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Objective: To report on the initial experience in a single Brazilian university clinic of the use of fetoscopic endotracheal occlusion (FETO) to treat severe isolated congenital diaphragmatic hernia (CDH). Methods: The inclusion criteria for FETO for this prospective study were isolated CDH and intrathoracic herniation of the liver, as well as the lung area to head circumference ratio (LHR) <1.0. The main variables evaluated were LHR and observed to expected (o/e) LHR before and after FETO, gestational age (GA) at FETO, reversal of tracheal occlusion (TO), and birth and discharge of a living child from the hospital. Results: Among 8 isolated left-sided CDH cases with normal karyotypes, the median LHR and o/e LHR before FETO were 0.7 (range: 0.6-0.9) and 0.27 (range: 0.22-0.32), respectively. The median LHR and o/e LHR after FETO were 1.2 (range: 0.9-1.8) and 0.45 (0.31-0.67), respectively. The median GA at FETO, reversal of TO and birth were 26.8 (range: 26-29), 32.5 (range: 31.0-34.0) and 37 weeks (range: 35-37), respectively. Neonatal survival at the time of hospital discharge was 50% (4/8). Conclusion: FETO is feasible at our institution and may help to improve postnatal survival of children with severe CDH in developing countries. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objective: To investigate glomerular development and expression of insulin and insulin-like growth factor receptors in an experimental model of intrauterine growth restriction (IUGR). Material and Methods: We studied three groups of Sprague-Dawley fetuses: IUGR - restricted by ligation of the right uterine artery; C-IUGR - left horn controls, and EC - external controls (non-manipulated). Body and organs were weighed, and glomerular number and volume were analyzed. Expression of IRβ, IRS-1, IRS-2 and IGF-IRβ was analyzed in liver, intestine and kidneys by immunoblotting. Results: Organ/body weight ratios were similar. In IUGR, glomerular number and volume were increased compared to C-IUGR and EC (p<0.001). In the IUGR liver, increases were found in IGF-IRβ compared to C-IUGR and EC; IRβ compared to EC, and IRS-2 compared to C-IUGR. However, decreases in IRβ were noted in IUGR compared to C-IUGR; IRS-1 compared to C-IUGR and EC, and IRS-2 compared to EC. In IUGR intestine, increases were detected in IRβ, IRS-1 and IGF-IRβ compared to C-IUGR and EC. In IUGR kidneys, increases were observed in IRβ and IGF-IRβ compared to C-IUGR and EC, and IRS-1 compared to EC. Decreased IRS-2 in the intestine and kidney were noticed in IUGR compared to C-IUGR and EC. Conclusion: IUGR fetuses had less glomeruli and alterations in insulin receptors, which may be associated with an increased risk of disease occurrence in adulthood. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Exposure of the spinal cord in myelomeningocele (MM) throughout gestation increases spinal injury. Astrocyte activation evidenced by glial fibrillary acidic proteins (GFAP) indicates the extent of injury. Corticosteroids modulate GFAP synthesis, but their effect in MM is unclear. The purpose of this study was to evaluate the GFAP expression in a fetal rat model of dysraphism and the effect of corticosteroid treatment on this marker and on clinical neurological disabilities. Methods: Dysraphism was surgically created in 2 groups of 48 rat fetuses; group 1: control, and group 2: treated with corticosteroid. Each group was subdivided into fetuses with surgically created MM, controls and shams on day 18.5 of gestation (term = 22 days). Fetuses were harvested on day 21.5, examined for evidence of neurological deficits, and the following clinical parameters were registered: kyphosis, tail deformities, leg deformities, leg paralysis or paresis and pain perception. The fetuses were fixed for GFAP immunostaining. Results: All fetuses with MM in group 1 presented neurological deficits and glial reactions with GFAP expression, as opposed to controls and shams. In group 2, corticosteroid treatment prevented some neurological deficits (18–25%), reducing glial response and GFAP expression. Conclusions: Experimentally induced dysraphism in the rat fetus is related to glial response and increased GFAP expression in the spinal cord. Corticoid treatment clinically improved nerve injury in some fetuses. It reduced glial reaction and GFAP expression. Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Amniotic fluid (AF) and its components, such as fetal urine and meconium, may lead to intestinal alterations in gastroschisis, which cause immaturity of the myenteric plexus and consequent intestinal hypomotility and malabsorption. In this study we identified morphological and histological alterations of the intestine and the myenteric plexus with two different times of exposure to AF. Methods: The experimental gastroschisis was achieved at two different gestational ages, on day 18.5 (E18.5) and day 19.5 (E19.5) of gestation, in fetal rats which were divided into 3 subgroups: control, sham and gastroschisis. We measured fetal body weight (BW), intestinal weight (IW) and intestinal length (IL). The layers of intestinal wall and myenteric plexus were evaluated by hematoxylin and eosin staining (HE staining) and immunofluorescence (α-internexin), respectively. Results: BW was not significantly different among the control, sham and gastroschisis groups at both ages. IW and IL were larger and shorter, respectively, in the gastroschisis fetuses (p < 0.001) at both ages. Intestinal diameters and wall layers presented significant differences among control, sham and gastroschisis fetuses at both ages (p < 0.001), but the time of exposure to AF compromised the serous membrane, D-II (diameter II, p < 0.001) and IL (p = 0.001). α-Internexin presented more intensive immunoreactivity in gastroschisis fetuses at E18.5. Conclusions: In gastroschisis, the longer the time of exposure to AF, the more severe bowel impairment will be, especially with regard to IL and the serous layer, and the more immature the myenteric plexus will be. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objectives: To describe the molecular analysis through comparative genomic hybridization (CGH) of fetuses with gastroschisis, and to observe if this technique could improve the resolution of the conventional cytogenetic techniques. Methods: Amniotic analysis of fetuses with gastroschisis, using both conventional (G-banding) and molecular (CGH) cytogenetics assays. Results: All of the seven fetuses studied displayed a normal G-band karyotype. Six fetuses displayed a normal disomic profile through CGH and one sample has displayed ish cgh enh 3q26→qter result (ICSN). The fetus with this imbalance of chromosome 3 was re-classified as a ruptured omphalocele, instead of gastroschisis, after birth. Conclusions: The molecular investigation through CGH technique can improve the resolution of the conventional karyotye analysis in cases of abdominal wall defects. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Fetuses with digestive anomalies such as gastroschisis may present intrauterine growth restriction (IUGR) and shortened intestines. Objective: The aim of this study was to assess the influence caused by amniotic fluid (AF) in intestinal length and somatic growth in an experimental gastroschisis fetal model at two distinct gestational ages. Material and Method: Fetal rats were operated according to Correia-Pinto on 2 different days of gestation: day 18.5 (group I) and day 19.5 (group II). Each group was divided into three sub-groups: fetuses with gastroschisis (G), control (C) and sham(S). Body measurements and histological analysis were done. Result: Body measurement analysis showed: average body weight (g) in group I was G = 5.32, C = 5.68, S = 5.86; group II was G = 5.32, C = 5.80, S = 5.66. Average intestine weight (g) in group I was G = 0.283, C = 0.238, S = 0.231; group II was G = 0.272, C = 0.231, S = 0.233. Average intestine length (mm) in group I was G = 125, C = 216, S = 209; group II was G = 148, C = 226, S = 226. Histological analysis showed a decrease in the number and size of the intestinal microvillae and a light edema of serosa. Conclusion: Gastroschisis had a direct correlation with IUGR and the time of exposure of the fetuses to AF had no influence on body weight in gastroschisis fetuses but did interfere with intestinal length. Copyright © 2005 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Dysraphism is a defect in neural tube development, leading to dysplastic growth of the spinal cord and meninges. Myelomeningocele (MM) is just one of its forms. Hydrocephalus is among the most important alterations in MM and occurs as a consequence of Arnold-Chiari malformation (AC). Experimental models have been developed in sheep, rabbits and rats to study MM physiopathology, allowing a more detailed evaluation of clinical parameters involved in this anomaly. Objective: Using the experimental model of dysraphism in fetal rats, the aim of this study was to evaluate the relevance of AC malformations, clinical parameters and grade of histological lesions. Materials and Methods: Three groups with 16 fetuses in each were compared, MM, Control and Sham, after intrauterine surgical creation of MM on day 18.5 of gestation (term = 22 days). AC was evaluated by photographic comparison of sagittal cuts of fetal heads. Clinical and histological evaluations were also made. Results: 88% of AC (14/16) in MM fetuses were obtained, besides 100% of clinical alterations. Necrosis and erosion of the spinal cord exposed to amniotic fluid were verified in histology. Conclusion: The presence of AC in the dysraphism rat model was high. These results allowed the use of this model to study alterations and intrauterine evolution of MM in a fashion similar to those observed in humans. Copyright © 2005 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Myelomeningocele (MM) is a congenital neural tube defect with serious consequences, including hydrocephaly. An important hope for intrauterine repair is that hydrocephaly may be prevented by reversing the Arnold-Chiari malformation. Three medical centers in the United States are doing trials with this objective. We describe an intrauterine correction of MM in a Brazilian research center of fetal medicine, which resulted in abruptio placentae and fetal death, to illustrate factors that influence fetal-maternal risks during this surgical procedure. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]