After correction for surgery, age at diagnosis, sex and attained age, both ACTH and Gn deficiencies were independently associated with increased mortality (RR 2·56, 95% CI 1·10–5·96, P = 0·01, and RR 2·26, 95% CI 1·15–4·47, P = 0·01, respectively, Table 3). The gene discussed is POMC; the disease is mucopolysaccharidosis type 3D.