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Un insulinome sécrétant peut survenir une ou plusieurs fois dans la vie de patients atteints de néoplasies endocriniennes multiples de type 1 (NEM1), seul ou parmi d’autres tumeurs neuro-endocrines (TNE) pancréatiques sécrétantes, glucagonome, en particulier. Plusieurs lésions pancréatiques sont fréquemment identifiées en imagerie, notamment en TEP 68Ga-DOTATOC. L’enjeu est de repérer la (ou les) lésion(s) responsable(s) de l’hyperinsulinisme pour guider la chirurgie. Nous rapportons la performance dans cette indication de la TEP/TDM à l’exendine-4 marquée au 68Ga (EX), un ligand du récepteur GLP1.

Mutations in the presenilin-1 gene (PS1) account for a majority of patients with early-onset familial AD. However, the clinical indications and algorithms for genetic testing in dementia are still evolving.

To describe the clinical and molecular genetic analysis of a large family of northern Chinese descent with a mutation at the SCA2 locus causing carbidopa-levodopa–responsive parkinsonism.

The immunoglobulin heavy chain variable region is encoded as three separate libraries of elements in germ‐line DNA: VH, D and JH. To examine the order and regulation of their joining, we have developed assays that distinguish their various combinations and have used the assays to study tumor cell analogs of B‐lymphoid cells as well as normal B‐lymphoid cells. Abelson murine leukemia virus (A‐MuLV) transformed fetal liver cells ‐ the most primitive B‐lymphoid cell analog available for analysis ‐ generally had DJH rearrangements at both JH loci. These lines continued DNA rearrangement in culture, in most cases by joining a VH gene segment to an existing DJH complex with the concomitant deletion of intervening DNA sequences. None of these lines or their progeny showed evidence of VHD or DD rearrangements. Heavy chain‐producing tumor lines, representing more mature stages of the B‐cell pathway, and normal B‐lymphocytes had either two VHDJH rearrangements or a VHDJH plus a DJH rearrangement at their two heavy chain loci; they also showed no evidence of VHD or DD rearrangements. These results support an ordered mechanism of variable gene assembly during B‐cell differentiation in which D‐to‐JH rearrangements generally occur first and on both chromosomes followed by VH‐to‐DJH rearrangements, with both types of joining processes occurring by intrachromosomal deletion. The high percentage of JH alleles remaining in the DJH configuration in heavy chain‐producing lines and, especially, in normal B‐lymphocytes supports a regulated mechanism of heavy chain allelic exclusion in which a VHDJH rearrangement, if productive, prevents an additional VH‐to‐DJH rearrangement.

The Medway Project is a 660 MW combined cycle power plant, which employs two of the world’s largest advanced technology MS9001FA combustion turbine generators and an advanced design reheat steam turbine generator in a power plant system designed for high reliability and efficiency. This paper discusses the power plant system optimization and design, including thermodynamic cycle selection, equipment arrangement, and system operation. The design of the MS9001FA combustion turbine generator and the steam turbine generator, including tailoring for the specific application conditions, is discussed.

Abelson murine leukemia virus (A-MuLV)-transformed cells, simian virus 40 (SV40)-transformed cells, and chemically transformed cells all have increased levels of a 50,000-molecular-weight host cell protein. The protein was detected with sera raised to the A-MuLV-transformed and chemically transformed cells and was tightly bound to T-antigen in extracts of SV40-transformed cells. Partial protease digests showed that the proteins from all three sources were indistinguishable. The three proteins were phosphorylated in cells, and the linkage of phosphate to the A-MuLV-associated P50 was to a serine residue. By immunofluorescence methods, P50-related protein was found on the surface of both normal lymphoid cells and A-MuLV-transformed lymphoid cells, but cell fractionation showed that the majority of P50 was free in the cytoplasm of the transformed cells. Immunofluorescence also showed that P50 was found in granules in the cytoplasm of both untransformed and SV40-transformed fibroblasts. Other cells gave indistinct patterns. Cocapping experiments showed that the A-MuLV-specified P120 protein is weakly associated with the surface P50-related protein of lymphoid cells, but no association of P120 and P50 could be demonstrated by immunoprecipitation methods. Although a monoclonal antiserum to P50 was used in many of these studies, the identity of the bulk P50 protein with the molecules that are reactive at the cell surface requires further study.

We examined the interaction of Abelson murine leukemia virus protein P120 with other cellular components after extraction with the nonionic detergent Triton X-100. Most of the Abelson murine leukemia virus P120-associated kinase activity was found in the detergent-insoluble matrix in both lymphoid and fibroblast cell lines. The P120 labeled during a short exposure of cells to [35S]-methionine was mainly in the detergent-insoluble matrix (lymphoid cells) or equally distributed in the detergent-insoluble matrix and the soluble fraction (fibroblasts). Steady-state-labeled P120 was distributed equally in the two fractions (lymphoid cells) or mostly in the soluble portion (fibroblasts). Thus, there was an apparent movement of P120 from the detergent-insoluble matrix to the detergent-soluble fraction and a concomitant loss of enzymatic activity. When the detergent-insoluble matrix was incubated with [32P]ATP in situ, phosphorylation of tyrosine residues of P120 was observed. We found an 80,000-molecular-weight fragment of P120 (designated F80) after extraction of fibroblast cells with detergent. F80 was not found in extracted lymphoid cells, but mixing labeled lymphoid cells and unlabeled fibroblasts before extraction produced the fragment. F80 contained the gag determinants of P120 but did not react with Abelson-specific serum. These data allowed us to assign various features of the protein to regions of the P120 molecule and to localize the Abelson-specific antigenic determinants to the C-terminal region of the molecule.

Body counter measurements have been made on individuals who have undergone accidental exposure to 239Pu. The liver, lungs and other parts of the body were measured. The exposures are the result of contaminated wounds and inhalations. 241Am is present in the plutonium to which the individuals have been exposed. 241Am, which emits a 60-keV gamma-ray, is used as a tracer for making plutonium measurements. A plastic phantom was used to calibrate the counter by placing known amounts of 239Pu and 241Am into the major internal organs. The data were collected with a 512-channel pulse-height analyzer and a 4-in. by 4-mm-thick, thin-window NaI(Tl) detector. A gas flow proportional detector presently under development was used to supplement the scintillation detectors. About 150 unexposed people have been examined in the counter in order to establish background levels for a variety of body weights and 137Cs-40K content. These background levels are used as a basis for determining internal deposition.

To confirm recent observations that some vitamin D deficient osteomalacic patients had low plasma creatinine concentrations, low urinary creatinine excretion, and a paradoxically low creatinine clearance, and whether these changes were due to vitamin D deficiency per se, these measurements were made in patients before and after vitamin D supplementation. The pretreatment levels were also compared with those in healthy controls and in non-osteomalacic patients without renal disease. The above changes were confirmed. After treatment with vitamin D, plasma creatinine concentrations rose, but urinary creatinine excretion and creatinine clearance did not alter. These data indicate the limitation of creatinine clearance as an index of renal function in vegetarian osteomalacic patients. Furthermore, they demonstrate the effect of vitamin D deficiency and its reversal on plasma creatinine concentrations.