Incidence and Relapse Triggers of Childhood Idiopathic Nephrotic Syndrome between 2006 and 2016: A Population-Based Study in Fukushima, Japan.

Childhood idiopathic nephrotic syndrome (NS) is defined by proteinuria and hypoproteinemia. The incidence of childhood idiopathic NS varies with age, race, residential areas, and social conditions. In Japan, its incidence was estimated to be 6.49 cases/100,000 children. Our study aimed to investigate the incidence, characteristics, and rate of relapse of idiopathic NS in Fukushima between 2006 and 2016. Overall, 158 children aged from 6 months to 15 years old (65.8% male) developed idiopathic NS (median age at onset, 5.3 years). The peak age at onset was three years. The average annual incidence of childhood idiopathic NS was 5.16 (range, 3.47-9.26) cases/100,000 children. The highest incidence was in 2011, which was the year of the Great East Japan Earthquake and nuclear power plant accident, and reportedly caused psychological distress in the children at the time. Conversely, the five-year birth cohort showed minor difference from 2008 to 2012. The rate of incidence in males aged < 5 years was thrice greater than in females of the same age and almost the same for males and females aged 11-15 years. Of 507 total relapses in 115 NS children, common triggers of relapses were steroid discontinuation or reduction and infection. The average annual incidence of childhood NS based on the Fukushima population was lower than previously reported in Japan, and the annual incidence has changed over an 11-year period. These changes may be affected by social or environmental factors, including mental stress associated with lifestyle changes after the disaster.

Prenatal nicotine exposure affects cardiovascular function and growth of the developing fetus.

AIM: Effects of nicotine on fetal hemodynamics are not well known, especially in the first trimester fetus. We investigated the acute and chronic effects of nicotine on hemodynamics in pregnant mice and their fetuses using ultrasound. Postnatal health status including growth and hemodynamics was also examined. METHODS: To investigate the acute effects of nicotine on fetal hemodynamics, we injected nicotine 0.2 mg/kg subcutaneously into pregnant mice on gestational days (GD) 9.5, 11.5 and 13.5 and compared with saline-injected group. To determine the chronic effects of nicotine on fetal hemodynamics, we administered nicotine in drinking water (0.1 mg/mL) to pregnant mice from GD 6.5 until they gave birth and compared hemodynamics with water-administered mice. RESULTS: Regarding the acute effects of nicotine, we found no intergroup difference in maternal hemodynamics; however, fetal blood flow through the dorsal aorta, carotid artery and umbilical artery tended to decrease, particularly on GD 11.5. Regarding the chronic effects of nicotine, we observed no intergroup difference in maternal body weight changes and hemodynamics; however, blood flow to all fetal organs tended to be lower in the nicotine water group than in the water group with significant difference on GD 13.5. The offspring of the nicotine water group had significantly low birth weights and continued to have low body weight until 9 weeks of age. In addition, these offspring developed postnatal cardiac hypertrophy. CONCLUSION: Nicotine adversely affects fetal hemodynamics acutely and chronically in early pregnancy, potentially leading to fetal tissue hypoxia, intrauterine growth restriction and adverse postnatal health effects.

Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection.

Chronic active Epstein-Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis-like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11-year-old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted. Two years after the PAH symptoms appeared, CAEBV was evident, based on deteriorated liver function, hepatosplenomegaly, and coronary artery aneurysms. CAEBV should be considered as a cause of secondary PAH, particularly when liver dysfunction coexists.

Outcome of fetal echocardiography: a 17 year single-institution experience in Japan.

BACKGROUND: The aim of this retrospective study was to evaluate the influence of prenatal diagnosis on perinatal outcomes of congenital heart disease (CHD) over a 17 year period at a single center. METHODS: The perinatal outcome of CHD in 146 patients diagnosed on fetal echocardiography between 1994 and 2010 were reviewed. The characteristics of 193 neonatal inpatients with CHD treated at the authors' department between 2001 and 2010 were also analyzed; among the inpatients, 61 were diagnosed before birth (prenatal group) and 132 were diagnosed after birth (postnatal group). RESULTS: Among the 146 patients prenatally diagnosed with CHD, the prenatal mortality, including abortion and stillbirth, decreased from 1994 to 2010. Among the 193 neonatal inpatients, the prenatal group had lower gestational age and bodyweight than the postnatal group. Further, the prenatal group had lower blood pH at admission, but no patient in that group experienced ductal shock, although six patients in the postnatal group did. The average dose of prostaglandin E1 used in duct-dependent CHD was significantly lower in the prenatal group than in the postnatal group (3.4 vs. 4.6 ng/kg per min; P = 0.015). CONCLUSIONS: Prenatal diagnosis of CHD enables planned labor, prevents ductal shock, and reduces prostaglandin E1 side-effects and medical expenditure.

Increasing the accuracy of lung perfusion scintigraphy in children with bidirectional Glenn circulation.

BACKGROUND: In children who have undergone a bidirectional Glenn procedure without antegrade or additional pulmonary blood flow, we have often noted a discrepancy between apparent lung perfusion on scintigraphy and superior vena cava angiography when evaluating right and left pulmonary blood flow. We found a tendency for radionuclide, tracer 99mTc-MAA, when administered through a single upper extremity vein, to preferentially accumulate in the ipsilateral lung. OBJECTIVE: In the present study, we examined whether the ratio of right-to-left pulmonary flow varied when 99mTc-MAA was administered via either the right upper or the left upper extremity vein. MATERIALS AND METHODS: We studied six children (median age 1.3 ± 0.23 years) who underwent a bidirectional Glenn before total cavopulmonary connection. Five children who underwent biventricular repair served as a control. Perfusion scintigraphy using 99mTc-labeled macroaggregated albumin (99mTc-MAA) was performed in all children. First, we injected radionuclide via the right upper extremity and calculated the pulmonary accumulation in both lungs (R-image). Second, we injected the same dose of radionuclide via the left upper extremity and calculated the pulmonary accumulation (B-image), which represented the resulting administration via both upper extremities. The lung accumulation that resulted from radionuclide administration via the left upper extremity (L-image) was determined by subtracting the R-image from the B-image. We evaluated the right-to-total pulmonary blood flow ratio (radionuclide accumulation in right lung / radionuclide accumulation in both lungs) in the R-, L- and B-images. RESULTS: The right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 815 ± 15.3%, 39.8 ± 11.7% and 61.3 ± 11.8%, respectively, and there were significant differences among the three images (P < 0.01). On the other hand, in the control group, the right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 59.3 ± 22.4%, 57.8 ± 26.4% and 58.8 ± 23.7%, respectively, and there was no significant difference. CONCLUSION: In children with bidirectional Glenn circulation without antegrade or additional pulmonary blood flow, the venous blood of each arm tends to flow into the ipsilateral lung. The administration of radionuclide via both arms is important for accurate evaluation of lung perfusion scintigraphy in children who have undergone a bidirectional Glenn procedure.

Gonadal mosaicism of a TAZ (G4.5) mutation in a Japanese family with Barth syndrome and left ventricular noncompaction.

TAZ (G4.5) was initially identified as the gene associated with Barth syndrome and left ventricular noncompaction (LVNC). The purpose of this study was to investigate patients with LVNC for disease-causing mutations in TAZ. In 124 Japanese patients, including 50 families, mutation analysis of TAZ was performed using DNA sequencing. A splice donor mutation was identified in two brothers with Barth syndrome and LVNC, and a sister who was asymptomatic. However, the variant was not identified in either parent or the maternal grandparents, all of whom were asymptomatic. Due to the recurrent inheritance of this variant by each of the children we concluded that this was evidence of gonadal mosaicism in the obligate carrier mother, the first reported occurrence of this in Barth syndrome.

[A study on measles epidemic in Sukagawa area, Fukushima Prefecture from 2002 to 2003].

Measles epidemic occurred in southern part of Fukushima Prefecture from April 2002 to July 2003. Public Iwase hospital in Sukagawa City was the central hospital in the measles epidemic area and 382 patients with measles were admitted to the hospital during the epidemic. Based on clinical records, age distribution, vaccination history, familiar infection and complications were retrospectively investigated. Moreover, the vaccination rates and their calculating methods in the area were compared and the problems on immunization against measles were discussed. As the result, we found that 1) measles epidemic centered on unvaccinated infants, 2) measles was still serious disease with many complications and sometimes fatal, 3) measles was highly infectious and there was no way for prevention except vaccination, 4) the actual condition of measles vaccination was not reflected exactly by the current calculating methods used for the local health reports by the Ministry of Health Labour and Welfare. For the prevention of measles epidemic, it is necessary to know the exact numbers of patients infected with measles and patients deceased due to measles, to calculate precise vaccination rate, and to form the consensus among parents, physicians and administrators to control measles epidemic by the vaccination.