EIF2AK4 and Pulmonary capillary hemangiomatosis: Patient #17 (Mussa 2021) died while on trametinib therapy, out of pulmonary capillary hemangiomatosis, a form of vascular lung disease typically associated with bi-allelic EIF2AK4 variants in its heritable form (52) or secondary to various parenchymal lung disorders (53), but not typical associated NS.