New era in treatment for phenylketonuria: Pharmacologic therapy with sapropterin dihydrochloride

Oral administration of sapropterin hydrochloride, recently approved for use by the US Food and Drug Administration and the European Commission, is a novel approach for the treatment of phenylketonuria (PKU), one of the most common inborn errors of metabolism. PKU is caused by an inherited deficiency of the enzyme phenylalanine hydroxylase (PAH), and the pathophysiology of the disorder is related to chronic accumulation of the free amino acid phenylalanine in tissues. Contemporary therapy is based upon restriction of dietary protein intake, which leads to reduction of blood phenylalanine levels. This therapy is difficult to maintain throughout life, and dietary noncompliance is commonplace. Sapropterin dihydrochloride is a synthetic version of tetrahydrobiopterin, the naturally occurring pterin cofactor that is required for PAH-mediated phenylalanine hydroxylation. In a subset of individuals with PAH deficiency, sapropterin administration leads to reduction in blood phenylalanine levels independent of dietary protein. For these individuals, sapropterin is an effective novel therapy for PKU

Is the Scottish population living dangerously? Prevalence of multiple risk factors: the Scottish Health Survey 2003

<b>Background:</b> Risk factors are often considered individually, we aimed to investigate the prevalence of combinations of multiple behavioural risk factors and their association with socioeconomic determinants.<p></p> <b>Methods:</b> Multinomial logistic regression was used to model the associations between socioeconomic factors and multiple risk factors from data in the Scottish Health Survey 2003. Prevalence of five main behavioural risk factors - smoking alcohol, diet, overweight/obesity, and physical inactivity, and the odds in relation to demographic, individual and area socioeconomic factors.<p></p> <b>Results:</b> Full data were available on 6,574 subjects (80.7% of the survey sample). Nearly the whole adult population (97.5%) reported to have at least one behavioural risk factor; while 55% have three or more risk factors; and nearly 20% have four or all five risk factors. The most important determinants for having four or five multiple risk factors were low educational attainment which conferred around a 3-fold increased odds compared to high education; and residence in the most deprived communities (relative to least deprived) which had greater than 3-fold increased odds.<p></p> <b>Conclusions:</b> The prevalence of multiple behavioural risk factors was high and the prevalence of absence of all risk factors very low. These behavioural patterns were socioeconomically determined. Policy to address factors needs to be joined up and better consider underlying socioeconomic circumstances.<p></p&gt

Detection of X-ray Emission from the Very Old Pulsar J0108-1431

PSR J0108-1431 is a nearby, 170 Myr old, very faint radio pulsar near the "pulsar death line" in the P-Pdot diagram. We observed the pulsar field with the Chandra X-ray Observatory and detected a point source (53 counts in a 30 ks exposure, energy flux (9+/-2)\times 10^{-15} ergs cm^{-2} s^{-1} in the 0.3-8 keV band) close to the radio pulsar position. Based on the large X-ray/optical flux ratio at the X-ray source position, we conclude that the source is the X-ray counterpart of PSR J0108-1431.The pulsar spectrum can be described by a power-law model with photon index Gamma \approx 2.2 and luminosity L_{0.3-8 keV} \sim 2\times 10^{28} d_{130}^2 ergs s^{-1}, or by a blackbody model with the temperature kT\approx 0.28 keV and bolometric luminosity L_{bol} \sim 1.3\times 10^{28} d_{130}^2 ergs s^{-1}, for a plausible hydrogen column density NH = 7.3\times 10^{19} cm^{-2} (d_{130}=d/130 pc). The pulsar converts \sim 0.4% of its spin-down power into the X-ray luminosity, i.e., its X-ray efficiency is higher than for most younger pulsars. From the comparison of the X-ray position with the previously measured radio positions, we estimated the pulsar proper motion of 0.2 arcsec yr^{-1} (V_\perp \sim 130 d_{130} km s^{-1}), in the south-southeast direction.Comment: 8 pages, 9 figures, accepted to ApJ; minor revisions in Sections 2.2 and 3.

Magnetic Photon Splitting: Computations of Proper-time Rates and Spectra

The splitting of photons in the presence of an intense magnetic field has recently found astrophysical applications in polar cap models of gamma-ray pulsars and in magnetar scenarios for soft gamma repeaters. Numerical computation of the polarization-dependent rates of this third order QED process for arbitrary field strengths and energies below pair creation threshold is difficult: thus early analyses focused on analytic developments and simpler asymptotic forms. The recent astrophysical interest spurred the use of the S-matrix approach by Mentzel, Berg and Wunner to determine splitting rates. In this paper, we present numerical computations of a full proper-time expression for the rate of splitting that was obtained by Stoneham, and is exact up to the pair creation threshold. While the numerical results derived here are in accord with the earlier asymptotic forms due to Adler, our computed rates still differ by as much as factors of 3 from the S-matrix re-evaluation of Wilke and Wunner, reflecting the extreme difficulty of generating accurate S-matrix numerics for fields below about \teq{4.4\times 10^{13}}Gauss. We find that our proper-time rates appear very accurate, and exceed Adler's asymptotic specializations significantly only for photon energies just below pair threshold and for supercritical fields, but always by less than a factor of around 2.6. We also provide a useful analytic series expansion for the scattering amplitude valid at low energies.Comment: 13 pages, AASTeX format, including 3 eps figures, ApJ in pres

Predictions of Gamma-ray Emission from Globular Cluster Millisecond Pulsars Above 100 MeV

The recent Fermi detection of the globular cluster (GC) 47 Tucanae highlighted the importance of modeling collective gamma-ray emission of millisecond pulsars (MSPs) in GCs. Steady flux from such populations is also expected in the very high energy (VHE) domain covered by ground-based Cherenkov telescopes. We present pulsed curvature radiation (CR) as well as unpulsed inverse Compton (IC) calculations for an ensemble of MSPs in the GCs 47 Tucanae and Terzan 5. We demonstrate that the CR from these GCs should be easily detectable for Fermi, while constraints on the total number of MSPs and the nebular B-field may be derived using the IC flux components.Comment: 12 pages, 2 figures, accepted for publication in ApJ

Fish farm effluent as a nutrient source for algae biomass cultivation

One of the challenges of microalgae biotechnology is the cost of growth media nutrients, with microalgae consuming enormous quantities of fertilisers, more than other oil crops. The traditional use of synthetic fertilisers in mass cultivation of microalgae is associated with rising prices of crude oil and competition from traditional agriculture. The fact that fish farm wastewater (FFW) nutrients are released in the form preferred by microalgae (NH3 for nitrogen and PO4 -3 for phosphate), and the ability of microalgae to use nitrogen from different sources, can be exploited by using fish farm effluent rich in nutrients (nitrogen and phosphorus) in the cultivation of cheaper microalgae biomass for production of biodiesel. The cultivationof algae biomass in FFW will also serve as wastewater treatment. We reviewed the benefits and potential of fish effluent in algae cultivation for the production of biodiesel. Microalgae can utilise nutrients in FFW for different applications desirable for the production of biomass, including the accumulation of lipids, and produce a fuel with desirable properties. Also, treating wastewater and reducing demand for fresh water are advantageous. The high lipid content and comparable biodiesel properties of Chlorella sorokiniana and Scenedesmus obliquus make both species viable for FFW cultivation for biodiesel production. Significance: The cost associated with microalgae growth media nutrients can be saved by using fish farm wastewater,which contains nutrients (nitrogen and phosphorus) suitable for microalgae cultivation. Fish farm wastewater has lower nutrient concentrations when compared to standard growth media suitable for higher lipid accumulation. Microalgae used as a biodiesel feedstock, cultivated in fish farm wastewater, has added benefits, including wastewater treatment