bluuebunny/crossref_metadata_2025_split huggingface dataset:
LEOPARD syndrome: Clinical diagnosis in the first year of life
Authors: Digilio M. Cristina , Sarkozy Anna , de Zorzi Andrea , Pacileo Giuseppe , Limongelli Giuseppe , Mingarelli Rita , Calabrò Raffaele , Marino Bruno , Dallapiccola Bruno 
Published: March 2006

AbstractLEOPARD syndrome (LS) is an autosomal dominant syndrome characterized by multiple lentigines and café‐au‐lait spots, electrocardiographic‐conduction abnormalities, ocular hypertelorism/obstructive cardiomyopathy, pulmonary stenosis, abnormalities of the genitalia in males, retardation of growth, and deafness. LS shares many features with Noonan syndrome (NS), in which lentigines and deafness are usually not present. Molecular studies have shown that LS and NS are allelic disorders, caused by different missense mutations in PTPN11, a gene encoding the protein tyrosine phosphatase SHP‐2 located at chromosome 12q22‐qter. The clinical diagnosis of LS is generally difficult in the first months of life because the distinctive lentigines are generally not present at birth and develop during childhood. From January 2002 to December 2004, we suspected LS clinically in 10 patients admitted to our genetic counseling services in the first 12 months of life. A PTPN11 gene mutation was detected in 8/10 (80%) patients. In one patient without a PTPN11 mutation a subsequent clinical diagnosis of neurofibromatosis type 1 (NF1) was made, following the evaluation of the mother, who had previously undiagnosed classic NF1. The age of LS patients with PTPN11 mutation ranged between 1 and 11 months (mean age ± SD 7.5 ± 3.96 months). Review of the clinical characteristics of patients with LS confirmed by molecular study during the first year of life demonstrates that the diagnosis of LS in the first months of age can be clinically suspected in patients presenting with three main features, that is, characteristic facial features (100%), hypertrophic cardiomyopathy (HCM) (87%), and cafe‐au‐lait spots (75%). Characteristic facial features can be mild or severe, and consist of hypertelorism, downslanting palpebral fissures, ptosis, and dysmorphic ears. The clinical suspicion of LS may be confirmed by molecular screening for PTPN11 mutations. An early diagnosis of the disease is useful for the prospective care of associated medical problems and for precise genetic counseling. © 2006 Wiley‐Liss, Inc.
Dural ectasia in children with Marfan syndrome: A prospective, multicenter, patient‐control study
Authors: Knirsch Walter , Kurtz Claudia , Häffner Nicole , Binz Gudrun , Heim Peter , Winkler Peter , Baumgartner Daniela , Freund‐Unsinn Karin , Stern Heiko , Kaemmerer Harald , Molinari Luciano , Kececioglu Deniz , Uhlemann Frank 
Published: March 2006

AbstractThe clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient‐control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome. Design: Prospective clinical trial, multicentric, cross‐sectional. Setting: MRI of the lumbosacral spine. Patients: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. Outcome measures: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine. Results: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6–8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls. Conclusion: Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome. © 2006 Wiley‐Liss, Inc.
Downstaging colorectal liver metastases by concomitant unilateral portal vein ligation and selective intra-arterial chemotherapy
Authors: Selzner N , Pestalozzi B C , Kadry Z , Selzner M , Wildermuth S , Clavien P-A 
Published: March 2006

Abstract

Background
Although selective intrahepatic arterial chemotherapy successfully downstaged irresectable colorectal liver metastases in a previous study, curative resection was rarely possible, as the remnant healthy liver volume was inadequate. This pilot study evaluated the efficacy of concomitant unilateral portal vein ligation and selective intrahepatic arterial chemotherapy in downstaging such tumours.

Methods
The study included 11 patients with irresectable colorectal liver metastases. Selective intrahepatic arterial chemotherapy was delivered using a subcutaneous pump, and each patient underwent concomitant unilateral portal vein ligation of the hemiliver judged to have the higher tumour load. Chemotherapy involved serial administration of floxuridine for 2 weeks every 4 weeks.

Results
All patients developed significant atrophy of the hemiliver subjected to portal vein ligation and contralateral hypertrophy. There was no increase in tumour load within 6 months of therapy, and the load decreased by 60 per cent in the hemiliver ipsilateral to the ligated vein. At 3 months, six of 11 patients showed a significant response to chemotherapy. In four patients, downstaging allowed curative resection after only three cycles of chemotherapy. These patients survived at least 20 months afterwards.

Conclusion
Combined unilateral portal vein ligation and selective intrahepatic arterial chemotherapy produced substantial atrophy of the ipsilateral hemiliver along with contralateral hypertrophy, without increased tumour growth in the regenerating hemiliver.
Surgical intervention in patients with necrotizing pancreatitis
Authors:   Dutch Acute Pancreatitis Study Group, Besselink M G , de Bruijn M T , Rutten J P , Boermeester M A , Hofker H S , Gooszen H G 
Published: March 2006

Abstract

Background
This study evaluated the various surgical strategies for treatment of (suspected) infected necrotizing pancreatitis (INP) and patient referrals for this condition in the Netherlands.

Methods
This retrospective study included all 106 consecutive patients who had surgical treatment for INP in the period 2000–2003 in one of eight Dutch university medical centres including three teaching hospitals. Surgical approaches included an open abdomen strategy, laparotomy with continuous postoperative lavage, minimally invasive procedures or laparotomy with primary abdominal closure. The National Hospital Registration System was searched to identify patients with acute pancreatitis who were admitted to the 90 Dutch hospitals that did not participate in the present study.

Results
The overall mortality rate was 34·0 per cent, 70 per cent (16 of 23) for the open abdomen strategy, 25 per cent (13 of 53) for continuous peritoneal lavage, 11 per cent (two of 18) for minimally invasive procedures and 42 per cent (five of 12) for primary abdominal closure (P &lt; 0·001). During the study interval, 44 (12·2 per cent) of 362 patients with acute pancreatitis who were likely to require surgical intervention had been referred to university medical centres.

Conclusion
Laparotomy with continuous postoperative lavage is the surgical strategy most often used in the Netherlands. The results of the open abdomen strategy are poor whereas a minimally invasive approach seems promising.
Performance characteristics of an analytical procedure for determining chloramphenicol residues in muscle tissue by gas chromatography–electron capture detection
Authors: Cerkvenik‐Flajs Vesna 
Published: March 2006

AbstractValidation of an analytical method for determining chloramphenicol residues in muscle tissue by gas chromatography–electron capture detection (GC‐ECD) was performed according to the latest European Union criteria for the analysis of veterinary drugs in food, laid down by Commission Decision 2002/657/EC. The method using the meta isomer of chloramphenicol as an internal standard proved to be very selective, specific to other related phenicols and accurate to within +3.6% at a concentration level of 8.9 µg/kg, as present in the certified reference material available. The correlation coefficient of the calibration curve was 0.9991. At all three fortification levels studied (0.3, 0.45 and 0.6 µg/kg), repeatability and intra‐laboratory reproducibility were <8 and ≤9%, respectively. The decision limit (CCα) and detection capability (CCβ) were 0.07 and 0.12 µg/kg, respectively. The validation results and the results of participation in an international inter‐laboratory proficiency test indicate that the method presented is completely suited for regulatory control to screen and quantify chloramphenicol residues in various muscle tissues on a routine basis. Copyright © 2006 John Wiley & Sons, Ltd.
An Atypical Approach Identifies TYR234 as the Key Base Catalyst in Chondroitin AC Lyase
Authors: Rye Carl S. , Matte Allan , Cygler Miroslaw , Withers Stephen G. 
Published: March 2006

AbstractChondroitin AC lyase from Flavobacterium heparinum catalyses the degradation of chondroitin by an anionic E1cb elimination mechanism that involves proton abstraction from C5 of glucuronic acid. The lyase also carries out efficient proton transfer to a sugar nitronate anion, which was designed originally as an inhibitor of the enzyme, with a second‐order rate constant of kcat/Km=2.7×106 M−1 s−; this is very similar to that of the natural chondroitin substrate (kcat/Km=1.3×106 M−1 s−1). Studies with this nitronate should therefore provide insight into the proton‐transfer step (general base catalysis) within this mechanism. Indeed, the Tyr234Phe mutant of the enzyme was essentially inactive with the natural substrate and correspondingly did not catalyse proton transfer to the nitronate, thereby implicating this residue as the general base catalyst. Parallel studies designed to identify the acid catalyst were carried out by using a substrate with a 2,4‐dinitrophenol leaving group that needs no acid assistance for departure. These results are consistent with Tyr234 also playing the role of acid catalyst. Not only do these studies confirm the suspected role of Tyr234, but also they validate a new methodology for identification of acid/base catalysts in lyases and epimerases of this type. In addition a structural and mechanistic rationale is provided for different active‐site acid/base configurations in syn and anti lyases.
Reactivity of 1‐Hydro‐5‐carbaphosphatrane Based on Tautomerization between Pentavalent Phosphorane and Trivalent Cyclic Phosphonite
Authors: Kobayashi Junji , Goto Kei , Kawashima Takayuki , Schmidt Michael W. , Nagase Shigeru 
Published: March 2006

AbstractThe reaction behavior of 1‐hydro‐5‐carbaphosphatrane (1 a) was examined. Treatment of 1 a with oxidants such as 3‐chloroperoxybenzoic acid (mCPBA) and tBuOCl gave cyclic phosphonate 2 and 1‐chloro‐5‐carbaphosphatrane (4), respectively, via cyclic phosphonite 3, a tautomer of 1 a. Compound 4 was readily hydrolyzed to afford 2. Compound 1 a was also sulfurized via 3 by elemental sulfur to afford cyclic thioxophosphonate 5, which was also obtained by reactions in the presence of bases. Treatment of 1 a with bases also proceeded through 3 to give an equilibrium mixture of the corresponding phenoxide anion 10 and the phosphoranide anion 9, which was quenched with MeI to afford a mixture of 11 and 1‐methyl‐5‐carbaphosphatrane (1 b). Such reactivities are typical for neutral phosphoranes. Theoretical investigations of these reactivities were also performed.
Tuning of Electronic Properties in Thienyl‐Phosphole π‐Conjugated Systems through P‐Functionalization Monitored by Raman Spectroscopy
Authors: Casado Juan , Réau Régis , López Navarrete Juan T. 
Published: March 2006

AbstractHerein, a Raman spectroscopic study of a new family of 2,5‐di(2‐thienyl)phospholes and thienyl‐capped 1,1′‐diphospholes is presented. The Raman spectra have been carefully assigned with the help of density functional calculations. For di(2‐thienyl)phospholes, two well‐differentiated groups of Raman bands exist that arise either from the central phosphole ring or from the outer thiophene substituents. These data reveal a segmentation of the electronic structure. This paper reports interesting relationships between geometrical data such as the BLA (bond‐length alternation) parameter and Raman band wavenumbers. These correlations are unprecedented in the chemistry of phospholes and have been used to interpret the evolution of the electronic structure (aromaticity⇄π‐conjugation) upon 1) substitution of the central sulfur atom of terthiophene by phosphorus and 2) P‐functionalization. Increasing the coordination number of the phosphole ring results in intramolecular charge transfer. The best scenario for phosphole aromaticity is found for 1,1′‐diphospholes.
Tetrabutylammonium Decatungstate‐Photosensitized Alkylation of Electrophilic Alkenes: Convenient Functionalization of Aliphatic CH Bonds
Authors: Dondi Daniele , Fagnoni Maurizio , Albini Angelo 
Published: March 2006

AbstractTetrabutylammonium decatungstate (TBADT, 2×10−3 m) is an effective photocatalyst for the alkylation of electrophilic alkenes (0.1 m, α,β‐unsaturated nitriles, esters, ketones) by alkanes, alcohols, and ethers. The products are in most cases obtained in >70 % isolated yields, through an experimentally very simple procedure. The kinetics of the radical processes following initial hydrogen abstraction by excited TBADT in deoxygenated MeCN have been studied. In the absence of a trap, back hydrogen transfer from reduced tungstate is the main pathway for alkyl radicals, while α‐hydroxyalkyl radicals are oxidized to ketones by ground‐state TBADT. With both radical types the reaction ceases at a few percent conversion. However, trapping by electrophilic alkenes is followed by reduction of the radical adduct and regeneration of the catalyst, which allows the alkylation to proceed up to complete alkene conversion with the mentioned good yields of products. With a nucleophilic (α‐hydroxyalkyl) radical, alkylation is efficient (Φ = 0.58) and can also be carried out when degassing is omitted, the only difference being a short induction period. With a less reactive (cyclohexyl) radical, the quantum yield is lower (Φ = 0.06) and the reaction is considerably slowed in aerated solutions, but the chemical yield remains good.
Synthesis, Characterization, Spectroscopic, and Electrochemiluminescence Properties of a Solvatochromic Azacrown‐Containing Cyanoruthenate(II): Potential Applications in Separation and Indirect Photometric Detection of Cations and Amino Acids in HPLC
Authors: Li Mei‐Jin , Chu Ben Wai‐Kin , Yam Vivian Wing‐Wah 
Published: March 2006

AbstractA new anionic ruthenium(II) complex, (Et4N)[Ru(tpyA18C6)(CN)3] (tpyA18C6=N‐[4′‐(2,2′:6′,2′′‐terpyridyl)]‐1,4,7,10,13‐pentaoxa‐16‐azacyclohexadodecane), has been synthesized and characterized. The complex was found to show pronounced solvatochromic behavior and, when dissolved in solution, changed its color from purple to yellow when the solvent system was varied from pure acetonitrile to pure water. Its absorption and emission energies in various solvents showed a linear dependence of the Gutman's acceptor number. The characteristic photoluminescence and electrochemiluminescence (ECL) of the complex were also found to be progressively quenched as the proportion of water in a water/acetonitrile mixture increased. Large changes in the chemical shifts of the 1H NMR and 13C NMR signals of [Ru(tpyA18C6)(CN)3]− in different solvents were observed. The complex has also been demonstrated to serve as a mobile‐phase additive in high‐performance liquid chromatography for separation of metal cations and amino acids. Comparison studies with the crown‐free analogue, (Et4N)[Ru(tpy)(CN)3] (tpy=2,2′:6′,2′′‐terpyridine), showed that other than the ion‐pair effect, the allosteric host–guest interaction provided by the presence of the pendant crown was essential to the separation performance of the complex. Indirect detection of nonabsorbing analytes has been achieved by monitoring the absorbance changes of the eluent at the metal‐to‐ligand charge‐transfer (MLCT) absorption band maximum of the complex at 445 nm. The effects of pH, ionic strength, and polarity of the mobile phase as well as the complex concentration on the selectivity and resolution have also been studied.
Triphenylantimony(v) Catecholates and o‐Amidophenolates: Reversible Binding of Molecular Oxygen
Authors: Cherkasov Vladimir K. , Abakumov Gleb A. , Grunova Ekaterina V. , Poddel'sky Andrey I. , Fukin Georgy K. , Baranov Evgenii V. , Kurskii Yury V. , Abakumova Ludmila G. 
Published: March 2006

AbstractNovel neutral antimony(V) complexes were isolated as crystalline materials and characterized by IR and NMR spectroscopy: o‐amidophenolate complexes [4,6‐di‐tert‐butyl‐N‐(2,6‐dimethylphenyl)‐o‐amidophenolato]triphenylantimony(V) (Ph3Sb[AP‐Me], 1) and [4,6‐di‐tert‐butyl‐N‐(2,6‐diisopropylphenyl)‐o‐amidophenolato]triphenylantimony(v) (Ph3Sb[AP‐iPr], 2); catecholate complexes (3,6‐di‐tert‐butyl‐4‐methoxycatecholato)triphenylantimony(V) (Ph3Sb[(MeO)Cat], 3), its methanol solvate 3⋅CH3OH (4); (3,6‐di‐tert‐butyl‐4,5‐di‐methoxycatecholato)triphenylantimony(V) (Ph3Sb[(MeO)2Cat], 5) and its acetonitrile solvate 5⋅CH3CN (6). Complexes 1–7 were synthesized by oxidative addition of the corresponding o‐iminobenzoquinones or o‐benzoquinones to Ph3Sb. In the case of the phenanthrene‐9,10‐diolate (PhenCat) ligand, two different complexes were isolated: Ph3Sb[PhenCat] (7) and [Ph4Sb]+[Ph2Sb(PhenCat)2]− (8). Complexes 7 and 8 were found to be in equilibrium in solution. Molecular structures of 2, 4, 6, and 8 were determined by X‐ray crystallography. Complexes 1–7 reversibly bind molecular oxygen to yield Ph3Sb[L‐Me]O2 (9), Ph3Sb[L‐iPr]O2 (10), Ph3Sb[(MeO)L′]O2 (11), Ph3Sb[(MeO)2L′]O2 (12) and Ph3Sb[PhenL′]O2 (13), which contain five‐membered trioxastibolane species (where L is the O,O′,N‐coordinated derivative of a 1‐hydroperoxy‐6‐(N‐aryl)‐iminocyclohexa‐2,4‐dienol, and L′ the O,O′,O′′‐coordinated derivative of 6‐hydroperoxy‐6‐hydroxycyclohexa‐2,4‐dienone). Complexes 9–13 were characterized by IR and 1H NMR spectroscopy and X‐ray crystallography.
Octahedral and Tetrahedral Coinage Metal Clusters: Is Three‐Dimensional d‐Orbital Aromaticity Viable?
Authors: Corminboeuf Clemence , Wannere Chaitanya S. , Roy Debjani , King R. Bruce , von Rague Schleyer Paul 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
New Tetragonal Structure Type for A2Ca10Sb9 (A: Li, Mg). Electronic Variability Around a Zintl Phase.
Authors: Ganguli Ashok K. , Gupta Shalabh , Corbett John D. 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Crystallographic and Magnetic Studies on Iron‐Rich Mixed Rare‐Earth Intermetallics (Nd/Tb)2(Fe/Al)17.
Authors: Xiao Y. G. , Rao G. H. , Zhang Q. , Liu G. Y. , Zhang Y. , Liang J. K. 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Crystal Structure and Some Magnetic Properties of Novel Compounds: U3Pt23Si11, U3Pt5Si and U6Pt30Si19.
Authors: Chotard J. N. , Tougait O. , Noel H. , Rogl P. , Zelinskiy A. , Bodak O. I. 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Silver Ion Dynamics in the Ag5Te2Cl‐Polymorphs Revealed by Solid State NMR Lineshape and Two‐ and Three‐Time Correlation Spectroscopies.
Authors: Brinkmann Christian , Faske Sandra , Vogel Michael , Nilges Tom , Heuer Andreas , Eckert Hellmut 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
The Heat of Formation of the Uranyl Dication: Theoretical Evaluation Based on Relativistic Density Functional Calculations.
Authors: Moskaleva Lyudmila V. , Matveev Alexei V. , Krueger Sven , Roesch Notker 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
The 673 and 1123 K Isothermal Sections (Partial) of the Phase Diagram of the Ce—Mg—Ni Ternary System.
Authors: Zhou Huaiying , Wang Yanchun , Yao Qingrong 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Phase Diagram of the Sn—Zn—Ti System.
Authors: Vassilev Gueorgui P. , Dobrev Evgeni S. , Tedenac Jean‐Claude 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Investigation of the Charge Compensation Mechanism on the Electrochemically Li‐Ion Deintercalated Li1‐xCo1/3Ni1/3Mn1/3O2 Electrode System by Combination of Soft and Hard X‐Ray Absorption Spectroscopy.
Authors: Yoon Won‐Sub , Balasubramanian Mahalingam , Chung Kyung Yoon , Yang Xiao‐Qing , McBreen James , Grey Clare P. , Fischer Daniel A. 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Mechanochemical Preparation and Investigation of Properties of Magnesium, Calcium and Lithium—Magnesium Alanates.
Authors: Mamatha M. , Bogdanovic B. , Felderhoff M. , Pommerin A. , Schmidt W. , Schueth F. , Weidenthaler C. 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Electronic Structure of Binary Phosphoric and Arsenic Triazides.
Authors: Zeng Xiaoqing , Wang Weigang , Liu Fengyi , Ge Maofa , Sun Zheng , Wang Dianxun 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Pr10 [Si10‐xAlxO9+xN17‐x]Cl with x ≈ 1 — An Oxonitridoaluminosilicate Chloride.
Authors: Lieb Alexandra , Lauterbach Rainer , Schnick Wolfgang 
Published: March 2006

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.