegrade nailing has become a feasible alternative to treat supracondylar fractures.

Pre- and postoperative rectal manometric assessment of patients with anorectal malformations: should we preserve the fistula?

Background: Surgical correction of congenital anorectal malformations could be complicated by fecal incontinence. Some authors believe that preservation of the fistula is associated with improved outcome. Rectal manometry is a well-established method to evaluate postoperative functional outcome in these patients and can demonstrate successful transplantation of the fistula.

Decreased expression of GATA4 in the diaphragm of nitrofen-induced congenital diaphragmatic hernia.

Background: The molecular mechanisms underlying the diaphragmatic defect in congenital diaphragmatic hernia (CDH) are still poorly understood. The transcription factor GATA4 is essential for normal development of the diaphragm. Recently, mutations in the GATA4 gene have been linked to human and rodent CDH.

Is there unity in Europe? First survey of EUPSA delegates on the management of gastroschisis.

Aim: To report the first European survey on the current management of gastroschisis and ascertain the degree of variability between centers.

Methods: A 10-question survey was administered at the 2011 European Paediatric Surgeons' Association (EUPSA) Congress. Questionnaires were completed by 205 delegates from 39 countries.

Trends in the treatment and outcome of congenital diaphragmatic hernia over the last decade.

Purpose: Congenital diaphragmatic hernia (CDH) remains a challenging and life-threatening congenital anomaly. The aim was to evaluate whether treatment and survival has changed during the last decade.

Methods: We retrospectively analysed all consecutive infants with CDH referred to two European tertiary paediatric surgical centres over 11 years (January 1999 to December 2009).

Closure of bronchopleural fistula with porcine dermal collagen and fibrin glue in an infant.

The management of an 11-month-old infant who developed a bronchopleural fistula (BPF) 3 weeks after video-assisted thoracic surgery for congenital cystic adenomatoid malformation of the right lower pulmonary lobe is presented. Being refractory to treatment with chest tubes, the BPF was managed using a bronchoscopic approach using porcine dermal collagen (PDC) combined with a fibrin glue plug. The single session was sufficient to manage the BPF and the postoperative course was uneventful.

The 100 most-cited articles in Pediatric Surgery International.

Purpose: Citation analysis within specific journals and subject areas has become a popular method to assess the impact of a journal, article or author. To date, only a few evaluations of citation reports have been published in the field of pediatric surgery. Twenty-six years after its inception, Pediatric Surgery International (PSI) is a firmly established journal in pediatric surgery.

Prenatal retinoic acid upregulates connexin 43 (Cx43) gene expression in pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia rat model.

Purpose: Connexin 43 (Cx43), a major gap junction protein, is necessary for alveologenesis and plays an important role in the differentiation of type II to type I alveolar epithelial cells. Knockout mice of Cx43 display severe pulmonary hypoplasia (PH). Prenatal administration of retinoic acid (RA) is known to stimulate alveologenesis in nitrofen-induced PH.

Treatment algorithm for complex injuries of the foot in paediatric patients.

Background: Complex injuries of the foot in the paediatric population present difficult treatment challenges. While standardised protocols exist for the adult population to achieve an optimal result in the treatment of such injuries, therapy in paediatric patientsmust be managed without a firm treatment algorithm.

Methods: Medical records of all patients with a complex trauma of the foot treated at our Department over a period of 13 years were evaluated.

Prenatal administration of retinoic acid upregulates insulin-like growth factor receptors in the nitrofen-induced hypoplastic lung.

Background: Pulmonary hypoplasia (PH) is the main cause of mortality in newborns with congenital diaphragmatic hernia (CDH). Prenatal administration of retinoic acid (RA) stimulates alveologenesis in the nitrofen-induced pulmonary hypoplasia. Insulin-like growth factor receptors (IGFRs) play a crucial role in alveologenesis during lung development.

Prenatal administration of retinoic acid upregulates connective tissue growth factor in the nitrofen CDH model.

Purpose: Recent studies have suggested that retinoids may be involved in the molecular mechanisms of pulmonary hypoplasia (PH) in congenital diaphragmatic hernia (CDH). Connective tissue growth factor (CTGF) plays a key role in foetal lung development and remodelling during later gestation. CTGF knockout mice exhibit PH with similar characteristics to the human and nitrofen-induced PH.

The role of primary myogenic regulatory factors in the developing diaphragmatic muscle in the nitrofen-induced diaphragmatic hernia.

Purpose: The nitrofen model of congenital diaphragmatic hernia (CDH) is widely used to investigate the pathogenesis of CDH. However, the exact pathomechanism of the diaphragmatic defect is still unclear. Diaphragmatic muscularization represents the last stage of diaphragmatic development.

Expression of Iroquois genes is up-regulated during early lung development in the nitrofen-induced pulmonary hypoplasia.

Background/purpose: Iroquois homeobox (Irx) genes have been implicated in the early lung morphogenesis of vertebrates. Irx1-3 and Irx5 gene expression is seen in fetal lung in rodents up to day (D) 18.5 of gestation.

Expression of the Wilm's tumor gene WT1 during diaphragmatic development in the nitrofen model for congenital diaphragmatic hernia.

Purpose: The nitrofen model of congenital diaphragmatic hernia (CDH) reproduces a typical diaphragmatic defect. However, the exact pathomechanism of CDH is still unknown. The Wilm's tumor 1 gene (WT1) is crucial for diaphragmatic development.

Downregulation of Midkine gene expression and its response to retinoic acid treatment in the nitrofen-induced hypoplastic lung.

Purpose: Nitrofen-induced congenital diaphragmatic hernia (CDH) model has been widely used to investigate the pathogenesis of pulmonary hypoplasia (PH) in CDH. Recent studies have suggested that retinoids may be involved in the molecular mechanisms of PH in CDH. Prenatal treatment with retinoic acid (RA) has been reported to improve the growth of hypoplastic lung in the nitrofen CDH model.

Prenatal retinoic acid treatment upregulates late gestation lung protein 1 in the nitrofen-induced hypoplastic lung in late gestation.

Purpose: Pulmonary hypoplasia (PH), the leading cause of mortality in congenital diaphragmatic hernia (CDH), is associated with arrested alveolarization. Late gestation lung protein 1 (LGL1) plays a crucial role in the regulation of alveolarization. Inhibition of LGL1 impairs alveolar maturation in fetal rat lungs.

Abnormal platelet-derived growth factor signaling accounting for lung hypoplasia in experimental congenital diaphragmatic hernia.

Purpose: The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is not fully understood. Platelet-derived growth factor A (PDGFA) and platelet-derived growth factor receptor α (PDGFRα) play a crucial role in lung development. It has been reported that PDGF induces H(2)O(2)-production and that oxidative stress may be an important mechanism for the impaired lung development in the nitrofen rat model.

Prenatal treatment with retinoic acid activates parathyroid hormone-related protein signaling in the nitrofen-induced hypoplastic lung.

Purpose: Prenatal treatment with retinoic acid (RA) has been reported to stimulate alveologenesis in hypoplastic lungs (HL) in the nitrofen model of congenital diaphragmatic hernia (CDH). Parathyroid hormone-related protein (PTHrP) promotes alveolar maturation by stimulating surfactant production, regulated by PTHrP receptor (PTHrP-R). PTHrP knockout and PTHrP-R null mice both exhibit pulmonary hypoplasia.

Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung's disease.

Purpose: The transanal one-stage pull-through procedure (TERPT) has gained worldwide popularity over open and laparoscopic-assisted one-stage techniques in children with Hirschsprung's disease (HD). It offers the advantages of avoiding laparotomy, laparoscopy, scars, abdominal contamination, and adhesions. However, enterocolitis associated with Hirschsprung's disease (HAEC) still remains to be a potentially life-threatening complication after pull-through operation.

Prenatal retinoic acid upregulates pulmonary gene expression of PI3K and AKT in nitrofen-induced pulmonary hypoplasia.

Background: The precise mechanism of pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) still remains unclear. Recently, prenatal treatment with retinoic acid (RA) has been reported to stimulate alveologenesis in hypoplastic lungs in the nitrofen model of CDH. The serine/threonine protein kinase B (AKT) plays a key role in lung morphogenesis through epithelial-mesenchymal interaction in phosphatidylinositide 3-kinase (PI3K)-dependent manner.

Insulinlike growth factor receptor type 1 and type 2 are downregulated in the nitrofen-induced hypoplastic lung.

Purpose: In congenital diaphragmatic hernia (CDH), high mortality rates are attributed to severe pulmonary hypoplasia. The insulinlike growth factor receptor type 1 (IGF-1R) and type 2 (IGF-2R) play a critical role in the alveologenesis during lung development. The IGF-1R null mutation mice die after birth because of respiratory failure.

Insulin receptor is downregulated in the nitrofen-induced hypoplastic lung.

Purpose: The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is still poorly understood. During fetal lung development, the insulin receptor (IR) plays an important role by mediating the cellular uptake of glucose, which is a major substrate for the biosynthesis of surfactant phospholipids. In fetal rat lung, IR gene expression has been revealed on type II pneumocytes.

Spatiotemporal alteration in phosphatidylinositide 3-kinase-serine/threonine protein kinase B signaling in the nitrofen-induced hypoplastic lung.

Purpose: The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is not fully understood. The serine/threonine protein kinase B (AKT) plays important roles for lung morphogenesis through epithelial-mesenchymal interaction in phosphatidylinositide 3-kinase (PI3K)-dependent manner. It has been reported that the lung explant morphogenesis in mice is interfered by inhibitors of the PI3K-AKT pathway.

Disturbance of parathyroid hormone-related protein signaling in the nitrofen-induced hypoplastic lung.

Purpose: Despite remarkable progress in resuscitation and intensive care, the morbidity and mortality rates in congenital diaphragmatic hernia (CDH) remain high due to severe pulmonary hypoplasia. The pathogenesis of pulmonary hypoplasia associated with CDH is still not clearly understood. Pulmonary parathyroid hormone-related protein (PTHrP) is expressed in the type II epithelial cells and stimulates surfactant production by a paracrine feedback loop regulated by PTHrP receptor (PTHrP-R), which is expressed in the mesenchyme, during terminal airway differentiation.