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Non-hepatic causes of hyperammonaemia are uncommon relative to hepatic aetiologies. An adolescent female was admitted to the hospital with a diagnosis of very severe aplastic anaemia. During her treatment with immunosuppressive therapy, she developed neutropenic enterocolitis, pseudomonal bacteraemia and hyperammonaemia. A combination of intermittent haemodialysis and high-volume continuous veno-venous haemodiafiltration (CVVHDF) was required to manage the hyperammonaemia. Despite a thorough investigation, there were no hepatic, metabolic or genetic aetiologies identified that explained the hyperammonaemia. The hyperammonaemia resolved only after the surgical resection of her inflamed colon, following which she was successfully weaned off from the renal support. This is a novel case report of hyperammonaemia of nonhepatic origin secondary to widespread inflammation of the colon requiring surgical resection in an immunocompromised patient. This case also highlights the role of high-volume CVVHDF in augmenting haemodialysis in the management of severe refractory hyperammonaemia. [ABSTRACT FROM AUTHOR]

Introduction: Pegaspargase can cause anti-asparaginase antibody formation, which can decrease its effectiveness without causing any clinically apparent reaction (silent inactivation). When a patient has silent inactivation, a switch to Erwinia anti-asparaginase is warranted, but there is currently a global shortage of Erwinia. The only way to identify silent inactivation is to measure an asparaginase level. However, routine asparaginase level monitoring is not currently standard of care at all Canadian centers. This study aims to identify variations in practice regarding asparaginase level monitoring and Erwinia use. Methods: A 21-item survey was developed using OPINIO software and distributed to all Pediatric Hematology–Oncologists in Canada from February to October 2020. Results: Respondents represented 15 hospitals across each region of Canada (response rate = 52%). Only 39.2% of respondents reported routinely measuring asparaginase levels, yet 53% of respondents have modified therapy from pegaspargase to Erwinia in up to half of their patients. The most common reason for not measuring asparaginase levels was not knowing how to use levels clinically (25.5%). There was variation in the timing of levels and their target. Conclusions: We identified substantial variation in asparaginase activity monitoring practices across Canada. Therefore, future research should aim to develop a national practice guideline on asparaginase activity monitoring. [ABSTRACT FROM AUTHOR]

ABSTRACT Aim Methods Results Conclusion Heavy menstrual bleeding (HMB) affects up to 37% of adolescents. Given the paucity of available tools to assess health‐related quality of life (HRQoL) in adolescents with HMB, we developed the adolescent menstrual bleeding questionnaire (aMBQ), a valid and reliable measure of bleeding‐related quality of life. The aim of this study was cross‐cultural translation and adaptation of the English aMBQ into French to ensure accessibility for more Canadian adolescents who menstruate.A five‐step process was followed: (1) forward translation from English to Canadian French; (2) backward translation from French to English; (3) review of source and translated aMBQ to create a reconciled version; (4) cognitive debriefing to ensure linguistic and clinical equivalence and (5) review of cognitive debriefings to produce the final version of the French aMBQ. Results of cognitive debriefings were reviewed after every three participants; items were revised if presented as an issue by ≥2 participants. These changes were implemented and tested in cognitive debriefings until saturation was reached.Linguistic changes were made to nine (33%) of the questions and one (3.7%) answer options. Major changes were made to four of the 27 questions (15%), and minor changes were made to five of the 27 questions (19%).Professional translators, clinical experts and patient input through cognitive debriefing are pivotal to successful cross‐cultural translation. Results of cognitive debriefing interviews suggest the French aMBQ is easily understood and confirms its face validity. [ABSTRACT FROM AUTHOR]

Background: Heavy menstrual bleeding (HMB) affects up to 37% of adolescents. Without recognition, HMB can lead to other medical conditions resulting in diminished health-related quality of life. WeThrive, a new mobile health (mHealth) app, implements the pictorial bleeding assessment chart to identify HMB, and the adolescent Menstrual Bleeding Questionnaire to measure the effects of HMB on adolescents' health-related quality of life. If HMB is identified, WeThrive will connect users to local clinics for further assessment of their menstrual bleeding with a health care provider., Objective: This study aimed to describe adolescents' experiences using WeThrive app., Methods: This qualitative study was approved by the local Research Ethics Board in Halifax, Nova Scotia, and informed consent was provided by all participants. Individual semistructured interviews were held via videoconference with adolescents younger than 18 years, who had at least 1 menstrual period and had used WeThrive at least once. Interview transcripts were thematically analyzed by 2 investigators (MP and NMN) independently, and the κ statistic was calculated to determine the strength of correlation in themes., Results: Five adolescents (mean age 15.5, range 13-18 years), participated in the interviews. All participants stated that WeThrive helps them better understand their menstrual periods by predicting period onset, recognizing menstrual symptoms, and identifying HMB. Four themes were identified: (1) the importance of visual features and usability, (2) newly obtained knowledge using WeThrive, (3) feature use depends on menstrual health, and (4) trustworthiness. There was substantial agreement on the identified themes (κ=0.73)., Conclusions: WeThrive is visually appealing, and trustworthy, and helps users better understand their menstrual periods, including identifying HMB. By identifying HMB early, WeThrive has the potential to improve the recognition of bleeding disorders and iron deficiency in adolescents. WeThrive is a useful tool to help adolescents better understand their menstrual periods., (©Nora MacNeil, Victoria Price, Meghan Pike. Originally published in JMIR Formative Research (https://formative.jmir.org), 03.10.2024.)