Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1

Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger–Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger–Ellison syndrome, excision of duodenal gastrinomas together with clearance of regional lymph node metastases. This strategy, with early and aggressive surgery before metastases have developed, is believed to reduce the risks for tumor recurrence and malignant progression

Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1

Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger-Ellison syndrome, excision of duodenal gastrinomas together with clearance of regional lymph node metastases. This strategy, with early and aggressive surgery before metastases have developed, is believed to reduce the risks for tumor recurrence and malignant progression

Introduction: Forensic Fail

Background: About 60% of Pheochromocytoma (PCC) and Paraganglioma (PGL) patients have either germline or somatic mutations in one of the 12 proposed disease causing genes; SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, EPAS1, RET, NF1, TMEM127, MAX and H-RAS. Selective screening for germline mutations is routinely performed in clinical management of these diseases. Testing for somatic alterations is not performed on a regular basis because of limitations in interpreting the results. Aim: The purpose of the study was to investigate genetic events and phenotype correlations in a large cohort of PCC and PGL tumours. Methods: A total of 101 tumours from 89 patients with PCC and PGL were re-sequenced for a panel of 10 disease causing genes using automated Sanger sequencing. Selected samples were analysed with Multiplex Ligation-dependent Probe Amplification and/or SNParray. Results: Pathogenic genetic variants were found in tumours from 33 individual patients (37%), 14 (16%) were discovered in constitutional DNA and 16 (18%) were confirmed as somatic. Loss of heterozygosity (LOH) was observed in 1/1 SDHB, 11/11 VHL and 3/3 NF1-associated tumours. In patients with somatic mutations there were no recurrences in contrast to carriers of germline mutations (P = 0.022). SDHx/VHL/ EPAS1 associated cases had higher norepinephrine output (P = 0.03) and lower epinephrine output (P<0.001) compared to RET/NF1/H-RAS cases. Conclusion: Somatic mutations are frequent events in PCC and PGL tumours. Tumour genotype may be further investigated as prognostic factors in these diseases. Growing evidence suggest that analysis of tumour DNA could have an impact on the management of these patients

Repeated non-epileptic seizures in a previously healthy young woman – a case report

Efst á síðunni er hægt að nálgast greinina í heild sinni með því að smella á hlekkinn18 ára hraust stúlka kom endurtekið á slysadeild á 6 vikna tímabili vegna krampakasta. Engin fyrri saga var um flogaveiki og hún tók engin lyf. Myndrannsóknir og heilalínurit bentu ekki til flogaveiki. Hún mældist með lækkaðan blóðsykur í tvígang á slysadeild, 1,3 mmól/L og 1,7 mmól/L (4,0-6,0 mmól/L). Vaknaði þá grunur um insúlínmyndandi æxli. Gerð var víðtæk leit að æxlisvexti sem bar engan árangur. Var hún því send erlendis í frekari uppvinnslu, meðal annars í jáeindaskanna og sérhæfða æðamyndatöku. Hún var að lokum greind með nesidioblastosis. Hér verður fjallað um sjúkratilfellið auk yfirferðar um þennan sjaldgæfa sjúkdóm og uppvinnslu á honum.A previously healthy 18 year old female has repeated admissions over a six week period to the emergency department because of seizures. She has no previous history of epilepsy and denies any drug use. Imaging and electroencephalogram do not indicate epilepsy. Blood sugar levels are low on two occasions, 1.3 mmol / L and 1.7mmól / L (4.0 - 6.0 mmol / L). After further investigations the suspicion of an insulin-producing tumor arises. Extensive research and imaging is conducted to look for tumor growth without any findings. Subsequently she was sent abroad for further evaluation with a 11C-5HTP-PET scan, selective angiography with celiacography and an intra-arterial calcium stimulation test. She was diagnosed with nesidioblastosis. Here we will discuss the presentation and work-up of the medical case and review this rare causative diseas

Desorption products during linear heating of copper zeolites with pre-adsorbed methanol

Desorption products from zeolites with medium (MFI) and small (CHA) pores and with and without ion-exchanged copper were studied during linear heating after the pre-adsorption of methanol using a chemical flow reactor with a gas phase Fourier transform infrared spectrometer. The methanol desorption profiles were deconvoluted and compared with those predicted from first-principles calculations. In situ diffuse reflectance infrared Fourier transform spectroscopy was used to study the samples during methanol desorption following a step-wise increase of the sample temperature. It is shown that well-dispersed copper species in the Cu-zeolite samples interact more strongly with methanol and its derivatives as compared to the bare zeolites, resulting in methanol desorption at higher temperatures. Moreover, the introduction of Cu leads to CO formation and desorption in larger amounts at lower temperatures compared to the bare zeolites. The formation and desorption of dimethyl ether (DME) from pre-adsorbed methanol takes place at different temperatures depending on both the influence of Cu and the zeolite topology. The Cu sites in zeolites lead to higher DME formation/desorption temperatures, while a small shift of DME desorption towards higher temperatures is observed for the CHA framework structure compared to the MFI framework structure

Synthesis and Characterization of Catalytically Active Au Core─Pd Shell Nanoparticles Supported on Alumina

A two-step seeded-growth method was refined to synthesize Au@Pd core@shell nanoparticles with thin Pd shells, which were then deposited onto alumina to obtain a supported Au@Pd/Al2O3 catalyst active for prototypical CO oxidation. By the strict control of temperature and Pd/Au molar ratio and the use of l-ascorbic acid for making both Au cores and Pd shells, a 1.5 nm Pd layer is formed around the Au core, as evidenced by transmission electron microscopy and energy-dispersive spectroscopy. The core@shell structure and the Pd shell remain intact upon deposition onto alumina and after being used for CO oxidation, as revealed by additional X-ray diffraction and X-ray photoemission spectroscopy before and after the reaction. The Pd shell surface was characterized with in situ infrared (IR) spectroscopy using CO as a chemical probe during CO adsorption-desorption. The IR bands for CO ad-species on the Pd shell suggest that the shell exposes mostly low-index surfaces, likely Pd(111) as the majority facet. Generally, the IR bands are blue-shifted as compared to conventional Pd/alumina catalysts, which may be due to the different support materials for Pd, Au versus Al2O3, and/or less strain of the Pd shell. Frequencies obtained from density functional calculations suggest the latter to be significant. Further, the catalytic CO oxidation ignition-extinction processes were followed by in situ IR, which shows the common CO poisoning and kinetic behavior associated with competitive adsorption of CO and O2 that is typically observed for noble metal catalysts

Para-chloro-2-[18F]fluoroethyl-etomidate: A promising new PET radiotracer for adrenocortical imaging.

Introduction: [11C]Metomidate ([11C]MTO), the methyl ester analogue of etomidate, was developed as a positron emission tomography (PET) radiotracer for adrenocortical tumours and has also been suggested for imaging in primary aldosteronism (PA). A disadvantage of [11C]MTO is the rather high non-specific binding in the liver, which impacts both visualization and quantification of the uptake in the right adrenal gland. Furthermore, the short 20-minute half-life of carbon-11 is a logistic challenge in the clinical setting. Objectives: The aim of this study was to further evaluate the previously published fluorine-18 (T1/2=109.5 min) etomidate analogue, para-chloro-2-[18F]fluoroethyl etomidate; [18F]CETO, as an adrenal PET tracer. Methods: In vitro experiments included autoradiography on human and cynomolgus monkey (non-human primate, NHP) tissues and binding studies on adrenal tissue from NHPs. In vivo studies with [18F]CETO in mice, rats and NHP, using PET and CT/MRI, assessed biodistribution and binding specificity in comparison to [11C]MTO. Results: The binding of [18F]CETO in the normal adrenal cortex, as well as in human adrenocortical adenomas and adrenocortical carcinomas, was shown to be specific, both in vitro (in humans) and in vivo (in rats and NHP) with an in vitro Kd of 0.66 nM. Non-specific uptake of [18F]CETO in NHP liver was found to be low compared to that of [11C]MTO. Conclusions: High specificity of [18F]CETO to the adrenal cortex was demonstrated, with in vivo binding properties qualitatively surpassing those of [11C]MTO. Non-specific binding to the liver was significantly lower than that of [11C]MTO. [18F]CETO is a promising new PET tracer for imaging of adrenocortical disease and should be evaluated further in humans

Steps and catalytic reactions: CO oxidation with preadsorbed O on Rh(553)

Industrial catalysts are often comprised of nanoparticles supported on high-surface-area oxides, in order to maximise the catalytically active surface area and thereby utilise the active material better. These nanoparticles expose steps and corners that, due to low coordination to neighboring atoms, are more reactive and, as a consequence, are often assumed to have higher catalytic activity. We have investigated the reaction between CO and preadsorbed O on a stepped Rh(553) surface, and show that CO oxidation indeed occurs faster than on the flat Rh(111) surface at the same temperature. However, we do find that this is not a result of reactions at the step sites but rather at the terrace sites close to the steps, due to in-plane relaxation enabled by the step. This insight can provide ways to optimize the shape of the nanoparticles to further improve the activity of certain reactions