Increased risk of clinically relevant neurodevelopmental disorders in survivors of congenital diaphragmatic hernia: a population-based study.

Purpose: Neurodevelopmental disorders (ND) have been recognized as the most common and potentially most disabling outcome of congenital diaphragmatic hernia (CDH). Our aim was to evaluate the incidence of ND in patients with a history of CDH referred for ND assessment on the basis of clinically relevant symptoms rather than through a screening process.

Methods: The study was a nationwide, population-based prospective nested case-control study within a cohort of newborn children who survived the newborn period in Sweden during the observational period from 1st January 1982 until 31st December 2015.

History of endoscopic treatment of vesicoureteral reflux.

Since its first clinical application in 1984, the endoscopic subureteral injection of bulking agents has become an alternative to long-term antibiotic prophylaxis and open surgical intervention in the treatment of VUR in children. The 15 min day care endoscopic procedure has gained worldwide popularity in the management of VUR in children. Over the years, multiple studies have demonstrated safety and long-term efficacy of this minimally invasive outpatient procedure.

Urinary Tract Infection in Children: Management in the Era of Antibiotic Resistance-A Pediatric Urologist's View.

Context: Antibiotic resistance to uropathogens has grown significantly worldwide. Today, pediatric urologist experience a situation that needs appropriate action because urinary tract infections are one of the most common bacterial infections in children.

Objective: In this overview we aimed at presenting the clinical aspects of antibiotic usage in pediatric urology.

Comments to Recent Guidelines on Undescended Testis.

During the past couple of decades, our understanding of the treatment of undescended testis (UDT) has hugely expanded and it is still dynamically changing: new diagnostic tools are available, and experimental procedures are becoming a real-life treatment options. Our community needs to continuously update our guidelines. It is also our responsibility to build up, not a uniform, but a patient-oriented guideline which can provide information for both primary care providers and pediatric surgeons.

Gonadoblastoma in patients with 45,X/46,XY mosaicism: A 16-year experience.

Background: It is recognised that individuals with a 45,X/46,XY karyotype, known as Turner mosaic syndrome with Y chromosome material (TMSY), have an increased risk of developing gonadoblastoma (GB), which may then devolve into one of a number of germ cell malignancies. Hence, children with TMSY are usually recommended to undergo prophylactic gonadectomy.

Objective: We designed this study to describe the phenotypic features of our series of children with TMSY who underwent prophylactic gonadectomy in order to evaluate the prevalence of GB and germ cell malignancies in their resected specimens.

Antenatal retinoic acid administration increases trophoblastic retinol-binding protein dependent retinol transport in the nitrofen model of congenital diaphragmatic hernia.

Background: Low pulmonary retinol levels and disrupted retinoid signaling pathway (RSP) have been implicated in the pathogenesis of congenital diaphragmatic hernia (CDH) and associated pulmonary hypoplasia (PH). It has been demonstrated that nitrofen disturbs the main retinol-binding protein (RBP)-dependent trophoblastic retinol transport. Several studies have demonstrated that prenatal treatment with retinoic acid (RA) can reverse PH in the nitrofen-induced CDH model.

Expression of hepatic lipid droplets is decreased in the nitrofen model of congenital diaphragmatic hernia.

Background: Prenatal mortality in newborn infants with congenital diaphragmatic hernia (CDH) has been attributed to increased amounts of liver hernia ion through the diaphragmatic defect. Antenatal studies in human and rodent fetus with CDH further demonstrated a contribution of the developing liver in the pathogenesis of CDH. The abnormal hepatic growth in experimental animal models, therefore, indicates a disruption of normal liver development in CDH.

Decidual β-carotene-15,15'-oxygenase-1 and 2 (BCMO1,2) expression is increased in nitrofen model of congenital diaphragmatic hernia.

Background: Retinoids are essential for fetal and lung development. Beta-carotene(BC) is the main dietary retinoid source and beta-carotene-15,15'-oxygenase-1 and 2 (Bcmo1,2) is the primary enzyme generating retinoid from BC in adult mammalian tissues. Placenta has a major role in the retinol homeostasis in fetal life: Since there is no fetal retinol synthesis, maternal retinol has to cross the placenta.

Prenatal administration of all-trans retinoic acid upregulates leptin signaling in hypoplastic rat lungs with experimental congenital diaphragmatic hernia.

Purpose: Pulmonary hypoplasia (PH), characterized by alveolar immaturity, is one of the leading causes of respiratory insufficiency in newborns with congenital diaphragmatic hernia (CDH). Leptin (Lep) and its receptor (Lep-R) play an important role in fetal lung growth by stimulating alveolar differentiation and maturation. Lep and Lep-R are strongly expressed by alveolar cells during the saccular stage of fetal lung development.

Nitrofen increases total retinol levels in placenta during lung morphogenesis in the nitrofen model of congenital diaphragmatic hernia.

Background: It has been shown that pulmonary retinol level is decreased during lung morphogenesis in the nitrofen-induced PH in congenital diaphragmatic hernia (CDH). Placenta has a major role in the retinol homeostasis in fetal life. Since there is no fetal retinol synthesis, maternal retinol has to cross the placenta.

Increased uptake of dietary retinoids at the maternal-fetal barrier in the nitrofen model of congenital diaphragmatic hernia.

Background/purpose: 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.

Prenatal administration of retinoic acid increases the trophoblastic insulin-like growth factor 2 protein expression in the nitrofen model of congenital diaphragmatic hernia.

Background: The high mortality rate in congenital diaphragmatic hernia (CDH) is attributed to pulmonary hypoplasia (PH). Insulin-like growth factor 2 (IGF2) is an important regulator of fetal growth. The highest levels of IGF2 expression are found in the placenta, which are negatively regulated by decidual retinoid acid receptor alpha (RARα).

Pulmonary FGF-18 gene expression is downregulated during the canalicular-saccular stages in nitrofen-induced hypoplastic lungs.

Purpose: Pulmonary hypoplasia (PH) associated with congenital diaphragmatic hernia (CDH) represents one of the major challenges in neonatal intensive care. However, the molecular pathogenesis of PH is still poorly understood. In developing fetal lungs, fibroblast growth factor 18 (FGF-18) plays a crucial role in distal airway maturation.

Appendicitis in obese children.

During the past two decades, the incidence of childhood obesity has increased at alarming rates throughout the world. Obesity is associated with a variety of physiological changes that may impair a patient's response to surgery. With the rising rates of childhood obesity, pediatric surgeons must appreciate differences in the management and outcomes of these patients.

Increased trophoblastic apoptosis mediated by neutrophil gelatinase-associated lipocalin (NGAL) activation in the nitrofen model of congenital diaphragmatic hernia.

Background: Retinoids play a key role in fetal lung development. It has been suggested that the maternal-fetal retinol transport is disrupted by trophoblastic apoptosis. The mechanism underlying nitrofen-induced apoptosis in placenta is not fully understood.

Single center experience with endoscopic subureteral dextranomer/hyaluronic acid injection as first line treatment in 1,551 children with intermediate and high grade vesicoureteral reflux.

Purpose: In recent years the endoscopic injection of dextranomer/hyaluronic acid has become an established alternative to long-term antibiotic prophylaxis and the surgical management of vesicoureteral reflux. We determined the safety and effectiveness of the endoscopic injection of dextranomer/hyaluronic acid as first line treatment for high grade vesicoureteral reflux.

Materials And Methods: Between 2001 and 2010, 1,551 children (496 male, 1,055 female, median age 1.

Pax3 gene expression is not altered during diaphragmatic development in nitrofen-induced congenital diaphragmatic hernia.

Background/purpose: Malformations of the pleuroperitoneal folds (PPFs) have been identified as the origin of the diaphragmatic defect in congenital diaphragmatic hernia (CDH). Pax3, expressed in muscle precursor cells (MPCs), plays a key role in regulating myogenesis and muscularization in the fetal diaphragm. Pax3 mutant mice display absence of muscular diaphragm.

Alterations of peroxisome proliferator-activated receptor γ and monocyte chemoattractant protein 1 gene expression in the nitrofen-induced hypoplastic lung.

Background/purpose: Peroxisome proliferator-activated receptor γ (PPARγ) plays a key role in normal lung development. Peroxisome proliferator-activated receptor γ messenger RNA (mRNA) is detectable at 18 days of gestation in fetal rat lungs, and levels peak just before birth. Peroxisome proliferator-activated receptor γ agonists are reported to stimulate lung development, whereas inhibition of PPARγ disrupts postnatal lung maturation.

Nitrofen interferes with trophoblastic expression of retinol-binding protein and transthyretin during lung morphogenesis in the nitrofen-induced congenital diaphragmatic hernia model.

Background: Retinoids play a key role in lung development. Retinoid signaling pathway has been shown to be disrupted in the nitrofen model of congenital diaphragmatic hernia (CDH) but the exact mechanism is not clearly understood. Retinol-binding protein (RBP) and transthyretin (TTR) are transport proteins for delivery of retinol to the tissues via circulation.

Urinary tract anomalies associated with high grade primary vesicoureteral reflux.

Background: Few studies have evaluated the significance of associated urological anomalies in vesicoureteral reflux (VUR). The aim of our study was to determine the incidence of associated urological anomalies in patients with high grade VUR and to assess their impact on renal parenchymal scarring.

Methods: We retrospectively reviewed the hospital records of 1,765 consecutive cases diagnosed with high grade VUR (Grade III-V) at our hospital between 1998 and 2010.

Upregulation of fibroblast growth factor receptor 2 and 3 in the late stages of fetal lung development in the nitrofen rat model.

Purpose: Nitrofen model of congenital diaphragmatic hernia (CDH) has been widely used to investigate the pathogenesis of pulmonary hypoplasia (PH). Fibroblast growth factor (FGF) signaling pathway plays a fundamental role in fetal lung development. FGF7 and FGF10, which are critical for lung morphogenesis, have been reported to be downregulated in nitrofen-induced PH.

Smad1 and WIF1 genes are downregulated during saccular stage of lung development in the nitrofen rat model.

Purpose: The exact pathogenesis of pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia (CDH) still remains unclear. Smad1, one of the bone morphogenesis protein (BMP) receptor downstream signaling proteins, plays a key role in organogenesis including lung development and maturation. Smad1 knockout mice display reduced sacculation, an important feature of pulmonary hypoplasia.

[Indication for and execution of rectal examination in children with suspected appendicitis].

Digital rectal examination is considered a non-evident diagnostic procedure in suspected appendicitis. It is rather unpleasant for children and there is a wide range of laboratory and imaging modalities available to contribute to the decision making in case a surgical intervention is necessary. In recent years digital rectal examination is not routinely used prior to surgery, however it may remain a useful screening method for patients with unclear clinical diagnosis.

Laparoscopic appendectomy is associated with lower morbidity in extremely obese children.

Purpose: In recent years there has been worldwide increase in childhood obesity. However, in the beginning laparoscopic appendectomy in obese children was contraindicated, now it is commonly used for the treatment of appendicitis. The purpose of this study was to compare open versus laparoscopic appendectomy in extremely obese children.

Increased incidence of negative appendectomy in childhood obesity.

Background: In recent years, there has been worldwide increase in childhood obesity. The diagnosis of acute appendicitis in very obese children can sometimes be difficult and challenging. The purpose of this study was to determine the incidence of histologically normal appendix in very obese and non-obese children undergoing emergency appendectomy for the clinical diagnosis of acute appendicitis.