Literature DB >> 17161202

New classification of hypoganglionosis: congenital and acquired hypoganglionosis.

Tomoaki Taguchi1, Kouji Masumoto, Satoshi Ieiri, Takanori Nakatsuji, Junko Akiyoshi.   

Abstract

AIM: Hypoganglionosis has been associated with fewer intestinal ganglion cells. However, current reports questioned the validity of this clinical entity. The aim of this study is to demonstrate the existence of hypoganglionosis.
MATERIALS AND METHODS: We have experienced 24 cases of functional intestinal obstruction with abnormalities of the intestinal ganglia. A precise histological examination was performed using quantitative morphometric studies. These results were compared with age-matched controls (n = 13).
RESULTS: Based on histological examination, disorders in the ganglion cells could be classified into 3 categories: immaturity of ganglia (n = 13), congenital hypoganglionosis (n = 7), and acquired hypoganglionosis (n = 4). In congenital hypoganglionosis, the number as well as the size of ganglion cells are small at birth. The size of ganglion cells tends to increase over time, but their numbers do not increase; as a result, the symptoms of dysmotility do not improve. On the other hand, acquired hypoganglionosis is late onset and characterized as a degeneration of ganglion cells and gliosis histologically. After performing a resection of the affected bowel, the prognosis is usually good.
CONCLUSION: Congenital and acquired hypoganglionosis are 2 distinct entities. The histological findings as well as the clinical characteristics of these 2 types of hypoganglionosis are different.

Entities:  

Mesh:

Year:  2006        PMID: 17161202     DOI: 10.1016/j.jpedsurg.2006.08.004

Source DB:  PubMed          Journal:  J Pediatr Surg        ISSN: 0022-3468            Impact factor:   2.545


  21 in total

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10.  Anorectal manometry with reference to operative rectal biopsy for the diagnosis/exclusion of Hirschprung's disease in children under 1 year of age.

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