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Journal articles on the topic 'Hirschsprungs'

Author: Grafiati

Published: 3 June 2025

Last updated: 31 July 2025

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1

Kagan, Anatoly V., Aleksey N. Kotin, Svetlana A. Karavaeva, and Tamara V. Kesaeva. "Diagnostic problems of hirschsprung’s disease in neonates: clinical examples." Pediatrician (St. Petersburg) 11, no. 1 (2020): 83–90. http://dx.doi.org/10.17816/ped11183-90.

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Hirschsprungs disease usually manifests from the first days of life and is diagnosed in the newborn period. In some patients Hirschsprungs disease cant be diagnosed in the newborn period because of different forms of disease and clinical features. From 2008 to 2019 75 patients with Hirschsprungs disease were operated in the City Childrens Hospital No. 1. 21 patients had delayed diagnosis. 11 newborns didnt have very clear clinical symptoms, intestinal obstruction disappeared after decompression. 2 patients with associated chromosomal disorders were diagnosed with Hirschsprungs disease later be

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2

Faishal, Mo. "Hirschsprungs Disease (Congenital Mega-Colon)." International Journal of Science and Research (IJSR) 10, no. 4 (2021): 62–63. https://doi.org/10.21275/sr21330105243.

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3

Wasito, Dennis Chrissandy, Iskandar Ali, Fransiska Kusumowidagdo, and I. Gusti Bagus Adria Hariastawa. "The serum acetylcholinesterase (AChE) levels as an alternative diagnostic tool in pre-operative Hirschsprung's disease." Bali Medical Journal 11, no. 3 (2022): 1543–47. http://dx.doi.org/10.15562/bmj.v11i3.3610.

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Background: The golden standard of Hirschsprung's disease diagnosis is through a histopathological examination of the aganglionic site. The significance of serum acetylcholinesterase (AChE) immunohistochemistry for diagnosing Hirschsprung's disease has been widely accepted. However, the study of serum AChE levels in Hirschsprung's disease patients is still scarce. We aimed to examine the serum levels of AChE in individuals with Hirschsprung's disease. Methods: An analytical observational study with a cross sectional design was conducted on 29 patients with Hirschsprung's disease. We divided th

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4

Adelina Shannen, Felicia, Andika x, and I. Made Adi Surya. "Adult Onset Hirschsprungs Disease: A Case Report." International Journal of Science and Research (IJSR) 10, no. 2 (2021): 305–7. https://doi.org/10.21275/sr21203113747.

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5

Reece, Albert Stuart, and Gary Kenneth Hulse. "Congenital Gastrointestinal Anomalies in Europe 2010–2019: A Geo-Spatiotemporal and Causal Inferential Study of Epidemiological Patterns in Relationship to Cannabis- and Substance Exposure." Gastroenterology Insights 14, no. 1 (2023): 64–109. http://dx.doi.org/10.3390/gastroent14010007.

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Introduction: Congenital anomalies (CA’s) of most of the gastrointestinal tract have been linked causally with prenatal or community cannabis exposure. Therefore, we studied this relationship in Europe. Methods: CA data were from Eurocat. Drug-use data were sourced from the European Monitoring Centre for Drugs and Drug Addiction. Income data were taken from the World Bank. Results: When countries with increasing rates of daily cannabis use were compared with those which were not, the overall rate of gastrointestinal CA’s (GCA’s) was higher in the former group (p = 0.0032). The five anomalies w

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Pimenova, Evgeniya S., Grigoriy A. Korolev, Maxim V. Klementyev, Kulyash M. Kezhenbayeva, Olga E. Romanova, and Dmitriy A. Morozov. "“Bowel Management” program for children with congenital malformations and neurogenic bowel after surgery." Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care 11, no. 3 (2021): 325–38. http://dx.doi.org/10.17816/psaic981.

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BACKGROUND: After the surgical treatment of children for anorectal malformations, spinal pathology, and Hirschsprungs disease, their quality of life is significantly reduced due to fecal incontinence. For patients with persistent defecation disorders, the Bowel Management program is offered. AIM: This study analyzes the Bowel Management program implemented in the clinic. MATERIALS AND METHODS: A prospective analysis of the program used in children after surgical correction of malformations was conducted. The program comprised the following components: lectures for patients/parents,

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7

Schreiner, Christina, Elisabeth Ralser, Christine Fauth, Ursula Kiechl-Kohlendorfer, and Elke Griesmaier. "Genetic mutation in Hirschsprungs/congenital central hypoventilation syndrome." Journal of Pediatric Surgery Case Reports 69 (June 2021): 101861. http://dx.doi.org/10.1016/j.epsc.2021.101861.

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8

., Sam W. Moore. "Do RET and APC Crosstalk in Hirschsprungs Disease Pathogenesis?" Trends in Medical Research 3, no. 1 (2008): 31–35. http://dx.doi.org/10.3923/tmr.2008.31.35.

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9

Noviello, C., M. Romano, F. Nino, et al. "Delayed diagnosis of hirschsprungs disease after esophageal atresia repair." Journal of Pediatric Surgery Case Reports 33 (June 2018): 35–36. http://dx.doi.org/10.1016/j.epsc.2018.03.009.

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10

Gaisie, G. "Hirschsprungs disease, Ondine's Curse, and neuroblastoma — manifestations of neurocristopathy." Pediatric Radiology 20, no. 1-2 (1989): 136. http://dx.doi.org/10.1007/bf02010663.

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11

Hatti, Ramesh B., Adarsh Eshappa Setra, Bhuvaneshwari C. Yelamali, and Uday Ambi. "Extended long-segment Hirschsprungs’ disease in the Waardenburg–Shah syndrome." Annals of Pediatric Surgery 8, no. 2 (2012): 59–61. http://dx.doi.org/10.1097/01.xps.0000412307.65985.e9.

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12

Haubrich, William S. "Hirschsprung of Hirschsprung’s disease." Gastroenterology 127, no. 5 (2004): 1299. http://dx.doi.org/10.1053/j.gastro.2004.09.081.

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13

Korolev, Grigoriy A., Evgeniya S. Pimenova, and Dmitriy A. Morozov. "Application of the PedsQL 4.0 questionnaire in assessing the quality of life of children with defecation disorders after surgical treatment of congenital malformations." Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care 12, no. 2 (2022): 177–86. http://dx.doi.org/10.17816/psaic1010.

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BACKGROUND: : Fecal incontinence significantly affects the quality of life of children with anorectal malformations, Hirschsprungs disease, and spinal pathologies. AIM: This work uses the PedsQL 4.0 questionnaire to assess the quality of life of children with defecation disorders after surgical treatment of congenital malformations and evaluate its effectiveness. MATERIALS AND METHODS: Interviews were conducted with 20 families in which children aged from 17 months to 18 years were operated on for spinal hernia (50%), anorectal defects (35%), and Hirschsprung disease (15%). Parents

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14

Kurtash, Oleh. "Surgical Correction of Hirschsprung's Disease in Children Using the Soave-Boley Technique with Manual Colorectal Anastomosis." Galician Medical Journal 27, no. 4 (2020): E2020410. http://dx.doi.org/10.21802/gmj.2020.4.10.

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Introduction. Current trends in surgical treatment of Hirschsprung's disease are aimed at minimally invasive interventions. However, the experience of using Soave-Boley procedure in surgical treatment of Hirschsprung's disease in children of different ages is valuable in the arsenal of differentiated approach to the treatment of this pathology. The objective of the research was to evaluate the results of surgical correction of Hirschsprung's disease in children using the Soave-Boley technique with manual colorectal anastomosis. Materials and Methods. The analysis of surgical treatm

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15

Zeraatian, S., B. Sabet, SMV Hosseini, and HR Foroutan. "Botulinium toxin, as bridge to transanal pullthrough in neonate with Hirschsprungs disease." Journal of Indian Association of Pediatric Surgeons 13, no. 2 (2008): 69. http://dx.doi.org/10.4103/0971-9261.43025.

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16

Gholamzadeh, Saeed, Mohammad Zarenezhad, and SeyedMohammad Vahid Hosseini. "New method of transanal pull through operation in patients with hirschsprungs disease." African Journal of Paediatric Surgery 11, no. 1 (2014): 94. http://dx.doi.org/10.4103/0189-6725.129258.

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17

Li, Ji Cheng. "The development of colon innervation in trisomy 16 mice and Hirschsprungs disease." World Journal of Gastroenterology 7, no. 1 (2001): 16. http://dx.doi.org/10.3748/wjg.v7.i1.16.

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18

Alam, Muhammad Rashedul, Tarafder Mohammad Atiquzzaman, Md Hasanuz zaman, and Sumona Haque. "Retrospective Analysis of Congenital Neonatal Intestinal Obstruction in a Tertiary Care Hospital in Bangladesh." SAS Journal of Surgery 8, no. 4 (2022): 199–203. http://dx.doi.org/10.36347/sasjs.2022.v08i04.002.

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Introduction: Patients with congenital neonatal intestinal obstruction are the main bulk of neonatal surgical departments. The incidence is approximately 1 in 1500-2000 live birth. Commonly caused by anorectal malformation (ARM), intestinal atresia, hirschsprungs disease (HD), meconium ileus, malrotation of gut, volvulus neonatorum, necrotizing enterocolitis (NEC) and rarely bands adhesion. These patients presented with vomiting, gradual abdominal distension and delayed or no passes of meconium. Usually, an abdominal radiograph is all that is necessary to make the diagnosis, since the gas patt

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19

V., Odogu, O. Chukwuka I., and Chinawa N. "Waardenburg Syndrome in an 8 Year Old African Child: Case Report." British Journal of Medicine & Medical Research 21, no. 9 (2017): 1–5. https://doi.org/10.9734/BJMMR/2017/32921.

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<strong>Aim: </strong>Waardenburg syndrome is a very rare condition, inherited autosomally with genetic heterogeneity and characterized by deafness, hair discoloration, iris discoloration and eyelid changes. <strong>Case Report: </strong>We report a case of an 8 year old female child with a history of a striking difference between the eyes since birth. Ocular examination revealed slightly widened medial canthal distances and hypertelorism. There was a lateral displacement of the right inner canthi [Dystopia Canthorum]. The Iris was hypopigmented and bluish in colour in the right eye, whilst th

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20

Li, Shuai, Yichun Zhang, Kang Li, et al. "Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis." International Journal of Molecular Sciences 24, no. 5 (2023): 4602. http://dx.doi.org/10.3390/ijms24054602.

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Despite the significant progress that has been made in terms of understanding the pathophysiology and risk factors of Hirschsprung-associated enterocolitis (HAEC), the morbidity rate has remained unsatisfactorily stable, and clinical management of the condition continues to be challenging. Therefore, in the present literature review, we summarized the up-to-date advances that have been made regarding basic research on the pathogenesis of HAEC. Original articles published between August 2013 and October 2022 were searched in a number of databases, including PubMed, Web of Science, and Scopus. T

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21

Lampus, Harsani. "Overview of Hirschsprung Disease: A Narrative Literature Review." Sriwijaya Journal of Pediatrics 1, no. 1 (2023): 14–16. http://dx.doi.org/10.59345/sjped.v1i1.14.

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Hirschsprung disease is a congenital malformation resulting from the failure of neural crest cells to migrate into the gastrointestinal tract. The exact cause of this disorder is unknown, but it is suspected that there is an interaction of several factors, including gene mutations and epigenetic mechanisms. This literature review aimed to describe the pathophysiology, clinical symptoms, and treatment of Hirschprung disease. Mild to severe constipation is a common manifestation of Hirschsprung's disease with poor diet, poor weight gain, and progressive abdominal distention. However, diarrhea ma

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22

O. Paprunia, Umesh, Girija A. Patil, and Bhushan H. Mahamulkar. "MULTIPLE SYNCHRONOUS SMALL BOWEL ADENOCARCINOMAS IN A CASE OF BOWEL OBSTRUCTION DUE TO FAECALOMAS SECONDARY TO MULTIPLE STRICTURES - A DIAGNOSTIC SURPRISE." International Journal of Advanced Research 9, no. 11 (2021): 1194–98. http://dx.doi.org/10.21474/ijar01/13858.

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Small bowel adenocarcinoma(SBA)is a rare neoplasm presenting usually in elderly patients as a single tumour. Multiple synchronous SBA is unique and difficult to diagnose due to non-specific presentations.Faecalomas have been described in association with Hirschsprungs disease, psychiatric patients, Chagas disease, both inflammatory and neoplastic conditions, and in patients suffering with chronic constipation.We herein report a case of multiple faecalomas secondary to multiple small bowel strictures leading to small bowel obstruction in a patient with previous history of pulmonary tuberculosis

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23

Damayanti, Fiana, Ananda K Ramadhan, Athalita A Nabil, Hasan W Almuwaffaq, Sima Smith, and Zikrul Haikal. "HIRSCHSPRUNG’S DISEASE: METODE DIAGNOSIS DAN TATALAKSANA." Jurnal Ilmu Kedokteran dan Kesehatan 10, no. 12 (2024): 3584–94. http://dx.doi.org/10.33024/jikk.v10i12.12334.

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Abstrak: Hirschsprung’s Disease: Metode Diagnosis Dan Tatalaksana. Penyakit Hirschsprung (HSCR) atau megakolon kongenital adalah penyakit yang ditandai dengan hilangnya kemampuan dilatasi dan peristaltik usus akibat tidak adanya sel ganglion pada pleksus Myentericus (Aurbach’s) dan pleksus submukosa (Meissner). HSCR 80% didiagnosis pada tahun pertama kehidupan atau pada neonatus, dan jarang terlihat pada masa remaja dan dewasa. Diagnosis penyakit Hirschsprung harus ditegakkan sedini mungkin mengingat berbagai komplikasi yang dapat terjadi dan sangat berbahaya bagi nyawa pasien seperti konstipa

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24

Haida, M., H. Ouaya, M. Michouar, et al. "Hirschsprung's Disease in Adults, A Case Report." Scholars Journal of Applied Medical Sciences 9, no. 9 (2021): 1427–30. http://dx.doi.org/10.36347/sjams.2021.v09i09.022.

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Hirschsprung's disease (HD) is a rare congenital disease characterized by the absence of ganglion cells in the distal rectum and which is usually diagnosed in early childhood. We speak of Hirschsprung’s disease of adults in patients where the diagnosis was made after the age of 10 years, it is a rare but not exceptional condition. This should be borne in mind in young adults with a history of chronic constipation. Due to the rarity of Hirschsprung’s disease in adults, patients are rarely referred for evaluation, including rectal biopsy, further delaying diagnosis. The elements of the diagnosis

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25

Patole, Monali Madhukar, Mandar Madhukar Patole, and Subhasish Paul. "A prospective observational study of associated anomalies in hirschsprung’s disease." International Journal of Contemporary Pediatrics 7, no. 1 (2019): 112. http://dx.doi.org/10.18203/2349-3291.ijcp20195737.

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Background: Hirschsprung’s Disease (HSCR) is the commonest congenital gut motility disorder and is characterized by the absence of ganglion cells in a variable length of the distal gut. According to literature , HRSCD may be associated with a chromosomal abnormality or other congenital anomalies in approximately 20% of cases HSCR appeared to be a multifactorial malformation with low, sex dependent penetrance and variable expression according to the length of the a ganglionic segment, suggesting the involvement of one or more gene (s) with low penetrance. So far, eight genes have been found to

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26

Levitt, Marc A., Belinda Dickie, and Alberto Peña. "The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through." Seminars in Pediatric Surgery 21, no. 4 (2012): 344–53. http://dx.doi.org/10.1053/j.sempedsurg.2012.07.009.

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O H, Radhika Krishna, Srinivas Srirampur, Vani Padmaja G J, and Ramesh Reddy Kota. "Pitfalls in the histological diagnosis of total colonic aganglionosis on appendicectomy specimens." Asian Journal of Medical Sciences 13, no. 1 (2022): 99–104. http://dx.doi.org/10.3126/ajms.v13i1.39556.

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Background: Hirschsprung’s disease is the most important cause of functional intestinal obstruction in children. It is characterized by the absence of ganglion cells in the submucosal and myenteric plexuses on histology. In 10% of Hirschsprungs disease patients, involvement of the entire colon is seen in a condition called total colonic aganglionosis (TCA). The absence of ganglion cells in the appendix on histology has been considered diagnostic of TCA. The validity of this histological finding being taken as criteria for diagnosis is not clear. Aims and Objectives: This study examines the pre

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Azalia, Salsa Novela, and Yuni Ahda. "Literature Review: The Role of RET Gene Mutations in The Emergence of Hirschsprung Disease." Jurnal Biologi Tropis 24, no. 2 (2024): 37–43. http://dx.doi.org/10.29303/jbt.v24i2.6740.

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Hirschsprung’s disease is a congenital condition characterized by a failure of nerve function along the gastrointestinal (GI) tract, resulting in the absence of enteric ganglia in the most distal segment of the colon. Although the prevalence rate is 1:5000 babies born, Hirschsprung’s disease can have a significant impact on patients, especially in terms of quality of life. One of the main genetic factors often associated with the etiopathogenesis of Hirschsprung’s disease is the RET gene. This study aims to provide scientific insight into advances in the understanding of genetic variants assoc

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Moore, S. W., D. Sidler, and P. A. W. Schubert. "Segmental aganglionosis (zonal aganglionosis or “skip” lesions) in Hirschsprungs disease: a report of 2 unusual cases." Pediatric Surgery International 29, no. 5 (2013): 495–500. http://dx.doi.org/10.1007/s00383-013-3286-8.

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30

Tudorache, Simona-Gabriela, Daniela Dobrițoiu, Silvia Răgălie, Alin Stoica, and Laura-Mihaela Ion. "THE MANAGEMENT OF CHRONIC CONSTIPATION IN PEDIATRIC PATIENTS." Romanian Journal of Pediatrics 70, no. 2 (2021): 141–47. http://dx.doi.org/10.37897/rjp.2021.2.9.

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Introduction. Chronic constipation is a common reason for a pediatric patient to visit a doctor and we think that the number of cases is increasing but only a few patients are diagnosed with Hirschsprung disease. Aim. This study aims to clarify the difference between the two most common causes of chronic constipation in pediatric patients: habitual constipation and Hirschsprung’s disease regarding symptoms, clinical findings, investigations and treatment and to identify what is more important for the patients with chronic constipation: to go further with investigations after the clinical exami

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31

Honggowarsito, Yohanes Santosa, Adria Hariastawa, Fendy Matulatan, and Alphania Rahniayu. "Correlation between the number of Interstitial cells of Cajal (ICC) and defecation pattern in patients with Hirschsprung's disease after Duhamel's surgery at General Hospital Dr. Soetomo." Bali Medical Journal 12, no. 1 (2023): 675–77. http://dx.doi.org/10.15562/bmj.v12i1.4154.

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Background: Hirschsprung is a congenital disorder of the distal bowel caused by the absence of a ganglion in the Aurbach and Meissner plexuses. Definitive treatment of the disorder is operative, namely by resection of the aganglionic segment of the intestine and performing a pull-through on the ganglionic segment. It cannot be denied that there are still disturbances in the pattern of defecation after definitive surgery, even though the pull-through segment has enough ganglion cells. Interstitial cells of Cajal (ICC) are the pacemaker in smooth muscle contraction in the intestine. In several s

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Prabakaran, S., and K. Kasthuri Thilagam. "Profile of Hirschsprung’s disease cases diagnosed in tertiary care teaching hospital: a retrospective observational study." International Journal of Contemporary Pediatrics 7, no. 3 (2020): 551. http://dx.doi.org/10.18203/2349-3291.ijcp20200678.

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Background: To date, there are very few studies of Hirschsprung’s disease. Hence, the study was conducted to profile Hirschsprung’s disease in a tertiary care setting.Methods: This is a retrospective observational study that evaluated the admitted patients with Hirschsprung’s disease in the pediatric surgery department. Biopsy was taken from the patient in the form of the full thickness of intestine, seromuscular biopsy, resection from the colostomy site when doing closure and or appendix was taken and subjected to histopathological study with routine eosin and haematoxylin stain.Results: Amon

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Darmajaya, I. Made, and I. Ketut Subhawa. "Validity of Barium Enema as Hirschsprung’s Disease Diagnostic Tools for Infant in Sanglah Hospital Denpasar." JBN (Jurnal Bedah Nasional) 5, no. 1 (2021): 16. http://dx.doi.org/10.24843/jbn.2021.v05.i01.p03.

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Aim: To determine the validity (sensitivity, specificity, likelihood ratio) of barium enema as Hirschsprung's disease diagnostic tool in infants. Methods: This study was a diagnostic test of barium enema compared with postoperative histopathology examinations as the gold standard. The population were all patients with indigestion symptom be discovered at the pediatric surgical clinic of Sanglah Hospital Denpasar. The sampling method was consecutive sampling, satisfy inclusion criteria (< 12 months old, indicate classic symptoms of Hirschsprung’s disease). The total sample of the study was 5

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Anandasari, Pande Putu Yuli, and Ni Nyoman Margiani. "Characteristics of the rectosigmoid index in pediatric patients with definitive Hirschsprung’s disease in radiology installation of Sanglah General Hospital, Denpasar from January 2018 to December 2019." Bali Medical Journal 12, no. 1 (2023): 1206–9. http://dx.doi.org/10.15562/bmj.v12i1.3949.

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Background: Hirschsprung's disease is a developmental disorder of the enteric nervous system of the distal intestines. Colon in-loop examination is used as a spearhead in the diagnosis of Hirschsprung's disease, and according to previous studies, it was found that the presence of transitional zone and rectosigmoid index of less than 1 were correlated with positive pathology results in 79%-87% of cases. The purpose of this study was to provide an overview of the rectosigmoid index of patients suspected of having Hirchsprung's disease as illustrated by a colon in-loop examination. Methods: The r

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Marinchuk, A. T., E. S. Pak, T. M. Petrova, A. S. Kharkov, and O. V. Bukhtin. "Hirschsprung’s disease: current state of the problem, clinical observation." South Russian Journal of Therapeutic Practice 6, no. 2 (2025): 101–5. https://doi.org/10.21886/2712-8156-2025-6-2-101-105.

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Hirschsprung's disease (HD) (congenital agangliosis of the colon) was isolated into an independent nosological unit in 1886 by the Copenhagen pediatrician Hirschsprung. This disease is diagnosed in children and adults. To date, the etiology and mechanisms of HD disease development have been revealed, effective methods of diagnosis, surgical treatment and conservative management of patients have been developed. Treatment can significantly improve the prognosis and quality of life of patients. The clinical observation of a patient with HD who underwent surgical treatment 16 years after the disea

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Md Shariful Islam, Abu Daud, Shams ud Din Elias Khan, Sabina Yasmeen, and Julia Akhter Nira. "Death of Paediatric Surgical Patients in NICU of CMH, Dhaka." Journal of Armed Forces Medical College, Bangladesh 16, no. 1 (2021): 26–28. http://dx.doi.org/10.3329/jafmc.v16i1.53829.

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Introduction: The purpose of this study was to explore clinical characteristics and primary surgical diagnosis associated with in-hospital death in pediatric surgical patients admitted to the neonatal intensive care unit (NICU) of Combined Miilitary Hospital (CMH), Dhaka. Aim: To explore the clinical factors associated with in-hospital death in paediatric surgical patients admitted to the NICU over a period of 4 years in CMH Dhaka. Methods: This retrospective study includes all patients admitted to NICU of CMH Dhaka for paediatric surgical diseases between July 2013 and December 2017. Data ana

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Upadhyaya, Vijai Datta, Ashwani Mishra, Yusuf Md, and Basant Kumar. "Colonic stenosis in neonates is not always congenital or complication of necrotizing enterocolitis." Journal of Neonatal Surgery 9 (July 29, 2020): 7. http://dx.doi.org/10.47338/jns.v9.524.

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Background: Bowel obstruction in early infancy may results from variety of congenital anomalies involving small and large bowel. Colonic stenosis (CS) congenital or acquired is among the rare causes of chronic bowel obstructions in early infancy. Methods: Medical record of 4 patients with colonic stenosis secondary to Hirschsprung’s disease was reviewed. Results: A total of 4 cases were included in the study. Age of presentation ranged from 25 days to 96 days. Most common presentation was gross abdominal distension with visible bowel loop, vomiting, difficulty in passing stool followed by hist

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Tran Viet, Hoang, Tuan Huynh Minh, Nhan Vu Truong, et al. "Pre- and Postoperative Risk Factors for Hirschsprung-Associated Enterocolitis in Vietnamese Children." Gastrointestinal Disorders 7, no. 1 (2025): 17. https://doi.org/10.3390/gidisord7010017.

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Background/Objective: Hirschsprung-associated enterocolitis (HAEC) can occur before and after surgery, increasing the complication rates, hospital stay, and treatment costs. This study aims to determine the incidence of preoperative and postoperative Hirschsprung-associated enterocolitis HAEC and the related risk factors. Methods: This study is a prospective cohort study of Hirschsprung’s disease patients under 16 years of age at two Children’s Hospitals in Ho Chi Minh City, Vietnam from December 2022 to June 2024. The postoperative follow-up is 12 months. Results: We enrolled 84 pediatric pat

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Pavkov, Danijela Jojkić, Melanija Vislavski, Nada Vučković, et al. "Immature colonic ganglion cells as a cause of megacolon in infancy: case report." Paediatria Croatica 58, no. 3 (2014): 227–30. http://dx.doi.org/10.13112/pc.618.

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Congenital megacolon or Hirschsprung’s disease was fi rst described by Harold Hirschsprung. It is a congenital intestinal aganglionosis as a result of arrested fetal development of the myenteric nervous system. Normal intestinal motility depends on a coordinated segmental contraction waves followed immediately by smooth muscle relaxation as it propagates caudally. Patients with Hirschsprung’s disease lack functional myenteric nervous system in the aff ected distal intestine and have ineff ective distal peristalsis. The clinical outcomes are failure to pass meconium shortly after birth, constip

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Okoro Philemon E*,Onyesoh Chinyeaka. "Prolapse of colostomy in children; characteristics, predisposing factors and preventive measures in four health facilities in Southern Nigeria." Innovative Journal of Medical and Health Science 9, no. 5 (2019): 440–46. http://dx.doi.org/10.15520/ijmhs.v9i5.2595.

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IntroductionThe protrusion of the bowel through the stoma in a colostomy is one of thecommon complications of colostomy. Though it rarely gets secondarilycomplicated, it causes significant morbidity by virtue of the increasing bowel massoutside. The predisposing factors and progression in children are not well reportedin our region.AimTo evaluate the characteristics and occurrence of colostomy prolapse in childrenand to identify any factors predisposing to this complication in our practice.Patients and MethodsThis is a five year prospective study of paediatric colostomy in the authors’ service

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Falah, Erfano Muhammad. "PROFIL PENGGUNAAN ANTIBIOTIK PASIEN HAEC PASCA OPERASI DI RSUD DR. SOETOMO TAHUN 2015-2018." Jurnal Kedokteran Syiah Kuala 18, no. 2 (2018): 76–79. http://dx.doi.org/10.24815/jks.v18i2.17994.

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Abstract. HAEC (Hirschsprung-associated Enterocolitis) is one of serious and life-threatening complication of patient with HD (Hirschsprung’s Disease). The etiology of HAEC is still unknown until now, however, the existence of gram-negative bacterial especially C. Dificille has been linked to occurrence of patient with HAEC. HAEC frequently found at patient with HD post-operative surgery. The treatment for HD patient post-operative with HAEC are rectal irrigation, fluid therapy, and antibiotics. Because of the wide variety of sign and symptoms in HAEC post operative patient, under treatment ha

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De Lima, Daniel Jhonatas Silvério, Nadine Nair De Pina Tavares, Lucas dos Santos Alcantara, Lúcia Caetano Pereira, and Andrea Silva Do Amaral. "CONGENITAL CENTRAL HYPOVENTILATION SYNDROME ASSOCIATED WITH HIRSCHSPRUNG’S DISEASE." Revista de Patologia do Tocantins 5, no. 3 (2018): 32–36. http://dx.doi.org/10.20873/uft.2446-6492.2018v5n3p32.

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RESUMO A síndrome de hipoventilação central congênita (SHCC) é uma desordem genética rara, associada a mutações no gene paired-like homeobox 2B (PHOX2B), caracterizada por disautonomia e apneia central. A doença de Hirschsprung (DH) está presente em cerca de 20% dos casos de SHCC. Este estudo objetiva relatar o caso de um neonato que apresentou apneia central recorrente, eliminação tardia de mecônio e distensão abdominal diagnosticado com DH associada a SHCC, comparando-se os aspectos clínicos esperados e os encontrados pela equipe que assistiu a criança. Nota-se um retardo no diagnóstic

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Mahmud, Dr S. M. Khalid, Dr Jahanara Laizu, and Dr Rakibul Islam. "Swenson like Full Thickness Transanal Pull through for Short Segment Hirschsprung Disease: My Experience in A Tertiary Care Hospital Bangladesh." SAS Journal of Surgery 8, no. 2 (2022): 46–51. http://dx.doi.org/10.36347/sasjs.2022.v08i02.001.

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Introduction: The Swenson procedure was the original pull-through procedure used to treat Hirschsprung disease. The aganglionic segment is resected down to the sigmoid colon and rectum, and an oblique anastomosis is performed between the normal colon and the low rectum. Given our Center’s experience with a full-thickness transanal pull rectal dissection for anorectal malformations we chose to apply the ideas to the primary treatment of HD, and describe technical aspects and impact on fecal, urinary, and sexual function. Aim of the study: The aim of the study was to analyze the short term outco

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Pușcașu, Maria, Lavinia Iftene, and D. N. Păduraru. "LAPAROSCOPIC INTERVENTION IN HIRSCHSPRUNG DISEASE - MARTIN DUHAMEL METHOD." Journal of Surgical Sciences 1, no. 3 (2018): 129–34. http://dx.doi.org/10.33695/jss.v1i3.188.

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Hirschsprung's disease or congenital megacolon is a developmental disorder characterized by the absence of nerve cells in the myenteric plexus in the distal colon. The specific symptoms are caused by the absence of the peristaltic movement in the affected segment. 75% of cases involve the recto-sigmoid region, and 60% of children affected have other associated congenital diseases (ophthalmological, urogenital, cardiac or central nervous system anomalies).We are presenting the case of a newborn delivered at term who develops symptoms represented by: abdominal distension, delay in the pass of me

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Abid Muhammad Fathul Islam, Ildsa Maulidya Mar'athus Nasokha, and Amril Mukmin. "Studi Kasus Prosedur Pemeriksaan Colon in Loop pada Pasien Pediatrik dengan Kasus Hirschprungs di RSUD Brebes." JURNAL RISET RUMPUN ILMU KESEHATAN 4, no. 2 (2025): 132–51. https://doi.org/10.55606/jurrikes.v4i2.5363.

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Colon In Loop examination is an important radiographic procedure in the evaluation of Hirschsprung cases in pediatric patients. At Brebes Regional Hospital, there are differences in procedures compared to existing literature, especially in the method of administering contrast media and the projections used. This study aims to review and examine the Colon In Loop examination procedure in pediatric patients with Hirschsprung cases at Brebes Regional Hospital. This study uses a qualitative descriptive approach with a case study method. Data were collected through observation, interviews, and docu

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Abbasiasl, Hourieh, Ashrafalsadat Hakim, and Kourosh Zarea. "Explaining the Care Experiences of Mothers of Children with Hirschsprung’s Disease: A Qualitative Study." Global Pediatric Health 8 (January 2021): 2333794X2110155. http://dx.doi.org/10.1177/2333794x211015520.

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Hirschsprung’s disease is a gastrointestinal anomalies that disrupts excretion. In this disease, like other chronic diseases; mothers undergo a lot of care. Considering the concept of nursing care, it is important to understand the care and its effects on the parents, the child, and care improvement. The purpose of this study is to understand the care experiences of parents of children with Hirschsprung’s disease. In this qualitative study which was performed by content analysis approach, 12 participants were selected from mothers of children with Hirschsprung’s disease who were referred to pe

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Askarpour, S., H. Javaherizadeh, M. Peyvasteh, A. Mohammadi, and S. Soma Seyedsalehi. "Investigating the Diagnostic and Risk Factors for Enterocolitis in Children with Hirschsprung’s Disease." Acta Medica Bulgarica 52, no. 1 (2025): 48–52. https://doi.org/10.2478/amb-2025-0007.

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Abstract Aim This study aimed to identify the clinical risk factors associated with Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung’s disease (HD). Method A total of 75 children diagnosed with HD were recruited in this observational prospective study. Then, the clinical and paraclinical symptoms of children with Hirschsprung’s disease (n = 57) were compared to those with HAEC (n = 18). P-value < 0.05 was considered statistically significant. Results No significant difference was found between the two groups in terms of age (P = 0.72), gender (P = 0.51), and family

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Arun Chander Yadav, K., D. Suganya, R. Kannan, and A. Priya. "A case report on long segment hirschprung’s disease." Indian Journal of Pharmacy and Pharmacology 8, no. 3 (2021): 235–37. http://dx.doi.org/10.18231/j.ijpp.2021.041.

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Hirschsprung disease is a complex congenital condition of the intestine, usually recognized to be genetic origin and reverberates from a disturbance of the normal development of the enteric nervous system. It is evident to be the most common cause of a low intestinal obstruction in the neonates as well as the older children. Study reveals that occurrence rate of cases in 70% may be associated with other congenital abnormalities together with number of syndromic phenotypes. A number of distinct genetic sites have been identified in these syndromic phenotypes, identifying potential genetic predi

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Culnan, Stephanie, Alyssa Carlson, and Meredith L. Farmer. "Bridging Knowledge and Practice: Insights Into Hirschsprung’s Disease and Home Management." Neonatal Network 44, no. 3 (2025): 167–76. https://doi.org/10.1891/nn-2024-0067.

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Hirschsprung’s disease (HD) is a congenital intestinal motility disorder characterized by the absence of ganglionic cells in the distal intestine, leading to functional bowel obstruction and affecting 1 in every 5,000 neonates. A serious complication of HD is Hirschsprung-associated enterocolitis (HAEC), a life-threatening systemic illness responsible for a significant proportion of HD-related mortality. Proper management of HD, especially preventing HAEC, includes the use of rectal irrigations, which caregivers must master before their neonate’s discharge. However, barriers such as short NICU

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Tomuschat, Christian, Anne Marie O’Donnell, David Coyle та Prem Puri. "Reduction of hydrogen sulfide synthesis enzymes cystathionine-β-synthase and cystathionine-γ-lyase in the colon of patients with Hirschsprungs disease". Journal of Pediatric Surgery 53, № 3 (2018): 525–30. http://dx.doi.org/10.1016/j.jpedsurg.2017.06.011.

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