bluuebunny/crossref_metadata_2025_split huggingface dataset:
Relationship between Cortisol Increment and Basal Cortisol: Implications for the Low-Dose Short Adrenocorticotropic Hormone Stimulation Test
Authors: Doi Suhail AR , Lasheen Ibrahim , Al-Humood Khaldoon , Al-Shoumer Kamal AS 
Published: February 2006

Abstract
Background: We analyzed the low-dose (1 μg) rapid adrenocorticotropic hormone test (LDST) in 17 patients with a normal hypothalamic-pituitary-adrenal axis to determine reference intervals for the LDST on the basis of poststimulation cortisol increments.
Methods: We analyzed test results for 17 patients (14 females and 3 males; age range, 18–46 years) who had received a 2-mL aliquot of low-dose (1 μg) adrenocorticotropic hormone prepared from one 250-μg vial of Synacthen diluted in 500 mL of sterile normal saline solution. Sampling took place at 0, 20, 30, and 60 min post stimulation. The cortisol increment was plotted against basal cortisol.
Results: We observed a marked interdependence of the basal cortisol concentration with the increase in cortisol concentration. The relationship was inverse and linear with the best fit observed at 30 min post stimulation. The lower 95% prediction limit for basal cortisol at the zero increment was 400 nmol/L with a mean concentration of 600 nmol/L.
Conclusions: We propose that a peak cortisol concentration &lt;400 nmol/L is a sufficient single criterion for abnormal adrenal function as assessed by the LDST. Concentrations of 400–600 nmol/L are in the gray area, and those &gt;600 nmol/L confirm normal adrenal function. Repeat analyses with larger sample sizes are warranted to confirm these observations.
Platelets promote coagulation factor XII-mediated proteolytic cascade systems in plasma
Authors: Johne Julia , Blume Constanze , Benz Peter M. , Pozgajová Miroslava , Ullrich Melanie , Schuh Kai , Nieswandt Bernhard , Walter Ulrich , Renné Thomas 
Published: February 2006

AbstractBlood coagulation factor XII (FXII, Hageman factor) is a plasma serine protease which is autoactivated following contact with negatively charged surfaces in a reaction involving plasma kallikrein and high-molecular-weight kininogen (contact phase activation). Active FXII has the ability to initiate blood clotting via the intrinsic pathway of coagulation and inflammatory reactions via the kallikrein-kinin system. Here we have determined FXII-mediated bradykinin formation and clotting in plasma. Western blotting analysis with specific antibodies against various parts of the contact factors revealed that limited activation of FXII is sufficient to promote plasma kallikrein activation, resulting in the conversion of high-molecular-weight kininogen and bradykinin generation. The presence of platelets significantly promoted FXII-initiated bradykinin formation. Similarly,in vitroclotting assays revealed that platelets critically promoted FXII-driven thrombin and fibrin formation. In summary, our data suggest that FXII-initiated protease cascades may proceed on platelet surfaces, with implications for inflammation and clotting.
Concentration of 99mTc-pertechnetate and 188Re-perrhenate
Authors: Mansur M. S. , Mushtaq A. , Jehangir M. 
Published: February 2006

Summary
The radioactive concentrations of 99mTc/188Re from their generators are dependent upon the specific activity of 99Mo/188W, which dictates the bed size of the alumina/gel column and the volume of physiological saline needed as eluent. Because of the high content of inactive molybdenum/tungsten in neutron irradiated MoO3/WO3, large columns containing alumina/gel are needed to produce chromatographic 99Mo → 99mTc/188W → 188Re generators. This results in large elution volumes containing relatively high 99Mo/188W contents and low concentrations of 99mTc/188Re. The decrease in radioactive concentration, or specific volume, places a limitation on some of the clinical procedures. We report post elution concentration of 99mTc/188Re using in house prepared silver cation exchange and alumina column. The high bolus volumes (10 − 60 ml saline) can conveniently be concentrated in 1 ml of saline. This approach also works very effectively to yield high specific volume solutions of 99mTc-pertechnetate from a fission 99Mo loaded alumina generator towards the end of its normal working life.
Syntheses of two potential dopamine D4 receptor radioligands: 18F labelled chromeno[3,4-c]pyridin-5-ones
Authors: Li Gu-Cai , Yin Duan-Zhi , Wang Ming-Wei , Cheng Deng-Feng , Wang Yong-Xian 
Published: February 2006

Summary
The dopamine D4 receptor is hypothesized to relate with the pathophysiology and pharmacotherapy of schizophrenia while its level in brain regions is much lower and to date no suitable tracer is available for the study of D4 receptor in vivo . Therefore, selective imaging agents for the D4 subtype are badly needed. Based on the structure-activity analysis of chromeno[3,4-c]pyridin-5-ones as dopamine D4 receptor ligands, two fluorine-18 labelled chromeno[3,4-c] pyridin-5-one derivatives, 3-(4-[18F]fluorobenzyl)-8-hydroxy-1,2,3,4-tetrahydrochromeno[3,4-c]pyridin-5-one and 3-(4-[18F]fluorobenzyl)-8,9-dimethoxy-1,2,3,4-tetrahydrochromeno[3,4-c]pyridin-5-one were synthesized through a two-step one-pot method. Their radiochemical yields were around 19.7% (decay-corrected) and radiochemical purities were higher than 95% with specific activities of about 120 GBq/μmol.
Soil-to-fungi transfer of 90Sr, 239+240Pu, and 241Am
Authors: Baeza A. , Guillén J. , Mietelski Jerzy W. , Gaca Pawel 
Published: February 2006

Summary
Fungi have up to now generally been used as bioindicators of radiocaesium in terrestrial ecosystems. However there is little knowledge about the behaviour of other man-made radionuclides. In the present work, we analyzed the soil-to-fungi transfer of 90Sr, 239+240Pu, and 241Am by collecting several species of fungi and the corresponding surface soil (0−5 cm) of a selected semi-natural ecosystem. The transfer of these radionuclides was then quantified by means of the traditional transfer factor, TF, defined as the ratio between the content in the fruiting bodies and in the total fraction of soil. As a general rule, the 90Sr TF values were higher than those of 239+240Pu and 241Am. This definition of the transfer factor can underestimate the transfer of radionuclides of which a large percentage are bound to soil particles. We therefore also used a definition based on the amount of radionuclide available to be transferred instead of the total content of the soil — the available transfer factor, ATF — which would be closer to the actual process of transfer. Hebeloma cylindrosporum and Lycoperdon perlatum presented 239+240Pu and 241Am ATF values that were greater than or similar to those of 90Sr. These species may thus be used as bioindicators for 239+240Pu and 241Am in a forest ecosystem.
Thermodynamic study of metal silicate complexation in perchlorate media
Authors: Pathak P.N. , Choppin Gregory R. 
Published: February 2006

SummaryThe thermodynamic parameters of formation of theo-silicate complexes of UO22+, Cm3+and Eu3+have been measured in the temperature range 5−45 °C in an aqueous medium of 0.20 M NaClO4) ionic strength. Solvent extraction with bis(2-ethylhexyl) phosphoric acid (HDEHP) was used to measure the stability constant (log β1) values of the silicate complexes as a function of temperature to obtain the enthalpies and entropies of complexation. The complexation reactions are entropy driven and opposed by the enthalpy. These thermodynamic values are consistent with the formation of an inner sphere complex.
Effect of pH and fulvic acid on sorption and complexation of cobalt onto bare and FA bound MX-80 bentonite
Authors: Xu Di , Shao Dadong , Chen Changlun , Ren Anping , Wang Xiangke 
Published: February 2006

SummaryBentonite has attracted great interest in nuclear waste management because of its outstanding properties. Herein, the sorption and complexation of cobalt on bare bentonite and fulvic acid (FA) coated bentonite are studied, respectively, under ambient conditions. The experiments are carried out atT= 25 ± 2 °C in 0.01 M KNO3solutions. Effect of pH, bentonite concentration, fulvic acid and cobalt solution concentration on cobalt sorption to bentonite is also investigated. The results show that cobalt sorption is strongly pH dependent and weakly bentonite content dependent. The presence of fulvic acid enhances Co2+sorption at low pH values and reduces the sorption of Co2+at high pH values. Effect of addition sequences of Co2+and FA to bentonite suspension on cobalt sorption to FA-bentonite colloids is also studied and the results indicate that the sorption of cobalt on FA coated bentonite is dependent on the addition sequences. The mechanism is discussed by the relative strength between both complexes of Co(II) with sorbed and soluble FA and with bentonite.
Polyunsaturated but not Conjugated Linoleic Acid Supplementation of Leukemic U937 Cells Can Act as an Amplification Factor for Photofrin‐mediated Photodynamic Therapy
Authors: Voigt Astrid , Agatha Gerhard , Zintl Felix 
Published: February 2006

ABSTRACTPolyunsaturated fatty acids located in leukemia cell membranes are excellent targets for peroxidation. They can significantly enhance the effectiveness of Photofrin‐mediated photodynamic therapy (PDT)‐induced cell killing. In this study, the peroxidizability of conjugated fatty acid isomers (9c,11t‐linoleic acid and 9c,11c‐linoleic acid) and polyunsaturated fatty acids (PUFAs; linoleic acid, γ‐linolenic acid and arachidonic acid) with 2,2′‐azo‐bis(2‐amidinpropane)dihydrochloride, soybean lipoxygenase and photomediated peroxidation are compared with each other. Peroxidation was determined using different methods: by means of gas chromatography to estimate the fatty acid (FA) consumption, by photometry for the level of FA peroxides or phospholipid peroxides and by definition of the content of malondialdehyde for thiobarbituric acid reactive substances (TBARS). The results suggest that the generation of oxidation products from individual FAs indicate a different formation rate of oxidation products. Radical FA peroxides were produced most by polyunsaturated arachidonic acid, followed by linoleic acid and γ‐linolenic acid, whereas conjugated FA isomers did not generate peroxides. Accordingly, the levels of lipid peroxides and TBARS were substantially increased after incorporation and oxidation of polyunsaturated FAs into U937 cells and could significantly enhance the effectiveness of Photofrin‐PDT‐induced cytotoxicity. The results showed that PUFA, but not conjugated FA supplementation of U937 cells, can act as a PDT amplification factor.
Shape evolution of 3‐dimensional faceted crystals
Authors: Zhang Yongchun , Sizemore Jacob P. , Doherty Michael F. 
Published: February 2006

AbstractA novel method for modeling the shape evolution of 3‐dimensional (3‐D) faceted crystals has been developed in which the normal distances to each face from an origin inside the crystal are represented by a system of ordinary differential equations. The model is initialized from an arbitrary initial seed shape and size but known crystallographic data (such as unit cell and symmetry). At each time step, the entire family of possible discrete shape evolution events (such as vertices bifurcating into edges or faces, etc.) are exhaustively enumerated and investigated using a new set of simple testable conditions. The evolving crystal shape is then determined from the evolving set of normal distances and the corresponding crystallographic planes. The model has been successfully applied to two organic crystal systems: adipic acid grown from water and α‐glycine grown from water. © 2006 American Institute of Chemical Engineers AIChE J, 2006
Design and scale‐up of chemical reactors for nanoparticle precipitation
Authors: Marchisio Daniele L. , Rivautella Liliana , Barresi Antonello A. 
Published: February 2006

AbstractIn recent years there has been a growing interest in production on an industrial scale of particles with size in the sub‐micron range (40‐200 nm). This can be done by controlling particle formation in order to nucleate very small particles and by tailoring the particle surface in order to avoid particle aggregation and produce stable suspensions. In this work we focus on the role of turbulent mixing on particle formation in confined impinging jet reactors. In particular, we show how computational fluid dynamics and simple precipitation models could be used to derive scale‐up criteria for the production of nanoparticles. © 2006 American Institute of Chemical Engineers AIChE J, 2006
Angelman syndrome 2005: Updated consensus for diagnostic criteria
Authors: Williams Charles A. , Beaudet Arthur L. , Clayton‐Smith Jill , Knoll Joan H. , Kyllerman Martin , Laan Laura A. , Magenis R. Ellen , Moncla Ann , Schinzel Albert A. , Summers Jane A. , Wagstaff Joseph 
Published: February 2006

AbstractIn 1995, a consensus statement was published for the purpose of summarizing the salient clinical features of Angelman syndrome (AS) to assist the clinician in making a timely and accurate diagnosis. Considering the scientific advances made in the last 10 years, it is necessary now to review the validity of the original consensus criteria. As in the original consensus project, the methodology used for this review was to convene a group of scientists and clinicians, with experience in AS, to develop a concise consensus statement, supported by scientific publications where appropriate. It is hoped that this revised consensus document will facilitate further clinical study of individuals with proven AS, and assist in the evaluation of those who appear to have clinical features of AS but have normal laboratory diagnostic testing. © 2006 Wiley‐Liss, Inc.
Dyggve–Melchior–Clausen syndrome and Smith–McCort dysplasia: Clinical and molecular findings in three families supporting genetic heterogeneity in Smith–McCort dysplasia
Authors: Neumann Luitgard M. , Ghouzzi Vincent El , Paupe Vincent , Weber Hans‐Peter , Fastnacht Elisabeth , Leenen Andreas , Lyding Sigrid , Klusmann Anne , Mayatepek Ertan , Pelz Jörg , Cormier‐Daire Valerie 
Published: February 2006

AbstractDyggve–Melchior–Clausen syndrome (DMC) (MIM 223800) and Smith–McCort dysplasia (SMC) (MIM 607326) are rare allelic autosomal recessive spondylo‐epi‐metaphyseal dysplasias (SEMDs) characterized by similar skeletal manifestations. Both phenotypes have been mapped to chromosome 18q21.1 and mutations in the DYM (dymeclin) gene were identified in 13 families with DMC and in two families with SMC. Most mutations identified in DMC predict a loss of function, while those identified in SMC are mainly missense mutations, presumably associated with residual DYM activity and a less severe phenotype. We studied three consanguineous families from Turkey, Lebanon, and Georgia, one with SMC and two with DMC and identified different homozygous DYM mutations (IVS3 194‐1G > A, 938_942delTGTCT) in the DMC families. No mutation was identified in the SMC family, possibly suggesting genetic heterogeneity of this disorder. © 2006 Wiley‐Liss, Inc.
Severe, fetal‐onset form of olivopontocerebellar hypoplasia in three sibs: PCH type 5?
Authors: Patel Millan S. , Becker Laurence E. , Toi Ants , Armstrong Dawna L. , Chitayat David 
Published: February 2006

AbstractWe present three siblings with a precise onset of fetal seizure‐like activity who had severe olivopontocerebellar hypoplasia (OPCH) and degeneration. Autopsies at 20, 27, and 37 weeks gestation showed diffuse central nervous system volume loss that was most marked for the cerebellum and brain stem structures. Neuropathological abnormalities included dysplastic, C‐shaped inferior olivary nuclei, absent or immature dentate nuclei, and cell paucity more marked for the cerebellar vermis than the hemispheres. Delayed development was seen in layer 2 of the cerebral cortex and in Purkinje cells of the cerebellum. Prenatal monitoring defined a developmental window of 16–18 weeks gestation when ultrasonic assessment of cerebellar width was used for prenatal diagnosis. We discuss our findings in the context of the differential diagnosis for infantile (O)PCH and propose a classification scheme for the pontocerebellar hypoplasias. These patients represent the earliest reported with OPCH and provide unique information regarding the developmental neuropathology of this condition. © 2006 Wiley‐Liss, Inc.
Minimal phenotype in a girl with trisomy 15q due to t(X;15)(q22.3;q11.2) translocation
Authors: Stankiewicz Paweł , Kuechler Alma , Eller C. Daniel , Sahoo Trilochan , Baldermann Christiane , Lieser Ulla , Hesse Martin , Gläser Christiane , Hagemann Monika , Yatsenko Svetlana A. , Liehr Thomas , Horsthemke Bernhard , Claussen Uwe , Marahrens York , Lupski James R. , Hansmann Ingo 
Published: February 2006

AbstractFew cases of de novo unbalanced X;autosome translocations associated with a normal or mild dysmorphic phenotype have been described. We report a 3‐year‐old dizygotic female twin with prenatally ascertained increased nuchal translucency. Prenatal chromosome studies revealed nearly complete trisomy 15 due to a de novo unbalanced translocation t(X;15)(q22;q11.2) confirmed postnatally. A mild phenotype was observed with normal birth measurements, minor facial dysmorphic features (hypertelorism, short broad nose, and a relatively long philtrum), and moderate developmental delay at the age of 3 years in comparison to her male fraternal twin. Replication timing utilizing BrdU and acridine‐orange staining showed that the der(X) chromosome was late‐replicating with variable spreading of inactivation into the translocated 15q segment. The der(X) was determined to be of paternal origin by analyses of polymorphic markers and CGG‐repeat at FMR1. Methylation analysis at the SNRPN locus and analysis of microsatellites on 15q revealed paternal isodisomy with double dosage for all markers and the unmethylated SNRPN gene. The Xq breakpoint was mapped within two overlapping BAC clones RP11‐575K24 and RP13‐483F6 at Xq22.3 and the 15q breakpoint to 15q11.2, within overlapping clones RP11‐509A17 and RP11‐382A4 that are all significantly enriched for LINE‐1 elements (36.6%, 43.0%, 26.6%, 22.0%, respectively). We speculate that the attenuated phenotype may be due to inactivation spreading into 15q, potentially facilitated by the enrichment of LINE‐1 elements at the breakpoints. In silico analysis of breakpoint regions revealed the presence of highly identical low‐copy repeats (LCRs) at both breakpoints, potentially involved in generating the translocation. © 2006 Wiley‐Liss, Inc.
Inverse association between severe nausea and vomiting in pregnancy and some congenital abnormalities
Authors: Czeizel Andrew E. , Puhó Erzsébet , Ács Nándor , Bánhidy Ferenc 
Published: February 2006

AbstractThe objective of the study was to investigate the possible association between nausea and vomiting in early pregnancy and congenital abnormalities. The prevalence of medically‐recorded severe nausea and vomiting in early pregnancy in cases with congenital abnormalities and their available matched population controls without any defect was compared in the population‐based large data set of the Hungarian Case‐Control Surveillance System of congenital abnormalities, 1980–1996. Of 22,843 cases with as 25 different congenital abnormality groups, 1,713 (7.5%) cases had mothers with medically recorded and treated severe nausea and vomiting during pregnancy. Of 38,151 matched population controls, 3,777 (9.9%) had mothers with severe nausea and vomiting (adjusted prevalence odds ratio (POR) with 95% CI: 0.74, 0.68–0.79). Five congenital abnormality groups: cleft lip with or without cleft palate (0.50, 0.37–0.70), posterior cleft palate (0.53, 0.32–0.89), renal a/dysgenesis (0.23, 0.06–0.96), obstructive defects of urinary tract (0.32, 0.18–0.58), and cardiovascular malformations (0.68, 0.57–0.81) had mothers with a lower prevalence of severe nausea and vomiting in pregnancy (adjusted PORs with 95% CI included in parentheses). Of 25 congenital abnormality groups, 22 had POR lower than 1. Thus in this study the mothers of cases with congenital abnormalities were 26% less likely to have had severe nausea and vomiting in early pregnancy than the mothers of population controls without congenital abnormalities. © 2006 Wiley‐Liss, Inc.
Genotype–phenotype correlation in hereditary hemorrhagic telangiectasia: Mutations and manifestations*
Authors: Bayrak‐Toydemir Pınar , McDonald Jamie , Markewitz Boaz , Lewin Susan , Miller Franklin , Chou Lan‐Szu , Gedge Friederike , Tang Wei , Coon Hillary , Mao Rong 
Published: February 2006

AbstractHereditary hemorrhagic telangiectasia (HHT) is a genetically heterogeneous vascular dysplasia with multiple telangiectases and arteriovenous malformations and it is caused by mutations in endoglin gene (ENG) (HHT1) and activin A receptor type II‐like 1 gene (ACVRL1) (HHT2). We evaluated 111 patients with HHT from 34 families by history, examination, screening for vascular malformations, and sequencing of both genes. We found mutations in 26 of the 34 kindreds (76%) analyzed—54% were in ENG and 46% were in ACVRL1. Mutations in ACVRL1 cluster largely in exons 7 and 8, but ENG mutations were widely distributed within that gene. We found that epistaxis had an earlier onset in patients with HHT1 than those with HHT2, but the severity by middle ages was similar. Pulmonary arteriovenous malformations were more frequent and on the average of larger size in HHT1. Hepatic vascular malformations were more common in patients with HHT2. Cerebral arteriovenous malformations were more common in patients with HHT1, but spinal arteriovenous malformations were seen only in patients with HHT2. Truncating mutations in ENG were associated with more affected organs and more severe hemorrhaging than were missense mutations. We conclude that HHT2 has a later onset than HHT1 and the former may disproportionately involve smaller vessels in tissues with more significant vascular remodeling. © 2006 Wiley‐Liss, Inc.
Klinefelter syndrome and mediastinal germ cell tumors
Authors: Völkl Thomas M.K. , Langer Thorsten , Aigner Thomas , Greess Holger , Beck Jörn D. , Rauch Anita M. , Dörr Helmuth G. 
Published: February 2006

AbstractPrecocious puberty is not a typical manifestation of patients with Klinefelter syndrome (KS). However, there is an increased incidence of mediastinal germ cell tumors (M‐GCT) in KS, whereas the discussion of a generally higher tumor risk in this condition is still controversial. A rare subgroup of KS patients consists of prepubertal children with precocious puberty due to human chorionic gonadotropin (hCG)‐producing M‐GCTs. We present clinical data on a boy with KS and sexual precocity, and summarize the published data on 12 boys with KS out of 54 cases of KS and M‐GCT. Clinical report: an 8.5‐year‐old boy presented with signs of precocious puberty. Laboratory analyses (suppressed gonadotropins, elevated testosterone) and thoracic CT demonstrated a β‐human chorionic gonadotropin (β‐hCG) and α1‐feto protein (α‐FP) secreting mediastinal tumor. Histological analysis showed a mixed germ cell tumor comprising choriocarcinoma (CH), embryonal carcinoma (EC), mature teratoma (MT), and yolk sac tumor (YS). He was successfully treated by surgery and adjuvant chemotherapy. Epianalysis of published cases: all KS patients (n = 12), age 4–9 years, presented with precocious sexual development (PP), whereas the older ones showed thorax‐associated symptoms, mainly chest pain, dyspnea, and cough. The histological distribution was also age‐dependent with mixed germ cell tumors predominantly in younger patients. Thus, M‐GCTs are strongly associated with precocious puberty in young boys with KS. Therefore, a karyotype analysis should be included in the clinical work‐up of boys with precocious puberty and M‐GCT. There is still no convincing explanation for the association of M‐GCTs and KS. © 2006 Wiley‐Liss, Inc.