PMID- 9088067 OWN - NLM STAT- MEDLINE DCOM- 19970527 LR - 20191101 IS - 8756-0437 (Print) IS - 1098-2388 (Linking) VI - 13 IP - 2 DP - 1997 Mar-Apr TI - Parathyroid glands and the multiple endocrine neoplasia syndromes and familial hypocalciuric hypercalcemia. PG - 114-24 AB - Hypercalcemia is a variable feature of inherited endocrine disorders. In the multiple endocrine neoplasia (MEN) syndromes, generalized hyperparathyroidism is a common feature. It occurs much more frequently in patients with MEN type 1 as compared to patients with MEN type 2A. Unlike the MEN syndromes, patients with familial hypocalciuric hypercalcemia (FHH) have only hypercalcemia with no associated endocrinopathies. The hyperparathyroidism in patients with either of the MEN syndromes is managed by parathyroidectomy, whereas patients with FHH are managed nonoperatively. The specific genetic defects associated with MEN type 2 syndromes and FHH have been identified. They explain, in part, the clinical and pathophysiologic features of these diseases. The genetic defect causative of MEN type 1 will doubtless soon be found and thereby provide further insights into the molecular basis of calcium homeostasis. We will review the clinical presentation and the management of patients with these disorders. We will also review the recent molecular discoveries in MEN 2A, MEN 2B, and FHH, and define how they have altered the management of patients who have these syndromes. FAU - Herfarth, K K AU - Herfarth KK AD - Department of Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA. FAU - Wells, S A Jr AU - Wells SA Jr LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Semin Surg Oncol JT - Seminars in surgical oncology JID - 8503713 SB - IM MH - Humans MH - *Hypercalcemia/genetics/physiopathology/therapy MH - *Hyperparathyroidism/etiology/therapy MH - *Multiple Endocrine Neoplasia/genetics/physiopathology/therapy MH - Parathyroid Glands/pathology/physiopathology MH - Parathyroidectomy RF - 57 EDAT- 1997/03/01 00:00 MHDA- 2000/06/22 10:00 CRDT- 1997/03/01 00:00 PHST- 1997/03/01 00:00 [pubmed] PHST- 2000/06/22 10:00 [medline] PHST- 1997/03/01 00:00 [entrez] AID - 10.1002/(SICI)1098-2388(199703/04)13:2<114::AID-SSU7>3.0.CO;2-C [pii] AID - 10.1002/(sici)1098-2388(199703/04)13:2<114::aid-ssu7>3.0.co;2-c [doi] PST - ppublish SO - Semin Surg Oncol. 1997 Mar-Apr;13(2):114-24. doi: 10.1002/(sici)1098-2388(199703/04)13:2<114::aid-ssu7>3.0.co;2-c.