bluuebunny/crossref_metadata_2025 huggingface dataset:
An Integrated Feasibility Analysis of a Stand‐alone Wind Power System, including No‐energy Fulfillment Cost
Authors: Kaldellis J. K.
Published: December 2003

Autonomous wind power systems are among the most interesting and environmentally friendly technological solutions for the electrification of remote consumers. However, the expected system operational cost is quite high, especially if the no‐load rejection restriction is applied. This article describes an integrated feasibility analysis of a stand‐alone wind power system, considering, beyond the total long‐term operational cost of the system, the no‐energy fulfilment (or the alternative energy coverage) cost of the installation. Therefore the impact of desired system reliability on the stand‐alone system configuration is included. Accordingly, a detailed parametric investigation is carried out concerning the influence of the hourly no‐energy fulfilment cost on the system dimensions and operational cost. Thus, by using the proposed method, one has the capability–in all practical cases–to determine the optimum wind power system configuration that minimizes the long‐term total cost of the installation, considering also the influence of the local economy basic parameters. Copyright © 2003 John Wiley & Sons, Ltd.
A New Tetranuclear Zinc Complex with a μ4‐bridging Carbonate Cap
Authors: Bauer‐Siebenlist Bernhard , Meyer Franc , Vidovic Denis , Pritzkow Hans
Published: December 2003

A bis(tridentate) compartmental pyrazolate‐based ligand forms the discrete dizinc(II) complex[LZn2(NO3)3] (1) with a singly O‐atom bridging nitrate and two terminal chelating nitrates. In the absence of coordinating counteranions, the dizinc(II) scaffold takes up atmospheric carbon dioxide to give a tetranuclear species 3 assembled from two dinuclear components. Its X‐ray crystal structure reveals a μ4‐bridging carbonate cap, which is a rare structural motif in zinc(II) coordination chemistry.
Hydrazin‐Addukte mit Dialkylindiumchloriden — Überraschende strukturelle Vielfalt bei einfachen Verbindungen
Authors: Uhl Werner , Emden Christian H. , Geiseler Gertraud , Harms Klaus
Published: December 2003

Dialkylindiumchloride reagieren mit Hydrazinderivaten zu unterschiedlichen Addukten, die eine bemerkenswerte strukturelle Vielfalt zeigen. Die äquimolare Umsetzung von Me2InCl mit Phenylhydrazin ergibt das erwartete Monoaddukt [Me2InCl·NH2NHC6H5] (1), in dem der Ligand über sein NH2‐Stickstoffatom an das zentrale Indiumatom koordiniert und das im festen Zustand über eine In—Cl—In‐Brücke dimer vorliegt. Bei der entsprechende Reaktion mit tert‐Butylhydrazin entsteht dagegen unter asymmetrischer Spaltung des Indiumhalogenids die ionische Verbindung [Me2InCl2]‐[Me2In(NH2NHCMe3)2]+ (3), in deren kationischem Bestandteil zwei NH2‐Gruppen am Indiumatom koordinieren. Im Gegensatz dazu ergibt sterisch höher abgeschirmtes (Me3C)2InCl mit diesem Hydrazin ein Addukt 2, das zwar analog zu 1 ist, im Festkörper aber nicht dimerisiert. Ein Unterschuß von Phenylhydrazin führt zur Bildung der Verbindung (Me2InCl)4[NH2N(H)C6H5]2 (4), in der ein Ausschnitt aus der Leiterstruktur von Me2InCl mit vier Formeleinheiten des Halogenids durch zwei terminale Hydrazinliganden stabilisiert wird. Nur in wenigen Kristallen wurde eine entsprechende Verbindung 5 mit tert‐Butylhydrazin erhalten, die eine komplizierte dreidimensionale Struktur aufweist. Daneben wird über die Molekülstruktur von In(CMe3)3 (6) berichtet.
Synthese, Schwingungsspektren und Kristallstrukturen der Chloroberyllate (Ph4P)2[BeCl4] und (Ph4P)2[Be2Cl6]
Authors: Neumüller Bernhard , Weller Frank , Dehnicke Kurt
Published: December 2003

Berylliumdichlorid reagiert mit Tetraphenylphosphoniumchlorid in Dichlormethan je nach dem angewandten molaren Verhältnis unter Bildung der leicht löslichen Chloroberyllate (Ph4P)2[BeCl4]·2, 5CH2Cl2 (1·2, 5CH2Cl2) und (Ph4P)2[Be2Cl6] · 2CH2Cl2 (2·2CH2Cl2). Beide Verbindungen werden durch Kristallstrukturanalysen charakterisiert.

1·2, 5CH2Cl2: Raumgruppe P1¯, Z = 2, Gitterkonstanten bei 193 K: a = 1185, 7(1); b = 1244, 5(1); c = 1939, 3(2) pm; α = 77, 62(1)°; β = 74, 13(1)° γ = 68, 17(1)°, R1 = 0, 0416. Das [BeCl4]2‐‐Ion bildet Tetraeder ohne spezielle Lagesymmetrie.

2·2CH2Cl2: Raumgruppe P1¯, Z = 1, Gitterkonstanten bei 193 K: a = 985, 3(1); b = 1107, 6(1); c = 1344, 7(1) pm; α = 65, 45(1)°; β = 79, 12(1)°; γ = 78, 49(1)°, R1 = 0, 0361. Das [Be2Cl6]2‐‐Ion bildet chloroverbrückte Doppeltetraeder der Lagesymmetrie 1¯ (Ci).

Im Vakuum verlieren die Verbindungen das eingelagerte Dichlormethan. Von 1 und 2 werden die Schwingungsspektren (IR, Raman) mitgeteilt.
A Merrifield Resin Functionalized with Molybdenum Peroxo Complexes: Synthesis and Catalytic Properties
Authors: Hinner Marlon J. , Grosche Manja , Herdtweck Eberhardt , Thiel Werner R.
Published: December 2003

CH2Cl functionalized polystyrene (Merrifield resin) was reacted with deprotonated 2‐(3‐pyrazolyl)pyridine to give a polymer bound chelate ligand system, which could be co‐ordinated to oxodiperoxo molybdenum(VI) units. The resulting heterogenized catalyst was investigated for its activity in the epoxidation of cyclooctene.
Lithium‐Uran‐Nitride: LiUN2 und Li2UN2
Authors: Jacobs H. , Heckers U. , Zachwieja U. , Kockelmann W.
Published: December 2003

Die Synthese von LiUN2 erfolgte aus einem Gemenge von Li3N und UH3 (3:1) unter N2 bei 900 °C innerhalb von 3 d. Die Verbindung kristallisiert nach Röntgen‐ und Neutronenpulverdaten tetragonal in der Raumgruppe I41/amd mit Z = 4 und a = 4, 3965 (2)Å, c = 11, 059 (4)Å. Die Struktur leitet sich z. B. von der des Anatas (TiO2 ≙ UN2) durch Auffüllen der verbleibenden Oktaederlücken mit Li ab. Die Verbindung ist ein paramagnetischer Halbleiter.

Bei Umsetzungen von Li3N mit UH3 (3 : 1) bei 600 °C entsteht als Hauptprodukt Li2UN2, das bereits bekannt ist. Im Gegensatz zu früheren Untersuchungen kristallisiert dieses Produkt nicht in der Raumgruppe P3¯ mit Z = 3 sondern in P3¯ m1 mit Z = 1.

Bei Reaktionsansätzen von Li3N mit UH3 (5 : 1) bei 500 °C ergibt sich ein kubisch flächenzentriert kristallisierendes Produkt mit a = 4, 890 (1)Å dessen Gitterkonstante derjenigen von UN entspricht. Bei einem hohen Li3N : UH3‐Verhältnis von 5 : 1 bis 10 : 1 und Reaktionstemperaturen > 800 °C gibt das Produkt ein linienreiches Guinier‐Diagramm, das auf eine weitere Phase im System Li/U/N hinweist.
Metallkomplexe mit N2O2S2‐Koordinationssphäre. Synthese und Charakterisierung der Nickel(II)‐, Cobalt(II)‐ und Kupfer(II)‐Komplexe eines N, N′‐bis(2‐hydroxybenzyl)‐substituierten vierzehngliedrigen trans‐N2S2‐Makrocyclus
Authors: Mattes Rainer , Entian Thomas
Published: December 2003

Der funktionalisierte makrocyclische Ligand ‐N, N′‐bis(2‐hydroxybenzyl)‐3, 4:10, 11‐dibenzo‐1, 8‐diaza‐5, 12dithia‐cyclotetradecan, C32H34N2O2S2 (H2L1), seine Komplexe [NiL1]·CH3CN (1), [CoL1] (2a), [CoL1]·4CH3OH (2b), [CuHL1]Cl·5H2O (3) und das Addukt [H3L1](ClO4) (4) wurden dargestellt und durch Röntgenstrukturanalysen charakterisiert. In 1, 2a und 2b sind die Metallatome oktaedrisch von den sechs Donoratomen eines Liganden koordiniert. Der Ligand ist um die N···N′‐Verbindungsachse gefaltet. Daraus resultiert eine trans‐N2‐cis‐O2‐cis‐S2‐Konfiguration an den Zentralatomen. Die Cobalt(II)‐Komplexe 2a und 2b unterscheiden sich in Bezug auf die Konformation des Liganden. In 3 ist das Kupfer(II)‐Ion verzerrt quadratisch‐pyramidal koordiniert, mit einer 2‐Hydroxybenzylgruppe als apikalem Ligand und dem N2S2‐Donorsatz als basale Liganden. In 4 sind beide Stickstoffatome protoniert; die Phenolatsauerstoffatome sind über eine kurze intramolekulare Wasserstoffbrückenbindung von 2.585(4)Å Länge miteinander verbunden.
[K36(μ6‐Cl)2(H2O)11(MeOH)32][SnSe4][Co4(μ4‐Se)(SnSe4)4]3: Synthesis and Crystal Structure of a Novel Compound Containing Ternary [Co4(μ4‐Se)(SnSe4)4]10— Anions and a Novel [(μ6‐Cl)2K9]7+ Coordination Aggregate
Authors: Melullis Maike , Zimmermann Christian , Anson Christopher E. , Dehnen Stefanie
Published: December 2003

Formation of the novel compound [K36(μ6‐Cl)2(H2O)11(MeOH)32][SnSe4][Co4(μ4‐Se)(SnSe4)4]3 (2) was achieved by variation of the crystallization conditions that recently enabled synthesis of the quaternary compound [K22(H2O)17(MeOH)8][SnSe4]3[Co4(μ4‐Se)(SnSe4)4] (1) by reaction of K4[SnSe4] with [Co(en)3]Cl3 in a methanol/water mixture (19:1). The crystal structure of 2, which was determined by means of single crystal X‐ray diffraction (orthorhombic space group Pbcm; lattice dimensions at 200 K: a = 18.7122(8), b = 20.9653(9), c = 57.340(3)Å, V = 22494.9(17)Å3; R1 [I > 2σ(I)] = 0.0674; wR2 = 0.1271) differs from 1 in that the [Co4(μ4‐Se)(SnSe)4]10— anions are embedded in an even more complex counterion aggregation; the latter contains solvated K+ cations, additional [SnSe4]4— groups and, in contrast to 1, Cl— anions. As a part of the counterion assembly, the Cl— and some of the K+ ions are arranged in a previously unknown [(μ6‐Cl)2K9]7+ coordination aggregate, formed by two face‐sharing [(μ6‐Cl)K6] octahedra.
Trinuclear Mixed‐Valent MnII/MnIV/MnII Complexes — Structure and Magnetic Behavior
Authors: Alexiou Maria , Zaleski Curtis M. , Dendrinou‐Samara Catherine , Kampf Jeff , Kessissoglou Dimitris P. , Pecoraro Vincent L.
Published: December 2003

Examples of mixed‐valent manganese compounds with 2e— difference between the oxidation states of the metal ions are rare. Herein we report the synthesis, crystal structure, spectroscopic and magnetic studies of a series of MnII‐MnIV‐MnII trinuclear compounds of the general composition MnIIMnIVMnII(pko)4(CH3O)2(X)2[X= Cl— (1), NCO—(2), NCS— (3)]. These molecules can be prepared by adding the ketonoxime ligand in the presence of sodium hydroxide and the analogous pseudohalide salt to MnCl2·4H2O in methanol and with exposure to air. The crystal structures for the three compounds are quite similar with one six coordinate MnIV ion in an MnO6 coordination environment and two six coordinate MnII ions having an N5O or N4OCl coordination environment. Four nitrogen atoms come from two ketonoximato ligands, an oxygen atom from a methoxide bridge and a chloro or nitrogen atom from the halide or pseudohalide terminal ligand. Each pko— ligand acts as a tridentate chelating agent by using a pyridine, and an oximato‐nitrogen, bound to the MnII ion and the oximato oxygen, coordinated to the MnIV central ion. The other nitrogen atom of the pko— ligand remains uncoordinated. The magnetic parameters obtained from the fitting procedure are J12 = +7.8 cm—1 and g = 2.07 for 1, J12 = + 8.1 cm—1 and g = 2.08 for 2, and J12 = +6.1 cm—1 and g = 2.09 for 3 which indicate a ferromagnetic behavior. The ground states for all the compounds are S = 13/2.
An exact solution to the moving boundary problem with fractional anomalous diffusion in drug release devices
Authors: Liu J.Y. , Xu M.Y.
Published: December 2003

An exact solution to the moving boundary problem with fractional anomalous diffusion (in time) in drug release devices is presented by means of fractional Green's function and Wright function in this paper. The result given by this paper coincides with the known semi‐empirical Ritger‐Peppas' formula in controlled drug release system [4]. For convenience of use, an approximate expression of the amount of drug released from polymeric matrix with high accuracy is given out at the end of the paper.
Effect of Helicobacter pylori Infection in Barrett’s Esophagus and the Genesis of Esophageal Adenocarcinoma
Authors: Clark Geoffrey W.B.
Published: November 2003

The relation between Helicobacter pylori and gastroesophageal reflux disease is unclear. Recent reports have suggested a possible protective role for H. pylori, particularly in preventing the complications of gastroesophageal reflux disease (GERD). The purpose of this article is to present a brief overview of the recent literature regarding the role of H. pylori in the genesis of the complications of GERD, focusing on Barrett’s esophagus and esophageal adenocarcinoma. The prevalence of H. pylori infection in the population of the West is around 40% and is not different in cohorts of patients with GERD. When the infection induces pangastritis or corpus‐predominant gastritis, there may be concomitant reduced gastric acid secretion. Eradication of the bacteria in this subgroup of patients may enhance gastric acid secretion and provoke reflux symptoms. H. pylori organisms do not colonize the specialized intestinal metaplasia characteristic of Barrett’s esophagus. H. pylori infection rates in gastric mucosa of patients with Barrett’s esophagus occur at a similar or slightly lower frequency than is found in controls. Gastric infection with cagA‐positive strains of H. pylori appears to be uncommon in patients with Barrett’s esophagus. Furthermore, epidemiologic studies indicate that cagA‐positive strains are protective against esophageal adenocarcinoma. Several investigators have proposed that the decreasing prevalence of H. pylori infection might be an important factor in the rising incidence of this tumor.
Placental Pathology in Fetal Bartter Syndrome
Authors: Ernst Linda M. , Parkash Vinita
Published: December 2003

Bartter syndrome, which presents clinically with polyuria, urinary potassium loss, hypokalemia, hypercalciuria, and alkalosis, is an autosomal recessive disorder with mutations in genes encoding the Na-K-2Cl cotransporter, the chloride channel CLC-NKB, and the potassium channel ROMK. Prenatal diagnosis of Bartter syndrome is now possible; however, there are no reports of the placental pathology associated with fetal Bartter syndrome. We present the placental pathologic findings in two siblings with fetal Bartter syndrome. Both pregnancies were complicated by polyhydramnios and preterm delivery. The first pregnancy delivered at 30 weeks, and Bartter syndrome was diagnosed in the perinatal period. The subsequent pregnancy required periodic therapeutic amniocentesis secondary to massive polyhydramnios and delivered at 32 weeks gestation. The suspicion of fetal Bartter syndrome was very high in this second pregnancy, and the infant was confirmed to have Bartter syndrome subsequently. Both placentas were large for gestational age, weighing greater than the 95th percentile. Microscopic examination showed extensive subtrophoblastic basement membrane mineralization (special stains positive for iron and calcium) in the chorionic villi. This striking finding was present in both placentas. Subtrophoblastic mineralization has been described in the literature in placentas of fetuses with abnormalities including anencephaly, trisomy 21, and other congenital abnormalities; however, it has also been described in normal pregnancies. Mechanisms of calcification in the placenta are not well understood, but these striking cases suggest that defects in fetal renal excretion of ions can lead to dystrophic calcification within the placenta, particularly in a subtrophoblastic pattern.
Severe Perinatal Liver Disease Associated with Fetal Thrombotic Vasculopathy
Authors: Dahms Beverly Barrett , Boyd Theonia , Redline Raymond W.
Published: December 2003

Three neonates with fetal thrombotic vasculopathy in the placenta and severe neonatal liver disease are described. Symptoms included a bleeding disorder on the first day of life, followed by direct hyperbilirubinemia and elevated liver transaminases. All patients also had evidence of thrombosis outside the placenta, including cerebral infarct in two infants and thrombosis of the inferior vena cava in one infant. Liver disease was demonstrated to be thrombotic in one infant who died with Budd-Chiari syndrome. Two infants survived and had liver biopsy with cholestasis, bile duct proliferation, and portal fibrosis demonstrated at 4 weeks and 11 weeks of age, respectively. The etiology of thrombosis is unknown, though in one patient an excessively long and coiled umbilical cord may be implicated. The prenatal onset of thrombosis suggests an inherited or acquired thrombophilic state. In cases of enigmatic neonatal liver disease, an association with thrombosis should be considered and thrombi sought in placenta, umbilical cord, major blood vessels, and other organs. Evaluation for a hypercoagulable state is also suggested.
Short Rib Polydactyly Syndrome Type III: Histopathogenesis of the Skeletal Phenotype
Authors: Corsi Alessandro , Riminucci Mara , Roggini Mario , Bianco Paolo
Published: December 2003

A morphological study of the skeletal system in a case of short rib polydactyly syndrome type III (SRPS-III) documented a “bajonet” deformity of the ribs for misalignment and overlap of cartilaginous and bony ends. This deformity resulted from a ‘tandem’ change in endochondral bone formation that is, arrested orthotopic cartilage maturation and etherotopic perichondral cartilage differention and ossification. At the cartilaginous end, cartilage maturation and vascular invasion were absent. At the bony end, longitudinal bone growth occurred by a perichondral ectopic growth plate. ‘Miniature’ versions of this ‘tandem’ change were also demonstrated in the long bones of the limbs and included focally arrested orthotopic cartilage maturation at the growth plates, perichondral cartilage differentiation, and ossification within cartilage canals. Our morphological study indicates that a generalized loss of syncrony in cartilage removal and osteogenic differentiation occurs in all growth plates, albeit with varied expressivity, and represents, at tissue level, the mechanism by which the SRPS-III skeletal phenotype develops.
Aspirin Intoxication in a Child Associated with Myocardial Necrosis: Is this a Drug-related Lesion?
Authors: Peña-Alonso Y. ROCíO , Montoya-Cabrera Miguel A. , Bustos-Córdoba Edgar , MarroqUín-Yáñez Lourdes , Olivar-López VíCTOR
Published: December 2003

A 5-year-old girl with a mild upper airways infection was admitted to the hospital because of sudden vomiting and drowsiness that evolved to stupor; she was dehydrated, hypotensive, and tachypneic; laboratory tests revealed noncompensated lactic acidosis. She also had hypoglycemia followed by hyperglycemia, and progressive bradycardia leading to reversible cardiac arrest. Her clinical condition complicated by sinus bradycardia, ventricular tachycardia, third-degree atrioventricular blockage and lethal asystole. At the final stage of her illness, the serum salicylate concentration was 383.8 mcg/mL. Based on this single data, a retrospective toxicological analysis estimated a theoretical peak level of serum salicylate of approximately 1570 mcg/mL (therapeutic range, 20–250 mcg/mL) although the real amount of aspirin that this child ingested is difficult to calculate because aspirin is a drug that shows a so-called zero order kinetics. At autopsy, the most striking finding was multiple foci of coagulative necrosis involving the entire thickness of the myocardium with scant inflammatory infiltrate composed mainly of macrophages and polymorphonuclear leukocytes. The morphologic characteristics of the myocardial lesion in addition to salicylate blood levels suggests the possibility of an adverse drug reaction of the type acute toxic myocarditis.
Parvovirus Infects Cardiac Myocytes in Hydrops Fetalis
Authors: O'Malley Aiveen , Barry-Kinsella Carole , Hughes Caroline , Kelehan Peter , Devaney Deirdre , Mooney Eoghan , Gillan John
Published: December 2003

Parvovirus infection during pregnancy is an important cause of hydrops fetalis. It is attributed to anemia caused by viral-induced destruction of red blood cells. Infection of other organs has been reported including the heart, liver, and lungs. Few of these reports, however, convincingly demonstrate virions within the functional parenchyma of the tissue. This is of particular concern regarding myocardium in the context of hydrops fetalis which is, in part, due to cardiac failure. The problem in routine pathology practice is that most fetuses with the infection are macerated. This, in part, probably explains the paucity of published information on cardiac involvement. This study examined five cases of fatal hydrops fetalis with variable maceration with serologically proven parvovirus B19 infection. Transmission electron microscopy of cardiac tissue demonstrated intranuclear virions in both erythroid precursor cells and in cardiac myocytes in three of these cases. In each of these, immunogold electron microscopy provided confirmatory evidence of parvovirus infection. Virions were not identifiable where maceration had caused disintegration of nuclei in the myocytes. In addition, virions were absent in the three negative control cases where retroplacental hemorrhage was confirmed as the cause of death. This study suggests that parvovirus infection of cardiac myocytes may play a more important role in causing hydrops fetalis than previously realized. It also demonstrates that maceration should not discourage the use of electron microscopy.
Chronic Rejection of Small Bowel Grafts: Pediatric and Adult Study of Risk Factors and Morphologic Progression
Authors: Parizhskaya Maria , Redondo Clara , Demetris Anthony , Jaffe Ronald , Reyes Jorge , Ruppert Kris , Martin Lillian , Abu-Elmagd Kareem
Published: December 2003

One hundred and seventy-two patients underwent small bowel transplantation at Children's Hospital of Pittsburgh and University of Pittsburgh Medical Center between May 1990 and August 2001. Thirty-four patients had complete or partial resection of their primary graft and in 15, histologic features of chronic rejection were present in the resected small bowel. This is a descriptive and correlative study of the demographic, perioperative, and histologic features associated with progression to intestinal graft failure. Variable features associated with an increased risk of chronic rejection included acute rejection within the 1st month, increased number and higher grade of acute rejection episodes, isolated small bowel grafts rather than small bowel–liver grafts, older recipient age, non-Caucasian race, and Caucasian to non-Caucasian transplant. The mucosal biopsies showed predictive changes many months before the grafts were excised. The mucosal biopsy diagnosis of chronic vascular rejection can be difficult because the affected vessels, the distal branches of the mesenteric arteries, and the larger arteries of the subserosa and submucosa are not routinely sampled. The possibility of underlying arteriopathy, however, can be inferred in some instances from the presence of secondary mucosal changes in the small bowel biopsies though the “early” changes lack specificity. It is the progression of biopsy findings over time that is predictive of outcome. It is important to recognize the persistence of “late” mucosal changes of chronic rejection so that patients are not subjected to increased immune suppression when it is unlikely to be of significant benefit.
Biochemical Abnormality Associated with Smith-Lemli-Opitz Syndrome in an Infant with Features of Rutledge Multiple Congenital Anomaly Syndrome Confirms that the Latter is a Variant of the Former
Authors: Rakheja Dinesh , Wilson Golder N. , Rogers Beverly B.
Published: December 2003

We describe a female infant with morphologic features of Rutledge multiple-congenital-anomaly syndrome (RM-CAS) and biochemical features of Smith-Lemli-Opitz syndrome (SLOS). She had microcephaly with hypoplastic cerebral frontal lobes and cerebellum, agenesis of the splenium of corpus callosum, abnormal facies including hypertelorism with bilateral inner epicanthal folds, a broad nasal bridge with slightly anteverted nares and patent choanae, low set ears and complex conchal formation, high-arched palate and thick maxillary alveolar ridges, and micrognathia. Her chest was broad, genitalia were ambiguous, and uterus was bicornuate. Skeletal abnormalities included a hypoplastic appendicular skeleton, post-axial hexadactyly of the right hand and the left foot, syndactyly of bilateral 2nd–3rd toes and left 5th–6th toes, right talipes varus and left talipes valgus, and fused L5–S1 vertebrae. Congenital heart disease consisted of hypoplastic left heart, coronary sinus agenesis, ostium secundum and ostium primum defects, and a thickened septum primum. The lungs were hypolobated and the kidneys manifested oligopapillary hypoplasia. Total colonic Hirschsprung disease was noted microscopically. Analysis of liver tissue taken at postmortem examination revealed the ratio of 7-dehydrocholesterol and cholesterol to be 143 (expected, 0.28 ± 0.28). Although initially described as a distinct syndrome, RMCAS was merged with the severe form of SLOS, because of significantly overlapping features [Online Mendelian Inheritance in Man (OMIM) #268670]. The biochemical data showing an excess of 7-dehydrocholesterol and low cholesterol in the liver tissue of our case supports this viewpoint.
Multifocal Infantile Myofibromatosis and Generalized Fibromuscular Dysplasia in a Child: Evidence for a Common Pathologic Process?
Authors: Wright Corrina , Corbally Martin T. , Hayes Roisin , McDermott Michael B.
Published: December 2003

Infantile myofibromatosis (IM) is a condition characterized by the formation of spindle cell tumors of skin, soft tissue, and viscera. Although small vessel involvement by the process is a frequently identified and indeed diagnostically useful histological finding, involvement of large vessels is not widely reported. Fibromuscular dysplasia (FMD) is a noninflammatory arteriopathy characterized by intimal, medial, and/or adventitial fibroplasias leading to luminal compromise and aneurysm formation. Although venous disease has been reported, involvement of arterioles and viscera has not been identified. We report a patient in whom IM was diagnosed, on the basis of multiple soft tissue tumors present from birth, who subsequently developed generalized and ultimately fatal FMD. These two conditions exhibit overlapping pathologic features, including pronounced intimal fibroplasia. Their occurrence in a single individual may provide insights into the pathogenesis of both conditions, suggesting that they represent part of the same spectrum of vascular myofibroblastic proliferations.
Transcription‐coupled TA and GC strand asymmetries in the human genome
Authors: Touchon M , Nicolay S , Arneodo A , d'Aubenton-Carafa Y , Thermes C
Published: December 2003

Analysis of the whole set of human genes reveals that most of them present TA and GC skews, that these biases are correlated to each other and are specific to gene sequences, exhibiting sharp transitions between transcribed and non‐transcribed regions. The GC asymmetries cannot be explained solely by a model previously proposed for (G+T) skew based on transitions measured in a small set of human genes. We propose that the GC skew results from additional transcription‐coupled mutation process that would include transversions. During evolution, both processes acting on a large majority of genes in germline cells would have produced these transcription‐coupled strand asymmetries.
Identification of pollen‐expressed pectin methylesterase inhibitors in Arabidopsis
Authors: Wolf Sebastian , Grsic-Rausch Slobodanka , Rausch Thomas , Greiner Steffen
Published: December 2003

Pectin methylesterases (PMEs) play an essential role during plant development by affecting the mechanical properties of the plant cell wall. Previous work indicated that plant PMEs may be subject to post‐translational regulation. Here, we report the analysis of two proteinaceous inhibitors of PME in Arabidopsis thaliana (AtPMEI1 and 2). The functional analysis of recombinant AtPMEI1 and 2 proteins revealed that both proteins are able to inhibit PME activity from flowers and siliques. Quantitative RT‐PCR analysis indicated that expression of AtPMEI1 and 2 mRNAs is tightly regulated during plant development with highest mRNA levels in flowers. Promotor::GUS fusions demonstrated that expression is mostly restricted to pollen.
Widespread production of novel soluble protein isoforms by alternative splicing removal of transmembrane anchoring domains
Authors: Xing Yi , Xu Qiang , Lee Christopher
Published: December 2003

We have investigated the effects of alternative splicing on transcripts encoding membrane proteins in 1001 human genes. Out of a total of 464 alternatively spliced genes encoding single‐pass transmembrane (TM) proteins, in 188 we observed a splice form that specifically removed the TM domain, producing a soluble protein isoform. For example, in syndecan‐4, the new alternative splice form closely parallels the proteolytic ectodomain shedding previously shown in this protein, and recognized as an important regulatory mechanism of receptor function. While many of the soluble isoforms produced by alternative splicing have already been validated, most are novel, and in 57 genes showed a statistically significant association (P‐value<0.01) with a specific tissue.
Subunit a of the E. coli ATP synthase: reconstitution and high resolution NMR with protein purified in a mixed polarity solvent
Authors: Dmitriev Oleg Y. , Altendorf Karlheinz , Fillingame Robert H.
Published: December 2003

Subunit a of the Escherichia coli ATP synthase, a 30 kDa integral membrane protein, was purified to homogeneity by a novel procedure incorporating selective extraction into a monophasic mixture of chloroform, methanol and water, followed by Ni‐NTA chromatography in the mixed solvent. Pure subunit a was reconstituted with subunits b and c and phospholipids to form a functional proton‐translocating unit. Nuclear magnetic resonance (NMR) spectra of the pure subunit a in the mixed solvent show good chemical shift dispersion and demonstrate the potential of the solvent mixture for NMR studies of the large membrane proteins that are currently intractable in aqueous detergent solutions.
MIXED FARMING SYSTEMS IN TRANSITION: THE CASE OF FIVE VILLAGES ALONG A RAINFALL GRADIENT IN NORTH-WEST SYRIA
Authors: RISCHKOWSKY B. , THOMSON E. F. , SHNAYIEN R. , KING J. M.
Published: December 2003

The changes taking place in the mixed farming systems of northwest Syria were examined by re-visiting, in 1996 and 2000, five villages along a rainfall gradient. The villages had been surveyed first in 1977–79. In those villages with moderate rainfall, intensification of crop production, namely a trend towards cereal monoculture and the planting of tree crops, did not lead to specialization in cropping at the expense of sheep ownership. In contrast, households in the areas too dry for most rainfed crops except barley (Hordeum vulgare) were more likely to sell their sheep because they depended heavily on off-farm income. Increases in crop yields were found but these only benefited the families in villages in the higher rainfall zones. Conversely, with the exception of ewe fertility, there was little evidence of improvements in sheep productivity. The mixed farming systems in the five villages sampled are still passing through a period of transition, and the cropping component will undoubtedly continue to change. This is less likely to happen to the small ruminant component in the near future unless the sector is given higher priority in national policy. As a strategy to increase feed production and balance the crop rotations, the prospects for closer crop/livestock integration at the farm level are limited by the many difficulties associated with the introduction of leguminous pasture and forage crops.
Putting the state in its place: the critique of state-centrism and its limits
Authors: Lacher Hannes
Published: December 2003

The critique of state-centrism is a crucial aspect of the restructuring of International Relations theory, widely seen as a precondition for the conceptualisation of international transformation. In this article, I argue that the terms on which this critique is framed lead to claims which are both too sweeping in their implications for a transformation to a post-Westphalian system, and not radical enough with respect to the Westphalian period itself. The critique of state-centrism is premised on the assumption that modernity was a territorial order in which states contained ‘their’ societies. But modern social relations always included global dimensions. If the modern social sciences discounted these global aspects of modernity, the way forward for the social sciences, and IR in particular, cannot be in embracing the notion of a contemporary shift from the national to the global, but in a reconsideration of modernity itself. Just as the new globalism is inadequate as a basis for understanding our supposedly postmodern times, so nation-statism was always defective as a basis for understanding modernity. I argue that the notion of a national/global dialectic provides a better basis for understanding the current socio-spatial transformation.
Sense and sensibility in the study of state socialisation: a reply to Kai Alderson
Authors: Thies Cameron G
Published: December 2003

In a recent article in the Review of International Studies, Kai Alderson subjected the concept of state socialisation to considerable scrutiny. This kind of conceptual clarification is fundamental to both theory building and empirical work in the study of international relations. Alderson should be commended for his work on the concept, since there are only a handful of previous studies that explicitly explore state socialisation in any detail. However, his attempt to produce a ‘consensus definition’ of the concept to bring clarity to an emerging research programme has left me with more questions than answers. This essay is designed to raise questions about Alderson's conceptualisation of state socialisation based on a comparison with the literature on socialisation from other disciplines. The overarching goal is to stimulate healthy debate about a concept that should be central to our understanding of the social aspects of international politics.
Forum on Chomsky
Authors: Herring Eric , Robinson Piers
Published: December 2003

This special section breaks an important silence in British international relations journals toward Noam Chomsky's political writings. Behind this silence lies the bigger matter of the silence in these journals about the issues contained in Chomsky's work. These issues include the use and sponsorship by the United States of terrorism on a massive scale for many years; the failure of most Western academics to take any interest in those US actions; and the dereliction by Western academics of their duty to help those who are trying to stop the United States from acting in this way. Once we started to read Chomsky's work, we concluded that there was a great deal to be learned from it. However, when we began to draw on it, we came up against widespread hostility towards his work combined with both ignorance and misrepresentation of precisely what he writes. In order to explore this undue marginalisation, we solicited a number of articles, including one from Chomsky, which resulted in this collection.
Discerning the patterns of world order: Noam Chomsky and international theory after the Cold War
Authors: Laffey Mark
Published: December 2003

In this article I argue that Chomsky's political writings, widely ignored in the discipline, are a significant resource for thinking about contemporary world politics, how we should analyse it, and to what ends. This claim is defended through an analysis of recent efforts by IR scholars to interpret the post-Cold War order. When viewed through the analytic perspective articulated by Chomsky, disciplinary accounts of the post-Cold War world as liberal and peaceful are shown to be insufficiently attentive to the empirical record. Chomsky's political writings are also shown to be compatible with standard accounts of critical social science.
Is there room for international law in realpolitik?: accounting for the US ‘attitude’ towards international law
Authors: Scott Shirley V.
Published: December 2003

The United States has in recent years come under considerable criticism for its apparently cynical attitude towards international law. While the US administration refers often to the importance of the international rule of law it appears unwilling to itself be bound by that law. While the US took the lead in the post World War II years in establishing international law and institutions it has in recent years appeared unwilling to give those same institutions its full support. This article begins by examining a range of explanations for the seemingly undesirable US attitude towards international law. Dismissing each as inadequate on its own, the article demonstrates how what often appear to be contradictory aspects of the United States' relationship with international law can in fact be reconciled through the application to United States' behaviour of the basic tenets of classic modern realism as it pertains to the conduct of foreign policy. This is surprising since international law and realpolitik are more often contrasted. The United States' ‘attitude’ towards international law is better explained not as a post-Cold War anomaly but as having been an integral aspect of the rise of the United States to sole superpower status.
Ambiguities of global civil society
Authors: Amoore Louise , Langley Paul
Published: December 2003

The concept of an emergent global civil society (GCS), an identifiable public sphere of voluntary association distinct from the architecture of states and markets, has become voguish in some approaches to international relations and international political economy, and in the practices of global governance. This article seeks to reveal the limitations of the prevailing commonsense framing of GCS. Challenging the idea that we can isolate an unambiguous GCS sphere, we focus instead on the particular uses of GCS – on the practices that are shaped in its name. We make a number of interventions to emphasise the conceptual and political ambiguity of GCS. First, we shift the emphasis from GCS as a bounded ‘non-governmental’; space to GCS as precisely a means of making global politics governable in particular ways. Second, we question the assumption of GCS as ‘voluntary association’, asking what it means for GCS to embody or represent the interests of social groups. Finally, we raise questions of the image of empowerment through GCS, highlighting the power relations, tensions and contradictions at the heart of a transformative politics.
Safe harbours are hard to find: the trans-Atlantic data privacy dispute, territorial jurisdiction and global governance
Authors: Kobrin Stephen J.
Published: December 2003

The trans-Atlantic dispute over application of the European Union's Data Directive (1995) is discussed as a case study of an emerging geographic incongruity between the reach and domain of the territorially-defined Westphalian state and the deep and dense network of economic relations. The article reviews significant EU-US differences about the meaning of privacy and the means to protect it, the history of attempts to apply its provisions to information transferred to the US, and the less than satisfactory attempt at resolution – the Safe Harbor agreement. It then argues that attempting to apply the Directive to transactions on the Internet raises fundamental questions about the meaning of borders, territorial sovereignty and political space and explores the implications for territorial jurisdiction and global governance at some length.
Diversity of Peracarida (Crustacea, Malacostraca) caught in a suprabenthic sampler
Authors: LÖRZ ANNE-NINA , BRANDT ANGELIKA
Published: December 2003

The composition of suprabenthic Peracarida from a depth between 1 and 1.33 m above the seafloor was investigated. In order to study the abundance and diversity quantitatively, the samples were taken by means of the supranet of an epibenthic sledge off Kapp Norvegia and off the South Shetland Islands. The samples were taken during ANT XVII-3 with RV Polarstern in April and May 2000. These samples of EASIZ III received during the Antarctic autumn are compared with those of EASIZ II taken during the summer. While the autumn samples show far less abundance of all peracarid taxa, the diversity is about twice as high as during summer. In total 115 species of peracarid crustaceans were sampled at six stations yielding 1336 individuals standardized to 1000 m3 hauls. Amphipoda clearly dominated in abundance and diversity.
Lipid composition of the euphausiids Euphausia vallentini and Thysanoessa macrura during summer in the Southern Indian Ocean
Authors: MAYZAUD P. , BOUTOUTE M. , ALONZO F.
Published: December 2003

Two species of euphausiids (Thysanoessa macrura Sars and Euphausia vallentini Stebbing) from the Southern Indian Ocean were analysed for their lipid content, fatty acid and fatty alcohol composition, in relation to developmental stage (T.m) and sex (E.v). Lipid composition showed wax esters and triacylglycerols as main neutral lipids for T. macrura and E. vallentini respectively. Allometric relationships between lipid class and total lipids indicated that changes in total lipids were size dependent and mainly related to wax esters in T. macrura and polar lipids (both phosphatidylcholine or PC and phosphatidylethanolamine or PE) in E. vallentini. No difference in lipid composition could be shown for male and female E. vallentini, while sampling location, developmental stage and sex were significantly influential in T. macrura. In this latter species wax esters displayed relatively similar fatty acid and alcohol composition in both juvenile and female stages, whereas a striking difference could be seen between females and juveniles in terms of polar lipids with a very low PE content in females. Male and female E. vallentini showed little differences in fatty acid structure with the exception of PC in females, which were low in 22:6n-3. The trophic status of these two species was established using multivariate discriminant analysis, which indicated for E. vallentini a degree of omnivory similar to E. superba, while these T. macrura appeared more omnivorous than individuals collected in other areas of the Southern Ocean. Clustering of polar lipid composition suggested a link between differences in PC fatty acid and the post-spawn stage of the female of E. vallentini collected. The same probably applies for the changes in PE recorded for T. macrura females.
Limited Pliocene/Pleistocene glaciation in Deep Freeze Range, northern Victoria Land, Antarctica, derived from in situ cosmogenic nuclides
Authors: OBERHOLZER PETER , BARONI CARLO , SCHAEFER JOERG M. , OROMBELLI GIUSEPPE , OCHS SUSAN IVY , KUBIK PETER W. , BAUR HEINRICH , WIELER RAINER
Published: December 2003

The question of how stable the climate in Antarctica has been during the last few million years compared to the rest of the planet is still controversial. This study attempts to add new information to the discussion by reconstructing the timing and spatial extent of glacial advances in northern Victoria Land over tens of thousands to millions of years. In Terra Nova Bay region, surface exposure ages and erosion rates of glacially rounded bedrock and glacial erratics have been determined using the cosmogenic nuclides 3He, 10Be and 21Ne. Three morphological units have been analysed. They yield minimum ages of 11 to 34 ka, 309 ka, and 2.6 Ma, respectively. Erosion rates were as low as 20 cm Ma−1 since middle Pliocene time. Taking erosion into account, the oldest surface is 5.3 Ma old. Pleistocene glacier advances had considerable extent, reaching up to 780 m above modern ice levels, but have been restricted to the valleys since at least mid-Pliocene. The existence of landscapes of mid-Pliocene age in northern Victoria Land implies that the climatic stability of the McMurdo Dry Valleys is not unique within the Transantarctic Mountains, but rather the expression of a constantly cold and hyperarid climate regime in entire Victoria Land.
Supraglacial debris along the front of the Larsen-A Ice Shelf, Antarctic Peninsula
Authors: EVANS JEFFREY , COFAIGH COLM Ó
Published: December 2003

Semi-continuous, linear accumulations of poorly-sorted debris are present on the surface of the remnant Larsen-A Ice Shelf, Antarctic Peninsula. These accumulations form a complex of debris bands extending parallel to the front of the ice shelf for several kilometres. Landsat imagery shows that the debris bands originated as lateral moraines along the Nordenskjöld Coast. Almost 80% of clasts sampled from these debris accumulations have shape/roundness characteristics consistent with glacier transport in the zone of basal traction. Angular and very angular clasts account for 15% and 22% of clasts in the pebble- and cobble/boulder-sized fractions, respectively, and originated by rockfall from valley/nunatak sides, with subsequent passive glacier transportation. Lithological analysis indicates that the debris is derived locally from the Nordenskjöld Coast, Cape Fairweather region and interior of the Antarctic Peninsula. Episodic melt-out and resedimentation of this debris from the front of the ice shelf would deliver pulses of coarse-grained sediment to the sea floor. Therefore, coarse-grained debris can also be released along the calving margin of small polar ice shelves fringing mountainous terrain, and could potentially be confused with sediment deposited at the grounding line of Antarctic ice-shelves. Sedimentological criteria to differentiate between these environments are proposed in this paper.
The International Polar Year in 2007
Authors: WALTON DAVID
Published: December 2003

Anniversaries punctuate all our lives, providing the semicolons that cause us to pause and think again. Everyone has plenty of personal anniversaries but there are also cultural and institutional ones that we can use as an opportunity to remember but also as a platform to leap forward. How far have we come and where do we want to be in the future? The recent announcement of an International Polar Year (IPY) in 2007 is linked to the fiftieth anniversary of the International Geophysical Year, an event that proved to have such momentous consequences both for Antarctic science and for international politics.
The uptake of gaseous organic nitrogen by leaves: A significant global nitrogen transfer process
Authors: Sparks Jed P. , Roberts James M. , Monson Russell K.
Published: December 2003

We report measurements of the uptake of peroxyacetyl nitrate (PAN) by plant leaves at mixing ratios typical of the continental lower troposphere. PAN uptake rates between 20 and 55 pmol m−2 s−1 were measured in eight plant species at an atmospheric mixing ratio of 250 pptv. Uptake rates in all species were strongly controlled by the leaf stomatal aperture. Foliar PAN uptake has important implications for balancing global and regional N cycles. From our measurements, we estimate 3% of the global annual emission of NOx to the atmosphere could be removed by foliar uptake of PAN. Regionally, the deposition rate of PAN can be as much as 20% the rate of total N deposition.