We collected information on patient status and cause of death for 545 insulin-dependent diabetic subjects who had cardiorespiratory reflex and nerve conduction tests performed to determine if presence and severity of autonomic and somatic neuropathy is associated with mortality and if a functioning pancreas transplantation (PTx) influences mortality. Follow-up was 12-138 months. Abnormal cardiorespiratory reflexes were present in 417 patients and there was abnormal nerve conduction in 392 patients. Mortality rates for patients with abnormal tests were higher (P < 0.0001) than for patients with normal tests. A total neuropathy score that included cardiorespiratory and nerve conduction test scores predicted survival better than separate scores. Patients with moderate neuropathy, but not those with severe neuropathy, who retained a functioning PTx, had longer survival times than patients whose PTx failed in the first 3 months. Considering only patients transplanted after 1985, those with moderate neuropathy who retained a functioning PTx had even longer survival times than nontransplanted patients.