Expanding diversity in developmental profiles of very-low-birth-weight infants during 6 years after birth.

Very-low-birth-weight infants (VLBWIs) are at high risk for neurodevelopmental problems after age 3 years. We investigated the association between the developmental quotient (DQ) of VLBWIs and their growth profiles during 6 years after birth. Participants were VLBWIs born at Kokura Medical Center (the first cohort) and Kyushu University Hospital (the second cohort) between 2012 and 2017.

Kawasaki Disease and Vaccination: Prospective Case-Control and Case-Crossover Studies among Infants in Japan.

The causal effects of vaccines on Kawasaki disease (KD) remain elusive. We aimed to examine the association between vaccines administered during infancy and the development of KD in Japan. We conducted a multicenter prospective case-control study using questionnaires and compared the vaccination status of infants (age: 6 weeks to 9 months) who developed KD (KD group; = 102) and those who did not develop KD (non-KD group; = 139).

Clarithromycin Plus Intravenous Immunoglobulin Therapy Can Reduce the Relapse Rate of Kawasaki Disease: A Phase 2, Open-Label, Randomized Control Study.

Background: We previously reported that biofilms and innate immunity contribute to the pathogenesis of Kawasaki disease. Therefore, we aimed to assess the efficacy of clarithromycin, an antibiofilm agent, in patients with Kawasaki disease.

Methods And Results: We conducted an open-label, multicenter, randomized, phase 2 trial at 8 hospitals in Japan.

Outcomes of cardiac surgery in trisomy 18 patients.

Objective: The objective was to clarify the outcomes of cardiac surgery in trisomy 18 patients.

Patients And Methods: We analysed 34 consecutive trisomy 18 patients, of whom 21 were males, with cardiac complications. They were divided into patients who underwent cardiac surgery and those who were conservatively treated.

Longitudinal growth of the autologous vessels above and below the Gore-Tex graft after the extracardiac conduit Fontan procedure.

Objective: Reflecting excellent mid-term outcomes, extracardiac conduit Fontan procedure (ECFP) using Gore-Tex tube graft has been performed with increasing frequency in patients with functional single ventricle. Nevertheless, due to the lack of growth potential of the artificial conduit, the status of the venous pathway along with somatic growth is a continuing concern. In this study, we evaluated the longitudinal growth of the autologous vessels above and below the Gore-Tex graft used in the ECFP.

Mid-term follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure.

Objective: Extracardiac conduit Fontan procedure (ECFP) using Gore-Tex graft has been performed with increasing frequency for the patients with functional single ventricle. However, lack of growth potential and longevity of the conduit are consistent concerns and main points of criticism of the ECFP. In this study, we investigated the mid-term status of the Gore-Tex graft used in the ECFP by comparing the internal diameter of the graft with the inferior vena cava (IVC) diameter at 1 month and 5.

Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association.

Background: Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated.

Methods: Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005.

Central retinal artery occlusion following transcatheter balloon aortic valvuloplasty in an adolescent with aortic valvular stenosis.

A 12-year-old girl with aortic valvular stenosis underwent transcatheter balloon aortic valvuloplasty (BAV) using a femoral artery approach. Anticoagulation with heparin during the procedure was used. The patient noted sudden onset of concentric constriction of the visual field in the right eye 40 min after BAV.

Genetic analysis of MMP gene polymorphisms in patients with Kawasaki disease.

Kawasaki disease (KD) is an acute febrile disorder characterized by systemic vasculitis primarily occurring in coronary arteries. Matrix metalloproteinases (MMPs) have been considered to play pathophysiologic roles in the development of coronary artery lesions (CALs); therefore, an evaluation of the genetic contributions of the MMP genes to the development of CALs in KD patients would be beneficial for the prediction of CAL formation. We focused on the known functional single nucleotide polymorphisms (SNPs) in the MMP genes (MMP-2-735C>T, MMP-3-1612 5A/6A, MMP-9-1562C>T, MMP-12-82A>G, and MMP-13-77A>G) and performed the association study between these SNPs and CAL formation in KD.

Tissue inhibitor of metalloproteinase 2 and coronary artery lesions in Kawasaki disease.

Objective: To identify cytokine genes uniquely expressed in peripheral blood mononuclear cells (PBMNCs) in the acute phase of Kawasaki disease (KD) with coronary artery lesions (CALs).

Study Design: We screened the mRNA expression levels of PBMNCs from 4 pairs of KD patients with and without CAL using DNA microarray. The result was confirmed by real-time polymerase chain reaction (RT-PCR).

Auxiliary partial orthotopic living donor liver transplantation for a child with congenital absence of the portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the mesenteric vasculature in which visceral venous blood bypasses the liver, completely draining into the systemic circulation through a congenital porto-systemic shunt. Liver transplantation has rarely been indicated for patients with this disease. We present a child with CAPV who was managed successfully by living donor auxiliary partial orthotopic liver transplantation (APOLT), while preserving the right lobe of the native liver.

Magnetic resonance studies of brain lesions in patients with Kawasaki disease.

We evaluated brain lesions in patients with coronary arterial lesions (CAL) as a complication of Kawasaki disease (KD) by magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Among 47 patients who underwent coronary angiography for the evaluation of CAL due to KD at Kyushu University Hospital from April 1996 to September 2004, 24 patients were evaluated prospectively by brain MRI and MRA 0.1-21.

Association of vascular endothelial growth factor (VEGF) and VEGF receptor gene polymorphisms with coronary artery lesions of Kawasaki disease.

We analyzed the genetic polymorphisms of vascular endothelial growth factor (VEGF) and its receptors [Fms-related tyrosine kinase-1, kinase insert domain receptor (KDR)] in Japanese patients with Kawasaki disease (KD) and normal control subjects to examine whether these genes would contribute to the KD occurrence and/or the development of coronary artery lesion (CAL) in KD. We found that the frequency of G allele of VEGF g.-634 G>C single-nucleotide polymorphism in the promoter region was significantly higher in KD patients with CAL than in those without CAL (p = 0.

CD25+CD4+ regulatory T cells in patients with Kawasaki disease.

Objective: To investigate whether the CD25 + CD4 + regulatory T-cell population, which plays important roles not only in maintaining immunologic self-tolerance but also in controlling the magnitude and character of antimicrobial immune responses, is related to the pathophysiology of Kawasaki disease (KD).

Study Design: The patient group consisted of 54 patients (median age, 30 months; 27 female and 27 male patients) fulfilling the criteria for KD. Age-matched control subjects included 17 patients with active infections and 24 healthy children.

Inducible and endothelial constitutive nitric oxide synthase gene polymorphisms in Kawasaki disease.

Background: Nitric oxide (NO) is secreted by immune and vascular endothelial cells, and appears to play important roles in the pathophysiology of Kawasaki disease (KD). Thus, genetic variations in NO synthase (NOS) genes may be involved in the development of coronary artery lesions (CAL) in KD.

Methods: The present study investigated the association of endothelial constitutive NOS (ecNOS) and inducible NOS (iNOS) gene polymorphisms with the development of CAL in KD in a Japanese population.

Serum hepatocyte growth factor combined with vascular endothelial growth factor as a predictive indicator for the occurrence of coronary artery lesions in Kawasaki disease.

Unlabelled: We investigated the possible use of serum hepatocyte growth factor (HGF) and vascular endothelial growth factor (VEGF) levels as a predictive indicator for the occurrence of coronary artery lesions (CAL) in Kawasaki disease (KD). Serum HGF and VEGF levels were measured by enzyme-linked immunosorbent assay in 41 patients with KD and 25 afebrile controls. Serum HGF levels of patients in the acute phase of KD were significantly higher than those of afebrile controls (Pc < 0.

Central nervous system vasculopathy associated with neonatal lupus.

Neonatal lupus erythematosus, characterized mainly by congenital heart block and transient skin lesions, is usually self-limited. A patient with history of neonatal lupus erythematosus and congenital heart block developed central nervous system vasculopathy resembling moyamoya disease and hypertension at 17 years of age. Careful examination and follow-up of possible central nervous system complications later in life might be important in infants with neonatal lupus erythematosus.