The Great Space Weather Event during February 1872 Recorded in East Asia

The study of historical great geomagnetic storms is crucial for assessing the possible risks to the technological infrastructure of a modern society, caused by extreme space-weather events. The normal benchmark has been the great geomagnetic storm of September 1859, the so-called "Carrington Event". However, there are numerous records of another great geomagnetic storm in February 1872. This storm, about 12 years after the Carrington Event, resulted in comparable magnetic disturbances and auroral displays over large areas of the Earth. We have revisited this great geomagnetic storm in terms of the auroral and sunspot records in the historical documents from East Asia. In particular, we have surveyed the auroral records from East Asia and estimated the equatorward boundary of the auroral oval to be near 24.3 deg invariant latitude (ILAT), on the basis that the aurora was seen near the zenith at Shanghai (20 deg magnetic latitude, MLAT). These results confirm that this geomagnetic storm of February 1872 was as extreme as the Carrington Event, at least in terms of the equatorward motion of the auroral oval. Indeed, our results support the interpretation of the simultaneous auroral observations made at Bombay (10 deg MLAT). The East Asian auroral records have indicated extreme brightness, suggesting unusual precipitation of high-intensity, low-energy electrons during this geomagnetic storm. We have compared the duration of the East Asian auroral displays with magnetic observations in Bombay and found that the auroral displays occurred in the initial phase, main phase, and early recovery phase of the magnetic storm.Comment: 28 pages, 5 figures, accepted for publication in the Astrophysical Journal on 31 May 201

Defect in proline synthesis: pyrroline-5-carboxylate reductase 1 deficiency leads to a complex clinical phenotype with collagen and elastin abnormalities

Pyrroline-5-carboxylate reductase 1 (PYCR1) catalyzes the last step in proline synthesis. Deficiency of PYCR1, caused by a defect in PYCR1, was recently described in patients with cutis laxa, intrauterine growth retardation, developmental dysplasia of the hips and mental retardation. In this paper, we describe additional six patients (ages ranging from 4 months to 55 years) from four Iranian families with clinical manifestations of a wrinkly skin disorder. All patients have distinct facial features comprising triangular face, loss of adipose tissue and thin pointed nose. Additional features are short stature, wrinkling over dorsum of hand and feet, visible veins over the chest and hyperextensible joints. Three of the patients from a large consanguineous family do not have mental retardation, while the remaining three patients from three unrelated families have mental and developmental delay. Mutation analysis revealed the presence of disease-causing variants in PYCR1, including a novel deletion of the entire PYCR1 gene in one family, and in each of the other patients the homozygous missense mutations c.616G > A (p.Gly206Arg), c.89T > A (p.Ile30Lys) and c.572G > A (p.Gly191Glu) respectively, the latter two of which are novel. Light- and electron microscopy investigations of skin biopsies showed smaller and fragmented elastic fibres, abnormal morphology of the mitochondria and their cristae, and slightly abnormal collagen fibril diameters with irregular outline and variable size. In conclusion, this study adds information on the natural course of PYCR1 deficiency and sheds light on the pathophysiology of this disorder. However, the exact pathogenesis of this new disorder and the role of proline in the development of the clinical phenotype remain to be fully explaine

Non-Equilibrium Processes in the Solar Corona, Transition Region, Flares, and Solar Wind \textit{(Invited Review)}

We review the presence and signatures of the non-equilibrium processes, both non-Maxwellian distributions and non-equilibrium ionization, in the solar transition region, corona, solar wind, and flares. Basic properties of the non-Maxwellian distributions are described together with their influence on the heat flux as well as on the rates of individual collisional processes and the resulting optically thin synthetic spectra. Constraints on the presence of high-energy electrons from observations are reviewed, including positive detection of non-Maxwellian distributions in the solar corona, transition region, flares, and wind. Occurrence of non-equilibrium ionization is reviewed as well, especially in connection to hydrodynamic and generalized collisional-radiative modelling. Predicted spectroscopic signatures of non-equilibrium ionization depending on the assumed plasma conditions are summarized. Finally, we discuss the future remote-sensing instrumentation that can be used for detection of these non-equilibrium phenomena in various spectral ranges.Comment: Solar Physics, accepte

SEA SURFACE TEMPERATURES RECONSTRUCTION OF THE LAST 16,000 YEARS IN THE EASTERN MEDITERRANEAN SEA

A detailed study has been performed on two eastern Mediterranean box cores (BC02 and BC06) and on a southern Adriatic piston core (AD91-17) on the alkenone unsaturation ratio, a molecular proxy for past sea surface temperatures. The aim was to identify climatic events of the last 16 Ky, with particular attention on the conditions during formation of sapropel S1. All three temperature curves lack evidence for cooling in the Younger Dryas stadial and warming in the Boelling/Alleroed interstadial events. Just prior to the sapropel S1 base, SST cooled and increased by about 5°C during the sapropel deposition interval. Within sapropel S1, SST show a marked warming followed by a clear cooling. In the topmost intervals of the cores SST are mostly constant, but a warming event is always observed. This warming phase may correspond to the Medieval climatic Optimum (in the AD91-17 core) and to the Roman Optimum (in the box cores)

COL1-Related Disorders: Case Report and Review of Overlapping Syndromes

Collagen type I mutations are related to wide phenotypic expressions frequently causing an overlap of clinical manifestations, in particular between Osteogenesis Imperfecta (OI) and Ehlers-Danlos syndrome (EDS). Both disorders present inter- and intra-familial clinical variability and several clinical signs are present in both diseases. Recently, after the observation that some individuals first ascertained by a suspicion of EDS resulted then carriers of pathogenic variants of genes known to primarily cause OI, some authors proposed the term “COL1-related overlap disorder” to describe these cases. In this paper, we report clinical, molecular, and biochemical information about an individual with a diagnosis of EDS with severe joint hypermobility who carries a pathogenic heterozygous variant in COL1A2 gene, and a benign variant in COL1A1 gene. The pathogenic variant, commonly ascribed to OI, as well as the benign variant, has been inherited from the individual's mother, who presented only mild signs of OI and the diagnosis of OI was confirmed only after molecular testing. In addition, we reviewed the literature of similar cases of overlapping syndromes caused by COL1 gene mutations. The reported case and the literature review suggest that the COL1-related overlap disorders (OI, EDS and overlapping syndromes) represent a continuum of clinical phenotypes related to collagen type I mutations. The spectrum of COL1-related clinical manifestations, the pathophysiology and the underlying molecular mechanisms support the adoption of the updated proposed term “COL1-related overlap disorder” to describe the overlapping syndromes

Severe early onset preeclampsia: short and long term clinical, psychosocial and biochemical aspects

Preeclampsia is a pregnancy specific disorder commonly defined as de novo hypertension and proteinuria after 20 weeks gestational age. It occurs in approximately 3-5% of pregnancies and it is still a major cause of both foetal and maternal morbidity and mortality worldwide1. As extensive research has not yet elucidated the aetiology of preeclampsia, there are no rational preventive or therapeutic interventions available. The only rational treatment is delivery, which benefits the mother but is not in the interest of the foetus, if remote from term. Early onset preeclampsia (<32 weeks’ gestational age) occurs in less than 1% of pregnancies. It is, however often associated with maternal morbidity as the risk of progression to severe maternal disease is inversely related with gestational age at onset2. Resulting prematurity is therefore the main cause of neonatal mortality and morbidity in patients with severe preeclampsia3. Although the discussion is ongoing, perinatal survival is suggested to be increased in patients with preterm preeclampsia by expectant, non-interventional management. This temporising treatment option to lengthen pregnancy includes the use of antihypertensive medication to control hypertension, magnesium sulphate to prevent eclampsia and corticosteroids to enhance foetal lung maturity4. With optimal maternal haemodynamic status and reassuring foetal condition this results on average in an extension of 2 weeks. Prolongation of these pregnancies is a great challenge for clinicians to balance between potential maternal risks on one the eve hand and possible foetal benefits on the other. Clinical controversies regarding prolongation of preterm preeclamptic pregnancies still exist – also taking into account that preeclampsia is the leading cause of maternal mortality in the Netherlands5 - a debate which is even more pronounced in very preterm pregnancies with questionable foetal viability6-9. Do maternal risks of prolongation of these very early pregnancies outweigh the chances of neonatal survival? Counselling of women with very early onset preeclampsia not only comprises of knowledge of the outcome of those particular pregnancies, but also knowledge of outcomes of future pregnancies of these women is of major clinical importance. This thesis opens with a review of the literature on identifiable risk factors of preeclampsia

Impact of Somatic DNA Repair Mutations on the Clinical Outcomes of Bone Metastases from Castration-Resistant Prostate Cancer

Up to 80% of castration-resistant prostate cancer (CRPC) patients develop bone metastases during the natural history of disease and about 25% harbor mutations in DNA damage repair (DDR) genes. This retrospective observational study evaluated the prevalence of DDR alterations in CRPC patients and their effect on the clinical outcomes associated with bone metastases. The mutational status of CRPC patients was analyzed per FoundationOne(&amp; REG;) analysis in tissue biopsy or, when it was not possible, in liquid biopsy performed at the onset of metastatic CRPC (mCRPC). The impact of DDR gene mutations on bone-related efficacy endpoints was evaluated at the time of mCRPC diagnoses. In total, 121 mCRPC patients with bone metastases were included: 38 patients had mutations in at least one DDR gene, the remaining 83 ones had a non-mutated DDR status. DDR mutated status was associated with bone metastases volume (p = 0.006), but did not affect SRE (skeletal-related events) incidence and time to SRE onset. Liquid and tissue biopsies were both available for 61 patients with no statistically significant difference in terms of incidence and type of molecular DDR alterations. Mutated DDR status was associated with higher bone metastasic volume, although a not detrimental effect on the other bone-related efficacy endpoints was observed

Penilaian Kinerja Keuangan Koperasi di Kabupaten Pelalawan

This paper describe development and financial performance of cooperative in District Pelalawan among 2007 - 2008. Studies on primary and secondary cooperative in 12 sub-districts. Method in this stady use performance measuring of productivity, efficiency, growth, liquidity, and solvability of cooperative. Productivity of cooperative in Pelalawan was highly but efficiency still low. Profit and income were highly, even liquidity of cooperative very high, and solvability was good