Bedeutung der PET/CT für die Chirurgie neuroendokriner Neoplasien

Neuroendocrine neoplasms (NEN) are rare malignancies with a wide spectrum of metastatic potential which originate from the endocrine cells of the body and express somatostatin receptors. The ⁶⁸gallium somatostatin receptor positron emission tomography-computed tomography (PET/CT) technique is the most sensitive method of assessment of well-differentiated NENs and for the detection of cancer of unknown primary (CUP syndrome) NENs. Imaging with 18F-fluorodeoxyglucose (18F-FDG PET/CT) is indicated in poorly differentiated neuroendocrine carcinomas. The receptor-dependent imaging of NENs has a decisive impact on further management.     

The role of therapeutic plasma exchange in pemphigus vulgaris

Background The treatment of pemphigus, an autoimmune bullous disease, is based on the combination of corticosteroids and adjuvant therapies, such as immunosuppressive drugs, anti‐inflammatory drugs and immunomodulatory procedures, such as intravenous immunoglobulin and therapeutic plasma exchange (TPE). Objective This study aims to assess our experience with TPE as a steroid‐sparing modality in moderate and severe intractable pemphigus patients. Methods A retrospective evaluation for all intractable pemphigus patients treated by TPE in a university‐affiliated tertiary referral medical centre between the years 1998 and 2008. Treatment protocol included three TPE treatments weekly for 1–3 months, combined with monthly pulse therapy of dexamethasone and/or cyclophosphamide. Maintenance therapy was based on once/bi weekly TPE treatments or monthly intravenous immunoglobulin. Results Seven patients were included in the study, four with severe pemphigus vulgaris and three with moderate disease. Six of the seven patients responded to TPE: Four patients (57%) achieved complete remission and two patients (28%) achieved partial remission on minimal therapy. Mild adverse effects related to TPE were observed in two patients and included dizziness and mild headache. Conclusion TPE is a well‐tolerated effective steroid‐sparing agent in recalcitrant pemphigus patients.     

Family Quality of Life: Canadian Results from an International Study

The quality of life of families that have a son or daughter with a disability has been an area overlooked by quality of life researchers until recently. The purpose of this study was to gather preliminary data on family quality of life and to report the findings of one part of an international study. The parents of 34 children and adults with intellectual disabilities were interviewed using the Family Quality of Life Survey, a measurement and assessment tool developed by a team of international researchers. Quantitative and qualitative data were collected for four concepts—opportunities available, initiative to access opportunities, attainment, and satisfaction—for each of the 9 key areas of family quality of life. In general, careers, spiritual and cultural beliefs, and family relationships were rated highly, while support from others and support from disability related services were rated quite low. Results provide a baseline for further research into the needs of these families, which is imperative for developing public policies and approaches to family-centered supports.     

Transgene optimization significantly improves SIN vector titers, gp91phox expression and reconstitution of superoxide production in X-CGD cells

Gene therapy has proven to be of potential value for the correction of inherited hematopoietic disorders. However, the occurrence of severe side effects in some of the clinical trials has questioned the safety of this approach and has hampered the use of long terminal repeat-driven vectors for the treatment of a large number of patients. The development of self-inactivating (SIN) vectors with reduced genotoxicity provides an alternative to the currently used vectors. Our initial attempts to use SIN vectors for the correction of a myeloid disorder, chronic granulomatous disease, failed due to low vector titers and poor transgene expression. The optimization of the transgene cDNA (gp91(phox)) resulted in substantially increased titers and transgene expression. Most notably, transgene optimization significantly improved expression of a second cistron located downstream of gp91(phox). Thus, optimization of the transgene sequence results in higher expression levels and increased therapeutic index allowing the use of low vector copy numbers per transduced cell and weaker internal promoters.     

Cardiovascular risk-associated allele frequencies for 15 genes in healthy elderly French and Chinese.

In order to investigate possible ethnic differences in genetic and environmental determinants, we investigated several cardiovascular disease-associated genetic variations in successful ageing populations of France (Nancy) and China (Hong Kong). Allelic frequencies of these genetic variations were compared between healthy elderly Chinese (n=103) and French populations (n=100). A multi-locus assay was used to genotype 15 genes for 29 biallelic sites, genes implicated in lipid and homocysteine metabolism, thrombosis, leukocyte adhesion, and blood pressure regulation. For most of the candidate markers within lipid metabolism genes, the less frequent alleles were more common in the Chinese population compared with the French population, while the less frequent alleles of the majority of the other markers were detected only or more commonly in the French population. In conclusion, polymorphisms in 13 genes exhibited statistically significant differences in allelic frequencies between the two populations. Since the two populations were selected as examples of successful ageing, we could hypothesise that genetic factors that could play a role in a successful ageing process may be different between the two populations.     

Analysis of the vasoconstrictor responses to potassium depolarization and norepinephrine and their antagonism by differing classes of vasodilators in the perfused rat hindquarters

Although the role of calcium in activation of vascular smooth muscle has received considerable attention, few studies have analyzed responses of resistance vessels. We therefore evaluated the role of calcium in vasoconstriction induced by norepinephrine (NE) and by high potassium depolarization in the resistance vessels of the Krebs'-perfused rat hindquarters. In addition, responses to vasodilators of differing classes were assessed. Injection of NE (0.3-100 micrograms) into the hindquarters increased perfusion pressure in a dose-related manner. Perfusion of the hindquarters with a depolarizing salt solution (80 mM K+) produced a sustained vasoconstriction (perfusion pressure = 143 +/- 4 mm Hg). Vasoconstriction to NE and high K+ was abolished during perfusion with a Ca-free salt solution containing 2 mM ethylene glycol bis(beta-aminoethyl ether)-N,N'-tetraacetic acid. In addition, the potassium-induced vasoconstriction was slightly reduced (perfusion pressure = 119 +/- 4 mm Hg) in animals pretreated with reserpine (5 mg/kg i.p.) indicating a slight neurogenic contribution to the response. The calcium channel blockers nifedipine (0.03-10 micrograms), diltiazem (0.3-30 micrograms) and verapamil (0.1-10 micrograms) produced dose-related vasodilation of hindquarters constricted with either NE (7.1 microM) or high potassium. These drugs were 4 to 10-fold more potent against potassium-induced vasoconstriction. Reserpine pretreatment enhanced the vasodilator response to nifedipine in potassium-depolarized preparations. Nitroprusside and nitroglycerin relaxed both NE and potassium-constricted hindquarters. The calmodulin blockers trifluoperazine and W-7 likewise produced vasodilation; trifluoperazine being 12.5 times more potent against NE-induced vasoconstriction.(ABSTRACT TRUNCATED AT 250 WORDS)