Ehlers-Danlos syndrome type VII: phenotype and genotype

1994

A patient suffering from a severe form of Ehlers-Danlos syndrome is presented (EDS type VII). The presence of bilateral congenital hip dislocation, generalized joint hypermobility and a soft hyperelastic skin with abnormal scarring suggested a specific collagen type I defect. SDS-PAGE analysis of collagens secreted into the medium of fibroblast cultures showed a retarded migration of more than half of the alpha 2(I) chains. CNBr peptide mapping of the HPLC-purified altered chain localized the mutant locus to the N-terminal region of the protein. cDNA analysis of the corresponding gene COL1A2 revealed, in addition to the expected collagen sequence, a transcript missing the entire exon 6. Thi…

Intraocular pressure in samples of elderly Finnish and Swedish men and women.

1994

Abstract Intraocular pressure was measured in 231 systematically sampled 75-year-old men and women in Goteborg, Sweden and in 284 75-year-old and 201 80-year-old residents in Jyvaskyla, Finland as part of a comparative study on functional capacity and health, NORA 75 (Nordic Research on Ageing). After excluding the cases reporting glaucoma in their medical history or using drugs for glaucoma treatment there were no significant differences in the distributions of intraocular pressure between the men (mean intraocular pressure in the right eye 15.1 mmHg, left eye 15.0 mmHg) and women (mean intraocular pressure in the right eye 15.5 mmHg, left eye 15.8 mmHg) in Goteborg. In Jyvaskyla the 75-ye…

Superconducting properties of polycrystalline YBCO prepared by a pyrolytic process

1994

Polycrystalline YBCO was prepared by a pyrolytic process starting from citrate and tartrate precursors. The effect of the precursor on the superconducting properties was investigated by means of magnetic measurements using a SQUID magnetometer, a Vibrating-Sample Magnetometer and an a.c. susceptometer. The critical temperature is not affected by the type of precursor (Tc=85 K for both). On the other hand, the precursor plays an important role on the critical current density, which is found to be an order of magnitude higher in the sample obtained from citrates (Jc(77K, H=0)=2.3·104 A/cm2 and 103 A/cm2 for the from-citrate and from-tartrate sample, respectively).

Binding of Insecticidal Crystal Proteins of Bacillus thuringiensis to the Midgut Brush Border of the Cabbage Looper, Trichoplusia ni (Hübner) (Lepido…

1994

The susceptibility of Trichoplusia ni larvae to several Bacillus thuringiensis insecticidal crystal proteins (ICPs) was tested. Neonatal larvae proved to be susceptible to solubilized trypsin-treated CryIA(a), CryIA(b), and CryIA(c) (50% lethal concentrations [LC 50 s], 570, 480, and 320 ng/cm 2 , respectively) but showed little susceptibility to CryIB and CryID (LC 50 s, 5,640 and 2,530 ng/cm 2 , respectively). The toxicity of ICPs was correlated to binding to the epithelial brush border of the midgut, as revealed by immunocytochemical staining with monoclonal antibodies. In vitro binding experiments with iodinated ICPs and brush border membrane vesicles indicated that CryIA(b) and CryIA(…

Association between reduced lymphocyte beta-adrenergic receptors and left ventricular dysfunction in young obese subjects

1994

This study was designed to evaluate total (t) and surface (s) beta-adrenergic receptors (BAR) density and their relationships with left ventricular function in young obese subjects. BAR density, plasma insulin, catecholamines and left ventricular function were evaluated in 27 young obese subjects (BMI30.5 kg/m2 for males and27.3 kg/m2 for females) without other risk factors for cardiovascular diseases (smoking, hypertension, diabetes and lipid abnormalities) and in 20 lean controls (BMI25 kg/m2 for males and24.7 kg/m2 for females). Both groups were matched for gender, age and body height. BAR density was evaluated according to Böyum and De Blasi methods. Plasma catecholamines by high perfus…

Charm production in the CERN hyperon beam

1994

First results on charm production by the Σ- hyperon beam experiment WA89 at CERN are presented. Estimates are given for thexF and nuclearA dependence of the inclusive cross-section of D-mesons. Preliminary results on charm baryon production are shown.

Überleben im Weltraumvakuum — Leben aus dem All? —

1994

Die Frage nach dem Ursprung des Lebens auf der Erde kann bisher niemand beantworten. Wir wissen nur, das alle heute bekannten Lebensformen auf einen gemeinsamen Ursprung zuruckgefuhrt werden konnen und das man diesen Ursprung etwa 3,8 Milliarden Jahre zuruckverfolgen kann [1]. Palaontologische und palaobiochemische Befunde machen wahrscheinlich, das die vermutlich einzelligen Lebewesen dieser Zeit hinsichtlich ihres Stoffwechsels schon hoch entwickelt waren. Isotopenuntersuchungen an fossilen Kohlenstoffverbindungen dieser Epoche lassen zum Beispiel vermuten, das Kohlendioxid reduktiv assimiliert wurde, moglicherweise bereits durch Photosynthese. Da andererseits vor mehr als 4 Milliarden Ja…

Ondasetron versus granisetron in the prevention of chemotherapy‐induced nausea and vomiting. Results of a prospective randomized trial

1994

Background. A single‐institution, prospective, randomized open trial was performed to compare ondansetron and granisetron in the prevention of chemotherapyrelated nausea and vomiting. The effect of antemetic drugs was analyzed indipendently for patients treated with highly emetogenic chemotherapy (Study 1), and those treated with moderately emetogenic regimens (Study 2). Methods. In Study 1. 182 patients treated with chemotherapeutic regimens containing high dose cisplatin (more than 70 mg/m2) were randomized to receive 24 mg of ondasentron intravenously (i. v.) or 3 mg of granisetron i. v. for the control of acute emesis. Patients treated with fractionated chemotherapy and those followed‐u…

Precambrian basement around Wadi Halfa, Sudan: a new perspective on the evolution of the East Saharan Craton

1994

This paper provides new geochemical and isotopic data on the evolution of the western foreland to the Nubian shield of north-east Africa. There is abundant evidence for early to middle Proterozoic crust west of the River Nile, but this was severely affected by the Pan-African (~ 500-900 Ma) orogenic cycle. The results are reported of Rb-Sr whole rock and zircon evaporation geochronological studies and whole rock Sm-Nd and feldspar Pb isotopic analyses for four rock units around Wadi Halfa in northern- most Sudan. These results indicate the presence of heterogeneous pre-Pan-African crustal components, preserved in mylonitic gneisses and in conglomerates that unconformably overlie the gneisse…

Modeling of Point Defects in Corundum Crystals

1994

Several different approaches including Hartree-Fock ab initio cluster calculations, semiempirical INDO calculations, and atom-atom potentials were used for modeling of the spatial and electronic structure as well as migration mechanisms of both intrinsic defects (self-trapped and defect-trapped holes, O and Al vacancies) and impurities (transition-metal ions like Co, Fe, Mg, Mn, Ti). The atomic structure of all hole centers is found to be similar to V[sub K] centers in alkali halides (two-site model); their formation is energetically favorable. The energy required for 60[degree] hole reorientations inside the basic oxygen triangles is found to be similar to both the energy for hops between …

On the significance of molecular surfaces and thermodynamic interactions for the excess viscosities of liquid mixtures

1994

The viscosities η of homogeneous binary mixtures of liquids are usually approximated as In η = ϕ1 In η1 + ϕ2 In η2 where ϕi and ηi are the volume fractions and the viscosities, resp., of the i-th pure substance; the behavior of real systems is then discussed in terms of Δ In η, the deviations from the above reference behavior. Here a semi-empirical approach is presented according to which volume fractions are replaced by the surface fractions Ωi to create a more realistic reference state, and the thermodynamic interaction parameter g is taken into account. The new equation reads (expressing it for practical purposes still in the terms of ϕi) γ is a geometric factor, measuring the difference…

PREDICTION OF SEVERE RHEUMATOID ARTHRITIS USING EPSTEIN-BARR VIRUS

1994

One of the immunological abnormalities in patients with RA is increased synthesis of immunoglobulins (Ig) in cultures of Epstein-Barr virus (EBV-) stimulated lymphocytes. We set out to investigate whether this feature, seen early in the disease, associated with later severe RA. We studied prospectively 45 patients with recent onset RA and, 41 healthy individuals. From 0-6 months after admission, blood lymphocytes were cultured in the presence of EBV for 4 weeks and Ig in the supernatants were assayed. To assess the severity of disease, clinical, laboratory and radiological evaluations were performed every 6 months for 2 yr. The association of increased Ig production with the severity of RA …

Quantitation of HCV-replication using one-step competitive reverse transcription-polymerase chain reaction and a solid phase, colorimetric detection …

1994

A solid phase assay for the colorimetric detection of competitively amplified HCV-cDNA has been established and used to investigate clinical samples from patients with chronic hepatitis. The assay is based on the reduction in the amplification of an hepatitis C virus-related competitor molecule by wild-type hepatitis C virus during polymerase chain reaction. The internal standard contains a lac operator sequence, allowing the amount of amplified competitor to be determined using a lac I-repressor/beta-galactosidase fusion protein. The reduction in the amplification of competitor is dependent upon the concentration of HCV-RNA in the original sample. External hepatitis C virus wild-type stand…

Disposition of ciprofloxacin following intravenous administration in dogs

1994

The pharmacokinetics of ciprofloxacin (CIP) following intravenous administration in dogs have been investigated. The drug was administered at three doses (2.5, 5 and 10 mg/kg body weight) and was assayed in biological fluid samples (plasma and urine) by an HPLC method. The plasma concentration-time curves were best described by a two-compartment open pharmacokinetic model. The drug was widely distributed (Vd(area) almost 3 l/kg), being distributed in the dog more rapidly than in other species (t1/2(lambda 1) 3 min approximately). The elimination half-life (t1/2 lambda 2) was 129-180 min which is similar to values obtained in other species. The unchanged drug eliminated in urine was less tha…

SPECIES RICHNESS WITHIN FAMILIES OF FLOWERING PLANTS

1994

Variation in species and genus richness among families of flowering plants was examined with respect to four classification variables: geographical distribution, growth form, pollination mode, and dispersal mode. Previous studies have estimated rates of species proliferation from age and contemporary diversity. Here we found that the earliest appearances in the fossil record are correlated with contemporary familial species richness, abundance in the fossil record, and the independent variables considered in this analysis. Thus, we believe that the fossil record does not provide reasonable estimates of the ages of families and that the rate of species proliferation cannot be calculated from…

Protein synthesisin vivoin rats fed on lipid-rich liquid diets

1994

Changes in tissue composition and protein synthesis ratio were studied in the major tissues of the body in young rats fed on lipid-rich, isonitrogenous purified liquid diets, a convenient method for inducing voluntary overfeeding under controlled nutritional conditions. Overfed rats showed faster growth induced by the energy excess. Analysis of tissue composition (protein, DNA and RNA contents) revealed that growth was due mainly to tissue hyperplasia in which protein and DNA contents increased in parallel. Fractional protein synthesis ratio measuredin vivoby the flooding-dose method of phenylalanine showed a marked increase in all tissues. This change could be attributed to an increase in …

Further Flavonoids and Other Phenolics of Thymus webbianus Rouy

1994

Abstract Seven different flavonoids from the aerial parts of Thymus webbianus were isolated. They were identified by UV spectra, TLC and HPLC-DAD as luteolin, api­genin, eryodictiol, naringenin, luteolin-7-O-glucoside, apigenin-7-O-glucoside and apigenin-6,8-di-C-glucoside. Other phenolics characterized by HPLC-DAD analysis were: protocatechuic, chlorogenic, syringic, p-coumaric and 3,5-dicaffeoylquinic acids.

Effect of Buthionine Sulfoximine on the Sensitivity to Doxorubicin of Parent and MDR Tumor Cell Lines

1994

We have studied the interaction of glutathione-depleting concentrations of buthionine sulfoximine (BSO) with the anti-proliferative activity of doxorubicin (DXR) in three tumor lines, the mouse B16 melanoma. Friend erythroleukemia and the human K562 leukemia, both as DXR-sensitive and-resistant (with typical multidrug resistance) variants. BSO significantly enhanced the DXR effects in the wild-type Friend and K562 leukemias, and especially in the drug-resistant subline of Friend leukemia. BSO did not modify DXR accumulation and retention in the latter clone. Moreover, neither BSO nor verapamil used alone completely reversed the resistance to DXR of this cell line; their combination was more…

Exercise-induced necrotic muscle damage and enzyme release in the four days following prolonged submaximal running in rats.

1994

Male Wistar rats were made to run uphill on a treadmill 5.5° incline at 17 m min−1 for 4 h, and killed for muscle and serum sampling 2, 4, 12, 24, 48 or 96 h after the exertion. To estimate the degree of muscle damage,β-glucuronidase activity, total protein concentration, water content and morphology were examined in the red parts of quadriceps femoris (MQF) and soleus (MS) muscles, the distal white part of the rectus femoris muscle (MRF) and the superficial part of triceps brachii muscle (MTB). Simultaneous serum samples were assayed for creatine kinase (CK) activity and carbonic anhydrase III (CA III) concentration. Fibre swelling and interstitial oedema were detected in MS at 4 h and in …

ON TOPOLOGICAL SPACES WITH A UNIQUE QUASI-PROXIMITY

1994

Abstract Trying to solve the question of whether every T 1 topological space with a unique compatible quasi-proximity should be hereditarily compact, we show that it is true for product spaces as well as for locally hereditarily Lindelof spaces.

Optimal shakedown design of beam structures

1994

The optimal design of plane beam structures made of elastic perfectly plastic material is studied according to the shakedown criterion. The design problem is formulated by means of a statical approach on the grounds of the shakedown lower bound theorem, and by means of a kinematical approach on the grounds of the shakedown upper bound theorem. In both cases two different types of design problems are formulated: one searches for the minimum volume design whose shakedown limit load is assigned; the other searches for the design of the assigned volume whose shakedown limit load is maximum. The optimality conditions of the four problems above are found by the use of a variational approach; such…

Primary angiosarcoma of the alveolar mucosa in a haemodialysis patient: case report and discussion

1994

A case of a haemodialysis patient with a primitive angiosarcoma of the alveolar mucosa is reported. The vascular origin of the tumor was confirmed by the immunohistochemical data which showed strong positivity for Factor VIII-related antigen and for vimentin, whereas stains for desmin and cytokeratins were negative.

The Effect of Indobufen on the Activities of Selected Rat Liver Phase I and Phase II Drug Metabolizing Enzymes, Peroxisomal β-oxidation and Hepatic G…

1994

Abstract Oral administration of indobufen to male rats for three days at daily doses of 5, 10 and 20 mg kg−1 resulted in no changes in liver total glutathione, cytosolic glutathione S-transferases or microsomal epoxide hydrolase. Reduced glutathione appeared slightly diminished to about 84% of control at the highest dose level. Microsomal cytochrome P450-dependent ethoxyresorufin O-de-ethylase and pentoxyresorufin de-alkylase activities were decreased to 64% (not significantly) and 67% of control at the lowest dose level. 6α- and 7α-Hydroxytestosterone activities were decreased to 67 and 68% of control at the highest dose level. Cyanide-insensitive peroxisomal fatty acid β-oxidation was inc…

Conversion-electron M�ssbauer spectroscopy using 121Sb and a study of powdered ?-Sb2O3

1994

It is shown that 121Sb Conversion-Electron Mossbauer Spectroscopy (CEMS) is possible even at room temperature. The parameters and peculiarities of 121Sb CEMS are summarized. An application to powdered Sb(III) oxide shows that the material on the surface of the grains is not oxidized to Sb(V) but has changed its crystal symmetry, while the bulk of the grains still exhibits the initial cubic-crystal phase.

Morphological transformation and DNA adduct formation by dibenz[a,h]anthracene and its metabolites in C3H10T1/2CL8 cells.

1994

The major routes of metabolic activation of dibenz[a,h]-anthracene (DBA) have been studied in transformable C3H10T1/2CL8 (C3H10T1/2) mouse embryo fibroblasts in culture. The morphological transforming activities of three potential intermediates formed by metabolism of DBA by C3H10T1/2 cells, trans-3,4-dihydroxy-3,4-dihydro-DBA-(DBA-3,4-diol), trans-dihydroxy-3,4-dihydro-DBA-anti-1,2-oxide (DBA-3,4-diol-1,2-oxide) and DBA-5,6-oxide were determined. DBA-3,4-diol-1,2-oxide was a strong morphological transforming agent giving a mean of 73% dishes with Type II or III foci and 1.63 Type II and III foci per dish at 0.5 microgram/ml. DBA-3,4-diol produced a mean of 42% dishes with Type II or III fo…

The poverty line and subsistence minimum in Latvia

1994

This article gives a picture of the difficulties of defining poverty and setting up a poverty line in a post-communist country in the period of transition to democracy and a market economy. The ongoing processes are linked with the deterioration of living standards and wellbeing of the population. The government has attempted to protect the population socially, accepting a subsistence minimum and later a crisis subsistence minimum. It is difficult to argue that the activities of the government have been successful and helped much in improving the living standards of the population of Latvia.

The polarization dependence of x‐ray photoelectron yield of a Au photocathode

1994

We report a measurement of the total photoelectron yield of gold as a function of the incident x‐ray polarization. Polarized x rays with energies of 2.5, 2.7, and 4.0 keV were used to excite a gold photocathode at a glancing incidence angle between 5° and 50°. Contrary to a previous report, we found no measurable polarization dependence for all three x‐ray energies. We conclude that the modulation factor, i.e., the fractional change of the total yield when the polarization state is varied, is less than 4%.

Optimization of synthesis of a titaniumcatalyst supported on alumina for synthesis of ultra-high molecular-weightpolyethylene (UHMWPE)

1994

Ethylene polymerized under low pressure over a catalytic system (modified Аl2Оз/ТlСl4 + AlEt2Cl) yields UHMWPE. Alumina gel chlorinated and calcined (above 773 K) was used as a catalyst carrier. The effect of the main variables of carrier chlorination (concentration of HCl, chlorination time and temperature) and of the calcination temperature on activity of the catalytic system was investigated. A mathematical method of planning experiments and evaluating experimental results was used in order to improve both the analysis of the conditions of the preliminary modification of the support and their optimization. Relationships between the surface properties of the alumina carrier and the activi…

Relaxation of human isolated mesenteric arteries by vasopressin and desmopressin.

1994

1. The effects of vasopressin and deamino-8-D-arginine vasopressin (DDAVP, desmopressin) were studied in artery rings (0.8-1 mm in external diameter) obtained from portions of human omentum during the course of abdominal operations (27 patients). 2. In arterial rings under resting tension, vasopressin produced concentration-dependent, endothelium-independent contractions with an EC50 of 0.59 +/- 0.12 nM. The V1 antagonist d(CH2)5Tyr(Me)AVP (1 microM) and the mixed V1-V2 antagonist desGly-d(CH2)5D-Tyr(Et)ValAVP (0.01 microM) displaced the control curve to vasopressin to the right in a parallel manner without differences in the maximal responses. In the presence of indomethacin (1 microM) the…

Conformational investigation of α,β‐dehydropeptides Part VI. Molecular and crystal structure of benzyloxycarbonylglycyl‐(Z )‐dehydrophenylalanine

1994

The structure of a peptide containing C-terminal dehydrophenylalanine, Z-Gly-(Z)-delta Phe (C19H18N2O5, MW = 354) was determined from single-crystal X-ray diffraction data. Needle-shaped crystals were grown from a 1:1 mixture of methanol-acetone in the monoclinic space group P2(1) with a = 14.717(4), b = 4.941(2), c = 12.073(4) A, beta = 103.72(4) degrees; V = 852.86(8) A3, Z = 2 and Dc = 1.32 g cm-3. The structure was solved by direct methods using SHELXS-86 and refined to a final R-index of 0.032 for 1714 observed reflections. The peptide adopts a conformation folded at the glycine residue, and principal torsion angles are omega 0 = -167.6(2) degrees, phi 1 = -71.8(3) degrees, psi 1 = -31…

Ovarian suppression reduces clinical and endocrine expression of late-onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

1994

Objective To determine the effectiveness of GnRH-agonist (GnRH-a) treatment in women with late onset congenital adrenal hyperplasia. Design Prospective assessment of GnRH-a treatment in six women with documented late-on-set congenital adrenal hyperplasia who were not preselected. Comparisons were made to previous responses in the same patients receiving dexamethasone. Eight age- and weight-matched ovulatory women served as controls. Setting Academic medical center. Intervention Baseline blood determinations before and after IV ACTH, before and after 6months of GnRH-a treatment. Estrogen and progestin replacement was begun in all women after the 3rd month of treatment. Main Outcome Measures …

A new approach to the identification of cognitive conflicts in the repertory grid: A nomothetic study

1994

Abstract Two separate but related investigations are reported. In the first, a score was derived from a construct correlation matrix obtained by grid data processing. This score, the Correlation Test (CT) score, expresses the percentage of unbalanced triads in the construct correlation matrix and is considered to assess logical inconsistency. It is discussed as being qualitatively different from other scores derived from construct correlation matrices, such as cognitive complexity scores. It is suggested that the CT score refers to cognitive integration, whereas other scores usually refer to cognitive differentiation. The second investigation was an attempt to trace the clinical implication…

The First sulfur-containing twin-DCNQI-type acceptor

1994

Immunohistochemical Staining of Retrobulbar Adipose Tissue in Graves' Ophthalmopathy

1994

An increase of retrobulbar adipose tissue has been shown by imaging techniques in patients with Graves' ophthalmopathy (GO). Immunohistochemical staining was applied to investigate the involvement of different retrobulbar (especially adipose) tissue components in the autoimmune process of the disease. Cryostat sections from retrobulbar tissues of 15 GO patients and 11 controls were analyzed with a battery of monoclonal antibodies against CD2, CD4, CD8, CD11a, CD19/22, CD25, CD54, CD57, CD68, C3b, HLA-A, B, C, and HLA-DR. In contrast to controls, the retrobulbar adipose tissue showed an increase of HLA-DR expression, an activation of intercellular adhesion molecule I (ICAM-1, CD54), as well …

Pancreatic digestive hydrolase activities in growing rats fed alternately on raw and heated soya-bean flour.

1994

The time-course effects of two diets containing raw (RSF) or heated (HSF) soya-bean flour on the digestive enzyme levels in the pancreas and in pancreatic juice were investigated in growing rats fed, alternately, on RSF or HSF diets for two 4-week periods. These values were compared with those obtained in a control group fed on a casein diet. RSF and HSF diets lowered N balance (84.8 (SE 0.9), 82.6 (SE 0.8) and 79.9 (SE 0.8) % with control, HSF and RSF diets respectively, at the third week). However, they increased protease activities compared with the control diet (3-fold for trypsin (EC3.4.21.4) and chymotrypsin (EC3.4.21.1) activities in pancreas contents and outputs with the RSF diet; 2…

Discriminant analysis in diagnosing carcinoma of the pancreas and of the papilla of Vater

1994

The clinical and biochemical presentation of carcinoma of the pancreas (PC) and of the papilla of Vater (CPV) are very similar, and, consequently, detailed investigations are required to correctly distinguish between them. The aim of the present study was to select the clinical and biochemical variables that would most efficiently discriminate the precise site of tumor origin. The study group consisted of 72 patients with PC and 22 patients with CPV consecutively hospitalized in our department. The following clinical parameters were considered: age, asthenia, anorexia, vomiting, weight loss, pain, fever, pruritis, and constipation; the biochemical parameters considered were total, direct, a…