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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 15 (1999), S. 192-194 
    ISSN: 1437-9813
    Schlagwort(e): Keywords Internal anal sphincter achalasia ; Innervation ; PGP 9.5 ; Synapse ; Synapsin I ; Immunohistochemistry
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 13 (1998), S. 243-252 
    ISSN: 1437-9813
    Schlagwort(e): Key words Infantile hypertrophic pyloric stenosis ; Pathogenesis ; Innervation ; Extracellular matrix proteins ; Growth factors
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 13 (1998), S. 464-467 
    ISSN: 1437-9813
    Schlagwort(e): Key words Hirschsprung's disease ; Hypoganglionosis ; Intestinal neuronal dysplasia ; Suction rectal biopsy ; NADPH-diaphorase histochemistry
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric surgery international 10 (1995), S. 459-464 
    ISSN: 1437-9813
    Schlagwort(e): Intestinal neuronal dysplasia ; Hirschsprung's disease ; Acetylcholinesterase staining ; Diagnostic criteria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The incidence of isolated intestinal neuronal dysplasia (IND) has varied from 0.3% to 62% of all suction rectal biopsies in different centres. The uncertainty regarding the incidence has resulted from the considerable confusion regarding the essential diagnostic criteria. In an attempt to clarify the diagnostic criteria for IND, we examined biopsy material from the following three groups using acetylcholinesterase (AChE) histochemistry: (1) full-thickness normal colon from 23 controls; (2) suction rectal biopsies from 9 patients who had isolated IND; and (3) full-thickness biopsies from 10 patients with Hirschsprung's disease (HD) who demonstrated IND in the proximal margin of the resected segment. Our data show that hyperganglionosis is the most consistent finding in both IND associated with HD and isolated IND. Other histochemical criteria of IND were dependent upon whether the biopsy was full-thickness or a suction rectal biopsy. Where full-thickness biopsies were available, giant ganglia and ectopic ganglion cells were seen in all cases. Increases in AChE-positive nerve fibres in the mucosa was a frequent finding in patients with IND diagnosed by suction rectal biopsies. We recommend that patients suspected to have IND on suction rectal biopsy should have a full-thickness biopsy for detailed examination of the submucous and myenteric plexuses.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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