PMID- 19309299
OWN - NLM
STAT- MEDLINE
DCOM- 20091102
LR  - 20090804
IS  - 1523-4681 (Electronic)
IS  - 0884-0431 (Linking)
VI  - 24
IP  - 8
DP  - 2009 Aug
TI  - Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical 
      expression and severity.
PG  - 1404-10
LID - 10.1359/jbmr.090304 [doi]
AB  - Primary hyperparathyroidism (PHPT) is a common endocrine disease that is 
      associated with multiple endocrine neoplasia type 1 (MEN1) in approximately 2% of 
      PHPT cases. Lack of a family history and other specific expressions may lead to 
      underestimated MEN1 prevalence in PHPT. The aim of this study was to identify 
      clinical or biochemical features predictive of MEN1 and to compare the severity 
      of the disease in MEN1-related versus sporadic PHPT (sPHPT). We performed a 36-mo 
      cross-sectional observational study in three tertiary referral centers on an 
      outpatient basis on 469 consecutive patients with sporadic PHPT and 64 with 
      MEN1-related PHPT. Serum calcium, phosphate, PTH, 25(OH)D(3), and creatinine 
      clearance were measured, and ultrasound examination of the urinary 
      tract/urography was performed in all patients. In 432 patients, BMD was measured 
      at the lumbar spine (LS) and femoral neck (FN). MEN1 patients showed lower BMD 
      Z-scores at the LS (-1.33 +/- 1.23 versus -0.74 +/- 1.4, p = 0.008) and FN (-1.13 
      +/- 0.96 versus -0.6 +/- 1.07, p = 0.002) and lower phosphate (2.38 +/- 0.52 
      versus 2.56 +/- 0.45 mg/dl, p = 0.003) and PTH (113.8 +/- 69.5 versus 173.7 +/- 
      135 pg/ml, p = 0.001) levels than sPHPT patients. Considering probands only, the 
      presence of MEN1 was more frequently associated with PTH values in the normal 
      range (OR, 3.01; 95% CI, 1.07-8.50; p = 0.037) and younger age (OR, 1.61; 95% CI, 
      1.28-2.02; p = 0.0001). A combination of PTH values in the normal range plus age 
      <50 yr was strongly associated with MEN1 presence (OR, 13.51; 95% CI, 3.62-50.00; 
      p = 0.0001). In conclusion, MEN1-related PHPT patients show more severe bone but 
      similar kidney involvement despite a milder biochemical presentation compared 
      with their sPHPT counterparts. Normal PTH levels and young age are associated 
      with MEN1 presence.
FAU - Eller-Vainicher, Cristina
AU  - Eller-Vainicher C
AD  - Unit of Endocrinology-Diabetology, Department of Medical Sciences, University of 
      Milan, Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, 
      IRCCS, Milan, Italy. eller.vainicher@gmail.com
FAU - Chiodini, Iacopo
AU  - Chiodini I
FAU - Battista, Claudia
AU  - Battista C
FAU - Viti, Raffaella
AU  - Viti R
FAU - Mascia, Maria Lucia
AU  - Mascia ML
FAU - Massironi, Sara
AU  - Massironi S
FAU - Peracchi, Maddalena
AU  - Peracchi M
FAU - D'Agruma, Leonardo
AU  - D'Agruma L
FAU - Minisola, Salvatore
AU  - Minisola S
FAU - Corbetta, Sabrina
AU  - Corbetta S
FAU - Cole, David E C
AU  - Cole DE
FAU - Spada, Anna
AU  - Spada A
FAU - Scillitani, Alfredo
AU  - Scillitani A
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - J Bone Miner Res
JT  - Journal of bone and mineral research : the official journal of the American 
      Society for Bone and Mineral Research
JID - 8610640
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Bone Density
MH  - Cross-Sectional Studies
MH  - Female
MH  - Humans
MH  - Hyperparathyroidism, Primary/*physiopathology
MH  - Male
MH  - Middle Aged
MH  - Proto-Oncogene Proteins/*physiology
MH  - Severity of Illness Index
MH  - Young Adult
EDAT- 2009/03/25 09:00
MHDA- 2009/11/03 06:00
CRDT- 2009/03/25 09:00
PHST- 2009/03/25 09:00 [entrez]
PHST- 2009/03/25 09:00 [pubmed]
PHST- 2009/11/03 06:00 [medline]
AID - 10.1359/jbmr.090304 [doi]
PST - ppublish
SO  - J Bone Miner Res. 2009 Aug;24(8):1404-10. doi: 10.1359/jbmr.090304.