PMID- 19155316
OWN - NLM
STAT- MEDLINE
DCOM- 20090416
LR  - 20161125
IS  - 1479-683X (Electronic)
IS  - 0804-4643 (Linking)
VI  - 160
IP  - 4
DP  - 2009 Apr
TI  - The syndrome of gastric carcinoid and hyperparathyroidism: a family study and 
      literature review.
PG  - 689-94
LID - 10.1530/EJE-08-0867 [doi]
AB  - OBJECTIVE: To present evidence supporting the hypothesis that the coexistence of 
      gastric carcinoids (GCs) and hyperparathyroidism may represent a distinct 
      clinical entity, not related to multiple endocrine neoplasia type 1 (MEN1). 
      METHODS: We studied a cohort of five young siblings (age range 26-42 years), one 
      of whom had been found to have GC and hyperparathyroidism. All siblings underwent 
      serial gastroscopies for the assessment of gastric neuroendocrine cell 
      proliferations over a mean follow-up period of 31.2 months. Imaging, biochemical 
      and hormonal as well as molecular genetic investigations were performed in the 
      direction of MEN1 syndrome. The literature was searched for cases with 
      coexistence of GCs and hyperparathyroidism not associated with MEN1. RESULTS: 
      Four of the siblings, all male, were found to have GCs in a background of 
      Helicobacter pylori-associated chronic atrophic gastritis and pernicious anaemia, 
      with no serological evidence of gastric autoimmunity. In two of them, 
      asymptomatic hyperparathyroidism was also present. Screening for MEN1 gene 
      mutations or large deletions was negative, and hormone and imaging investigations 
      did not support a diagnosis of familial MEN1 syndrome. A literature search 
      revealed sporadic reports of cases with GC and hyperparathyroidism not 
      attributable to MEN1. CONCLUSIONS: The association of GCs and hyperparathyroidism 
      appears to constitute a distinct syndrome that can be encountered in genetically 
      predisposed individuals, and should not be regarded as 'atypical' or 'incomplete' 
      expression of MEN1. Its prevalence and aetiology should be the subject of future 
      studies. Screening for hyperparathyroidism seems to be justified in patients with 
      GC of any type.
FAU - Christopoulos, C
AU  - Christopoulos C
AD  - The Greek MEN-1 Study Group, First Department of Internal Medicine, A. Fleming 
      General Hospital, Athens, Greece. cgchrist@otenet.gr
FAU - Balatsos, V
AU  - Balatsos V
FAU - Rotas, E
AU  - Rotas E
FAU - Karoumpalis, I
AU  - Karoumpalis I
FAU - Papavasileiou, D
AU  - Papavasileiou D
FAU - Kontogeorgos, G
AU  - Kontogeorgos G
FAU - Dupasquier, S
AU  - Dupasquier S
FAU - Calender, A
AU  - Calender A
FAU - Skandalis, N
AU  - Skandalis N
FAU - Economopoulos, P
AU  - Economopoulos P
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20090120
PL  - England
TA  - Eur J Endocrinol
JT  - European journal of endocrinology
JID - 9423848
RN  - 0 (Chromogranin A)
RN  - 0 (Hormones)
RN  - 0 (Pancreatic Hormones)
RN  - 0 (Pituitary Hormones)
RN  - 9007-49-2 (DNA)
SB  - IM
MH  - Adult
MH  - Carcinoid Tumor/diagnostic imaging/*genetics/*pathology
MH  - Cell Proliferation
MH  - Chromogranin A/blood
MH  - DNA/genetics
MH  - Endoscopy
MH  - Enterochromaffin Cells/pathology
MH  - Female
MH  - Helicobacter Infections/drug therapy
MH  - Helicobacter pylori
MH  - Hormones/blood
MH  - Humans
MH  - Hyperparathyroidism/*genetics/*pathology
MH  - Immunohistochemistry
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/genetics/pathology
MH  - Mutation/genetics
MH  - Pancreas/diagnostic imaging
MH  - Pancreatic Hormones/blood
MH  - Pituitary Hormones/blood
MH  - Stomach Neoplasms/diagnostic imaging/*genetics/*pathology
MH  - Ultrasonography
EDAT- 2009/01/22 09:00
MHDA- 2009/04/17 09:00
CRDT- 2009/01/22 09:00
PHST- 2009/01/22 09:00 [entrez]
PHST- 2009/01/22 09:00 [pubmed]
PHST- 2009/04/17 09:00 [medline]
AID - EJE-08-0867 [pii]
AID - 10.1530/EJE-08-0867 [doi]
PST - ppublish
SO  - Eur J Endocrinol. 2009 Apr;160(4):689-94. doi: 10.1530/EJE-08-0867. Epub 2009 Jan 
      20.