Scoping review of initiation criteria for inhaled nitric oxide in preterm infants (born <34 weeks) after 7 days of age.

Objectives: Inhaled nitric oxide (iNO) is a known treatment for pulmonary hypertension (PH) associated with bronchopulmonary dysplasia in preterm infants after 7 days of age (postacute phase). However, a consensus regarding the optimal criteria for initiating iNO therapy in this population in the postacute phase is currently lacking. This study, therefore, aimed to identify the criteria for initiating iNO therapy, alongside the associated clinical and echocardiographic findings, in this population.

Aberrant Methylation of is Associated with the Pathogenesis of Autoimmune Pancreatitis.

IgG4-related disease (IgG4-RD) is an autoimmune disease of unknown cause. is a transcription factor involved in immune responses, and its dysfunction leads to uncontrolled immune responses. We performed, to our knowledge, the first methylation analysis in type 1 autoimmune pancreatitis (denoted simply as AIP), a representative IgG4-RD.

Long-Term Outcomes of the Non-Fenestrated Strategy for Extracardiac Total Cavopulmonary Connection.

Background: The discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy.

Methods: Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy.

Outcomes Following Close Collaboration With Parents Intervention in Neonatal Intensive Care Units: A Nonrandomized Clinical Trial.

Importance: Family-centered care (FCC) in neonatal intensive care units (NICUs) is critical for parental involvement and infant well-being, yet few studies have evaluated the impact of FCC interventions on practice or examined how implementation fidelity may affect these outcomes.

Objectives: To evaluate the association between the Close Collaboration With Parents intervention and FCC practices and how implementation fidelity may modify these outcomes.

Design, Setting, And Participants: This nonrandomized clinical trial had a before-and-after design.

Epidemiology and treatment trends for acute encephalopathy under the impact of SARS-CoV-2 pandemic based on a prospective multicenter consecutive case registry.

Background: Acute encephalopathy is a severe condition predominantly affecting children with viral infections. The purpose of this study was to elucidate the epidemiology, treatment, and management of acute encephalopathy. The study also aimed to understand how the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has affected epidemiological trends.

Three-year prognoses after low-dose oral food challenge for cow's milk.

Background: Low-dose (LD) oral food challenge (OFC) with heated cow's milk (CM; 3 mL) effectively prevents CM elimination in children with CM allergy (CMA). We investigated the long-term prognoses after an LD-OFC for CMA.

Methods: Children with immediate CMA symptoms after consuming <25 mL of CM within 2 years of a baseline LD-OFC were retrospectively analyzed.

Usefulness of the "En face view" method for diagnosing perimembranous ventricular septal defects.

Background: Perimembranous ventricular septal defect (VSD) can be classified as having trabecular, inlet, or outlet extension. The surgical approach used in patch closure depends on the which valve of the tricuspid valve to suture around and the avoidance of the specialized conducting system. This retrospective study evaluated the usefulness of the "En face view" method for classifying perimembranous VSD.

Fetal bradycardia associated with left ventricle noncompaction diagnosed as HCN4 mutations.

A 35-year-old pregnant woman was referred to our hospital because of fetal bradycardia. Fetal echocardiography revealed a normal fetal heart except for slightly prominent trabeculae in the left ventricle, normal cardiac function, and fetal sinus bradycardia with a heart rate of 100 bpm. Electrocardiography (ECG) after birth revealed sinus bradycardia with a heart rate of 70-80 bpm.

Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts.

Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain.

A Novel Synonymous Variant in SQSTM1 Causes Neurodegeneration With Ataxia, Dystonia, and Gaze Palsy Revealed by Urine-Derived Cells-Based Functional Analysis.

Background: Heterozygous variants of sequestosome-1 gene (SQSTM1) have been reported in patients with various neurological disorders, whereas biallelic pathogenic variants of SQSTM1 can cause child-onset and multisystem neurodegeneration, including cerebellar ataxia, dystonia, and vertical gaze palsy (NADGP). Here, we describe two cases of NADGP in a Japanese family.

Methods: We performed clinical and genetic laboratory evaluations of the two patients and their healthy parents.

Effect of couplet care on early parent-infant closeness among preterm infants.

Aim: To evaluate the effect of couplet care on parent-infant closeness among preterm infants.

Methods: A comparison study in a Level III neonatal intensive care unit (NICU) before and after the introduction of couplet care, including stabilising infants in the delivery unit for early skin-to-skin contact, providing mothers' postpartum care in the infant's room and providing the father's bed in the infant's room. The study included parents of preterm infants born below 35 weeks.

Associations between maternal per- and polyfluoroalkyl substances exposure and lipid levels in maternal and cord blood: The Japan environment and Children's study.

Despite numerous studies, the associations between per- and polyfluoroalkyl substances (PFAS) exposure and various lipid levels in pregnant women remain ambiguous, especially concerning the association with cord blood lipids. This analysis included 20,960 pregnant women enrolled in the Japan Environment and Children's Study, recruited between 2011 and 2014. Non-fasting plasma samples collected before 22 weeks of gestation were examined for PFAS concentrations.

Impact of Introducing a Multiplex Polymerase Chain Reaction Blood Culture Panel on Anti-Methicillin-Resistant Staphylococcus aureus (MRSA) and Carbapenem Antimicrobial Agents in a Children's Hospital.

Background: With the advent of multiplex polymerase chain reaction (PCR) using samples from a positive blood culture, the time required to identify a pathogen has significantly shortened to a few hours. It can help us select appropriate antimicrobial agents more quickly. The present study aimed to assess the impact of using a multiplex PCR blood culture panel on the appropriate administration of antimicrobial agents.

Robust growth hormone responses to GH-releasing peptide 2 in adolescents.

Objectives: GH-releasing peptide-2 (GHRP2) can be used for provocative growth hormone testing (GHT). Since it acts as a powerful stimulus for GH secretion, cut-off peak GH level in GHRP2 loading test (GHRP2T) is higher than in other GHT. Nevertheless, data on response at adolescents are limited.