An Update and Report Failure of Surgical Syndactyly Repair in Harlequin Ichthyosis.

Harlequin ichthyosis (HI) is a rare congenital skin disorder caused by irregular epidermal differentiation. Syndactyly in HI is associated with thick hyperkeratotic skin flexion and angulation deformity of the hand and fingers resulting in limited function of the upper extremity. Traditional syndactyly release is limited as full-thickness skin grafts typically used in reconstruction are composed of diseased skin and require donor sites in a patient predisposed for adverse wound healing. This case report is a follow-up to a previous viewpoint written about digital escharotomies in a newborn with HI and outlines a second and fourth webspace syndactyly release with a dermal substitute. Despite early evidence of adequate release and improved hand function, recurrence of syndactyly was observed within 4 months of surgical release. Our experience described within this case report may suggest the limitations and possible alternatives of surgical release of syndactyly in the HI population

Genomic Characterization of Methanomicrobiales Reveals Three Classes of Methanogens

BACKGROUND:Methanomicrobiales is the least studied order of methanogens. While these organisms appear to be more closely related to the Methanosarcinales in ribosomal-based phylogenetic analyses, they are metabolically more similar to Class I methanogens. METHODOLOGY/PRINCIPAL FINDINGS:In order to improve our understanding of this lineage, we have completely sequenced the genomes of two members of this order, Methanocorpusculum labreanum Z and Methanoculleus marisnigri JR1, and compared them with the genome of a third, Methanospirillum hungatei JF-1. Similar to Class I methanogens, Methanomicrobiales use a partial reductive citric acid cycle for 2-oxoglutarate biosynthesis, and they have the Eha energy-converting hydrogenase. In common with Methanosarcinales, Methanomicrobiales possess the Ech hydrogenase and at least some of them may couple formylmethanofuran formation and heterodisulfide reduction to transmembrane ion gradients. Uniquely, M. labreanum and M. hungatei contain hydrogenases similar to the Pyrococcus furiosus Mbh hydrogenase, and all three Methanomicrobiales have anti-sigma factor and anti-anti-sigma factor regulatory proteins not found in other methanogens. Phylogenetic analysis based on seven core proteins of methanogenesis and cofactor biosynthesis places the Methanomicrobiales equidistant from Class I methanogens and Methanosarcinales. CONCLUSIONS/SIGNIFICANCE:Our results indicate that Methanomicrobiales, rather than being similar to Class I methanogens or Methanomicrobiales, share some features of both and have some unique properties. We find that there are three distinct classes of methanogens: the Class I methanogens, the Methanomicrobiales (Class II), and the Methanosarcinales (Class III)

Natalizumab treatment shows low cumulative probabilities of confirmed disability worsening to EDSS milestones in the long-term setting.

Abstract Background Though the Expanded Disability Status Scale (EDSS) is commonly used to assess disability level in relapsing-remitting multiple sclerosis (RRMS), the criteria defining disability progression are used for patients with a wide range of baseline levels of disability in relatively short-term trials. As a result, not all EDSS changes carry the same weight in terms of future disability, and treatment benefits such as decreased risk of reaching particular disability milestones may not be reliably captured. The objectives of this analysis are to assess the probability of confirmed disability worsening to specific EDSS milestones (i.e., EDSS scores ≥3.0, ≥4.0, or ≥6.0) at 288 weeks in the Tysabri Observational Program (TOP) and to examine the impact of relapses occurring during natalizumab therapy in TOP patients who had received natalizumab for ≥24 months. Methods TOP is an ongoing, open-label, observational, prospective study of patients with RRMS in clinical practice. Enrolled patients were naive to natalizumab at treatment initiation or had received ≤3 doses at the time of enrollment. Intravenous natalizumab (300 mg) infusions were given every 4 weeks, and the EDSS was assessed at baseline and every 24 weeks during treatment. Results Of the 4161 patients enrolled in TOP with follow-up of at least 24 months, 3253 patients with available baseline EDSS scores had continued natalizumab treatment and 908 had discontinued (5.4% due to a reported lack of efficacy and 16.4% for other reasons) at the 24-month time point. Those who discontinued due to lack of efficacy had higher baseline EDSS scores (median 4.5 vs. 3.5), higher on-treatment relapse rates (0.82 vs. 0.23), and higher cumulative probabilities of EDSS worsening (16% vs. 9%) at 24 months than those completing therapy. Among 24-month completers, after approximately 5.5 years of natalizumab treatment, the cumulative probabilities of confirmed EDSS worsening by 1.0 and 2.0 points were 18.5% and 7.9%, respectively (24-week confirmation), and 13.5% and 5.3%, respectively (48-week confirmation). The risks of 24- and 48-week confirmed EDSS worsening were significantly higher in patients with on-treatment relapses than in those without relapses. An analysis of time to specific EDSS milestones showed that the probabilities of 48-week confirmed transition from EDSS scores of 0.0–2.0 to ≥3.0, 2.0–3.0 to ≥4.0, and 4.0–5.0 to ≥6.0 at week 288 in TOP were 11.1%, 11.8%, and 9.5%, respectively, with lower probabilities observed among patients without on-treatment relapses (8.1%, 8.4%, and 5.7%, respectively). Conclusions In TOP patients with a median (range) baseline EDSS score of 3.5 (0.0–9.5) who completed 24 months of natalizumab treatment, the rate of 48-week confirmed disability worsening events was below 15%; after approximately 5.5 years of natalizumab treatment, 86.5% and 94.7% of patients did not have EDSS score increases of ≥1.0 or ≥2.0 points, respectively. The presence of relapses was associated with higher rates of overall disability worsening. These results were confirmed by assessing transition to EDSS milestones. Lower rates of overall 48-week confirmed EDSS worsening and of transitioning from EDSS score 4.0–5.0 to ≥6.0 in the absence of relapses suggest that relapses remain a significant driver of disability worsening and that on-treatment relapses in natalizumab-treated patients are of prognostic importance

Beauty Beyond Compare: Effects of Context Extremity and Categorization On Hedonic Contrast

Three studies investigated the effects of extreme context stimuli and categorizationon hedonic contrast by having subjects judge the attractiveness of faces. Experiment 1 demonstrated hedonic contrast in both directions by using 2 sets of stimuli presented in different orders. Preceding moderately unattractive faces with moderately attractive facesmade the unattractive faces more unattractive. When the order of presentation was reversed, the moderately attractive faces became more attractive. Experiment 2 found that this hedonic contrast was eliminated when the moderately attractive faces were replaced with extremely attractive faces. Experiment 3 showed that even with those 2 sets of extremely different stimuli, hedonic contrast occurred if subjects were instructed to think of both sets of stimuli as belonging to the same category. These findings, using hedonic judgments, parallel Sarris\u27s (1967, 1968) finding with weights that when 2 sets of stimuli are toodifferent in the dimension being judged, no contrast occurs. They also lend support to his explanation for this result. When the 2 sets of stimuli are too different they are not seen as belonging to the same category. They are therefore not compared, and contrast does not occur. The authors propose that these principles might apply to contrast in all settings

Attractiveness Difference Magnitude Affected by Context, Range, and Categorization

Previous studies have shown that, when subjects view hedonically positive stimuli followed by stimuli of lesser hedonic value, their preference between the stimuli of lesser hedonic value decreases. This is hedonic condensation. In addition, its opposite, hedonic expansion, occurs when subjects view less hedonically positive stimuli followed by more hedonically positive stimuli. Experiment 1 showed both hedonic condensation and expansion in subjects who viewed pictures of unattractive and moderately attractive faces. Experiment 2 showed that, when subjects were instructed to view the stimuli as coming from two different groups, hedonic expansion but not hedonic condensation was eliminated. Experiment 3 showed that increasing the attractiveness difference between the attractive and unattractive faces eliminated all effects of context on subjects\u27 attractiveness difference judgments. Experiment 4 showed that forcing subjects to categorize the extremely attractive and unattractive faces into the same group resulted in hedonic expansion among attractive faces but not condensation among the unattractive faces. These results suggest that, as hedonic contrast changes the hedonic values of stimuli, it also changes the attention paid to those stimuli, thereby altering the degree of preference between them. Manipulations that prevent a shift in hedonic value also block a shift in the magnitude of the preference judgments

Chocolate Craving and the Menstrual Cycle

Spanish and American female chocolate cravers reported the usual times when they craved chocolate by answering an open-ended question. They also were asked directly if they craved chocolate perimenstrually. American women (40% open-ended, 60% direct) were more likely than were Spanish women (4% open-ended, 24% direct) to report that their chocolate cravings occurred perimenstrually when responding to both questions. The most frequently reported times (other than perimenstrually) that chocolate was craved were the same for Spanish men and women (after eating, studying) and for American men and women (evening), differing cross-culturally but not across gender. The results suggest a cultural origin rather than a physiological basis for chocolate craving

Use of Neuronavigation in Suturectomy for Craniosynostosis

Smaller operative exposures associated with suturectomy for craniosynostosis may result in difficulties visualizing the prematurely fused suture during surgery. The authors report cases of suturectomy for lambdoid and metopic craniosynostosis in which neuronavigation or frameless stereotaxy was used to assist with incision planning and intraoperative localization of the fused suture. In both cases, neuronavigation integrated easily and safely into established workflows and was associated with complete suture release. To our knowledge, this is the first report of applying this noninvasive technology, which does not require cranial pinning or rigid fixation, to suturectomy, and the authors demonstrate its use as an adjunct, especially for surgeons beginning in practice. Larger studies are needed to determine if neuronavigation in suturectomy is associated with a clinically significant reduction in blood loss or operative time or an increase in the rate of complete suturectomy

Compared to What? Effects of Categorization On Hedonic Contrast

Test stimuli are rated less good following very good context stimuli than when presented either alone or following neutral context stimuli. This diminution in rating is called hedonic contrast. In two experiments, the degree of hedonic contrast depended on how subjects were instructed to categorize context and test stimuli. Contrast was substantially attenuated if context and test stimuli were said to belong to different categories. The effect was demonstrated for beverages (Experiment 1) and birds (Experiment 2). Stimuli\u27s hedonic ratings were far less affected by other stimuli declared to belong to a different category than by stimuli declared to belong to a common category

Zygoma Fractures Are Associated With Increased Morbidity and Mortality in the Pediatric Population

BACKGROUND: Trauma involving the facial bones has been shown to be associated with high severity in previous studies. Characteristics of facial fractures in adults have been well described in the adult population, less so in the pediatric literature. Our investigation aims to define these epidemiological measures and risk factors for poor outcomes using the most recent data. METHODS: The 2016 Trauma Quality Improvement Program data bank was examined to study facial fracture pattern, mechanism of injury, and demographic descriptive data to characterize pediatric trauma patients. Multivariable regression analysis was performed to assess risk factors for morbidity and mortality in pediatric facial fracture patients. RESULTS: Of 51,168 total pediatric trauma patients, 2917 (5.7%) presented with facial fractures. Motor vehicle trauma was the most common mechanism of injury. Maxillary/malar fractures was the most common fracture type overall. Mandibular fractures were most common in the 0 to 1 age category while nasal bone fractures were more common in older patients. Patients with mandible fractures experienced the highest rate of operative management. Zygoma fracture was highly associated with concomitant traumatic brain injury. Multivariable regression analysis showed that fracture of the zygoma, concomitant traumatic brain injury, and cervical spine injury were risk factors for increased mortality. CONCLUSION: Facial fractures are a rare but significant form of trauma in the pediatric population. Our data suggests a slight change in fracture patterns compared to previous studies. Zygoma fractures, traumatic brain injury, and cervical spine injury are risk factors of increased mortality that clinicians should be aware of

Characteristics and Patterns of Facial Fractures in the Elderly Population in the United States Based on Trauma Quality Improvement Project Data

With the increase in the elderly population in the United States, the number of people seeking care for trauma injuries is expected to increase. However, nationwide studies on epidemiological profile of elderly facial fractures remain sparse. The authors present the epidemiological measures of elderly facial fractures on national scale. Characteristics of facial fractures among younger adults (18-64 years old) and the elderly population (65-74 years old, 75-84 years old and 85 years and above) have been examined using the 2016 American College of Surgeons-Trauma Quality Improvement Program databank. Variables studied included facial fracture patterns, mechanisms of injury, treatment variables, and demographic descriptive data. Of 104,183 elderly trauma patients, 3415 presented with facial fractures, making up 3.3% (versus 6.5% in younger adults). Majority of facial fractures in 85 and over group (60.7%) were experienced by females, compared to only 19.5% in the younger adult group. Most common mechanism of injury in the elderly was falls, versus motor vehicle trauma in younger adults. The most common type of facial fractures among both groups were nasal fractures. Elderly patients presented with fewer zygoma, mandibular and nasal fractures when compared to younger adults, whereas showing more maxillary/malar and orbital bone fractures. Elderly patients experienced less operative management (4.3%-8.2%) compared to younger adults (15.6%). Concurrently, mortality rates were higher in the elderly patients (9.9%-11.8%) when compared to the younger adults (8.0%). Elderly patients presented with different causes of injury, distribution of fractures, and rates of operative management compared to younger adults