This population-based registry was designed to provide robust and updated information on the characteristics and the epidemiology of chronic myeloid leukemia (CML). All cases of newly diagnosed ...Philadelphia positive, BCR-ABL1+ CML that occurred in a sample of 92.5 million adults living in 20 European countries, were registered over a median period of 39 months. 94.3% of the 2904 CML patients were diagnosed in chronic phase (CP). Median age was 56 years. 55.5% of patients had comorbidities, mainly cardiovascular (41.9%). High-risk patients were 24.7% by Sokal, 10.8% by EURO, and 11.8% by EUTOS risk scores. The raw incidence increased with age from 0.39/100,000/year in people 20-29 years old to 1.52 in those >70 years old, and showed a maximum of 1.39 in Italy and a minimum of 0.69 in Poland (all countries together: 0.99). The proportion of Sokal and Euro score high-risk patients seen in many countries indicates that trial patients were not a positive selection. Thus from a clinical point of view the results of most trials can be generalized to most countries. The incidences observed among European countries did not differ substantially. The estimated number of new CML cases per year in Europe is about 6370.
To date, it is unclear which treatment modality, liver resection (LR) or transarterial chemoembolization (TACE) is the more appropriate for patients with huge (≥ 10 cm) hepatocellular carcinoma ...(HCC). The study aim was to compare, using propensity score matching, short- and long-term outcomes of patients with huge HCC who underwent potentially curative LR or TACE. Patients with huge HCC who had been managed at the Clinical Center by curative-intent LR or by palliative TACE between November 2001 and December 2018 were retrospectively identified. The morbidity and mortality rates and overall survival were compared between the groups before and after the propensity score matching. Independent predictors of long-term survival were determined by multivariate analysis. A total of 103 patients with huge HCC were included; 68 were assigned to the LR group and 35 to the TACE group. The overall morbidity rate was higher in the LR group than in the TACE group before matching (64.7% vs. 37.1%, p = 0.012), while there was no difference after matching (60% vs. 30%, p = 0.055). The major morbidity and 30-days mortality were similar between the groups before and after matching. The LR group was associated with longer overall survival than the TACE group before matching (p = 0.032) and after matching (p = 0.023). Total bilirubin and TACE treatment were independent prognostic factors associated with long-term survival. In patients with huge HCC, liver resection provides better long-term survival than TACE and should be considered as the initial treatment whenever possible.
The European Treatment and Outcome Study (EUTOS) population-based registry includes data of all adult patients newly diagnosed with Philadelphia chromosome-positive and/or BCR-ABL1+ chronic myeloid ...leukemia (CML) in 20 predefined countries and regions of Europe. Registration time ranged from 12 to 60 months between January 2008 and December 2013. Median age was 55 years and median observation time was 29 months. Eighty percent of patients were treated first line with imatinib, and 17% with a second-generation tyrosine kinase inhibitor, mostly according to European LeukemiaNet recommendations. After 12 months, complete cytogenetic remission (CCyR) and major molecular response (MMR) were achieved in 57% and 41% of patients, respectively. Patients with high EUTOS risk scores achieved CCyR and MMR significantly later than patients with low EUTOS risk. Probabilities of overall survival (OS) and progression-free survival for all patients at 12, 24 and 30 months was 97%, 94% and 92%, and 95%, 92% and 90%, respectively. The new EUTOS long-term survival score was validated: the OS of patients differed significantly between the three risk groups. The probability of dying in remission was 1% after 24 months. The current management of patients with tyrosine kinase inhibitors resulted in responses and outcomes in the range reported from clinical trials. These data from a large population-based, patient sample provide a solid benchmark for the evaluation of new treatment policies.
Purpose
The purpose of this study is to present a concept combining three modifications of the component separation technique (CST) in one procedure as an original solution for the management of ...complex subcostal abdominal wall hernia.
Methods
Between January 2010 and January 2020, seven patients presenting at the high-volume academic center with complex subcostal hernia underwent surgery in which three modifications of CST were combined into one procedure. Major complex subcostal hernia was defined by either width or length of the defect being greater than 10 cm. The following were the stages of the operative technique: (a) the “method of wide myofascial release” at the side of the hernia defect; (b) “open-book variation” of the component separation technique at the opposite side of the hernia defect; (c) a modified component separation technique for closure of midline abdominal wall hernias in the presence of enterostomies; (d) suturing of the myofascial flaps to each other to cover the defect; and (e) repair augmentation with an absorbable mesh in the onlay position.
Results
The median length and width of the complex subcostal hernias were 15 cm (10–19) and 15 cm (8–24), respectively. The overall morbidity rate was 57.1% (wound infection occurred in three patients, seroma in two patients, and skin necrosis in one patient). There was no hernia recurrence during the median follow-up period of 19 months.
Conclusion
The operative technique integrating three modifications of CST in one procedure with onlay absorbable mesh reinforcement is a feasible solution for the management of complex subcostal abdominal wall hernia.
Purpose
To investigate short and long-term outcome after the open preperitoneal flat mesh technique (OPFMT) for umbilical, epigastric, spigelian, small incisional and “port-site” hernia performed as ...a day case procedure.
Methods
We retrospectively analyzed records of patients who underwent OPFMT for umbilical, epigastric, Spigelian, small incisional and “port-site” hernia in ambulatory settings between 2004 and 2020 at Clinical Center of Serbia. Demographic and clinical characteristics, operative data and postoperative complications were compared between the groups. Univariate and multivariate analyses were performed to identify predictive factors for mesh infection and recurrence.
Results
Overall, 476 patients were divided according to the type of hernia. Early postoperative complications were similar in all study groups. Mesh infection, chronic pain and recurrence were different between groups (
p
= 0.013,
p
= 0.019 and
p
= 0.011, respectively). Overall recurrence rate after OPFMT was 2.5%. Hernia defect, hematoma and length of postoperative stay at the Day Surgery Unit were identified as potential predictors of mesh infection (Odds ratio 6.449, 22.143 and 1.546, respectively;
p
= 0.027,
p
= 0.011 and
p
= 0.038, respectively) while mesh infection was the only potential predictor of recurrence in univariate analysis. Hematoma was an independent predictor of recurrence (Odds ratio 27.068; 95% Confidence interval 2.355–311.073;
p
= 0.008).
Conclusion
The OPFMT performed under local anesthesia as a day case procedure is a safe technique associated with favorable long-term outcome. Hematoma is an independent predictor of mesh infection occurrence.
Telomerase Reverse Transcriptase (TERT) encodes the telomerase reverse transcriptase enzyme and is the most frequently mutated gene in patients with telomeropathies. Heterozygous variants impair ...telomerase activity by haploinsufficiency and pathogenic variants are associated with bone marrow failure syndrome and predisposition to acute myeloid leukaemia. Owing to their rarity, telomeropathies are often unrecognised and misdiagnosed. Herein, we report a novel TERT gene variant, c.2605G > A p.(Asp869Asn) in a family with hereditary aplastic anaemia. This report emphasises the importance of routine deep genetic screening for rare TERT variants in patients with a family history of cytopenia or aplastic anaemia, which could identify clinically inapparent telomere disorders.
The earthquake that took place at 03:56 local time on November 26, 2019, with Mw = 6.4, struck the west part of Albania and caused heavy damages to many public and residential buildings in the ...districts of Durres, Tirana, Lezha, Shkodra, Diver, Berat and the surrounding areas. Immediately after the earthquake, teams from the Institute of Earthquake Engineering and Engineering Seismology arrived and made rapid visual assessment of 169 damaged buildings in the affected area. During the inspection, severe damages to structural and nonstructural elements were found. This paper shows the analytical proof of the seismic behaviour of the structures in which damages were observed during visual assessment. Elastic (static and equivalent seismic force) analyses of elements were performed up to ultimate state of strength along with dynamic analyses of low-, mid- and high-rise buildings with different structural systems and characteristics corresponding to the period of their construction and the design legislation for such type of structures in Albania. The ductility and displacement capacities were defined for each of these groups of structures, while dynamic analysis was performed to define the displacements and the ductility caused by the real earthquake record and the intensity of the earthquake recorded in Durres. The results from the analyses confirm the behaviour of low- and mid-rise structures under this earthquake considering that damages observed on-site completely correspond to damages that occur under the displacements obtained from the analytical investigations. Since high-rise buildings were not found in the stricken region, their behaviour under an earthquake with the same characteristics as the one that took place in November 2019 was predicted applying the same methodology used for the low- and mid- rise buildings.
High‐resolution X‐ray diffraction data and Hansen–Coppens multipole formalism have been used to obtain the charge density distribution in two selenium adducts with the N‐heterocyclic carbenes ...1,3‐dimesitylimidazolidin‐2‐ylidene (1) and 1,3‐dimesityl‐4,5‐dioxoimidazolidin‐2‐ylidene (2). Application of Bader's quantum theory of atoms in molecules and calculation of the atomic charges by integration of the experimental electron density over the atomic basins showed that the main electrostatic difference between the Se adducts is in the significantly different accumulation of electron density in their C(carbene)–Se fragments (–0.10 vs. +0.30 e in 1 and 2). Deformation electron density features along the C–Se bond and topological values at the bond critical point indicate that this bond has π character in both compounds but it is more pronounced in the case of 2. This is in agreement with stronger C–N bonds and a larger N–C–N angle in 1. The experimental charge density distribution also clearly indicates the important role of the N‐mesityl substituent as an electron‐donating group, which increases the electron density in the imidazole ring thus contributing to the electronic stabilization of the carbene C atom. Because the N‐mesityl substituents donate similar amounts of electron density in both molecules (0.75 and 0.64 e in 1 and 2), it was concluded that the decisively different electronic properties of the two NHCs have to be attributed to their different backbone structures.
High‐resolution X‐ray diffraction experiments (100 K) have been used to investigate and quantitatively compare the electron density distribution in two carbene adducts of selenium.
At 03:56 local time on November 26, 2019, an earthquake with a Mw = 6.4 struck western part of Albania. The duration of the tremor lasted less than 50 s and was felt largely also in Albania's capital ...Tirana, and in places as far away more than 300 km northeast of the epicenter. It caused damage to many public and residential buildings in districts of Durres, Tirana, Lezha, Shkodra, Diver, Berat and surrounding areas. This paper describes rapid visual assessment of the damaged buildings (169 in total) in affected areas by IZIIS teams’ inspection of damaged buildings. Severe damages were identified in structural and non-structural elements as a result of inconsistent application of recent knowledge in design, construction and quality control of earthquake resistant structures. Structural errors in design and construction as well as inappropriate quality of built-in materials have been observed. Such results from the rapid damage assessment leads to the necessity of taking specific measures as detailed engineering inspection of vital structures as a basis of definition corresponding technical solutions for repair and strengthening with aim of restoring their operational mode. Last but not least, the biggest effect of earthquake damage was observed in non-structural elements which made the structures not-usable for citizens of the earthquake region.
Statistical analysis of data extracted from the Cambridge Structural Database (CSD) has been used to investigate the crystal structure properties of o-dialkoxybenzene derivatives, compounds ...containing two ether oxygen acceptors in ortho positions of benzene ring. It has been shown that in more than 90% of cases, the fragment has predictable geometrical characteristics where the two ether oxygens form short interatomic O···O contact (2.57 Å in average), while O-substitutents take trans position, both approximately coplanar with the benzene ring. Such arrangement of oxygen acceptors produces a large and uniform area of the negative electrostatic potential suitable for multiple hydrogen bonding. The acceptor abilities of the O···O system have been investigated by the statistical CSD analysis. The ab initio estimation of the interaction energy in the dimer of o-dimethoxybenzene (DMB) and H2O, employed as a model system, is achieved via high-level electron correlation CCSD(T) calculation with the complete basis set extrapolation. The interaction energy is estimated to be −6.5 kcal/mol. The results indicate the existence of a very flat potential energy surface in the region between methoxy oxygens and that DMB–water is a highly flexible system. The structural role of the O···O acceptor system is particularly interesting considering its ability to form multiple hydrogen bonding.
PDF
