2005 Volume 5 Issue 2 Pages 82-88
Both multiple endocrine neoplasia（MEN）type 1 and 2 include parathyroid disease. We previously reported cases of familial hyperparathyroidism（ FHPT） including hyperparathyroidism with MEN and familial isolated（FIHPT）. Herein, we report the long-term postoperative follow-up for these FHPT cases. Subjects were 19 patients（twelve families）who underwent parathyroidectomy for FHPT at our department with over one year of post operative follow up. These casese included 13 MEN 1 cases（8 families）, 2 MEN2 cases（2 families）, and 4 FIHPT cases（2 families）. The average observation period was 59 months（ range from 12 months to 51 months） . Hyperplasia was noted in 11 cases（ in one case, supernumerary parathyroid glands relapsed as cancer）, and 8 adenomas. Gene examinations were performed in 16 cases. At present, all cases are continuing to be followed at our department outpatient. Results: Two of the 19 cases died due to gastrinoma and an accident, respectively, while the remaining 17 cases have survived postoperatively. There was no relapse of parathyroid adenoma after parathyroidectomy. Recurrence of hyperparathyroidism in one case was due to supernumerary gland. Only one case developed an other organ disease due to MEN during this follow-up period. This case developed ploractionoma and tumor: ACTHindependent macronodular hyperplasia（AIMAH）after parathyroidectomy. There were four cases demonstrating supernumerary parathyroid glands. In two of these cases, serum Ca and PTH.have remained within normal ranges for over 90 months after parathyroidectomy, respectively. Five persons who were single at operation have not yet married, one married patient had a second child postoperatively. At present, two cases who did not want to undergo gene examination still do not want to undergo this examination. Therefore, the appearance of disease in the lineage can not be confirmed, excluding the lineage investigation performed immediately after surgery. Conclusively, our long-term postoperative follow-up study for FHPT demonstrated few cases of concurrent disease in other organs and no newly diagnosed cases among followed families. FHPT might be a slowly developing disease. It seemed that FHPT follow-up continues to be necessary.
