Combination of Conservative and Interventional Therapy Strategies for Intra- and Extrahepatic Cholangiocellular Carcinoma: A Retrospective Survival Analysis

Background. Due to the predominantly advanced stage at the time of diagnosis treatment of cholangiocarcinoma is difficult. Apart from surgical resection, interventional treatment strategies are increasingly used in advanced stage tumours. The aim of the study was a retrospective comparison of the effect of the various forms of treatment on morbidity and mortality. Method. A total of 195 patients, received either chemotherapy or a combination of photodynamic therapy (PDT) or transarterial chemoembolization (TACE) and chemotherapy. Results. The median survival rate for all patients was 15.6 months, 50.8% were still alive 1 year after diagnosis. Patients, who had previously undergone surgery, survived 17.1 months longer than those without surgical treatment (P < .01). Chemotherapy prolonged the survival by 9.2 months (P = .47). Palliative patients under combination of chemotherapy and PDT survived on average 1.8 months longer (P = .28), with chemotherapy and TACE 9.8 months longer (P = .04) compared to chemotherapy alone. Conclusions. It appears that surgical treatment and chemotherapy combined with PDT or TACE may prolong survival

Hematopoietic stem cell transplantation in a patient with proteasome-associated autoinflammatory syndrome (PRAAS)

Background: Proteasome-associated autoinflammatory syndromes (PRAASs) form a family of recently described rare autosomal recessive disorders of disturbed proteasome assembly and proteolytic activity caused by mutations in genes coding for proteasome subunits. The treatment options for these proteasome disorders consist of lifelong immunosuppressive drugs or Janus kinase inhibitors, which may have partial efficacy and noticeable side effects. Because proteasomes are ubiquitously expressed, it is unknown whether hematopoietic stem cell transplantation (HSCT) may be a sufficient treatment option. Objective: Our aim was to report the case of a young boy with a treatment-resistant cutaneous vasculitis that was initially suspected to be associated with a gene variant in SH2D1A. Methods: Whole-exome sequencing was performed to identify the genetic defect. Molecular and functional analyses were performed to assess the impact of variants on proteasomal function. The immune characterization led to the decision to perform HSCT on our patient and conduct follow-up over the 7-year period after the transplant. Because loss of myeloid chimerism after the first HSCT was associated with relapse of autoinflammation, a second HSCT was performed. Results: After the successful second HSCT, the patient developed mild symptoms of lipodystrophy, which raised the suspicion of a PRAAS. Genetic analysis revealed 2 novel heterozygous variants in PSMB4 (encoding proteasomal subunit beta 7). Retrospective analysis of patient cells stored before the first HSCT and patient cells obtained after the second HSCT demonstrated that HSCT successfully rescued proteasome function, restored protein homeostasis, and resolved the interferon-stimulated gene signature. Furthermore, successful HSCT alleviated the autoinflammatory manifestations in our patient. Conclusion: Patients with treatment-resistant PRAAS can be cured by HSCT.Transplantation and immunomodulatio

Survival rate in patients with hepatocellular carcinoma: a retrospective analysis of 389 patients

Hepatocellular carcinoma (HCC) is the fifth most common cancer worldwide. However, treatment options are limited and often inefficient. The aim of this study was to determine current survival rates for patients diagnosed with HCC and to identify prognostic factors, which will help in choosing optimal therapies for individual patients. A retrospective analysis of medical records was performed on 389 patients who were identified through the central tumour registry at our institution from 1998 to 2003. Clinical parameters, treatments received and survival curves from time of diagnosis were analysed. Overall median survival was 11 months. Liver cirrhosis was diagnosed in 80.5% of all patients. A total of 170 patients received transarterial chemoembolisation (TACE) and/or percutaneous ethanol injections (PEI) with a median survival rate of 16 months for patients receiving TACE, 11 months for patients receiving PEI and 24 months for patients receiving TACE followed by PEI. Independent negative prognostic parameters for survival were the presence of portal vein thrombosis, advanced liver cirrhosis (Child–Pugh score B or C) and a score of >2. This study will help to estimate survival rates for patients with HCC according to their clinical status at diagnosis and the treatments received

Metabolic, hormonal and performance effects of isomaltulose ingestion before prolonged aerobic exercise: a double-blind, randomised, cross-over trial

Background Isomaltulose has been discussed as a low glycaemic carbohydrate but evidence concerning performance benefits and physiological responses has produced varying results. Therefore, we primarily aimed to investigate the effects of isomaltulose ingestion compared to glucose and maltodextrin on fat and carbohydrate oxidation rates, blood glucose levels and serum hormone concentrations of insulin and glucose-dependent insulinotropic polypeptide (GIP). As secondary aims, we assessed running performance and gastrointestinal discomfort. Methods Twenty-one male recreational endurance runners performed a 70-min constant load trial at 70% maximal running speed (Vmax), followed by a time to exhaustion (TTE) test at 85% Vmax after ingesting either 50 g isomaltulose, maltodextrin or glucose. Fat and carbohydrate oxidation rates were calculated from spiroergometric data. Venous blood samples for measurement of GIP and insulin were drawn before, after the constant load trial and after the TTE. Capillary blood samples for glucose concentrations and subjective feeling of gastrointestinal discomfort were collected every 10 min during the constant load trial. Results No between-condition differences were observed in the area under the curve analysis of fat (p = 0.576) and carbohydrate oxidation rates (p = 0.887). Isomaltulose ingestion led to lower baseline postprandial concentrations of blood glucose compared to maltodextrin (percent change [95% confidence interval], − 16.7% [− 21.8,-11.6], p < 0.001) and glucose (− 11.5% [− 17.3,-5.7], p = 0.001). Similarly, insulin and GIP concentrations were also lower following isomaltulose ingestion compared to maltodextrin (− 40.3% [− 50.5,-30.0], p = 0.001 and − 69.1% [− 74.3,-63.8], p < 0.001, respectively) and glucose (− 32.6% [− 43.9,-21.2], p = 0.012 and − 55.8% [− 70.7,-40.9], p < 0.001, respectively). Furthermore, glucose fluctuation was lower after isomaltulose ingestion compared to maltodextrin (− 26.0% [− 34.2,-17.8], p < 0.001) and glucose (− 17.4% [− 29.1,-5.6], p < 0.001). However, during and after exercise, no between-condition differences for glucose (p = 0.872), insulin (p = 0.503) and GIP (p = 0.244) were observed. No between-condition differences were found for TTE (p = 0.876) or gastrointestinal discomfort (p = 0.119). Conclusion Isomaltulose ingestion led to lower baseline postprandial concentrations of glucose, insulin and GIP compared to maltodextrin and glucose. Consequently, blood glucose fluctuations were lower during treadmill running after isomaltulose ingestion, while no between-condition differences were observed for CHO and fat oxidation rates, treadmill running performance and gastrointestinal discomfort. Further research is required to provide specific guidelines on supplementing isomaltulose in performance and health settings

Einsatzmoeglichkeiten nativer Oele und Fette als Chemierohstoffe. 1.2: Biokatalytische Reaktionen von ungewoehnlichen Fettsaeuren mit Alkoholen und Zuckern Schlussbericht

SIGLEAvailable from TIB Hannover: F95B306+a / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Forschung und Technologie (BMFT), Bonn (Germany)DEGerman

Kritik der Jugendstrafvollzugsreform

UuStB Koeln(38)-5G7086 / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekSIGLEDEGerman