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Autopsy study of 17 newborn infants with lethal autosomal recessive disease presenting as growth retardation with lactic acidosis, Fanconi aminoaciduria, and hepatic hemosiderosis is reported. The patients succumbed between day 1 and 4 months of life; 9 patients died within the first month. All patients showed severe pathologic changes of liver with cholestasis in all livers. Extensive accumulatio

Objective. To evaluate CAT-192, a recombinant human antibody that neutralizes transforming growth factor beta 1 (TGF beta 1), in the treatment of early-stage diffuse cutaneous systemic sclerosis (dcSSc). Methods. Patients with SSc duration of < 18 months were randomly assigned to the placebo group or to 1 of 3 CAT-192 treatment groups: 10 mg/kg, 5 mglkg, 0.5 mg/kg. Infusions were given on day 0 an

Background: Cytokine response is an important factor in the development of shock and organ failure. The aim of this study was to investigate intraperitoneal (peritoneal) and venous (systemic) postoperative cytokine release after major surgery. Methods: Major abdominal surgery was performed in 19 patients. Preoperative systemic measurements and postoperative systemic and peritoneal measurements of

The use of molecular mechanics calculations to supplement experimental data in standard X-ray crystallography and NMR refinements is discussed and it is shown that structures can be locally improved by the use of quantum chemical calculations. Such calculations can also be used to interpret the structures, e. g. to decide the protonation state of metal-bound ligands. They have shown that metal sit

In a 5-year follow-up study of 88 animal exposed laboratory technicians, the incidence of laboratory animal allergy (LAA), lung function, and the development of allergy test reactivity were investigated. Only two individuals developed test positive LAA rhinitis during the follow-up period. Furthermore, one subject who had previously had LAA rhinitis developed LAA asthma. In the remaining subjects

Three separate proteins, BchD, BchH, and BchI, together with ATP, insert magnesium into protoporphyrin IX. An analysis of ATP utilization by the subunits revealed the following: BchH catalyzed ATP hydrolysis at the rate of 0.9 nmol per min per mg of protein. BchI and BchD, tested individually, had no ATPase activity but, when combined, hydrolyzed ATP at the rate of 117.9 nmol/min per mg of protein

Europe has positioned itself as a front runner for climate mitigation policy globally. However, to reach climate mitigation targets that go far beyond Kyoto commitments, climate policy must become more integrated with sectoral policies such as energy, transport and agriculture. To achieve such policy integration, policy refraining and dilemma sharing among sectoral actors are important mechanisms.

When assessing the health status of patients after orthopaedic surgery, such as knee arthroplasty, general health and disease-specific questionnaires are gaining in popularity because of their precision in detecting subtle differences. Self-administered postal surveys using extensive questionnaires have associated patient burden, however, which may have an impact on response rate and completeness.

In this paper we consider a general problem of astrophysical lasersoperating with optical pumping by accidental resonance (PAR) with strongspontaneous emission lines of other elements. The origin of populationinversion and the coefficient of amplification due to the opticalexcitation of atoms (ions) by a four-level scheme with PAR are analyzed.We elucidate conclusively the question of the manifest

Objective To evaluate a calibration method for disk diffusion antibiotic susceptibility tests, using zone diameter values generated in the individual laboratory as the internal calibrator for combinations of antibiotic and bacterial species. Methods The high-zone side of zone histogram distributions was first analyzed by moving averages to determine the peak position of the susceptible population

We present a software solution that enables faster and more accurate data analysis of 2DE/MALDI TOF MS data. The software supports data analysis through a number of automated data selection functions and advanced graphical tools. Once protein identities are determined using MALDI TOF MS, automated data retrieval from online databases provides biological information. The software, called 2DDB, redu

It has been proposed that hyaluronan-binding proteoglycans play an important role as guiding cues during neural crest (NC) cell migration, but their precise function has not been elucidated. In this study, we examine the distribution, structure and putative role of the two major hyaluronan-binding proteoglycans, PG-M/versicans and aggrecan, during the course of avian NC development. PG-M/versicans

The complex [Mn-III(HL)(L)(py)(CH3OH)](CH3OH)-C-. (H2L=2-ortho-hydroxyphenylbenzimidazole, py pyridine), 1, has been characterized spectroscopically and by X-ray crystallography. The complex is triclinic, space group P-1(-) with a = 10.396(2), b = 10.7340(10), c = 15.193(2) angstrom, alpha = 73.193(4)degrees, beta = 76.283(8)degrees, gamma = 61.400(40)degrees, V = 1415.1(4)angstrom(3), Z = 2, D-c

Superlattices with magnetic layers containing from 8 to 16 GaMnAs monolayers corresponding to the mixed crystal composition between 5 and 6% of Mn and from 4 to 10 GaAs monolayers were grown by the low temperature MBE technique and characterized by Raman scattering. Folded acoustic phonons were observed for all superlattices in the Raman scattering spectra. The interlayer exchange coupling, found

Cardiac index, systemic and pulmonary arterial pressures, carbon dioxide elimination and ventilation of each lung were studied during thoracotomy. Seventeen patients, placed in the full lateral position, were ventilated mechanically through a Carlens' tube to moderate hypocapnia. Mean cardiac index increased by 12% as the pleura was opened (P less than 0.05), with no further change during surgery

We have examined the short-term effects of three volatile anaesthetics, halothane, isoflurane and desflurane, on mucociliary activity in the rabbit maxillary sinus in vivo. Mucociliary activity was recorded photoelectrically and the signal processed by fast Fourier transformation. Administration of 1.0 MAC of halothane, isoflurane or desflurane caused a temporary increase in mucociliary activity,

Six healthy volunteers were exposed to gaseous hexahydrophthalic anhydride (HHPA) concentrations of 10, 40, or 80 micrograms.m-3 (65, 260 or 520 nmol.m-3, respectively) for 8 h. The respiratory uptake of the inhaled HHPA was almost complete. Rapid increases in plasma and urinary levels of hexahydrophthalic acid (HHP acid) were seen. During the first 4 h after the end of exposure, the half-time of

This article addresses the role of proximity for knowledge collaboration between dedicated biotechnology firms (DBFs) and related actors. Innovation projects managed by a selection of eight Swedish DBFs are analysed in detail and classified with regard to their specific knowledge characteristics. Based on this classification, explanations to the relative importance of functional and relational pro