PMID- 18376196
OWN - NLM
STAT- MEDLINE
DCOM- 20080421
LR  - 20211027
IS  - 0003-4932 (Print)
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 247
IP  - 3
DP  - 2008 Mar
TI  - Prospective study of surgery for primary hyperparathyroidism (HPT) in multiple 
      endocrine neoplasia-type 1 and Zollinger-Ellison syndrome: long-term outcome of a 
      more virulent form of HPT.
PG  - 501-10
LID - 10.1097/SLA.0b013e31815efda5 [doi]
AB  - BACKGROUND: Primary hyperparathyroidism (HPT) in multiple endocrine neoplasia 
      type 1 (MEN1) patients with Zollinger-Ellison syndrome (ZES) is caused by 
      parathyroid hyperplasia. Surgery for parathyroid hyperplasia is tricky and 
      difficult. Long-term outcome in ZES/MEN1/HPT is not well known. METHODS: 
      Eighty-four consecutive patients (49 F/35 M) with ZES/MEN1/HPT underwent initial 
      parathyroidectomy (PTX) and were followed at 1- to 3-year intervals. RESULTS: Age 
      at PTX was 36 +/- 2 years. Mean follow-up was 17 +/- 1 years. Before PTX, mean Ca 
      = 2.8 mmol/L (normal level (nl <2.5), PTH i = 243 pg/mL (nl <65), and gastrin = 
      6950 pg/mL (nl < 100). Sixty-one percent had nephrolithiasis. Each patient had 
      parathyroid hyperplasia. Fifty-eight percent of patients had 4 parathyroid glands 
      identified. Nine of 84 (11%) had 4 glands removed with immediate autograft, 40/84 
      (47%) 3 to 3.5 glands, whereas 35/84 (42%) <3 glands removed. 
      Persistent/recurrent HPT occurred in 42%/48% of patients with <3 glands, 12%/44% 
      with 3 to 3.5 glands, and 0%/55% with 4 glands removed. Hypoparathyroidism 
      occurred in 3%, 10%, and 22%, respectively. The disease-free interval after 
      surgery was significantly longer if >3 glands were removed. After surgery to 
      correct the HPT, each biochemical parameter of ZES was improved and 20% of 
      patients no longer had laboratory evidence of ZES. CONCLUSIONS: HPT/MEN1/ZES is a 
      severe form of parathyroid hyperplasia with a high rate of nephrolithiasis, 
      persistent and recurrent HPT. Surgery to correct the hypercalcemia significantly 
      ameliorates the ZES. Removal of less than 3.5 glands has an unacceptably high 
      incidence of persistent HPT (42%), whereas 4-gland resection and transplant has a 
      high rate of permanent hypoparathyroidism (22%). More than 3-gland resection has 
      a longer disease-free interval. The surgical procedure of choice for patients 
      with HPT/MEN1/ZES is 3.5-gland parathyroidectomy. Careful long-term follow-up is 
      necessary as a significant proportion will develop recurrent HPT.
FAU - Norton, Jeffrey A
AU  - Norton JA
AD  - Department of Surgery, Stanford University Medical Center, Stanford, CA 94305, 
      USA. janorton@stanford.edu
FAU - Venzon, David J
AU  - Venzon DJ
FAU - Berna, Marc J
AU  - Berna MJ
FAU - Alexander, H R
AU  - Alexander HR
FAU - Fraker, Douglas L
AU  - Fraker DL
FAU - Libutti, Stephen K
AU  - Libutti SK
FAU - Marx, Stephen J
AU  - Marx SJ
FAU - Gibril, Fathia
AU  - Gibril F
FAU - Jensen, Robert T
AU  - Jensen RT
LA  - eng
GR  - Z01 DK053200-16/ImNIH/Intramural NIH HHS/United States
GR  - Z01 DK053215-01/ImNIH/Intramural NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
MH  - Adult
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Hyperparathyroidism, Primary/pathology/*surgery
MH  - Hyperplasia
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*complications
MH  - Nephrolithiasis/etiology
MH  - Parathyroidectomy
MH  - Prospective Studies
MH  - Recurrence
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/*complications
PMC - PMC2717476
MID - NIHMS126257
EDAT- 2008/04/01 09:00
MHDA- 2008/04/22 09:00
PMCR- 2009/07/29
CRDT- 2008/04/01 09:00
PHST- 2008/04/01 09:00 [pubmed]
PHST- 2008/04/22 09:00 [medline]
PHST- 2008/04/01 09:00 [entrez]
PHST- 2009/07/29 00:00 [pmc-release]
AID - 00000658-200803000-00016 [pii]
AID - 10.1097/SLA.0b013e31815efda5 [doi]
PST - ppublish
SO  - Ann Surg. 2008 Mar;247(3):501-10. doi: 10.1097/SLA.0b013e31815efda5.