Children with Hirschsprung's disease have an abnormal colonic mucus defensive barrier independent of the bowel innervation status

Aslam, Adil; Spicer, R. D.; Corfield, Anthony P.

doi:10.1016/s0022-3468(97)90683-7

Cited by 39 publications

(28 citation statements)

References 31 publications

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“…Despite the advances in management of children with Hirschsprung disease, the pathogenesis of HAEC remains incompletely understood. Several authors have extensively studied the clinical [2,3,5,10,13,15], histologic [14], and immunologic [17,27] [13] reported that an age at diagnosis Hirschsprung disease of more than 1 week increased the risk of preoperative HAEC. Another study by Hackam et al [5] did not show any significant increase in postoperative HAEC with delayed diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…A subsequent study did not show significant increase in postoperative HAEC associated with delayed diagnosis [5]. Anecdotally, it is believed that the frequency of HAEC admission decreases with increasing age, and that children eventually outgrow the episodes of enterocolitis [2,17]. However, consensus is lacking on the impact of age at diagnosis on the development of enterocolitis.…”

mentioning

confidence: 98%

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Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not

Haricharan

Seo

Kelly

et al. 2008

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 98%

Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not

Haricharan

Seo

Kelly

et al. 2008

Journal of Pediatric Surgery

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“…In Bristol, Aslam et al [33] demonstrated that the total mucin turnover was significantly reduced in Hirschsprung's patients as opposed to age-matched normal controls. Although ganglionated colon demonstrated similar mucin turnover alterations, the changes were more significant in the aganglionic bowel.…”

Section: Mucinmentioning

confidence: 99%

New insights into the pathogenesis of Hirschsprung’s associated enterocolitis

Murphy

Puri

2005

Ped Surgery Int

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The management of Hirschsprung's disease (HD) has made dramatic strides over the last 20 years. Research into the embryological development and migration of ganglion cell has enabled a greater understanding of the pathogenesis of the disease. Coupled with new techniques in surgery, such as laparoscopy-assisted pull-through and the transanal pull-through, this knowledge has led to improved outcomes for children with HD. However, although our appreciation of Hirschsprung's associated enterocolitis and its aetiology has increased, there are continued references in the literature to a multitude of theories of pathogenesis. The purpose of this review is to delineate the theories and demonstrate the evidence supporting or otherwise contradicting each other.

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“…Studies of the mucus layer in HSCR have shown changes in both mucins and secreted immunoglobulin in patients with HAEC [309][310][311]. A study of mucin turnover in HSCR patients showed that development of enterocolitis was specifically related to an increase in the ratio of intracellular to secreted mucins [312].…”

Section: Hirschsprung's Diseasementioning

confidence: 99%

Exploring the role and diversity of mucins in health and disease with special insight into non-communicable diseases

et al. 2015

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Mucins are major glycoprotein components of the mucus that coats the surfaces of cells lining the respiratory, digestive, gastrointestinal and urogenital tracts. They function to protect epithelial cells from infection, dehydration and physical or chemical injury, as well as to aid the passage of materials through a tract i.e., lubrication. They are also implicated in the pathogenesis of benign and malignant diseases of secretory epithelial cells. In Human there are two types of mucins, membrane-bound and secreted that are originated from mucous producing goblet cells localized in the epithelial cell layer or in mucous producing glands and encoded by MUC gene. Mucins belong to a heterogeneous family of high molecular weight proteins composed of a long peptidic chain with a large number of tandem repeats that form the so-called mucin domain. The molecular weight is generally high, ranging between 0.2 and 10 million Dalton and all mucins contain one or more domains which are highly glycosylated. The size and number of repeats vary between mucins and the genetic polymorphism represents number of repeats (VNTR polymorphisms), which means the size of individual mucins can differ substantially between individuals which can be used as markers. In human it is only MUC1 and MUC7 that have mucin domains with less than 40% serine and threonine which in turn could reduce number of PTS domains. Mucins can be considered as powerful two-edged sword, as its normal function protects from unwanted substances and organisms at an arm's length while, malfunction of mucus may be an important factor in human diseases. In this review we have unearthed the current status of different mucin proteins in understanding its role and function in various non-communicable diseases in human with special reference to its organ specific locations. The findings described in this review may be of direct relevance to the major research area in biomedicine with reference to mucin and mucin associated diseases.

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Children with Hirschsprung's disease have an abnormal colonic mucus defensive barrier independent of the bowel innervation status

Cited by 39 publications

References 31 publications

Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not

Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not

New insights into the pathogenesis of Hirschsprung’s associated enterocolitis

Exploring the role and diversity of mucins in health and disease with special insight into non-communicable diseases

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