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Hirschsprung

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https://www.readbyqxmd.com/read/29238171/a-5-year-follow-up-study-of-neonates-with-hirschsprung-s-disease-undergoing-transanal-soave-or-swenson-surgery
#1
Jianjun Zhang, Tongsheng Ma, Yuan Peng, Guangfeng Huang, Fengli Liu
Objective: To investigate different outcomes and long-term efficacy of transanal Soave or Swenson surgery in treatment for neonates with Hirschsprung's disease (HD). Methods: In the present study, a total of 29 neonatal patients were included, with 20 patients undergoing the Soave procedure and nine patients undergoing the Swenson procedure. Data collected from the patients included basic demographics, age and weight at the time of operation, pathological typing, type of operation, operative time, blood loss, length of hospital stay, bowel function, and complications...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/29224790/increased-expression-of-semaphorin-3a-in-the-endothelin-receptor-b-null-mouse-model-of-hirschsprung-disease
#2
Naho Fujiwara, Katsumi Miyahara, Nana Nakazawa-Tanaka, Chihiro Akazawa, Atsuyuki Yamataka
PURPOSE: Semaphorins are guidance cues for developing neurons, implicated in the determination of the migratory pathway of neural crest-derived neural precursors during enteric nervous system development. Recently, it has been reported that Semaphorin 3A (SEMA3A) expression is up-regulated in the aganglionic colon in Hirschsprung disease (HD) patients, suggesting that increased SEMA3A expression may be a risk factor for HD. Thus, the aim of our study was to determine the expression of SEMA3A using Sox10-Venus mice gut...
November 14, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29223545/the-bowel-function-and-quality-of-life-of-hirschsprung-disease-patients-who-have-reached-18-years-of-age-or-older-the-long-term-outcomes-after-undergoing-the-transabdominal-soave-procedure
#3
Shun Onishi, Kazuhiko Nakame, Tatsuru Kaji, Masato Kawano, Tomoe Moriguchi, Koshiro Sugita, Keisuke Yano, Mioko Nomura, Koji Yamada, Waka Yamada, Ryuta Masuya, Takafumi Kawano, Seiro Machigashira, Motoi Mukai, Satoshi Ieiri
BACKGROUND/PURPOSE: Hirschsprung disease (HD) is considered curable, but the postoperative bowel function is not always satisfactory. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17years of age who had undergone definitive operations in childhood. METHOD: From 1984 to 2016, 110 patients with HD underwent definitive surgery at our institute. Sixty-three patients who had undergone the transabdominal Soave procedure reached 18years of age...
December 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29217941/intestinal-obstruction-in-early-neonatal-period-a-3-year-review-of-admitted-cases-from-a-tertiary-hospital-in-ethiopia
#4
Mustefa Mohammed, Tadesse Amezene, Moges Tamirat
Background: Failure to pass meconium by a full-term neonate within the first 24 hours should raise a suspicion of bowel obstruction. The objective of this study was to determine pattern of presentation, diagnosis and outcome of management of intestinal obstruction in the early neonatal period in the Neonatal Care Unit of Tikur Anbessa Specialized Hospital in Addis Ababa, Ethiopia. Methods: Retrospective chart review of admitted cases from January 2011 to December 2013 was done...
July 2017: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/29212617/diagnostic-role-of-anal-sphincter-relaxation-integral-in-high-resolution-anorectal-manometry-for-hirschsprung-disease-in-infants
#5
Jia-Feng Wu, Cheng-Hsun Lu, Chia-Hsiang Yang, I-Jung Tsai
OBJECTIVE: To investigate the possible diagnostic role of anal sphincter relaxation integral (ASRI) in high-resolution anorectal manometry (HRAM) for Hirschsprung disease. STUDY DESIGN: We performed conventional anorectal manometry (ARM) in 24 infants (8 with Hirschsprung disease and 16 without Hirschsprung disease) and HRAM in another 21 infants (9 with Hirschsprung disease and 12 without Hirschsprung disease) before and after October 2014. All infants underwent rectal suction biopsy for confirmation of Hirschsprung disease...
December 4, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29212517/is-hematoxylin-eosin-staining-in-rectal-mucosal-and-submucosal-biopsies-still-useful-for-the-diagnosis-of-hirschsprung-disease
#6
Suellen Serafini, Maria Mercês Santos, Ana Cristina Aoun Tannuri, Maria Claudia Nogueira Zerbini, Maria Cecília de Mendonça Coelho, Josiane de Oliveira Gonçalves, Uenis Tannuri
BACKGROUND: Hematoxylin-eosin (HE) staining of a full-thickness rectal wall fragment is classically used for the diagnosis of Hirschsprung disease (HD). However, this technique requires large fragments for a better diagnosis. Additionally, the histochemical and immunohistochemical methods of staining small fragments of rectal mucosal and submucosal biopsies are not available in all centers. Therefore, the possibility of diagnosing HD through HE staining in these biopsies could be a valuable alternative for centers that do not have more specific techniques...
December 6, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/29209677/laparoscopic-assisted-endorectal-pull-through-for-hirschsprung-s-disease-a-retrospective-study
#7
Xiaogang Li, Xiaoyun Li, Jun Cheng, Yongkang Zhang, Wei Zou, Fei Xie, Zhenggui Tao, Shaotao Tang, Mingguo Du, Xiaofeng Liao
OBJECTIVES: To summarize the efficacy of the laparoscopic-assisted transanal-endorectal pull-through procedure for Hirschsprung's disease (HD). METHODS: Between May 2006 and May 2013, 22 children with HD undergoing laparoscopic-assisted endorectal pull-through procedures were retrospectively analyzed. The operative time, pathology, intraoperative blood loss, recovery time for gastrointestinal function, postoperative hospital stay, complications, and defecation functions of the patients were analyzed...
December 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/29196262/collagen-vi-suppresses-fibronectin-induced-enteric-neural-crest-cell-migration-by-downregulation-of-focal-adhesion-proteins
#8
Shoichi Nishida, Hisayoshi Yoshizaki, Yoshitomo Yasui, Tsuyoshi Kuwahara, Etsuko Kiyokawa, Miyuki Kohno
The enteric nervous system (ENS) is a network of neurons and glia that are derived from enteric neural crest cells (ENCCs) and essential for regulating peristaltic activity of the colon. ENCCs migrate along the gastrointestinal tract to form the ENS, and disruption of ENCC motility leads to ENS disorders, such as Hirschsprung's disease. Previous ENCC-transplant experiments show that ENCCs can invade into isolated mouse intestines by age E13.5, but not after E15.5. We hypothesized that altered age-specific micro-environments in the intestine are responsible for ENCC invasion/migration...
November 28, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29192291/kif1bp-loss-in-mice-leads-to-defects-in-the-peripheral-and-central-nervous-system-and-perinatal-death
#9
Caroline S Hirst, Lincon A Stamp, Annette J Bergner, Marlene M Hao, Mai X Tran, Jan M Morgan, Matthias Dutschmann, Andrew M Allen, George Paxinos, Teri M Furlong, Sonja J McKeown, Heather M Young
Goldberg-Shprintzen syndrome is a poorly understood condition characterized by learning difficulties, facial dysmorphism, microcephaly, and Hirschsprung disease. GOSHS is due to recessive mutations in KIAA1279, which encodes kinesin family member 1 binding protein (KIF1BP, also known as KBP). We examined the effects of inactivation of Kif1bp in mice. Mice lacking Kif1bp died shortly after birth, and exhibited smaller brains, olfactory bulbs and anterior commissures, and defects in the vagal and sympathetic innervation of the gut...
November 30, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29190183/a-long-term-follow-up-of-a-new-surgery-method-laparoscope-assisted-heart-shaped-anastomosis-for-hirschsprung-s-disease
#10
Chunlei Jiao, Donghai Yu, Dandan Li, Guo Wang, Jiexiong Feng
BACKGROUND: Laparoscopic surgery is widely used in the treatment of Hirschsprung's disease (HD). However, constipation and soiling are still the main long-term complications. A new type of anastomosis, which is characterized by a heart-shaped colorectal anastomosis after splitting the posterior rectum wall to 0.5 cm above the dentate line, has been improved by our medical center. The purpose of this study was to determine the long-term effects of laparoscope-assisted heart-shaped anastomosis (LHSA) and to compare it with a more generally applied approach, the laparoscope-assisted Soave procedure (LSP)...
November 30, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29180902/validation-of-algorithms-to-determine-incidence-of-hirschsprung-disease-in-ontario-canada-a-population-based-study-using-health-administrative-data
#11
Ahmed Nasr, Katrina J Sullivan, Emily W Chan, Coralie A Wong, Eric I Benchimol
Objective: Incidence rates of Hirschsprung disease (HD) vary by geographical region, yet no recent population-based estimate exists for Canada. The objective of our study was to validate and use health administrative data from Ontario, Canada to describe trends in incidence of HD between 1991 and 2013. Study design: To identify children with HD we tested algorithms consisting of a combination of diagnostic, procedural, and intervention codes against the reference standard of abstracted clinical charts from a tertiary pediatric hospital...
2017: Clinical Epidemiology
https://www.readbyqxmd.com/read/29179287/interest-of-anorectal-manometry-during-long-term-follow-up-of-patients-operated-on-for-hirschsprung-s-disease
#12
Viet Q Tran, Tania Mahler, Patrick Bontems, Dinh Q Truong, Annie Robert, Philippe Goyens, Henri Steyaert
Background/Aims: Although many advances in the management of Hirschsprung's disease have recently been achieved, postoperative outcomes of these patients remain difficult in a non-negligible number of cases. Therefore, this study aims at investigating characteristics of anorectal manometry and its relationship with postoperative outcomes during long-term follow-up in Hirschsprung patients. Methods: Patients over 4 years of age operated on for Hirschsprung's disease were interviewed to complete detailed questionnaires on bowel function...
November 25, 2017: Journal of Neurogastroenterology and Motility
https://www.readbyqxmd.com/read/29170900/the-challenges-of-closing-an-ileostomy-in-patients-with-total-intestinal-aganglionosis-after-small-bowel-transplant
#13
Fereshteh Salimi Jazi, Tiffany J Sinclair, Chad M Thorson, Ricardo Castillo, Andrew C Bonham, Carlos O Esquivel, Matias Bruzoni
We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were interested in closing the ileostomy and undergoing pull-through surgery. The complexity of the case includes the presence of an aganglionic rectal segment-a short root of the mesentery due to the small bowel transplant-and significant immunosuppression...
November 23, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29162749/role-of-synaptophysin-in-the-intraoperative-assessment-of-quadrantic-innervation-of-the-proximal-doughnut-in-hirschsprung-disease
#14
Lokendra Yadav, M K Babu, Kanishka Das, Suravi Mohanty, P Divya, Gowri Shankar, Usha Kini
BACKGROUND: Symptoms may persist in a retained aganglionic segment of the colon after corrective (pull-through) surgery in Hirschsprung disease (HD). Thus, it is important to assess the proximal doughnut for innervation abnormalities intraoperatively by frozen sections stained with conventional haematoxylin and eosin stain and supported by rapid acetylcholinesterase (AChE) histochemistry. When the doughnut is proximal to the sigmoid colon, AChE is not useful and requires ratification by yet another rapid technique and hence this study...
July 2017: National Medical Journal of India
https://www.readbyqxmd.com/read/29137805/duhamel-operation-vs-transanal-endorectal-pull-through-procedure-for-hirschsprung-disease-a-systematic-review-and-meta-analysis
#15
Yong-Zhong Mao, Shao-Tao Tang, Shuai Li
OBJECTIVE: To compare treatment outcomes in children with Hirschsprung's disease who underwent treatment using the Duhamel or TERPT surgical procedures. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through December 26, 2016. Search strings included Hirschsprung's disease, fecal incontinence, transanal endorectal pull-through, and Duhamel operation. Randomized controlled studies (RCTs) and retrospective studies that compared the treatment of Hirschsprung's disease in with TERPT or Duhamel surgical procedures in neonates, infants, or children were included...
October 16, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29127965/minimally-invasive-neonatal-surgery-hirschsprung-disease
#16
REVIEW
Atsuyuki Yamataka, Go Miyano, Masahiro Takeda
Transanal pull-through (TAPT) is the procedure of choice for treating Hirschsprung disease and should be performed with laparoscopic assistance using the anorectal line (ARL) to ensure optimum postoperative bowel function (POBF). The dentate line (DL) has traditionally been used as the landmark for commencing dissection during TAPT, but we prefer the ARL because the DL is too subjective and can be associated with risk for injury to delicate sensory innervation required for normal defecation in the anal transition zone...
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29110829/hirschsprung-disease-bowel-function-beyond-childhood
#17
Tomas Wester, Anna Löf Granström
Hirschsprung disease is a developmental defect of the enteric nervous system characterized by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term outcomes indicating that impaired bowel function is common. Recently, several controlled studies show that bowel function outcomes are affected beyond childhood, in adolescents and adults, compared with healthy control subjects. Constipation and fecal incontinence are common. The impaired bowel function appears to have a negative impact on quality of life, although, a majority of patients have adapted to their symptoms...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29108502/colonic-adventitial-fibromuscular-dysplasia-a-nonspecific-arteriopathy-associated-with-hirschsprung-disease-and-other-obstructive-disorders
#18
Ameet I Thaker, Raj P Kapur
Background Smooth muscle differentiation ("adventitial fibromuscular dysplasia," AFD) was purported as specific to arteries in the transition zone of Hirschsprung disease (HSCR) patients. We investigated AFD in an HSCR population and controls and consider the pathogenesis and significance of the vascular pathology. Design Vascular histology in sections from colonic HSCR resections (n = 55) was compared with age- and site-matched controls with (n = 19) and without (n = 28) non-HSCR obstructive conditions...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29098737/structural-and-functional-differences-in-phox2b-frameshift-mutations-underlie-isolated-or-syndromic-congenital-central-hypoventilation-syndrome
#19
Simona Di Lascio, Roberta Benfante, Eleonora Di Zanni, Silvia Cardani, Annalisa Adamo, Diego Fornasari, Isabella Ceccherini, Tiziana Bachetti
Heterozygous mutations in the PHOX2B gene are causative of Congenital Central Hypoventilation Syndrome (CCHS), a neurocristopathy characterised by defective autonomic control of breathing due to the impaired differentiation of neural crest cells (NCCs). Among PHOX2B mutations, polyalanine (polyAla) expansions are almost exclusively associated with isolated CCHS, whereas frameshift variants, although less frequent, are often more severe than polyAla expansions and identified in syndromic CCHS. This paper provides a complete review of all the frameshift mutations identified in cases of isolated and syndromic CCHS reported in the literature as well as those identified by us and not yet published...
November 2, 2017: Human Mutation
https://www.readbyqxmd.com/read/29093530/sporadic-hirschsprung-disease-mutational-spectrum-and-novel-candidate-genes-revealed-by-next-generation-sequencing
#20
Zhen Zhang, Qi Li, Mei Diao, Na Liu, Wei Cheng, Ping Xiao, Jizhen Zou, Lin Su, Kaihui Yu, Jian Wu, Long Li, Qian Jiang
Hirschsprung disease (HSCR) is a common cause of functional colonic obstruction in children. The currently available genetic testing is often inadequate as it mainly focuses on RET and several other genes, accounting for only 15-20% of cases. To identify novel, potentially pathogenic variants, we isolated a panel of genes from a whole-exome sequencing study and from the published mouse aganglionosis phenotypes, enteric nervous system development, and a literature review. The coding exons of 172 genes were analyzed in 83 sporadic patients using next-generation sequencing...
November 1, 2017: Scientific Reports
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