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  • 1
    ISSN: 1437-9813
    Keywords: Congenital diaphragmatic hernia ; Newborn ; Pulmonary hypertension ; Calcitonin gene-related peptide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A congenital diaphragmatic hernia (CDH) model was induced in pregnant rats following administration of 100 mg nitrofen. The fetuses were stored and fixed in Bouin's solution for 24 h after caesarean section at term. After fixation, the lungs were dissected out. Immunostaining of the CDH lungs and controls with rabbit anti-rat calcitonin gene-related peptide (CGRP) antibody at “optimal” and “supraoptimal” dilution levels was obtained by examining the intensity of staining with a series of dilutions of the antisera from 1: 1,000 to 1: 20,000. Supraoptimal dilution detects variations in antigen concentration that may be masked if the routine optimal dilution is used. Immunostaining of the lung by antisera to platelet-derived growth factor (PDGF) and alpha-smooth-muscle actin (ASMA) was performed to examine vascular remodelling. The number of CGRP-immunoreactive cells was significantly (P 〈0.001) greater in the lungs of CDH rats (n = 26) (0.74 +-0.19 NEB [neuroepithelial bodies]/mm2; mean +- SEM) compared with controls (n = 21) (0.30+-0.16 NEB/mm2) seen at supraoptimal dilution (1:20,000). Since CGRP is a vasodilator, this could have important implications in the development of pulmonary hypertension. The pattern of ASMA and PDGF immunostaining was similar in CDH lungs and controls, and therefore, vascular remodelling is not a feature of CDH lungs in fetuses delivered by caesarean section and not exposed to hypoxia.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 13 (1998), S. 464-467 
    ISSN: 1437-9813
    Keywords: Key words Hirschsprung's disease ; Hypoganglionosis ; Intestinal neuronal dysplasia ; Suction rectal biopsy ; NADPH-diaphorase histochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The purpose of this study was to investigate the specificity and sensitivity of NADPH-diaphorase (NADPH-D) staining in suction rectal biopsies (SRB) to determine whether it can be used as a diagnostic test for Hirschsprung's disease (HD) and related disorders. We studied SRB material in 80 patients suspected of having such disorders taken at 3, 5, and 7 cm above the pectinate line. Eight-micron sections were stained with hematoxylin and eosin, acetylcholinesterase histochemistry, and NADPH-D histochemistry. Normal biopsy specimens demonstrated strong NADPH-D reactivity in the submucosal ganglia and a large number of NADPH-D-positive fibers in the muscularis mucosae (MM). In contrast, there were no NADPH-D-positive fibers in the MM in patients with HD and hypertrophic nerve trunks stained weakly. Patients with hypoganglionosis (HYPG) demonstrated only a few NADPH-D-positive fibers in the MM and scant submucosal ganglia. Our results show that it is possible to diagnose HD and HYPG in mucosal rectal biopsies containing MM only and stained by NADPH-D histochemistry. As there is no background staining in NADPH-D histochemistry, it is easy to detect NADPH-D-positive fibers. NADPH-D histochemical staining may be an important additional technique for diagnosing HD and related disorders.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 12 (1997), S. 95-100 
    ISSN: 1437-9813
    Keywords: Congenital diaphragmatic hernia ; Fetal repair ; Persistent pulmonary hypertension ; Pulmonary hypoplasia ; Prognostic factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The first description diaphragmatic hernia appeared in 1575. In 1848, Bochdalek described congenital diaphragmatic hernia (CDH) occurring through a posterolateral defect. Successful surgical treatment of CDH in an infant was first performed in 1902, whereas the first neonate operated within 24 hours of life was reported in 1946. However, early surgery did not improve survival rates and the mortality was in the region of 50%. One reason for this was that more neonates underwent surgery who previously would have died without the repair of the CDH. Pulmonary hypoplasia and pulmonary hypertension were early recognised as important reasons for the high mortality rate. In recent years, an enormous effort has been made by research groups all over the world to describe the pathogenesis and pathophysiology of CDH, and apply these findings to clinical practice. Attempts have been made to define prognostic factors. Extracorporeal membrane oxygenation (ECMO) has produced encouraging results. Fetal surgical therapy remains an option in selected cases despite huge technical and ethical problems. Recently, several new therapeutic methods have been suggested, such as high frequency oscillatory ventilation, partial liquid ventilation, nitric oxide inhalation, surfactant therapy, and fetal tracheal ligation. However, more experience is required before the value of these approaches is clear. Despite these efforts, the mortality remains unacceptably high. The challenge for the future is to continue development of therapeutic approaches in order to improve survival of neonates with CDH.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1437-9813
    Keywords: Key words Hirschsprung's disease ; Neuromuscular junction ; Synapse-associated proteins ; Neurotransmission
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We examined immunohistochemically the expression and localisation of synapse-associated proteins, syntaxin (SNT) and synaptotagmin (STG) in the entire resected specimens of colon obtained at the time of pull-through operation from 15 patients with Hirschsprung's disease (HD) and 6 age-matched controls. Both antibodies showed a similar pattern of staining. In the normal colon and ganglionic colon from HD, there was strong reactivity in the submucous and myenteric plexuses in addition to staining of nerve fibres in the smooth-muscle layers. In the aganglionic colon, there was an absence or marked decrease in SNT and STG-positive nerve fibres in the smooth-muscle layers and in hypertrophic nerve trunks. Our data indicate that important proteins necessary for the docking of synaptic vesicles at the presynaptic plasma membrane are lost in fibres innervating the smooth muscle of HD and suggest that abnormal neurotransmission may have a role in the maintenance of muscle spasticity.
    Type of Medium: Electronic Resource
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  • 5
    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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