Histologic studies of rectocolic aganglionosis and allied diseases

Munakata, Keimei; Okabe, Ichiro; Morita, Ken

doi:10.1016/s0022-3468(78)80215-2

Cited by 48 publications

(20 citation statements)

References 11 publications

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“…The size of Auerbach plexus is also small ( Table 2). These findings are consistent with those of bhypogenesisQ of the ganglion cells [5]. Chronologically, the diameter of the nucleus of ganglion cells increased over time, but their numbers did not increase at all (Figs.…”

Section: Congenital Hypoganglionosis (N = 7)supporting

confidence: 90%

New classification of hypoganglionosis: congenital and acquired hypoganglionosis

Taguchi¹,

Masumoto²,

Ieiri³

et al. 2006

Journal of Pediatric Surgery

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Section: Congenital Hypoganglionosis (N = 7)supporting

confidence: 90%

New classification of hypoganglionosis: congenital and acquired hypoganglionosis

Taguchi¹,

Masumoto²,

Ieiri³

et al. 2006

Journal of Pediatric Surgery

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“…The intensity of AChE staining appears to increase with increasing age [24]: In one reported series, abnormal AChE activity was identified in the lamina propria in only 83% of infants with HSCR presenting under 3 months of age, whereas the majority of those over 1 year of age demonstrated the classical AChE staining pattern associated with HSCR [17]. Therefore, the absence of an AChE reaction in neonates (within the first 3 weeks after birth) does not exclude HSCR [32,71].…”

Section: Problems In Interpreting Ache Histochemistrymentioning

confidence: 94%

“…The concept of a dysplastic ENS in normally ganglionated bowel is supported by other evidence of anatomical ENS abnormalities in the proximal ganglionated bowel in HSCR, observed by silver staining [71] or other immunocytochemical techniques [95]. In addition, the The main focus of the current debate surrounding IND is whether the features associated with IND refer to a descriptive histological appearance rather than a distinct clinicopathological entity [54,88] and what the significance of these findings is on postoperative anorectal function.…”

Section: Problems In Interpreting Ache Histochemistrymentioning

confidence: 99%

Acetylcholinesterase in Hirschsprung?s disease

Moore

Johnson

2005

Ped Surgery Int

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The association between the congenital absence of colonic ganglion cells and an increased acetylcholinesterase (AChE) expression in the affected tissue is of diagnostic importance in Hirschsprung's disease (HSCR). Investigation of AChE's function in development may also help unravel some of the complex pathophysiology in HSCR. Normal nerves do not stain for AChE, but increased AChE expression is associated with the hypertrophied extrinsic nerve fibres of the aganglionic segment in HSCR. Although a high degree of histochemical diagnostic accuracy exists, results are not always uniform, and false positives and false negatives are reported. False negative results are primarily related to age, and an absence of AChE reaction does not exclude HSCR in neonates within the first 3 weeks after birth. AChE staining results may lack uniformity, resulting in a number of technical modifications that have been made to improve standardization of AChE staining. At least two distinct histological patterns are described, types A and B. The interpretation of increased AChE staining patterns in ganglionated bowel at the time of surgical pull-through remains a problem in patients with HSCR. The development of rapid staining techniques has helped to identify normal ganglionated bowel with greater certainty. The presence of fine AChE neurofibrils in the ganglionated segment has contributed to the debate surrounding intestinal neuronal dysplasia. Quantitative assay of cholinesterase activity confirms the pattern of histochemical staining. AChE is particularly increased in relation to butrylcholinesterase, with one molecular form, the G4 tetrameric form, predominating. It is likely that the raised levels of AChE in aganglionic tissue are the transcriptional consequence of the abnormalities in signalling molecules that characterize HSCR. Evidence suggests that this AChE is functioning in a nonenzymatic capacity to promote cell adhesion and differentiation and that the hypertrophied nerves and neurofibrils may be the result of this increased AChE expression.

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“…That pattern of abnormal innervation contrasts with classical Hirschsprung disease in which the ganglion cells end abruptly and the aganglionic zone extends from that level to the anal margin. Although there are reports of hypoganglionosis or NCD in humans [Fadda et al, 1987;Munakata et al, 1978;Puri et al, 1977;Kadair et al, 19771 and mice [Bielschowsky and Schofield, 1962;Bolande and Towler, 1972;Jacobs-Cohen et al, 19871, this appears to be the only report of abnormal aggregation of melanocytes in humans with this syndrome.…”

Section: Discussionmentioning

confidence: 87%

Piebaldism‐Waardenburg syndrome: Histopathologic evidence for a neural crest syndrome

Kaplan

Chaderévian

Opitz³

et al. 1988

Am. J. Med. Genet.

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Piebaldism, an autosomal dominant trait, is characterized by patchy hypopigmentation of the face, anterior chest, abdomen, and limbs, heterochromia/bicolored irises, congenital megacolon, and deafness. A 4-month-old Inuit (Eskimo) boy with these manifestations also had left pulmonic artery stenosis, ocular ptosis, and unilateral duplication of the renal collecting system. Evidence is presented for both qualitative and quantitative derangement of neural crest derivatives in this syndrome. Histologically, hypoganglionosis, hyperganglionosis, and ectopic ganglia in lamina propria (neuronal colonic dysplasia [NCD]) were documented in the rectum. The appendix, proximal to the clinical transition zone, showed similar dysplasia. In the hypopigmented skin, multiple microscopic sections were devoid of melanocytes, with no melanin in adjacent basal cells. The hyperpigmented skin contained melanin throughout the basal layer, but the melanocytes were unevenly distributed. Most tissues affected in this boy are of neural crest origin; pathogenesis could be due to faulty migration along the established pathways involving either the borders (basal laminae) or the components of the extracellular matrix (fibronectin, cytotactin, laminin, glycosaminoglycans, and collagen). The similarities between piebaldism and the Waardenburg syndromes are discussed.

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Histologic studies of rectocolic aganglionosis and allied diseases

Cited by 48 publications

References 11 publications

New classification of hypoganglionosis: congenital and acquired hypoganglionosis

New classification of hypoganglionosis: congenital and acquired hypoganglionosis

Acetylcholinesterase in Hirschsprung?s disease

Piebaldism‐Waardenburg syndrome: Histopathologic evidence for a neural crest syndrome

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