caa a22 4500 475803582 CHVBK 20180406123744.0 cr unu---uuuuu 170329e20001101xx s 000 0 eng 10.1007/s002680010237 doi (NATIONALLICENCE)springer-10.1007/s002680010237 Are Patients with Multiple Endocrine Neoplasia Type I Prone to Premature Death? [Elektronische Daten] [Patrick G. Dean, Jon A. van Heerden, David R. Farley, Geoffrey B. Thompson, Clive S. Grant, W. Scott Harmsen, Duane M. Ilstrup] Multiple endocrine neoplasia type I (MEN-I) is an autosomal dominant disorder characterized by endocrinopathies involving the anterior pituitary gland, parathyroid glands, and pancreas. The long-term prognosis for patients affected with this disorder is uncertain. To better characterize this prognosis, we performed a retrospective review of all patients with MEN-I treated at a single institution during the period 1951-1997. A group of 233 patients served as the study population. Their records were analyzed for confirmation of diagnosis, treatments received, long-term survival, and cause of death. Altogether, 108 eight male patients (46%) and 125 female patients (54%) were identified. At the conclusion of the study, 164 (70%) were alive and 69 (30%) were deceased, with a median follow-up for patients alive at last contact of 13.4 years (range < 1 month to 54.3 years). The cause of death was reliably obtained in 60 patients. Of these patients, 17 (28%) died of causes related to MEN-I, most commonly metastatic islet cell tumors (10 patients). The remaining patients died of causes unrelated to MEN-I, most commonly coronary artery disease and nonendocrine malignancies (14% each). The overall 20-year survival of MEN-I patients was 64% (95% CI was 56-72%), and that of an age- and gender-matched upper Midwest population was 81% (p < 0.001). Patients with MEN-I appear to be at increased risk of premature death. Earlier diagnosis and treatment of potentially malignant pancreatic islet cell neoplasms may result in a decrease of this premature mortality. Société Internationale de Chirurgie, 2000 Dean Patrick G. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut van Heerden Jon A. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut Farley David R. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut Thompson Geoffrey B. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut Grant Clive S. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut Harmsen W. Scott Section of Biostatistics, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut Ilstrup Duane M. Section of Biostatistics, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut World Journal of Surgery Springer-Verlag 24/11(2000-11-01), 1437-1441 0364-2313 24:11<1437 2000 24 268 https://doi.org/10.1007/s002680010237 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002680010237 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Dean Patrick G. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- van Heerden Jon A. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- Farley David R. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- Thompson Geoffrey B. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- Grant Clive S. Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- Harmsen W. Scott Section of Biostatistics, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 700 1- Ilstrup Duane M. Section of Biostatistics, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905, USA, US aut NATIONALLICENCE 773 0- World Journal of Surgery Springer-Verlag 24/11(2000-11-01), 1437-1441 0364-2313 24:11<1437 2000 24 268 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer