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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 15 (1999), S. 192-194 
    ISSN: 1437-9813
    Keywords: Keywords Internal anal sphincter achalasia ; Innervation ; PGP 9.5 ; Synapse ; Synapsin I ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Internal anal sphincter achalasia (IASA) is a condition with a clinical presentation similar to Hirschsprung's disease, but with the presence of ganglion cells on rectal biopsy. The diagnosis of IASA is made on anorectal manometry, which demonstrates the absence of a rectosphincteric reflux on rectal balloon inflation. In order to understand the nature of neuronal abnormalities in this condition, we performed immunohistochemistry using PGP 9.5 (a general neuronal marker) and synapsin I (a presynaptic marker) in IAS specimens from 10 patients with IASA and 8 normal controls. In the IAS of normal controls, there were many PGP 9.5 and synapsin I-positive nerve fibers. In IASA PGP 9.5-immunoreactive fibers were markedly reduced and synapsin I-positive fibers were either absent or markedly reduced. Our findings demonstrate that the IAS in achalasia patients has defective intramuscular innervation as well as defective innervation of the neuromuscular junction, thereby contributing to the motility dysfunction.
    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
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 13 (1998), S. 243-252 
    ISSN: 1437-9813
    Keywords: Key words Infantile hypertrophic pyloric stenosis ; Pathogenesis ; Innervation ; Extracellular matrix proteins ; Growth factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Although infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring surgery in the first few months of life, its pathogenesis is not fully understood. Reviews of the recent progress in the pathogenesis of IHPS show: (1) there is increasing evidence to suggest that smooth-muscle cells in IHPS are not properly innervated; (2) because non-adrenergic, non-cholinergic nerves are mediators of smooth-muscle relaxation, it is likely that the absence of these nerves in pyloric muscle is the cause of excessively contracted hypertrophic circular pyloric muscle; (3) there are abnormal amounts of extracellular matrix proteins in hypertrophic pyloric muscle. Circular muscle cells in IHPS are actively synthesizing collagen, and this may be responsible for the characteristic “firm” nature of the pyloric tumor; and (4) the increased expression of insulin-like growth factor-I, transforming growth factor-ß1, and platelet-derived growth factor-BB and their receptors in hypertrophic pyloric muscle suggests that increased local synthesis of growth factors may play an important role in smooth-muscle hypertrophy in IHPS.
    Type of Medium: Electronic Resource
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