Review
Microbiology
Sireekarn Chantakhow, Jiraporn Khorana, Kanokkan Tepmalai, Nonglak Boonchooduang, Nipon Chattipakorn, Siriporn C. Chattipakorn
Summary: Hirschsprung disease (HSCR) is commonly associated with a life-threatening complication known as Hirschsprung-associated enterocolitis (HAEC), related to gut dysbiosis. Studies have shown controversial changes in alpha diversity of microbiota in HAEC patients, but potential improvement through probiotics. Furthermore, differences in microbiota between proximal ganglionic and distal aganglionic intestines have been observed.
Review
Biochemistry & Molecular Biology
Shuai Li, Yichun Zhang, Kang Li, Yuan Liu, Shuiqing Chi, Yong Wang, Shaotao Tang
Summary: Despite advances in understanding the pathophysiology and risk factors of Hirschsprung-associated enterocolitis (HAEC), its morbidity rate remains unsatisfactorily stable and clinical management is challenging. This literature review summarizes recent research progress on the pathogenesis of HAEC, examining 50 eligible articles obtained from various databases. The review concludes that HAEC is a multifactorial clinical syndrome and emphasizes the importance of gaining deep insights into its pathogenesis to effectively manage the disease.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, General & Internal
Konstantine Chakhunashvili, Davit G. Chakhunashvili, Eka Kvirkvelia, Eka Gozalishvili
Summary: This article presents a rare case of total colonic aganglionosis with unusual clinical, laboratory, and instrumental data. A 2-day-old Caucasian female newborn presented with reverse peristalsis, abdominal distention, and inability to pass stool. Diagnosis was achieved through ileostomy operation and full-thickness biopsy, leading to significant improvement.
JOURNAL OF MEDICAL CASE REPORTS
(2023)
Article
Surgery
Ruth A. Lewit, Laura V. Veras, Robert A. Cowles, Kathryn Fowler, Sebastian King, Eveline Lapidus-Krol, Jacob C. Langer, Christine J. Park, Fouad Youssef, Sergey Vavilov, Ankush Gosain
Summary: This study evaluated existing HAEC scoring systems and developed a new one, finding that the new system outperformed existing ones by decreasing underdiagnosis in this patient cohort.
JOURNAL OF SURGICAL RESEARCH
(2021)
Article
Genetics & Heredity
M. Erculiani, F. Poluzzi, G. Mottadelli, E. Felici, M. L. Novi, M. Caraccia, A. Grandi, S. Casella, L. Giacometti, G. Montobbio, I Ceccherini, E. Di Marco, C. Bonaretti, R. Biassoni, M. Squillario, A. Pietrantoni, V. Villanacci, A. Pini Prato
Summary: The study revealed a 10-fold higher incidence of chronic inflammation in TCSA patients, with up to 50% developing IBD-like lesions postoperatively. However, specific risk factors for prevention strategies were not identified. Recommendations include screening all TCSA patients with retrograde ileoscopy regardless of fecal markers.
ORPHANET JOURNAL OF RARE DISEASES
(2023)
Article
Pediatrics
Chuanping Xie, Jiayu Yan, Zhiyi Zhang, Wang Kai, Zengmeng Wang, Yajun Chen
Summary: This study identified long-segment aganglionosis as an independent risk factor for preoperative HAEC and found that a history of preoperative HAEC is strongly associated with the development of HAEC after Soave.
Article
Medicine, General & Internal
Raed Al-Taher, Hebah Tawfiq Daradkeh, Hiba Hadadin, Abdelrahman Obiedat, Yazan Hijazein, Laith Hijazein, Sarah Obiedat, Yazeed Hadadin, Abdel rahman Al Manasra, Hamza Alduraidi, Malik Juweid
Summary: Chronic constipation is a common complaint in children, and Hirschsprung's disease is an important pathological cause. This study compared the presentation and predictive factors for positive rectal biopsy results in Jordanian children with refractory constipation.
Article
Immunology
Isabelle Mueller, Urs Kym, Virginie Galati, Sasha Tharakan, Ulrike Subotic, Thomas Krebs, Eleuthere Stathopoulos, Peter Schmittenbecher, Dietmar Cholewa, Philipp Romero, Bertram Reingruber, Stefan Holland-Cunz, Simone Keck
Summary: Infants affected by Hirschsprung disease lack ganglia in a variable length of the colon and are prone to developing severe Hirschsprung-associated enterocolitis. Cholinergic signaling has been found to reduce inflammatory response, and the lack of cholinergic innervation correlates with higher incidence of enterocolitis. Cholinergic signals suppress the secretion of IL-8, a pro-inflammatory cytokine, in intestinal epithelial cells via the alpha 7 nicotinic acetylcholine receptor.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Wendy Yang, Jenny Pham, Sebastian K. King, Donald F. Newgreen, Heather M. Young, Lincon A. Stamp, Marlene M. Hao
Summary: Hirschsprung disease is characterized by the absence of enteric ganglia in the distal bowel. This study introduces a novel method using muscle units as a distinctive landmark to quantify the density of enteric ganglia in Hirschsprung disease patients. The muscle unit to ganglion ratio is higher in the transition zone compared to the normoganglionated region for long-segment Hirschsprung disease patients.
Article
Pediatrics
Nelson Rosen, Monica Holder
Summary: This article discusses the challenges and importance of counseling parents about Hirschsprung's disease (HD), emphasizing the need for parents to be informed and educated about their child's condition and treatment process. It covers the most common concerns parents have and provides evidence-based information. The article also highlights the complexity of long-term management of children with HD and underscores the importance of establishing a good parent-surgeon relationship.
SEMINARS IN PEDIATRIC SURGERY
(2022)
Review
Pediatrics
Ellen M. Encisco, Irene Isabel P. Lim, Cristine S. Velazco, Nelson G. Rosen, Aaron P. Garrison, Beth Rymeski, Jason S. Frischer
Summary: This study evaluated a historic cohort of patients with Hirschsprung disease (HD) to determine the incidence of Hirschsprung-associated enterocolitis (HAEC) and to assess the effect of Botox on HAEC incidence. The results showed that 49.5% of the patients experienced at least one episode of HAEC, and 2.9% of the patients received Botox injections at the time of surgery. Further research is needed to determine the impact of Botox on HAEC incidence.
JOURNAL OF PEDIATRIC SURGERY
(2023)
Article
Pediatrics
Xiaopan Chang, Shuai Li, Kang Li, Guoqing Cao, Xi Zhang, Dehua Yang, Shaotao Tang
Summary: Establishing a definitive diagnosis and initiating timely management for SSHD is challenging. Techniques such as barium enema and laparoscopic-guided multipoint biopsy play important roles in assisting diagnosis and treatment.
PEDIATRIC SURGERY INTERNATIONAL
(2021)
Article
Genetics & Heredity
Hong Zhang, Jing-Lu Zhao, Yi Zheng, Xiao-Li Xie, Li-Hua Huang, Le Li, Yun Zhu, Li-Feng Lu, Tu-Qun Hu, Wei Zhong, Qiu-Ming He
Summary: Hirschsprung disease (HSCR) is a hereditary defect characterized by the absence of enteric ganglia. IL-11 gene polymorphisms were studied in relation to HSCR and HAEC susceptibility, with results showing no association with HSCR risk in the Chinese population, but a potential link between rs8104023 and HAEC susceptibility. Further research in larger cohorts is needed for replication.
BMC MEDICAL GENOMICS
(2021)
Article
Pediatrics
Jixin Yang, Tianqi Zhu, Xiaojuan Wu, Mingfa Wei, Guo Wang, Jiexiong Feng
Summary: Colonoscopy is a safe, accurate, and timely examination for postoperative lower gastrointestinal bleeding in children with Hirschsprung's Disease. It helps diagnose the causes of hematochezia and provides accurate treatment options. All patients had uneventful recovery during the follow-up period, demonstrating the efficacy of colonoscopy in this subset of patients.
FRONTIERS IN PEDIATRICS
(2021)
Review
Gastroenterology & Hepatology
Sanne J. Verkuijl, Florian Friedmacher, Patrick N. Harter, Udo Rolle, Paul M. A. Broens
Summary: Hirschsprung's disease is a congenital disorder characterized by aganglionosis, with patients often experiencing persistent bowel dysfunction even after surgical resection. There is growing evidence of neuropathological abnormalities in HD patients, including imbalances in neurotransmitters and abnormal expression of enteric pacemaker cells.
WORLD JOURNAL OF GASTROINTESTINAL SURGERY
(2021)
Article
Surgery
Rafik Shalaby, Mohamed I. Elsawaf, Soliman Mohamad, Ashraf Hamed, Mohmed Mahfouz
JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES
(2020)
Article
Surgery
Mostafa Zain, Sameh Shehata, Ahmed Khairi, Khaled Ashour, Ahmed F. Khalil, Mohamed El-Sawaf, Mohamed Abouheba
Summary: The study found that in pediatric patients undergoing laparoscopic Nissen fundoplication, minimal esophageal dissection and preservation of the phrenoesophageal ligament can effectively prevent wrap transmigration, while wrap fixation does not provide any additional benefits.
JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES
(2021)
Article
Surgery
Rafik Shalaby, Mohamed Elsawaf, Soliman Mohamad, Ashraf Hamed, Mohamad Mahfouz
Summary: The study presented a new minimally invasive technique for laparoscopic appendectomy, known as needlescopic appendectomy (NAP), which achieved good treatment results in 48 patients with excellent cosmetic outcomes.
JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES
(2021)
Article
Surgery
Rafik Shalaby, Mohamed Negm, Mohamed El-Sawaf, Adham Elsaied, Sherif Shehata, Ashraf Hamed, Sameh Shehata, Ahmed B. Radwan
Summary: This study describes a novel technique for pediatric needlescopic inguinal hernia repair, which achieves excellent cosmetic outcomes without recurrence.
SURGICAL LAPAROSCOPY ENDOSCOPY & PERCUTANEOUS TECHNIQUES
(2022)
Article
Pediatrics
Mohamed El Ghazeery, Mohamed Elsawaf, Mohamed Ashour, Mohamed Metwaly, Mohamed Hashish
Summary: LSG surgery in obese adolescents has shown significant short-term clinical and metabolic success, with high average excess weight loss and BMI reduction, as well as improvement in fatty infiltration of the liver and other comorbidities postoperatively.
ANNALS OF PEDIATRIC SURGERY
(2021)
Article
Pediatrics
Hisham F. Salama, Mohammad G. Khirallah, Mohamed I. Elsawaf, Mahmoud A. ELafify
ANNALS OF PEDIATRIC SURGERY
(2018)
Article
Pediatrics
Hisham A. Almetaher, Mohamed I. El-Sawaf, Ashraf Elattar, Essam A. Elhalaby
ANNALS OF PEDIATRIC SURGERY
(2018)
Article
Pediatrics
Mohamed S. Hashish, Mohamed Elsawaf
ANNALS OF PEDIATRIC SURGERY
(2017)
Article
Pediatrics
Mohamed S. Hashish, Mohamed Elsawaf, Mohamed A. Moussa
ANNALS OF PEDIATRIC SURGERY
(2017)
Article
Surgery
Ariel U. Spencer, Xiaoyi Sun, Mohammed El-Sawaf, Emir Q. Haxhija, Diann Brei, Jonathan Luntz, Hua Yang, Daniel H. Teitelbaum
Article
Pediatrics
Mohamed I. El-Sawaf, Robert A. Drongowski, Jennifer N. Chamberlain, Arnold G. Coran, Daniel H. Teitelbaum
JOURNAL OF PEDIATRIC SURGERY
(2007)
Meeting Abstract
Gastroenterology & Hepatology
Hiroyuki Koga, Xiaoyi Sun, Hua Yang, Keisuke Nose, Mohamed El-sawaf, Khalil Bitar, Sita Somara, Daniel H. Teitelbaum