PMID- 8924487
OWN - NLM
STAT- MEDLINE
DCOM- 19961122
LR  - 20131121
IS  - 1121-421X (Print)
IS  - 1121-421X (Linking)
VI  - 42
IP  - 3
DP  - 1996 Sep
TI  - [New etiopathogenic, clinical and therapeutic findings in multiple endocrine 
      neoplasia type 1].
PG  - 133-43
AB  - Multiple Endocrine Neoplasia type 1 (MEN 1) syndrome comprises tumors or 
      hyperplasia of different glands, including parathyroid, pituitary, adrenal cortex 
      and the gastroenteropancreatic system. The vast majority of MEN 1 are found in 
      familial clusters, although a few cases are sporadic. Hypercalcemia and/or 
      nephrocalcinosis are the first and most common clinical manifestation in familial 
      MEN 1 syndrome, followed by islet cell tumors (especially those secreting gastrin 
      or insulin) and pituitary dysfunction due to either functioning or 
      non-functioning microadenomas. Genetic studies indicate that familial MEN 1 
      syndrome is inherited through a dominant gene with incomplete penetrance and 
      variable expression. The diagnosis of MEN 1 syndrome is mainly based on the 
      careful assessment of the clinical history, symptoms physical evaluation along 
      with the assay of serum electrolytes (i.e., calcium, phosphorus, etc.) and 
      hormonal substances (i.e., gastrin, insulin, pancreatic polypeptide, prolactin, 
      adrenocorticotropic hormone, etc.). In addition, several provocative tests have 
      been used to identify endocrine tumors (particularly those of the 
      gastroenteropancreatic system) and imaging techniques play a crucial role for the 
      diagnostic approach in MEN 1 syndrome. Even though in the long term, the 
      prognosis of MEN 1 syndrome is unfavourable. Recently, however, many therapeutic 
      strategies, including both surgical and pharmacological options, have been 
      developed to reduce the size of the neoplasm and control symptoms associated with 
      hormone oversecretion.
FAU - Tomassetti, P
AU  - Tomassetti P
AD  - Dipartimento di Medicina Interna e Gastroenterologia, Policlinico Sant'Orsola, 
      Universita degli Studi, Bologna.
FAU - De Giorgio, R
AU  - De Giorgio R
FAU - Bosoni, D
AU  - Bosoni D
FAU - Del Vecchio, E
AU  - Del Vecchio E
FAU - Migliori, M
AU  - Migliori M
FAU - Stanghellini, V
AU  - Stanghellini V
FAU - Corinaldesi, R
AU  - Corinaldesi R
LA  - ita
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Nuove acquisizioni eziopatogenetiche, cliniche e terapeutiche nella neoplasia 
      endocrina multipla di tipo 1.
PL  - Italy
TA  - Minerva Gastroenterol Dietol
JT  - Minerva gastroenterologica e dietologica
JID - 9109791
RN  - 0 (Antineoplastic Agents, Hormonal)
RN  - RWM8CCW8GP (Octreotide)
SB  - IM
MH  - Antineoplastic Agents, Hormonal/therapeutic use
MH  - Female
MH  - Gastrinoma/drug therapy/pathology/*surgery
MH  - Humans
MH  - Hyperparathyroidism
MH  - Male
MH  - *Multiple Endocrine Neoplasia Type 1/drug therapy/genetics/pathology/surgery
MH  - Octreotide/therapeutic use
MH  - Pancreatic Neoplasms/drug therapy/pathology/*surgery
MH  - Parathyroid Neoplasms/drug therapy/pathology
MH  - Pituitary Neoplasms/drug therapy/pathology/*surgery
MH  - Prognosis
RF  - 53
EDAT- 1996/09/01 00:00
MHDA- 1996/09/01 00:01
CRDT- 1996/09/01 00:00
PHST- 1996/09/01 00:00 [pubmed]
PHST- 1996/09/01 00:01 [medline]
PHST- 1996/09/01 00:00 [entrez]
PST - ppublish
SO  - Minerva Gastroenterol Dietol. 1996 Sep;42(3):133-43.