Biliatresone induces cholangiopathy in C57BL/6J neonates.

Exposure to plant toxins or microbiota that are able to digest common food ingredients to toxic structures might be responsible for biliary atresia (BA). An isoflavonoid, biliatresone is known to effectively alter the extrahepatic bile duct (EHBD) development in BALB/c mice. Biliatresone causes a reduction of Glutathione (GSH) levels, SOX17 downregulation and is effectively countered with N-Acetyl-L-cysteine treatment in vitro. Therefore, reversing GSH-loss appears to be a promising treatment target for a translational approach. Since BALB/c mice have been described as sensitive in various models, we evaluated the toxic effect of biliatresone in robust C57BL/6J mice and confirmed its toxicity. Comparison between BALB/c and C57BL/6J mice revealed similarity in the toxic model. Affected neonates exhibited clinical symptoms of BA, such as jaundice, ascites, clay-colored stools, yellow urine and impaired weight gain. The gallbladders of jaundiced neonates were hydropic and EHBD were twisted and enlarged. Serum and histological analysis proved cholestasis. No anomalies were seen in the liver and EHBD of control animals. With our study we join a chain of evidence confirming that biliatresone is an effective agent for cross-lineage targeted alteration of the EHBD system.

Appendicitis and Peritonitis in Children with a Ventriculo-Peritoneal Shunt.

The purpose of this study was to outline the management of patients with appendicitis and ventriculoperitoneal shunt (VPS) in the largest pediatric surgery department in Germany. Patients with VPS presenting with an acute abdomen between 2012 and 2022 at a tertiary-care pediatric facility were the subject of a retrospective descriptive analysis. Patients were divided into two groups based on their diagnoses: group A (appendicitis) and group B (primary peritonitis). Medical records were analyzed to look at the diagnostics, operative approach, complications, peritoneal and liquor culture, and antibiotic treatment. A total of seventeen patients were examined: seven patients in group A and ten individuals in group B. In the present study patients in group A typically presented younger, sicker, and with more neurological symptoms than those in group B. All patients with appendicitis had their VPS exteriorized, and a new shunt system into the peritoneum was reimplanted 20 days later. Surgery should be aggressively administered to patients who present with an acute abdomen and a VPS. Change of the whole shunt system is suggested. Shunt infection and dysfunction should be ruled out in patients with abdominal symptoms, and surgical care should be started with a low threshold.

Extrahepatic Bile Duct and Gall Bladder Dissection in Nine-Day-Old Mouse Neonates.

The dissection of murine neonatal bile ducts has been described as difficult. The main aim of the described standard operating procedure is the isolation of the extrahepatic bile duct (EBD) in mouse neonates without damaging the bile duct during preparation. Because of its exceptionally close preparation compared to the cholangiocytes cell line and harvesting of the entire extrahepatic bile duct system (EBDS), the described approach is extremely useful in researching animal models of newborn bile duct disorders, such as biliary atresia. After euthanasia, the peritoneal cavity was accessed, and the bile duct system, duodenum, and liver were extracted with the unique En-bloc-Resection (EbR). The extracted sample is placed on a foam mat, and the EBD is dissected from contaminating cells atraumatically without necessary touch. The dissection of the entire EBDS is a significant advantage of this method. Caution must be taken due to the small size and amount of bile duct tissue. Using the described technique, there is no damage to the cholangiocytes. Further, the purity of the technique is reproducible (n = 10). Therefore, optimally comparable samples can be harvested. Furthermore, no bile duct tissue is harmed, because any contact with the bile duct system can be avoided during preparation, leaving the bile fluid inside the gall bladder. Most importantly, while performing the final gall bladder and bile duct dissection, atraumatic microinstruments were used only slightly lateral of the bile duct without squeezing it. This is the key to a clean and intact sample, and essential for further histological investigation or the isolation of cholangiocytes. To summarize, the described innovative dissection technique enables especially inexperienced operators with the necessary equipment to isolate the EBDS as cleanly as possible.

Limits in Laparoscopic Partial Splenectomy in Children.

The aim of this paper is to assess the effectiveness and perioperative complications of splenic surgeries in children. In 41 splenectomies, an anterior abdominal laparoscopic approach was used, with 35 including a partial laparoscopic splenectomy. Of these, three needed a conversion to open. Six patients had a total splenectomy, three of which were open. Patients ranged in age from 5 to 18 years. Splenectomy was performed for a variety of causes, including hereditary spherocytosis (n = 20), splenic cysts (n = 13), sickle cell disease (n = 3), primary malignancy (n = 1), sepsis (n = 1), embolism (n = 1), anemia (n = 1), and hypersplenism (n = 1). The average length of stay was 7.6 days, and the average operation time was 169.3 min. Pleural effusion in the left hemithorax was found in 31.6% of the patients, with 5.3% requiring a thorax drain. The majority of patients had the highest platelet count two weeks after surgery. There was no evidence of wound infection, pancreatic leak, colon perforation, or postoperative sepsis. The most encountered perioperative complication was bleeding with the need of transfusion (n = 6), and one patient needed a diaphragm repair. A partial splenectomy (PS) can be a difficult procedure with a steep learning curve. For most children who require a splenic operation, this should be the primary procedure of choice.

Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis.

BACKGROUND: Patients suffering from complete colonic aganglionosis (TCA) require the best surgical care possible. Only a few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. This study adds to the current literature a thorough clinical and functional outcomes group. METHODS: Between 2011 and 2021, medical records of Hirschsprung disease (HD) patients who underwent J-Pouch reconstruction during infancy (n = 12) were reviewed. In close follow-up, bowel function and satisfaction with operation results were evaluated. The median age at the time of J-Pouch reconstruction was 16 months, and covering ileostomies were closed four months later. There were no postoperative problems. After the final repair, Pouch-related problems (PRP) occurred in 27% of the children and were treated conservatively. There was no histological evidence of pouchitis in any of the individuals. The median 24-h stooling frequency was 4-5 at the latest follow-up 51 months following enterostomy closure. CONCLUSIONS: The current study's findings support the existing literature and advocate for J-pouch repair in TCA patients. However, more research will be needed to determine the best time to undergo pouch surgery and ileostomy closure in TCA patients.