Incidence and risk factors of graft failure in allogeneic hematopoietic stem cell transplantation for mucopolysaccharidosis in a nationwide pediatric cohort. A study on behalf of the Francophone Society of Bone Marrow Transplantation and Cellular Therapy.

Context: Mucopolysaccharidosis (MPS) requires urgent treatment to prevent neurological damage. While gene therapy holds promise for effectively treating these diseases with minimal toxicity, access remains limited for most patients. Consequently, advancing allogeneic hematopoietic stem cell transplantation (HSCT) for young children is crucial.

Contribution of simulation in paediatric laparoscopic surgery: improving suturing skills before performing a laparoscopic procedure in children.

Introduction: Mastering paediatric laparoscopic surgery competency (PLSC) is technically challenging. The present study aimed to determine whether the inter-academic PLSC degree (IAD_PLSC) practical training program enables trainees to improve their skills.

Methods: This retrospective study included trainees enrolled in the IAD_PLSC program in 2021 and 2022 which included two separate 12 h-sessions.

Executive functions disorders in children with frontal and temporal epilepsy.

Introduction: Children with focal epilepsy often present with executive functions (EFs) deficits. EFs deficits can contribute to adaptive challenges and have a negative impact on academic achievement. The purpose of this study was to investigate the EFs profiles of children diagnosed with frontal lobe epilepsy or temporal lobe epilepsy.

Comparison of surgical management and outcomes of acute right colic and sigmoid diverticulitis: a French national retrospective cohort study.

Background: Acute right colic diverticulitis (ARD) is less frequent in Western countries than acute sigmoid diverticulitis (ASD). We aimed to compare the management of ARD and ASD operated on in emergency.

Methods: All consecutive patients who had emergency surgery for ASD and ARD (2010-2021) were included in a retrospective, multicenter, cohort study.

Itching for Answers: A Comprehensive Review of Cholestatic Pruritus Treatments.

Cholestasis is a clinical and laboratory syndrome indicating impaired bile production or excretion. One of the hallmark symptoms of cholestasis is pruritus. Itch can be severe and debilitating for patients, impacting their quality of life similarly to pain, and, in some cases, it can be refractory.

Impact of facial nerve resection in parotid cancer abutting the facial nerve without preoperative paralysis: A multicentric propensity score-based analysis.

Objectives: The management of the facial nerve (FN) is a major issue in parotid cancer, especially when there is no preoperative facial palsy and FN invasion is discovered intraoperatively. The aim of this study was to assess the impact of FN resection in patients with parotid cancer abutting the FN, without pretreatment facial palsy, using a propensity score matching.

Materials And Methods: Data from all patients treated between 2009 and 2020 for a primary parotid cancer abutting or invading the FN but without pretreatment facial palsy were extracted from the national multicentric REFCOR database.

Early parental vocal contact in neonatal units: rationale and clinical guidelines for implementation.

This paper aims to present clear and evidence-based proposals for the integration of Early Parental Vocal Contact into the clinical practices of neonatal units. In the first part, we present a comprehensive rationale exploring the ontogenesis of voice perception in both term and preterm newborns that establishes a foundational understanding. This knowledge serves as a crucial starting point for developing evidence-based auditory and multisensory interventions aimed at fostering the developmental trajectory of preterm infants.

Early-Onset Osteoporosis: Molecular Analysis in Large Cohort and Focus on the PLS3 Gene.

Osteoporosis is a skeletal disorder characterized by abnormal bone microarchitecture and low bone mineral density (BMD), responsible for an increased risk of fractures and skeletal fragility. It is a common pathology of the aging population. However, when osteoporosis occurs in children or young adults, it strongly suggests an underlying genetic etiology.

Review of 2022 PSOGI/RENAPE Consensus on HIPEC.

The 2022 PSOGI (Peritoneal Surface Oncology Group International) and RENAPE (French Network for Rare Peritoneal Malignancies) consensus on hyperthermic intraperitoneal chemotherapy (HIPEC) was a comprehensive effort aimed at standardizing treatment protocols for various peritoneal malignancies. This initiative is critical due to the wide range of technical variations in HIPEC procedures and the resulting need for standardization to ensure consistent and effective patient care and meaningful audit of multicenter data.

No differences in clinical and radiographic outcomes between standard offset and high offset short cementless stems.

Purpose: To compare clinical and radiographic outcomes of total hip arthroplasty (THA) using standard offset versus high offset short cementless stems.

Methods: We reviewed a consecutive series of 204 primary THAs performed over 5 years using a short cementless collared stem. At a minimum follow-up of 2 years, 6 patients had deceased, 6 were not evaluated radiographically and, 2 were lost to follow-up.

The 2022 PSOGI International Consensus on HIPEC Regimens for Peritoneal Malignancies: Pseudomyxoma Peritonei.

Background: The combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) constitutes the established standard of care for pseudomyxoma peritonei patients. However, the role of HIPEC lacks validation through randomized trials, leading to diverse proposed treatment protocols. This consensus seeks to standardize HIPEC regimens and identify research priorities for enhanced clarity.

Stenosis of the colorectal anastomosis after surgery for diverticulitis: A national retrospective cohort study.

Aim: The aim of this work was to investigate the association between early postoperative anastomotic leakage or pelvic abscess (AL/PA) and symptomatic anastomotic stenosis (SAS) in patients after surgery for left colonic diverticulitis.

Method: This is a retrospective study based on a national cohort of diverticulitis surgery patients carried out by the Association Française de Chirurgie. The assessment was performed using path analyses.

Standardizing eligibility and patient selection for Pressurized Intraperitoneal Aerosol Chemotherapy: A Delphi consensus statement.

Introduction: Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC) is a procedure for minimally invasive drug administration in patients with peritoneal metastasis. Previous studies have emphasized the importance of uniformity in treatment protocols and standardization of this practice. This study aimed to reach a consensus on eligibility, patient selection, and choice of chemotherapy for PIPAC.

Risk factors for emergency surgery for diverticulitis: A retrospective multicentric French study at 41 hospitals.

Background: The observed increase in the incidence of complicated diverticulitis may lead to the performance of more emergency surgeries. This study aimed to assess the rate and risk factors of emergency surgery for sigmoid diverticulitis.

Method: The primary outcomes were the rate of emergency surgery for sigmoid diverticulitis and its associated risk factors.

Knowledge-based, computerized, patient clinical decision support system for perioperative pain, nausea and constipation management: a clinical feasibility study.

Opioid administration is particularly challenging in the perioperative period. Computerized-based Clinical Decision Support Systems (CDSS) are a promising innovation that might improve perioperative pain control. We report the development and feasibility validation of a knowledge-based CDSS aiming at optimizing the management of perioperative pain, postoperative nausea and vomiting (PONV), and laxative medications.

Parastomal hernia repair according to Modified Stapled Mesh Stoma Reinforcement Technique (mSMART): which are the results ?

Introduction: Parastomal hernia repair is a real surgical challenge because of the high rate of recurrence. The Stapled Mesh Stoma Reinforcement Technique (SMART) is a keyhole-like technique in which the mesh is stapled to the fascia using a circular mechanical stapler.

Methods: A prospective study from January 2021 to February 2023 was conducted including all patients operated with the SMART technique.

CNS tumors with PLAGL1-fusion: beyond ZFTA and YAP1 in the genetic spectrum of supratentorial ependymomas.

A novel methylation class, "neuroepithelial tumor, with PLAGL1 fusion" (NET-PLAGL1), has recently been described, based on epigenetic features, as a supratentorial pediatric brain tumor with recurrent histopathological features suggesting an ependymal differentiation. Because of the recent identification of this neoplastic entity, few histopathological, radiological and clinical data are available. Herein, we present a detailed series of nine cases of PLAGL1-fused supratentorial tumors, reclassified from a series of supratentorial ependymomas, non-ZFTA/non-YAP1 fusion-positive and subependymomas of the young.

The Ala1186Val Variant Linked to Cytoplasmic Body Myopathy and Cardiomyopathy Causes Protein Instability.

Filamin C-related disorders include myopathies and cardiomyopathies linked to variants in the gene. Filamin C belongs to a family of actin-binding proteins involved in sarcomere stability. This study investigates the pathogenic impact of the c.