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  • Congenital diaphragmatic hernia  (1)
  • Electron-microscopy  (1)
  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 4 (1989), S. 326-331 
    ISSN: 1437-9813
    Schlagwort(e): Hirschsprung's disease ; Electron-microscopy ; Schwann cells ; Monoclonal antibodies
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The pathophysiology of Hirschsprung's disease is not fully understood. Using light microscopy we have previously demonstrated the absence of a unique Schwann-cell antigen in the circular muscle of aganglionic colon identified by D7 monoclonal antibody. In an attempt to characterise the morphological changes in neuronal cells at subcellular level, we studied innervation patterns in normal and aganglionic colon by electron microscopy. The most striking observation on ultrastructural serial examination of the entire resected specimen of colon from patients with Hirschsprung's disease was the presence of grossly swollen monoaxonal or oligoaxonal Schwann cell units with loss of cellular contents in the circular muscle of aganglionic colon. The extent of subcellular changes in Schwann cells and axons corresponded with a diminution of immunoreactivity with a panel of neuronal cell antibodies. These ultrastructural findings suggest that degenerative changes in Schwann cells and axons within the circular muscle coat of aganglionic segment may be a significant factor in the pathogenesis of Hirschsprung's disease.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 2 (1987), S. 327-330 
    ISSN: 1437-9813
    Schlagwort(e): Congenital diaphragmatic hernia ; Incidence ; Mortality ; Associated anomalies ; Prenatal correction
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Advances in ultrasonography have made it possible to accurately diagnose congenital diaphragmatic hernia (CDH) prenatally, and recently interest has been expressed in in-utero correction of CDH as a solution to the problem of high mortality. In an attempt to elucidate the natural history of CDH, we analysed the incidence and mortality of this disorder in a large maternity hospital with an autopsy rate of 100% for all stillbirths and neonatal deaths. Between 1973 and 1985, there were 47 cases of CDH among 99,062 births, an incidence of 1 in 2,107 birhts. There were 15 (32%) stillbirths and 32 (68%) live births. All 15 stillborns had lethal associated non-pulmonary anomalies. Of the 32 liveborn patients, 17 died prior to transfer to the referral centre and 11 of these had major associated anomalies. Prematurity was a feature in 65% of patients who died prior to transfer to the referral centre. Nine of the 15 patients who arrived at the referral centre survived. Our date suggest that the only patient who may benefit from prenatal correction of CDH is a fetus who has serious persistent pulmonary hypertension. However, in the absence of reliable criteria for the in-utero prediction of persistent pulmonary hypertension in association with CDH, surgical correction of CDH prenatally is not practicable at present even for this group of patients. Newer therapeutic approaches in the management of persistent pulmonary hypertension should be addressed as the most promising approach in the management of a CDH patient who develops respiratory failure in the first few hours of life.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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