SBDS and hyperinsulinemic hypoglycemia, familial, 4: Common events in SDS patients are the development of many somatic clones in the bone marrow with variants in either EIF6 or TP53 genes, producing different effects: i) variants in EIF6 have a compensatory role on the impact of SBDS deficiency, in turn reducing the risk of developing MDS and AML by improving the ribosome maturation, enhancing translation, and reducing p53 upregulation (Kennedy et al., 2021), ii) variants in TP53 act in a different pathway and decrease tumor suppressor checkpoint activation.