Should a percutaneous dilational tracheostomy be guided with a bronchoscope?
Our data about PDT in critically ill patients do not indicate any clear-cut difference in complication rates or practicability in the absence of bronchoscopic guidance when adequate skills and experience have been acquired and simple but effective precautions at each step are adopted. However, randomisation and long-term laryngotracheal followup should be considered in future studies.
Our results suggest that HMGCR loss-of-function primarily affects postmigratory cranial neural crest cells through abnormal Sonic Hedgehog signaling, probably induced by reduction in metabolites of the cholesterol pathway. Malformation severity correlates with the grade of HMGCR inhibition, developmental stage of its disruption, and probably with availability of maternal lipids. Together, our results might help to understand the spectrum of orofacial phenotypes found in cholesterol synthesis disorders. Birth Defects Research (Part A) 106:814-830, 2016. © 2016 Wiley Periodicals, Inc.