Immune profiling and functional analysis of NK and T cells in ataxia telangiectasia.

Ataxia telangiectasia (AT) is a rare autosomal-recessive disorder characterized by profound neurodegeneration, combined immunodeficiency, and an increased risk for malignant diseases. Treatment options for AT are limited, and the long-term survival prognosis for patients remains grim, primarily due to the emergence of chronic respiratory pathologies, malignancies, and neurological complications. Understanding the dysregulation of the immune system in AT is fundamental for the development of novel treatment strategies.

Use of a new vertical traction device for early traction-assisted staged closure of congenital abdominal wall defects: a prospective series of 16 patients.

Purpose: Abdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns.

Methods: Four tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023.

[The acute abdomen in children].

Background: Because 8-10% of children in the emergency room present with acute abdominal pain, a systematic work-up is essential to rule out acute abdomen.

Objectives: This article highlights the etiology, symptoms, diagnostic workup, and treatment of acute abdomen in children.

Materials And Methods: Review of the current literature.

MUC-FIRE: Study protocol for a randomized multicenter open-label controlled trial to show that MUCous FIstula REfeeding reduces the time from enterostomy closure to full enteral feeds.

Background: After enterostomy creation, the distal bowel to the ostomy is excluded from the physiologic passage of stool, nutrient uptake, and growth of this intestinal section. Those infants frequently require long-term parenteral nutrition, continued after enterostomy reversal due to the notable diameter discrepancy of the proximal and distal bowel. Previous studies have shown that mucous fistula refeeding (MFR) results in faster weight gain in infants.

Ovarian lesions and tumors in infants and older children.

Objectives: Ovarian lesions are rare but frequent in children. Patients could present with abdominal pain, but ovarian lesions could also be incidentally found on ultrasound. Awareness is required in cases with acute, severe lower abdominal pain, as ovarian torsion could be the cause.

Multidisciplinary Treatment Strategies for Wilms Tumor: Recent Advances, Technical Innovations and Future Directions.

Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed.

Prenatal Detection of Congenital Duodenal Obstruction-Impact on Postnatal Care.

Background: Duodenal obstruction is a rare cause of congenital bowel obstruction. Prenatal ultrasound could be suggestive of duodenal atresia if polyhydramnios and the double bubble sign are visible. Prenatal diagnosis should prompt respective prenatal care, including surgery.

Outcome of esophageal atresia: inborn versus outborn patients.

Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume-outcome association in EA management remains unconfirmed.

Comparison of perioperative outcomes between laparoscopic and open partial splenectomy in children and adolescents.

Background: In order to avoid consequences of total splenectomy, partial splenectomy (PS) is increasingly reported. The purpose of this study was to compare perioperative outcomes of laparoscopic PS (LPS) and open PS (OPS) in children and adolescents.

Aim: To compare perioperative outcomes of patients with LPS and OPS.

Epidemiology and One-Year Follow-Up of Neonates with CDH-Data from Health Insurance Claims in Germany.

Congenital diaphragmatic hernia (CDH) is a major congenital malformation with high mortality. Outcome data on larger unselected patient groups in Germany are unavailable as there is no registry for CDH. Therefore, routine data from the largest German health insurance fund were analyzed for the years 2009-2013.

Incidence of penalty corner injuries in international field hockey.

Field hockey penalty corners (PCs) put players at potential risk for injuries. This study evaluated the incidence of PC-related injuries with special regards to head injuries. Video sequences provided by the Fédération Internationale de Hockey (FIH) of 295 matches in 11 elite field hockey tournaments (2015-2016) were analysed for overall injuries and PC-related injuries in detail.

Injury surveillance in elite field hockey: a pilot study of three different recording techniques.

Objective: In field hockey, injuries are assessed by various recording techniques leading to a heterogenic collection of poorly comparable injury data.

Methods: Injury data were prospectively collected at the 2016 Men's Hockey Junior World Cup using the match injury reports (MIRs), video injury clips provided by the Fédération Internationale de Hockey, and daily medical reports (DMRs). A pilot study comparing injury type, mechanism, location on the field, injured body part and overall injury incidence among the different injury recording techniques was performed.

Video Analysis of Acute Injuries in Elite Field Hockey.

Objective: With this study, we aim to determine the value of video assessment of acute injuries in field hockey.

Design: Retrospective video analysis for acute injuries.

Setting: Professional field hockey.

Comparison of outcomes between complete and incomplete congenital duodenal obstruction.

Background: Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes.

Aim: To quantify and compare the association between CCDO and ICDO with outcome parameters.

Laparoscopic versus open surgery for the repair of congenital duodenal obstructions in infants and children.

Background: Laparoscopic repair of congenital duodenal obstruction (LCDO) was described more than 15 years ago. However, studies comparing outcomes of LCDO with open repair (OCDO) are rare. Standardized assessments of complications using the Clavien-Dindo classification (CDC) and the comprehensive complication index (CCI) are not available.

Crizotinib in ALK inflammatory myofibroblastic tumors-Current experience and future perspectives.

Inflammatory myofibroblastic tumor (IMT) and its subtype epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue tumors. As about 50% of IMT and 100% of EIMS contain activating rearrangements of the anaplastic lymphoma kinase (ALK) gene, targeted kinase inhibition of ALK by compounds such as crizotinib is a potential treatment option. We performed a literature review and analyzed a total of 30 patients with IMT/EIMS treated with crizotinib.

Radical Surgery Improves Survival in Patients with Stage 4 Neuroblastoma.

Background: Neuroblastoma (NBL) is the most common extracranial solid tumor in children. Despite a good overall prognosis in NBL patients, the outcome of children with stage 4 disease, even with multimodal intensive therapy, remains poor. The role of extended surgical resection of the primary tumor is in numerous studies controversial.

Identification of intestinal ganglioneuromatosis leads to early diagnosis of MEN2B: role of rectal biopsy.

Background/purpose: Gastrointestinal symptoms are very common in patients with multiple endocrine neoplasia type 2B (MEN2B) syndrome. Herein, we present a case of intestinal ganglioneuromatosis (IGN) in MEN2B syndrome and a systematic literature review with a special focus on gastrointestinal symptoms prior to the diagnosis of MEN2B.

Methods: Literature search was performed (years 1966-2015) using the "Pubmed" and "Scopus" databases.

Injury data of major international field hockey tournaments.

Background: Detailed injury data are not available for international tournaments in field hockey. We investigated the epidemiology of field hockey injuries during major International Hockey Federation (Fédération Internationale de Hockey, FIH) tournaments in 2013.

Materials And Methods: FIH injury reports were used for data collection.

Pancreatic cancer exosomes initiate pre-metastatic niche formation in the liver.

Pancreatic ductal adenocarcinomas (PDACs) are highly metastatic with poor prognosis, mainly due to delayed detection. We hypothesized that intercellular communication is critical for metastatic progression. Here, we show that PDAC-derived exosomes induce liver pre-metastatic niche formation in naive mice and consequently increase liver metastatic burden.

Aging-dependent reduction in glyoxalase 1 delays wound healing.

Methylglyoxal (MG), the major dicarbonyl substrate of the enzyme glyoxalase 1 (GLO1), is a reactive metabolite formed via glycolytic flux. Decreased GLO1 activity in situ has been shown to result in an accumulation of MG and increased formation of advanced glycation endproducts, both of which can accumulate during physiological aging and at an accelerated rate in diabetes and other chronic degenerative diseases. To determine the physiological consequences which result from elevated MG levels and the role of MG and GLO1 in aging, wound healing in young (≤12 weeks) and old (≥52 weeks) wild-type mice was studied.

Desmoid fibromatosis in children and adolescents: a conservative approach to management.

Purpose: Desmoid fibromatosis is associated with frequent recurrence and significant morbidity, but no metastases. To examine the impact of initial non-operative management on event-free survival (EFS) in children, we reviewed our institutional experience with this tumor.

Methods: We retrospectively reviewed our institutional database for pediatric cases of desmoid fibromatosis treated between 1970 and 2010.