: The novel agent pd-FVIIa/FX is a 1 : 10 protein weight mixture of activated factor VII (FVIIa) and factor X (FX) derived from donated blood plasma. A phase III clinical trial of pd-FVIIa/FX revealed high efficacy for bleeding episodes in haemophilia patients with inhibitors. However, up to now, only one case of this new agent being used for surgery had been reported. The objective of this study is to evaluate the perioperative haemostatic efficacy and safety of pd-FVIIa/FX in haemophilia patients with inhibitors. We retrospectively reviewed 25 operation charts from 14 haemophilia patients with high-responding inhibitors using pd-FVIIa/FX during the perioperative period. Efficacy was evaluated by attending physicians and results divided into four groups (excellent, good, fair, and poor). The operation chart was provided by nine Japanese medical institutes with expertise in haemophilia management. Out of the total of 25 surgical procedures, 44% (11/25) were classified as major surgery and the remainders were minor surgeries. In all of the surgeries but one, rFVIIa and/or APCC were administered in combination or sequential method. In all cases except one, the haemostatic efficiency rate was judged as excellent or good by treating physicians for an overall efficacy rate of 96%. No thrombotic adverse effects were reported. This study's results suggest that both combination and sequential therapy of pd-FVIIa/FX and other bypassing agents are well tolerated and effective for the control of perioperative bleeding in haemophilia patients with high-responding inhibitors.
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.
Abstract Background Examining the predictors of summative assessment performance is important for improving educational programs and structuring appropriate learning environments for trainees. However, predictors of certification examination performance in pediatric postgraduate education have not been comprehensively investigated in Japan. Methods The Pediatric Board Examination database in Japan, which includes 1578 postgraduate trainees from 2015 to 2016, was analyzed. The examinations included multiple-choice questions (MCQs), case summary reports, and an interview, and the predictors for each of these components were investigated by multiple regression analysis. Results The number of examination attempts and the training duration were significant negative predictors of the scores for the MCQ, case summary, and interview. Employment at a community hospital or private university hospital were negative predictors of the MCQ and case summary score, respectively. Female sex and the number of academic presentations positively predicted the case summary and interview scores. The number of research publications was a positive predictor of the MCQ score, and employment at a community hospital was a positive predictor of the case summary score. Conclusion This study found that delayed and repeated examination taking were negative predictors, while the scholarly activity of trainees was a positive predictor, of pediatric board certification examination performance.
Summary: Hidradenomas are relatively rare benign tumors in the dermis that differentiate into eccrine or apocrine sweat glands. They often present as round or oval nodules and vary in color. Generally, they occur in the head and neck region. Keloid scars are often red, elevated lesions that are caused by chronic inflammation in the reticular dermis. These scars demonstrate a preference for high skin-tension sites, including the scapular region. Herein, we describe a case of a dark red hidradenoma on the scapular region with a high incidence of acne surrounding the lesion area that was initially diagnosed as an acne-initiated keloid. However, local steroid injection did not cure the lesion. After excision, histopathology revealed typical findings for hidradenoma, namely mucinous, polygonal, and clear cell composition. In some cases, as presented it may be challenging for clinicians to differentiate between hidradenoma and keloid due to the similar clinical features. Thus, hidradenoma should be taken in consideration as a differential diagnosis when encountering steroid-refractory keloid-like lesions. Moreover, early biopsy or surgical resection should be considered.
Background: Blood culture contamination (BCC) leads to unnecessary interventions including hospitalization, antibiotic administration and additional laboratory tests. Previous studies in adults revealed that BCC was associated with unnecessary financial expenditures. However, information pertaining to the pediatric population is limited. Therefore, we investigated the details of the adverse economic impact associated with BCC in a pediatric emergency department (ED) in Japan. Methods: This study was a retrospective, observational study. We collected data on blood cultures performed in patients 18 years of age in a pediatric ED. Medical records of patients with positive blood cultures were reviewed, and the information regarding adverse events related to BCC was extracted. Medical costs related to BCC were estimated from the data. Results: In total, 13,139 sets of blood cultures were performed from April 2013 to June 2016, and 141 cases (1.1%) of BCC were identified. Among these, 106 patients (75%) experienced at least 1 adverse event associated with BCC. The total medical cost due to BCC was 4,076,713 Japanese yen. Multifaceted approaches targeting ED physicians including lectures on optimal blood collection methods and monthly feedback on BCC rates were effective in reducing the BCC rate and its related costs. Conclusions: Interventions associated with BCC were common and accounted for significant adverse economic impact on pediatric patients. Regular education and monitoring were effective in reducing BCC and its related costs.
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.
Abstract Tongue-base cysts, which are occasionally categorized as vallecular cysts, are a rare yet potentially life-threatening cause of stridor in pediatric patients. Studies reporting the use of point-of-care ultrasound (POCUS) to identify tongue-base cysts are lacking. We present the case series of four infants in whom tongue-base cysts were detected using neck POCUS.
