Bleeding complications associated with the molecular adsorbent recirculating system: a retrospective study

Background The molecular adsorbent recirculating system (MARS) is a hepatic replacement system that supports excretory liver function in patients with liver failure. However, since MARS has been employed in our hospital, bleeding complications have occurred in many patients during or after MARS. The objective of this study was to determine how MARS affects coagulopathy and identify specific factors associated with bleeding complications. Methods We retrospectively analyzed data from 17 patients undergoing a total of 41 MARS sessions. Complete blood count, coagulation profiles, and blood chemistry values were compared before and after MARS. To identify pre-MARS factors associated with increased bleeding after MARS, we divided patients into bleeder and non-bleeder groups and compared their pre-MARS laboratory values. Results MARS significantly reduced bilirubin and creatinine levels. MARS also increased prothrombin time and reduced platelet and fibrinogen, thus negatively impacting coagulation. Pre-MARS hemoglobin was significantly lower in the bleeder group than in the non-bleeder group (P=0.015). When comparing the upper and lower 33% of MARS sessions based on the hemoglobin reduction rate, hemoglobin reduction was significantly greater in MARS sessions involving patients with low pre-MARS international normalized ratio of prothrombin time (PT-INR) and factor V (P=0.038 and P=0.023, respectively). Conclusions MARS could appears to alter coagulation-related factors such as factor V and increase the risk of bleeding complications particularly in patient with low hemoglobin. However, individual differences among patients were large, and various factors, such as low hemoglobin, PT-INR, and factor V levels, appear to be involved

New virtual orthodontic treatment system for indirect bonding using the stereolithographic technique

The purpose of this article is to introduce a new virtual orthodontic treatment (VOT) system, which can be used to construct three-dimensional (3D) virtual models, establish a 3D virtual setup, enable the placement of the virtual brackets at the predetermined position, and fabricate the transfer jig with a customized bracket base for indirect bonding (IDB) using the stereolithographic technique. A 26-year-old woman presented with anterior openbite, crowding in the upper and lower arches, and narrow and tapered upper arch, despite having an acceptable profile and balanced facial proportion. The treatment plan was rapid palatal expansion (RPE) without extraction. After 10 days of RPE, sufficient space was obtained for decrowding. After a 10-week retention period, accurate pretreatment plaster models were obtained using silicone rubber impression. IDB was performed according to the protocol of the VOT system. Crowding of the upper and lower arches was effectively resolved, and anterior openbite was corrected to normal overbite. Superimposition of the 3D virtual setup models (3D-VSM) and post-treatment 3D virtual models showed that the latter deviated only slightly from the former. Thus, the use of the VOT system helped obtain an acceptable outcome in this case of mild crowding treated without extraction. More cases should be treated using this system, and the pre- and post-treatment virtual models should be compared to obtain feedback regarding the procedure; this will support doctors and dental laboratory technicians during the learning curve. (Korean J Orthod 2011;41(2):138-146)

Prenatally Detected Congenital Perineal Mass Using 3D Ultrasound which was Diagnosed as Lipoblastoma Combined with Anorectal Malformation: Case Report

We report a case of prenatally diagnosed congenital perineal mass which was combined with anorectal malformation. The mass was successfully treated with posterior sagittal anorectoplasty postnatally. On ultrasound examination at a gestational age of 23 weeks the fetal perineal mass were found on the right side. Any other defects were not visible on ultrasonography during whole gestation. Amniocentesis was performed to evaluate the fetal karyotyping and acetylcholinesterase which were also normal. As the fetus grew up, the mass size was slowly increased more and more. At birth, a female neonate had a perineal mass on the right side as expected. During operation, the anal sphincteric displacement was found near the mass and reconstructed through posterior sagittal incision. This is the first reported case of prenatally diagnosed congenital perineal mass, after birth which was diagnosed as lipoblastoma and even combined with anorectal malformation. This case shows that it can be of clinical importance to be aware of this rare fetal perineal mass in prenatal diagnosis and counseling

A Double Mutation of the Ryanodine Receptor Type 1 Gene in a Malignant Hyperthermia Family with Multiminicore Myopathy

Background and PurposeᄏAt least 100 Ryanodine receptor type 1 (RYR1) mutations associated with malignant hyperthermia (MH) and central core disease (CCD) have been identified, but 2 RYR1 mutations accompanying multiminicore myopathy in an MH and/or CCD family have been reported only rarely. MethodsᄏFifty-three members of a large MH family were investigated with clinical, histopathologic, RYR1 mutation, and haplotyping studies. Blood creatine kinase (CK) and myoglobin levels were also measured where possible. ResultsᄏSequencing of the entire RYR1 coding region identified a double RYR1 mutation (R2435H and A4295V) in MH/CCD regions 2 and 3. Haplotyping analysis revealed that the two missense heterozygous mutations (c.7304G>A and c.12891C>T) were always present on a common haplotype allele, and were closely cosegregated with histological multiminicores and elevated serum CK. All the subjects with the double mutation showed elevated serum CK and myoglobin, and the obtained muscle biopsy samples showed multiminicore lesions, but only two family members presented a late-onset, slowly progressive myopathy. ConclusionsᄏWe found multiminicore myopathy with clinical and histological variability in a large MH family with an unusual double RYR1 mutation, including a typical CCD-causing known mutant. These results suggest that multiminicore lesions are associated with the presence of more than two mutations in the RYR1 gene.ope

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1.

In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to examine autophagy and related processes, and for reviewers to provide realistic and reasonable critiques of reports that are focused on these processes. These guidelines are not meant to be a dogmatic set of rules, because the appropriateness of any assay largely depends on the question being asked and the system being used. Moreover, no individual assay is perfect for every situation, calling for the use of multiple techniques to properly monitor autophagy in each experimental setting. Finally, several core components of the autophagy machinery have been implicated in distinct autophagic processes (canonical and noncanonical autophagy), implying that genetic approaches to block autophagy should rely on targeting two or more autophagy-related genes that ideally participate in distinct steps of the pathway. Along similar lines, because multiple proteins involved in autophagy also regulate other cellular pathways including apoptosis, not all of them can be used as a specific marker for bona fide autophagic responses. Here, we critically discuss current methods of assessing autophagy and the information they can, or cannot, provide. Our ultimate goal is to encourage intellectual and technical innovation in the field