China
Africa
Explore chapters and articles related to this topic
Hirschsprung disease
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Matthew W. Ralls, Arnold G. Coran
Typically, the diagnosis of Hirschsprung disease can be suspected based on the history alone, but confirmation is required by histopathologic examination of a rectal biopsy. The infant should receive rectal washouts, and broad-spectrum IV antibiotics should be given just prior to the incision. No formal bowel preparation is required.
Pediatric colorectal surgery in low- and middle-income settings: Adaptation to the resources available
Published in Alejandra Vilanova-Sánchez, Marc A. Levitt, Pediatric Colorectal and Pelvic Reconstructive Surgery, 2020
Chris Westgarth-Taylor, Marion Arnold
Hirschsprung disease: Anastomotic (mucosal) stricture post Hirschsprung endorectal pull-throughObstructive symptoms despite patency after ganglionated endorectal pull-through
The gastrointestinal tract
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
The underlying cause of Hirschsprung disease should be considered. It may be (1) chromosomal, in about 12% of cases, with the most frequent single condition being Down syndrome. Then (2) there are the monogenic, syndromic forms. Hirschsprung disease is a significant complication of Mowat-Wilson syndrome and FG syndrome and is also a feature of Bardet-Biedl syndrome, congenital central hypoventilation, cartilage hair hypoplasia, Goldberg-Shprintzen syndrome and Waardenburg syndrome type 4 (among others). (3) There are the monogenic non-syndromic forms, associated especially with variants of low penetrance in the RET proto-oncogene but variants in other genes can be associated (including EDN3 and EDNRB). Pathogenic variants in RET act as a Mendelian predisposition to the condition but are not sufficient to cause it. Then, finally (4) there are those cases that are non-syndromic but without a recognised molecular predisposition.
Sigmoid volvulus: a rare but unique complication of enteric fever
Published in Journal of Community Hospital Internal Medicine Perspectives, 2020
Muhammad Sohaib Asghar, Abubakar Tauseef, Hiba Shariq, Maryam Zafar, Rumael Jawed, Uzma Rasheed, Mustafa Dawood, Haris Alvi, Saad Aslam, Marium Tauseef
The mesentery of the sigmoid colon is different in a way that it is more elongated in length but tapered at the base, which increases the chance of twisting of Sigmoid Colon as compare to other bowel segments [1,4]. Overall it is less likely to be found in females, but during pregnancy, gravid uterus occupies more space in the pelvis, causing outward pushing of the sigmoid colon in the abdominal cavity, which explains the increasing incidence of sigmoid volvulus in pregnancy [1]. The acquired causes of sigmoid volvulus mainly rely on conditions that cause an increase in its span, like chronic constipation, infections, neuropsychiatric disorders, and electrolyte abnormalities [5]. Intra-abdominal adhesions make torsions more easily which can be acquired secondary to surgery, injury, and infections [6]. Hirschsprung Disease, i.e. congenital absence of ganglions in the bowel segment, most commonly in the colon, causes dilatation and elongation of proximal bowel thus increase the chance of volvulus [7].
Prospective study reveals a microbiome signature that predicts the occurrence of post-operative enterocolitis in Hirschsprung disease (HSCR) patients
Published in Gut Microbes, 2020
Weibing Tang, Yang Su, Chen Yuan, Yuqing Zhang, Lingling Zhou, Lei Peng, Pin Wang, Guanglin Chen, Yang Li, Hongxing Li, Zhengke Zhi, Hang Chang, Bo Hang, Jian-Hua Mao, Antoine M. Snijders, Yankai Xia
Hirschsprung disease (HSCR) is a birth defect caused by a congenital absence of ganglion cells (aganglionosis) in the distal colon, which extends for varying distances into the more proximal colon but rarely involves the small intestine.1–3 Consequently, varying lengths of the distal colon are unable to relax, causing functional colonic obstruction over time. While HSCR can be treated by a pull-through surgery, HSCR patients are at high risk of developing enterocolitis before or after the surgery, with classic manifestations including fever, abdominal distention, diarrhea, and sepsis. Hirschsprung-associated enterocolitis (HAEC) is the leading cause of serious morbidity and mortality in HSCR patients.4
Disruption of the network between Onuf’s nucleus and myenteric ganglia, and developing Hirschsprung-like disease following spinal subarachnoid haemorrhage: an experimental study
Published in International Journal of Neuroscience, 2019
Ozgur Caglar, Binali Firinci, Mehmet Dumlu Aydin, Erdem Karadeniz, Ali Ahiskalioglu, Sare Altas Sipal, Murat Yigiter, Ahmet Bedii Salman
We used the method of Zanesco et al. [25], and found that the neuron density of both Onuf’s nucleus and myenteric ganglia had been decreased in spinal SAH group. This changes were characteristic and resembles the Hirschsprung disease. Another interesting finding of the present study, Hirschsprung-like histopathological findings may be less in males (our unpublished data), because the innervations of female rat’s intestines by both Onuf’s nucleus network and vagal nerves, but not males [26].