Search

Researchers at Nippon Medical School in Tokyo, Japan, have reported a rare case of a choledochal cyst with acute cholangitis that was diagnosed during pregnancy and treated after delivery. The patient, a 31-year-old Japanese woman, presented with right upper quadrant pain at 37 weeks' gestation. The cyst was successfully excised and biliary reconstruction was performed postpartum. The patient is now healthy with normal liver function seven years after the operation. The researchers emphasize the importance of carefully considering treatment decisions for choledochal cysts diagnosed during pregnancy to ensure good outcomes for both the mother and fetus. [Extracted from the article]

A comparative study conducted by researchers at Fujian Medical University in China examined the efficacy of two surgical procedures, robotic-assisted single-incision-plus-one port laparoscopic choledochal cyst excision (R-SILC + 1) and single-incision laparoscopic choledochal cyst (SILC), in treating pediatric choledochal cyst (CDC). The study found that the R-SILC + 1 group had advantages over the SILC group in terms of reduced bleeding volume, shorter indwelling time of the abdominal drainage tube, shorter postoperative fasting time, and shorter postoperative discharge time. However, the R-SILC + 1 group had a longer operation time and higher hospitalization costs. The researchers concluded that with improvements in physician experience and technological advancements, the potential of R-SILC + 1 will be further realized. [Extracted from the article]

A recent study conducted by researchers at Zanjan University of Medical Sciences explored the use of immunohistochemical staining to improve the diagnosis of Hirschsprung's disease (HD), a neurogenic intestinal disorder. The study involved 70 pediatric patients suspected of HD, and the researchers found that the calretinin marker exhibited a consistent accuracy of 100% in diagnosing the disease. Additionally, markers for Cajal cells showed a specificity and sensitivity of 100% in diagnosing HD. The researchers concluded that further research is needed to better understand the complexities of HD and its associated complications. [Extracted from the article]

A study conducted by researchers at the University of Utah explores the feasibility of implementing a two-stage newborn screening program for biliary atresia (BA) in an integrated healthcare system. The study found that out of 12,276 newborns eligible for screening, 98.2% underwent first-stage screening, and 95.7% underwent second-stage screening or were recommended to do so. However, no cases of BA were identified through screening, although two infants who were excluded from the study were subsequently diagnosed with BA. The researchers concluded that program infrastructure is necessary for successful implementation and sustainability. [Extracted from the article]

A recent study conducted by researchers at the University of Chile aimed to identify associated comorbidities, syndromes, and anatomical findings in newborns with esophageal atresia using bronchoscopy. The study analyzed data from 37 patients who underwent surgery for esophageal atresia or tracheoesophageal fistula between 2016 and 2023. The findings revealed that 62.1% of the patients had comorbidities, and 56.75% had airway anomalies such as tracheomalacia and laryngomalacia. The researchers concluded that performing an endoscopic airway study in all patients with esophageal atresia is crucial due to the high percentage of patients with airway anomalies. [Extracted from the article]

A recent report from Massachusetts General Hospital discusses a study on Hirschsprung Disease (HSCR), a condition that affects the digestive system. The researchers aimed to determine if transplantation of enteric neural stem cells (ENSCs) could improve gut motility and reduce inflammation in mice with HSCR. The study found that ENSC transplantation resulted in improved gut motility, reduced inflammation, and increased survival in the mice. This research suggests that ENSC transplantation may be a potential treatment for HSCR in humans. [Extracted from the article]

A recent study conducted by the University Medical Center of the Johannes Gutenberg University in Mainz, Germany, examined the physical activity levels of children with esophageal atresia (EA) and congenital heart disease (CHD). The study found that both EA and CHD were associated with reduced physical activity, particularly in females and teenagers. The researchers recommend promoting physical activity during follow-up to prevent additional morbidity associated with sedentary behavior. The study provides valuable insights into the impact of these conditions on motor development and physical activity in children. [Extracted from the article]

A recent study conducted by researchers at the Karolinska Institute in Stockholm, Sweden, explored the prevalence and risk of attention deficit/hyperactivity disorder (ADHD), autism spectrum disorders (ASD), and intellectual disability (ID) in individuals with esophageal atresia (EA). The study found that individuals with EA had a higher risk of ASD and ID compared to those without the condition. However, the risk of ADHD was not significantly increased. These findings are important for establishing follow-up programs and providing timely detection and support for children with EA, particularly before they start school. [Extracted from the article]

A recent study conducted at Ealing Hospital in London, United Kingdom, reported on an extremely rare case of mucinous adenocarcinoma originating from Meckel diverticulum (MD) in a young woman with Crohn's disease. The woman, who had a history of Crohn's disease, presented with symptoms of vomiting, abdominal pain, and constipation. A computed tomography scan revealed a small-bowel obstruction, leading to urgent surgery. The study highlights the challenges in diagnosing and treating this rare malignancy, which initially presented with symptoms similar to Crohn's disease. Further information on this research can be found in the ACG Case Reports Journal. [Extracted from the article]

A recent study conducted by Baylor University College of Medicine in Houston, Texas, explored the effectiveness of population-based screening strategies for biliary atresia (BA) in newborns. The researchers reviewed 15 studies involving over 1.8 million participants and found that assessing direct/conjugated bilirubin (DB/CB) in the first few days of life had the highest diagnostic accuracy for BA screening. The study also suggested that stool color card (SCC) screening at one month of age could be moderately effective. The researchers recommended further research on cost-effectiveness and combinations of screening strategies. [Extracted from the article]

A study conducted by researchers at the University of Geneva explores the challenges faced by clinicians when diagnosing hepatic hilar cysts in prenatal patients. These cysts can either indicate cystic biliary atresia (BA) or a choledochal cyst (CC), two conditions with different management and prognosis. The study presents four case reports and proposes a management algorithm for prenatal hepatic hilar cysts. The researchers emphasize the importance of caution when differentiating between BA and CC, as transitional types can be challenging to diagnose. [Extracted from the article]

A recent study conducted by the University of California San Francisco (UCSF) investigated the genomic alterations associated with persistent or recurrent Barrett's esophagus (BE) in patients who underwent endoscopic therapy. The study found that patients with persistent disease were more likely to have pre-treatment alterations involving the receptor tyrosine kinase pathway, amplifications of oncogenes, and deletions of tumor suppressor genes. However, these associations were no longer significant after adjusting for patient age and BE length. The study also revealed that many recurrences may represent undetected minimal residual disease. [Extracted from the article]

A recent study conducted at the National University of Medical Sciences (NUMS) in Pakistan examined the clinical parameters and outcomes of children with biliary atresia, a condition affecting the bile ducts. The study found that intervention in the form of Kasai Hepatic Portoenterostomy significantly improved outcomes for patients with biliary atresia. The research concluded that this procedure is an important factor in improving the prognosis for these patients. The study provides valuable insights into the management and treatment of biliary atresia in children. [Extracted from the article]

A new report from VMMC and Safdarjung Hospital in New Delhi, India, presents fresh data on choledochal cysts, a rare congenital disease of the biliary tract that is more common in females. The report describes a case of an 11-year-old girl with a type IV choledochal cyst that contained dual heterotropic elements, including pancreatic and splenic tissues. This is the first documented case of a choledochal cyst containing both ectopic spleen and pancreas tissue. The researchers emphasize the importance of being aware of this entity and its potential complications, such as bleeding, blockage, and malignant transformation. [Extracted from the article]

A report from Tripler Army Medical Center in Honolulu, Hawaii discusses research findings on the timing of the Kasai procedure for biliary atresia, a rare liver disease affecting children. The study used the American College of Surgeons National Surgical Quality Improvement Program Pediatric database to analyze the age at which patients in the United States receive the procedure. The research found that the median age at surgery was 57 days old, which exceeds the goal of 45 days. The study suggests that further investigation is needed to understand factors that may affect the timing of diagnosis and treatment. [Extracted from the article]

A recent study conducted by Guangzhou Medical University in southern China has found a genetic association between a specific gene, GJA8, and long-segment Hirschsprung's disease (L-HSCR) in children. Hirschsprung's disease is a congenital disorder that affects the colon and is caused by both genetic and environmental factors. The study analyzed a single nucleotide polymorphism (SNP) in the GJA8 gene and found that it increased the risk of L-HSCR in the southern Chinese population. The research suggests that this SNP may play a regulatory role in gene expression and contribute to the susceptibility of L-HSCR in children from southern China. [Extracted from the article]

A recent study conducted in Kota Kinabalu, Malaysia, examined the quality of life (QOL) in children with anorectal malformation (ARM) and Hirschsprung's disease (HD) compared to healthy controls. The study found that there were no significant differences between parent-reported and child-reported QOL scores in the overall, psychosocial health, and physical health components for ARM, HD, and controls. However, parents tended to overestimate the child's general well-being. The researchers emphasized the importance of considering both parent and child perspectives to improve patient care. [Extracted from the article]

Researchers in the Department of Hepatobiliary Surgery have conducted a study on choledochal cysts, a rare condition that requires surgical treatment to prevent complications such as obstructive jaundice and cyst rupture. The researchers recommend complete cyst excision followed by reconstruction with a Roux-en-Y hepaticojejunostomy as the best treatment option. They present a case study with a video demonstrating the complete resection of a type I choledochal cyst and reconstruction of two separate ducts using a laparoscopic approach. This approach offers the advantages of minimally invasive surgery and better visualization of structures, but also presents a surgical challenge that requires specialized training. [Extracted from the article]

Researchers at Yonsei University College of Medicine conducted a study to determine the association between overweight/obesity, metabolically unhealthy (MU), and the risk of developing Barrett's esophagus (BE). They analyzed data from 402,510 individuals in the UK Biobank and found that both MU and overweight/obesity were independently associated with BE incidence. The study also revealed that BE incidence was significantly higher in individuals who were metabolically unhealthy and overweight/obese. This research suggests that MU and overweight/obesity are independent risk factors for BE and have a synergistic effect on its development. [Extracted from the article]

A recent study conducted by University Gadjah Mada in Yogyakarta, Indonesia, examined the outcomes of children with long-segment and total colon Hirschsprung disease (HSCR) following pull-through surgery. HSCR is a congenital disorder that causes a functional obstruction in infants due to the absence of ganglion cells. The study found that children with long-segment HSCR generally had good functional outcomes, while some children with total colon HSCR experienced soiling and failed to achieve voluntary bowel movement. Hirschsprung-associated enterocolitis (HAEC) was noted in both groups, and complications such as surgical site infection and diaper rash were observed. The researchers emphasized the importance of long-term follow-up to identify complications early and provide appropriate treatment. [Extracted from the article]

A new report from Mahidol University in Bangkok, Thailand discusses research findings on the prevalence and association of congenital heart disease (CHD) with Hirschsprung's disease. The study found that 14.4% of Hirschsprung's disease patients also exhibited CHDs. Echocardiograms were found to be more sensitive than chest X-rays in diagnosing CHDs. The presence of concomitant deformities, syndromes, and/or associations associated with Hirschsprung's disease significantly increased the likelihood of concurrent CHDs. The study concludes that echocardiograms should be selectively used in Hirschsprung's disease patients with Down syndrome, other syndromes, or concomitant deformities. [Extracted from the article]

The article reports on new clinical practice guidelines from the Center for Clinical Management Research regarding endoscopic eradication therapy (EET) for Barrett's esophagus (BE) and related neoplasia. Topics discussed include evidence-based recommendations for EET based on dysplasia severity, comparative effectiveness of focal endoscopic mucosal resection (EMR) versus stepwise EMR, and considerations for shared decision-making and future research directions in the management of BE.

The article focuses on a case study from Zhejiang University addressing the challenges of managing congenital high airway obstruction syndrome (CHAOS) complicated by esophageal atresia (EA) and tracheoesophageal fistula (TOF). Topics include the difficulties in intubation in preterm infants with these conditions, the critical need for thorough antenatal diagnosis, and the potential for new tracheal replacement strategies to improve patient outcomes.

A new report discusses research findings on biliary atresia, a condition characterized by fibro-obliterative changes in the extrahepatic bile duct and congenital malformations. The study investigated the role of the gene Pkd1l1 in the development of biliary atresia and found that Pkd1l1-deficient mice exhibited congenital anomalies, hypertrophic and fibrotic bile ducts, and delayed biliary drainage. The mice also showed increased expression of fibrosis and extracellular matrix remodeling genes and decreased expression of genes mediating ciliary signaling. The researchers concluded that Pkd1l1-deficient mice serve as an authentic genetic model for studying the pathogenesis of biliary atresia. [Extracted from the article]

A new report discusses research findings on choledochal cysts, a rare condition with varying presentations depending on the patient's age and context. Early diagnosis and complete resection are crucial to prevent complications and malignancies. The most common type is Type Ia, which can rarely reach a giant size. Patients with giant cysts often have a long clinical history and limited access to specialist care. The management of giant cysts presents challenges, including the risk of permanent liver damage. For more information, readers can refer to the article "Successful Management of a Late Referred Giant Choledochal Cyst Type Ia in 8-Year-old Girl" in the Annals of African Medicine. [Extracted from the article]

A recent study conducted at Chelsea and Westminster Hospital NHS Foundation Trust in London, United Kingdom, focused on the diagnostic pathway of Hirschsprung's disease (HD) in pediatric patients. The study aimed to describe the diagnostic process and the indication for a rectal biopsy. The researchers collected data from 33 patients who underwent 34 rectal biopsies, with constipation and chronic abdominal distension plus vomiting being the most common symptoms. The study concluded that a protocolized approach to assessing infants and children with suspected HD ensures the appropriate use of invasive procedures such as biopsy. [Extracted from the article]

A recent research study conducted at University Hospital Rostock in Germany explored the use of transrectal diameter (TRD) assessment with ultrasound to evaluate symptoms of faecal load in children with Hirschsprung Disease (HD). The study found that children with HD without obstructive symptoms had a TRD of less than 3 cm, similar to healthy controls. The TRD measurement allowed clinicians to assess faecal load without exposing children to radiation. The findings suggest that TRD assessment can be a useful tool for monitoring bowel management in children with HD. [Extracted from the article]

A recent study conducted at the University of KwaZulu-Natal in South Africa examined the transition from thoracotomy to thoracoscopy for esophageal atresia repair (TEAR) in a low- and middle-income country (LMIC) setting. The study analyzed data from 65 patients with esophageal atresia, comparing the outcomes of those who underwent TEAR versus open esophageal atresia repair (OEAR). The results showed that TEAR had comparable outcomes to OEAR, with lower mortality rates. The study suggests that implementing minimally invasive surgery for esophageal atresia in LMICs can be successful with a dedicated quality improvement program. [Extracted from the article]

A recent study conducted by researchers at the University of Heidelberg in Germany has shed light on the role of inflammation and the NF-kB signaling pathway in Hirschsprung's disease (HSCR). HSCR is a congenital abnormality caused by the failure of neural crest-derived precursors to develop properly in the enteric nervous system. The study found that there was decreased gene expression of the NF-kB main subunit RELA, as well as other NF-kB-related genes, in the pathologic distal aganglionic segments of HSCR tissue samples compared to the proximal ganglionic segments. The researchers concluded that the modulation of NF-kB signaling in the neuro-enteric system contributes to the pathological effects of HSCR. [Extracted from the article]

A study conducted in Nanchang, China, has found that gene variants in the ZNF717 and PABPC1 genes contribute to congenital esophageal atresia (EA) by interfering with normal esophageal growth. EA is a condition where the esophagus does not develop properly in infants and is often accompanied by tracheoesophageal fistula (TEF). The researchers analyzed esophageal tissue samples from children with EA-TEF and established esophageal organoid units as an in vitro model. They found that the mutated genes ZNF717 and PABPC1 were associated with slower growth and dysplasia of cell development in the organoids. The study suggests that normal expressions of ZNF717 and PABPC1 are essential for esophageal development, and their variant or deficiency may lead to EA-TEF. [Extracted from the article]

Researchers at Xi'an Jiaotong University in Shaanxi, China have conducted a study on the use of totally laparoscopic surgery for choledochal cysts (CCs) in pediatric patients. The study evaluated the clinical data of 28 children who underwent the procedure, which involved cyst excision and Roux-en-Y hepaticojejunostomy. The results showed that the surgery can be safely performed in pediatric patients, with minimal complications and faster recovery compared to conventional laparoscopic surgery. The researchers suggest that totally laparoscopic surgery with manual sutures may be a viable option for minimally invasive surgery in pediatric patients with CCs. [Extracted from the article]

A recent study conducted by Fujian Medical University in China aimed to develop a prediction nomogram based on clinical factors to assess the risk of postoperative complications in children with congenital choledochal cysts. The study analyzed the clinical data of 131 children who underwent choledochal cyst resection and Roux-en-Y hepaticojejunostomy. The researchers identified several predictors associated with postoperative complications, including age, Todani classification type, cyst wall thickness, chronic cholecystitis, and choledochal cyst diameter. The study concluded that the prediction nomogram could provide a more accurate strategy for procedure and postoperative follow-up in children with choledochal cysts. [Extracted from the article]

The article from the University of Hong Kong details new research on the anti-fibrotic effects of steroids in biliary atresia. Topics include the use of animal and organoid models to assess steroid efficacy, the evaluation of liver fibrosis and other biomarkers, and the differences observed in liver morphology and gene expression between steroid and placebo-treated groups. The study concludes that steroids show potential in reducing liver fibrosis associated with biliary atresia.

The article from Cincinnati Children's Hospital Medical Center details findings in biliary atresia. Topics include the presence of portal vein hypoplasia in patients with biliary atresia at diagnosis, the comparison of portal vein and hepatic artery diameters between patients with biliary atresia and other forms of cholestasis, and the association of portal vein hypoplasia with clinical outcomes such as jaundice clearance and liver survival.

The article from the University of Chile reveals new insights into personalized medicine by discussing a rare case involving visceral myopathy (VM) and Hirschsprung's disease (HD). Topics include the clinical significance of these conditions, the diagnostic challenges requiring full-thickness biopsy, and the need for personalized treatment approaches.