Yukihiko Kawasaki

C3 glomerulopathy (C3G) is a type of complement-associated glomerulopathy caused by a defect in the alternative complement pathway and is regarded as a rare disease. We report a 14-year-old boy with C3G and Sjögren’s syndrome (SS) whose school urinary screening revealed abnormalities, but the diagnosis was delayed because he refused to consult a medical institution. Histopathological examination revealed histologic features of membranoproliferative glomerulonephritis. He was diagnosed with C3G and Sjögren’s syndrome based on proteinuria, the histopathological findings, high levels of antibodies against SS-A, and lymphocytic infiltration around the salivary glands. He was treated with combination therapy, consisting of methylprednisolone pulse, prednisolone, and mycophenolate mofetil. At 3 months after treatment, urinary protein excretion was decreased and hematuria had disappeared, and improvement in hypocomplementemia and the levels of antibodies against SS-A were observed. The course of our case suggests that the management of patients with C3G should take into consideration the possibility of the coexistence of SS, and that those with positive findings on school urine screening should be encouraged to visit a medical institution.

Aim:To clarify the frequency and characteristics of patients with attention deficit hyperactivity disorder (ADHD) born with low birth weight (LBW), very low birth weight (VLBW), small for gestational age (SGA), or preterm, we examined the clinical characteristics of ADHD patients. Methods:We collected data for 168 ADHD children, and the patients were retrospectively divided into each group according to birth weight, height, and gestational age. Results:1) Patients with ADHD born with LBW, VLBW, SGA, or preterm accounted for 10.1%, 3.0%, 3.6%, or 6.5% of all patients, respectively. 2) ADHD-RS scores for hyperactivity disorder and the frequency of patients requiring ADHD drugs in the LBW infant group were higher than those in the non-LBW infant group. 3) ADHD-RS scores for hyperactivity disorder and total in the VLBW infant group were higher than those in the non-VLBW infant (birth weight:1,500-2,500 g) group. 4) The ADHD scores for attention-deficit and trouble score at the most recent follow-up patients born with SGA were higher than for non-SGA patients. Conclusions:These results suggest that it is necessary to pay attention to the appearance of neurodevelopment disorders such as ADHD when monitoring the progress of patients born with LBW or VLBW, and it is important to provide more appropriate management and therapeutic guidance for patients born with VLBW or SGA.

To clarify the characteristics of children with attention deficit hyperactivity disorder (ADHD) requiring early drug therapy and changes in ADHD treatment over time, we undertook a clinical survey of ADHD children. We retrospectively classified 168 children with ADHD into groups based on the presence [Group 1, (the early treatment Group A, non-early treatment Group B)] or absence (Group 2) of ADHD drug therapy. The older age at first examination and the frequency of patients born with low birth weight in Group 1 were higher than those in Group 2. The ADHD-Rating Scale (ADHD-RS) scores for total (ADHD-RS-T) and trouble scores (TS) in Group 1 were higher than those in Group 2. All patients requiring early drug medication were diagnosed with ADHD at age 6 or older and showed significantly higher ADHD-RS hyperactivity disorder (ADHD-RS-H) and TS. Guanfacine was the main drug prescribed, being the first-line drug in 45.0% of cases. These results suggest that characteristics of patients requiring early ADHD drug medication include age 6 years or older at first examination and high ADHD-RS-H, ADHD-RS-T and TS. Furthermore, an increase in the frequency of prescriptions for guanfacine was observed as a prescription trend for ADHD medications.

Background: The objectives of this study were to determine the longer‐term trends in childhood obesity and glucose metabolism abnormalities among residents of Fukushima Prefecture 5 years after the Great East Japan Earthquake.Methods: We evaluated the changes in height, weight, body mass index (BMI), BMI SD score, fasting plasma glucose (FPG) concentration, and hemoglobin A1c (HbA1c) among elementary and junior high school residents who had lived in the evacuation zone between 2011 and 2015.Results: Of the residents, 11,112 received health checks in 2011, while in 2012, 2013, 2014, and 2015, 5,737, 4522, 4297 and 3405 received health checks, respectively. The mean BMI SD score for all participants in 2011 was 0.149, and this score gradually decreased from 2011 to 2015. FPG levels and HbA1c levels for all participants with a BMI value +2SD or more in 2011 were higher than those in residents with a BMI value of less than +2SD. The frequency of participants with a FPG level of 126 mg/dl or more and the frequency of participants with a HbA1c level of 6.5% or more in 2011 were higher than those in 2012, 2013, and 2015.Conclusions: These results suggest that a number of pediatric residents suffered from obesity and glucose metabolism abnormalities. However, the longer‐term observations indicated an improvement in obesity and glucose metabolism abnormalities. There was a strong association observed between obesity and glucose metabolism, thus, it is important to continue with health checks for children with obesity and strive to improve their health.

Aim: To clarify whether the response to treatment of IgA nephropathy (IgAN) differs depending on patient age, we examined the response to treatment according to age of onset in children with IgAN. Methods: We collected data for 44 children with severe IgAN. The children were retrospectively divided into three groups based on their age at disease onset. Group 1 consisted of 24 children under 11 years old, group 2 consisted of 9 children aged 12 to 13 years, and group 3 consisted of 11 children aged over 14 years old. The clinical features and prognosis were analyzed for each group. Results: The urinary protein excretion and serum IgA values in group 3 were higher than those in groups 1 and 2 at the most recent follow up, and histological findings showed that the MESTCG scores in group 3 were higher than those in group 1. Furthermore, the incidence of patients with persistent nephropathy or renal insufficiency in group 3 was higher than those in groups 1 and 2. Conclusions: Patients aged 14 years and older with IgAN may respond poorly to treatment compared with those younger than 14 years old. Therefore, care must be taken regarding response to treatment and relapse when treating older children.

BackgroundThe Great East Japan Earthquake and the Fukushima Daiichi nuclear disaster forced the evacuation of residents and led to many changes in lifestyle for the evacuees. The Comprehensive Health Check was implemented to support the prevention of lifestyle-related disease and we analyzed the effect of prolonged evacuation (average of 3.0 years) on the new onset of hyper-LDL cholesterolemia.MethodsThe study participants were Japanese adults living near the Fukushima Daiichi nuclear power plant in Fukushima Prefecture. Annual health checkups focusing on metabolic syndromes were conducted for persons ≥ 40 years by the Specific Health Checkup. Based on data from annual checkups from 2011 or 2012, we followed 18,670 non-hyper-LDL cholesterolemia who underwent at least one other annual checkup during 2013-2015.ResultsWe found that the new onset of hyper-LDL cholesterolemia was significantly by 31% higher in evacuees than in non-evacuees. Evacuees had a significantly higher prevalence of obesity, hypertension, and diabetes, and higher frequency of weight change. Furthermore, logistic regression model analysis showed that the evacuation was significantly associated with the new onset of hyper-LDL cholesterolemia after adjusting age, gender, body mass index (BMI), smoking habit, alcohol consumption, diabetes, weight change, sleep deprivation, and exercise.ConclusionsThe findings of the present study suggest that prolonged evacuation after a disaster is a risk factor for the new onset of hyper-LDL cholesterolemia, and lead to an increase in cardiovascular disease. It is therefore important to follow-up evacuees and recommend lifestyle changes where necessary.

Background IgA nephropathy is a typical chronic glomerulonephritis that tends to occur in childhood. Method We reviewed the report on pathogenesis, treatment strategy with multidrug therapy and tonsillectomy pulse therapy for childhood-onset severe IgA nephropathy to clarify the pathophysiology and treatment of IgA nephropathy in childhood. Results In recent years, it has been found that the pathogenesis at onset is associated with aberrant glycosylation at the IgA1 hinge. Given this genetic background, the aberrantly glycosylated IgA1immune complex produced by antigen-stimulated T cells and B cells is deposited in the glomeruli. Inflammation is induced via activation of the complement, macrophages and mesangial cells, and glomerular damage progresses thereafter. Treatment is selected according to the severity of IgA nephropathy. In order to prevent the development of renal damage, it is important to control the associated immune responses. For severe IgA nephropathy, in particular, multidrug therapy with prednisolone, immunosuppressants, and angiotensin enzyme synthesis inhibitors and tonsillectomy methylprednisolone pulse therapy are now performed- and, as a result, the number of renal deaths has decreased and the long-term prognosis has improved. Conclusion The prognosis of IgA nephropathy is improving. In the future, it will be important to develop a treatment method that takes into consideration the fact that children are in their growth and development stage and, therefore, seeks to minimizes side effects.

Background: The survival rate of children with cancer has increased substantially in recent years. Shared decision making (i.e., the ability of children with cancer to express their will and share it with medical personnel) has become a particularly important issue. The nature and developmental processes of children’s decision making in hospital should be understood. There is, however, a lack of research in this area. Methods: From January 2016 to March 2018, we conducted a longitudinal qualitative observational study, within the context of medical anthropology, in a hospital pediatric ward in Japan. We investigated the nature and development of decision making among seven children aged 5–12 years with hematologic cancers. We recorded their everyday behaviors, interactions, narratives, and events in the ward. The recording was conducted systematically and it was analyzed thematically using both variable‐oriented and process‐oriented modes to assess causal relationships between phenomena. Results: The thematic analysis identified three thematic scenes in which children developed their will regarding cancer treatment: (1) adjusting to hospital life; (2) forming friendships with other children; and (3) communicating with medical personnel. Sharing information, building trusting relationships, and sharing treatment goals with medical personnel were identified as forms of children’s participation in medical decision making. Through cultivated friendships, children’s peer groups were sources of resilience and strength in overcoming difficulties in hospital life. Conclusions: The development of children’s decision making in a pediatric oncology ward was based on various rich human relationships. Such relationships should be promoted to improve shared decision making substantially.