Purpose: Hirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD), TCA and the type of operation used. Methods: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC. Results: 4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques. Conclusion: The prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.

Early life is characterized by extraordinary challenges, including rapid tissue growth and immune adaptation to foreign antigens after birth. During this developmental stage, infants have an increased risk to immune-mediated diseases. Here, we demonstrate that tissue-resident, IL-13 and IL-4-producing ILC2s are enriched in human infant intestines compared to adult intestines. Organoid systems were employed to assess the role of infant intestinal ILC2s in intestinal development and showed that IL-13 and IL-4 increased epithelial cell proliferation and skewed cell differentiation towards secretory cells. IL-13 furthermore upregulated the production of mediators of type-2 immunity by infant intestinal epithelial cells, including VEGF-A and IL-26, a chemoattractant for eosinophils. In line with these in vitro findings increased numbers of eosinophils were detected in vivo in infant intestines. Taken together, ILC2s are enriched in infant intestines and can support intestinal development, while inducing an epithelial secretory response associated with type 2 immune-mediated diseases.

The Federal Republic of Germany counts among the earliest States parties to the European Convention on Human Rights (ECHR). It ratified the ECHR on 5 December 1952, three years ahead of Italy, and hence found itself among the original members of the treaty system when the ECHR entered into force on 3 September 1953. For the new democratic Government, it was a decision of principle to affirm its willingness to cooperate peacefully within the group of European States, submitting to an international review mechanism with regard to all of its activities. Therefore, very shortly afterwards, it accepted also the individual application under Article 25 ECHR, which at that time was not yet compulsory for all States parties. For many years under the Nazi dictatorship, Germany had brought death and destruction to its neighbours. Now, organized under a democratic and liberal constitution, the Basic Law (BL), it wanted to manifest its newfound identity as a civilized State abiding by the rule of law.

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.

Background: The transition zone (TZ) is defined by specific histological findings in patients with Hirschsprung Disease (HSCR). HSCR treatment includes surgical removal of the aganglionic zone (AZ). During the pull-through procedure, it is critical to resect the TZ. Given the TZ’s wide histological heterogeneity, we wanted to know how extensive the histological transition zone is. Methods: A retrospective study of patients who had pull-through surgery for rectosigmoid HSCR between January 2010 and December 2020 was carried out. Demographics, length of TZ and AZ, age and symptoms upon presentation, and complications after surgery were also obtained. Results: The inclusion criteria were met by 50 patients. The mean age of all patients was 10 months (0.1–107.5 months), with a mean age at pull-through of 16.3 months (3–112 months). Thirty-one out of fifty patients (62%) received primary laparoscopic endorectal pull-through surgery (LEPT). The average TZ length of all patients was 2.6 cm (0–10 cm), and the AZ length was 9.6 cm (1–30 cm). The length of the AZ and TZ were shown to have no correlation (r² = 0.237). Conclusions: The current study found that the mean length of the TZ in individuals with rectosigmoid HSCR is less than 5 cm in most cases and has no correlation with the length of the AZ.