Laminin-1 promotes enteric nervous system development in mouse embryo.

Background/aim: Neuronal development is regulated by extracellular environmental factors including nerve growth factor (NGF) and laminin. We have previously demonstrated that laminin-1 promotes neurite outgrowth of dorsal root ganglion cells by modulating NGF and integrin signaling. However, information about their effects on the enteric nervous system (ENS) is limited.

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.

Tubularized incised plate urethroplasty with dorsal inlay graft prevents meatal/neourethral stenosis: a single surgeon's experience.

Background/purpose: Snodgrass tubularized incised plate urethroplasty (SUP) is versatile and has good cosmesis. However, postoperative meatal/neourethral stenosis (M/N-S) is common enough for some surgeons to add a dorsal inlay graft (DIG) harvested from the inner prepuce and sutured to cover the longitudinal midline incision of the urethral plate. This is the first formal assessment of the effectiveness of DIG for preventing M/N-S.

Increase in fetal pulmonary artery diameters during late gestation is a predictor of outcome in congenital diaphragmatic hernia with liver herniation.

Aim: Liver herniation (LH) in congenital diaphragmatic hernia (CDH) may not be a reliable prognostic indicator. We measured pulmonary artery (PA) diameters in CDH + LH as an alternative.

Methods: Of 41 consecutive cases of prenatally diagnosed left-sided CDH treated from 2002 to 2010, 19 had CDH + LH and 22 had CDH - LH.

Radiographic signs predictive of success of hydrostatic reduction of intussusception.

Aim: Outcome of hydrostatic reduction of intussusception (HRI) was analyzed according to specific radiographic signs to improve success.

Methods: At our institution, a pediatric surgical team performs HRI using a standardized protocol. We reviewed 266 consecutive HRI performed from 1998 to 2008 according to patient demographics, symptomatology, parameters of inflammation (peak WBC, peak CRP), position of the tip of the intussuscepted bowel and an intussusception bowel ratio (IBR).

Treves' field pouch hernia: our experience and literature review.

Treves' field pouch hernia (TFPH) is an unusual type of congenital internal hernia. Although eight cases of TFPH have been reported in the English literature, they were seldom diagnosed preoperatively with high mortality rates. We describe a 12-year-old girl with TFPH diagnosed as an internal hernia on computed tomography and confirmed laparoscopically, and review the literature.

Congenital diaphragmatic hernia and retinoids: searching for an etiology.

Congenital diaphragmatic hernia (CDH) is a major life-threatening cause of respiratory failure in the newborn. Recent data reveal the role of a retinoid-signaling pathway disruption in the pathogenesis of CDH. We describe the epidemiology and pathophysiology of human CDH, the metabolism of retinoids and the implications of retinoids in the development of the diaphragm and lung.

Prenatal treatment with retinoic acid accelerates type 1 alveolar cell proliferation of the hypoplastic lung in the nitrofen model of congenital diaphragmatic hernia.

Purpose: Retinoids play an important role in lung development. A recent study has demonstrated that prenatal treatment with retinoic acid (RA) stimulates alveologenesis in hypoplastic lungs in the nitrofen model of congenital diaphragmatic hernia (CDH). Furthermore, it has also been demonstrated that the differentiation from alveolar epithelial cells type II (AECs-II) into alveolar epithelial cells type I (AECs-I), which is the key process in lung development, is disturbed in this model.

Down-regulation of Wnt signal pathway in nitrofen-induced hypoplastic lung.

Purpose: The pathogenesis of pulmonary hypoplasia associated with congenital diaphragmatic hernia is poorly understood. Recently, it has been reported that Wnt signaling pathway plays a critical role in branching lung morphogenesis. Mice lacking Wnt7b gene die soon after birth because of respiratory failure and display severe lung hypoplasia.

Reduced expression of aquaporin 5 water channel in nitrofen-induced hypoplastic lung with congenital diaphragmatic hernia rat model.

Purpose: Pulmonary hypoplasia remains the principal cause of high morbidity and mortality in patients with congenital diaphragmatic hernia (CDH). The precise mechanisms causing lung hypoplasia remains unclear. Aquaporins (AQPs) are reported to constitute a family of water channels that facilitate membrane water permeability in various tissues of animals.

Kidney development in the nitrofen-induced pulmonary hypoplasia and congenital diaphragmatic hernia in rats.

Background/purpose: The relationship of the developing lung and kidney is not completely understood. Renal enlargement has been reported in association with pulmonary hypoplasia in congenital diaphragmatic hernia (CDH). Recent studies suggest that retinoids may be involved in the pathogenesis of CDH.

Altered regulation of retinoic acid synthesis in nitrofen-induced hypoplastic lung.

Retinoids are a group of molecules derived from vitamin A, which play an important role in lung development. Within the cell, retinol can either be oxidized to retinal or esterified to retinyl esters by lecithin : retinol acyltransferase (LRAT) for storage. Retinal is then oxidized to an active metabolite of vitamin A, retinoic acid (RA) by retinal dehydrogenase (RALDH).

Reduction of intussusception in infants by a pediatric surgical team: improvement in safety and outcome.

Commonly, reduction of intussusception is performed by experienced radiologists. We review the performance of a pediatric surgical team for treating intussusception according to a standard protocol and present our findings. Three hundred and seventy eight patients with signs and symptoms of intussusception we treated from 1980 to 2005 were reviewed.

Altered renin-angiotensin system gene expression causes renal hypoplasia in the rats with nitrofen-induced diaphragmatic hernia.

The association between renal hypoplasia and pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) has become recently appreciated. However, the underlying mechanisms responsible for this association are still unknown. Renin-angiotensin system (RAS) plays an important role in renal and somatic growth, angiogenesis and reproduction.

Retinoic acid rescues lung hypoplasia in nitrofen-induced hypoplastic foetal rat lung explants.

There is increasing evidence to suggest that the retinoid pathway is involved in the pathogenesis of congenital diaphragmatic hernia (CDH). We hypothesised that retinoids are involved in the pathogenesis of associated pulmonary hypoplasia in CDH and therefore designed this study to investigate the effects of retinoid acid on nitrofen-induced hypoplastic lungs. Pregnant rats were exposed to either olive oil or 100 mg nitrofen on day 9.

Prenatal detection of isolated gastric duplication cyst.

We present a rare case of gastric duplication cyst that was suspected prenatally. A routine prenatal ultrasonography (US) showed an abdominal cyst with peristalsis and a provisional diagnosis of enteric duplication was made. A healthy male infant was born at 39 weeks gestation and postnatal US identified a cyst, 5x3x2 cm in size, adjacent to the pancreas.