caa a22 4500 477035175 CHVBK 20180405111303.0 cr unu---uuuuu 170330e19960701xx s 000 0 eng 10.1007/s002689900124 doi (NATIONALLICENCE)springer-10.1007/s002689900124 Prophylactic Surgery for Multiple Endocrine Neoplasia Type IIa after Genetic Diagnosis: Is Parathyroid Transplantation Indicated? [Elektronische Daten] [Ruth A. Decker, James D. Geiger, Charles E. Cox, Marilyn Mackovjak, Minakshi Sarkar, Michael L. Peacock] Abstract. Identification of germline mutations in the: RET proto-oncogene predisposing to multiple endocrine neoplasia type IIa (MEN-IIa) has allowed a DNA-based approach to diagnosis and treatment by prophylactic thyroidectomy in children testing genetically positive. Although total thyroidectomy is the accepted operation for C cell disease, the necessity of routine total parathyroidectomy and autotransplantation as previously described in these asymptomatic children is questionable, particularly given the low occurrence of hyperparathyroidism in MEN-IIa (10-20%). Thirty-six children (ages 1 month to 12 years) from four MEN-IIa kindreds at risk for disease underwent genetic testing. Mutational analysis was done using a highly sensitive PCR-based denaturing gradient gel electrophoresis technique. Parathyroid or serum calcium concentrations were determined preoperatively. Of the 36 children at risk, 18 were found to have a MEN-IIa mutation; 11 have undergone prophylactic thyroidectomy at ages ranging from 2 to 12 years (mean 7.5 years). In each case, there was no biochemical evidence of hypercalcemia preoperatively, and all parathyroid glands were identified and were found to be grossly normal at exploration. Glands were carefully dissected and left in situ. Postoperatively, 10 of the 11 children maintained normocalcemia, allowing discharge within 24 to 36 hours. Resected thyroid glands contained C cell hyperplasia in nine, medullary carcinoma in one, and normal histology in one. We conclude that an alternative to routine parathyroidectomy may be desirable for prophylactic treatment of MEN-IIa. In situ parathyroid preservation can be safely achieved without compromising the completeness of the thyroid resection. This conservative approach obviates the potential morbidity associated with total parathyroidectomy and autotransplantation. 1996 by the Société Internationale de Chir, ugie Decker Ruth A. Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Geiger James D. Department of Pediatric Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Cox Charles E. Department of Pediatric Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Mackovjak Marilyn Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Sarkar Minakshi Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Peacock Michael L. Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut https://doi.org/10.1007/s002689900124 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002689900124 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Decker Ruth A. Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut NATIONALLICENCE 700 1- Geiger James D. Department of Pediatric Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut NATIONALLICENCE 700 1- Cox Charles E. Department of Pediatric Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut NATIONALLICENCE 700 1- Mackovjak Marilyn Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut NATIONALLICENCE 700 1- Sarkar Minakshi Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut NATIONALLICENCE 700 1- Peacock Michael L. Department of Surgery, Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor, Michigan 48109-0331, U.S.A., US aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer