Understanding how biological communities assemble in the presence of rapid evolution is becoming an important topic in ecology. Previous studies demonstrated that community assembly can be affected by two types of eco-evolutionary dynamics: evolution-mediated priority effect (EPE) and ecological character displacement (ECD). In EPE, early-arriving species prevent colonization of late-arriving species via local adaptation (i.
View Article and Find Full Text PDFNeurofibromatosis type 1 (NF1) is an autosomal dominant nevus disease characterized by multiple manifestations, primarily café-au-lait macules and neurofibromas. Here, we present the case of an NF1 patient with 47,XYY mosaicism whose diagnosis was prompted by café-au-lait macules on the skin and mandibular neurofibromas. Targeted next-generation sequencing of the patient's blood sample revealed a novel frameshift mutation in NF1 (NM_000267.
View Article and Find Full Text PDFPurpose: Congenital tracheal stenosis (CTS) has been reported to occur in 50-65% of cases of left pulmonary artery sling (LPAS), but the exact incidence rate is unknown. This study aimed to determine the actual rate using bronchoscopy and to elucidate morphological features in computed tomography (CT) diagnosis.
Methods: We performed a single institutional retrospective review of all patients with LPAS between January 2010 and March 2022.
Purpose: A tracheoesophageal fistula (TEF) associated with complete tracheal rings (CTR) is extremely rare. This study aimed to elucidate the clinical features of this combination.
Methods: Records of 39 patients diagnosed with TEF with or without CTR between January 2013 and February 2023 were retrospectively reviewed.
Infantile fibrosarcoma (IFS) commonly harbors ETS variant transcription factor 6 ()-neurotrophic receptor tyrosine kinase 3 () fusion. However, the recent accessibility to clinical next-generation sequencing (NGS) has revealed negative spindle cell sarcomas resembling IFS morphologically, involving and . The present report describes a pediatric case of spindle cell sarcoma with resembling IFS morphologically.
View Article and Find Full Text PDFAim: To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients.
Methods: Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately.
Aim: To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients.
Methods: Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately.
Pediatr Surg Int
December 2022
Purpose: The severity of congenital tracheal stenosis (CTS) is commonly evaluated based on the degree of stenosis. However, it does not always reflect the clinical respiratory status. We applied computational fluid dynamics (CFD) to the assessment of CTS.
View Article and Find Full Text PDFPurpose: Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination.
Methods: Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed.
Congenital tracheal stenosis (CTS) with unilateral pulmonary agenesis (UPA) is characterized by the absence of one or both lungs in the hemithorax and is often associated with airway distortion. Some UPA patients have high mortality and morbidity even postoperatively, and it remains unclear whether surgery increases the energy flux needed to drive airflow. Here, we used pre- and postoperative patient-specific airway models to numerically investigate tracheal flow in patients with CTS, especially flow associated with right UPA (CTS-RUPA).
View Article and Find Full Text PDFObjective: To evaluate the relationship between the changes in condylar volume and maxillofacial skeletal morphology according to sex as well as the relationship between condylar volume reduction and skeletal relapse in patients who underwent orthognathic surgery.
Methods: Ninety-five patients were categorized into skeletal Class III, Class II, and facial asymmetry groups. Computed tomography scans taken preoperatively and at 1 year postoperatively were used for quantitative measurement.
Background: Perioperative management of congenital tracheal stenosis (CTS) is challenging. In the present study, compared the effect of closed-pediatric intensive care unit (PICU) perioperative management by pediatric intensivists and open-PICU management by surgeons. Outcomes in terms of ventilator-free days (VFD) and length of postoperative PICU stay in children with CTS were evaluated.
View Article and Find Full Text PDFEuropean J Pediatr Surg Rep
January 2021
Fetal lung interstitial tumor (FLIT) is a rare primary lung mass in neonates. Classical incisions, such as posterolateral thoracotomy or median sternotomy, do not provide optimal exposure of the operative field for the resection of pediatric thoracic giant tumors. Herein, we report a rare case of a FLIT in a full-term male neonate, with complete resection achieved using a hemi-clamshell approach, which provided the required visualization of the operative field.
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