Clinical symptoms of patients with Kawasaki disease include smoldering fever.

Background: Persistent low-grade fever has been observed in some patients during intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD); however, smoldering fever (SF) has not previously been reported in patients with KD. This study aimed to clarify the clinical characteristics of SF in patients with KD.

Methods: A single-center retrospective cohort study, which included a total of 621 patients who received IVIG therapy, was conducted.

New predictors to identify severe cases during intravenous immunoglobulin therapy in Kawasaki disease.

Background: The purpose of this study was to predict which patients would require plasma exchange therapy (PEX) using laboratory findings during intravenous immunoglobulin (IVIG) therapy in Kawasaki disease.

Methods: A retrospective, single-center, cohort study was conducted. Of the 621 IVIG-treated patients, 166 patients who received a second IVIG dose on the second day after initiation of IVIG were included.

Poor response to intravenous immunoglobulin in patients with a parental history of Kawasaki disease.

Background: Patients whose parents had a history of Kawasaki disease (KD) often show a poor response to intravenous immunoglobulin (IVIG). There are very few reports describing the clinical characteristics and no reports on changes in C-reactive protein (CRP) level after IVIG administration in such cases.

Methods: A single-center cohort study including 950 patients hospitalized for KD and treated with IVIG was conducted.

Ascending Aortic Aneurysm in a Child With Fibulin-4 Deficiency.

EFEMP2 (alias FBLN4) encodes extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B and leads to severe aortopathy with aneurysm formation and vascular tortuosity. A 4-month-old child presented with a large ascending aortic aneurysm, and genetic testing revealed an EFEMP2 mutation. We achieved successful repair of the ascending aortic aneurysm at 33 months of age and report the macroscopic and microscopic findings.

Impact of rotavirus vaccination on the burden of acute gastroenteritis in Nagoya city, Japan.

Background: In Nagoya city, Japan, rotavirus (RV) vaccination has been available since 2011 with estimated coverage reaching 92% by 2015 after the introduction of a public subsidy in 2012. This study assessed the impact of vaccination on the RV gastroenteritis (RVGE) burden in children aged <5 years old (y) by comparing RVGE hospitalizations and outpatient visits during pre-vaccination (2007-2011), transition (2011-2012) and subsidization (2012-2016) periods.

Methods: All hospitalizations and outpatient visits in children aged <5 y from 2 administrative districts of Nagoya city were identified from the hospital-based electronic databases of 4 hospitals.

Self-perception of children with autism spectrum disorders.

Background: We aimed to evaluate self-perceived competence and self-esteem of primary school children with autism spectrum disorder (ASD) in Japan and compare to those of children with other chronic physical diseases and healthy controls.

Methods: Data were collected from 227 children: ASD (N.=91), nephrotic syndrome (NS) (N.

A case of atypical hemolytic uremic syndrome due to anti-factor H antibody in a patient presenting with a factor XII deficiency identified two novel mutations.

A 9-year-old boy with pallor and macrohematuria showed hemolytic anemia, thrombocytopenia and renal failure. There was no history of diarrhea and the stool culture was negative. A diagnosis of atypical hemolytic uremic syndrome (HUS) was confirmed; however, the cause of the prolonged activated partial thromboplastin time (APTT) was unknown.

Comprehensive genetic analysis of relevant four genes in 49 patients with Marfan syndrome or Marfan-related phenotypes.

In order to evaluate the contribution of FBN1, FBN2, TGFBR1, and TGFBR2 mutations to the Marfan syndrome (MFS) phenotype, the four genes were analyzed by direct sequencing in 49 patients with MFS or suspected MFS as a cohort study. A total of 27 FBN1 mutations (22 novel) in 27 patients (55%, 27/49), 1 novel TGFBR1 mutation in 1 (2%, 1/49), and 2 recurrent TGFBR2 mutations in 2 (4%, 2/49) were identified. No FBN2 mutation was found.

Less invasive surgical closure of patent ductus arteriosus in extremely low birth weight infants.

Minimally invasive surgery is widely used in pediatric surgery. Extremely low birth weight infants (ELBWI) are literally so fragile to surgical stress that the minimum invasive procedures should be required. We report 15 ELBWI cases with patent ductus arteriosus (PDA), who underwent surgical closure.

Neonatal sudden death due to Legionella pneumonia associated with water birth in a domestic spa bath.

We report the first case of neonatal Legionnaires' disease associated with water birth in a spa bath at home. Legionella pneumophila serogroup 6 was detected from postmortem lung tissue.