Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.3.5.1 (succinate dehydrogenase)
 8,177 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this study was to review critically the diagnostic features of intestinal neuronal dysplasia type B (IND B). Over a period of 5 years colonic mucosal biopsies of 773 children with symptoms of chronic constipation were examined. Four biopsies taken 2-10 cm above the pectinate line were cut in serial sections and histochemical lactate dehydrogenase, succinate dehydrogenase, (SDH) and acetylcholinesterase (AChE) reactions performed. Presence of giant ganglia of the submucosal plexus, being characterized by more than seven nerve cells, established the diagnosis of IND B. Giant ganglia were found to be age-independent changes, while hyperplasia of the submucosal plexus, increase of AChE activity in nerve fibres of the lamina propria and low SDH activity in nerve cells proved to be age-dependent findings which disappear during the maturation of the enteric nervous system. Using these criteria IND B was diagnosed in 209 children. In 64 of these patients a combination of IND B and aganglionosis (Hirschsprung's disease) was found. IND B seems to be related to premature expression of laminin A during embryogenesis, resulting in premature nerve cell differentiation in the myenteric and submucosal plexus, which in turn blocks neuroblast colonization of the rectum. IND B, hypoganglionosis and aganglionosis, which are often combined, may therefore be considered to be different manifestations of the same developmental abnormality.
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PMID:Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B) 754 72

In colonic motility disorders, a pathohistological diagnosis based solely on formalin-fixed gut is often inconclusive. Classical histological techniques or immunohistochemistry represent a static staining. In contrast, native tissue submitted to enzyme histochemistry provides functional information about the effectiveness of the cellular performance. Routinely, a complementary set of reactions is performed and includes acetylcholinesterase (AChE), lactic and succinic dehydrogenase, as well as nitroxide synthase reactions. In this monograph, the whole spectrum of different anomalies of the colonic wall is illustrated in a systematic fashion: Hirschsprung's disease is characterized by an increase in AChE activity of parasympathetic nerve fibers of the rectosigmoid. In ultrashort Hirschsprung's disease, only enzyme histochemistry renders a reliable diagnosis possible in biopsies of the anal ring. Aganglionosis of the musculus corrugator cutis ani shows a localized increase of AChE activity in nerve fibers, similar to Hirschsprung's disease, not detectable in conventional histology. Immaturity, hypoganglionosis and neuronal dysganglionosis can be clearly recognized in dehydrogenase reactions. Enzyme histochemical reactions are complemented by picrosirius red staining for assessment of the collagen texture of the muscularis propria. Absence or intertenial interruption of the continuous connective tissue layer between circular and longitudinal muscle of the muscularis propria has been termed aplastic or atrophic desmosis, respectively. Many of the entities described are also observed in adults. Atrophic hypoganglionosis or atrophic desmosis with loss of the myenteric plexus connective tissue fascia is implied as a frequent cause of chronic constipation in adults. The essential contribution of a functional histopathological technique towards a reliable diagnosis of gut dysfunction in native tissue is extensively demonstrated in great detail in more than two hundred figures.
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PMID:Pathology of chronic constipation in pediatric and adult coloproctology. 1590 1