bluuebunny/crossref_metadata_2025 huggingface dataset:
Extraordinary Transmission‐based Plasmonic Nanoarrays for Axially Super‐Resolved Cell Imaging
Authors: Choi Jong‐ryul , Kim Kyujung , Oh Youngjin , Kim Ah Leum , Kim Sook Young , Shin Jeon‐Soo , Kim Donghyun
Published: October 2013

Extraordinary transmission based axial imaging (EOT‐AIM) for cell microscopy is reported. EOT‐AIM uses linear arrays of nanoapertures, each of which samples target fluorescence up to a preset axial distance from surface, in combination with wide‐field microscopy for acquisition of lateral images. Current design of nanoapertures provides EOT‐AIM with axial super‐resolution that is as small as 20 nm for a depth range of 500 nm. Experiments were performed for the measurement of the axial distribution of ganglioside in mouse macrophage (RAW264.7) cells using FITC‐conjugated cholera toxin subunit B. The results were successfully confirmed with conventional confocal and total internal reflection fluorescence microscopy.
Stereoretentive Copper(II)‐Catalyzed Ritter Reactions of Secondary Cycloalkanols
Authors: Al‐huniti Mohammed H. , Lepore Salvatore D.
Published: October 2013

A Ritter‐like coupling reaction of cyclic alcohols and both aryl and alkyl nitriles to form amides catalyzed by copper(II) triflate is described. These reactions proceed in good yields under mild and often solvent‐free conditions. With 2‐ and 3‐substituted cycloalkanols, amide products are formed with near complete retention of configuration. This is likely due to fast nucleophilic capture of non‐planar carbocations (hyperconjomers) stabilized by ring hyperconjugation. A critical aspect of this novel catalytic cycle is the in situ activation of the alcohol substrates by thionyl chloride to form chlorosulfites.

magnified image
Asymmetric Hydrogenation of β‐Keto Sulfonamides and β‐Keto Sulfones with a Chiral Cationic Ruthenium Diamine Catalyst
Authors: Huang Xiao‐Fei , Zhang Shao‐Yun , Geng Zhi‐Cong , Kwok Chun‐Yuen , Liu Peng , Li Hai‐Yan , Wang Xing‐Wang
Published: October 2013

Optically active β‐hydroxy sulfonamides and β‐hydroxy sulfones are very important building blocks for the preparation of bioactive compounds and pharmaceuticals. In this work, a highly efficient asymmetric hydrogenation of β‐keto sulfonamides and β‐keto sulfones has been developed using the phosphine‐free chiral ruthenium complex Ru(OTf)(TsDPEN)(η6‐p‐cymene) as the catalyst, to afford the corresponding β‐hydroxy sulfonamides and β‐hydroxy sulfones in high yields with excellent optical purities. In addition, a cascade asymmetric hydrogenation/dynamic kinetic resolution (DKR) of racemic cyclic β‐keto sulfonamides and β‐keto sulfones was also realized using the same catalyst, to give the corresponding chiral cyclic β‐hydroxy sulfonamides and β‐hydroxy sulfones in good yields with excellent enantio‐ and diastereoselectivities.

magnified image
Copper‐Catalyzed Domino Synthesis of 4‐Oxopyrimido[1,2‐a]indole Derivatives
Authors: Wang Yuyuan , Wang Ruji , Jiang Yuyang , Tan Chunyan , Fu Hua
Published: October 2013

A novel, convenient and efficient copper‐catalyzed domino method for the synthesis of 4‐oxopyrimido[1,2‐a]indole derivatives has been developed, and the corresponding target products were obtained in moderate to good yields. The domino reactions involved sequential copper‐catalyzed intermolecular C‐arylation, intramolecular addition of NH to nitrile and nucleophilic attack of amino to carbonyl, and the obtained N‐(2‐halophenyl)‐3‐oxoalkanamides carry various functional groups.

magnified image
Efficient Indirect Electrochemical Synthesis of 2‐Substituted Benzoxazoles using Sodium Iodide as Mediator
Authors: Li Wei‐Cui , Zeng Cheng‐Chu , Hu Li‐Ming , Tian Hong‐Yu , Little R. Daniel
Published: October 2013

An electrochemical strategy for the efficient synthesis of 2‐substituted benzoxazoles was developed using a catalytic amount of sodium iodide (NaI) as a redox catalyst in a two‐phase buffer system.

magnified image
Magnetic Nanoparticles (CoFe2O4)‐Supported Phosphomolybdate as an Efficient, Green, Recyclable Catalyst for Synthesis of β‐Hydroxy Hydroperoxides
Authors: Li Pei‐He , Li Bao‐Le , An Zhi‐Min , Mo Li‐Ping , Cui Zhen‐Shui , Zhang Zhan‐Hui
Published: October 2013

Magnetic nanoparticles (CoFe2O4)‐supported phosphomolybdate ([CoFe2O4@SiO2‐PrNH2‐PMo]) was readily prepared and identified as an efficient catalyst for ring‐opening of various epoxides with ethereal hydrogen peroxide (H2O2). The reaction proceeded under mild conditions to give β‐hydroxy hydroperoxides (HHPs) in high yields at room temperature, and the catalyst could be magnetically separated and recycled.

magnified image
N,N′‐Dioxide–Scandium(III)‐Catalyzed Asymmetric Michael Addition of β,γ‐Unsaturated Butenolides to α,β‐Unsaturated γ‐Keto Esters
Authors: Ji Jie , Lin Lili , Zhou Lin , Zhang Yuheng , Liu Yangbin , Liu Xiaohua , Feng Xiaoming
Published: October 2013

A highly efficient N,N′‐dioxide–scandium(III) complex catalytic system has been developed for the asymmetric vinylogous Michael reaction of α‐angelica lactone and its derivatives to α,β‐unsaturated γ‐keto esters, affording the corresponding γ,γ‐disubstituted butenolide products in moderate to good yields (up to 93%) with high dr (up to >19:1) and ee values (up to 97%) under mild reaction conditions.

magnified image
Asymmetric Hydrogenation of Quinoxalines, Benzoxazines, and a Benzothiazine Catalyzed by Chiral Ruthenabicyclic Complexes
Authors: Arai Noriyoshi , Saruwatari Yu , Isobe Kotaro , Ohkuma Takeshi
Published: October 2013

The ruthenabicyclic complex RuCl[(R)‐daipena][(R)‐dm‐segphos] with potassium tert‐butoxide catalyzes the hydrogenation of 2‐alkylquinoxalines and a 3‐methyl‐2H‐1,4‐benzoxazine in toluene under 20–100 atm of hydrogen at 40 °C to afford S‐configured cyclic amino products in greater than 97% enantiomeric excess {DAIPENA=anion of DAIPEN at the 2‐position of an anisyl group, DAIPEN=1,1‐di(4‐anisyl)‐2‐isopropyl‐1,2‐ethylenediamine, DM‐SEGPHOS=(4,4′‐bi‐1,3‐benzodioxole)‐5,5′‐diylbis[di(3,5‐xylyl)phosphine]}. The high catalytic activity results in a turnover number as high as 9400. Hydrogenation of the benzoimine heterocycles with the RuCl[(R)‐daipena][(R)‐segphos]/potassium tert‐butoxide system yields the R‐configured products in high enantiomeric excess [SEGPHOS=(4,4′‐bi‐1,3‐benzodioxole)‐5,5′‐diylbis(diphenylphosphine)]. The mode of enantioselection is discussed based on transition state models involving six‐membered pericyclic structures.

magnified image
Vinylogous Organocatalytic Triple Cascade Reaction: Forging Six Stereocenters in Complex Spiro‐Oxindolic Cyclohexanes
Authors: Chatterjee Indranil , Bastida David , Melchiorre Paolo
Published: October 2013

We report a triple vinylogous cascade reaction, yielding valuable spiro‐oxindolic cyclohexane derivatives. The three‐component domino process proceeds by way of a catalyzed Michael/1,6‐addition/vinylogous aldol sequence affording the products with six stereogenic centers and very high control over the stereochemistry. The chemistry is based on a rare example of asymmetric 1,6‐addition to linear 2,4‐dienals proceeding with complete δ‐site selectivity. Key to the reaction development was a directing group positioned at the β‐dienal position, which was essential for achieving highly predictable reaction outcomes.

magnified image
Photoredox Catalysis for Organic Syntheses
Authors: Reckenthäler Melissa , Griesbeck Axel G.
Published: October 2013

Current examples of photoredox catalytic processes for CO, CN, CHal and CC bond formation are described with emphasis on the basic mechanistic scenarios. The focus is on the special aspects of photochemical redox catalysis especially on the electron transfer energetics, the use of electron transfer (ET) sensitizers and mediators as well as sacrificial reagents.

magnified image
Guiding the Selection of Processing Additives for Increasing the Efficiency of Bulk Heterojunction Polymeric Solar Cells
Authors: Vongsaysy Uyxing , Pavageau Bertrand , Wantz Guillaume , Bassani Dario M. , Servant Laurent , Aziz Hany
Published: October 2013

In bulk heterojunction (BHJ) polymeric organic solar cells (OSCs), the use of processing additives in the material formulation has emerged as a promising, cost‐effective, and widely applicable method for optimizing the phase separation between the donor (D) and acceptor (A) materials, thus increasing their efficiency. So far, however, there has been no systematic approach for identifying suitable processing additives for a given D:A system. A method based on the Hansen solubility parameters (HSPs) is proposed for guiding the selection of processing additives for a given D:A combination. The method is applied to the archetypical poly(3‐hexylthiophene) (P3HT) and [6,6]‐phenyl‐C61‐butyric acid methyl ester (PCBM) system. The HSPs of these materials are determined and used to define a set of numerical criteria that need to be satisfied by a processing additive in order for it to be effective in realizing a higher efficiency OSC. Applying the selection criteria results in the identification of three novel processing additives. OSCs made of these formulations demonstrate an increase in their short‐circuit current density (JSC) and power conversion efficiency (PCE). These results demonstrate the efficiency of these novel processing additives and show that the HSPs represent a useful tool to determine and explore new types of processing additives for BHJ‐OSCs.
Silicon‐Based Nanomaterials for Lithium‐Ion Batteries: A Review
Authors: Su Xin , Wu Qingliu , Li Juchuan , Xiao Xingcheng , Lott Amber , Lu Wenquan , Sheldon Brian W. , Wu Ji
Published: October 2013

There are growing concerns over the environmental, climate, and health impacts caused by using non‐renewable fossil fuels. The utilization of green energy, including solar and wind power, is believed to be one of the most promising alternatives to support more sustainable economic growth. In this regard, lithium‐ion batteries (LIBs) can play a critically important role. To further increase the energy and power densities of LIBs, silicon anodes have been intensively explored due to their high capacity, low operation potential, environmental friendliness, and high abundance. The main challenges for the practical implementation of silicon anodes, however, are the huge volume variation during lithiation and delithiation processes and the unstable solid‐electrolyte interphase (SEI) films. Recently, significant breakthroughs have been achieved utilizing advanced nanotechnologies in terms of increasing cycle life and enhancing charging rate performance due partially to the excellent mechanical properties of nanomaterials, high surface area, and fast lithium and electron transportation. Here, the most recent advance in the applications of 0D (nanoparticles), 1D (nanowires and nanotubes), and 2D (thin film) silicon nanomaterials in LIBs are summarized. The synthetic routes and electrochemical performance of these Si nanomaterials, and the underlying reaction mechanisms are systematically described.
Solution‐Processed Hydrogen Molybdenum Bronzes as Highly Conductive Anode Interlayers in Efficient Organic Photovoltaics
Authors: Soultati Anastasia , Douvas Antonios M. , Georgiadou Dimitra G. , Palilis Leonidas C. , Bein Thomas , Feckl Johann M. , Gardelis Spyros , Fakis Mihalis , Kennou Stella , Falaras Polycarpos , Stergiopoulos Thomas , Stathopoulos Nikolaos A. , Davazoglou Dimitris , Argitis Panagiotis , Vasilopoulou Maria
Published: October 2013

Highly efficient and stable organic photovoltaic (OPV) cells are demonstrated by incorporating solution‐processed hydrogen molybdenum bronzes as anode interlayers. The bronzes are synthesized using a sol‐gel method with the critical step being the partial oxide reduction/hydrogenation using an alcohol‐based solvent. Their composition, stoichiometry, and electronic properties strongly correlate with the annealing process to which the films are subjected after spin coating. Hydrogen molybdenum bronzes with moderate degree of reduction are found to be highly advantageous when used as anode interlayers in OPVs, as they maintain a high work function similar to the fully stoichiometric metal oxide, whereas they exhibit a high density of occupied gap states, which are beneficial for charge transport. Enhanced short‐circuit current, open‐circuit voltage and, fill factor, relative to reference devices incorporating either PEDOT‐PSS or a solution processed stoichiometric molybdenum oxide, are obtained for a variety of bulk heterojunction mixtures based on different polymeric donors and fullerene acceptors. In particular, high power conversion efficiencies are obtained in devices that employed the s‐HxMoO2.75 as the hole extraction layer.
Achieving Efficiency and Equity in Sugar Factories and Sugar‐Cropping Patterns Under Climate Change in Upper and Middle Egypt
Authors: Hamada Youssef M.
Published: October 2013

Drought as a natural phenomenon, due to variability of meteorological conditions, strikes worldwide but the severity of its impact depends on the vulnerabilities of water supply systems and economical and sectors as well as on the effectiveness of the adopted mitigation measures. The aim of this paper is to study achieving efficiency and equity in sugar factories and sugar‐cropping patterns in Upper and Middle Egypt by focusing on the Strategic Water Shortage Preparedness Plan, introduction methodologies, and specific action to fight drought within the general water‐planning framework. The Linear Programming Model was applied to calculate the sugar crops, sugar cane and sugar beet acreage, production, and income of both Upper and Middle Egypt. As a result of an optimal cropping pattern, the cultivation season would lose acreage by 7.260%, farm income increase by 1.774%, water uses decrease by 18.511%, and CO2emission and energy reduced by 14.96%. Overall, as a result of an optimal sugar‐cropping pattern, Egyptian sugar exports would decrease by $130.086 million US. [EconLit classification: Q130 and Q000].
Tomography images to analyze the deformation of the cavern in the continuous‐flow mixing of non‐Newtonian fluids
Authors: Patel Dineshkumar , Ein‐Mozaffari Farhad , Mehrvar Mehrab
Published: October 2013

Tomography, an efficient nonintrusive technique, was employed to visualize the flow in continuous‐flow mixing and to measure the cavern volume (Vc) in batch mixing. This study has demonstrated an efficient method for flow visualization in the continuous‐flow mixing of opaque fluids using two‐dimensional (2‐D) and 3‐D tomograms. The main objective of this study was to explore the effects of four inlet‐outlet configurations, fluid rheology (0.5–1.5% xanthan gum concentration), high‐velocity jet (0.317–1.660 m s−1), and feed flow rate (5.3 × 10−5−2.36 × 10−4m3s−1) on the deformation of the cavern. Dynamic tests were also performed to estimate the fully mixed volume (Vfully mixed) for the RT, A310, and 3AM impellers in a continuous‐flow mixing system, and it was found that Vfully mixedwas greater than Vc. Incorporating the findings of this study into the design criteria will minimize the extent of nonideal flows in the continuous‐flow mixing of complex fluids and eventually improve the quality of end‐products. © 2013 American Institute of Chemical EngineersAIChE J, 60: 315–331, 2014
Tightening methods for continuous‐time mixed‐integer programming models for chemical production scheduling
Authors: Merchan Andres F. , Velez Sara , Maravelias Christos T.
Published: October 2013

Significance

Important advances in modeling chemical production scheduling problems have been made in recent years, yet effective solution methods are still required. We use an algorithm that uses process network and customer demand information to formulate powerful valid inequalities that substantially improve the solution process. In particular, we extend the ideas recently developed for discrete‐time formulations to continuous‐time models and show that these tightening methods lead to a significant decrease in computational time, up to more than three orders of magnitude for some instances. © 2013 American Institute of Chemical Engineers AIChE J, 59: 4461–4467, 2013
Effect of race on outcomes after allogeneic hematopoietic cell transplantation for severe aplastic anemia
Authors: Eckrich Michael J. , Ahn Kwang‐Woo , Champlin Richard E. , Coccia Peter , Godder Kamar , Horan John , Margolis David , Deeg H.Joachim , Eapen Mary
Published: October 2013

We compared outcomes after hematopoietic cell transplantation in patients of African American (n = 84) and Caucasian (n = 215) descent with severe aplastic anemia. African Americans and Caucasians were matched for age, donor–recipient human leukocyte antigen match, graft type, and transplantation year. The median follow‐up of surviving patients was 5 years. In multivariate analysis, overall mortality risks were higher for African Americans compared to Caucasians (relative risk 1.73, P = 0.01). The 5‐year probabilities of overall survival adjusted for interval from diagnosis to transplantation, and performance score was 58% for African Americans and 73% for Caucasians. The day‐100 cumulative incidence of grade III–IV, but not grade II–IV acute graft‐versus‐host disease (GVHD), was higher in African Americans compared to Caucasians (29% vs. 13%, P = 0.006). Although the 5‐year cumulative incidence of chronic GVHD was not significantly different between the racial groups, African Americans were more likely to have extensive chronic GVHD compared to Caucasians (72% vs. 49%, P = 0.06). Survival differences between Caucasians and African Americans can be attributed to multiple factors. Our data suggest that some of the observed survival differences between Caucasians and African Americans may be explained by higher rates of acute GVHD and severity of chronic GVHD. Am. J. Hematol. 89:125–129, 2014. © 2013 Wiley Periodicals, Inc.
IDH mutations are closely associated with mutations of DNMT3A, ASXL1 and SRSF2 in patients with myelodysplastic syndromes and are stable during disease evolution
Authors: Lin Chien‐Chin , Hou Hsin‐An , Chou Wen‐Chien , Kuo Yuan‐Yeh , Liu Chieh‐Yu , Chen Chien‐Yuan , Lai Yan‐Jun , Tseng Mei‐Hsuan , Huang Chi‐Fei , Chiang Ying‐Chieh , Lee Fen‐Yu , Liu Ming‐Chih , Liu Chia‐Wen , Tang Jih‐Luh , Yao Ming , Huang Shang‐Yi , Ko Bor‐Sheng , Wu Shang‐Ju , Tsay Woei , Chen Yao‐Chang , Tien Hwei‐Fang
Published: October 2013

Current information about clinical significance of IDH mutations in myelodysplastic syndromes (MDS), their association with other genetic alterations and the stability during disease progression is limited. In this study, IDH mutations were identified in 4.6% of 477 patients with MDS based on the FAB classification and in 2.2 % of 368 patients based on the 2008 WHO classification. IDH mutations were closely associated with older age, higher platelet counts, and mutations of DNMT3A (36.4% vs. 8.7%, P < 0.001), ASXL1 (47.6% vs. 22.0%, P = 0.007), and SRSF2 (45.5% vs. 11.8%, P < 0.001). IDH2 mutation was a poor prognostic factor for overall survival in patients with lower‐risk MDS, based on international prognosis scoring system (IPSS), FAB classification, WHO classification, or revised IPSS (all P ≦ 0.001), but not in higher‐risk groups. Sequential studies in 151 patients demonstrated that all IDH‐mutated patients retained the same mutation during disease evolution while none of the IDH‐wild patients acquired a novel mutation during follow‐ups. In conclusion, IDH mutation is a useful biomarker for risk stratification of patients with lower‐risk MDS. IDH mutations are stable during the clinical course. The mutation, in association with other genetic alterations, may play a role in the development, but not progression of MDS.Am. J. Hematol. 89:137–144, 2014. © 2013 Wiley Periodicals, Inc.
Evaluation and management of anemia in the elderly
Authors: Goodnough Lawrence Tim , Schrier Stanley L.
Published: October 2013

Anemia is now recognized as a risk factor for a number of adverse outcomes in the elderly, including hospitalization, morbidity, and mortality. What constitutes appropriate evaluation and management for an elderly patient with anemia, and when to initiate a referral to a hematologist, are significant issues. Attempts to identify suggested hemoglobin levels for blood transfusion therapy have been confounded for elderly patients with their co‐morbidities. Since no specific recommended hemoglobin threshold has stood the test of time, prudent transfusion practices to maintain hemoglobin thresholds of 9–10 g/dL in the elderly are indicated, unless or until evidence emerges to indicate otherwise. Am. J. Hematol. 89:88–96, 2014. © 2013 Wiley Periodicals, Inc.
TEG and ROTEM: Technology and clinical applications
Authors: Whiting David , DiNardo James A.
Published: October 2013

Initially described in 1948 by Hertert thromboelastography (TEG) provides a real‐time assessment of viscoelastic clot strength in whole blood. Rotational thromboelastometry (ROTEM) evolved from TEG technology and both devices generate output by transducing changes in the viscoelastic strength of a small sample of clotting blood (300 µl) to which a constant rotational force is applied. These point of care devices allow visual assessment of blood coagulation from clot formation, through propagation, and stabilization, until clot dissolution. Computer analysis of the output allows sophisticated clot formation/dissolution kinetics and clot strength data to be generated. Activation of clot formation can be initiated with both intrinsic (kaolin, ellagic acid) and extrinsic (tissue factor) activators. In addition, the independent contributions of platelets and fibrinogen to final clot strength can be assessed using added platelet inhibitors (abciximab and cytochalasin D). Increasingly, ROTEM and TEG analysis is being incorporated in vertical algorithms to diagnose and treat bleeding in high‐risk populations such as those undergoing cardiac surgery or suffering from blunt trauma. Some evidence suggests these algorithms might reduce transfusions, but further study is needed to assess patient outcomes. Am. J. Hematol. 89:228–232, 2014. © 2013 Wiley Periodicals, Inc.
Treatment with bortezomib‐based regimens improves overall response and predicts for survival in patients with primary or secondary plasma cell leukemia: Analysis of the Greek myeloma study group
Authors: Katodritou Eirini , Terpos Evangelos , Kelaidi Charikleia , Kotsopoulou Maria , Delimpasi Sossana , Kyrtsonis Marie‐Christine , Symeonidis Argiris , Giannakoulas Nikos , Stefanoudaki Aikaterini , Christoulas Dimitrios , Chatziaggelidou Christina , Gastari Vassiliki , Spyridis Nikos , Verrou Evgenia , Konstantinidou Pavlina , Zervas Kostas , Dimopoulos Meletios A.
Published: October 2013

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, with poor outcome. Bortezomib‐based regimens (BBR) are highly effective in myeloma, but there is limited information about their efficacy and safety in PCL. Thus, we retrospectively collected data from 42 consecutive PCL patients (25 with primary PCL‐pPCL and 17 with secondary PCL‐sPCL) to explore the role of BBR in this entity. BBR were administered in 29 of 42 patients, while 6 of 25 patients with pPCL underwent autologous transplantation. Objective response (≥partial response) was significantly higher in patients treated with BBR versus conventional therapies (69% vs. 30.8%, P = 0.04); 27.5% of patients treated with BBR achieved at least very good partial response (vgPR). The highest ORR was observed in pPCL patients treated with BBR (88.9%; ≥vgPR: 33.3%). In BBR‐group, grade 3 of 4 hematological, neurological and renal toxicity and neutropenic infections were observed in 41.4%, 7%, 3.4%, and 31%, respectively. With a median follow‐up of 51 months, median overall survival (OS) for patients treated with BBR versus conventional therapies was 13 versus 2 months (P < 0.007). Median OS of patients with pPCL and sPCL treated with BBR was 18 and 7 months, respectively (P < 0.001). In the multivariate analysis normal PLTs, treatment with BBR and high quality response were the only powerful predictors for survival. Our study carrying the longest reported median follow‐up, demonstrated that treatment of PCL with BBR induces high response rates and prolongs survival over conventional therapies, regardless of additional autologous transplantation rescue or established high risk features, with manageable toxicity. Am. J. Hematol. 89:145–150, 2014. © 2013 Wiley Periodicals, Inc.
Predictive factors for latency period and a prognostic model for survival in patients with therapy‐related acute myeloid leukemia
Authors: Ornstein Moshe C. , Mukherjee Sudipto , Mohan Sanjay , Elson Paul , Tiu Ramon V. , Saunthararajah Yogenthiran , Kendeigh Cassie , Advani Anjali , Kalaycio Matt , Maciejewski Jaroslaw P. , Sekeres Mikkael A.
Published: October 2013

Therapy‐related acute myeloid leukemia (t‐AML) is an increasingly recognized sequela in patients receiving chemotherapy or radiotherapy for a primary malignancy or autoimmune disease. This study assessed factors related to the latency period (LP) between the antecedent disorder (AD) and t‐AML diagnosis and developed a comprehensive prognostic model to predict overall survival (OS). We evaluated a cohort of newly diagnosed t‐AML patients treated with cytarabine‐based induction therapy from 2001 to 2011. Multivariable linear and proportional hazards models were used to assess the impact of different classes of chemotherapy on the LP and to identify independent prognostic factors for OS. Of 730 treated AML patients, 58 (7.9%) had t‐AML. Median LP to t‐AML was 5.6 years (range, 0.5–38.4). 64% of patients achieved CR and median OS was 10.7 months. Independent prognostic factors of short LP were age at AD (P < 0.0001) and prior treatment with mitotic inhibitors (P = 0.05). Unfavorable cytogenetics (P = 0.004), antecedent hematologic or autoimmune disease (P = 0.01), age >60 (P = 0.03), and platelet count <30,000 μL (P = 0.04) at the time of t‐AML diagnosis were prognostic for inferior OS. A prognostic model using these factors was developed that risk stratified t‐AML patients into two groups: favorable and unfavorable. Patients in the favorable group had a median OS of 37.6 months compared with 6.4 months in patients comprising the unfavorable group (P < 0.0001). Multicomponent prognostic models integrating disease or treatment‐related covariates can help better understand how t‐AML evolves; and can be clinically useful in risk stratifying t‐AML patients undergoing induction therapy. Am. J. Hematol. 89:168–173, 2014. © 2013 Wiley Periodicals, Inc.
Hypercoagulability panel testing predicts thrombosis in neonates undergoing cardiac surgery
Authors: Emani Sirisha , Zurakowski David , Baird Christopher W. , Pigula Frank A. , Trenor Cameron , Emani Sitaram M.
Published: October 2013

Thrombosis contributes to morbidity and mortality in neonates following cardiac surgery. Alterations in hemostatic factors following cardiac surgery have been described, but there is no data correlating these changes with risk of thrombosis in neonates. The aim of this study is to predict thrombosis in neonates undergoing cardiac surgery by assessment of a panel of hypercoagulability markers. Neonates undergoing cardiac surgery were enrolled preoperatively and prospectively followed. Preoperative hypercoagulability panel testing included thrombin generation assay (TGA), immunoassays for antithrombin III, protein C, protein S, factor VIII, thrombin‐activatable fibrinolytic inhibitor (TAFI), plasminogen activator inhibitor‐1 (PAI‐1), and cardiolipin antibody. Postoperative thrombosis was defined by clinical events (shunt thrombosis, limb ischemia, and stroke) or imaging (intravascular or intracardiac thrombus). Risk factors for thrombosis were assessed. One hundred neonates were enrolled in the study over a two‐year period. The incidence of postoperative in‐hospital thrombosis was 20%. The only significant clinical risk factor associated with thrombosis was the single ventricle physiology. Hypercoagulability factors associated with increased risk of thrombosis by univariate analysis were elevated PAI‐1, TAFI, and TGA, and presence of anticardiolipin antibodies. Multivariable logistic regression analysis demonstrated that elevated PAI‐1 (P = 0.015), TAFI (P = 0.028), and TGA (P = 0.007) were independent predictors of thrombosis. Hypercoagulability panel testing may help identify neonates at high risk for thrombosis following cardiac surgery. Future studies are warranted to determine if high risk patients benefit from targeted anticoagulation therapies. Am. J. Hematol. 89:151–155, 2014. © 2013 Wiley Periodicals, Inc.
Retracted: Joined at the hip? A paleoepidemiological study of developmental dysplasia of the hip and its relation to swaddling practices among indigenous peoples of North America
Authors: Blatt Samantha H.
Published: October 2013

The above article, published online on 7 October 2013 in Wiley Online Library (wileyonlinelibrary.com), and in Volume 25, No. 6, pp. 821–834, November/December 2013, has been retracted by agreement between the author, the journal Editor‐in‐Chief, and Wiley Periodicals, Inc. The retraction has been agreed due to unattributed use of methodological statements and diagnostic criteria from “Brief communication: Developmental dysplasia of the hip in medieval London” by Piers D. Mitchell and Rebecca C. Redfern (American Journal of Physical Anthropology 144:3, March 2011, doi: 10.1002/ajpa.21448), from which Figure 1 was also adapted without proper acknowledgement, and from “Diagnostic criteria for developmental dislocation of the hip in human skeletal remains” by P.D. Mitchell and R.C. Redfern (International Journal of Osteoarchaeology 18:1, January/February 2008, doi: 10.1002/oa.919).
Pathogenic and obesogenic factors associated with inflammation in Chinese children, adolescents and adults
Authors: Thompson Amanda L. , Houck Kelly M. , Adair Linda , Gordon‐Larsen Penny , Du Shufa , Zhang Bing , Popkin Barry
Published: October 2013

Objectives

Influenced by pathogen exposure and obesity, inflammation provides a critical biological pathway linking changing environments to the development of cardiometabolic disease. This study tests the relative contribution of obesogenic and pathogenic factors to moderate and acute CRP elevations in Chinese children, adolescents and adults.

Methods

Data come from 8795 participants in the China Health and Nutrition Study. Age‐stratified multinomial logistic models were used to test the association between illness history, pathogenic exposures, adiposity, health behaviors and moderate (1–10 mg/L in children and 3–10 mg/L in adults) and acute (>10mg/L) CRP elevations, controlling for age, sex and clustering by household. Backward model selection was used to assess which pathogenic and obesogenic predictors remained independently associated with moderate and acute CRP levels when accounting for simultaneous exposures.

Results

Overweight was the only significant independent risk factor for moderate inflammation in children (RRR 2.10, 95%CI 1.13‐3.89). History of infectious (RRR 1.28, 95%CI 1.08‐1.52) and non‐communicable (RRR 1.37, 95%CI 1.12‐1.69) disease, overweight (RRR 1.66, 95%CI 1.45‐1.89) and high waist circumference (RRR 1.63, 95%CI 1.42‐1.87) were independently associated with a greater likelihood of moderate inflammation in adults while history of infectious disease (RRR 1.87, 95%CI 1.35‐2.56) and overweight (RRR 1.40, 95%CI 1.04‐1.88) were independently associated with acute inflammation. Environmental pathogenicity was associated with a reduced likelihood of moderate inflammation, but a greater likelihood of acute inflammation in adults.

Conclusions

These results highlight the importance of both obesogenic and pathogenic factors in shaping inflammation risk in societies undergoing nutritional and epidemiological transitions. Am. J. Hum. Biol. 26:18–28, 2014. © 2013 Wiley Periodicals, Inc.
Predictors for return to work for those with occupational respiratory disease: Clinical and structural factors
Authors: Zoeckler Jeanette M. , Cibula Donald A. , Morley Christopher P. , Lax Michael B.
Published: October 2013

Background

Few occupational researchers have examined “return to work” among patients with work‐related respiratory diseases. In addition, prior studies have emphasized individual patient characteristics rather than a more multi‐dimensional approach that includes both clinical and structural factors.

Methods

A retrospective chart review identified patients with occupational respiratory diseases in the Occupational Health Clinical Center, Syracuse, NY between 1991 and 2009. We assessed predictors of work status using an exploratory, sequential mixed methods research design, multinomial (n = 188) and Cox regressions (n = 130).

Results

The findings suggest that patients with an increased number of diagnoses, non‐union members, and those who took more than a year before clinical presentation had significantly poorer work status outcomes, after adjusting for age, education level, and relevant diagnoses.

Conclusions

Efforts to prevent slow return to work after developing occupational respiratory disease should recognize the importance of timely access to occupational health services, disease severity, union membership, and smoking status. Am. J. Ind. Med. 56:1371–1382, 2013. © 2013 Wiley Periodicals, Inc.
Investigation of respiratory and dermal symptoms associated with metal working fluids at an aircraft engine manufacturing facility
Authors: Meza Francisco , Chen Lilia , Hudson Naomi
Published: October 2013

Background

Each year, 1.2 million metalworkers are exposed to metalworking fluids (MWFs), which can cause dermal and respiratory disease. The National Institute for Occupational Safety and Health (NIOSH) conducted a health hazard evaluation of MWF exposures at an aircraft engine manufacturing facility. The objectives were to determine employee exposures to endotoxin and MWFs in the air, characterize symptoms experienced by employees working with MWFs, compare them to symptoms of employees unexposed to MWFs, and make recommendations for reducing exposures based on results.

Methods

Four hundred seven workers were categorized as MWF exposed or MWF unexposed and completed questionnaires. Estimated prevalence ratios (PR) of dermatitis, asthma, and work‐related asthma (WRA) symptoms were calculated. Airborne concentrations of MWF and endotoxin were measured, and work practices observed.

Results

MWF exposed workers had a significantly higher prevalence of dermatitis on wrists/forearms (PR 2.59; 95% CI 1.22, 5.46), asthma symptoms (PR 1.49; 95% CI 1.05, 2.13), and WRA symptoms (PR 2.10; 95% CI 1.22, 3.30) than unexposed workers. Airborne concentrations of MWF were below the NIOSH recommended exposure limit (REL) for MWF aerosols (thoracic particulate mass).

Conclusions

Despite MWF exposures below the NIOSH REL, exposed workers had a higher prevalence of asthma, WRA, and dermatitis symptoms than unexposed workers. Recommendations to reduce exposure included configuring mist collectors to automatically turn on when the machine is in use, and enforcing enclosure use. Am. J. Ind. Med. 56:1394–1401, 2013. © 2013 Wiley Periodicals, Inc.
Opioid poisonings and opioid adverse effects in workers in Washington State
Authors: Fulton‐Kehoe Deborah , Garg Renu K. , Turner Judith A. , Bauer Amy M. , Sullivan Mark D. , Wickizer Thomas M. , Franklin Gary M.
Published: October 2013

Objective

To examine trends in opioid poisonings and adverse effects in Washington (WA) State and nationally.

Methods

We calculated rates of opioid poisonings and adverse effects and examined opioid prescriptions in the WA workers' compensation system, 2004–2010. Using Health Care Cost and Utilization Project (HCUP), Nationwide Inpatient Sample (NIS) data, we also calculated national rates of opioid poisonings and adverse effects, 1993–2010.

Results

We identified 96 opioid poisonings and 312 opioid‐related adverse effects in WA, 2004–2010. The rates did not change substantially over these years. Most poisonings and adverse effects occurred in cases without chronic opioid use and with prescribed doses <120 mg/day morphine‐equivalent dose. Nationally, the rates of opioid poisonings and adverse effects increased significantly from 1993 to 2010.

Conclusions

Many poisonings and adverse effects occurred in patients without high dose or long‐term opioid therapy, suggesting that opioid dosing and duration guidelines may not be sufficient to reduce morbidity related to prescription opioid use. Am. J. Ind. Med. 56:1452–1462, 2013. © 2013 Wiley Periodicals, Inc.
MED12 related disorders
Authors: Graham John M. , Schwartz Charles E.
Published: October 2013

MED12

is a member of the large Mediator complex, which has a critical and central role in RNA polymerase II transcription. As a multiprotien complex, Mediator regulates signals involved in cell growth, development, and differentiation, and it is involved in a protein network required for extraneuronal gene silencing and also functions as a direct suppressor of Gli3‐dependent Sonic hedgehog signaling. This may explain its role in several different X‐linked intellectual disability syndromes that share some overlapping clinical features. This review will compare and contrast four different clinical conditions that have been associated with different mutations in MED12, which is located at Xq13. To date, these conditions include Opitz–Kaveggia (FG) syndrome, Lujan syndrome, Ohdo syndrome (Maat–Kievit–Brunner type, or OSMKB), and one large family with profound X‐linked intellectual disability due to a novel c.5898insC frameshift mutation that unlike the other three syndromes, resulted in affected female carriers and truncation of the MED12 protein. It is likely that more MED12 mutations will be detected in sporadic patients and X‐linked families with intellectual disability and dysmorphic features as exome sequencing becomes more commonly utilized, and this overview of MED12‐related disorders may help to correlate MED12 genotypes with clinical findings. © 2013 Wiley Periodicals, Inc.
Genetic counseling for susceptibility loci and neurodevelopmental disorders: The del15q11.2 as an example
Authors: De Wolf Veerle , Brison Nathalie , Devriendt Koenraad , Peeters Hilde
Published: October 2013

In recent years, several recurrent copy number variations (CNVs) that confer risk of neurodevelopmental disorders have been identified (e.g., del and dup 16p11.2, del15q13.3, del and dup 1q21.1, del16p13.3, del15q11.2). They are often inherited from an unaffected parent and lack phenotypic specificity. Although there is growing evidence from association studies to consider them as susceptibility CNVs, their clinical utility is debated. Yet the clinician is frequently challenged to deal with these counseling situations without guidelines or consensus. In this report, counseling issues and research opportunities are discussed, with the recurrent 15q11.2 BP1‐BP2 (including CYFIP1, NIPA1, NIPA2, TUBGCP5) as an example. Several clinical reports have been published describing patients with del15q11.2 featuring intellectual disability, developmental delay, neurological problems, autism spectrum disorder (ASD), attention problems, speech delay, and dysmorphism. The del15q11.2 was found to be significantly associated with intellectual disability, schizophrenia, epilepsy, and ASD. In this report we discuss how patient‐specific and family‐specific information may alter the interpretation of del15q11.2 as a contributing factor to the disorder in practical counseling situations. In addition, an association study for ASD in a Belgian Flemish cohort and an overview of reported association studies, clinical reports and genomics data for del15q11.2 are presented. © 2013 Wiley Periodicals, Inc.
Elements of morphology: General terms for congenital anomalies
Authors: Hennekam Raoul C. , Biesecker Leslie G. , Allanson Judith E. , Hall Judith G. , Opitz John M. , Temple I Karen , Carey John C. ,   Elements of Morphology Consortium
Published: October 2013

An international group of clinicians working in the field of dysmorphology has established a process for the standardization of terms used to describe human morphology. The goals are to standardize these terms and develop consensus regarding their definitions. This project will increase the usefulness and precision of descriptions of the human phenotype and facilitate reliable comparisons of phenotypic findings among clinicians and researchers in medicine, developmental biology, and genetics. Here we define and illustrate the general terms that describe congenital anomalies as related to human conditions. © 2013 Wiley Periodicals, Inc.
Biallelic SEMA3A defects cause a novel type of syndromic short stature
Authors: Hofmann Kristin , Zweier Markus , Sticht Heinrich , Zweier Christiane , Wittmann Wolfgang , Hoyer Juliane , Uebe Steffen , van Haeringen Arie , Thiel Christian T. , Ekici Arif B. , Reis André , Rauch Anita
Published: October 2013

Chromosomal microarray testing is commonly used to identify disease causing de novo copy number variants in patients with developmental delay and multiple congenital anomalies. In such a patient we now observed an 150 kb deletion on chromosome 7q21.11 affecting the first exon of the axon guidance molecule gene SEMA3A (sema domain, immunoglobulin domain (Ig), short basic domain, secreted, (semaphorin) 3A). This deletion was inherited from the healthy father, but considering the function of SEMA3A and phenotypic similarity to the knock‐out mice, we still assumed a pathogenic relevance and tested for a recessive second defect. Sequencing of SEMA3A in the patient indeed revealed the de novo in‐frame mutation p.Phe316_Lys317delinsThrSerSerAsnGlu. Cloning of the mutated allele in combination with two informative SNPs confirmed compound heterozygosity in the patient. While the altered protein structure was predicted to be benign, aberrant splicing resulting in a premature stop codon was proven by RT‐PCR to occur in about half of the transcripts from this allele. Expression profiling in human fetal and adult cDNA panels, confirmed a high expression of SEMA3A in all brain regions as well as in adult and fetal heart and fetal skeletal muscle. Normal intellectual development in the patient was surprising but may be explained by the remaining 20% of SEMA3A expression level demonstrated by quantitative RT‐PCR. We therefore report a novel autosomal recessive syndrome characterized by postnatal short stature with relative macrocephaly, camptodactyly, septal heart defect and several minor anomalies caused by biallelic mutations in SEMA3A. © 2013 Wiley Periodicals, Inc.
The growing complexity of the intestinal polyposis syndromes
Authors: Lucci‐Cordisco Emanuela , Risio Mauro , Venesio Tiziana , Genuardi Maurizio
Published: October 2013

Familial adenomatous polyposis has been the first form of inherited intestinal polyposis to be recognized. For a long time it has been considered the main polyposis syndrome, associated with an easily recognizable phenotype, with a marginal role attributed to a few very rare hamartomatous conditions. More recently, it has been gradually demonstrated that the intestinal polyposes encompass a range of conditions within a wide spectrum of disease severity, polyp histology, and extraintestinal manifestations. A growing number of genes and phenotypes has been identified, and heterogeneity of somatic molecular pathways underlying epithelial transformation in different syndromes and associated tumors has been documented. Increasing knowledge on the molecular bases and more widespread use of genetic tests has shown phenotypic overlaps between conditions that were previously considered distinct, highlighting diagnostic difficulties. With the advent of next generation sequencing, the diagnosis and the classification of these syndromes will be progressively based more on genetic testing results. However, the phenotypic variability documented among patients with mutations in the same genes cannot be fully explained by different expressivity, indicating a role for as yet unknown modifying factors. Until the latter will be identified, the management of patients with polyposis syndromes should be guided by both clinical and genetic findings. © 2013 Wiley Periodicals, Inc.
Science, art, and mistery in the statues and in the anatomical machines of the prince of sansevero: The masterpieces of the “Sansevero Chapel”
Authors: Della Monica Matteo , Galzerano Domenico , Di Michele Sara , Acquaviva Fabio , Gregorio Giovanni , Lonardo Fortunato , Sguazzo Francesca , Scarano Francesca , Lama Diana , Scarano Gioacchino
Published: October 2013

During the 18th century in Naples, Raimondo di Sangro, Prince of Sansevero, completed works on the family chapel, the so‐called “Cappella Sansevero.” The chapel houses statues of extraordinary beauty and spectacularly detailed but also, in the basement, two human skeletons known as the “Anatomical Machines” (“Macchine Anatomiche”). These two skeletons, a man and a pregnant woman, are entirely surrounded by their circulatory systems, just as if these were suddenly fixed. Legend, believed as truth until few years ago, says that Prince Raimondo had prepared and injected an unknown embalming substance in the blood vessels of two of his servants convicting them to eternal fixity. Recent investigations have demonstrated that, while the bones are authentic, the blood vessels are actually extraordinary artifacts that also reproduce some congenital malformations. The dreadful aspect of these two skeletons appears to be in strident contrast with the classic beauty of the statues which glorify and celebrate the ideal of morphology. Conversely, the two Anatomical Machines, protagonists of legends and superstitions since centuries, represent a marvelous example of science mixed with art. © 2013 Wiley Periodicals, Inc.
Multiple tumor types including leiomyoma and Wilms tumor in a patient with Gorlin syndrome due to 9q22.3 microdeletion encompassing the PTCH1 and FANC‐C loci
Authors: Garavelli Livia , Piemontese Maria Rosaria , Cavazza Alberto , Rosato Simonetta , Wischmeijer Anita , Gelmini Chiara , Albertini Enrico , Albertini Giuseppe , Forzano Francesca , Franchi Fabrizia , Carella Massimo , Zelante Leopoldo , Superti‐Furga Andrea
Published: October 2013

Gorlin syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant condition mainly characterized by the development of mandibular keratocysts which often have their onset during the second decade of life and/or multiple basal cell carcinoma (BCC) normally arising during the third decade. Cardiac and ovarian fibromas can be found. Patients with NBCCS develop the childhood brain malignancy medulloblastoma (now often called primitive neuro‐ectodermal tumor [PNET]) in 5% of cases. The risk of other malignant neoplasms is not clearly increased, although lymphoma and meningioma can occur in this condition. Wilms tumor has been mentioned in the literature four times. We describe a patient with a 10.9 Mb 9q22.3 deletion spanning 9q22.2 through 9q31.1 that includes the entire codifying sequence of the gene PTCH1, with Wilms tumor, multiple neoplasms (lung, liver, mesenteric, gastric and renal leiomyomas, lung typical carcinoid tumor, adenomatoid tumor of the pleura) and a severe clinical presentation. We propose including leiomyomas among minor criteria of the NBCCS. © 2013 Wiley Periodicals, Inc.