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  • 1
    ISSN: 1437-9813
    Keywords: Key words CDH ; Heart ; Basic fibroblast growth factor ; Platelet-derived growth factor ; Polymerase chain reaction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Newborn infants with congenital diaphragmatic hernia (CDH) still have high mortality. Recently, the possible role of a cardiac maldevelopment in the high mortality has been suggested. Human and animal studies have demonstrated that heart weight is significantly reduced in the presence of CDH. Basic fibroblast growth factor (bFGF) and platelet-derived growth factor (PDGF) are pleiotropic regulatory peptides that are expressed in myocardium in precise developmental and spatial programs. PDGF and bFGF both stimulate cardiac growth by inducing cell proliferation and stimulating the synthesis of extracellular matrix. The aim of this study was to investigate the presence of heart hypoplasia in nitrofen-induced CDH in rats and the role of specific tissue growth factors (bFGF and PDGF) in its genesis. CDH was induced in pregnant rats following administration of 100 mg nitrofen on day 9.5 of gestation (term 22 days). In control animals the same dose of olive oil was given without nitrofen. Cesarean section was performed on day 21 of gestation. The fetuses were divided in two groups: normal controls (n = 8) and nitrofen-induced CDH (n = 8). Total RNA, DNA, and soluble proteins were extracted from the heart in each group and measured. mRNA was extracted from total RNA and a reverse transcription-polymerase chain reaction (RT-PCR) was performed to evaluate mRNA expression of bFGF and PDGF. The heart/body weight ratio (HBWR) and DNA content were significantly decreased (P 〈 0.01) in CDH animals compared to controls. RNA and protein content were also reduced in CDH. The expression of bFGF and PDGF mRNA was significantly reduced in the CDH group compared to controls (P 〈 0.01). The decreased HBWR, DNA, RNA, and protein content in the CDH heart indicates that the heart is hypoplastic in nitrofen-induced left CDH. The downregulation of bFGF and PDGF gene expression in the CDH heart suggests that these regulating peptides may play an important role in the genesis of cardiac hypoplasia in CDH.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 16 (2000), S. 282-284 
    ISSN: 1437-9813
    Keywords: Key words Desmin ; Infantile hypertrophic pyloric stenosis ; Immunohistochemistry ; Fetus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Recent reports indicate that extracellular matrix and cytoskeleton plasmalemmal elements are altered in infantile hypertrophic pyloric stenosis (IHPS). Desmin is a cytoskeletal protein that is important for the organization and function of muscular fibers. It has been found to be increased in the smooth muscle in chronic intestinal pseudo-obstruction and in skeletal muscle in some forms of myopathies as well as in unexplained hypertrophic cardiomyopathies. The aim of this study was to analyze the expression of desmin in IHPS. Full-thickness muscle-biopsy specimens were obtained from 8 IHPS patients (age range 23 to 41 days) at pyloromyotomy, from 8 age-matched controls without evidence of gastrointestinal (GI) disease at autopsy, and from 2 stillborns who died at 27 and 30 weeks of gestation without evidence of GI disease. Indirect immunohistochemistry was performed using the avidin-biotin-peroxidase complex method with anti-desmin and visualized by development with 3-diaminobenzidine tetrahydrochloride. Pyloric muscle in IHPS demonstrated strong desmin immunoreactivity. The expression of desmin was also strong in the muscular layers of fetal pylorus. In the age-matched controls absent or weak desmin immunoreactivity was seen in the pyloric muscle layer. The increased amount of desmin in hypertrophied pyloric muscle in IHPS may result in inco-ordination of contraction and relaxation of the pylorus, thus causing motility dysfunction. The similar pattern of desmin expression in IHPS and fetal pylorus suggests that the organization of intermediate filaments in IHPS is in a fetal stage of development.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1437-9813
    Keywords: Key words Atrial natriuretic peptide ; Congenital diaphragmatic hernia ; Hypoplastic lung ; Reverse transcription polymerase chain reaction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Atrial natriuretic peptide (ANP) plays a major role in electrolyte and volume homeostasis through potent biological effects including vasorelaxation, bronchorelaxation, lung permeability, and clearance. There are two distinct biochemical and functional classes of ANP receptors, guanylate cyclase receptor (GC-R) and clearance receptors (clearance-R). Two subtypes of GC-R have been described, GCA-R and GCB-R. Antenatal glucocorticoid therapy (AGT) has been demonstrated to improve pulmonary immaturity and abnormal structure of pulmonary arteries in animal models of congenital diaphragmatic hernia (CDH). The aim of this study was to investigate the effect of antenatal glucocorticoid administration on the ANP system in nitrofen-induced CDH hypoplastic lung in rats. A CDH model was induced in pregnant rats following administration of nitrofen on day 9.5 of gestation. Dexamethasone (Dex) was given intraperitoneally on days 18.5 and 19.5; cesarean section was performed on day 21. Reverse transcription polymerase chain reaction was performed to evaluate the relative amounts of GCA-R, GCB-R and clearance-R mRNA expression. The mRNA expression of GCA-R, GCB-R, and clearance-R was significantly increased in CDH compared to control lung. ANP receptor mRNA expression was significantly decreased in CDH lung with compared to without Dex treatment. Our finding of increased ANP receptor mRNA expression in CDH lung suggests that the hypoplastic lung has high sensitivity for ANP. Decreased mRNA expression of ANP receptors in CDH lung after Dex treatment suggests that AGT may improve pulmonary physiological function of ANP in hypoplastic CDH lung.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 16 (2000), S. 473-477 
    ISSN: 1437-9813
    Keywords: Key words Congenital diaphragmatic hernia (CDH) ; Surfactant-associated protein ; Hypoplastic lung ; Ventilation ; Oxygen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The hypoplastic lung in congenital diaphragmatic hernia (CDH) has both a quantitative and qualitative reduction in surfactant. Recently, the role of oxygen (O2) as a regulator of pulmonary surfactant-associated protein (SP) gene expression has been reported. The mRNA level of SP has been demonstrated to be increased in the lungs of animals exposed to hyperoxia. The aim of this study was to investigate SP mRNA expression in hypoplastic CDH lung in rats during mechanical ventilation in order to determine the effect of O2 on SP synthesis in CDH. A CDH model was induced in pregnant rats following administration of nitrofen. The newborn rats with CDH and controls were intubated and ventilated. Ventilation was continued for 6 h under 100% oxygen. Reverse-transcription polymerase chain reaction (RT-PCR) was performed to evaluate the relative amounts of mRNA expression of SP-A, SP-B, SP-C, and SP-D. Relative amounts of SP-A, SP-B, and SP-D mRNA expression in CDH lung were significantly decreased compared to controls at birth and 6 h after ventilation. There was no significant difference in SP-C mRNA expression between CDH animals and controls. Upregulated mRNA expression of SP-A, SP-B, and SP-D in lungs of control animals at 6 h after ventilation suggests that oxygenation accelerates postnatal SP synthesis in normal lungs. The inability of O2 to increase SP mRNA expression in hypoplastic CDH lung suggests that the hypoplastic lung is not responsive to increased oxygenation for the synthesis of SP.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1435-2451
    Keywords: Key words Gastroesophageal reflux ; Neurologically impaired children ; Fundoplication ; Thal ; Nissen ; Gastrostomy ; Pyloroplasty
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: It is difficult to give guidelines when approaching gastroesophageal disease in neurologically impaired children. Indication for surgery has been increasing over recent years, but there is no consensus on the surgical technique of choice. Nothing has been written specifically comparing the results of different procedures in these patients, so far. Study design: We retrospectively compare the short- and long-term results of two different types of fundoplication in a series of children operated on for documented gastroesophageal reflux disease at our institution. Results: One group (group A) of 27 patients, operated on between 1977 and 1993, underwent Nissen fundoplication, the other (group B), formed of 20 patients all of whom were operated on between 1993 and 1995, underwent Thal fundoplication. We compared the results in terms of positive outcome (recovery) and negative outcome (minor and major complication), computing the relative odds of group A versus group B in terms of risk of complication, and we compared the mean operative time and the length of hospital stay by means of a student's t-test analysis. Conclusions: Our results show that there is no statistical difference between the two procedures in terms of relative risk of complication and success rate. The duration of surgery and hospital stay were significantly shorter in group B. The Thal procedure can, therefore, be proposed as first choice in the management of these patients.
    Type of Medium: Electronic Resource
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