[Onboarding of young professionals in the intensive care unit : Nationwide online survey by young DIVI - an initiative of the German interdisciplinary association for intensive and emergency medicine (DIVI)]. / Einarbeitung junger Fachkräfte auf der Intensivstation : Eine bundesweite Onlineumfrage der Jungen DIVI ­ einer Initiative der Deutschen Interdisziplinären Vereinigung für Intensiv- und Notfallmedizin (DIVI).

BACKGROUND: Onboarding of junior staff in the intensive care unit is vital to ensure high-quality critical care treatment. This process depends on beginner's training. AIM: We aimed to determine structure and duration of intensive care onboarding and the job satisfaction of junior professionals in German intensive care units. MATERIALS AND METHODS: We conducted an anonymous, interprofessional online survey regarding quality of onboarding and job satisfaction among young professionals. RESULTS: A total of 554 young professionals participated, about two thirds were physicians. A written concept was used in 59% of the nurse's and 27% of physicians' training. Median duration of training before taking full charge of patient treatment was 30 days among nurses and 7 days among physicians. About one third of nurses and 17% of physicians stated that they were sufficiently prepared after the training period, whereby 49% of physicians often felt overwhelmed. More than 42% can imagine working in critical care longer than the next 3 years. CONCLUSION: Fundamental methods for training of critical care professionals starting their intensive care career are underused in Germany and the duration of training blatantly differs from national guideline recommendations. Although there seem to be deficits concerning material and staff resources, participants are satisfied with learning progress and teamwork.

A rare PALB2 germline variant causing G2/M cell cycle arrest is associated with isolated myelosarcoma in infancy.

BACKGROUND: Isolated myelosarcoma of infancy is a rare presentation of acute myelogenous leukaemia (AML). Because of its rarity and early onset in infancy underlying genetic predisposition is potentially relevant in disease initiation. METHODS AND RESULTS: We report an oncologic emergency in an infant with thoracic and intraspinal aleukaemic myeloid sarcoma causing acute myelon compression and lower leg palsy. Whole-exome sequencing of the patient's germline DNA identified a rare PALB2 (OMIM 610355) variant (p.A1079S), which is located in a domain critical for the gene's proper function within the homology-directed repair pathway. In line with potential DNA damage repair defects mediated by the PALB2 deregulation, the patient's fibroblasts showed increased sensitivity towards radiation and DNA intercalating agents. CONCLUSION: Therefore, we suggest PALB2 p.A1079S as a pathogenic variant potentially contributing to the here observed patient phenotype.