Peer Learning Has Double Effects in Clinical Research Education: A Qualitative Study.

Background: Peer learning has been recognized for its effectiveness in health professional education. However, its effects on clinical research education are not clear and were explored qualitatively in this study. Methods: The peer-learning method was implemented in a clinical research education seminar for early-career physicians at a children's and mothers' hospital in 2019. We conducted semistructured interviews with participants about peer-learning experience and qualitatively analyzed verbatim transcripts using Engeström's "activity theory" framework. Results: From framework analysis, learning processes were extracted mainly in four domains, namely, (a) instrument and its usage: research design and its match with research question, (b) outcome: research result, (c) community: seminar, and (d) division of labor: roles of participants and staff. Conclusions: In this report of a peer-learning trial in postgraduate clinical research education, the following two pathways of peer-learning effects were abstracted. The indirect pathway was the presentations by experienced participants providing concrete examples of research processes. The direct pathway was the questions from experienced participants to beginners about specific and concrete questions. There were also two points to consider in peer learning in clinical research education: gaps in premise knowledge and beginners' frustration about expected outcomes. We believe that these extracted pathways and points imply the significance and considerations for continuing the peer-learning trial in clinical research education. Future tasks are to promote clinical research education with a view to the learning effects, not only on individuals, but also on groups.

Association between residents' scholarly activities and publication requirement policy.

BACKGROUND: Scholarship is recognized as important in residency training worldwide. The Japan Pediatric Society (JPS) enacted a reform in 2017 to require publication of an article as a prerequisite for taking the board certification test, with the goal of increasing scholarly activity. METHODS: The purpose of this study was to provide a detailed description of the trends in residents' scholarly activities related to the JPS reform. A secondary analysis was performed on the cross-sectional database of pediatrics residents who took the certification test in 2015-2018. RESULTS: The enrolled participants were 2399 residents of which 79.7% passed the test. Publication of any type of article increased significantly (21%-22% to 100%; 0.1 to 0.3/person-year) after the implementation of the JPS reform, whereas academic presentations did not (89% to 91%; 1.2 to 1.3/person-year), both in terms of the percentage of the number of those who created them and the average rate of research production. Not only Japanese articles (11%-13% to 49%-53%; 0.04 to 0.15-0.17/person-year) or case reports (10%-14% to 51%-52%; 0.03-0.05 to 0.16-0.17/person-year), but also English articles (4%-5% to 15%-16%; 0.01-0.02 to 0.05/person-year) and original articles (5% to 11%-17%; 0.01 to 0.03-0.05/person-year) increased significantly. The number of each type of article publication was correlated with success in the board certification test (odds ratio 1.5-1.8). CONCLUSIONS: Scholarly activities of pediatrics residents were enhanced by the JPS implementation of the article requirement policy, which is crucial to fostering a scholarly culture. The most efficient measures to promote scholarship need to be persistently investigated.

Long-term impact of overnight shiftwork implementation on pediatric residents' mental wellness: A repeated cross-sectional survey.

OBJECTIVES: The Japanese government partially enacted the "Work Style Reform Bill" in 2019. The National Center for Child Health and Development (NCCHD) introduced an Overnight Call Shift (OCS) system for pediatrician training. We conducted a follow-up survey in 2019 to investigate the long-term effectiveness of the OCS system to improve the pediatric residents' mental wellness at NCCHD. METHODS: We conducted a questionnaire-based cross-sectional survey for pediatric residents in 2019 to compare the data with those of the previous survey in 2012. The questionnaire includes demographic data, working conditions data, and mental wellness assessment by the Center for Epidemiologic Studies Depression scale (CES-D) and the Maslach Burnout Inventory (MBI). RESULTS: The collection rate for the 2019 survey was 94.5% (37 participants/39 eligible). Compared to 2012, there were no significant changes in demographic data and working hours, a significant increase by about 30% in residents who took daytime off after night work, about 10% decrease in residents who scored 16 and above on the CES-D, and a significant decrease in the mean score for depersonalization (DP) in the MBI. Multiple regression analyses showed that daytime off after night work was the decreasing factor for CES-D and Emotional exhaustion (EE). CONCLUSIONS: The overnight shiftwork system shortened the pediatric residents' duty hours somewhat, and imposed an impact on the pediatric residents' mental wellness.

Plasma exchange and infliximab as a third-line therapy for refractory infantile Kawasaki disease.

BACKGROUND: The treatment for Kawasaki disease (KD) patients refractory to intravenous immunoglobulin (IVIG) therapy is still controversial, and the efficacy of plasma exchange (PE) and infliximab (IFX) therapy for infantile KD is unknown. METHODS: A total of 22 infantile KD patients refractory to initial and additional IVIG, who received either PE or IFX as third-line therapy from October 2008 to February 2020 were examined retrospectively. The patients' sex, age, days of first IVIG, days of PE or IFX therapy, laboratory data preceding PE or IFX therapy, coronary artery lesions (CALs), and adverse effects were investigated. RESULTS: Thirteen patients received PE and nine patients received IFX as the third-line therapy. For the median age at onset, the median days of first IVIG and PE or IFX, and pre-PE or IFX therapy blood test results, there were no significant between-group differences. At admission, and before and after the third-line therapy, there were also no significant differences in occurrence of CALs. The frequency of serious adverse events was significantly higher in the PE group than in the IFX group. CONCLUSIONS: Although there were no significant differences in patient background, blood test results, or frequency of CALs, the frequency of adverse events was significantly higher in the PE group. With the trend of expansion of IFX therapy for KD patients refractory to IVIG, the role of PE as the additional therapy may become more limited.

The most severe form of LMNA-associated congenital muscular dystrophy.

Alterations in the LMNA gene cause a wide spectrum of diseases collectively called laminopathies. LMNA-associated congenital muscular dystrophy is a form of laminopathy, which usually causes infantile onset of muscle weakness, predominantly in the cervical-axial muscles, and motor developmental retardation. Cardiac symptoms during the first decade of life are rare. We report a case of LMNA-associated congenital muscular dystrophy in which the patient did not achieve head control and experienced facial muscle weakness. Cardiac dysrhythmias were observed at 5 years with development of dilated cardiomyopathy and ischemic strokes at 7 years. Despite intensive medical intervention, he died suddenly at 9 years. This report broadens the spectrum of phenotypes of this disorder with the most severe symptoms during the first decade of life. Our case underscores the need for early genetic testing for LMNA in patients with congenital muscular dystrophy to screen for cardiac manifestations and intervene as necessary.

Transient hypertension with urinary tract infection in congenital hydronephrosis.

Introduction: Hypertension is occasionally associated with congenital hydronephrosis. Case report: The authors report a four-month-old boy with severe left congenital hydronephrosis and transient hypertension triggered by his first urinary tract infection (UTI). Despite the satisfactory UTI clinical course, he suddenly developed severe hypertension (130/80 mmHg) on the ninth day of UTI. His aldosterone level was within the reference range and his plasma renin activity was slightly elevated. Although his hypertension was refractory to calcium channel blockers, an angiotensin-converting enzyme inhibitor was effective for hypertension. On day 24, he was able to discontinue the antihypertensive without organ damage. Conclusions: Although the precise cause of hypertension was unclear in our case, we considered it to be temporary renin-associated hypertension due to decreasing renal blood flow due to UTI in a patient with severe congenital hydronephrosis. In pediatric UTI, particularly in patients with unilateral hydronephrosis, blood pressure monitoring is very important.

Anti-complement factor H (CFH) antibodies and a novel CFH gene mutation in an atypical hemolytic uremic syndrome patient with complement activation of the classical pathway.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by overactivation of the complement alternative pathway. aHUS involves the presence of antibodies against complement factor H and its mutations in the complement genes. A 2-month-old boy presented with discoid rash, hemolytic anemia, thrombocytopenia, multiple antibodies, and hypocomplementemia with a very low level of C4 (< 3 mg/dL), indicating activation of the complement pathway, together fulfilling the systemic lupus erythematosus (SLE) criteria of the American College of Rheumatology at 5 months of age. However, most of these findings normalized spontaneously without any intervention. Further investigations revealed a high level of anti-complement factor H antibodies and a novel heterozygous missense mutation (p.Glu1172Ala, located in exon 22) in a complement gene, CFH. At 2 years of age, his SLE-like symptoms have not recurred, but hematuria and schistocytes were persistent. Eventually, aHUS was diagnosed rather than SLE. Our findings suggest that multiple antibody complex, including anti-complement factor H antibody, may temporarily activate the classical pathway, resulting in SLE-like findings.

Slow Elevation in Protein C Activity without a PROC Mutation in a Neonate with Intracranial Hemorrhage.

Severe protein C (PC) deficiency leads to purpura fulminans and stroke in newborns. However, the clinical impact of plasma PC activity on the development of neonatal cerebral disease remains elusive. We report a case of hemorrhagic stroke associated with neonatal asphyxia and severe PC deficiency. Plasma PC and protein S activity 7 days after birth was 12% and 43%, respectively. No PROC mutation was found. PC levels did not exceed 20% until 2 months of age, even in the absence of consumption coagulopathy or vitamin K deficiency. Neither thromboembolic nor hemorrhagic events occurred during the infusion of activated PC concentrate (twice weekly, up to 68 days after birth). The PC activity levels gradually increased to the standard value for age by 9 months of age. The present case showed that neonatal PC deficiency without a PROC mutation caused an intracranial hemorrhage before a slow increase in PC activity.

Acquired idiopathic thrombotic thrombocytopenic purpura successfully treated with intravenous immunoglobulin and glucocorticoid: A case report.

RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level. INTERVENTIONS & OUTCOMES: Fresh frozen plasma was initially effective. Prednisolone and immunoglobulin resolved the condition with no adverse effects and rendered plasma exchange unnecessary. LESSONS: Compared with biological agents, immunoglobulin is cost-effective, readily available, and has a proven long-term safety record, making it a possible treatment option for acquired thrombotic thrombocytopenic purpura.

Complete atrioventricular block as a complication of varicella in children.

Although varicella is a benign and self-limited disease in children, serious complications can occur. We herein report a case of a 15-month-old boy who required a permanent pacemaker because of complete atrioventricular block as a complication of varicella. Universal vaccination is warranted to prevent such a potentially fatal complication in Japan where varicella is still endemic.