ジンエン シンコウ ニオケル サイボウガイ キシツ レセプター インテグリン ノ ヤクワリ ト ソノ キノウ セイギョホウ

Sustained mesangial cell-mediated abnormal mesangial extracellular matrix (ECM) remodeling is the central biologic feature of progressive glomerulonephritis (GN) leading to glomerular sclerosis. Therefore, elucidating the molecular and cellular mechanisms involved in pathological mesangial remodeling is essential to understand the pathogenesis of progressive glomerular sclerosis. Accumulating evidences indicate that β1 integrin family regulate physiological and pathological ECM remodeling (fibrosis) in various organs. This overview will focus on our recent understanding of pathophysiological role of β1 integrin family in the progression of GN. Finally, we discuss the strategies targeting the integrin-mediated signaling pathway that permit the development of new therapeutic methods for inhibition of pathological ECM remodeling in GN

ショウニ ノ マンセイ ジンゾウビョウ CKD ノ ビョウタイ ト チリョウ センリャク

The progressive nature of chronic kidney disease（CKD）is well known and CKD occurs in allages groups, including children. Problem is that the patients with CKD have an increased risk ofnot only end-stage renal disease（ESRD）, but poor cardiovascular outcomes and death in both childrenand adults. It was estimated there are about 20.0 million japanese with CKD. Althoughthere is not definitely defined about correct number of pediatric patients and onset time of CKD, itis assumed that much number of pediatric CKD often carried over adult ages. Furthermore, mostof children with CKD have several problems such as the limitation of social activities, growth failure,cognitive impairment and mental fatigue. Therefore, the establishment of screening approachfor detecting pediatric CKD and appropriate treatment aiming the remission of CKD isrequired.This article aims to give a short review of school urinary screening system in Tokushima prefectureand treatment strategy for children with CKD, especially IgA nephropathy

ガッコウ ケンシン ニオケル ニョウ ケンサ : ショウニカ ノ タチバ カラ

Since 1974, the school urinary screening system in japan has been established to detectand treat earlier the pediatric patients with asymptomatic glomerlar diseases. It is wellknown that this project has contributed to improve not only the understanding ofpathophysiology in glomerular diseases, but the diagnosis, treatment and prognosis ofglomerular diseases in children. Of note, the recent data showed the decrease in thenumber of pediatric patients and adults patients from age 20 to 40, who have been treatedwith dialysis, supporting the substantial merit of this screening system. This reviewsummarized the details of renal diseases detected by school urinary screening and thenexplained the practical use of a revised manual of school urinary screening systemperformed in Tokushima prefecture for better management and treatment in children withabnormal urinary findings

RV Stiffness and Relaxation in PAH

We hypothesized that the kinematic model-based parameters obtained from the transtricuspid E-wave would be useful for evaluating RV diastolic property in pediatric pulmonary arterial hypertension (PAH) patients. The model was parametrized by stiffness/elastic recoil k, relaxation/damping c, and load x. These parameters were determined as the solution of m⋅d2x/dt2 + c⋅dx/dt + kx = 0, which is based on the theory that the E-wave contour is determined by the interplay of stiffness/restoring force, damping/relaxation force, and load. The PAH group had a significantly higher k and c versus the control group (182.5 ± 72.4 g/s2 vs. 135.7 ± 49.5 g/s2, p = 0.0232 and 21.9 ± 6.5 g/s vs. 10.6 ± 5.2 g/s, p <0.0001, respectively). These results show that RV has a higher stiffness/elastic recoil and inferior cross-bridge relaxation in the PAH group. Present findings indicate the feasibility and utility of kinematic model parameters for assessing RV diastolic function

A novel index equivalent to the myocardial performance index for right ventricular functional assessment in children and adolescent patients

The aims of the present study were to develop and check the utility and feasibility of a novel right ventricular (RV) functional index (RV angular velocity; RVω, s−1) derived from the angular velocity in harmonic oscillator kinematics obtained from the RV pressure waveform. We hypothesized that RVω reflects the myocardial performance index (MPI), which represents global RV function. A total of 132 consecutive patients, ranging in age from 3 months to 34 years with various cardiac diseases were included in this prospective study. RVω was defined as the difference between the peak derivative of pressure (dP/dt_max − dP/dt_min) divided by the difference between the maximum and minimum pressure (Pmax – Pmin). RVω showed significant negative correlations with the pulsed-wave Dopplerderived myocardial performance index (PWD-MPI) and the tissue Doppler imaging-derived MPI (TDI-MPI) (r = −0.52 and −0.51, respectively; both p < 0.0001). RVω also showed significant positive correlations with RV fractional area change (RVFAC) and RV ejection fraction (RVEF) (r = 0.41 and 0.39, respectively; both p < 0.0001), as well as a significant negative correlation with tricuspid E/e′ (r = −0.19, p = 0.0283). The clinical feasibility and utility of RVω for assessing global RV performance, incorporating both systolic and diastolic function, were demonstrated

OCT for observation of vasa vasorum

Background Hypoxia and low pulmonary arterial (PA) blood flow stimulate the development of systemic-to-pulmonary collateral blood vessels, which can be an adverse factor when performing the Fontan operation. The aim of this study was to use optical coherence tomography (OCT) to elucidate the morphological changes in PA vasculature after creation of a bidirectional cavopulmonary connection (BCPC) in children. Methods This prospective study evaluated PA wall thickness and development of PA vasa vasorum (VV) in the distal PA of eight patients (BCPC group, 1.3 ± 0.3 years) and 20 age-matched children with normal pulmonary artery hemodynamics and morphology (Control group, 1.4 ± 0.3 years). VV development was defined by the VV area ratio, defined as the VV area divided by the adventitial area in cross-sectional images. Results There was no significant difference in PA wall thickness between the BCPC and control groups (0.12 ± 0.03 mm vs. 0.12 ± 0.02 mm, respectively). The VV area ratio was significantly greater in the BCPC group than in the Control group (14.5 ± 3.5% vs. 5.3 ± 1.6%, respectively; p<0.0001). Conclusion OCT is a promising new tool for evaluating PA pathology, including the development of VV in patients after BCPC

PA compliance using harmonic oscillator kinematics

We hypothesized that KPA, a harmonic oscillator kinematics-derived spring constant parameter of the pulmonary artery pressure (PAP) profile, reflects PA compliance in pediatric patients. In this prospective study of 33 children (age range=0.5–20 years) with various cardiac diseases, we assessed the novel parameter designated as KPA calculated using the pressure phase plane and the equation KPA=(dP/dt_max)2/([Pmax – Pmin])/2)2, where dP/dt_max is the peak derivative of PAP, and Pmax – Pmin is the difference between the minimum and maximum PAP. PA compliance was also calculated using two conventional methods: systolic PA compliance (sPAC) was expressed as the stroke volume/Pmax – Pmin; and diastolic PA compliance (dPAC) was determined according to a two-element Windkessel model of PA diastolic pressure decay. In addition, data were recorded during abdominal compression to determine the influence of preload on KPA. A significant correlation was observed between KPA and sPAC (r=0.52, P=0.0018), but not dPAC. Significant correlations were also seen with the time constant (τ) of diastolic PAP (r=-0.51, P=0.0026) and the pulmonary vascular resistance index (r=-0.39, P=0.0242). No significant difference in KPA was seen between before and after abdominal compression. KPA had a higher intraclass correlation coefficient than other compliance and resistance parameters for both intra-observer and inter-observer variability (0.998 and 0.997, respectively). These results suggest that KPA can provide insight into the underlying mechanisms and facilitate the quantification of PA compliance

Pulmonary annular motion velocity in repaired CHD

Right ventricular (RV) dysfunction is generally evaluated using analyses of tricuspid annular motion. However, it represents only one aspect of RV performance. Whether measuring pulmonary annular motion velocity could serve as a novel way to evaluate global RV and/or RV outflow tract (RVOT) performance in pediatric congenital heart disease (CHD) patients with surgically repaired RVOT was evaluated. In this prospective study, tissue Doppler-derived pulmonary annular motion velocity was measured in children (aged 2-5 years) with RVOT reconstruction (RVOTR group, n = 48) and age-matched healthy children (Control, n = 60). The types of RVOTR procedures were as follows: pulmonary valve-sparing procedure (PVS, n = 7); transannular patch with monocusp valve reconstruction (TAP, n = 29); and RV-to-PA conduit reconstruction using a pericardial valve with expanded polytetrafluoroethylene conduit (Rastelli, n = 12). Pulmonary annular motion velocity waveforms comprised systolic bimodal (s1’ and s2’) and diastolic e’ and a’ waves in all participants. The peak velocities of s1’, s2’, e’, and a’ were significantly lower in the RVOTR group than in the control group (all p < 0.0001). Furthermore, these parameters depended significantly on the type of surgical procedure. The peak velocities of s1’, s2’, and e’ had significant correlations with RVOT ejection fraction (RVOT-EF) (r = 0.56, 0.49, and 0.34, respectively) and RVOT fractional shortening (RVOT-FS) (r = 0.72, 0.55, and 0.41, respectively), although there were no significant correlations between pulmonary annular motion and global RV function, including RV ejection fraction (RVEF) and RV fractional area change (RVFAC) in the assessment of all RVOTR group patients. The pulmonary annular motion parameters in the PVS group had significant correlations with both global RV and RVOT performance. The TAP group showed significant correlations between RVOT function and pulmonary annular motion. The Rastelli group showed almost no significant correlations between RV/RVOT function and tissue Doppler parameters. Pulmonary annular motion velocity is a simple, rapid, reproducible, and useful method of assessing RVOT function in children with surgically repaired CHD