JCCG ALL-B12: Evaluation of Intensified Therapies With Vincristine/Dexamethasone Pulses and Asparaginase and Augmented High-Dose Methotrexate for Pediatric B-ALL.

Purpose: The JCCG ALL-B12 clinical trial aimed to evaluate the effectiveness of unvalidated treatment phases for pediatric ALL and develop a safety-focused treatment framework.

Patients And Methods: Patients age 1-19 years with newly diagnosed B-ALL were enrolled in this study. These patients were stratified into standard-risk (SR), intermediate-risk (IR), and high-risk (HR) groups.

Effect of early tethered cord release on urodynamic findings and lower urinary tract function in myelomeningocele patients.

Objective: Detecting neurological deterioration and diagnosing tethered cord syndrome (TCS) in patients with myelomeningocele (MMC) can be challenging due to the presence of symptoms at birth and the lack of objective indicators. This retrospective analysis focused on urological manifestations and evaluated whether tethered cord release (TCR) for TCS at an early stage could improve or stabilize video urodynamic study (VUDS) findings and lower urinary tract function.

Methods: This study analyzed 55 of 64 children who underwent MMC repair at Miyagi Children's Hospital, Sendai, Japan, between 2003 and 2016.

A Prevalent TMEM260 Deletion Causes Conotruncal Heart Defects, Including Truncus Arteriosus.

Conotruncal heart defects are severe congenital malformations of the outflow tract, including truncus arteriosus (TA) and double-outlet right ventricle (DORV). TA is a severe congenital heart disease (CHD) in which the main arterial outflow tract of the heart fails to separate. We recently reported TMEM260 (NM_017799.

Combined procedure using a double flap as a surgical option for coarctation of the aorta with delayed diagnosis.

Simple coarctation of the aorta is repaired in an infant by direct end-to-end anastomosis of the aorta or subclavian flap aortoplasty. However, some cases are not detected until late childhood. For school-age patients, greater consideration must be given to risks such as postoperative limb ischemia and the potentially harmful effects of any artificial material on future growth.

Urgent Notification Intervention of Home Blood Pressure in Cohort Studies of the Tohoku Medical Megabank Project.

Introduction: The Tohoku Medical Megabank (TMM) was established for creative reconstruction from the Great East Japan Earthquake and tsunami in 2011. Two prospective genome cohort studies in Miyagi prefecture have successfully recruited approximately 127,000 participants. The health status of these individuals was evaluated at the initial recruitment, and follow-up health checkups have been conducted every 5 years.

Biallelic null variants in PNPLA8 cause microcephaly by reducing the number of basal radial glia.

Patatin-like phospholipase domain-containing lipase 8 (PNPLA8), one of the calcium-independent phospholipase A2 enzymes, is involved in various physiological processes through the maintenance of membrane phospholipids. Biallelic variants in PNPLA8 have been associated with a range of paediatric neurodegenerative disorders. However, the phenotypic spectrum, genotype-phenotype correlations and the underlying mechanisms are poorly understood.

Extubation and removal of supraglottic airway devices in pediatric anesthesia.

In pediatric anesthesia, respiratory adverse events often occur during emergence from anesthesia and at the time of endotracheal tube or supraglottic device removal. The removal of airway devices and extubation are conducted either while patients are deeply anesthetized or when patients awaken from anesthesia and have regained consciousness. The airways of children are easily irritated by external stimuli and are structurally prone to collapse, and the timing of both methods of airway device removal is similarly associated with various airway complications, including upper airway obstruction, coughing, or serious adverse events such as laryngospasm and desaturation.

[CURRENT STATUS OF SCHOOL LUNCHES RELATED TO FOOD ALLERGY AND UNMET NEEDS OF TEACHERS IN MIYAGI PREFECTURE].

Background: The number of students with food allergies is on the increase, while the problems and burdens of school teachers and staff are not yet clear. Our study was designed to identify the unmet needs of school teachers and staff dealing with food allergy in school lunches.

Methods: A written questionnaire was sent by mail to 600 elementary and junior high schools in Miyagi Prefecture.

A case of long-term survival of SADDAN treated with growth hormone for marked short stature.

Severe achondroplasia with developmental delay and acanthosis nigricans (SADDAN) is a bone dysplasia caused by a pathogenic variant of fibroblast growth factor receptor 3 (). Pathogenic variants in also cause thanatophoric dysplasia (TD) and achondroplasia. Although the findings of SADDAN and TD during the fetal and neonatal periods are similar, they differ in their long-term prognoses.

Persistent Depression of the Lambda Site with Progressive Skull Deformity May Be a Sign of Suture Closure: Case Series and Pathologic Consideration.

Background: Flat head syndrome (FHS) sometimes occurs when a baby maintains the same head position during the first several months of life, causing a skull deformity. FHS usually improves with time and natural growth, although some show aggravation against conservative treatment. We reviewed pathologically proved early closure of skull suture that may be seen secondary to FHS.

How do we treat severely prolapsed true ectopia cordis?

Ectopia cordis is an extremely rare condition where the heart displaces outside the thoracic cavity. Treating this, especially when accompanied by congenital heart diseases and severe protrusion, is challenging. Here, we present a case of successful treatment involving intracardiac repair and delayed sternal closure using local skin flaps.