Continuous linear growth, delayed bone maturation, tall stature, eunuchoid body proportions, genu valgum,  Normal libido,  Normal testicular volume  Normal spermogram.  Increased serum basal FSH, with normal LH and testosterone levels.  Overweight, acanthosis nigricans, Dyslipidemia and severe steatohepatitis.  Impaired glucose tolerance and hyperinsulinemia.  Osteopenia. The gene discussed is PLOD1; the disease is hyperinsulinism.