PMID- 22470073
OWN - NLM
STAT- MEDLINE
DCOM- 20120629
LR  - 20120705
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 255
IP  - 6
DP  - 2012 Jun
TI  - Primary hyperparathyroidism in MEN1 patients: a cohort study with longterm 
      follow-up on preferred surgical procedure and the relation with genotype.
PG  - 1171-8
LID - 10.1097/SLA.0b013e31824c5145 [doi]
AB  - OBJECTIVE: To identify the optimal surgical strategy for multiple endocrine 
      neoplasia type 1 (MEN1)-related primary hyperparathyroidism (pHPT). To describe 
      the course of postoperative hypoparathyroidism and to assess whether genotype is 
      associated with persistent/recurrent pHPT. BACKGROUND: Surgery is the preferred 
      treatment in MEN1-related pHPT, but the surgical procedure of choice is still 
      uncertain. METHODS: This retrospective cohort study was performed at the 
      Departments of Endocrinology of the University Medical Centers of Utrecht and 
      Nijmegen, the Netherlands. Patients were selected from the Dutch MEN1 database, 
      including all patients 16 years or older treated for MEN1 from 1990 to 2009. Data 
      were collected by medical record review. RESULTS: Seventy-three patients 
      underwent parathyroid surgery. Persistent/recurrent pHPT occurred in 53% after 
      less than 3 parathyroids resected (<SPTX), 17% after subtotal resection (SPTX), 
      and 19% after total resection with autotransplantation (TPTX). Persistent (>/=6 
      months) postoperative hypoparathyroidism occurred in 24% after <SPTX, 39% after 
      SPTX, and 66% after TPTX. Median duration of hypoparathyroidism was 1.5 years, in 
      65% successful cessation of vitamin D/calcium was possible, even after more than 
      10 years. After <SPTX, patients with nonsense or frameshift mutations in exons 2, 
      9, and 10 had a significantly lower risk of persistent/recurrent pHPT than 
      patients with other mutations. After SPTX/TPTX persistence/recurrence did not 
      differ with genotype. After SPTX/TPTX persistence/recurrence was more frequent (P 
      = 0.07) in patients without bilateral transcervical thymectomy (TCT). 
      CONCLUSIONS: SPTX with bilateral TCT is the procedure of choice for MEN1-related 
      pHPT. Genotype seems to affect the chance of recurrence. Postoperative 
      hypoparathyroidism lasting 6 months or more should not be considered permanent in 
      MEN1.
FAU - Pieterman, Carolina R C
AU  - Pieterman CR
AD  - Department of Internal Medicine, University Medical Center Utrecht, The 
      Netherlands.
FAU - van Hulsteijn, Leonie T
AU  - van Hulsteijn LT
FAU - den Heijer, Martin
AU  - den Heijer M
FAU - van der Luijt, Rob B
AU  - van der Luijt RB
FAU - Bonenkamp, J J
AU  - Bonenkamp JJ
FAU - Hermus, Ad R M M
AU  - Hermus AR
FAU - Borel Rinkes, Inne H M
AU  - Borel Rinkes IH
FAU - Vriens, Menno R
AU  - Vriens MR
FAU - Valk, Gerlof D
AU  - Valk GD
CN  - DutchMEN1 Study Group
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
CIN - Nat Rev Endocrinol. 2012 Jun;8(6):321. PMID: 22525736
MH  - Adult
MH  - Female
MH  - Follow-Up Studies
MH  - Genotype
MH  - Humans
MH  - Hyperparathyroidism, Primary/genetics/*surgery
MH  - Hypoparathyroidism/etiology/*physiopathology
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*surgery
MH  - Parathyroidectomy/*adverse effects
MH  - Recurrence
MH  - Retrospective Studies
MH  - Treatment Outcome
FIR - Valk, G D
IR  - Valk GD
FIR - Zonnenberg, B A
IR  - Zonnenberg BA
FIR - Hermus, A R M M
IR  - Hermus AR
FIR - Dekkers, O M
IR  - Dekkers OM
FIR - de Herder, W W
IR  - de Herder WW
FIR - van der Horst-Schrivers, A N A
IR  - van der Horst-Schrivers AN
FIR - Drent, M L
IR  - Drent ML
FIR - Bisschop, P H L T
IR  - Bisschop PH
FIR - Havekes, B
IR  - Havekes B
FIR - Vasen, H F A
IR  - Vasen HF
EDAT- 2012/04/04 06:00
MHDA- 2012/06/30 06:00
CRDT- 2012/04/04 06:00
PHST- 2012/04/04 06:00 [entrez]
PHST- 2012/04/04 06:00 [pubmed]
PHST- 2012/06/30 06:00 [medline]
AID - 10.1097/SLA.0b013e31824c5145 [doi]
PST - ppublish
SO  - Ann Surg. 2012 Jun;255(6):1171-8. doi: 10.1097/SLA.0b013e31824c5145.