|1.||Multiple Endocrine Neoplasia Type 1 (MEN I)
|5.||Primitive Neuroectodermal Tumors (PNET)
|1.||Hua, Xianxin: 16 articles (01/2014 - 07/2003)|
|2.||Marx, Stephen J: 15 articles (08/2015 - 01/2003)|
|3.||Marx, S J: 12 articles (12/2001 - 01/2000)|
|4.||Thakker, Rajesh V: 11 articles (04/2014 - 06/2002)|
|5.||Jensen, Robert T: 11 articles (11/2013 - 03/2003)|
|6.||Tsukada, Toshihiko: 11 articles (08/2013 - 10/2002)|
|7.||Brandi, Maria Luisa: 10 articles (08/2014 - 06/2002)|
|8.||Calender, Alain: 10 articles (05/2010 - 02/2002)|
|9.||Libutti, Steven K: 9 articles (11/2015 - 11/2003)|
|10.||Klöppel, Günter: 8 articles (05/2015 - 05/2006)|
11/01/2013 - "Controversies include: the best approach to a patient with hypergastrinemia suspected of possibly having ZES, the appropriate gastrin assay to use, the role of surgery in patients with ZES, especially those with multiple endocrine neoplasia type 1, and the precise order of therapeutic modalities in the treatment of patients with advanced disease. "
09/15/2012 - "Therefore, we examined whether conditional deletion of Men1 (Villin-Cre and Lgr5-EGFP-IRES-CreERT2), with subsequent loss of menin from the gastrointestinal epithelium, increases gastrin expression. "
05/01/2007 - "The observation of distinct deletion patterns in small synchronous tumours supports the concept that each gastrin-producing tumour in an individual MEN1 patient arises from an independent cell clone."
08/01/2006 - "Of patients with MEN 1, uncontrolled hypercalcaemia (P < 0.05) and elevated serum gastrin (P < 0.05) were more common amongst patients diagnosed with abnormal glycaemia than those with normoglycaemia. "
08/01/2002 - "The tumour suppressor menin (which is mutated in MEN-1 syndrome) inhibited the expression of MMP-9-luc by gastrin. "
|2.||cinacalcet (Sensipar)FDA Link
12/01/2013 - "Cinacalcet seems to be highly effective in controlling PHPT in patients with MEN1 either in naïve patients or in those with postsurgical recurrence. "
12/01/2013 - "If cinacalcet will be confirmed to ensure a long-time control of PHPT or even to prevent the development and progression of PHPT, this may led to modify the therapeutic strategy of MEN1 PHPT."
12/01/2013 - "We present an experience with cinacalcet-hydrochloride in two patients with MEN1 PHPT. "
12/01/2013 - "Medical therapy with cinacalcet-hydrochloride may modify the therapeutic strategy of MEN1 related PHPT. "
08/01/2012 - "Although long-term safety and efficacy data are required, cinacalcet might be considered a treatment option in MEN1 patients who have contraindications to surgery or persistent PHPT after surgery."
|3.||Octreotide (Sandostatin)FDA LinkGeneric
06/01/2014 - "To evaluate the efficacy of the long-acting SSA octreotide in MEN1 patients with early-stage duodeno-pancreatic NETs. "
05/12/2002 - "[Octreotide therapy in multiple endocrine neoplasia type-1]."
11/15/1999 - "Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1). "
01/01/2010 - "Long-term suppressive effect of octreotide on progression of metastatic gastrinoma with multiple endocrine neoplasia type 1: seven-year follow up."
04/01/1998 - "Thirty-five of 48 patients had positive [111In]-octreotide scintigraphy (23/25 (92%) carcinoids, 8/9 (89%) PETs, 4/11 (36%) MEN-I & ZES). "
|4.||DNA (Deoxyribonucleic Acid)IBA
02/01/2013 - "The MEN1, RET and CDKN1B molecular study was carried out in the germline DNA by PCR/SSCP and direct sequencing. "
10/01/2004 - "The DNA study for germline mutations confirmed the clinical diagnosis of MEN1 syndrome in the patient and evidenced the same MEN1 mutation in her father and twin sister. "
12/01/1998 - "We performed biochemical studies and DNA analyses of the MEN1 gene simultaneously and independently as family screening studies. "
10/01/2015 - "The coding sequence, including 9 coding exons and 16 splice junctions of the MEN1 gene of leukocyte DNA was determined. "
01/01/2015 - "Genomic DNA isolated from blood was subjected to MEN1 gene amplification followed by direct sequencing. "
07/01/2012 - "The study (open-label prospective, conducted in a single tertiary center) included 12 sporadic and 2 multiple endocrine neoplasia type 1 PHPT patients with serum calcium greater than 11.2 mg/dl. "
09/01/2011 - "Serum Calcium was highest in the HPT-JT group (3.6 mM), recurrencies of HPT were highest in the MEN1 group (40.5%). "
03/01/2004 - "These findings strongly favour a recommendation for MEN1 and CASR genotyping of patients with multiglandular FIHP, irrespective of urinary calcium excretion. "
04/01/1997 - "Twenty percent of ZES patients have multiple endocrine neoplasia type I, some of whom may also have elevated levels of serum calcium and a family history of ZES. "
10/01/1993 - "To evaluate this contention, intra platelet free calcium concentrations (platelet [Ca2+]i) were determined in 20 young adult men, i.e. "
|6.||Glucose (Dextrose)FDA LinkGeneric
11/23/2010 - "Men1 excision improved glucose metabolism at least partly through increasing proliferation of endogenous β cells and islet size. "
07/17/2013 - "Gene knock-out/knock-in studies indicate that inactivation of factors including MEN1 and abnormal PI3K/mTOR signaling uncouples endocrine cell cycle progression from the control of environmental cues such as glucose, leading to islet cell overgrowth. "
01/01/2012 - "Impaired fasting glucose was more prevalent in MEN1 compared with controls (17%vs 6%, P < 0·05). "
01/01/2012 - "Increased prevalence of impaired fasting glucose in MEN1 gene mutation carriers."
08/01/2006 - "Six (8.3%) MEN 1 patients had impaired fasting glucose compared to 4 (3%) of controls (P < 0.05). "
|7.||Ethanol (Ethyl Alcohol)IBA
01/01/2015 - "This study sought to evaluate the safety and efficacy of percutaneous ethanol ablation (PEA) for the treatment of recurrent PHP in patients with MEN1. "
03/01/2012 - "This analysis allowed the determination of the following change-points: among men: 1 change-point for vegetables, legumes, cereals, fish and seafood and dairy products and 2 change-points for fruit and nuts, meat and meat products and ethanol; among women: 1 change-point for legumes and fish and seafood and 2 change-points for the remaining food groups. "
12/01/2008 - "Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1."
04/01/1993 - "Plasma TG change over 8 h was determined following a liquid fatty meal in 14 male habitual runners (R) and 13 physically inactive men (I) after 3 weeks of alcohol abstinence and 3 weeks of drinking approximately 41 g (1.44 oz) of ethanol per day. "
01/01/2015 - "Safety and efficacy of percutaneous parathyroid ethanol ablation in patients with recurrent primary hyperparathyroidism and multiple endocrine neoplasia type 1."
|8.||Complementary DNA (cDNA)IBA
11/01/1999 - "Intron borders should thus be screened for mutations in MEN1 diagnostics and cDNA sequence analysis is helpful in identifying pathophysiological consequences of intron mutations."
01/01/2015 - "Nucleotide sequences obtained were compared with published MEN1 cDNA sequences. "
10/22/1999 - "Here we report the sequencing and characterization of the MEN1 gene in zebrafish (Danio rerio) at the cDNA level. "
09/03/1999 - "No MEN1 gene mutations were identified in endocrine islet tumor cell lines RIN 5AH (rat) and NIT-1 (mouse) as compared to wild type cDNA. "
06/01/1998 - "One of the novel genes that we have identified by cDNA selection in this region, SCG2 (Suppressor Candidate Gene 2), proved to be identical to the recently published MEN1 gene. "
|9.||Genetic Markers (Genetic Marker)IBA
10/01/1995 - "Improved carrier testing for multiple endocrine neoplasia, type 1, using new microsatellite-type DNA markers."
09/01/2001 - "LOH analysis was performed using four polymorphic DNA markers at the MEN1 gene locus; D11S480, PYGM, D11S449, and INT-2. "
02/01/2000 - "The extension of genetic diagnosis to asymptomatic family members allowed the identification of 10 MEN1 mutant gene carriers, one newly described and nine previously detected by linkage analysis with DNA markers from the 11q13 region. "
01/01/1997 - "Linkage testing with DNA markers is still required for patients with multiple endocrine neoplasia type I because the responsible gene has not yet been isolated. "
10/01/1995 - "Eight Finnish families, including 46 individuals carrying the risk haplotype, have been typed for four polymorphic microsatellite DNA markers spanning the MEN1 chromosomal region. "
06/01/1977 - "Twenty-three patients with hyperparathyroidism from six families with the multiple endocrine adenomatosis (MEA) I-syndrome were tested by secretin provocation. "
06/01/1988 - "Two adult patients with multiple B-cell adenomas and hyperplasia (not associated with multiple endocrine neoplasia type I) hyperresponded to secretin, whereas patients with multiple endocrine neoplasia, type I, without hyperinsulinism responded normally. "
11/01/1991 - "Secretin injection caused gastrin to increase in the patient with MEN I and in another hypergastrinemic patient. "
06/01/1977 - "In 34 normal subjects, 23 nonaffected members of families with MEA I-syndrome, and 42 patients with various diseases the maximum gastrin response to secretin was 21 pg/ml. We conclude that secretin provocation is helpful in the diagnosis of the Zollinger-Ellison syndrome, especially when basal serum gastrin levels are only slightly elevated."
11/01/1989 - "There was no significant difference in the responses of patients with different extents or locations of the tumor, presence or absence of multiple endocrine neoplasia, type-I, or with fasting gastrin less than or greater than 1000 pg/mL. In patients with fasting gastrin of less than 1000 pg/mL, the sensitivity of the secretin test using the criterion of an increase in gastrin of at least 110 pg/mL was 93% (CI, 76% to 99%) and for an increase of 200 pg/mL it was 85% (CI, 66% to 96%), (P greater than 0.05). "
04/01/2014 - "Preoperative MEN1 evaluation helped guide the extent of initial parathyroidectomy and was associated with lower rates of recurrence in sporadic and familial PHPT in pediatric patients. "
09/01/2015 - "This study investigated the ultrasonographic features of parathyroid glands in 10 patients with MEN1 who underwent preoperative neck ultrasonography and parathyroidectomy between 2006 and 2010 at Toranomon Hospital. "
08/01/2013 - "The study population included the patients, positive for the MEN1 gene mutation, who underwent parathyroidectomy between 1983 and 2009 at a single tertiary referral center. "
06/01/2012 - "The objective of the present study was to evaluate the utility of preoperative localizing studies in patients with MEN1 undergoing initial bilateral neck exploration (BNE) and parathyroidectomy for pHPT. "
06/01/2012 - "Preoperative localizing studies for initial parathyroidectomy in MEN1 syndrome: is there any benefit?"
06/01/2009 - "Several end points may be helpful for future guidelines: (1) earlier detection of Th-NET in MEN1 patients is required; (2) screening of both sexes is necessary; (3) a prospective study comparing MRI vs. CT scan in yearly screening for Th-NET is needed; (4) a reinforced screening program must be established for patients who belong to clustered families; and (5) thymectomies must be performed in specialized centers."
11/01/2008 - "Since transcervical thymectomy does not prevent all MEN1 patients from developing NETT, existing surveillance guidelines for MEN1 should consider CT scan of the thorax on a regular basis."
06/01/2012 - "This suggests that routine preoperative localizing studies to identify ectopic and supernumerary enlarged parathyroid glands is not useful in the majority of patients with MEN1 undergoing bilateral neck exploration and subtotal parathyroidectomy with cervical thymectomy."
04/01/2014 - "Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (≥ 3 gland parathyroidectomy with transcervical thymectomy, 13/22, 59% vs. 0/4, 0%; P=0.03) and less likely to have recurrent disease (10/22, 45% vs. 3/4, 75%; P<0.001) during follow up than patients diagnosed postoperatively. "
09/01/2004 - "According to our results, tPTX+T (total parathyroidectomy+thymectomy+autotransplantation) seems to be a more favorable surgical approach in patients with MEN1 pHPT than sDE (selective gland exstirpation) and stPTX (subtotal parathyroidectromy) without cervical thymectomy, because recurrences or persistence of the disease are rare. "
10/01/2004 - "The patient with Zollinger-Ellison and MEN1 syndrome has been followed 3 years after diagnosis and 2 years after total gastrectomy. "
11/01/1992 - "Ninety-five percent of patients (20/21) without multiple endocrine neoplasia type I, severe gastroesophageal reflux disease, or previous partial gastrectomy had safe reductions of doses. "
03/01/1977 - "Early cancers were found in ten patients (23%), and four of these ten (3 men, 1 woman, aged 49 to 76 years), had been operated on 20-33 years previosuly with a Billroth II partial gastrectomy. "
12/01/1989 - "This experience suggests the following: (1) that patients with AGCH, duodenal gastrinomas, or lymph node gastrinomas can usually be rendered eugastrinemic by resection; (2) that patients with pancreatic gastrinomas, whether sporadic or familial (MEN I), are rarely cured by resection and frequently develop hepatic metastases; and (3) that patients in whom the source of the hypergastrinemia is not identified and removed frequently require total gastrectomy, but antroduodenectomy should be considered because it may uncover an occult duodenal microneurogastrinoma or may correct AGCH."
11/01/2004 - "Recent studies have resolved some of the previous controversies including the surgical cure rate in patients with and without Multiple Endocrine Neoplasia-type1 (MEN1), the biological behavior of duodenal and pancreatic gastrinomas, role of imaging studies to localize tumor, and gastrectomy to manage acid output. "
05/01/2006 - "Among 579 MEN1 patients in the registry, 65 had NFPET < or = 2 cm. Fifteen (23%) underwent pancreatectomy, 9 at least segmental pancreatectomies and 6 biopsies or enucleations (the surgery group), and 50 (77%) were followed conservatively (the no surgery group). "
07/01/1993 - "Two of the three with MEN-I were also eugastrinemic after similar procedures, with the addition of distal pancreatectomy. "
06/01/2012 - "From 2000 to 2008, 52 MEN 1 patients [32 (62%) female; median age = 43 years, range 19-74 years] underwent 56 pancreatic operations [49 (88%) distal pancreatectomies] for suspected PNETs. "
08/01/1998 - "In patients with MEN 1, subtotal pancreatectomy with enucleation of tumours from the pancreatic head and uncinate process is recommended over simple enucleation because of frequent multiple tumors."
01/01/1994 - "Similarly, in patients with pancreatic and duodenal gastrinomas as a manifestation of multiple endocrine neoplasia type 1, the additional enucleation of pancreatic lesions with or without distal pancreatectomy has resulted in cure rates of 67% to 100%."
02/01/2013 - "Partial pancreaticoduodenectomy can provide cure for duodenal gastrinoma associated with multiple endocrine neoplasia type 1."
02/01/2006 - "Surgery to remove the gastrinoma in patients with multiple endocrine neoplasia type 1 is seldom curative unless a more extensive Whipple pancreaticoduodenectomy is performed. "
12/01/1994 - "We treated a diabetic patient with familial multiple endocrine neoplasia type 1 (MEN 1) who had undergone total pancreatoduodenectomy. "
01/01/2012 - "The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. "
11/01/2004 - "Current controversies as identified in the literature include the role of endoscopic ultrasound (EUS), surgery in ZES patients with MEN1, pancreaticoduodenectomy (Whipple procedure), lymph node primary gastrinoma, parietal cell vagotomy, reoperation and surgery for metastatic tumor, and the use of minimally invasive surgical techniques to localize and remove gastrinoma. "