Local dexamethasone improves the intestinal lesions of gastroschisis in chick embryos.

Background And Aims: Eviscerated bowel in gastroschisis (Gx) undergoes changes that lead to dysfunctions and create management difficulties. This study tests the hypothesis that exposure of the eviscerated bowel of chick embryos with Gx to dexamethasone might have beneficial effects on the parietal lesions.

Methods: Gx was created in chick embryos on incubation day 15 and either dexamethasone (0.

[Prognostic factors in pediatric liver transplantation. Multivariate analysis].

Aim: To analyze independent risk factors associated with poor graft and patient survival in a series of 292 pediatric liver transplants (PLT) performed in 234 children during a 15 years period. MATERIAL AND METHODS. 1.

Effects of retinoic acid on the neural crest-controlled organs of fetal rats.

Prenatal exposure of rat embryos to retinoic acid induces severe malformations involving various organs. The mechanisms of this embryopathy are known only in part. This study describes the malformations of the neural crest-derived organs in this model and shows that many of them fit into the pattern of disturbed neural crest organogenic control.

Prenatal vitamin E improves lung and heart hypoplasia in experimental diaphragmatic [correction of diaphragamatic] hernia.

Nitrofen induces in rats diaphragmatic hernia (CDH) with heart and lung hypoplasia by a mechanism involving oxidation. The aim of this study was to examine if prenatal administration of the anti-oxidant agent vitamin E (VitE) prevents to some extent heart and lung hypoplasia. Pregnant rats received on E9.

Effects of prenatal dexamethasone on the intestine of rats with gastroschisis.

Background/purpose: Intestinal lesions observed in gastroschisis (Gx) are accompanied by neonatal gastrointestinal dysfunction. This study examines the effects of transplacental dexamethasone on the eviscerated intestine of fetal rats with Gx.

Methods: Gx was created surgically in rat fetuses on gestational day 18, and the dams were treated either with 0.

The adrenal cortex in experimental congenital diaphragmatic hernia.

Background/purpose: Adrenal cortical malfunction was found recently in patients with severe congenital diaphragmatic hernia (CDH). The current study tests the hypothesis that the development and function of the adrenal cortex could be abnormal in an experimental model of CDH.

Methods: Pregnant rats were exposed on day 9.

[Kasai operation in the age of liver transplantation. Healing or merely palliative technique?].

Aim: To assess the results of portoenteroanastomosis (PEA) and liver transplantation (OLT) in extrahepatic biliary atresia (EHBA).

Methods: Out of a series of 148 EHBA, 92 cases primarily treated by us were selected. Survival with the native liver (end point = death or OLT) and its relationship with the age at PEA, type of EHBA, ductal size and bile flow restablishment were assessed.

Effects of early embryonal exposure to dexamethasone on malformations of neural-crest derivatives induced by nitrofen in rats.

Prenatal corticosteroids reverse to some extent lung and heart hypoplasia in nitrofen-exposed rat pups. The present study examines the effects of early exposure to dexamethasone on the neural crest-related malformations of the cardiovascular system, thymus, parathyroids, and thyroid observed in this model. Pregnant rats were exposed on gestational day 9.

Effects of vitamin A on malformations of neural-crest-controlled organs induced by nitrofen in rats.

Vitamin A (vit A) alleviates the effects of nitrofen in exposed rat pups. The present study examines the effects of early exposure to vitamin A on the neural-crest-related cardiovascular, thymic, parathyroid, and thyroid malformations previously reported in the rat model of congenital diaphragmatic hernia (CDH). Pregnant rats were exposed on gestational day 9.

Neural crest-derived defects in experimental congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is often associated with other malformations. This study tests the hypothesis that the heart and great vessels, thymus, parathyroids, and thyroid might be abnormal in the rat model of CDH as a result of disturbed neural-crest development. Time-mated pregnant rats were fed either 100 mg 2-4-dichlorophenyl-p-nitrophenyl ether (nitrofen) or vehicle on gestational day 9.

Prenatal dexamethasone rescues heart hypoplasia in fetal rats with congenital diaphragmatic hernia.

Background/purpose: Patients and rats with congenital diaphragmatic hernia (CDH) have lung and heart hypoplasia. Prenatal steroids improve lung hypoplasia in CDH rats. The current study tests the hypothesis that prenatal dexamethasone could rescue heart hypoplasia in rats with CDH.

The contribution of the adriamycin-induced rat model of the VATER association to our understanding of congenital abnormalities and their embryogenesis.

The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with the VATER association. Most interest has been centered on the foregut, where the model has clarified some aspects of the development of esophageal atresia (EA), tracheal agenesis, and other communicating bronchopulmonary foregut malformations. It has demonstrated aberrations in the nerve supply to the esophagus in EA and allowed the study of tracheomalacia.

Down-regulation of thyroid transcription factor-1 gene expression in fetal lung hypoplasia is restored by glucocorticoids.

The thyroid transcription factor (TTF)-1 has an essential role in lung morphogenesis and development. It is involved in the transcription of surfactant proteins (SP), which are critical in respiratory function. Neonates with congenital diaphragmatic hernia die of respiratory failure caused by pulmonary hypoplasia with associated biochemical immaturity.

Neural crest-derived defects in experimental esophageal atresia.

Esophageal atresia (EA) is often associated with cardiovascular and other malformations that are likely neural crest derived. The present study tests the hypothesis that the heart and great vessels and the thymus and parathyroids may be abnormal in the rat model of EA as a result of disturbed neural crest development. Time-mated pregnant rats received intraperitoneally on d 8 and 9 of gestation either 2 mg/kg adriamycin or vehicle.

Muscular architecture and manometric image of gastroesophageal barrier in the rat.

The two components of the gastroesophageal barrier, the sphincter and the crural sling, closely overlap in humans, whereas they are widely separated in the rat. This investigation correlates the anatomical components of the barrier and their manometric counterparts in this animal. Sphincteric and crural sling pressures were measured in four quadrants in 23 rats.

Skeletal malformations associated with congenital diaphragmatic hernia: experimental and human studies.

Background/purpose: Skeletal malformations are seen occasionally in infants with congenital diaphragmatic hernia (CDH). This study examines whether nitrofen, able to produce CDH in fetal rats, also induces skeletal anomalies and, if so, whether these are similar to those seen in CDH patients.

Methods: Pregnant rats received either nitrofen (100 mg, n = 7) or no treatment (n = 2) on gestational day 9.

Gastroesophageal reflux after combined lower esophageal sphincter and diaphragmatic crural sling inactivation in the rat.

This study tests the hypothesis that either selective or combined destruction of the lower esophageal sphincter and the diaphragmatic crural sling should induce reflux in the rat. Pull-through perfusion manometry was performed before and after lower esophageal myectomy, crural myotomy, or both. pH monitoring was used to detect reflux.

[Tissue adhesives in closing of fistulas after surgery of esophageal atresia].

Aims: Recurrent tracheoesophageal fistula is a severe postoperative complication after esophageal atresia repair. Endoscopic obliteration with tissue adhesives has been used as an alternative to reoperation. The aim of this study is to review our experience with such procedure.

[Respiratory malformations associated with esophageal atresia].

Aims: Since trachea, lungs and esophagus develop from foregut and esophageal atresia is a defect of its normal division, we examined the occurrence of respiratory malformations in a large clinical series of esophageal atresia.

Materials And Methods: The records of 415 patients born with esophageal atresia between 1965 and 1996 and 129 autopsies of the same patients were retrospectively reviewed. The presence of other associated anomalies was carefully studied and noted.

Skeletal malformations associated with esophageal atresia: clinical and experimental studies.

Background/purpose: Patients with esophageal atresia (EA) often have skeletal malformations. The purpose of this study is to examine if similar defects occur in rat fetuses prenatally exposed to Adriamycin, a chemical capable of causing EA in these animals.

Methods: The charts of 443 babies with EA were reviewed to assess the incidence and nature of these defects in them.

Cardiovascular malformations in congenital diaphragmatic hernia: human and experimental studies.

Background/purpose: Cardiovascular malformations (CVM) associated with congenital diaphragmatic hernia (CDH) account in part for the high mortality caused by this defect. The aim of this study is to examine the nature of these malformations in a large series of autopsies and to assess if similar defects are also present in rat fetuses with experimental CDH.

Methods: The incidence of CVM and their nature were examined in the autopsy records of 136 stillborns and neonates with CDH admitted to our institution in the last 30 years.

Notochord involvement in experimental esophageal atresia.

Esophageal atresia (EA) is often accompanied by vertebral defects and other anomalies. The adriamycin rat model of EA has disclosed the embryology of the malformation and shown that the vertebrae and notochord are also abnormal. This study describes the nature of notochord malformations in rat embryos exposed to adriamycin.

Lung hypoplasia caused by nitrofen is mediated by down-regulation of thyroid transcription factor TTF-1.

Prenatal exposure to nitrofen induces lung hypoplasia and diaphragmatic hernias very similar to those in human disease, but the mechanisms are still unknown. Thyroid transcription factor 1 (TTF-1) is involved in lung ontogeny and regulation of the expression of surfactant proteins, and is likely abnormally expressed in nitrofen-induced lung hypoplasia. This study examines the effect of nitrofen on TTF-1 messenger RNA (mRNA) expression in the lungs of prenatal rat fetuses and a human lung-cell line (NCI-H441) that expresses both TTF-1 and surfactant proteins in vivo.

The tracheobronchial tree is abnormal in experimental congenital diaphragmatic hernia.

Neonates with congenital diaphragmatic hernia (CDH) have other malformations that contribute to the high mortality. The nitrofen rat model allows experimental study of these anomalies. This study examines whether the tracheobronchial tree is also abnormal in this model.

Heart hypoplasia in experimental congenital diaphragmatic hernia.

Background/purpose: Heart hypoplasia is associated with congenital diaphragmatic hernia (CDH) and decisively influences survival rate. This study examines whether nitrofen-exposed fetal rats have heart hypoplasia.

Methods: Pregnant rats received either 100 mg nitrofen or vehicle on gestational day 9.