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Sample records for loss causing severe

  1. Cochlear implantation for severe sensorineural hearing loss caused by lightning.

    Science.gov (United States)

    Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun

    2012-01-01

    Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation. Copyright © 2012 Elsevier Inc. All rights reserved.

  2. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

    Directory of Open Access Journals (Sweden)

    An-Shine Chao

    2009-01-01

    Full Text Available We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebral artery was 0.8 m/s, indicating severe fetal anemia. Multiple intrauterine transfusions for the anemic fetus were administered. However, persistent severe fetal anemia and placentomegaly caused poor neonatal death and mirror syndrome in the mother. Uncommon red blood cell alloimmunization has to be watched for early in any population, especially in a woman with a history of unexplained perinatal loss.

  3. Desynchronizations in bee-plant interactions cause severe fitness losses in solitary bees.

    Science.gov (United States)

    Schenk, Mariela; Krauss, Jochen; Holzschuh, Andrea

    2018-01-01

    Global warming can disrupt mutualistic interactions between solitary bees and plants when increasing temperature differentially changes the timing of interacting partners. One possible scenario is for insect phenology to advance more rapidly than plant phenology. However, empirical evidence for fitness consequences due to temporal mismatches is lacking for pollinators and it remains unknown if bees have developed strategies to mitigate fitness losses following temporal mismatches. We tested the effect of temporal mismatches on the fitness of three spring-emerging solitary bee species, including one pollen specialist. Using flight cages, we simulated (i) a perfect synchronization (from a bee perspective): bees and flowers occur simultaneously, (ii) a mismatch of 3 days and (iii) a mismatch of 6 days, with bees occurring earlier than flowers in the latter two cases. A mismatch of 6 days caused severe fitness losses in all three bee species, as few bees survived without flowers. Females showed strongly reduced activity and reproductive output compared to synchronized bees. Fitness consequences of a 3-day mismatch were species-specific. Both the early-spring species Osmia cornuta and the mid-spring species Osmia bicornis produced the same number of brood cells after a mismatch of 3 days as under perfect synchronization. However, O. cornuta decreased the number of female offspring, whereas O. bicornis spread the brood cells over fewer nests, which may increase offspring mortality, e.g. due to parasitoids. The late-spring specialist Osmia brevicornis produced fewer brood cells even after a mismatch of 3 days. Additionally, our results suggest that fitness losses after temporal mismatches are higher during warm than cold springs, as the naturally occurring temperature variability revealed that warm temperatures during starvation decreased the survival rate of O. bicornis. We conclude that short temporal mismatches can cause clear fitness losses in solitary bees

  4. Causes of visual loss in uveitis

    Directory of Open Access Journals (Sweden)

    Stanković Zora

    2009-01-01

    Full Text Available Background/Aim. Epidemiological studies of blindness in a working age population require a precise definition of the true connection of uveitis and visual damage. Since most patients with more severe types of uveitis are hospitalized in tertiary referral uveitis service, our aim was to determine whether age, sex and age of onset of uveitis, as well as duration of visual loss and its causes influence the degree of visual damage in patients with different types of uveitis. Methods. The data were collected from medical records of 237 patients at the Department for Uveitis of the Institute for Eye Diseases in Belgrade over a three-year period (March 2005 to March 2008. Results. Visual acuity reduction (≤ 0.3 was found in 161/237 (67.9% patients, 85 of whom had visual acuity of ≤ 0.1 later. Working age patients (up to 60 years of age most often suffered from uveitis (173/237; 73%. The highest number of patients with visual loss was in the group suffering from panuveitis (77/94; 81.91%. The age of onset of uveitis and sex have no statistically significant influence on visual loss. The most common causes of visual loss (34/161; 21.1% were cystoid macular oedema (CMO (43/161; 26.7%, cataract (28/161; 17.39% and combination of CMO and cataract. Conclusion. The risk factors for severe visual loss (≤ 0.1 are panuveitis, bilateral inflammation, prolonged visual reduction and a significant number of relapses. The main causes of visual loss in 65.2% of our patients were CMO and cataract.

  5. 7 CFR 760.611 - Qualifying losses, eligible causes and types of loss.

    Science.gov (United States)

    2010-01-01

    ... final planting date; (4) The cause of loss was due to water contained or released by any governmental... containment or release of the water; (5) The cause of loss was due to conditions or events occurring outside...) Losses caused by a failure of power supply or brownout as defined in § 760.602; (2) Losses caused by the...

  6. 7 CFR 1437.9 - Causes of loss.

    Science.gov (United States)

    2010-01-01

    ... 7 Agriculture 10 2010-01-01 2010-01-01 false Causes of loss. 1437.9 Section 1437.9 Agriculture... Provisions § 1437.9 Causes of loss. (a) To be eligible for benefits under this part, an eligible cause of loss must result in: (1) A loss of production greater than 50 percent of the approved yield in...

  7. Genetic Causes of Recurrent Pregnancy Loss.

    Science.gov (United States)

    Page, Jessica M; Silver, Robert M

    2016-09-01

    Pregnancy loss is one of the most common obstetric complications, affecting over 30% of conceptions. A considerable proportion of losses are due to genetic abnormalities. Indeed, over 50% of early pregnancy losses have been associated with chromosomal abnormalities. Most are due to de novo nondisjunctional events but balanced parental translocations are responsible for a small but important percentage of genetic abnormalities in couples with recurrent pregnancy loss. In the past, assessment of genetic abnormalities was limited to karyotype performed on placental or fetal tissue. However, advances in molecular genetic technology now provide rich genetic information about additional genetic causes of and risk factors for pregnancy loss. In addition, the use of preimplantation genetic testing in couples undergoing in vitro fertilization has the potential to decrease the risk of pregnancy loss from genetic abnormalities. To date, efficacy is uncertain but considerable potential remains. This chapter will review what is known about genetic causes of recurrent pregnancy loss with a focus on novel causes and potential treatments. Remaining knowledge gaps will be highlighted.

  8. Systemic causes of hair loss.

    Science.gov (United States)

    Lin, Richard L; Garibyan, Lilit; Kimball, Alexandra B; Drake, Lynn A

    2016-09-01

    Hair loss is both a common chief complaint by patients and a clinical challenge for physicians, especially general practitioners, yet few dermatological problems yield as much patient satisfaction when resolved as hair loss. The diagnosis is often attributed to androgen-related hair loss, while other causes, some of which are life-threatening but treatable, are overlooked. We searched for relevant literature on hair loss and supported these findings with our clinical experience to identify seven major systemic etiologies of hair loss, ranging from infectious agents to consumption of unsafe supplements. Many causes are only described in the literature through case studies, though some original articles and meta-analyses are available. Careful history taking, proper examination techniques, and judicious use of laboratory tests are essential to reach at the correct diagnosis in a cost-effective manner when performing patient work-up. Such methodical evaluation of hair loss can result in the appropriate treatment plan and provide significant patient satisfaction. Key messages Hair loss is a common chief complaint and a difficult challenge for both general practitioners and dermatology consultants. We identified seven major categories of systemic hair loss etiology and present a framework for their clinical evaluation. A methodical approach to hair loss can result in the appropriate treatment plan and provide significant patient satisfaction.

  9. Oral Health Problems And Causes Of Tooth Loss At The University ...

    African Journals Online (AJOL)

    Context Several oral diseases and conditions are associated with tooth loss. Objective: The aim of this study was to determine the prevalence of oral health problems seen at the University of Nigeria Teaching Hospital (UNTH) in one year with the view to highlighting causes of tooth loss in this environment. Methods This ...

  10. Acute sensorineural hearing loss and severe otalgia due to scrub typhus

    Directory of Open Access Journals (Sweden)

    Kim Dong-Min

    2009-10-01

    Full Text Available Abstract Background Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi. Case presentations We encountered a patient with sensorineural hearing loss complicating scrub typhus, and three patients with scrub typhus who complained of otalgia, which was sudden onset, severe, paroxysmal, intermittent yet persistent pain lasting for several seconds, appeared within 1 week after the onset of fever and rash. The acute sensorineural hearing loss and otalgia were resolved after antibiotic administration. Conclusion When patients in endemic areas present with fever and rash and have sensorineural hearing loss or otalgia without otoscopic abnormalities, clinicians should suspect scrub typhus and consider empirical antibiotic therapy.

  11. Hair Loss: Common Causes and Treatment.

    Science.gov (United States)

    Phillips, T Grant; Slomiany, W Paul; Allison, Robert

    2017-09-15

    Hair loss is often distressing and can have a significant effect on the patient's quality of life. Patients may present to their family physician first with diffuse or patchy hair loss. Scarring alopecia is best evaluated by a dermatologist. Nonscarring alopecias can be readily diagnosed and treated in the family physician's office. Androgenetic alopecia can be diagnosed clinically and treated with minoxidil. Alopecia areata is diagnosed by typical patches of hair loss and is self-limited. Tinea capitis causes patches of alopecia that may be erythematous and scaly and must be treated systemically. Telogen effluvium is a nonscarring, noninflammatory alopecia of relatively sudden onset caused by physiologic or emotional stress. Once the precipitating cause is removed, the hair typically will regrow. Trichotillomania is an impulse-control disorder; treatment is aimed at controlling the underlying psychiatric condition. Trichorrhexis nodosa occurs when hairs break secondary to trauma and is often a result of hair styling or overuse of hair products. Anagen effluvium is the abnormal diffuse loss of hair during the growth phase caused by an event that impairs the mitotic activity of the hair follicle, most commonly chemotherapy. Physician support is especially important for patients in this situation.

  12. When Telomerase Causes Telomere Loss.

    Science.gov (United States)

    Glousker, Galina; Lingner, Joachim

    2018-02-05

    Telomerase counteracts telomere shortening, preventing cellular senescence. Telomerase deficiency causes telomere syndromes because of premature telomere exhaustion in highly proliferative cells. Paradoxically, in a recent issue of Cell, Margalef et al. (2018) demonstrate that telomerase causes telomere loss in cells lacking the RTEL1 helicase, which is defective in Hoyeraal-Hreidarsson syndrome (HHS). Copyright © 2018 Elsevier Inc. All rights reserved.

  13. Genetic mutation susceptibility of hearing loss in child with severe neonatal jaundice

    International Nuclear Information System (INIS)

    Zahedi, F.D.; Rahman, R.A.; Abdullah, A.

    2015-01-01

    This case report demonstrates a case of 5-year-old non-syndromic Malay boy who passed the hearing screening test however he was confirmed has bilateral profound sensorineural hearing loss diagnosed at 3 months of age by brain stem evoked response (BSER). He has background history of severe neonatal jaundice and male siblings of hearing impairment. The antenatal and birth history was uneventful apart from maternal hypothyroidism. His other two elder brothers have bilateral sensorineural hearing loss and history of severe neonatal jaundice as well. The ear examinations, computed tomography scan and magnetic resonance imaging revealed normal findings. Right sided cochlear implantation was done at the age of 3 years old and he is still under audiology follow-up. Conclusion: Genetic studies are important to determine the cause of genetic mutation in susceptibility to hearing impairment that run in his family after severe neonatal jaundice. Those baby with risk of developing hearing loss required diagnostic hearing assessment. (author)

  14. Diagnosis of Hair Loss: Clinical features of common causes of hair loss

    OpenAIRE

    Coupe, Robert L.M.

    1992-01-01

    Common causes of hair loss include androgenic hair loss, alopecia areata, trichotillomania, tinea capitis, telogen effluvium, and traction alopecia. The author discusses their distinguishing clinical features and those of less common alopecias.

  15. The severity of temporomandibular joint disorder by teeth loss in the elderly

    Directory of Open Access Journals (Sweden)

    Indry Herdiyani

    2011-07-01

    Full Text Available Temporomandibular joint dysfunction is a term that covers a number of clinical problems that involves masticatory muscles, temporomandibular joints, and related structures, or both. Loss of tooth was an etiology of temporomandibular joint dysfunction. The purpose of this study was to obtain the description of temporomandibular joint dysfunction level that caused by tooth loss of elderly in three nursing home Bandung. This was a descriptive study using the survey method of the elderly in three nursing home Bandung. A total of 34 people consist 6 males and 28 females. The subjects were examined by symptoms of temporomandibular joint dysfunction and the dysfunction level was assessed by Helkimo Clinical Dysfunction Index. The result of this study shows that elderly in Nursing Home Bandung have mild dysfunction level was 7 (14.71%, moderate dysfunction level was 22 (64.71%, and severe dysfunction level is 5 (20.58%. It can be concluded that loss of the teeth is one of the etiologies of temporomandibular joint disorder. Based on the research conducted, it can be concluded that all elderly with teeth loss will have the temporomandibular joint disorder and the most severity happens based on teeth loss by using the Helkimo Clinical Disfunction Index score was the moderate disorder.

  16. Recurrent Pregnancy Loss: Generally Accepted Causes and Their Management.

    Science.gov (United States)

    Kaiser, Jennifer; Branch, D Ware

    2016-09-01

    Recurrent pregnancy loss (RPL), commonly defined as 3 consecutive losses causes include uterine malformation, antiphospholipid syndrome, and parental chromosomal abnormalities; however, the majority of RPL cases are idiopathic (up to 75%). This chapter covers these accepted causes of RPL and provides diagnosis and management strategies for patients falling within the above categories.

  17. Allocation of Load-Loss Cost Caused by Voltage Sag

    Science.gov (United States)

    Gao, X.

    2017-10-01

    This paper focuses on the allocation of load-loss cost caused by voltage sag in the environment of electricity market. To compensate the loss of loads due to voltage sags, the load-loss cost is allocated to both sources and power consumers. On the basis of Load Drop Cost (LDC), a quantitative evaluation index of load-loss cost caused by voltage sag is identified. The load-loss cost to be allocated to power consumers themselves is calculated according to load classification. Based on the theory of power component the quantitative relation between sources and loads is established, thereby a quantitative calculation method for load-loss cost allocated to each source is deduced and the quantitative compensation from individual source to load is proposed. A simple five-bus system illustrates the main features of the proposed method.

  18. Schizophrenia illness severity is associated with reduced loss aversion.

    Science.gov (United States)

    Currie, James; Buruju, Dheeraj; Perrin, Jennifer S; Reid, Ian C; Steele, J Douglas; Feltovich, Nick

    2017-06-01

    Loss aversion, whereby losses weigh more heavily than equal-sized gains, has been demonstrated in many decision-making settings. Previous research has suggested reduced loss aversion in schizophrenia, but with little evidence of a link between loss aversion and schizophrenia illness severity. In this study, 20 individuals with schizophrenia and 16 control participants, matched by age and sex, played two versions of the Iterated Prisoners' Dilemma, one version with only positive payoffs and another version in which negative payoffs were possible, with the second version being derived from the first by subtracting a constant value from all payoffs. The control group demonstrated significantly lower cooperation rates under negative payoffs, compared with the version with only positive payoffs, indicative of loss aversion. The patient group on average showed no loss aversion response. Moreover, the extent of loss aversion in patients was found to be negatively correlated with schizophrenia illness severity, with less ill patients showing loss aversion more similar to controls. Results were found to be robust to the inclusion of potential confounding factors as covariates within rigorous probit regression analyses. Reduced loss aversion is a feature of schizophrenia and related to illness severity. Copyright © 2017. Published by Elsevier B.V.

  19. [Intractable diarrhoea and severe weight loss by roflumilast].

    Science.gov (United States)

    Horna, Oihana; Toyas, Carla

    2013-08-04

    Roflumilast is a recently marketed drug, indicated for maintenance treatment of severe chronic obstructive pulmonary disease associated with chronic bronchitis in adult patients with a history of frequent exacerbations as add on to bronchodilator treatment. The safety data of this drug have always been subjected to controversy and concerns. The Food and Drug Administration rejected the drug after the first evaluation, asking the company to clarify the adverse reactions during the investigation process, the European Medicines Agency approved the drug including a Risk Management Plan, designed to promote a safe use of the drug. During the first months after the marketing process, the Spanish Pharmacovigilance System has already been acquainted of several adverse events notifications; therefore, these patients may be closely monitored, mainly because of digestive and psychiatric disorders. Here we report the case of a female patient who showed a serious digestive clinical profile and a severe weight loss, more than 25% of her initial weight, when a treatment with roflumilast was started. The suspicion of a side effect as the cause of the reported clinical profile and its resolution required 3 hospital admissions. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  20. Shortened stapedius tendon: a rare cause of conductive hearing loss.

    Science.gov (United States)

    Zawawi, F; Varshney, R; Schloss, M D

    2014-01-01

    Anomalies of the stapedius tendon have been reported to cause conductive hearing loss; in theory, such anomalies limit the movement of the stapes. To demonstrate a rare cause of conductive hearing loss resulting from anomaly of the stapedius tendon and to compare the clinical findings of this patient to other stapedius tendon anomalies reported in the literature. Case report of a single case of shortened stapedius tendon and a review of the English literature on stapedius tendon anomalies. This is a case report of a 15-year-old boy with shortened stapedius tendon causing unilateral hearing loss, accompanied by a review of the literature. Contrary to other reported cases, this patient did not have an ossified tendon, but rather an extremely short tendon. The boy regained normal hearing following excision of the stapedius tendon. A shortened stapedius tendon is a very rare diagnosis, yet it should be considered as a possible cause of conductive hearing loss.

  1. A statistical study of GPS loss of lock caused by ionospheric disturbances

    Science.gov (United States)

    Tsugawa, T.; Nishioka, M.; Otsuka, Y.; Saito, A.; Kato, H.; Kubota, M.; Nagatsuma, T.; Murata, K. T.

    2010-12-01

    Two-dimensional total electron content (TEC) maps have been derived from ground-based GPS receiver networks and applied to studies of various ionospheric disturbances since mid-1990s. For the purpose of monitoring and researching ionospheric disturbances which can degrade GNSS navigations and cause loss-of-lock on GNSS signals, National Institute of Information and Communications Technology (NICT), Japan has developed TEC maps over Japan using the dense GPS network, GEONET, which consists of more than 1,200 GPS receivers and is operated by Geophysical Survey Institute, Japan. Currently, we are providing two-dimensional maps of absolute TEC, detrended TEC with 60, 30, 15-minute window, rate of TEC change index (ROTI), and loss-of-lock (LOL) on GPS signal over Japan. These data and quick-look maps since 1997 are archived and available in the website of NICT (http://wdc.nict.go.jp/IONO/). Recently developed GPS receiver networks in North America and Europe make it possible to obtain regional TEC maps with higher spatial and temporal resolution than the global weighted mean TEC maps in the IONEX format provided by several institutes such as International GNSS Service (IGS) and another global TEC map provided by MIT Haystack observatory. Recently, we have also developed the regional TEC maps over North America and Europe. These data and quick-look maps are also available in the NICT website. In this presentation, we will show some severe ionospheric events such as high latitude storm-time plasma bubbles and storm enhanced density events observed over Japan using the GPS-TEC database. These events cause loss-of-lock of GPS signals and large GPS positioning errors. We also discuss about the statistical characteristics of LOL on the GPS signal caused by ionospheric disturbances.

  2. Clinical and experimental studies on the sensorineural hearing loss caused by irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, M [Kobe Univ. (Japan). School of Medicine

    1979-08-01

    In our study, 43 patients whose ears were presumably irradiated, were examined periodically before and after the irradiation; 10 were found to be affected by the sensorineural hearing loss, and these 10 were classified into two types. Type I; Patients of this group had a hearing impairment during or soon after irradiation and usually revealed marked deterioration of bone conduction in high frequencies accompanied by worsening of air conduction in low frequencies. The impaired hearing of this group soon recovered to the pre-treatment level. Type II; Patients of this group developed a slow progressive sensorineural hearing loss. They noticed the deafness with tinnitus several months after irradiation. To clarify the mechanism of the sensorineural hearing loss, histopathological investigations were done using nembutal anesthetized guinea pigs which were irradiated in the confined ear region unilaterally. We also examined histopathologically one human temporal bone belonging to a patient who had been irradiated for middle ear carcinoma. The histopathology of the guinea pigs and human case revealed the following conclusions: Type I hearing loss may be caused by toxic labyrinthitis secondary to the radiation otitis media or by the aseptic labyrinthitis as the result of hyperemia and increased permeability of the irradiated blood vessels in the cochlea. Type II hearing loss may be caused by the late rediation response of the cochleal blood vessels i.e. by the vasculitis which gives rise to obliteration of the vascular lumen and affects the blood supply of the hair cells.

  3. Clinical and experimental studies on the sensorineural hearing loss caused by irradiation

    International Nuclear Information System (INIS)

    Yamamoto, Matsunori

    1979-01-01

    In our study, 43 patients whose ears were presumably irradiated, were examined periodically before and after the irradiation; 10 were found to be affected by the sensorineural hearing loss, and these 10 were classified into two types. Type I; Patients of this group had a hearing impairment during or soon after irradiation and usually revealed marked deterioration of bone conduction in high frequencies accompanied by worsening of air conduction in low frequencies. The impaired hearing of this group soon recovered to the pre-treatment level. Type II; Patients of this group developed a slow progressive sensorineural hearing loss. They noticed the deafness with tinnitus several months after irradiation. To clarify the mechanism of the sensorineural hearing loss, histopathological investigations were done using nembutal anesthetized guinea pigs which were irradiated in the confined ear region unilaterally. We also examined histopathologically one human temporal bone belonging to a patient who had been irradiated for middle ear carcinoma. The histopathology of the guinea pigs and human case revealed the following conclusions: Type I hearing loss may be caused by toxic labyrinthitis secondary to the radiation otitis media or by the aseptic labyrinthitis as the result of hyperemia and increased permeability of the irradiated blood vessels in the cochlea. Type II hearing loss may be caused by the late rediation response of the cochleal blood vessels i.e. by the vasculitis which gives rise to obliteration of the vascular lumen and affects the blood supply of the hair cells. (author)

  4. Viral Causes of Hearing Loss: A Review for Hearing Health Professionals

    Science.gov (United States)

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

    A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. PMID:25080364

  5. Economic Loss Caused by GMOs in Denmark

    DEFF Research Database (Denmark)

    Ulfbeck, Vibe Garf

    2008-01-01

    This book presents how European jurisdictions currently respond to economic losses caused by the admixture of gentically modified crops with conventional or organic crops and what alternatives there are from a comparative perspective. Country reports from most European countries are complemented ...

  6. GABAergic Neuron-Specific Loss of Ube3a Causes Angelman Syndrome-Like EEG Abnormalities and Enhances Seizure Susceptibility.

    Science.gov (United States)

    Judson, Matthew C; Wallace, Michael L; Sidorov, Michael S; Burette, Alain C; Gu, Bin; van Woerden, Geeske M; King, Ian F; Han, Ji Eun; Zylka, Mark J; Elgersma, Ype; Weinberg, Richard J; Philpot, Benjamin D

    2016-04-06

    Loss of maternal UBE3A causes Angelman syndrome (AS), a neurodevelopmental disorder associated with severe epilepsy. We previously implicated GABAergic deficits onto layer (L) 2/3 pyramidal neurons in the pathogenesis of neocortical hyperexcitability, and perhaps epilepsy, in AS model mice. Here we investigate consequences of selective Ube3a loss from either GABAergic or glutamatergic neurons, focusing on the development of hyperexcitability within L2/3 neocortex and in broader circuit and behavioral contexts. We find that GABAergic Ube3a loss causes AS-like increases in neocortical EEG delta power, enhances seizure susceptibility, and leads to presynaptic accumulation of clathrin-coated vesicles (CCVs)-all without decreasing GABAergic inhibition onto L2/3 pyramidal neurons. Conversely, glutamatergic Ube3a loss fails to yield EEG abnormalities, seizures, or associated CCV phenotypes, despite impairing tonic inhibition onto L2/3 pyramidal neurons. These results substantiate GABAergic Ube3a loss as the principal cause of circuit hyperexcitability in AS mice, lending insight into ictogenic mechanisms in AS. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Heat losses in power boilers caused by thermal bridges

    Directory of Open Access Journals (Sweden)

    Kocot Monika

    2017-01-01

    Full Text Available In this article the analysis of heat losses caused by thermal bridges that occur in the steam boiler OP-140 is presented. Identification of these bridges were conducted with use of thermographic camera. Heat losses were evaluated based on methodology of VDI 4610 standard, but instead of its simplified equations, criterial equations based on Nusselt number were used. Obtained values of annual heat losses and heat flux density corresponding to the fully insulated boiler surfaces were compared to heat losses generated by thermal bridges located in the same areas. The emphasis is put on the role of industrial insulation in heat losses reduction.

  8. Heat stress causes substantial labour productivity loss in Australia

    Science.gov (United States)

    Zander, Kerstin K.; Botzen, Wouter J. W.; Oppermann, Elspeth; Kjellstrom, Tord; Garnett, Stephen T.

    2015-07-01

    Heat stress at the workplace is an occupational health hazard that reduces labour productivity. Assessment of productivity loss resulting from climate change has so far been based on physiological models of heat exposure. These models suggest productivity may decrease by 11-27% by 2080 in hot regions such as Asia and the Caribbean, and globally by up to 20% in hot months by 2050. Using an approach derived from health economics, we describe self-reported estimates of work absenteeism and reductions in work performance caused by heat in Australia during 2013/2014. We found that the annual costs were US$655 per person across a representative sample of 1,726 employed Australians. This represents an annual economic burden of around US$6.2 billion (95% CI: 5.2-7.3 billion) for the Australian workforce. This amounts to 0.33 to 0.47% of Australia’s GDP. Although this was a period when many Australians experienced what is at present considered exceptional heat, our results suggest that adaptation measures to reduce heat effects should be adopted widely if severe economic impacts from labour productivity loss are to be avoided if heat waves become as frequent as predicted.

  9. Blackleg (Leptosphaeria maculans) Severity and Yield Loss in Canola in Alberta, Canada

    Science.gov (United States)

    Hwang, Sheau-Fang; Strelkov, Stephen E.; Peng, Gary; Ahmed, Hafiz; Zhou, Qixing; Turnbull, George

    2016-01-01

    Blackleg, caused by Leptosphaeria maculans, is an important disease of oilseed rape (Brassica napus L.) in Canada and throughout the world. Severe epidemics of blackleg can result in significant yield losses. Understanding disease-yield relationships is a prerequisite for measuring the agronomic efficacy and economic benefits of control methods. Field experiments were conducted in 2013, 2014, and 2015 to determine the relationship between blackleg disease severity and yield in a susceptible cultivar and in moderately resistant to resistant canola hybrids. Disease severity was lower, and seed yield was 120%–128% greater, in the moderately resistant to resistant hybrids compared with the susceptible cultivar. Regression analysis showed that pod number and seed yield declined linearly as blackleg severity increased. Seed yield per plant decreased by 1.8 g for each unit increase in disease severity, corresponding to a decline in yield of 17.2% for each unit increase in disease severity. Pyraclostrobin fungicide reduced disease severity in all site-years and increased yield. These results show that the reduction of blackleg in canola crops substantially improves yields. PMID:27447676

  10. Blackleg (Leptosphaeria maculans Severity and Yield Loss in Canola in Alberta, Canada

    Directory of Open Access Journals (Sweden)

    Sheau-Fang Hwang

    2016-07-01

    Full Text Available Blackleg, caused by Leptosphaeria maculans, is an important disease of oilseed rape (Brassica napus L. in Canada and throughout the world. Severe epidemics of blackleg can result in significant yield losses. Understanding disease-yield relationships is a prerequisite for measuring the agronomic efficacy and economic benefits of control methods. Field experiments were conducted in 2013, 2014, and 2015 to determine the relationship between blackleg disease severity and yield in a susceptible cultivar and in moderately resistant to resistant canola hybrids. Disease severity was lower, and seed yield was 120%–128% greater, in the moderately resistant to resistant hybrids compared with the susceptible cultivar. Regression analysis showed that pod number and seed yield declined linearly as blackleg severity increased. Seed yield per plant decreased by 1.8 g for each unit increase in disease severity, corresponding to a decline in yield of 17.2% for each unit increase in disease severity. Pyraclostrobin fungicide reduced disease severity in all site-years and increased yield. These results show that the reduction of blackleg in canola crops substantially improves yields.

  11. Early Pregnancy Loss

    Science.gov (United States)

    ... go on to have successful pregnancies. Repeated pregnancy losses are rare. Testing and evaluation can be done ... find a cause if you have several pregnancy losses. Even if no cause is found, most couples ...

  12. Study of the thermal noise caused by inhomogeneously distributed loss

    CERN Document Server

    Yamamoto, K; Ando, M; Kawabe, K; Tsubono, K

    2002-01-01

    The normal modal expansion is the most frequently used method to estimate the thermal noise of interferometric gravitational wave detectors. However, the method does not agree with new estimation methods, direct approaches, when the loss is distributed inhomogeneously. We have checked the modal expansion and direct approaches experimentally using a mechanical oscillator, such as a mirror. The experiments showed that the modal expansion is invalid. On the other hand, the measured spectra are consistent with the direct approaches. We calculated the thermal noise of a real mirror with inhomogeneous loss using the direct approaches. This calculation showed that the thermal motions caused by loss in the reflective coating and at coil-magnet actuators are comparable with the sensitivity goals of future gravitational wave detector projects. In addition, according to our calculation, a mechanical loss may cause much larger or much smaller thermal motion than is expected in modal expansion, depending on the loss distr...

  13. ECONOMIC LOSSES CAUSED BY TRAUMATIC BRAIN INJURY IN CHILDREN

    Directory of Open Access Journals (Sweden)

    S. A. Valiulina

    2015-01-01

    Full Text Available Background: Currently, analyzing the economic losses caused by health problems in population is of particular importance since it stipulates calculations of the volumes invested in healthcare systems in order to improve population’s health. Objective: The aim of our study was to find out economic losses caused by traumatic brain injury (TBI in children. Methods: The given work has utilized governmental statistical reports for Russia, for federal regions as well as for individual subjects. Direct medical expenses (medical services and indirect expenses (losses due to a temporary disability of parents having a sick child were calculated both in general and per patient. Results: Among all the direct medical costs of treatment of children with TBI inpatient care costs account for 85%. In the Central and Volga Federal District accounted for half of nationwide spending in general, brain injury and to provide certain kinds of healthcare. The structure of Russian costs as a result of the incidence of TBI children Moscow accounts for 20%. In Moscow, the cost of treating cases of traumatic brain injury in children is 3.2 times higher than the average for Russia. The resulting calculations of the value of health care costs attributable to a case of child head injury, behind the cost of treatment of the case of a child with head trauma, calculated according to the standards of Russia and the territories. This difference in the whole RF is 23%. Conclusion: The obtained findings have shown that in 2010 in Russia the magnitude of losses caused by TBI incidence in children amounted to 3 billion roubles or 0.008% of the gross product 1.2 billion roubles of which were direct expenses. However, this figure is considerably lower of the real amount; it becomes evident after the analysis of direct medical expenses per one case of pediatric TBI. Our calculations have shown that in Russia and in its regions the amount of expenses per one TBI patient is a quarter less

  14. SIX2 haploinsufficiency causes conductive hearing loss with ptosis in humans.

    Science.gov (United States)

    Guan, Jing; Wang, Dayong; Cao, Wenjian; Zhao, Yali; Du, Renqian; Yuan, Hu; Liu, Qiong; Lan, Lan; Zong, Liang; Yang, Ju; Yin, Zifang; Han, Bing; Zhang, Feng; Wang, Qiuju

    2016-11-01

    The ossicles represent one of the most fundamental morphological features in evolutionary biology of the mammalians. The mobile ossicular morphology abnormalities result in the severe conductive hearing loss. Development and patterning of the middle ear malformation depend on genetic and environmental causes. However, the genetic basis for the risk of congenital ossicle malformation is poorly understood. We show here nine affected individuals in a Chinese pedigree who had bilateral conductive hearing loss with ptosis. We performed whole-genome sequencing and array comparative genomic hybridization (CGH) analysis on DNA samples from the Chinese pedigree. We confirmed the presence of a novel 60 kb heterozygous deletion in size, encompassing SIX2 in our family. Mutation screening in 169 sporadic cases with external ear and middle ear malformations identified no pathogenic variant or polymorphism. We suggest SIX2 haploinsufficiency as a potential congenital factor could be attributed to developmental malformation of the middle ear ossicles and upper eyelid. To the best of our knowledge, this is the first report to provide a description of copy number variation in the SIX2 gene resulting in syndromic conductive hearing loss.

  15. Weeds Cause Losses in Field Crops through Allelopathy

    Directory of Open Access Journals (Sweden)

    Ali ZOHAIB

    2016-03-01

    Full Text Available A large number of weeds are known to be associated with crops and causing economic losses. Weeds interfere with crops through competition and allelopathy. They produce secondary metabolites known as allelochemicals, which belong to numerous chemical classes such as phenolics, alkaloids, fatty acids, indoles, terpens etc. However, phenolics are the predominant class of allelochemicals. The allelochemicals release from weed plants takes place through leaf leachates, decomposition of plant residues, volatilization and root exudates. Weeds leave huge quantities of their residues in field and affect the associated, as well as succeeding crops, in various cropping systems. Liberation of allelochemicals from weeds affects the germination, stand establishment, growth, yield and physiology of crop plants. They cause substantial reduction in germination and growth of the crop plants by altering various physiological processes such as enzyme activity, protein synthesis, photosynthesis, respiration, cell division and enlargement, which ultimately leads to a significant reduction in crop yield. In crux, allelopathic weeds represent a potential threat for crop plants and cause economic losses.

  16. Loss of binocular vision as direct cause for misrouting of temporal retinal fibers in albinism.

    Science.gov (United States)

    Banihani, Saleh M

    2015-10-01

    In humans, the nasal retina projects to the contralateral hemisphere, whereas the temporal retina projects ipsilaterally. The nasotemporal line that divides the retina into crossed and uncrossed parts coincides with the vertical meridian through the fovea. This normal projection of the retina is severely altered in albinism, in which the nasotemporal line shifted into the temporal retina with temporal retinal fibers cross the midline at the optic chiasm. This study proposes the loss of binocular vision as direct cause for misrouting of temporal retinal fibers and shifting of the nasotemporal line temporally in albinism. It is supported by many observations that clearly indicate that loss of binocular vision causes uncrossed retinal fibers to cross the midline. This hypothesis may alert scientists and clinicians to find ways to prevent or minimize the loss of binocular vision that may occur in some diseases such as albinism and early squint. Hopefully, this will minimize the misrouting of temporal fibers and improve vision in such diseases. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Differing causes of pregnancy loss in type 1 and type 2 diabetes.

    Science.gov (United States)

    Cundy, Tim; Gamble, Greg; Neale, Leonie; Elder, Rose; McPherson, Paul; Henley, Patrick; Rowan, Janet

    2007-10-01

    Women with type 2 and type 1 diabetes have differing risk factors for pregnancy loss. We compared the rates and causes of pregnancy loss in women with type 1 and type 2 diabetes. We utilized prospectively collected data on all pregnancies in a 20-year period (1986-2005) from a single center with a high prevalence of type 2 diabetes. Pregnancy losses included terminations for medical reasons and deaths up to 1 month postpartum but not spontaneous pregnancy losses pregnancies in women with known diabetes (330 with type 1 and 540 with type 2 diabetes) and 325 in women with diabetes diagnosed in pregnancy but persisting postpartum (97% type 2 diabetes). The rate of pregnancy loss was similar in type 1 and type 2 diabetes (2.6 vs. 3.7%, P = 0.39), but the causes of pregnancy loss differed. In type 1 diabetes >75% were attributable to major congenital anomalies or prematurity; in type 2 diabetes >75% were attributable to stillbirth or chorioamnionitis (P = 0.017). Women with type 2 and type 1 diabetes had similar A1C at presentation and near term, but the former were older (P causes of pregnancy loss in women with type 1 and type 2 diabetes. The higher rates of stillbirth in women with type 2 diabetes, suggest that other features, such as obesity, contribute significantly to pregnancy losses.

  18. Megadolicho basilar artery as a cause of asymmetrical sensorineural hearing loss - case report

    Directory of Open Access Journals (Sweden)

    Melo, Antonio Antunes

    2011-07-01

    Full Text Available Introduction: At the differentiated diagnosis of asymmetrical sensorineural hearing losses, vascular disorders are present, one of which is megadolicho basilar artery. This disease is generally asymptomatic, and when symptoms are found, they can be caused by a compression or ischemia. Clinically, sensorineural hearing loss, tinnitus, headache, facial hypoesthesia, trigeminal neuralgia, vertigo, diplopia and facial palsy, among others, are likely to occur. The image examination of choice for its diagnosis is nuclear magnetic resonance. The megadolicho basilar artery therapy can be surgical or conservative, according to the associated findings. A multidisciplinary approach, including a neurologist, neurosurgeon and an otorhinolaryngologist is recommended for a proper administration of the case. Objective: Report the case of a patient with asymmetrical sensorineural hearing loss, diagnosed of megadolicho basilar artery. Case report: JBS, 57-year-old white male with a history of asymmetrical sensorineural hearing loss and bilateral whistle-like tinnitus for several years. The otorhinolaryngologic evaluation, including otoscopy, anterior rhinoscopy and oral pharynx, was normal. Final Comments: The treatment consisted in following up with the patient, controlling the tinnitus by drugs and using an individual sound amplification apparatus on the left ear.

  19. Loss of Ikbkap Causes Slow, Progressive Retinal Degeneration in a Mouse Model of Familial Dysautonomia.

    Science.gov (United States)

    Ueki, Yumi; Ramirez, Grisela; Salcedo, Ernesto; Stabio, Maureen E; Lefcort, Frances

    2016-01-01

    Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy that is caused by a mutation in the gene for inhibitor of kappa B kinase complex-associated protein ( IKBKAP ). Although FD patients suffer from multiple neuropathies, a major debilitation that affects their quality of life is progressive blindness. To determine the requirement for Ikbkap in the developing and adult retina, we generated Ikbkap conditional knockout (CKO) mice using a TUBA1a promoter-Cre ( Tα1-Cre ). In the retina, Tα1-Cre expression is detected predominantly in retinal ganglion cells (RGCs). At 6 months, significant loss of RGCs had occurred in the CKO retinas, with the greatest loss in the temporal retina, which is the same spatial phenotype observed in FD, Leber hereditary optic neuropathy, and dominant optic atrophy. Interestingly, the melanopsin-positive RGCs were resistant to degeneration. By 9 months, signs of photoreceptor degeneration were observed, which later progressed to panretinal degeneration, including RGC and photoreceptor loss, optic nerve thinning, Müller glial activation, and disruption of layers. Taking these results together, we conclude that although Ikbkap is not required for normal development of RGCs, its loss causes a slow, progressive RGC degeneration most severely in the temporal retina, which is later followed by indirect photoreceptor loss and complete retinal disorganization. This mouse model of FD is not only useful for identifying the mechanisms mediating retinal degeneration, but also provides a model system in which to attempt to test therapeutics that may mitigate the loss of vision in FD patients.

  20. An unusual cause of conductive hearing loss: bilateral complete meatal obstruction following otoplasty.

    Science.gov (United States)

    Toplu, Yuksel; Toplu, Sibel Altunisik; Sapmaz, Emrah; Deliktas, Hacim

    2014-01-01

    There are many causes of conductive hearing loss. Otoplasty is a commonly performed cosmetic surgery to correction for prominent ear. We discussed an unusual cause of conductive hearing loss, bilateral complete meatal obstruction following otoplasty, in this article. This complication was probably due to unsuitable placement of the Furnas sutures. In the literature, as a cause of conductive hearing loss, unilateral complete meatal obstruction has been described rarely, but bilateral complete obstruction has not been defined. Hearing loss recovered completely after revision surgery. Correct diagnosis, accurate preoperative or perioperative examination, choice of the proper technique and surgeon experience are essential for avoiding inadequate results or complications of otoplasty. The surgeon should be aware of the cartilage elasticity and suture techniques to avoid this complication.

  1. Prediction of yield losses in wheat (triticum aestivum l.) caused by yellow rust in relation to epidemiological factors in Faisalabad

    International Nuclear Information System (INIS)

    Ahmad, S.; Afzal, M.; Noorka, I.R.; Iqbal, Z.; Akhtar, N.; Iiftikhar, Y.; Kamran, M.

    2010-01-01

    Thirty six genotypes were screened against yellow rust to check their level of susceptibility or resistance. Among 36 genotypes screened against yellow rust, 18 were susceptible, 6 were moderately susceptible to susceptible, 7 were moderately resistant to moderately susceptible and 5 genotypes remained resistant. Yield losses were predicted in wheat on the basis of varying level of yellow rust severities. It was observed that susceptible genotypes showed higher yield losses as compared to resistant genotypes. Maximum severity of 90% of yellow rust resulted in 54% to 55% calculated and predicted losses, respectively. While 40, 50, 60 and 70% disease severity of yellow rust caused 35-34%, 38-37%, 42-40% and 46-47% calculated and predicted losses, respectively. However, the decline in losses was observed as the genotypes changed their reaction from susceptible to moderate susceptible. Similarly, losses were diminished as the varieties/lines showed moderate resistant reaction from moderate susceptible. Minimum temperature and relative humidity remained positively correlated while the maximum temperature showed negative correlation with stripe rust severity. With the increase of minimum temperature and relative humidity a rise up in stripe rust infection was seen while as the maximum temperature increased stripe rust infection decreased on different genotypes. It may be concluded from the study that environmental factors played major role in the spread of the disease which result in yield losses. (author)

  2. Weight loss alters severity of individual nocturnal respiratory events depending on sleeping position

    International Nuclear Information System (INIS)

    Kulkas, A; Leppänen, T; Tiihonen, P; Mervaala, E; Töyräs, J; Sahlman, J; Seppä, J; Kokkarinen, J; Randell, J; Tuomilehto, H

    2014-01-01

    Weight loss is an effective treatment for obstructive sleep apnea (OSA). The mechanisms of how weight loss affects nocturnal breathing are not fully understood. The severity of OSA is currently estimated by the number of respiratory events per hour of sleep (i.e. apnea-hypopnea-index, AHI). AHI neglects duration and morphology of individual respiratory events, which describe the severity of individual events. In the current paper, we investigate the novel Adjusted-AHI parameter (incorporating individual event severity) and AHI after weight loss in relation to sleeping position. It was hypothesised that there are positional differences in individual event severity changes during weight loss. Altogether, 32 successful (> 5% of weight) and 34 unsuccessful weight loss patients at baseline and after 1 year follow-up were analysed. The results revealed that individual respiratory event severity was reduced differently in supine and non-supine positions during weight loss. During weight loss, AHI was reduced by 54% (p = 0.004) and 74% (p < 0.001), while Adjusted-AHI was reduced by 14% (p = 0.454) and 48% (p = 0.003) in supine and non-supine positions, respectively. In conclusion, the severity of individual respiratory events decreased more in the non-supine position. The novel Adjusted-AHI parameter takes these changes into account and might therefore contribute additional information to the planning of treatment of OSA patients. (paper)

  3. Profound bilateral visual loss after hysterectomy indicated for severe postpartum haemorrhage

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Zibrandtsen, Nathalie; Larsen, Michael

    2014-01-01

    We present a case of a patient with bilateral posterior ischaemic optic neuropathy in the previously unreported setting of hysterectomy indicated for severe postpartum haemorrhage. The diagnosis was based on clinical and paraclinical examinations, including MRI of the head, electroretinography (ERG....... The diagnosis of perioperative posterior ischaemic optic neuropathy is mostly a clinical diagnosis. However, MRI plays a major role in excluding other causes of visual loss, and VEP, ERG and OCT are valuable supplemental diagnostic tools.......) and visual evoked potentials (VEP) testing. During 1 year of follow-up, repeated optical coherence tomography (OCT) scans demonstrated optic disc atrophy, which was interpreted as a sign of direct retrograde ganglion cell degeneration after ischaemic damage to the retrolaminar part of the optic nerves...

  4. A pilot study of sensory feedback by transcutaneous electrical nerve stimulation to improve manipulation deficit caused by severe sensory loss after stroke.

    Science.gov (United States)

    Kita, Kahori; Otaka, Yohei; Takeda, Kotaro; Sakata, Sachiko; Ushiba, Junichi; Kondo, Kunitsugu; Liu, Meigen; Osu, Rieko

    2013-06-13

    Sensory disturbance is common following stroke and can exacerbate functional deficits, even in patients with relatively good motor function. In particular, loss of appropriate sensory feedback in severe sensory loss impairs manipulation capability. We hypothesized that task-oriented training with sensory feedback assistance would improve manipulation capability even without sensory pathway recovery. We developed a system that provides sensory feedback by transcutaneous electrical nerve stimulation (SENS) for patients with sensory loss, and investigated the feasibility of the system in a stroke patient with severe sensory impairment and mild motor deficit. The electrical current was modulated by the force exerted by the fingertips so as to allow the patient to identify the intensity. The patient had severe sensory loss due to a right thalamic hemorrhage suffered 27 months prior to participation in the study. The patient first practiced a cylindrical grasp task with SENS for 1 hour daily over 29 days. Pressure information from the affected thumb was fed back to the unaffected shoulder. The same patient practiced a tip pinch task with SENS for 1 hour daily over 4 days. Pressure information from the affected thumb and index finger was fed back to the unaffected and affected shoulders, respectively. We assessed the feasibility of SENS and examined the improvement of manipulation capability after training with SENS. The fluctuation in fingertip force during the cylindrical grasp task gradually decreased as the training progressed. The patient was able to maintain a stable grip force after training, even without SENS. Pressure exerted by the tip pinch of the affected hand was unstable before intervention with SENS compared with that of the unaffected hand. However, they were similar to each other immediately after SENS was initiated, suggesting that the somatosensory information improved tip pinch performance. The patient's manipulation capability assessed by the Box

  5. Severe Hemorrhage from the Umbilical Cord at Birth: A Preventable Cause of Neonatal Shock

    Directory of Open Access Journals (Sweden)

    Neetu Singh

    2013-01-01

    Full Text Available Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe that umbilical cord bleeding from errors in cord clamping could be an important cause of acute blood loss in the delivery room and that it may result in significant clinical morbidity, especially in extremely premature infants.

  6. Severe hemorrhage from the umbilical cord at birth: a preventable cause of neonatal shock.

    Science.gov (United States)

    Singh, Neetu; Suresh, Gautham

    2013-01-01

    Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe that umbilical cord bleeding from errors in cord clamping could be an important cause of acute blood loss in the delivery room and that it may result in significant clinical morbidity, especially in extremely premature infants.

  7. FEMA Hazard Mitigation Assistance Severe Repetitive Loss (SRL) Data

    Data.gov (United States)

    Department of Homeland Security — This dataset contains closed and obligated projects funded under the following Hazard Mitigation Assistance (HMA) grant programs: Severe Repetitive Loss (SRL). The...

  8. Myopic loss aversion: Potential causes of replication failures

    Directory of Open Access Journals (Sweden)

    Alexander Klos

    2013-09-01

    Full Text Available This paper presents two studies on narrow bracketing and myopic loss aversion. The first study shows that the tendency to segregate multiple gambles is eliminated if subjects face a certainty equivalent or a probability equivalent task instead of a binary choice. The second study argues that the behavioral differences previously attributed entirely to myopic loss aversion are partly because long-term return properties are simply easier to grasp if the return information is already provided in the form of long-term returns rather than one-year returns. Both results may be related to recent failures to replicate myopic loss aversion. When the choice situation is structured in such a way that it draws respondents' attention to the final outcome distribution and/or if severe misestimations of long-term returns based on short-term return information are unlikely, behavioral differences consistent with myopic loss aversion are less likely to be observed.

  9. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice

    Directory of Open Access Journals (Sweden)

    Yanfei Wang

    2017-12-01

    Full Text Available Calcium and integrin-binding protein 2 (CIB2 belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR-associated Cas9 nuclease (CRISPR/Cas9 genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  10. Celiac disease causing severe osteomalacia: an association still present in Morocco!

    Science.gov (United States)

    Tahiri, Latifa; Azzouzi, Hamida; Squalli, Ghita; Abourazzak, Fatimazahra; Harzy, Taoufik

    2014-01-01

    Celiac disease (CD), a malabsorption syndrome caused by hypersensitivity to gliadin fraction of gluten. CD can manifest with classic symptoms; however, significant myopathy and multiple fractures are rarely the predominant presentation of untreated celiac disease. Osteomalacia complicating celiac disease had become more and more rare. We describe here a case of osteomalacia secondary to a longstanding untreated celiac disease. This patient complained about progressive bone and muscular pain, weakness, fractures and skeletal deformities. Radiological and laboratory findings were all in favor of severe osteomalacia. Improvement of patient's weakness and laboratory abnormalities was obvious after treatment with gluten free diet, vitamin D, calcium and iron. This case affirms that chronic untreated celiac disease, can lead to an important bone loss and irreversible complications like skeletal deformities.

  11. Pinpointing Synaptic Loss Caused by Alzheimer?s Disease with fMRI

    OpenAIRE

    Brickman, Adam M.; Small, Scott A.; Fleisher, Adam

    2009-01-01

    During its earliest stage, before cell loss and independent of amyloid plaques and neurofibrillary tangles, Alzheimer's disease (AD) causes synaptic loss affecting the basal functional properties of neurons. In principle, synaptic loss can be detected by measuring AD-induced changes in basal function, or by measuring stimulus-evoked responses on top of basal changes. Functional magnetic resonance imaging (fMRI) is sensitive to both basal changes and evoked-responses, and there are therefore t...

  12. The judged seriousness of an environmental loss is a matter of what caused it

    Science.gov (United States)

    Thomas C. Brown; George L. Peterson; R. Marc Brodersen; Valerie Ford; Paul A. Bell

    2005-01-01

    Environmental losses, each described along with its cause, were judged for seriousness. Four types of cause were studied: illegal behavior, carelessness, economic and population growth, and natural events. Identical environmental losses (e.g., of a herd of elk or a large stand of trees) were considered most serious when caused by illegal behavior or carelessness, and...

  13. Bilateral External Auditory Exostoses Causing Conductive Hearing Loss: A Case Report and Literature Review of the Surfer's Ear.

    Science.gov (United States)

    Barbon, Dennis A; Hegde, Rahul; Li, Shuo; Abdelbaki, Ahmed; Bajaj, Divyansh

    2017-10-30

    In patients with repeated exposure to cold water, such as cold water surfers and kayakers, the reactive exostoses can occur in the external auditory canal. The external auditory canal exostoses are multiple, benign bony growths. They can cause external auditory canal stenosis, leading to repeated otitis externa and potentially conductive hearing loss. It is vital to consider this entity in susceptible patients who report hearing loss, as timely intervention such as proper ear protection equipment can lower the risk of developing severe external auditory canal exostoses. We present a case of a 42-year-old male, cold water surfer with conductive hearing loss and bilateral external auditory canal (EAC) stenosis demonstrated on the computed tomography.

  14. Vitreous loss-causes, associations, and outcomes: Eight-year analysis in Melaka Hospital

    Directory of Open Access Journals (Sweden)

    Thanigasalam Thevi

    2018-01-01

    CONCLUSION: Vitreous loss occurred in almost all types of cataract surgeries, especially by junior surgeons, among those aged <40 years and significantly caused poor visual outcome compared to other complications. Pseudoexfoliation had higher occurrence of vitreous loss. Vitreous loss patients had impaired/poor visual outcome due to preexisting comorbidity and astigmatism. Patients at risk and junior surgeons should be closely monitored to improve outcomes. Further studies need to be done to see why and when the vitreous loss occurred.

  15. A novel mutation in the EDAR gene causes severe autosomal recessive hypohidrotic ectodermal dysplasia.

    Science.gov (United States)

    Henningsen, Emil; Svendsen, Mathias Tiedemann; Lildballe, Dorte Launholt; Jensen, Peter Kjestrup Axel

    2014-08-01

    We report on a 2-year-old girl presenting with a severe form of hypohidrotic ectodermal dysplasia (HED). The patient presented with hypotrichosis, anodontia, hypohidrosis, frontal bossing, prominent lips and ears, dry, pale skin, and dermatitis. The patient had chronic rhinitis with malodorous nasal discharge. The girl was the second born child of first-cousin immigrants from Northern Iraq. A novel homozygous mutation (c.84delC) in the EDAR gene was identified. This mutation most likely causes a frameshift in the protein product (p.S29fs*74). This results in abolition of all ectodysplasin-mediated NF-kB signalling. This complete loss-of-function mutation likely accounts for the severe clinical abnormalities in ectodermal structures in the described patient. © 2014 Wiley Periodicals, Inc.

  16. The disease that caused weight loss in King David the Great.

    Science.gov (United States)

    Ben-Noun, Liubov Louba

    2004-02-01

    Older people have suffered from loss of weight since the dawn of history. This research is unique in character, as it combines contemporary medical knowledge with the presentation of a case taken from Ancient History. To analyze from a modern perspective the biblical description of a geriatric patient who suffered from weight loss. Biblical texts associated with the aged were examined and passages relating to geriatric patients who suffered from loss of weight were closely studied. This study is based on the evaluation of the biblical passages, and not on the interpretations of various rabbis and scholars. Passages such as: ". I forget to eat my bread" and "My knees are weak through fasting; and my flesh failed of fatness" and ". my bones cleave to my skin" indicate anorexia, fasting, extreme loss of weight, and subsequent cachexia. Among the numerous causes associated with weight loss, malignancy, social problems such as loneliness, social isolation and neglect by others, and psychological causes including depressed mood were most likely responsible. With regard to malignancy, it seems that the King was affected by primary carcinoma of the prostate or kidney with subsequent metastases to bones. This report demonstrates that the roots of geriatric medicine can be traced to biblical times.

  17. Science 101: What Causes Severe Thunderstorms and Tornadoes?

    Science.gov (United States)

    Robertson, Bill

    2016-01-01

    What causes severe thunderstorms and tornadoes? Tornadoes, often accompanied by severe thunderstorms and hail, form in pretty much the same way as severe thunderstorms. In the continental United States, tornadoes usually form in spring and summer, when warm, humid air from the Gulf of Mexico moves across the continent from southeast to northwest…

  18. Mutations in plasmalemma vesicle-associated protein cause severe syndromic protein-losing enteropathy.

    Science.gov (United States)

    Broekaert, Ilse Julia; Becker, Kerstin; Gottschalk, Ingo; Körber, Friederike; Dötsch, Jörg; Thiele, Holger; Altmüller, Janine; Nürnberg, Peter; Hünseler, Christoph; Cirak, Sebahattin

    2018-04-16

    Protein-losing enteropathy (PLE) is characterised by gastrointestinal protein leakage due to loss of mucosal integrity or lymphatic abnormalities. PLE can manifest as congenital diarrhoea and should be differentiated from other congenital diarrhoeal disorders. Primary PLEs are genetically heterogeneous and the underlying genetic defects are currently emerging. We report an infant with fatal PLE for whom we aimed to uncover the underlying pathogenic mutation. We performed whole exome sequencing (WES) for the index patient. Variants were classified based on the American College of Medical Genetics and Genomics guidelines. WES results and our detailed clinical description of the patient were compared with the literature. We discovered a novel homozygous stop mutation (c.988C>T, p.Q330*) in the Plasmalemma Vesicle-Associated Protein ( PLVAP ) gene in a newborn with fatal PLE, facial dysmorphism, and renal, ocular and cardiac anomalies. The Q330* mutation is predicted to result in complete loss of PLVAP protein expression leading to deletion of the diaphragms of endothelial fenestrae, resulting in plasma protein extravasation and PLE. Recently, another single homozygous stop mutation in PLVAP causing lethal PLE in an infant was reported. Our findings validate PLVAP mutations as a cause of syndromic PLE. Prenatal anomalies, severe PLE and syndromic features may guide the diagnosis of this rare disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Illness Severity and Work Productivity Loss Among Working Adults With Medically Attended Acute Respiratory Illnesses: US Influenza Vaccine Effectiveness Network 2012-2013.

    Science.gov (United States)

    Petrie, Joshua G; Cheng, Caroline; Malosh, Ryan E; VanWormer, Jeffrey J; Flannery, Brendan; Zimmerman, Richard K; Gaglani, Manjusha; Jackson, Michael L; King, Jennifer P; Nowalk, Mary Patricia; Benoit, Joyce; Robertson, Anne; Thaker, Swathi N; Monto, Arnold S; Ohmit, Suzanne E

    2016-02-15

    Influenza causes significant morbidity and mortality, with considerable economic costs, including lost work productivity. Influenza vaccines may reduce the economic burden through primary prevention of influenza and reduction in illness severity. We examined illness severity and work productivity loss among working adults with medically attended acute respiratory illnesses and compared outcomes for subjects with and without laboratory-confirmed influenza and by influenza vaccination status among subjects with influenza during the 2012-2013 influenza season. Illnesses laboratory-confirmed as influenza (ie, cases) were subjectively assessed as more severe than illnesses not caused by influenza (ie, noncases) based on multiple measures, including current health status at study enrollment (≤7 days from illness onset) and current activity and sleep quality status relative to usual. Influenza cases reported missing 45% more work hours (20.5 vs 15.0; P productivity as impeded to a greater degree (6.0 vs 5.4; P productivity loss were noted for vaccinated subjects. Influenza illnesses were more severe and resulted in more missed work hours and productivity loss than illnesses not confirmed as influenza. Modest reductions in illness severity for vaccinated cases were observed. These findings highlight the burden of influenza illnesses and illustrate the importance of laboratory confirmation of influenza outcomes in evaluations of vaccine effectiveness. © The Author 2015. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.

  20. Neonatal severe hyperparathyroidism caused by homozygous mutation in CASR: A rare cause of life-threatening hypercalcemia.

    Science.gov (United States)

    Murphy, Heidi; Patrick, Jessica; Báez-Irizarry, Eileen; Lacassie, Yves; Gómez, Ricardo; Vargas, Alfonso; Barkemeyer, Brian; Kanotra, Sohit; Zambrano, Regina M

    2016-04-01

    Neonatal severe hyperparathyroidism (NSHPT) is a rare, life-threatening condition that presents with severe hypercalcemia, hyperparathyroidism, and osteopenia in the newborn period. Treatment of NSHPT traditionally includes hydration and bisphosphonates; however newer calcimimetic agents, such as cinacalcet, are now being utilized to prevent or delay parathyroidectomy which is technically difficult in the newborn. Medical treatment success is related to calcium sensing receptor (CaSR) genotype. We report a 4-day-old infant who presented with hyperbilirubinemia, poor feeding, weight loss, severe hypotonia and was ultimately diagnosed with NSHPT. The patient's total serum calcium level of 36.8 mg/dL (reference range: 8.5-10.4 mg/dL) is, to our knowledge, the highest ever documented in this setting. Exome data previously obtained on the infant's parents was re-analyzed demonstrating bi-parental heterozygosity for a mutation of the CASR gene: c.206G > A, and Sanger sequencing data confirmed the patient was a homozygote for the same mutation. Though a patient with the same CaSR gene mutation described here has responded to cinacalcet, our patient did not respond and required parathyroidectomy. Though this case has previously been published as a surgical case report, a full report of the medical management and underlying genetic etiology is warranted; this case underscores the importance of disclosing bi-parental heterozygosity for a gene causing severe neonatal disease particularly when treatment is available and illustrates the need for further in vitro studies of this CaSR mutation. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  1. Energy losses in the D0 β solenoid cryostat caused by current changes

    International Nuclear Information System (INIS)

    Visser, A.T.

    1993-11-01

    The proposed D0 β solenoid is a superconducting solenoid mounted inside an aluminum tube which supports the solenoid winding over it's full length. This aluminum support tube, also called bobbin, is therefore very tightly coupled to magnetic flux changes caused by solenoid current variations. These current changes in the solenoid, will cause answer currents to flow in the resistive bobbin wall and therefore cause heat losses. The insertion of an external dump resistor in the solenoid current loop reduces energy dissipation inside the cryostat during a quench and will shorten the discharge time constant. This note presents a simple electrical model for the coupled bobbin and solenoid and makes it easier to understand the circuit behavior and losses. Estimates for the maximum allowable rate of solenoid current changes, based on the maximum permissible rate of losses can be made using this model

  2. Risk finance for catastrophe losses with Pareto-calibrated Lévy-stable severities.

    Science.gov (United States)

    Powers, Michael R; Powers, Thomas Y; Gao, Siwei

    2012-11-01

    For catastrophe losses, the conventional risk finance paradigm of enterprise risk management identifies transfer, as opposed to pooling or avoidance, as the preferred solution. However, this analysis does not necessarily account for differences between light- and heavy-tailed characteristics of loss portfolios. Of particular concern are the decreasing benefits of diversification (through pooling) as the tails of severity distributions become heavier. In the present article, we study a loss portfolio characterized by nonstochastic frequency and a class of Lévy-stable severity distributions calibrated to match the parameters of the Pareto II distribution. We then propose a conservative risk finance paradigm that can be used to prepare the firm for worst-case scenarios with regard to both (1) the firm's intrinsic sensitivity to risk and (2) the heaviness of the severity's tail. © 2012 Society for Risk Analysis.

  3. On losses caused in RF cavities by longitudinal electric fields

    International Nuclear Information System (INIS)

    Halbritter, J.

    1976-02-01

    Rf modes with large longitudinal electric fields (div E vector unequal to 0) at the cavity wall systematically show worse rf properties than modes with div E vector identical with 0; e.g. enlarged rf residual losses. While magnetic residual losses R sub(res) proportional f 2 are due to uncharged inhomogeneities in the oxide coating the metal, the electric residual losses R sub(orthogonal) occur via charged states in the oxide: the recharging of those states by tunnel exchange causes excitation across the energy gap of the superconductor yielding residual losses at high rf field strengths. The interaction of E sub(orthogonal) with the charges generate (longitudinal) phonons showing up as contribution to R sub(orthogonal). The resulting R sub(orthogonal) increases with E sub(orthogonal) and is nearly independent of frequency f, indicating the importance of R sub(orthogonal) for low frequency sc cavities, especially at high field strengths. In addition R sub(orthogonal) can account for the observed large residual losses of strip line modes in narrow junctions and joints between superconductors. (orig.) [de

  4. Investigation And Comparison of Fifth Grade Elementary Student’s Reading Skills with Severe Hearing Loss and Hearing in Tehran

    Directory of Open Access Journals (Sweden)

    Mohammad Razaei

    2013-04-01

    Full Text Available Objective: Since the written language is based on spoken language, hearing impairments may cause delays and defects in reading skills. This study is aimed to investigate reading problems in children with hearing loss and comparison of reading skills of fifth-grade elementary students’ reading skills suffering severe hearing loss. Materials & Methods: In this cross-sectional comparative study, 16 children with hearing loss were selected based on inclusion criteria from the whole fifth-grade elementary students with severe hearing loss in the Baghcheban schools and compared with 16 normal children matched upon the grade with sample group. To gather the data, Reading Test in elementary students was used as well as SPSS for data analysis. Results: Results showed children with hearing loss performed similarly as the control group on some skills, including naming speed skills (P=0.385, auditory-verbal sounds (reverse memory (P=0.345, visual-verbal pictures memory (P=1, phonological deletion (P=0.817 and nonword reading accuracy (P=0.633, however, they had poorer functions in the other domains. Conclusion: According to the result, it is concluded that auditory processing plays the key role in all prerequisite reading skills and children with hearing loss performed poorly on tasks based on auditory and language processing, whereas, the same perform on visual-processing-base tasks to normal children.

  5. Recent Advances in Cerebellar Ischemic Stroke Syndromes Causing Vertigo and Hearing Loss.

    Science.gov (United States)

    Kim, Hyun-Ah; Yi, Hyon-Ah; Lee, Hyung

    2016-12-01

    Cerebellar ischemic stroke is one of the common causes of vascular vertigo. It usually accompanies other neurological symptoms or signs, but a small infarct in the cerebellum can present with vertigo without other localizing symptoms. Approximately 11 % of the patients with isolated cerebellar infarction simulated acute peripheral vestibulopathy, and most patients had an infarct in the territory of the medial branch of the posterior inferior cerebellar artery (PICA). A head impulse test can differentiate acute isolated vertigo associated with PICA territory cerebellar infarction from more benign disorders involving the inner ear. Acute hearing loss (AHL) of a vascular cause is mostly associated with cerebellar infarction in the territory of the anterior inferior cerebellar artery (AICA), but PICA territory cerebellar infarction rarely causes AHL. To date, at least eight subgroups of AICA territory infarction have been identified according to the pattern of neurotological presentations, among which the most common pattern of audiovestibular dysfunction is the combined loss of auditory and vestibular functions. Sometimes acute isolated audiovestibular loss can be the initial symptom of impending posterior circulation ischemic stroke (particularly within the territory of the AICA). Audiovestibular loss from cerebellar infarction has a good long-term outcome than previously thought. Approximately half of patients with superior cerebellar artery territory (SCA) cerebellar infarction experienced true vertigo, suggesting that the vertigo and nystagmus in the SCA territory cerebellar infarctions are more common than previously thought. In this article, recent findings on clinical features of vertigo and hearing loss from cerebellar ischemic stroke syndrome are summarized.

  6. Loss of Dignity in Severe Chronic Obstructive Pulmonary Disease.

    Science.gov (United States)

    Solomon, Brahm K; Wilson, Keith G; Henderson, Peter R; Poulin, Patricia A; Kowal, John; McKim, Douglas A

    2016-03-01

    The maintenance of dignity is an important concept in palliative care, and the loss of dignity is a significant concern among patients with advanced cancer. The goals of this study were to examine whether loss of dignity is also a concern for patients receiving interdisciplinary rehabilitation for Stage III or IV chronic obstructive pulmonary disease. We examined the prevalence and correlates of loss of dignity and determined whether it improves with treatment. Inpatients underwent a structured interview inquiry around their sense of dignity and completed measures of pulmonary, physical, and psychological function at admission (n = 195) and discharge (n = 162). Loss of dignity was identified as a prominent ongoing concern for 13% of patients. It was correlated with measures of depression and anxiety sensitivity, but not with pulmonary capacity or functional performance. A robust improvement in loss of dignity was demonstrated, with 88% of those who reported a significant problem at admission no longer reporting one at discharge. The prevalence of a problematic loss of dignity among patients with severe chronic obstructive pulmonary disease is at least as high as among those receiving palliative cancer care. Loss of dignity may represent a concern among people with medical illnesses more broadly, and not just in the context of "death with dignity" at the end of life. Furthermore, interdisciplinary care may help to restore a sense of dignity to those individuals who are able to participate in rehabilitation. Copyright © 2016 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.

  7. Causes of severe visual impairment and blindness in children in schools for the blind in eastern Africa: changes in the last 14 years.

    Science.gov (United States)

    Njuguna, Margaret; Msukwa, Gerald; Shilio, Bernadeth; Tumwesigye, Cillasy; Courtright, Paul; Lewallen, Susan

    2009-01-01

    To determine the causes of severe visual impairment and blindness in children attending schools for the blind in Kenya, Malawi, Uganda, and Tanzania and to compare the findings with those of a 1994 study. Children attending schools for the blind or annexes in 4 eastern African countries were examined. The major anatomical site of and underlying etiology of severe visual impairment and blindness was recorded using the standardized World Health Organization (WHO) reporting form. A total of 1062 children aged below 16 years were examined of whom 701 (65.2%) had severe visual impairment or blindness. The major anatomical sites of visual loss overall (% and 95% CI) were cornea scar/phthisis bulbi (19%,16.1-21.9), whole globe lesions (15.7%,13.0-18.4), retina (15.4 %, 12.7-18.1), lens related disorders (13.1%, 10.7-15.5), and optic nerve disorders (12.3%, 9.9-14.7). Corneal scar/phthisis was not distributed equally among the countries and was highest in Malawi, similar to findings in 1995. The major etiology of visual loss was childhood factors (29.9%) and an estimated 40% of severe visual impairment and blindness was due to potentially avoidable causes. The major causes of severe visual impairment and blindness overall have not changed appreciably since 1995. There are important differences among countries, however, and using overall estimates for planning may be misleading.

  8. The losses at power grid caused by small nonlinear loads

    Directory of Open Access Journals (Sweden)

    Stevanović Dejan

    2013-01-01

    Full Text Available The difference between registered active power and spent unregistered power represents the losses at power grid. This paper treats problems related to losses caused by nonlinear loads connected to the power grid. In recent years the load profile of power consumers turned from energy-waster linear to energysaver but non-linear loads. The main cause of the losses appears due to the lack of adequate measurement equipment. Namely, common household power meters register only active energy, while power meters for industrial application register reactive energy as well. This approach does not follow the change of the enduser profile. Tendency of improving energy efficiency brought wide use of switching mode regulators and replace old incandescent light bulb with new energy saving lamps (CFL - compact fluorescent lamp, LED bulb. Therefore, the number of non-linear load drastically increased. Registering only active component of power at consumer’s side does not depict the real profile of the consumption. Therefore, in this paper we analyse and quantify the effects of nonlinear loads at power losses. As a result we suggest an efficient method for measuring the distortion component of power. The method relays on low-cost upgrade of commercial electronic power meters. The presented results of measurements on small non-linear loads confirm the proposed technique. Besides, they prove the importance of measuring all components of apparent power and justified their use in billing policy. The implemented set-up is based on power meter manufactured by EWG of Niš. [Projekat Ministarstva nauke Republike Srbije, br. TR32004

  9. Exposure to low-dose barium by drinking water causes hearing loss in mice.

    Science.gov (United States)

    Ohgami, Nobutaka; Hori, Sohjiro; Ohgami, Kyoko; Tamura, Haruka; Tsuzuki, Toyonori; Ohnuma, Shoko; Kato, Masashi

    2012-10-01

    We continuously ingest barium as a general element by drinking water and foods in our daily life. Exposure to high-dose barium (>100mg/kg/day) has been shown to cause physiological impairments. Direct administration of barium to inner ears by vascular perfusion has been shown to cause physiological impairments in inner ears. However, the toxic influence of oral exposure to low-dose barium on hearing levels has not been clarified in vivo. We analyzed the toxic influence of oral exposure to low-dose barium on hearing levels and inner ears in mice. We orally administered barium at low doses of 0.14 and 1.4 mg/kg/day to wild-type ICR mice by drinking water. The doses are equivalent to and 10-fold higher than the limit level (0.7 mg/l) of WHO health-based guidelines for drinking water, respectively. After 2-week exposure, hearing levels were measured by auditory brain stem responses and inner ears were morphologically analyzed. After 2-month exposure, tissue distribution of barium was measured by inductively coupled plasma mass spectrometry. Low-dose barium in drinking water caused severe hearing loss in mice. Inner ears including inner and outer hair cells, stria vascularis and spiral ganglion neurons showed severe degeneration. The Barium-administered group showed significantly higher levels of barium in inner ears than those in the control group, while barium levels in bone did not show a significant difference between the two groups. Barium levels in other tissues including the cerebrum, cerebellum, heart, liver and kidney were undetectably low in both groups. Our results demonstrate for the first time that low-dose barium administered by drinking water specifically distributes to inner ears resulting in severe ototoxicity with degeneration of inner ears in mice. Copyright © 2012 Elsevier Inc. All rights reserved.

  10. An economic assessment of the health effects and crop yield losses caused by air pollution in mainland China.

    Science.gov (United States)

    Miao, Weijie; Huang, Xin; Song, Yu

    2017-06-01

    Air pollution is severe in China, and pollutants such as PM 2.5 and surface O 3 may cause major damage to human health and crops, respectively. Few studies have considered the health effects of PM 2.5 or the loss of crop yields due to surface O 3 using model-simulated air pollution data in China. We used gridded outputs from the WRF-Chem model, high resolution population data, and crop yield data to evaluate the effects on human health and crop yield in mainland China. Our results showed that outdoor PM 2.5 pollution was responsible for 1.70-1.99 million cases of all-cause mortality in 2006. The economic costs of these health effects were estimated to be 151.1-176.9 billion USD, of which 90% were attributed to mortality. The estimated crop yield losses for wheat, rice, maize, and soybean were approximately 9, 4.6, 0.44, and 0.34 million tons, respectively, resulting in economic losses of 3.4 billion USD. The total economic losses due to ambient air pollution were estimated to be 154.5-180.3 billion USD, accounting for approximately 5.7%-6.6% of the total GDP of China in 2006. Our results show that both population health and staple crop yields in China have been significantly affected by exposure to air pollution. Measures should be taken to reduce emissions, improve air quality, and mitigate the economic loss. Copyright © 2016. Published by Elsevier B.V.

  11. Is otolithic vertigo accompanied by hearing loss caused by sacculocochlear endolymphatic hydrops?

    Science.gov (United States)

    Murofushi, Toshihisa; Komiyama, Sakurako; Hayashi, Yushi; Yoshimura, Eriko

    2016-01-01

    Otolithic vertigo is sometimes accompanied by hearing loss. Otolithic vertigo accompanied by hearing loss seems to be caused by sacculocochlear endolymphatic hydrops. To clarify the lesion site and pathophysiology of otolithic vertigo (OV) accompanied by hearing loss. The clinical records of four patients (two men and two women) that had been diagnosed with OV accompanied by hearing loss according to pre-determined diagnostic criteria were reviewed. The patients' main symptoms involved a sensation of movement in the pitch plane. All of the patients had low frequency-dominant hearing loss and either exhibited decreased cervical vestibular evoked myogenic potentials (cVEMP) or did not produce cVEMP. Two patients produced normal ocular VEMP (oVEMP). Caloric tests obtained normal results in all patients.

  12. Drugs and hair loss.

    Science.gov (United States)

    Patel, Mansi; Harrison, Shannon; Sinclair, Rodney

    2013-01-01

    Hair loss is a common complaint, both in men and women, and use of prescription medications is widespread. When there is a temporal association between the onset of hair loss and commencement of a medication, the medication is commonly thought to have caused the hair loss. However, hair loss and in particular telogen effluvium may occur in response to a number of triggers including fever, hemorrhage, severe illness, stress, and childbirth, and a thorough exclusion of these potential confounders is necessary before the hair loss can be blamed on the medication. Certain medications are known to cause hair loss by a variety of mechanisms including anagen arrest, telogen effluvium, or accentuation of androgenetic alopecia by androgens. Crown Copyright © 2013. Published by Elsevier Inc. All rights reserved.

  13. Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4-insufficient mouse model of large vestibular aqueduct syndrome.

    Science.gov (United States)

    Ito, T; Nishio, A; Wangemann, P; Griffith, A J

    2015-12-03

    Hearing loss of patients with enlargement of the vestibular aqueduct (EVA) can fluctuate or progress, with overall downward progression. The most common detectable cause of EVA is mutations of SLC26A4. We previously described a transgenic Slc26a4-insufficient mouse model of EVA in which Slc26a4 expression is controlled by doxycycline administration. Mice that received doxycycline from conception until embryonic day 17.5 (DE17.5; doxycycline discontinued at embryonic day 17.5) had fluctuating hearing loss between 1 and 6 months of age with an overall downward progression after 6 months of age. In this study, we characterized the cochlear functional and structural changes underlying irreversible hearing loss in DE17.5 mice at 12 months of age. The endocochlear potential was decreased and inversely correlated with auditory brainstem response thresholds. The stria vascularis was thickened and edematous in ears with less severe hearing loss, and thinned and atrophic in ears with more severe hearing loss. There were pathologic changes in marginal cell morphology and gene expression that were not observed at 3 months. We conclude that strial dysfunction and degeneration are the primary causes of irreversible progressive hearing loss in our Slc26a4-insufficient mouse model of EVA. This model of primary strial atrophy may be used to explore the mechanisms of progressive hearing loss due to strial dysfunction. Published by Elsevier Ltd.

  14. An Unexpected Cause of Severe Hypokalemia

    Directory of Open Access Journals (Sweden)

    Fernando Caravaca-Fontan

    2015-01-01

    Full Text Available We describe an unusual case of severe hypokalemia with electrocardiographic changes, due to licorice consumption, in a 15-year-old female student with no previous medical history. Prompt replacement of potassium and cessation of licorice ingestion resulted in a favourable outcome. We also discuss the pathophysiology and diagnosis, emphasizing the importance of a detailed anamnesis to rule out an often forgotten cause of hypokalemia as the licorice poisoning.

  15. A Hospital-based Study to Determine Causes of Diffuse Hair Loss in Women.

    Science.gov (United States)

    Malkud, Shashikant

    2015-08-01

    Diffuse hair loss is a common complaint encountered by dermatologists in their daily clinical practice. Hair loss in women is a distressing condition. Various underlying factors individually or in combination contribute to the pathogenesis. To determine causes of diffuse hair loss in women and to find the association between probable causes and relevant laboratory parameters, wherever applicable. One hundred and eighty women with diffuse hair loss were included in the study. Detailed history and clinical examination including hair pull test and hair microscopy were done in all study subjects. Specific laboratory investigations for determining iron deficiency anaemia, thyroid dysfunction and parasitic infestation were done. Among 180 patients, 116 (64.44%) had telogen effluvium, 28 (15.55%) had CTE, 21 (11.66%) had FPHL and 1 (0.55%) had AE. Fourteen patients (7.77%) had more than one aetiological diagnosis of diffuse hair loss. TE was the commonest type of diffuse hair loss. Incidence of TE and FPHL were highest in the age group of 21-30 years, whereas CTE in 30-40 years. Psychological stress and iron deficiency anaemia were the most common underlying aetiological factors for TE, which is statistically significant (phair loss is a multifactorial condition. A detailed history, thorough clinical examination and appropriate investigations help to identify the causative factors and treat them accordingly.

  16. Causes of severe visual impairment and blindness in students in schools for the blind in Northwest Ethiopia.

    Science.gov (United States)

    Asferaw, Mulusew; Woodruff, Geoffrey; Gilbert, Clare

    2017-01-01

    To determine the causes of severe visual impairment and blindness (SVI/BL) among students in schools for the blind in Northwest Ethiopia and to identify preventable and treatable causes. Students attending nine schools for the blind in Northwest Ethiopia were examined and causes assigned using the standard WHO record form for children with blindness and low vision in May and June 2015. 383 students were examined, 357 (93%) of whom were severely visually impaired or blind (blind and four were SVI, total 104. The major anatomical site of visual loss among those 0-15 years was cornea/phthisis (47.1%), usually due to measles and vitamin A deficiency, followed by whole globe (22.1%), lens (9.6%) and uvea (8.7%). Among students aged 16 years and above, corneal/phthisis (76.3%) was the major anatomical cause, followed by lens (6.3%), whole globe (4.7%), uvea (3.6%) and optic nerve (3.2%). The leading underlying aetiology among students aged blindness, mainly as the result of measles and vitamin A deficiency, is still a public health problem in Northwest Ethiopia, and this has not changed as observed in other low-income countries. More than three-fourth of causes of SVI/BL in students in schools for the blind are potentially avoidable, with measles/vitamin A deficiency and cataract being the leading causes.

  17. Gastric antral vascular ectasia causing severe anemia.

    Science.gov (United States)

    Toyota, M; Hinoda, Y; Nakagawa, N; Arimura, Y; Tokuchi, S; Takaoka, A; Kitagawa, S; Usuki, T; Yabana, T; Yachi, A; Imai, K

    1996-10-01

    Gastric antral vascular ectasia (GAVE) that caused continuous gastrointestinal bleeding is reported in a 76-year-old woman who had been treated with repeated blood transfusions because of severe anemia. Endoscopic examination was performed and diffuse speckled telangiectasia of the entire antrum was observed. Laboratory data showed SGOT > SGPT, decreased chE level and the increased levels of serum gastrin and ICG at 15 min. Anti-HCV antibody was positive. Image examination revealed splenomegaly. There was no family history of telangiectasia, and no telangiectasia was found in other organs. The diagnosis was established as GAVE with liver cirrhosis. Surgical resection of the distal stomach resulted in termination of the bleeding, and the cirrhotic changes of the surface of the liver were revealed at that time, providing further evidence of liver cirrhosis. Although the pathogenesis of GAVE is unknown, liver cirrhosis and hypergastrinemia are thought to be associated with the condition. Importantly, this condition is a cause of severe gastrointestinal bleeding in elderly patients.

  18. Cause of Death and the Quest for Meaning after the Loss of a Child

    Science.gov (United States)

    Lichtenthal, Wendy G.; Neimeyer, Robert A.; Currier, Joseph M.; Roberts, Kailey; Jordan, Nancy

    2013-01-01

    This study examined patterns of making meaning among 155 parents whose children died from a variety of violent and non-violent causes. Findings indicated 53% of violent loss survivors could not make sense of their loss, as compared to 32% of non-violent loss survivors. Overall, there was overlap in sense-making strategies across different causes…

  19. Chloroform ingestion causing severe gastrointestinal injury, hepatotoxicity and dermatitis confirmed with plasma chloroform concentrations.

    Science.gov (United States)

    Jayaweera, Dushan; Islam, Shawkat; Gunja, Naren; Cowie, Chris; Broska, James; Poojara, Latesh; Roberts, Michael S; Isbister, Geoffrey K

    2017-02-01

    Poisoning due to chloroform ingestion is rare. The classic features of acute chloroform toxicity include central nervous system (CNS) and respiratory depression, and delayed hepatotoxicity. A 30-year-old female ingested 20-30 mL of 99% chloroform solution, which caused rapid loss of consciousness, transient hypotension and severe respiratory depression requiring endotracheal intubation and ventilation. She was alert by 12 h and extubated 16 h post-overdose. At 38-h post-ingestion, her liver function tests started to rise and she was commenced on intravenous acetylcysteine. Her alanine transaminase (1283 U/L), aspartate transaminase (734 U/L) and international normalized ratio (2.3) peaked 67- to 72-h post-ingestion. She also developed severe abdominal pain, vomiting and diarrhoea. An abdominal CT scan was consistent with severe enterocolitis, and an upper gastrointestinal endoscopy showed erosive oesophagitis, severe erosive gastritis and ulceration. She was treated with opioid analgesia, proton pump inhibitors, sucralfate and total parenteral nutrition. Secretions caused a contact dermatitis of her face and back. Nine days post-ingestion she was able to tolerate food. Her liver function tests normalized and the dermatitis resolved. Chloroform was measured using headspace gas chromatograph mass spectrometry, with a peak concentration of 2.00 μg/mL, 4 h 20 min post-ingestion. The concentration-time data fitted a 1-compartment model with elimination half-life 6.5 h. In addition to early CNS depression and delayed hepatotoxicity, we report severe gastrointestinal injury and dermatitis with chloroform ingestion. Recovery occurred with good supportive care, acetylcysteine and management of gastrointestinal complications.

  20. Early age conductive hearing loss causes audiogenic seizure and hyperacusis behavior.

    Science.gov (United States)

    Sun, Wei; Manohar, Senthilvelan; Jayaram, Aditi; Kumaraguru, Anand; Fu, Qiang; Li, Ji; Allman, Brian

    2011-12-01

    Recent clinical reports found a high incidence of recurrent otitis media in children suffering hyperacusis, a marked intolerance to an otherwise ordinary environmental sound. However, it is unclear whether the conductive hearing loss caused by otitis media in early age will affect sound tolerance later in life. Thus, we have tested the effects of tympanic membrane (TM) damage at an early age on sound perception development in rats. Two weeks after the TM perforation, more than 80% of the rats showed audiogenic seizure (AGS) when exposed to loud sound (120 dB SPL white noise, hearing loss recovered. The TM damaged rats also showed significantly enhanced acoustic startle responses compared to the rats without TM damage. These results suggest that early age conductive hearing loss may cause an impaired sound tolerance during development. In addition, the AGS can be suppressed by the treatment of vigabatrin, acute injections (250 mg/kg) or oral intakes (60 mg/kg/day for 7 days), an antiepileptic drug that inhibits the catabolism of GABA. c-Fos staining showed a strong staining in the inferior colliculus (IC) in the TM damaged rats, not in the control rats, after exposed to loud sound, indicating a hyper-excitability in the IC during AGS. These results indicate that early age conductive hearing loss can impair sound tolerance by reducing GABA inhibition in the IC, which may be related to hyperacusis seen in children with otitis media. Published by Elsevier B.V.

  1. All-Cause and Cause-Specific Mortality Associated with Bariatric Surgery: A Review.

    Science.gov (United States)

    Adams, Ted D; Mehta, Tapan S; Davidson, Lance E; Hunt, Steven C

    2015-12-01

    The question of whether or not nonsurgical intentional or voluntary weight loss results in reduced mortality has been equivocal, with long-term mortality following weight loss being reported as increased, decreased, and not changed. In part, inconsistent results have been attributed to the uncertainty of whether the intentionality of weight loss is accurately reported in large population studies and also that achieving significant and sustained voluntary weight loss in large intervention trials is extremely difficult. Bariatric surgery has generally been free of these conflicts. Patients voluntarily undergo surgery and the resulting weight is typically significant and sustained. These elements, combined with possible non-weight loss-related mechanisms, have resulted in improved comorbidities, which likely contribute to a reduction in long-term mortality. This paper reviews the association between bariatric surgery and long-term mortality. From these studies, the general consensus is that bariatric surgical patients have: 1) significantly reduced long-term all-cause mortality when compared to severely obese non-bariatric surgical control groups; 2) greater mortality when compared to the general population, with the exception of one study; 3) reduced cardiovascular-, stroke-, and cancer-caused mortality when compared to severely obese non-operated controls; and 4) increased risk for externally caused death such as suicide.

  2. Constipation severity is associated with productivity losses and healthcare utilization in patients with chronic constipation.

    Science.gov (United States)

    Neri, Luca; Basilisco, Guido; Corazziari, Enrico; Stanghellini, Vincenzo; Bassotti, Gabrio; Bellini, Massimo; Perelli, Ilaria; Cuomo, Rosario

    2014-04-01

    We sought to evaluate the association between constipation severity, productivity losses and healthcare utilization in a national sample of Italian patients with chronic non-organic constipation (CC). We enrolled 878 outpatients with CC. Clinical and demographic data were collected by physicians during clinical examinations. Patients completed a self-administered questionnaire (Patient Assessment of Constipation-Symptoms, PAC-SYM; Work Productivity and Activity Impairment; healthcare utilization, and Symptoms Checklist 90 Revised - Somatization Scale, SCL-90 R). Mean PAC-SYM score was 1.62 ± 0.69. Mean weekly sick time due to constipation was 2.7 ± 8.6 h and productivity losses due to presenteeism was 19.7% ± 22.3%. Adjusted productivity losses in patients with severe CC (PAC-SYM score 2.3-4.0) compared to patients with mild symptoms (PAC-SYM score 0.0-1.0) was Italian Purchase Power Parity US$ 6160. Constipation severity (PAC-SYM quintiles) was associated with higher healthcare utilization (RRPAC-SYM 4/01.84; p-value for linear trend <0.01). After adjustment for somatization scores, the association of constipation severity with productivity losses and healthcare utilization rates was attenuated yet statistically significant. We observed a graded increase in productivity losses and healthcare utilization with increasing constipation severity. Further studies should evaluate whether significant savings might be achieved with regimens aimed at reducing the constipation severity.

  3. Severe hypertriglyceridemia in Japan: Differences in causes and therapeutic responses.

    Science.gov (United States)

    Murase, Toshio; Okubo, Minoru; Ebara, Tetsu; Mori, Yasumichi

    Severe hypertriglyceridemia (>1000 mg/dL) has a variety of causes and frequently leads to life-threating acute pancreatitis. However, the origins of this disorder are unclear for many patients. We aimed to characterize the causes of and responses to therapy in rare cases of severe hypertriglyceridemia in a group of Japanese patients. We enrolled 121 patients from a series of case studies that spanned 30 years. Subjects were divided into 3 groups: (1) primary (genetic causes); (2) secondary (acquired); and (3) disorders of uncertain causes. In the last group, we focused on 3 possible risks factors for hypertriglyceridemia: obesity, diabetes mellitus, and heavy alcohol intake. Group A (n = 20) included 13 patients with familial lipoprotein lipase deficiency, 3 patients with apolipoprotein CII deficiency, and other genetic disorders in the rest of the group. Group B patients (n = 15) had various metabolic and endocrine diseases. In Group C (uncertain causes; n = 86), there was conspicuous gender imbalance (79 males, 3 females) and most male subjects were heavy alcohol drinkers. In addition, 18 of 105 adult patients (17%) had histories of acute pancreatitis. The cause of severe hypertriglyceridemia is uncertain in many patients. In primary genetic forms of severe hypertriglyceridemia, genetic diversity between populations is unknown. In the acquired forms, we found fewer cases of estrogen-induced hypertriglyceridemia than in Western countries. In our clinical experience, the cause of most hypertriglyceridemia is uncertain. Our work suggests that genetic factors for plasma triglyceride sensitivity to alcohol should be explored. Copyright © 2017 National Lipid Association. Published by Elsevier Inc. All rights reserved.

  4. Severity and pattern of bone mineral loss in endocrine causes of osteoporosis as compared to age-related bone mineral loss

    Directory of Open Access Journals (Sweden)

    D Dutta

    2016-01-01

    Full Text Available Background: Data are scant on bone health in endocrinopathies from India. This study evaluated bone mineral density (BMD loss in endocrinopathies [Graves′ disease (GD, type 1 diabetes mellitus (T1DM, hypogonadotrophic hypogonadism (HypoH, hypergonadotropic hypogonadism (HyperH, hypopituitarism, primary hyperparathyroidism (PHPT] as compared to age-related BMD loss [postmenopausal osteoporosis (PMO, andropause]. Materials and Methods: Retrospective audit of records of patients >30 years age attending a bone clinic from August 2014 to January 2016 was done. Results: Five-hundred and seven records were screened, out of which 420 (females:male = 294:126 were analyzed. A significantly higher occurrence of vitamin D deficiency and insufficiency was noted in T1DM (89.09%, HyperH (85%, and HypoH (79.59% compared to age-related BMD loss (60.02%; P < 0.001. The occurrence of osteoporosis among females and males was 55.41% and 53.97%, respectively, and of osteopenia among females and males was 28.91% and 32.54%, respectively. In females, osteoporosis was significantly higher in T1DM (92%, HyperH (85%, and HypoH (59.26% compared to PMO (49.34%; P < 0.001. Z score at LS, TF, NOF, and greater trochanter (GT was consistently lowest in T1DM women. Among men, osteoporosis was significantly higher in T1DM (76.67% and HypoH (54.55% compared to andropause (45.45%; P = 0.001. Z score at LS, TF, NOF, GT, and TR was consistently lowest in T1DM men. In GD, the burden of osteoporosis was similar to PMO and andropause. BMD difference among the study groups was not significantly different after adjusting for body mass index (BMI and vitamin D. Conclusion: Low bone mass is extremely common in endocrinopathies, warranting routine screening and intervention. Concomitant vitamin D deficiency compounds the problem. Calcium and vitamin D supplementations may improve bone health in this setting.

  5. Loss of Arf4 causes severe degeneration of the exocrine pancreas but not cystic kidney disease or retinal degeneration.

    Directory of Open Access Journals (Sweden)

    Jillian N Pearring

    2017-04-01

    Full Text Available Arf4 is proposed to be a critical regulator of membrane protein trafficking in early secretory pathway. More recently, Arf4 was also implicated in regulating ciliary trafficking, however, this has not been comprehensively tested in vivo. To directly address Arf4's role in ciliary transport, we deleted Arf4 specifically in either rod photoreceptor cells, kidney, or globally during the early postnatal period. Arf4 deletion in photoreceptors did not cause protein mislocalization or retinal degeneration, as expected if Arf4 played a role in protein transport to the ciliary outer segment. Likewise, Arf4 deletion in kidney did not cause cystic disease, as expected if Arf4 were involved in general ciliary trafficking. In contrast, global Arf4 deletion in the early postnatal period resulted in growth restriction, severe pancreatic degeneration and early death. These findings are consistent with Arf4 playing a critical role in endomembrane trafficking, particularly in the pancreas, but not in ciliary function.

  6. Sensorineural hearing loss in children.

    LENUS (Irish Health Repository)

    Wormald, R

    2010-02-01

    The objective of the study was to examine the aetiology of sensorineural hearing loss (SNHL) in a paediatric population presenting to the National Centre of Medical Genetics. A retrospective chart review from 1998 to 2006. One hundred and twenty nine children were investigated for SNHL. The average age of diagnosis of hearing loss was 36 months. The degree of hearing loss was mild in 8 children, moderate in 33 children, severe in 31 children and profound in 57 children. Eighty-five children (66%) were diagnosed with a hereditary hearing loss, 11 (8%) children had an acquired hearing loss and no cause found in 33 (26%) children. This is the first report of the causes of hearing loss in Irish children. The mean age of diagnosis in our cohort is high and emphasises the need for a neonatal screening programme. There remains a number of children for whom the cause of hearing loss remains unknown.

  7. Judged seriousness of environmental losses: reliability and cause of loss

    Science.gov (United States)

    Thomas C. Brown; Dawn Nannini; Robert B. Gorter; Paul A. Bell; George L. Peterson

    2002-01-01

    Public judgments of the seriousness of environmental losses were found to be internally consistent for most respondents, and largely unaffected by attempts to manipulate responses by altering the mix of losses being judged. Both findings enhance confidence in the feasibility of developing reliable rankings of the seriousness of environmental losses to aid resource...

  8. Human error as the root cause of severe accidents at nuclear reactors

    International Nuclear Information System (INIS)

    Kovács Zoltán; Rýdzi, Stanislav

    2017-01-01

    A root cause is a factor inducing an undesirable event. It is feasible for root causes to be eliminated through technological process improvements. Human error was the root cause of all severe accidents at nuclear power plants. The TMI accident was caused by a series of human errors. The Chernobyl disaster occurred after a badly performed test of the turbogenerator at a reactor with design deficiencies, and in addition, the operators ignored the safety principles and disabled the safety systems. At Fukushima the tsunami risk was underestimated and the project failed to consider the specific issues of the site. The paper describes the severe accidents and points out the human errors that caused them. Also, provisions that might have eliminated those severe accidents are suggested. The fact that each severe accident occurred on a different type of reactor is relevant – no severe accident ever occurred twice at the same reactor type. The lessons learnt from the severe accidents and the safety measures implemented on reactor units all over the world seem to be effective. (orig.)

  9. Severe Bone Loss induced by Orthodontic Elastic Separator: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    A E Vishwanath

    2013-01-01

    Full Text Available A displaced orthodontic elastic separator was proposed as being the source of a gingival abscess that progressed to severe bone loss and exfoliation in a healthy adolescent patient with sound periodontal status prior to commencement of orthodontic treatment. After 1 year of undergoing orthodontic treatment, the patient presented with dull pain and mobility in the left upper permanent molar for which there was no apparent etiology. On clinical examination, the patient had gingival inflammation, associated with a deep pocket and severe mobility (grade III in relation to the same teeth. Radiographic examination of an orthopantomogram and intraoral periapical radiography (IOPAR revealed a chronic periodontal abscess with severe necrosis of the periodontal ligament and severe alveolar bone loss. A radiopaque mass on the distal surface below the cementoenamel junction (CEJ was also observed. The patient was referred to the department of periodontics for assessment and appropriate treatment. On curettage, it was found that there was orthodontic elastic separator which was displaced subgingivally.

  10. Novel mutations in DNAJB6 gene cause a very severe early-onset limb-girdle muscular dystrophy 1D disease.

    Science.gov (United States)

    Palmio, Johanna; Jonson, Per Harald; Evilä, Anni; Auranen, Mari; Straub, Volker; Bushby, Kate; Sarkozy, Anna; Kiuru-Enari, Sari; Sandell, Satu; Pihko, Helena; Hackman, Peter; Udd, Bjarne

    2015-11-01

    DNAJB6 is the causative gene for limb-girdle muscular dystrophy 1D (LGMD1D). Four different coding missense mutations, p.F89I, p.F93I, p.F93L, and p.P96R, have been reported in families from Europe, North America and Asia. The previously known mutations cause mainly adult-onset proximal muscle weakness with moderate progression and without respiratory involvement. A Finnish family and a British patient have been studied extensively due to a severe muscular dystrophy. The patients had childhood-onset LGMD, loss of ambulation in early adulthood and respiratory involvement; one patient died of respiratory failure aged 32. Two novel mutations, c.271T > A (p.F91I) and c.271T > C (p.F91L), in DNAJB6 were identified by whole exome sequencing as a cause of this severe form of LGMD1D. The results were confirmed by Sanger sequencing. The anti-aggregation effect of the mutant DNAJB6 was investigated in a filter-trap based system using transient transfection of mammalian cell lines and polyQ-huntingtin as a model for an aggregation-prone protein. Both novel mutant proteins show a significant loss of ability to prevent aggregation. Copyright © 2015 Elsevier B.V. All rights reserved.

  11. Tooth loss caused by displaced elastic during simple preprosthetic orthodontic treatment

    Science.gov (United States)

    Dianiskova, Simona; Calzolari, Chiara; Migliorati, Marco; Silvestrini-Biavati, Armando; Isola, Gaetano; Savoldi, Fabio; Dalessandri, Domenico; Paganelli, Corrado

    2016-01-01

    The use of elastics to close a diastema or correct tooth malpositions can create unintended consequences if not properly controlled. The American Association of Orthodontists recently issued a consumer alert, warning of “a substantial risk for irreparable damage” from a new trend called “do-it-yourself” orthodontics, consisting of patients autonomously using elastics to correct tooth position. The elastics can work their way below the gums and around the roots of the teeth, causing damage to the periodontium and even resulting in tooth loss. The cost of implants to replace these teeth would well exceed the cost of proper orthodontic care. This damage could also occur in a dental office, when a general dentist tries to perform a simplified orthodontic correction of a minor tooth malposition. The present case report describes a case of tooth loss caused by a displaced intraoral elastic, which occurred during a simple preprosthetic orthodontic treatment. PMID:27672645

  12. First report of Oryctes rhinoceros nudivirus (Coleoptera: Scarabaeidae) causing severe disease in Allomyrina dichotoma in Korea.

    Science.gov (United States)

    Lee, Seokhyun; Park, Kwan-Ho; Nam, Sung-Hee; Kwak, Kyu-Won; Choi, Ji-Young

    2015-01-01

    Oryctes rhinoceros nudivirus (OrNV) has been known to cause severe disease in coconut palm rhinoceros beetle, Oryctes rhinoceros, in Southeastern Asia and is used as a biological control to reduce the pest population. Here, we report for the first time that the OrNV may have landed on Korea and may be the major pathogen for diseased larvae of Korean horn beetle, Allomyrina dichotoma. After peroral inoculation, over 60% of infected larvae perished in 6 wk. This viral disease spreads very fast in several locations throughout Korea. This threat not only makes economic loss of local farms rearing A. dichotoma larvae but also may disturb the ecosystem by transmitting to wild A. dichotoma. © The Author 2015. Published by Oxford University Press on behalf of the Entomological Society of America.

  13. Language Outcomes in Young Children with Mild to Severe Hearing Loss.

    Science.gov (United States)

    Tomblin, J Bruce; Harrison, Melody; Ambrose, Sophie E; Walker, Elizabeth A; Oleson, Jacob J; Moeller, Mary Pat

    2015-01-01

    This study examined the language outcomes of children with mild to severe hearing loss during the preschool years. The longitudinal design was leveraged to test whether language growth trajectories were associated with degree of hearing loss and whether aided hearing influenced language growth in a systematic manner. The study also explored the influence of the timing of hearing aid fitting and extent of use on children's language growth. Finally, the study tested the hypothesis that morphosyntax may be at particular risk due to the demands it places on the processing of fine details in the linguistic input. The full cohort of children in this study comprised 290 children who were hard of hearing (CHH) and 112 children with normal hearing who participated in the Outcomes of Children with Hearing Loss (OCHL) study between the ages of 2 and 6 years. CHH had a mean better-ear pure-tone average of 47.66 dB HL (SD = 13.35). All children received a comprehensive battery of language measures at annual intervals, including standardized tests, parent-report measures, and spontaneous and elicited language samples. Principal components analysis supported the use of a single composite language score for each of the age levels (2, 3, 4, 5, and 6 years). Measures of unaided (better-ear pure-tone average, speech intelligibility index) and aided (residualized speech intelligibility index) hearing were collected, along with parent-report measures of daily hearing aid use time. Mixed modeling procedures were applied to examine the rate of change (227 CHH; 94 children with normal hearing) in language ability over time in relation to (1) degree of hearing loss, (2) aided hearing, (3) age of hearing aid fit and duration of use, and (4) daily hearing aid use. Principal components analysis was also employed to examine factor loadings from spontaneous language samples and to test their correspondence with standardized measures. Multiple regression analysis was used to test for

  14. A rare cause of severe hepatomegaly with an improving outcome

    DEFF Research Database (Denmark)

    Godskesen, Line Elberg; Abildgaard, Niels; Kjeldsen, Jens

    2014-01-01

    A previously healthy 43-year-old man presented with dyspnoea, 15 kg weight loss, severe hepatomegaly and alkaline phosphatase at 5400 U/L. Examinations seemed to suggest cirrhosis, but blood samples did not show any signs of underlying liver disease. Liver biopsy revealed amyloid light chain (AL...

  15. [Acute hearing loss and tinnitus caused by amplified recreational music].

    Science.gov (United States)

    Metternich, F U; Brusis, T

    1999-11-01

    Hearing loss resulting from exposure to permanent or repeated amplified music in professional musicians and music consumers is described in literature. The risk of hearing loss does not exist only after prolonged exposure to music. Short-term exposure to very high sound levels, for example in concerts, can also cause hearing loss and tinnitus. The retrospective study includes 24 patients who required rheologic therapy between 1994 and 1997 due to a music related acoustic trauma. The type, intensity, and length of music exposure as well as the distance and the position to the source of noise were examined. The type of hearing damage and its development during rheological treatment was studied by pure-tone audiometry. In the majority of examined patients (67%) the hearing loss developed on the basis of one-time exposure at a rock concert or pop concert, followed by hearing loss from attending discotheques (17%) or parties (12%), and music exposure from personal cassette players (4%). The majority of patients showed a maximum hearing loss of 40-60 dB (A) in a frequency between 3 kHz and 4 kHz. Pure-tone audiometry in 58% of the patients exhibited a unilateral threshold in a frequency between 3 kHz and 4 kHz combined with ipsilateral tinnitus of the same frequency. Twenty-one percent of the patients showed a symmetric bilateral threshold and tinnitus between 3 kHz and 4 kHz. In 8% there was a unilateral tinnitus, and in 13% a bilateral tinnitus without any hearing loss. All patients improved their hearing loss during rheologic treatment. Improvement in the tinnitus was only achieved in 33% of the examined cases. The risk of permanent hearing loss resulting from short-term exposure to amplified music is low compared to the risk of continuous tinnitus. Given the lack of acceptance of personal ear protectors, the risk of acute hearing damage due to amplified music could be reduced by avoiding the immediate proximity to the speakers.

  16. Oral creatine supplementation attenuates muscle loss caused by limb immobilization: a systematic review

    Directory of Open Access Journals (Sweden)

    Camila Souza Padilha

    Full Text Available Abstract Introduction: Recent studies have pointing creatine supplementation as a promising therapeutic alterna- tive in several diseases, especially myopathies and neurodegenerative disorder. Objective: elucidate the role of creatine supplementation on deleterious effect caused by limb immobilization in humans and rats. Methods: Analyzed articles were searched by three online databases, PubMed, SportDicus e Scielo. After a review and analysis, the studies were included in this review articles on effect of creatine supplementation on skeletal muscle in humans and rat, before, during and after a period of limb immobilization. Results: Studies analyzed demonstrated positive points in use of creatine supplementation as a therapeutic tool to mitigating the deleterious effects of limb immobilization, in humans and rat. Conclusion: The dataset of this literature review allows us to conclude that creatine supplementation may reduce muscle loss and/or assist in the recovery of muscle atrophy caused by immobilization and disuse in rats and humans. Also, we note that further research with better methodological rigor is needed to clarify the mechanisms by which creatine support the recovery of muscle atrophy. Moreover, these effects are positive and promising in the field of muscle rehabilitation, especially after member’s immobilization.

  17. An Index of Loss Aversion

    NARCIS (Netherlands)

    Köbberling, V.; Wakker, P.P.

    2005-01-01

    To a considerable extent, risk aversion as it is commonly observed is caused by loss aversion. Several indexes of loss aversion have been proposed in the literature. The one proposed in this paper leads to a clear decomposition of risk attitude into three distinct components: basic utility,

  18. An Index of Loss Aversion

    NARCIS (Netherlands)

    V. Köbberling (Veronika); P.P. Wakker (Peter)

    2005-01-01

    textabstractTo a considerable extent, risk aversion as it is commonly observed is caused by loss aversion. Several indexes of loss aversion have been proposed in the literature. The one proposed in this paper leads to a clear decomposition of risk attitude into three distinct components: basic

  19. [Rare cause for severe hypertriglyceridemia - case 9/2013].

    Science.gov (United States)

    Kahl, Sabine; Roden, Michael; Mörike, Klaus; Müssig, Karsten

    2013-11-01

    We report on a 48-year-old female patient with recently developed severe hypertriglyceridemia. Medical history was remarkable for breast cancer with breast-preserving surgery and chemoradiotherapy. The patient has been treated with 20 mg tamoxifen per day for three months. Laboratory results showed hypertriglyeridemia, hypercholesterolemia and lowered HDL-cholesterol. Findings were consistent with a drug-induced hypertriglyceridemia caused by anti-estrogenic therapy with tamoxifen. After consulting the patient's gynaecologist, we discontinued tamoxifen treatment. Thereupon, triglyceride levels fell consistently. There were no signs of pancreatitis, serum amylase and lipase were in the normal range. Patients with pre-diagnosed metabolic disorders, especially dyslipidemia and type 2 diabetes, should undergo regular controls of serum triglycerides during tamoxifen treatment. Also, one should keep in mind that a subacute, severe rise in serum triglyceride levels may be caused, in rare cases, by tamoxifen treatment. © Georg Thieme Verlag KG Stuttgart · New York.

  20. Output Loss Severity across EU Countries. Evidence for the 2008 Financial Crisis

    Directory of Open Access Journals (Sweden)

    Iustina Alina Boitan

    2015-08-01

    Full Text Available Financial crises are complex phenomena, in terms of the triggering factors, duration and severity, impact on both financial system and macroeconomic fundamentals and the full range of costs arising from its occurrence. The paper aims at providing an updated picture on the magnitude the 2008 financial crisis had, in terms of economic costs incurred by EU member states. It has been briefly reviewed crises’ main monetary and economic effects and costs. Then it has been employed International Monetary Fund’s approach for measuring crisis severity, expressed as an output loss indicator. To check the stability of the results, the basic methodology relying on a 3-year GDP trend has been complemented with a 7-year GDP trend. The output losses recorded by each EU country, under both trend assumptions, showed that Baltic states and Greece had been the most affected as they cumulated the highest losses. The lowest output losses have been registered in Western Europe countries (Austria, Belgium, France, Germany and Poland. Most EU economies’ growth still hasn’t entered on a robust ascending path, as they haven’t reached the level of GDP trend computed for the period preceding the onset of the financial crisis.

  1. What may cause fetus loss from acute pancreatitis in pregnancy: Analysis of 54 cases.

    Science.gov (United States)

    Tang, Min; Xu, Jian-Ming; Song, Sha-Sha; Mei, Qiao; Zhang, Li-Jiu

    2018-02-01

    Acute pancreatitis in pregnancy (APIP) poses a serious threat to the mother and her fetus, and might lead to fetal loss including miscarriage and stillbirth in certain patients. We sought to identify possible factors that affect fetal distress and evaluated outcomes of patients with APIP.We retrospectively reviewed clinical records of 54 pregnant women with APIP, who were treated at 2 tertiary clinical centers over a 6-year period. Clinical characteristics including etiology and severity of APIP, fetal monitoring data, and maternofetal outcomes were analyzed.Etiology of APIP included acute biliary pancreatitis (ABP, n = 14), hyperlipidemic pancreatitis (HLP, n = 22), and other etiologies (n = 18). Severity was classified as mild acute pancreatitis (MAP, n = 23), moderately severe acute pancreatitis (MSAP, n = 24), and severe acute pancreatitis (SAP, n = 7). The incidence of preterm delivery, fetal distress, and fetal loss increased with the progression of severity of APIP (P < .05). The severity of HLP was significantly higher than that of ABP and APIP of other etiology (P < .01). HLP was more likely to lead to fetal distress than other APs (P < .01). Only 12 (22.2%) patients had fetal monitoring including non-stress test (NST); 1 case of SAP (14.3%) and 15 cases of MSAP (62.5%) were not transferred to intensive care unit for intensive monitoring.The incidence of fetal distress and fetal loss increased with worsening of APIP severity. HLP tends to result in worse fetal outcomes. The deficiencies of fetal state monitoring, lack of assessment, and management of pregnant women might increase the fetal loss in APIP.

  2. Gradual Recovery from Bilateral Severe Sensorineural Hearing Loss post Motor Vehicle Accident.

    Science.gov (United States)

    Yaroko, A A; Shahrjerdi, B; M D, Md Khairi

    2013-04-01

    Sensorineural hearing loss following trauma is a common finding in daily clinical practice and usually associated with a poor prognosis. Our case illustrates a patient who was involved in motor vehicle accident sustaining bilateral severe to profound sensorineural hearing loss but subsequently recovered fully after two years. Unless there is clear trauma to the cochlea or auditory nerve, a substantial duration of follow up is needed in the treatment of such cases.

  3. Major losses of nutrients following a severe drought in a boreal forest.

    Science.gov (United States)

    Houle, Daniel; Lajoie, Geneviève; Duchesne, Louis

    2016-11-28

    Because of global warming, the frequency and severity of droughts are expected to increase, which will have an impact on forest ecosystem health worldwide 1 . Although the impact of drought on tree growth and mortality is being increasingly documented 2-4 , very little is known about the impact on nutrient cycling in forest ecosystems. Here, based on long-term monitoring data, we report nutrient fluxes in a boreal forest before, during and following a severe drought in July 2012. During and shortly after the drought, we observed high throughfall (rain collected below the canopy) concentrations of nutrient base cations (potassium, calcium and magnesium), chlorine, phosphorus and dissolved organic carbon (DOC), differing by one to two orders of magnitude relative to the long-term normal, and resulting in important canopy losses. The high throughfall fluxes had repercussions in the soil solution at a depth of 30 cm, leading to high DOC, chlorine and potassium concentrations. The net potassium losses (atmospheric deposition minus leaching losses) following the drought were especially important, being the equivalent of nearly 20 years of net losses under 'normal' conditions. Our data show that droughts have unexpected impacts on nutrient cycling through impacts on tree canopy and soils and may lead to important episodes of potassium losses from boreal forest ecosystems. The potassium losses associated with drought will add to those originating from tree harvesting and from forest fires and insect outbreaks 5-7 (with the last two being expected to increase in the future as a result of climate change), and may contribute to reduced potassium availability in boreal forests in a warming world.

  4. Neurogenic vision loss: Causes and outcome. An experience from a tertiary center in Northern India

    Directory of Open Access Journals (Sweden)

    Rajesh Verma

    2014-01-01

    Full Text Available Introduction: Vision loss can be a consequence of numerous disorders of eye and neural pathway conveying visual input to brain. A variety of conditions can affect visual pathway producing neurogenic vision loss. The presentation and course of vision loss depends on the site of involvement and underlying etiology. We conducted this unprecedented study to evaluate the characteristics and outcome of various diseases of the visual pathway. Materials and Methods: In this prospective cohort study, we evaluated 64 patients with neurogenic visual impairment. Ophthalmological causes were excluded in all of them. Their presentation, ophthalmological characteristics and investigation findings were recorded. These patients were followed up till 6 months. Results: Out of 69 patients evaluated, 5 were excluded as they had ophthalmological abnormalities. The remaining 64 cases (113 eyes were enrolled. 54 cases were due to diseases of anterior visual pathway and rest 10 had cortical vision loss. The etiologic distribution is as follows: Isolated optic neuritis- 12 (19%, multiple sclerosis- 4 (6.3%, neuromyelitis optica- 5 (7.9%, tubercular meningitis- 15 (23.8%, non-arteritic ischemic optic neuropathy, ischemic optic neuropathy complicating cavernous sinus thrombosis, cryptococcal meningitis, malignant infiltration of optic nerve, Crouzon′s syndrome, calvarial thickening and traumatic occipital gliosis- 1 (1.6% case each, idiopathic intracranial hypertension, pituitary adenoma, acute disseminated encephalomyelitis, posterior reversible leukoencephalopathy- 3 (4.8% cases each, cortical venous thrombosis 5 (7.9%, subacute scleroing panencephalitis- 4 (6.3% cases. Conclusions: The diseases of anterior visual pathway were much more common than cortical vision loss. A majority of our patients had severe impairment of vision at presentation.

  5. Prevalence and Causes of Visual Loss Among the Indigenous Peoples of the World: A Systematic Review.

    Science.gov (United States)

    Foreman, Joshua; Keel, Stuart; van Wijngaarden, Peter; Bourne, Rupert A; Wormald, Richard; Crowston, Jonathan; Taylor, Hugh R; Dirani, Mohamed

    2018-05-01

    Studies have documented a higher disease burden in indigenous compared with nonindigenous populations, but no global data on the epidemiology of visual loss in indigenous peoples are available. A systematic review of literature on visual loss in the world's indigenous populations could identify major gaps and inform interventions to reduce their burden of visual loss. To conduct a systematic review on the prevalence and causes of visual loss among the world's indigenous populations. A search of databases and alternative sources identified literature on the prevalence and causes of visual loss (visual impairment and blindness) and eye diseases in indigenous populations. Studies from January 1, 1990, through August 1, 2017, that included clinical eye examinations of indigenous participants and, where possible, compared findings with those of nonindigenous populations were included. Methodologic quality of studies was evaluated to reveal gaps in the literature. Limited data were available worldwide. A total of 85 articles described 64 unique studies from 24 countries that examined 79 598 unique indigenous participants. Nineteen studies reported comparator data on 42 085 nonindigenous individuals. The prevalence of visual loss was reported in 13 countries, with visual impairment ranging from 0.6% in indigenous Australian children to 48.5% in native Tibetans 50 years or older. Uncorrected refractive error was the main cause of visual impairment (21.0%-65.1%) in 5 of 6 studies that measured presenting visual acuity. Cataract was the main cause of visual impairment in all 6 studies measuring best-corrected acuity (25.4%-72.2%). Cataract was the leading cause of blindness in 13 studies (32.0%-79.2%), followed by uncorrected refractive error in 2 studies (33.0% and 35.8%). Most countries with indigenous peoples do not have data on the burden of visual loss in these populations. Although existing studies vary in methodologic quality and reliability, they suggest that most

  6. Severe signal loss in diamond beam loss monitors in high particle rate environments by charge trapping in radiation-induced defects

    CERN Document Server

    Kassel, Florian; Dabrowski, Anne; de Boer, Wim

    2016-01-01

    The beam condition monitoring leakage (BCML) system is a beam monitoring device in the compact muon solenoid (CMS) experiment at the large hadron collider (LHC). As detectors 32 poly-crystalline (pCVD) diamond sensors are positioned in rings around the beam pipe. Here, high particle rates occur from the colliding beams scattering particles outside the beam pipe. These particles cause defects, which act as traps for the ionization, thus reducing the charge collection efficiency (CCE). However, the loss in CCE was much more severe than expected from low rate laboratory measurements and simulations, especially in single-crystalline (sCVD) diamonds, which have a low initial concentration of defects. The reason why in real experiments the CCE is much worse than in laboratory experiments is related to the ionization rate. At high particle rates the trapping rate of the ionization is so high compared with the detrapping rate, that space charge builds up. This space charge reduces locally the internal electric field,...

  7. Estimating grain yield losses caused by septoria leaf blotch on durum wheat in Tunisia

    Directory of Open Access Journals (Sweden)

    Samia Berraies

    2014-12-01

    Full Text Available Septoria leaf blotch (SLB, caused by Zymoseptoria tritici (Desm. Quaedvlieg & Crous, 2011 (teleomorph: Mycosphaerella graminicola (Fuckel J. Schrot., is an important wheat disease in the Mediterranean region. In Tunisia, SLB has become a major disease of durum wheat (Triticum turgidum L. subsp. durum [Desf.] Husn. particularly during favorable growing seasons where significant yield losses and increase of fungicides use were recorded over the last three decades. The objectives of this study were to evaluate the effect of SLB severity on grain yield of new elite durum wheat breeding lines and to measure the relative effect of fungicide control on grain yield. Experiments were conducted during 2007-2008 and 2008-2009 cropping seasons. A set of 800 breeding lines were screened for reaction to SLB under natural infection at Beja research station. To estimate the disease effect, correlation between disease severity at early grain filling stage and grain yield was performed. Results showed that susceptible varieties yield was significantly reduced by SLB. Average yield reduction was as high as 384 and 325 kg ha-1 for every increment in disease severity on a 0-9 scale in both seasons, respectively. A negative correlation coefficient varied between -0.61 and -0.66 in both seasons. Treated and untreated trials conducted during 2008-2009 and 2009-2010 showed that yield of treated plots increased by 50% on the commonly cultivated susceptible varieties. The results of this investigation suggested that septoria incidence is related to large grain yield losses particularly on susceptible high yielding cultivars. However, appropriate fungicide application at booting growth stage could be beneficial for farmers. The development and use of more effective fungicide could be sought to alleviate the disease effects and therefore could be considered as a part of the integrated pest management and responsible use strategy on septoria leaf blotch in Tunisia.

  8. Mental stress as consequence and cause of vision loss: the dawn of psychosomatic ophthalmology for preventive and personalized medicine.

    Science.gov (United States)

    Sabel, Bernhard A; Wang, Jiaqi; Cárdenas-Morales, Lizbeth; Faiq, Muneeb; Heim, Christine

    2018-06-01

    The loss of vision after damage to the retina, optic nerve, or brain has often grave consequences in everyday life such as problems with recognizing faces, reading, or mobility. Because vision loss is considered to be irreversible and often progressive, patients experience continuous mental stress due to worries, anxiety, or fear with secondary consequences such as depression and social isolation. While prolonged mental stress is clearly a consequence of vision loss, it may also aggravate the situation. In fact, continuous stress and elevated cortisol levels negatively impact the eye and brain due to autonomous nervous system (sympathetic) imbalance and vascular dysregulation; hence stress may also be one of the major causes of visual system diseases such as glaucoma and optic neuropathy. Although stress is a known risk factor, its causal role in the development or progression of certain visual system disorders is not widely appreciated. This review of the literature discusses the relationship of stress and ophthalmological diseases. We conclude that stress is both consequence and cause of vision loss. This creates a vicious cycle of a downward spiral, in which initial vision loss creates stress which further accelerates vision loss, creating even more stress and so forth. This new psychosomatic perspective has several implications for clinical practice. Firstly, stress reduction and relaxation techniques (e.g., meditation, autogenic training, stress management training, and psychotherapy to learn to cope) should be recommended not only as complementary to traditional treatments of vision loss but possibly as preventive means to reduce progression of vision loss. Secondly, doctors should try their best to inculcate positivity and optimism in their patients while giving them the information the patients are entitled to, especially regarding the important value of stress reduction. In this way, the vicious cycle could be interrupted. More clinical studies are now

  9. [Causes and management of severe acute liver damage during pregnancy].

    Science.gov (United States)

    Sepulveda-Martinez, Alvaro; Romero, Carlos; Juarez, Guido; Hasbun, Jorge; Parra-Cordero, Mauro

    2015-05-01

    Abnormalities in liver function tests appear in 3% of pregnancies. Severe acute liver damage can be an exclusive condition of pregnancy (dependent or independent of pre-eclampsia) or a concomitant disease. HELLP syndrome and acute fatty liver of pregnancy are the most severe liver diseases associated with pregnancy. Both appear during the third trimester and have a similar clinical presentation. Acute fatty liver may be associated with hypoglycemia and HELLP syndrome is closely linked with pre-eclampsia. Among concomitant conditions, fulminant acute hepatitis caused by medications or virus is the most severe disease. Its clinical presentation may be hyper-acute with neurological involvement and severe coagulation disorders. It has a high mortality and patients should be transplanted. Fulminant hepatic failure caused by acetaminophen overdose can be managed with n-acetyl cysteine. Because of the high fetal mortality rate, the gestational age at diagnosis is crucial.

  10. Perceived causes of severe mental disturbance and preferred interventions by the Borana semi-nomadic population in southern Ethiopia: a qualitative study

    Science.gov (United States)

    2012-01-01

    Background Culture affects the way people conceptualize causes of severe mental disturbance which may lead to a variation in the preferred intervention methods. There is a seemingly dichotomous belief regarding what causes severe mental disturbance: people living in western countries tend to focus mainly on biological and psychosocial risk factors; whereas, in non-western countries the focus is mainly on supernatural and religious factors. These belief systems about causation potentially dictate the type of intervention preferred. Studying such belief systems in any society is expected to help in planning and implementation of appropriate mental health services. Methods A qualitative study was conducted among the Borana semi-nomadic population in southern Ethiopia to explore perceived causes of severe mental disturbance and preferred interventions. We selected, using purposive sampling, key informants from three villages and conducted a total of six focus group discussions: three for males and three for females. Results The views expressed regarding the causes of mental disturbance were heterogeneous encompassing supernatural causes such as possession by evil spirits, curse, bewitchment, ‘exposure to wind’ and subsequent attack by evil spirit in postnatal women and biopsychosocial causes such as infections (malaria), loss, ‘thinking too much’, and alcohol and khat abuse. The preferred interventions for severe mental disturbance included mainly indigenous approaches, such as consulting Borana wise men or indigenous healers, prayer, holy water treatment and seeking modern mental health care as a last resort. Conclusions These findings will be of value for health care planners who wish to expand modern mental health care to this population, indicating the need to increase awareness about the causes of severe mental disturbance and their interventions and collaborate with influential people and indigenous healers to increase acceptability of modern mental

  11. [IDENTIFICATION OF CAUSES OF EARLY PREGNANCY LOSSES ACCORDING TO HYSTOMORPHOLOGIC FEATURES].

    Science.gov (United States)

    Gotsiridze, K; Kintraia, N; Pailodze, M; Gogia, T; Tsaava, F

    2017-01-01

    Retrospective analysis of the early spontaneous abortions has been conducted (486 cases). Histomorphologic analysis of the curettage material contents erveledinvolutive-regressive developmental chsnges in 108 (22,28%) cases, in 370 cases (77.78%) pathologic changes, like inflammatory changes of deciduas and chorionic villis in 302 cases (80.2%), pathologic prematurity of chorionic vili in 48 cases (12.6%), hydatidiform mole in 28 cases (7.4%). As most cases of pregnancy loss has been reported at 7-9 weeks /172 cases/, we compared histomorphologic data revealed at 7-8 (71 cases) weeks to 5-6 (135 cases) and 10-12 weeks of gestation. Morphologic research data confimed, that at 7-8 weeks compared to 5-6 weeks leading reason ofpregnancy loss was inflammatory changes (OR-1,584), what can be cause of 110 pregnancy losses at 1000 pregnant women (AR-0.11). Data comparing7-9 weeks to 10-12 weeks pregnancy losses, confirm the priority of pathologic immaturity (OR-1,279) and hydatidiform mole (OR-1,557) and could be the risk of 100 cases of pregnancy termination at 1000 pregnant women (AR-0.10).Existing results are of great importance for the preconceptional preparation of women.

  12. Effectiveness of a Low-Calorie Weight Loss Program in Moderately and Severely Obese Patients

    Directory of Open Access Journals (Sweden)

    Julia K. Winkler

    2013-10-01

    Full Text Available Aims: To compare effectiveness of a 1-year weight loss program in moderately and severely obese patients. Methods: The study sample included 311 obese patients participating in a weight loss program, which comprised a 12-week weight reduction phase (low-calorie formula diet and a 40-week weight maintenance phase. Body weight and glucose and lipid values were determined at the beginning of the program as well as after the weight reduction and the weight maintenance phase. Participants were analyzed according to their BMI class at baseline (30-34.9 kg/m2; 35-39.9 kg/m2; 40-44.9 kg/m2; 45-49.9 kg/m2; ≥50 kg/m2. Furthermore, moderately obese patients (BMI 2 were compared to severely obese participants (BMI ≥ 40 kg/m2. Results: Out of 311 participants, 217 individuals completed the program. Their mean baseline BMI was 41.8 ± 0.5 kg/m2. Average weight loss was 17.9 ± 0.6%, resulting in a BMI of 34.3 ± 0.4 kg/m2 after 1 year (p Conclusion: 1-year weight loss intervention improves body weight as well as lipid and glucose metabolism not only in moderately, but also in severely obese individuals.

  13. Systematic review of severe acute liver injury caused by terbinafine.

    Science.gov (United States)

    Yan, Jun; Wang, Xiaolin; Chen, Shengli

    2014-08-01

    Terbinafine is an effective antimicrobial agent against dermatophytes, cryptococcus and other fungi. It is the preferred drug to treat onychomycosis. However, severe acute hepatitis from oral terbinafine administration has been recently reported. To describe a representative case, and review the literature regarding the best evidence on treatment and prognosis of severe acute hepatitis caused by oral terbinafine. The literature was searched for publications on severe hepatitis caused by terbinafine using MEDLINE, China Biology Medicine Disc, and the VIP Medical Information Resource System. Related references were searched manually. Seventeen English and three Chinese references of case reports were included after eliminating duplicate publications. No randomized control studies were found. Liver enzyme levels were found to have been increased significantly. Abdominal ultrasound demonstrated cholestasis. Severe acute liver injury is a known, but unusual complication of terbinafine exposure. The prognosis is often good with appropriate treatment. Liver function assessment before treatment and periodic monitoring 4-6 weeks after initiation of treatment is recommended.

  14. Prandial subcutaneous injections of glucagon-like peptide-1 cause weight loss in obese human subjects

    DEFF Research Database (Denmark)

    Näslund, Erik; King, N; Mansten, S

    2004-01-01

    Recombinant glucagon-like peptide-1 (7-36)amide (rGLP-1) was recently shown to cause significant weight loss in type 2 diabetics when administered for 6 weeks as a continuous subcutaneous infusion. The mechanisms responsible for the weight loss are not clarified. In the present study, rGLP-1 was ...... as a probable mechanism of action of increased satiety, decreased hunger and, hence, reduced food intake with an ensuing weight loss....

  15. Psychological adjustment to amputation: variations on the bases of sex, age and cause of limb loss

    International Nuclear Information System (INIS)

    Ali, S.; Haider, S.K.F.

    2017-01-01

    Amputation is the removal of a limb or part of a limb by a surgical procedure in order to save the life of a person. The underlying reasons behind the occurrence of this tragic incidence may be varied. However, irrespective of its cause limb loss is associated with wide range of life challenges. The study was done to investigate the psychological sequel of an individual after losing a limb and to know the level of strain and pressure they experience after this traumatic event. It also attempts to examine the moderating role of some demographic traits such as age, sex and cause of limb loss in psychosocial adjustment to amputation. Methods: The study includes 100 adult amputees of both genders and the data was collected from major government and private hospitals of Peshawar district. Demographic data sheet was constructed in order to know the demographics traits of amputees and a standardize Psychological Adjustment Scale developed by Sabir (1999) was used to find out the level of psychological adjustment after limb loss. Results: Nearly all the amputees' exhibit signs of psychological maladjustment at varying degrees. Males showed much greater signs of maladjustment than women and young adults were much psychologically shattered and disturbed as a result of limb loss. Amputation caused by planned medical reasons leads to less adjustment issues as compared to unplanned accidental amputation in which patient were not mentally prepare to accept this loss. Conclusion: Psychological aspect of amputation is an important aspect of limb loss which needs to be addressed properly in order to rehabilitate these patients and helps them to adjust successfully to their limb loss. (author)

  16. Staged Custom, Intramedullary Antibiotic Spacers for Severe Segmental Bone Loss in Infected Total Hip Arthroplasty

    Directory of Open Access Journals (Sweden)

    Atul F. Kamath

    2011-01-01

    Full Text Available Introduction. Total hip arthroplasty (THA infections with severe bone loss pose significant reconstructive challenges. We present our experience with two-stage hip reimplantation using an intramedullary, antibiotic-impregnated nail. Methods. Three patients with infected THA with severe proximal femoral bone loss (Mallory type IIIB or greater were treated using a custom antibiotic spacer. Clinical outcomes and any complications were recorded. Average followup was 49 months from final reimplantation. Results. Mean age at spacer placement (stage 1 was 53 years. The mean Harris Hip Score at final followup was 80. Two patients had asymptomatic heterotopic ossification, and one patient had a 2 cm leg-length discrepancy. Conclusions. A custom intramedullary nail antibiotic spacer is a reliable option in the staged management of the infected THA with severe proximal femoral bone loss. Benefits of this technique include limb salvage with maintenance of leg length, soft tissue tension, and functional status.

  17. Prevalence of Auditory Neuropathy in a Population of Children with Severe to Profound Hearing Loss

    Directory of Open Access Journals (Sweden)

    Nader Saki

    2013-04-01

    Full Text Available Background: The purpose of this investigation is to determine auditory neuropathy in the students with severe to profound hearing losses in Ahwaz.Materials and Methods: In this cross-sectional study, 212 children of 7-11 year old with severe to profound hearing loss performed ordinary audiometric evaluations as well as ABR and OAE. The patients with normal DPOAE who had no record of acoustic reflex having normal ABR, were considered as the patients with auditory neuropathy. Results: The neuropathic complication found in 14 children was appeared in 8 ones as one-sided (57.14% and in 6 ones (42.86% as two-sided. 68% of the patients as diagnosed had a very low Speech Discrimination Score (SDS.Conclusion: we must be very vigilant in auditory neuropathy diagnosis for the purpose to be successful in appropriate treatment of severe to profound hearing losses.

  18. Glaucoma Severity and Participation in Diverse Social Roles: Does Visual Field Loss Matter?

    Science.gov (United States)

    Yang, Yelin; Trope, Graham E; Buys, Yvonne M; Badley, Elizabeth M; Gignac, Monique A M; Shen, Carl; Jin, Ya-Ping

    2016-07-01

    To assess the association between glaucoma severity and participation in diverse social roles. Cross-sectional survey. Individuals with glaucoma, 50+, with visual acuity in the better eye >20/50 were enrolled. They were classified into 3 groups based on visual field loss in the better eye: mild [mean deviation (MD)>-6 dB], moderate (MD, -6 to -12 dB), and severe (MDSocial Role Participation Questionnaire assessed respondents' perceptions of the importance, difficulty, and satisfaction with participation in 11 social role domains (eg, community events, travel). Differences between groups were examined using multivariate linear regression analyses. A total of 118 participants (52% female) were included: 60 mild, 29 moderate, and 29 severe. All social role domains were rated as important by all participants except for education and employment. Women (Psocial activities. Compared with those with mild glaucoma, individuals with severe glaucoma reported significantly more difficulty participating in community/religious/cultural events (Psocial events (P=0.04). Participation in diverse social roles is valued by individuals with glaucoma. Severe visual field loss impedes involvement in and satisfaction with activities in community/religious/cultural events, travelling, and relationships with family members. Appropriate community and targeted interventions are needed to allow people with severe glaucoma to maintain active social participation-a key component to successful aging.

  19. PPIB mutations cause severe osteogenesis imperfecta.

    Science.gov (United States)

    van Dijk, Fleur S; Nesbitt, Isabel M; Zwikstra, Eline H; Nikkels, Peter G J; Piersma, Sander R; Fratantoni, Silvina A; Jimenez, Connie R; Huizer, Margriet; Morsman, Alice C; Cobben, Jan M; van Roij, Mirjam H H; Elting, Mariet W; Verbeke, Jonathan I M L; Wijnaendts, Liliane C D; Shaw, Nick J; Högler, Wolfgang; McKeown, Carole; Sistermans, Erik A; Dalton, Ann; Meijers-Heijboer, Hanne; Pals, Gerard

    2009-10-01

    Deficiency of cartilage-associated protein (CRTAP) or prolyl 3-hydroxylase 1(P3H1) has been reported in autosomal-recessive lethal or severe osteogenesis imperfecta (OI). CRTAP, P3H1, and cyclophilin B (CyPB) form an intracellular collagen-modifying complex that 3-hydroxylates proline at position 986 (P986) in the alpha1 chains of collagen type I. This 3-prolyl hydroxylation is decreased in patients with CRTAP and P3H1 deficiency. It was suspected that mutations in the PPIB gene encoding CyPB would also cause OI with decreased collagen 3-prolyl hydroxylation. To our knowledge we present the first two families with recessive OI caused by PPIB gene mutations. The clinical phenotype is compatible with OI Sillence type II-B/III as seen with COL1A1/2, CRTAP, and LEPRE1 mutations. The percentage of 3-hydroxylated P986 residues in patients with PPIB mutations is decreased in comparison to normal, but it is higher than in patients with CRTAP and LEPRE1 mutations. This result and the fact that CyPB is demonstrable independent of CRTAP and P3H1, along with reported decreased 3-prolyl hydroxylation due to deficiency of CRTAP lacking the catalytic hydroxylation domain and the known function of CyPB as a cis-trans isomerase, suggest that recessive OI is caused by a dysfunctional P3H1/CRTAP/CyPB complex rather than by the lack of 3-prolyl hydroxylation of a single proline residue in the alpha1 chains of collagen type I.

  20. Treatment of severe mitral regurgitation caused by lesions in both ...

    African Journals Online (AJOL)

    Mitral valve plasty (MVP) is preferred over mitral valve replacement (MVR) for mitral regurgitation in humans because of its favorable effect on quality of life. In small dogs, it is difficult to repair multiple lesions in both leaflets using MVP. Herein, we report a case of severe mitral regurgitation caused by multiple severe lesions ...

  1. TDP-43 Loss-of-Function Causes Neuronal Loss Due to Defective Steroid Receptor-Mediated Gene Program Switching in Drosophila

    Directory of Open Access Journals (Sweden)

    Lies Vanden Broeck

    2013-01-01

    Full Text Available TDP-43 proteinopathy is strongly implicated in the pathogenesis of amyotrophic lateral sclerosis and related neurodegenerative disorders. Whether TDP-43 neurotoxicity is caused by a novel toxic gain-of-function mechanism of the aggregates or by a loss of its normal function is unknown. We increased and decreased expression of TDP-43 (dTDP-43 in Drosophila. Although upregulation of dTDP-43 induced neuronal ubiquitin and dTDP-43-positive inclusions, both up- and downregulated dTDP-43 resulted in selective apoptosis of bursicon neurons and highly similar transcriptome alterations at the pupal-adult transition. Gene network analysis and genetic validation showed that both up- and downregulated dTDP-43 directly and dramatically increased the expression of the neuronal microtubule-associated protein Map205, resulting in cytoplasmic accumulations of the ecdysteroid receptor (EcR and a failure to switch EcR-dependent gene programs from a pupal to adult pattern. We propose that dTDP-43 neurotoxicity is caused by a loss of its normal function.

  2. Loss in species caused by tropical deforestation and their recovery through management

    Science.gov (United States)

    Ariel E. Lugo; John A. Parrotta; Sandra Brown

    1993-01-01

    The loss of species as a result of deforestation and degradation of tropical forest lands is widely discussed. Models based on island biogeography theory are used to evaluate the relationship between extinctions of species and deforestation. The analysis shows that natural resiliency causes the models to overestimate the rates of species extinctions for given...

  3. Analysis of HFETR shut-down state caused by loss of off-site power supply

    International Nuclear Information System (INIS)

    Wang Jinghu

    1997-01-01

    During the last 15 years, there are more than 40 unplanned shut-downs caused by loss of off-site power in HFETR. Because HFETR is a special research reactor, the author describes the shut-down state as three period. The author also discusses the influence of the number of shut-down due to loss of off-site power supply on the reactor safety, and propose some suggestions and measures to reduce the effects

  4. Incidence and main causes of severe maternal morbidity in São Luís, Maranhão, Brazil: a longitudinal study

    Directory of Open Access Journals (Sweden)

    Ana Paula Pierre Moraes

    Full Text Available CONTEXT AND OBJECTIVE: Evaluation of severe maternal morbidity has been used in monitoring of maternal health. The objective of this study was to estimate its incidence and main causes in São Luís, Maranhão, Brazil. DESIGN AND SETTING: Prospective longitudinal study, carried out in two public high-risk maternity hospitals and two public intensive care units (ICUs for referral of obstetric cases from the municipality. METHODS: Between March 1, 2009, and February 28, 2010, all cases of severe maternal morbidity according to the Mantel and Waterstone criteria were identified. The sociodemographic and healthcare characteristics of the extremely severe cases were compared with the less severe cases, using the Fisher, Χ2, Student t and Mann-Whitney tests, with a significance level of < 0.05. RESULTS: 127 cases of severe maternal morbidity were identified among 8,493 deliveries, i.e. an incidence of 15.0/1000 deliveries. Out of 122 cases interviewed, 121 cases were within the Waterstone criteria and 29 were within the Mantel criteria, corresponding to incidences of 14.1/1000 and 3.4/1000 deliveries, respectively. These rates were lower than those described in the literature, possibly due to case loss. The main causes were hypertension during pregnancy, which was more frequent in less severe cases (P = 0.001 and obstetric hemorrhage, which was more common among extremely severe cases (P = 0.01. CONCLUSIONS: Direct obstetric disorders were the main causes of severe maternal morbidity in São Luís, Maranhão. Investigation and monitoring of severe morbidity may contribute towards improving obstetric care in the municipality.

  5. Methodology used in Cuba for estimating economic losses caused by forest fires

    Science.gov (United States)

    Marcos Pedro Ramos Rodríguez; Raúl González Rodríguez

    2013-01-01

    Assessment of economic losses caused by forest fires is a highly complex but important activity. It is complicated first by the large number of effects, in different periods, brought about in the social, economic and environmental fields. Secondly, the difficulty of assigning a market value to resources such as biodiversity or endangered species should be mentioned. It...

  6. Sensorineural hearing loss and Mondini dysplasia caused by a deletion at locus DFN3.

    Science.gov (United States)

    Arellano, B; Ramírez Camacho, R; García Berrocal, J R; Villamar, M; del Castillo, I; Moreno, F

    2000-09-01

    To study a family with inner ear malformations and sensorineural hearing loss. Clinical, radiological, and genetic study of the members of a family with different degrees of sensorineural hearing loss. The males in the family manifested profound congenital hearing loss with severe inner ear malformations, while the only affected female had progressive hearing loss that had begun during puberty. Computed tomography showed inner ear malformations in both males, with enlarged internal auditory meatus and Mondini dysplasia. Genetic analysis disclosed a microdeletion at the locus DFN3 on chromosome X. A familial Mondini dysplasia is associated to a microdeletion at the deafness locus DFN3.

  7. Aging causes collateral rarefaction and increased severity of ischemic injury in multiple tissues

    Science.gov (United States)

    Faber, James E.; Zhang, Hua; Lassance-Soares, Roberta M.; Prabhakar, Pranay; Najafi, Amir H.; Burnett, Mary Susan; Epstein, Stephen E.

    2011-01-01

    Objective Aging is a major risk factor for increased ischemic tissue injury. Whether collateral rarefaction and impaired remodeling contribute to this is unknown. We quantified the number and diameter of native collaterals, and their remodeling in 3-, 16-, 24-, and 31-months-old mice. Methods and Results Aging caused an “age-dose-dependent” greater drop in perfusion immediately after femoral artery ligation, followed by a diminished recovery of flow and increase in tissue injury. These effects were associated with a decline in collateral number, diameter and remodeling. Angiogenesis was also impaired. Mechanistically, these changes were not accompanied by reduced recruitment of T-cells or macrophages to remodeling collaterals. However, eNOS signaling was dysfunctional, as indicated by increased protein nitrosylation and less phosphorylated eNOS and VASP in collateral wall cells. The cerebral circulation exhibited a similar age-dose-dependent loss of collateral number and diameter and increased tortuosity, resulting in an increase in collateral resistance and infarct volume (e.g., 6- and 3-fold, respectively, in 24-months-old mice) after artery occlusion. This was not associated with rarefaction of similarly-sized arterioles. Collateral remodeling was also reduced. Conclusions Our findings demonstrate that aging causes rarefaction and insufficiency of the collateral circulation in multiple tissues, resulting in more severe ischemic tissue injury. PMID:21617137

  8. Are patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia satisfied with nostril closure surgery?

    Science.gov (United States)

    Ichimura, Keiichi; Kikuchi, Hisashi; Imayoshi, Shoichiro; Yamauchi, Tomohiko; Ishikawa, Kotaro

    2012-02-01

    Recurrent epistaxis as a manifestation of hereditary hemorrhagic telangiectasia (HHT) is usually difficult to control. Although no treatment is regarded to be completely efficacious, nostril closure is considered a modality of choice for the most severe cases. The cessation of airflow resulting from this procedure can stop bleeding by minimizing risk factors. However, loss of nasal functions is a disadvantage of nostril closure. We conducted a questionnaire survey of patients who underwent nostril closure surgery, regarding the effects and disadvantages of the operation. Seven patients were asked questions on issues including frequency and severity of epistaxis pre- and post-operatively, satisfaction of treatment, and impairment in daily living activities. Most patients reported complete cessation of bleeding. Some still had bleeding, but the frequency and severity were far lower. No transfusions were required in any of the cases. Patients reported some disadvantages, for example, respiratory, olfactory, and phonatory issues. Six out of seven patients were very satisfied with the outcome of surgery. Nostril closure surgery can remarkably reduce frequency and volume of epistaxis. Our survey indicated that satisfactory results were achieved. However, difficulties caused by complete nasal obstruction varied. Thus, individualized coping strategies are required. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  9. Evaluation of the apparent losses caused by water meter under-registration in intermittent water supply.

    Science.gov (United States)

    Criminisi, A; Fontanazza, C M; Freni, G; Loggia, G La

    2009-01-01

    Apparent losses are usually caused by water theft, billing errors, or revenue meter under-registration. While the first two causes are directly related to water utility management and may be reduced by improving company procedures, water meter inaccuracies are considered to be the most significant and hardest to quantify. Water meter errors are amplified in networks subjected to water scarcity, where users adopt private storage tanks to cope with the intermittent water supply. The aim of this paper is to analyse the role of two variables influencing the apparent losses: water meter age and the private storage tank effect on meter performance. The study was carried out in Palermo (Italy). The impact of water meter ageing was evaluated in laboratory by testing 180 revenue meters, ranging from 0 to 45 years in age. The effects of the private water tanks were determined via field monitoring of real users and a mathematical model. This study demonstrates that the impact on apparent losses from the meter starting flow rapidly increases with meter age. Private water tanks, usually fed by a float valve, overstate meter under-registration, producing additional apparent losses between 15% and 40% for the users analysed in this study.

  10. Congenital cytomegalovirus (CMV) infection as a cause of permanent bilateral hearing loss: a quantitative assessment.

    Science.gov (United States)

    Grosse, Scott D; Ross, Danielle S; Dollard, Sheila C

    2008-02-01

    Congenital cytomegalovirus (CMV) infection is a cause of sensorineural hearing loss (SNHL) in children, but the magnitude of its contribution is uncertain. Quantifying the impact of congenital CMV infection requires an evidence-based assessment using a standard case definition of hearing loss. To determine the frequency of bilateral moderate to profound SNHL in children with congenital CMV infection and to estimate the CMV-attributable fraction of bilateral moderate to profound SNHL. A systematic review of studies of children with congenital CMV infection ascertained in an unbiased manner through universal newborn screening for CMV using viral culture in urine or saliva specimens in combination with a review of the literature on congenital CMV infection and hearing loss, including articles of all types. Approximately, 14% of children with congenital CMV infection develop SNHL of some type, and 3-5% develop bilateral moderate to profound SNHL. Among all children with bilateral moderate to profound SNHL, we estimate that 15-20% of cases are attributable to congenital CMV infection. Congenital CMV infection is one of the most important causes of hearing loss in young children, second only to genetic mutations, and is potentially preventable.

  11. Group points to underlying causes of ecosystem, blodiversity loss

    Science.gov (United States)

    Showstack, Randy

    Freshwater diversion, urban water pollution,and overfishing are leading to the decline of some of Pakistan's coastal mangrove ecosystems. In Mexico's Calakmul Biosphere Reserve, near the border of Guatemala, population growth and poverty are pushing forest clearing. Meanwhile, in Chilika Lake in southeast India, changes in economic policies and global markets have led to changes in commercial aquaculture that is partly responsible for the decline of local fisheries and the bird population.These are the conclusions of some of the 10 case studies contained in a World Wildlife Fund (WWF) report, issued on July 6, that examines forests, wetlands, steppes, mangroves, and other habitats to determine the underlying causes for biodiversity loss.

  12. Hibernation Based Therapy to Improve Survival of Severe Blood Loss

    Science.gov (United States)

    2016-06-01

    leaks extravascularly • Necrosis and inflammation involving the ear tip is considered to be a more severe manifestation of vascular damage associated...similar lesions to the 2M test solution, it appears that 2M test solution is more likely to cause vascular necrosis and inflammation (noted at 24 hours...injections • Although DMSO induced similar lesions to the 4M test solution, it appears that 4M test solution is more likely to cause vascular necrosis and

  13. Increasing losses caused by natural disasters: what are the drivers, how is the insurance industry affected, what has to be done?

    Science.gov (United States)

    Hoppe, P.

    2016-12-01

    Losses caused by natural disasters are a major factor influencing the balance sheet of insurers, especially reinsurers. Such events have a high potential of creating extreme accumulation losses by affecting different business lines at the same time. The basis for long term profitable business in the insurance industry is to be able to calculate and also get risk adequate premiums for the covers of natural perils. Risk adequate means that on average over several years the losses occurring can be paid by the premium income. Losses caused by natural disasters have increased tremendously worldwide in the last decades. The main drivers have been changes in the exposed values, i.e. growth of population and wealth in affected regions. For the insurance industry this loss increasing process in general is not a problem as the premiums normally are proportional to the sum insured, i.e. the exposed value. These factors can be quantified and the loss trends can be adjusted for these changes. Munich Re just recently has developed a very sophisticated method for such a normalisation of losses. After this normalization in respect to exposed values a still residual loss trend can be either driven by changes in the vulnerability of assets or on the hazard side. Trends in both factors are not automatically considered in the premiums, they have to be detected, quantified and then built into the risk models of the insurers. Many studies as well as data from the Munich Re NatCatSERVICE suggest that weather related hazards already have changed for some perils and in some regions. Climate research implies that due to global warming such trends will increase in the coming decades. Very little quantitative data, however, on such changes still is available to allow the adjustment of the risk models of insurers. In order to do this, data with high regional resolution and also probabilities of certain scenarios would be necessary. The other gap of information is quantitative data on changes

  14. De Gustibus: time scale of loss and recovery of tastes caused by radiotherapy

    International Nuclear Information System (INIS)

    Maes, Annelies; Huygh, Ingrid; Weltens, Caroline; Vandevelde, Guy; Delaere, Pierre; Evers, Georges; Bogaert, Walter van den

    2002-01-01

    Purpose: To quantify the prevalence and distress of taste loss at different intervals after radiotherapy (RT) for head and neck cancer. Materials and methods: In four different groups of head and neck cancer patients (73 patients in total), taste loss and distress due to taste loss were evaluated by taste acuity tests and taste questionnaires. Group 1 (n=17) was analyzed prior to RT. Groups 2 (n=17), 3 (n=17) and 4 (n=22) were at 2, 6 and 12-24 months after treatment, respectively. A cross-sectional analysis was performed between these four groups. Results: Prior to initiation of RT (group 1), partial taste loss was observed in 35, 18 and 6% of patients for bitter, salt and sweet, respectively. At 2 months after RT (group 2), taste loss (partial or total) was seen in 88, 82, 76 and 53% for bitter, salt, sweet and sour, respectively. At 6 months (group 3), partial taste loss was seen in 71, 65, 41 and 41% (bitter, salt, sweet, sour) and after 1-2 years (group 4) in 41, 50, 27 and 27% (bitter, salt, sweet, sour). Distress caused by taste loss was most frequent in group 2 (82%). Conclusions: In this study, loss of taste after RT was found to be most pronounced after 2 months. Bitter and salt qualities were most impaired. Gradual recovery was seen during the first year after treatment. Partial taste loss still persisted 1-2 years after treatment and was responsible for slight to moderate discomfort

  15. Can an "Aesthetic" Intervention (Braided Hair Coil) Cause Hair Loss After an Aesthetic Operation?

    Science.gov (United States)

    Dionyssopoulos, Alexander; Papaconstantinou, Antony; Stoltidou, Alexandra; Spyropoulou, Georgia-Alexandra

    2014-07-01

    Postoperative pressure alopecia (PPA), defined as hair loss caused by prolonged pressure on the patient's scalp during surgery, is an uncommon condition after aesthetic surgery. Originally, it was described for patients who underwent lengthy cardiovascular and gynecologic operations. The authors present a rare case, in which hair loss occurred after secondary breast augmentation (replacement of breast implants). The PPA appeared in the occipitoparietal region of the patient's scalp approximately 2 weeks after surgery. The operation was completed in less than 3 hours, without any fluctuations in the patient's blood pressure or any unusual blood loss. There were no other precipitating factors such as anemia or coagulopathies. The probable cause of this unexpected result was the patient's braided hair coil, which had not been noted before the operation. The patient habitually, and on the day of her operation, combed her hair into a braided coil, which placed extra pressure on the occipitoparietal region. The hair loss was temporary, and hair regrowth was complete within 2 months. This incident may have been avoided if the braided hair coil had been noted by nursing or other medical staff preoperatively. Repositioning the head every 30 minutes and providing adequate head padding during surgery are advised to protect the patient and prevent such incidents. 5. © 2014 The American Society for Aesthetic Plastic Surgery, Inc.

  16. Conditional deletion of pejvakin in adult outer hair cells causes progressive hearing loss in mice.

    Science.gov (United States)

    Harris, Suzan L; Kazmierczak, Marcin; Pangršič, Tina; Shah, Prahar; Chuchvara, Nadiya; Barrantes-Freer, Alonso; Moser, Tobias; Schwander, Martin

    2017-03-06

    Mutations in the Pejvakin (Pjvk) gene cause autosomal recessive hearing loss DFNB59 with audiological features of auditory neuropathy spectrum disorder (ANSD) or cochlear dysfunction. The precise mechanisms underlying the variable clinical phenotypes of DFNB59 remain unclear. Here, we demonstrate that mice with conditional ablation of the Pjvk gene in all sensory hair cells or only in outer hair cells (OHCs) show similar auditory phenotypes with early-onset profound hearing loss. By contrast, loss of Pjvk in adult OHCs causes a slowly progressive hearing loss associated with OHC degeneration and delayed loss of inner hair cells (IHCs), indicating a primary role for pejvakin in regulating OHC function and survival. Consistent with this model, synaptic transmission at the IHC ribbon synapse is largely unaffected in sirtaki mice that carry a C-terminal deletion mutation in Pjvk. Using the C-terminal domain of pejvakin as bait, we identified in a cochlear cDNA library ROCK2, an effector for the small GTPase Rho, and the scaffold protein IQGAP1, involved in modulating actin dynamics. Both ROCK2 and IQGAP1 associate via their coiled-coil domains with pejvakin. We conclude that pejvakin is required to sustain OHC activity and survival in a cell-autonomous manner likely involving regulation of Rho signaling. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

  17. Hormonal Treatment for Severe Hydronephrosis Caused by Bladder Endometriosis

    Directory of Open Access Journals (Sweden)

    Erkan Efe

    2014-01-01

    Full Text Available The incidence of endometriosis cases involving the urinary system has recently increased, and the bladder is a specific zone where endometriosis is most commonly seen in the urinary system. In the case presented here, a patient presented to the emergency department with the complaint of side pain and was examined and diagnosed with severe hydronephrosis and bladder endometriosis was determined in the etiology. After the patient was pathologically diagnosed, Levonorgestrel-Releasing Intrauterine System (LNG-IUS was administered to the uterine cavity. At the 12-month follow-up, endometriosis was not observed in the cystoscopy and symptoms had completely regressed. Hydronephrosis may be observed after exposure of the ureter, and silent renal function loss may develop in patients suffering from endometriosis with bladder involvement. For patients with moderate or severe hydronephrosis associated with bladder endometriosis, LNG-IUS application may be separately and successfully used after conservative surgery.

  18. The efficacy of fuel treatment in mitigating property loss during wildfires: Insights from analysis of the severity of the catastrophic fires in 2009 in Victoria, Australia.

    Science.gov (United States)

    Price, Owen F; Bradstock, Ross A

    2012-12-30

    Treatment of fuel (e.g. prescribed fire, logging) in fire-prone ecosystems is done to reduce risks to people and their property but effects require quantification, particularly under severe weather conditions when the destructive potential of fires on human infrastructure is maximised. We analysed the relative effects of fuel age (i.e. indicative of the effectiveness of prescribed fire) and logging on remotely sensed (SPOT imagery) severity of fires which occurred in eucalypt forests in Victoria, Australia in 2009. These fires burned under the most severe weather conditions recorded in Australia and caused large losses of life and property. Statistical models of the probability of contrasting extremes of severity (crown fire versus fire confined to the understorey) were developed based on effects of fuel age, logging, weather, topography and forest type. Weather was the primary influence on severity, though it was reduced at low fuel ages in Moderate but not Catastrophic, Very High or Low fire-weather conditions. Probability of crown fires was higher in recently logged areas than in areas logged decades before, indicating likely ineffectiveness as a fuel treatment. The results suggest that recently burnt areas (up to 5-10 years) may reduce the intensity of the fire but not sufficiently to increase the chance of effective suppression under severe weather conditions. Since house loss was most likely under these conditions (67%), effects of prescribed burning across landscapes on house loss are likely to be small when weather conditions are severe. Fuel treatments need to be located close to houses in order to effectively mitigate risk of loss. Copyright © 2012 Elsevier Ltd. All rights reserved.

  19. Loss of neuronal integrity: a cause of hypometabolism in patients with traumatic brain injury without MRI abnormality in the chronic stage

    International Nuclear Information System (INIS)

    Shiga, Tohru; Matsuyama, Tetsuaki; Kageyama, Hiroyuki; Kohno, Tomoya; Tamaki, Nagara; Ikoma, Katsunori; Isoyama, Hirotaka; Katoh, Chietsugu; Kuge, Yuji; Terae, Satoshi

    2006-01-01

    Traumatic brain injury (TBI) causes brain dysfunction in many patients. However, some patients have severe brain dysfunction but display no abnormalities on magnetic resonance imaging (MRI). There have been some reports of hypometabolism even in such patients. The purpose of this study was to investigate the relationship between metabolic abnormality and loss of neuronal integrity in TBI patients with some symptoms but without MRI abnormalities. The study population comprised ten patients with TBI and ten normal volunteers. All of the patients were examined at least 1 year after the injury. 15 O-labelled gas PET and [ 11 C]flumazenil (FMZ) positron emission tomography (PET) were carried out. The cerebral metabolic rate of oxygen (CMRO 2 ) and binding potential (BP) images of FMZ were calculated. Axial T2WI, T2*WI and FLAIR images were obtained. Coronal images were added in some cases. All of the patients had normal MRI findings, and all showed areas with abnormally low CMRO 2 . Low uptake on BP images was observed in six patients (60%). No lesions that showed low uptake on BP images were without low CMRO 2 . On the other hand, there were 14 lesions with low CMRO 2 but without BP abnormalities. These results indicate that there are metabolic abnormalities in TBI patients with some symptoms after brain injury but without abnormalities on MRI. Some of the hypometabolic lesions showed low BP, indicating a loss of neuronal integrity. Thus, FMZ PET may have potential to distinguish hypometabolism caused by neuronal loss from that caused by other factors. (orig.)

  20. Technetium-99m DMSA preparation: Trivial issues causing severe problems

    International Nuclear Information System (INIS)

    Kumar, V.

    1997-01-01

    Urinary tract infection (UTI) in children involving renal parenchyma, upper collecting system or bladder is one of the major causes for consideration in the diagnosis and management of paediatric nuclear medicine. Acute pyelonephritis is one of the prime causes of morbidity associated with urinary tract infection in children which can lead to progressive renal damage. Technetium-99m dimercaptosuccinic acid (DMSA) is used extensively for the assessment of UTI in paediatrics. The radiopharmaceutical preparation could be influenced by several factors, most of them are trivial, but invariably have severe impact on the quality of the scintiphotographs. This communication is mainly to highlight some of the issues related to 99 mTc-DMSA preparation and the possible precautionary measures that need to be taken to obviate unwarranted problems. (author)

  1. A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart.

    Science.gov (United States)

    Manji, Shehnaaz S M; Williams, Louise H; Miller, Kerry A; Ooms, Lisa M; Bahlo, Melanie; Mitchell, Christina A; Dahl, Hans-Henrik M

    2011-03-15

    Hearing impairment is the most common sensory impairment in humans, affecting 1:1,000 births. We have identified an ENU generated mouse mutant, Mozart, with recessively inherited, non-syndromic progressive hearing loss caused by a mutation in the synaptojanin 2 (Synj2), a central regulatory enzyme in the phosphoinositide-signaling cascade. The hearing loss in Mozart is caused by a p.Asn538Lys mutation in the catalytic domain of the inositol polyphosphate 5-phosphatase synaptojanin 2. Within the cochlea, Synj2 mRNA expression was detected in the inner and outer hair cells but not in the spiral ganglion. Synj2(N538K) mutant protein showed loss of lipid phosphatase activity, and was unable to degrade phosphoinositide signaling molecules. Mutant Mozart mice (Synj2(N538K/N538K)) exhibited progressive hearing loss and showed signs of hair cell degeneration as early as two weeks of age, with fusion of stereocilia followed by complete loss of hair bundles and ultimately loss of hair cells. No changes in vestibular or neurological function, or other clinical or behavioral manifestations were apparent. Phosphoinositides are membrane associated signaling molecules that regulate many cellular processes including cell death, proliferation, actin polymerization and ion channel activity. These results reveal Synj2 as a critical regulator of hair cell survival that is essential for hair cell maintenance and hearing function.

  2. A case of fetal intestinal volvulus without malrotation causing severe anemia.

    Science.gov (United States)

    Nakagawa, Tomoko; Tachibana, Daisuke; Kitada, Kohei; Kurihara, Yasushi; Terada, Hiroyuki; Koyama, Masayasu; Sakae, Yukari; Morotomi, Yoshiki; Nomura, Shiho; Saito, Mika

    2015-01-01

    Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

  3. A guinea pig model of selective severe high-frequency hearing loss.

    Science.gov (United States)

    Havenith, Sarah; Klis, Sjaak F L; Versnel, Huib; Grolman, Wilko

    2013-10-01

    Using an appropriate dose of an aminoglycoside antibiotic cotreated with a loop diuretic a guinea pig model of high-frequency loss can be obtained mimicking cochlear implant candidates with low-frequency residual hearing. We examined the stability of this model over time. A well-established method to create an animal model for profound deafness is cotreatment with an aminoglycoside antibiotic and a loop diuretic. Recent data indicated that reduction of the aminoglycoside dose might yield selective high-frequency hearing loss. Such a model is relevant for studies related to hybrid cochlear implant devices, for example, with respect to preservation of residual hearing. Guinea pigs received an electrode for chronic recording of compound action potentials to tones to assess thresholds. They were treated with a coadministration of kanamycin (200 mg/kg) and furosemide (100 mg/kg), after which, the animals were sacrificed for histologic analysis at 2, 4, or 7 weeks. After 2 to 7 weeks threshold shifts were greater than 50 dB for 8 to 16 kHz in 15 of 17 animals, whereas threshold shifts at 2 kHz or lower were less than 50 dB in 13 animals. Major threshold shifts occurred the first 2 to 4 days; subsequently, some spontaneous recovery occurred and, after 2-3 weeks thresholds, remained stable. Inner hair cell loss still progressed between 2 and 4 weeks in the most basal cochlear region; thereafter, hair cell loss was stable. An appropriate animal model for selective severe high-frequency hearing loss was obtained, which is stable at 4 weeks after ototoxic treatment.

  4. An overall estimation of losses caused by diseases in the Brazilian fish farms.

    Science.gov (United States)

    Tavares-Dias, Marcos; Martins, Maurício Laterça

    2017-12-01

    Parasitic and infectious diseases are common in finfish, but are difficult to accurately estimate the economic impacts on the production in a country with large dimensions like Brazil. The aim of this study was to estimate the costs caused by economic losses of finfish due to mortality by diseases in Brazil. A model for estimating the costs related to parasitic and bacterial diseases in farmed fish and an estimative of these economic impacts are presented. We used official data of production and mortality of finfish for rough estimation of economic losses. The losses herein presented are related to direct and indirect economic costs for freshwater farmed fish, which were estimated in US$ 84 million per year. Finally, it was possible to establish by the first time an estimative of overall losses in finfish production in Brazil using data available from production. Therefore, this current estimative must help researchers and policy makers to approximate the economic costs of diseases for fish farming industry, as well as for developing of public policies on the control measures of diseases and priority research lines.

  5. No-Reference Video Quality Assessment Model for Distortion Caused by Packet Loss in the Real-Time Mobile Video Services

    Directory of Open Access Journals (Sweden)

    Jiarun Song

    2014-01-01

    Full Text Available Packet loss will make severe errors due to the corruption of related video data. For most video streams, because the predictive coding structures are employed, the transmission errors in one frame will not only cause decoding failure of itself at the receiver side, but also propagate to its subsequent frames along the motion prediction path, which will bring a significant degradation of end-to-end video quality. To quantify the effects of packet loss on video quality, a no-reference objective quality assessment model is presented in this paper. Considering the fact that the degradation of video quality significantly relies on the video content, the temporal complexity is estimated to reflect the varying characteristic of video content, using the macroblocks with different motion activities in each frame. Then, the quality of the frame affected by the reference frame loss, by error propagation, or by both of them is evaluated, respectively. Utilizing a two-level temporal pooling scheme, the video quality is finally obtained. Extensive experimental results show that the video quality estimated by the proposed method matches well with the subjective quality.

  6. A Case of Fetal Intestinal Volvulus without Malrotation Causing Severe Anemia

    Directory of Open Access Journals (Sweden)

    Tomoko Nakagawa

    2015-01-01

    Full Text Available Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

  7. Losses in electric motors caused by harmonics; Harmoniske overtoners betydning for tabene i elmotorer

    Energy Technology Data Exchange (ETDEWEB)

    Johansson, M. [Dansk Energi Analyse A/S, Glostrup (Denmark); Kehr, J.M. [ABB A/S, Skovlunde (Denmark); Hoejte Hansen, H. [Balslev A/S, Glostrup (Denmark)

    2012-03-15

    the motor. The results of the lab measurements correspond very well to the losses calculated. On this basis it is concluded that harmonic currents and voltages cause energy losses in induction motors directly supplied, but that the energy losses are fairly low at the levels of distortion, a THDU of up to 6 to 8 %, which occur in Danish industry. Motors, which are supplied through a frequency converter, are not influenced by the harmonics in the supply mains. The analysis of the motor losses shows that the losses from the load torque, caused by the 5., 11., 17. etc harmonics, are considerably lower than the (very small) current heat losses in the motor, which have been caused by the same harmonics. The analysis also shows that the current displacement in the motors reduces the currents and the losses from the higher harmonics. Considering losses consequently the lower harmonics and especially 5. and 7. harmonics are most important. (LN)

  8. Measuring welfare loss caused by air pollution in Europe. A CGE analysis

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Kyung-Min [Joint Program on the Science and Policy of Global Change, Massachusetts Institute of Technology, Cambridge (United States); Department of Urban Studies and Planning, Massachusetts Institute of Technology, Cambridge (United States); Selin, Noelle E. [Joint Program on the Science and Policy of Global Change, Massachusetts Institute of Technology, Cambridge (United States); Engineering Systems Division and Department of Earth, Atmospheric and Planetary Sciences, Massachusetts Institute of Technology, Cambridge (United States); Reilly, John M.; Paltsev, Sergey [Joint Program on the Science and Policy of Global Change, Massachusetts Institute of Technology, Cambridge (United States)

    2010-09-15

    To evaluate the socio-economic impacts of air pollution, we develop an integrated approach based on computable general equilibrium (CGE). Applying our approach to 18 western European countries shows that even there, where air quality is relatively high compared with other parts of the world, health-related damages caused by air pollution may be substantial. We estimate that as of 2005, Europe experienced an annual loss in consumption of about 220 billion Euro in year 2000 prices (about 3% of total consumption) with a range based on 95% high and low epidemiological response functions of 107-335 billion Euro and a total welfare loss of about 370 billion Euro (range of 209-550) including both consumption and broader welfare losses (around 2% of welfare level) due to the accumulated effects of three decades of air pollution in Europe. In addition, we estimate that a set of air quality improvement policy scenarios as proposed in the 2005 CAFE program would bring 18 European countries as a whole a welfare gain of 37-49 billion Euro (year 2000 prices) in year 2020 alone. (author)

  9. Measuring welfare loss caused by air pollution in Europe: A CGE analysis

    International Nuclear Information System (INIS)

    Nam, Kyung-Min; Selin, Noelle E.; Reilly, John M.; Paltsev, Sergey

    2010-01-01

    To evaluate the socio-economic impacts of air pollution, we develop an integrated approach based on computable general equilibrium (CGE). Applying our approach to 18 western European countries shows that even there, where air quality is relatively high compared with other parts of the world, health-related damages caused by air pollution may be substantial. We estimate that as of 2005, Europe experienced an annual loss in consumption of about 220 billion Euro in year 2000 prices (about 3% of total consumption) with a range based on 95% high and low epidemiological response functions of 107-335 billion Euro and a total welfare loss of about 370 billion Euro (range of 209-550) including both consumption and broader welfare losses (around 2% of welfare level) due to the accumulated effects of three decades of air pollution in Europe. In addition, we estimate that a set of air quality improvement policy scenarios as proposed in the 2005 CAFE program would bring 18 European countries as a whole a welfare gain of 37-49 billion Euro (year 2000 prices) in year 2020 alone.

  10. Meal replacements as a weight loss tool in a population with severe mental illness.

    Science.gov (United States)

    Gelberg, Hollie A; Kwan, Crystal L; Mena, Shirley J; Erickson, Zachary D; Baker, Matthew R; Chamberlin, Valery; Nguyen, Charles; Rosen, Jennifer A; Shah, Chandresh; Ames, Donna

    2015-12-01

    Weight gain and worsening metabolic parameters are often side effects of antipsychotic medications used by individuals with severe mental illness. To address this, a randomized, controlled research study of a behavioral weight management program for individuals with severe mental illness was undertaken to assess its efficacy. Patients unable to meet weight loss goals during the first portion of the year-long study were given the option of using meal replacement shakes in an effort to assist with weight loss. Specific requirements for use of meal replacement shakes were specified in the study protocol; only five patients were able to use the shakes in accordance with the protocol and lose weight while improving metabolic parameters. Case studies of two subjects are presented, illustrating the challenges and obstacles they faced, as well as their successes. Taking responsibility for their own weight loss, remaining motivated through the end of the study, and incorporating the meal replacement shakes into a daily routine were factors found in common with these patients. Use of meal replacements shakes with this population may be effective. Published by Elsevier Ltd.

  11. Different clinical features of anaphylaxis according to cause and risk factors for severe reactions.

    Science.gov (United States)

    Kim, Sang-Yoon; Kim, Min-Hye; Cho, Young-Joo

    2018-01-01

    Anaphylaxis is a life-threatening allergic reaction. Several studies reported different anaphylactic reactions according to the causative substances. However, a comparison of anaphylaxis for each cause has not been done. This study was conducted to identify common causes of anaphylaxis, characteristics of anaphylactic reaction for each cause and to analyze the factors related to the severity of the reaction. Medical records of patients who visited the emergency room of Ewha Womans University Mokdong Hospital from March 2003 to April 2016 and diagnosed with anaphylactic shock were retrospectively reviewed. We compared the clinical features of anaphylaxis according to the cause. In addition, the severity of anaphylaxis was analyzed and contributing factors for severe anaphylaxis were reviewed. A total of 199 patients with anaphylaxis were analyzed. Food was the most common cause (49.7%), followed by drug reaction (36.2%), bee venom (10.1%), and unknown cause (4.0%). Cardiovascular symptoms of syncope and hypotension were more common in drug-induced anaphylaxis. The incidence of severe anaphylaxis was the highest in anaphylaxis due to drugs (54.2%). Urticaria and other skin symptoms were significantly more common in food-induced anaphylaxis. Risk factors for severe anaphylaxis included older age, male, and drug-induced one. Epinephrine treatment of anaphylaxis was done for 69.7% and 56.9% of patients with food-induced and drug-induced anaphylaxis, respectively. More severe anaphylaxis developed with drug treatment and in males. Low rate of epinephrine prescription was also observed. Male patients with drug induced anaphylaxis should be paid more attention. Copyright © 2017 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.

  12. Evaluation of human dental loss caused by carbamide peroxide bleacher compared with phosphoric acid conditioning - radioactive method

    International Nuclear Information System (INIS)

    Adachi, Eduardo Makoto; Yousseff, Michel Nicolau; Saiki, Mitiko

    2002-01-01

    The radiometric method was applied to the evaluation of dental loss caused by carbamide peroxide when it is applied on the surface layers of enamel and dentin tissues. Also the dental loss caused by the etching with 37% phosphoric acid procedure used in aesthetic restoration was assessed for comparison with those results obtained. The tooth samples irradiated with a P standard in a thermal neutron flux of the nuclear reactor were placed in contact with 10% carbamide peroxide or with 37% phosphoric acid solution. The radioactivity of 32 P transferred from the radioactive teeth to the bleaching gel or to etching acid was measured using a Geiger Muller detector to calculate the mass of P removed in this treatment and losses were calculated after obtaining their P concentrations. Results obtained indicated that enamel and dentin exposed to carbamide peroxide bleaching agent lose phosphorus. The extent of enamel loss was smaller than that obtained for dentin. In the case of acid etching, there was no difference between the results obtained for enamel and dentin loss. Also the dentin loss obtained after a treatment of 30 applications of 10% carbamide peroxide was the same magnitude of that one application of 37% phosphoric acid. (author)

  13. Increased risk of all-cause mortality and renal graft loss in stable renal transplant recipients with hyperparathyroidism.

    Science.gov (United States)

    Pihlstrøm, Hege; Dahle, Dag Olav; Mjøen, Geir; Pilz, Stefan; März, Winfried; Abedini, Sadollah; Holme, Ingar; Fellström, Bengt; Jardine, Alan G; Holdaas, Hallvard

    2015-02-01

    Hyperparathyroidism is reported in 10% to 66% of renal transplant recipients (RTR). The influence of persisting hyperparathyroidism on long-term clinical outcomes in RTR has not been examined in a large prospective study. We investigated the association between baseline parathyroid hormone (PTH) levels and major cardiovascular events, renal graft loss, and all-cause mortality by Cox Proportional Hazard survival analyses in 1840 stable RTR derived from the Assessment of LEscol in Renal Transplantation trial. Patients were recruited in a mean of 5.1 years after transplantation, and follow-up time was 6 to 7 years. Significant associations between PTH and all 3 outcomes were found in univariate analyses. When adjusting for a range of plausible confounders, including measures of renal function and serum mineral levels, PTH remained significantly associated with all-cause mortality (4% increased risk per 10 units; P=0.004), and with graft loss (6% increased risk per 10 units; PHyperparathyroidism is an independent, potentially remediable, risk factor for renal graft loss and all-cause mortality in RTR.

  14. Early Subchondral Bone Loss at Arthritis Onset Predicted Late Arthritis Severity in a Rat Arthritis Model.

    Science.gov (United States)

    Courbon, Guillaume; Cleret, Damien; Linossier, Marie-Thérèse; Vico, Laurence; Marotte, Hubert

    2017-06-01

    Synovitis is usually observed before loss of articular function in rheumatoid arthritis (RA). In addition to the synovium and according to the "Inside-Outside" theory, bone compartment is also involved in RA pathogenesis. Then, we investigated time dependent articular bone loss and prediction of early bone loss to late arthritis severity on the rat adjuvant-induced arthritis (AIA) model. Lewis female rats were longitudinally monitored from arthritis induction (day 0), with early (day 10) and late (day 17) steps. Trabecular and cortical microarchitecture parameters of four ankle bones were assessed by microcomputed tomography. Gene expression was determined at sacrifice. Arthritis occurred at day 10 in AIA rats. At this time, bone erosions were detected on four ankle bones, with cortical porosity increase (+67%) and trabecular alterations including bone volume fraction (BV/TV: -13%), and trabecular thickness decrease. Navicular bone assessment was the most reproducible and sensitive. Furthermore, strong correlations were observed between bone alterations at day 10 and arthritis severity or bone loss at day 17, including predictability of day 10 BV/TV to day 17 articular index (R 2  = 0.76). Finally, gene expression at day 17 confirmed massive osteoclast activation and interestingly provided insights on strong activation of bone formation inhibitor markers at the joint level. In rat AIA, bone loss was already observed at synovitis onset and was predicted late arthritis severity. Our results reinforced the key role of subchondral bone in arthritis pathogenesis, in favour to the "Inside-Outside" theory. Mechanisms of bone loss in rat AIA involved resorption activation and formation inhibition changes. J. Cell. Physiol. 232: 1318-1325, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Severe hypoglycaemia requiring the assistance of emergency medical services - frequency, causes and symptoms

    Czech Academy of Sciences Publication Activity Database

    Krnačová, V.; Kuběna, Aleš Antonín; Macek, K.; Bezděk, M.; Šmahelová, A.; Vlček, J.

    2012-01-01

    Roč. 156, č. 3 (2012), s. 271-277 ISSN 1213-8118 Grant - others:GA UK(CZ) SVV-2010-261-004 Keywords : regression trees * causes * symptoms * incidence * emergency medical service * severe hypoglycaemia Subject RIV: EI - Biotechnology ; Bionics Impact factor: 0.990, year: 2012 http://library.utia.cas.cz/separaty/2013/E/kubena-severe hypoglycaemia requiring the assistance of emergency medical services - frequency causes and symptoms.pdf

  16. Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    Hyun Jin Kim

    2017-03-01

    Full Text Available Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL, respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL. She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.

  17. Edge-related loss of tree phylogenetic diversity in the severely fragmented Brazilian Atlantic forest.

    Science.gov (United States)

    Santos, Bráulio A; Arroyo-Rodríguez, Víctor; Moreno, Claudia E; Tabarelli, Marcelo

    2010-09-08

    Deforestation and forest fragmentation are known major causes of nonrandom extinction, but there is no information about their impact on the phylogenetic diversity of the remaining species assemblages. Using a large vegetation dataset from an old hyper-fragmented landscape in the Brazilian Atlantic rainforest we assess whether the local extirpation of tree species and functional impoverishment of tree assemblages reduce the phylogenetic diversity of the remaining tree assemblages. We detected a significant loss of tree phylogenetic diversity in forest edges, but not in core areas of small (phylogenetic distance between any two randomly chosen individuals from forest edges; an increase of 17% in the average phylogenetic distance to closest non-conspecific relative for each individual in forest edges; and to the potential manifestation of late edge effects in the core areas of small forest remnants. We found no evidence supporting fragmentation-induced phylogenetic clustering or evenness. This could be explained by the low phylogenetic conservatism of key life-history traits corresponding to vulnerable species. Edge effects must be reduced to effectively protect tree phylogenetic diversity in the severely fragmented Brazilian Atlantic forest.

  18. Sparks, signals and shock absorbers: how dystrophin loss causes muscular dystrophy.

    Science.gov (United States)

    Batchelor, Clare L; Winder, Steve J

    2006-04-01

    The dystrophin-glycoprotein complex (DGC) can be considered as a specialized adhesion complex, linking the extracellular matrix to the actin cytoskeleton, primarily in muscle cells. Mutations in several components of the DGC lead to its partial or total loss, resulting in various forms of muscular dystrophy. These typically manifest as progressive wasting diseases with loss of muscle integrity. Debate is ongoing about the precise function of the DGC: initially a strictly mechanical role was proposed but it has been suggested that there is aberrant calcium handling in muscular dystrophy and, more recently, changes in MAP kinase and GTPase signalling have been implicated in the aetiology of the disease. Here, we discuss new and interesting developments in these aspects of DGC function and attempt to rationalize the mechanical, calcium and signalling hypotheses to provide a unifying hypothesis of the underlying process of muscular dystrophy.

  19. "Loss of breath" as a cause of postoperative hypoxia and bradycardia in children submitted to tonsillectomy.

    Science.gov (United States)

    Moro, Eduardo Toshiyuki; Goulart, Alexandre Palmeira

    2015-01-01

    the "shortness of breath" or "breathing interruption" crisis can be considered a cause of hypoxia in childhood. It is characterized by the presence of a triggering factor followed by weeping and apnea in expiration accompanied by cyanosis or pallor. The sequence of events may include bradycardia, loss of consciousness, abnormal postural tone and even asystole. A review of the literature revealed only two reports of postoperative apnea caused by "shortness of breath". this article describes the case of a child with a history of "shortness of breath" undiagnosed before the adenotonsillectomy, but that represented the cause of episodes of hypoxemia and bradycardia in the postoperative period. the "shortness of breath" crisis should be considered as a possible cause of perioperative hypoxia in children, especially when there is a history suggestive of this problem. As some events may be accompanied by bradycardia, loss of consciousness, abnormal postural tone and even asystole, observation in a hospital setting should be considered. Copyright © 2013 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  20. Functional photoreceptor loss revealed with adaptive optics: an alternate cause of color blindness.

    Science.gov (United States)

    Carroll, Joseph; Neitz, Maureen; Hofer, Heidi; Neitz, Jay; Williams, David R

    2004-06-01

    There is enormous variation in the X-linked L/M (long/middle wavelength sensitive) gene array underlying "normal" color vision in humans. This variability has been shown to underlie individual variation in color matching behavior. Recently, red-green color blindness has also been shown to be associated with distinctly different genotypes. This has opened the possibility that there may be important phenotypic differences within classically defined groups of color blind individuals. Here, adaptive optics retinal imaging has revealed a mechanism for producing dichromatic color vision in which the expression of a mutant cone photopigment gene leads to the loss of the entire corresponding class of cone photoreceptor cells. Previously, the theory that common forms of inherited color blindness could be caused by the loss of photoreceptor cells had been discounted. We confirm that remarkably, this loss of one-third of the cones does not impair any aspect of vision other than color.

  1. Climate impact of idealized winter polar mesospheric and stratospheric ozone losses as caused by energetic particle precipitation

    Science.gov (United States)

    Meraner, Katharina; Schmidt, Hauke

    2018-01-01

    Energetic particles enter the polar atmosphere and enhance the production of nitrogen oxides and hydrogen oxides in the winter stratosphere and mesosphere. Both components are powerful ozone destroyers. Recently, it has been inferred from observations that the direct effect of energetic particle precipitation (EPP) causes significant long-term mesospheric ozone variability. Satellites observe a decrease in mesospheric ozone up to 34 % between EPP maximum and EPP minimum. Stratospheric ozone decreases due to the indirect effect of EPP by about 10-15 % observed by satellite instruments. Here, we analyze the climate impact of winter boreal idealized polar mesospheric and polar stratospheric ozone losses as caused by EPP in the coupled Max Planck Institute Earth System Model (MPI-ESM). Using radiative transfer modeling, we find that the radiative forcing of mesospheric ozone loss during polar night is small. Hence, climate effects of mesospheric ozone loss due to energetic particles seem unlikely. Stratospheric ozone loss due to energetic particles warms the winter polar stratosphere and subsequently weakens the polar vortex. However, those changes are small, and few statistically significant changes in surface climate are found.

  2. Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model.

    Science.gov (United States)

    Carraro, Mattia; Almishaal, Ali; Hillas, Elaine; Firpo, Matthew; Park, Albert; Harrison, Robert V

    2017-04-01

    Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks. Vascular casts of mCMV-damaged cochleas, and those of untreated control animals, were examined using scanning electron microscopy. We find very large variations in the degree of vascular damage in animals given identical viral injections (2000 pfu). The primary lesion caused by CMV infection is to the stria vascularis and to the adjacent spiral limbus capillary network. Capillary beds of the spiral ligament are generally less affected. The initial vascular damage is found in the mid-apical turn and appears to progress to more basal cochlear regions. After viral migration to the inner ear, the stria vascularis is the primary affected structure. We suggest that initial auditory threshold losses may relate to the poor development or maintenance of the endocochlear potential caused by strial dysfunction. Our increased understanding of the pathogenesis of CMV-related hearing loss is important for defining methods for early detection and treatment.

  3. Gender-Based Violence Causing Severe Multiple Injuries; a Case Report

    Directory of Open Access Journals (Sweden)

    Adalard Falschung

    2018-04-01

    Full Text Available Introduction: Gender-based violence (GBV against women has been identified as a global health and development issue. We reported a case of GBV causing sever, multiple injuries in a middle-aged female. Case report: A 47-year-old woman presented to emergency room with disturbed level of consciousness, shortness of breath and multiple patches of skin discoloration. On examination, the patient was semi-conscious, with multiple ecchymosis and bilateral decreased air entry. Computed tomography scan of the neck and chest showed six rib fractures on the left side, and eight rib fractures on the right side, sternal fracture, manubriosternal dislocation, bilateral hemothorax, fracture of body of eleventh thoracic vertebra, and fracture of cervical spine of fifth and seventh vertebrae. The patient was intubated and admitted to intensive care unit. She was discharged with good health condition after 23 days of hospital admission. Conclusion: GBV is still a cause of severe trauma that puts the patient’s life at risk.

  4. Another cause for conductive hearing loss with present acoustic reflexes.

    Science.gov (United States)

    Ebert, Charles S; Zanation, Adam M; Buchman, Craig A

    2008-11-01

    There are numerous potential causes of conductive hearing loss (HL). It is important to obtain a thorough history and perform a complete examination, including audiometric testing and radiographic evaluation when necessary. In this report, we present a patient with an intact tympanic membrane, no history of ear disease or trauma who as an adult developed progressive, conductive HL because of an anomalous course of a dehiscent facial nerve. In the patient with a conductive HL and at least partially intact reflexes, superior semicircular canal dehiscence, fracture of the stapes superstructure proximal to the tendon, other third window phenomena, and now dehiscence of the facial nerve resulting in decreased mobility of the ossicular chain must be considered.

  5. [Gastroduodenal intussusception causing gastric retention.

    DEFF Research Database (Denmark)

    Alamili, M.; Berg, J.O.; Lindstrom, C.

    2008-01-01

    A case of gastroduodenal intussusception caused by a duodenal lipoma is presented. The condition was characterized by severe upper gastrointestinal retention, epigastric pain and weight loss. The mass was diagnosed by CT scan. The diagnosis was confirmed by operation. The patient was treated...

  6. Relation between diagnoses on severity, sick leave and loss of job among patients with occupational hand eczema

    DEFF Research Database (Denmark)

    Cvetkovski, Rikke Skoet; Rothman, Kenneth J; Olsen, J

    2005-01-01

    the worst prognosis. OBJECTIVES: To evaluate and compare the severity and consequences of recognized OHE in different diagnostic and subdiagnostic groups. METHODS: Between October 2001 and November 2002, all new cases of recognized OHE were identified from the Danish National Board of Industrial Injuries...... occupation and loss of job. RESULTS: The overall response rate to the questionnaire was 82%. We observed substantially greater severity among those with occupational irritant contact dermatitis (ICD) and AD than for any other diagnoses. Age above 50 years was also associated with increased severity of OHE...... that they had lost their job at least once during the past 12 months due to OHE. The only strong association with loss of job was food-related occupations. CONCLUSIONS: Occupational ICD and AD appear to be strongly associated with severity of OHE. AD and severity of OHE were independently associated...

  7. Mortality caused by intracranial bleeding in non-severe hemophilia A patients.

    Science.gov (United States)

    Loomans, J I; Eckhardt, C L; Reitter-Pfoertner, S E; Holmström, M; van Gorkom, B Laros; Leebeek, F W G; Santoro, C; Haya, S; Meijer, K; Nijziel, M R; van der Bom, J G; Fijnvandraat, K

    2017-06-01

    Essentials Data on bleeding-related causes of death in non-severe hemophilia A (HA) patients are scarce. Such data may provide new insights into areas of care that can be improved. Non-severe HA patients have an increased risk of dying from intracranial bleeding. This demonstrates the need for specialized care for non-severe HA patients. Background Non-severe hemophilia (factor VIII concentration [FVIII:C] of 2-40 IU dL -1 ) is characterized by a milder bleeding phenotype than severe hemophilia A. However, some patients with non-severe hemophilia A suffer from severe bleeding complications that may result in death. Data on bleeding-related causes of death, such as fatal intracranial bleeding, in non-severe patients are scarce. Such data may provide new insights into areas of care that can be improved. Aims To describe mortality rates, risk factors and comorbidities associated with fatal intracranial bleeding in non-severe hemophilia A patients. Methods We analyzed data from the INSIGHT study, an international cohort study of all non-severe hemophilia A patients treated with FVIII concentrates during the observation period between 1980 and 2010 in 34 participating centers across Europe and Australia. Clinical data and vital status were collected from 2709 patients. We report the standardized mortality rate for patients who suffered from fatal intracranial bleeding, using a general European male population as a control population. Results Twelve per cent of the 148 deceased patients in our cohort of 2709 patients died from intracranial bleeding. The mortality rate between 1996 and 2010 for all ages was 3.5-fold higher than that in the general population (95% confidence interval [CI] 2.0-5.8). Patients who died from intracranial bleeding mostly presented with mild hemophilia without clear comorbidities. Conclusion Non-severe hemophilia A patients have an increased risk of dying from intracranial bleeding in comparison with the general population. This demonstrates the

  8. Mechanisms causing reduced Arctic sea ice loss in a coupled climate model

    Directory of Open Access Journals (Sweden)

    A. E. West

    2013-03-01

    Full Text Available The fully coupled climate model HadGEM1 produces one of the most accurate simulations of the historical record of Arctic sea ice seen in the IPCC AR4 multi-model ensemble. In this study, we examine projections of sea ice decline out to 2030, produced by two ensembles of HadGEM1 with natural and anthropogenic forcings included. These ensembles project a significant slowing of the rate of ice loss to occur after 2010, with some integrations even simulating a small increase in ice area. We use an energy budget of the Arctic to examine the causes of this slowdown. A negative feedback effect by which rapid reductions in ice thickness north of Greenland reduce ice export is found to play a major role. A slight reduction in ocean-to-ice heat flux in the relevant period, caused by changes in the meridional overturning circulation (MOC and subpolar gyre in some integrations, as well as freshening of the mixed layer driven by causes other than ice melt, is also found to play a part. Finally, we assess the likelihood of a slowdown occurring in the real world due to these causes.

  9. The loss of efficiency caused by agents' uncoordinated routing in transport networks.

    Science.gov (United States)

    Xu, Zhongzhi; Sun, Li; Wang, Junjie; Wang, Pu

    2014-01-01

    Large-scale daily commuting data were combined with detailed geographical information system (GIS) data to analyze the loss of transport efficiency caused by drivers' uncoordinated routing in urban road networks. We used Price of Anarchy (POA) to quantify the loss of transport efficiency and found that both volume and distribution of human mobility demand determine the POA. In order to reduce POA, a small number of highways require considerable decreases in traffic, and their neighboring arterial roads need to attract more traffic. The magnitude of the adjustment in traffic flow can be estimated using the fundamental measure traffic flow only, which is widely available and easy to collect. Surprisingly, the most congested roads or the roads with largest traffic flow were not those requiring the most reduction of traffic. This study can offer guidance for the optimal control of urban traffic and facilitate improvements in the efficiency of transport networks.

  10. Upper gastrointestinal bleeding caused by severe esophagitis: a unique clinical syndrome.

    Science.gov (United States)

    Guntipalli, Prathima; Chason, Rebecca; Elliott, Alan; Rockey, Don C

    2014-12-01

    We have recognized a unique clinical syndrome in patients with upper gastrointestinal bleeding who are found to have severe esophagitis. We aimed to more clearly describe the clinical entity of upper gastrointestinal bleeding in patients with severe esophagitis. We conducted a retrospective matched case-control study designed to investigate clinical features in patients with carefully defined upper gastrointestinal bleeding and severe esophagitis. Patient data were captured prospectively via a Gastrointestinal Bleeding Healthcare Registry, which collects data on all patients admitted with gastrointestinal bleeding. Patients with endoscopically documented esophagitis (cases) were matched with randomly selected controls that had upper gastrointestinal bleeding caused by other lesions. Epidemiologic features in patients with esophagitis were similar to those with other causes of upper gastrointestinal bleeding. However, hematemesis was more common in patients with esophagitis 86% (102/119) than in controls 55% (196/357) (p bleeding than those without cirrhosis. We have described a unique clinical syndrome in patients with upper gastrointestinal bleeding who have erosive esophagitis. This syndrome is manifest by typical clinical features and is associated with favorable outcomes.

  11. [Gastroduodenal intussusception causing gastric retention.

    DEFF Research Database (Denmark)

    Alamili, M.; Berg, J.O.; Lindstrom, C.

    2008-01-01

    A case of gastroduodenal intussusception caused by a duodenal lipoma is presented. The condition was characterized by severe upper gastrointestinal retention, epigastric pain and weight loss. The mass was diagnosed by CT scan. The diagnosis was confirmed by operation. The patient was treated succ...

  12. Hypocretin (orexin) loss in Alzheimer's disease.

    NARCIS (Netherlands)

    Fronczek, R.; Geest, S. de; Frolich, M.; Overeem, S.; Roelandse, F.W.; Lammers, G.J.; Swaab, D.F.

    2012-01-01

    Sleep disturbances in Alzheimer's disease (AD) patients are associated with the severity of dementia and are often the primary reason for institutionalization. These sleep problems partly resemble core symptoms of narcolepsy, a sleep disorder caused by a general loss of the neurotransmitter

  13. Hypocretin (orexin) loss in Alzheimer's disease

    NARCIS (Netherlands)

    Fronczek, Rolf; van Geest, Sarita; Frölich, Marijke; Overeem, Sebastiaan; Roelandse, Freek W. C.; Lammers, Gert Jan; Swaab, Dick F.

    2012-01-01

    Sleep disturbances in Alzheimer's disease (AD) patients are associated with the severity of dementia and are often the primary reason for institutionalization. These sleep problems partly resemble core symptoms of narcolepsy, a sleep disorder caused by a general loss of the neurotransmitter

  14. Climate impact of idealized winter polar mesospheric and stratospheric ozone losses as caused by energetic particle precipitation

    Directory of Open Access Journals (Sweden)

    K. Meraner

    2018-01-01

    Full Text Available Energetic particles enter the polar atmosphere and enhance the production of nitrogen oxides and hydrogen oxides in the winter stratosphere and mesosphere. Both components are powerful ozone destroyers. Recently, it has been inferred from observations that the direct effect of energetic particle precipitation (EPP causes significant long-term mesospheric ozone variability. Satellites observe a decrease in mesospheric ozone up to 34 % between EPP maximum and EPP minimum. Stratospheric ozone decreases due to the indirect effect of EPP by about 10–15 % observed by satellite instruments. Here, we analyze the climate impact of winter boreal idealized polar mesospheric and polar stratospheric ozone losses as caused by EPP in the coupled Max Planck Institute Earth System Model (MPI-ESM. Using radiative transfer modeling, we find that the radiative forcing of mesospheric ozone loss during polar night is small. Hence, climate effects of mesospheric ozone loss due to energetic particles seem unlikely. Stratospheric ozone loss due to energetic particles warms the winter polar stratosphere and subsequently weakens the polar vortex. However, those changes are small, and few statistically significant changes in surface climate are found.

  15. Primary and Secondary Yield Losses Caused by Pests and Diseases: Assessment and Modeling in Coffee.

    Science.gov (United States)

    Cerda, Rolando; Avelino, Jacques; Gary, Christian; Tixier, Philippe; Lechevallier, Esther; Allinne, Clémentine

    2017-01-01

    The assessment of crop yield losses is needed for the improvement of production systems that contribute to the incomes of rural families and food security worldwide. However, efforts to quantify yield losses and identify their causes are still limited, especially for perennial crops. Our objectives were to quantify primary yield losses (incurred in the current year of production) and secondary yield losses (resulting from negative impacts of the previous year) of coffee due to pests and diseases, and to identify the most important predictors of coffee yields and yield losses. We established an experimental coffee parcel with full-sun exposure that consisted of six treatments, which were defined as different sequences of pesticide applications. The trial lasted three years (2013-2015) and yield components, dead productive branches, and foliar pests and diseases were assessed as predictors of yield. First, we calculated yield losses by comparing actual yields of specific treatments with the estimated attainable yield obtained in plots which always had chemical protection. Second, we used structural equation modeling to identify the most important predictors. Results showed that pests and diseases led to high primary yield losses (26%) and even higher secondary yield losses (38%). We identified the fruiting nodes and the dead productive branches as the most important and useful predictors of yields and yield losses. These predictors could be added in existing mechanistic models of coffee, or can be used to develop new linear mixed models to estimate yield losses. Estimated yield losses can then be related to production factors to identify corrective actions that farmers can implement to reduce losses. The experimental and modeling approaches of this study could also be applied in other perennial crops to assess yield losses.

  16. A "high severity" spruce beetle outbreak in Wyoming causes moderate-severity carbon cycle perturbations

    Science.gov (United States)

    Berryman, E.; Frank, J. M.; Speckman, H. N.; Bradford, J. B.; Ryan, M. G.; Massman, W. J.; Hawbaker, T. J.

    2017-12-01

    Bark beetle outbreaks in Western North American forests are often considered a high-severity disturbance from a carbon (C) cycling perspective, but field measurements that quantify impacts on C dynamics are very limited. Often, factors out of the researcher's control complicate the separation of beetle impacts from other drivers of C cycling variability and restrict statistical inference. Fortuitously, we had four years of pre-spruce beetle outbreak C cycle measurements in a subalpine forest in southeastern Wyoming (Glacier Lakes Ecosystem Experiments Site, or GLEES) and sustained intermittent monitoring for nearly a decade after the outbreak. Here, we synthesize published and unpublished pre- and post-outbreak measurements of key C cycle stocks and fluxes at GLEES. Multiple lines of evidence, including chamber measurements, eddy covariance measurements, and tracking of soil and forest floor C pools over time, point to the GLEES outbreak as a moderate-severity disturbance for C loss to the atmosphere, despite 70% to 80% of overstory tree death. Reductions in NEE were short-lived and the forest quickly returned to a carbon-neutral state, likely driven by an uptick in understory growth. Effect of mortality on the C cycle was asymmetrical, with a 50% reduction in net carbon uptake (NEE) two years into the outbreak, yet no measureable change in either ecosystem or growing season soil respiration. A small pulse in soil respiration occurred but was only detectable during the winter and amounted to < 10% of NEE. Possible reasons for the lack of measureable respiration response are discussed with emphasis on lessons learned for monitoring and modeling future outbreaks. We suggest a comprehensive assessment and definition of "moderate-severity" disturbances for Western forests and suggest that all tree mortality events may not be high-severity when it comes to C fluxes.

  17. Mutations in the Heme Exporter FLVCR1 Cause Sensory Neurodegeneration with Loss of Pain Perception.

    Science.gov (United States)

    Chiabrando, Deborah; Castori, Marco; di Rocco, Maja; Ungelenk, Martin; Gießelmann, Sebastian; Di Capua, Matteo; Madeo, Annalisa; Grammatico, Paola; Bartsch, Sophie; Hübner, Christian A; Altruda, Fiorella; Silengo, Lorenzo; Tolosano, Emanuela; Kurth, Ingo

    2016-12-01

    Pain is necessary to alert us to actual or potential tissue damage. Specialized nerve cells in the body periphery, so called nociceptors, are fundamental to mediate pain perception and humans without pain perception are at permanent risk for injuries, burns and mutilations. Pain insensitivity can be caused by sensory neurodegeneration which is a hallmark of hereditary sensory and autonomic neuropathies (HSANs). Although mutations in several genes were previously associated with sensory neurodegeneration, the etiology of many cases remains unknown. Using next generation sequencing in patients with congenital loss of pain perception, we here identify bi-allelic mutations in the FLVCR1 (Feline Leukemia Virus subgroup C Receptor 1) gene, which encodes a broadly expressed heme exporter. Different FLVCR1 isoforms control the size of the cytosolic heme pool required to sustain metabolic activity of different cell types. Mutations in FLVCR1 have previously been linked to vision impairment and posterior column ataxia in humans, but not to HSAN. Using fibroblasts and lymphoblastoid cell lines from patients with sensory neurodegeneration, we here show that the FLVCR1-mutations reduce heme export activity, enhance oxidative stress and increase sensitivity to programmed cell death. Our data link heme metabolism to sensory neuron maintenance and suggest that intracellular heme overload causes early-onset degeneration of pain-sensing neurons in humans.

  18. Mutations in the Heme Exporter FLVCR1 Cause Sensory Neurodegeneration with Loss of Pain Perception.

    Directory of Open Access Journals (Sweden)

    Deborah Chiabrando

    2016-12-01

    Full Text Available Pain is necessary to alert us to actual or potential tissue damage. Specialized nerve cells in the body periphery, so called nociceptors, are fundamental to mediate pain perception and humans without pain perception are at permanent risk for injuries, burns and mutilations. Pain insensitivity can be caused by sensory neurodegeneration which is a hallmark of hereditary sensory and autonomic neuropathies (HSANs. Although mutations in several genes were previously associated with sensory neurodegeneration, the etiology of many cases remains unknown. Using next generation sequencing in patients with congenital loss of pain perception, we here identify bi-allelic mutations in the FLVCR1 (Feline Leukemia Virus subgroup C Receptor 1 gene, which encodes a broadly expressed heme exporter. Different FLVCR1 isoforms control the size of the cytosolic heme pool required to sustain metabolic activity of different cell types. Mutations in FLVCR1 have previously been linked to vision impairment and posterior column ataxia in humans, but not to HSAN. Using fibroblasts and lymphoblastoid cell lines from patients with sensory neurodegeneration, we here show that the FLVCR1-mutations reduce heme export activity, enhance oxidative stress and increase sensitivity to programmed cell death. Our data link heme metabolism to sensory neuron maintenance and suggest that intracellular heme overload causes early-onset degeneration of pain-sensing neurons in humans.

  19. Severe Bone Loss induced by Orthodontic Elastic Separator: A Rare Case Report

    OpenAIRE

    A E Vishwanath; B K Sharmada; Sandesh S Pai; Nandini Nelvigi

    2013-01-01

    A displaced orthodontic elastic separator was proposed as being the source of a gingival abscess that progressed to severe bone loss and exfoliation in a healthy adolescent patient with sound periodontal status prior to commencement of orthodontic treatment. After 1 year of undergoing orthodontic treatment, the patient presented with dull pain and mobility in the left upper permanent molar for which there was no apparent etiology. On clinical examination, the patient had gingival inflammation...

  20. Hyper-responsivity to losses in the anterior insula during economic choice scales with depression severity.

    Science.gov (United States)

    Engelmann, J B; Berns, G S; Dunlop, B W

    2017-12-01

    Commonly observed distortions in decision-making among patients with major depressive disorder (MDD) may emerge from impaired reward processing and cognitive biases toward negative events. There is substantial theoretical support for the hypothesis that MDD patients overweight potential losses compared with gains, though the neurobiological underpinnings of this bias are uncertain. Twenty-one unmedicated patients with MDD were compared with 25 healthy controls (HC) using functional magnetic resonance imaging (fMRI) together with an economic decision-making task over mixed lotteries involving probabilistic gains and losses. Region-of-interest analyses evaluated neural signatures of gain and loss coding within a core network of brain areas known to be involved in valuation (anterior insula, caudate nucleus, ventromedial prefrontal cortex). Usable fMRI data were available for 19 MDD and 23 HC subjects. Anterior insula signal showed negative coding of losses (gain > loss) in HC subjects consistent with previous findings, whereas MDD subjects demonstrated significant reversals in these associations (loss > gain). Moreover, depression severity further enhanced the positive coding of losses in anterior insula, ventromedial prefrontal cortex, and caudate nucleus. The hyper-responsivity to losses displayed by the anterior insula of MDD patients was paralleled by a reduced influence of gain, but not loss, stake size on choice latencies. Patients with MDD demonstrate a significant shift from negative to positive coding of losses in the anterior insula, revealing the importance of this structure in value-based decision-making in the context of emotional disturbances.

  1. Trends in the incidence and causes of severe visual impairment and blindness in children from Israel.

    Science.gov (United States)

    Mezer, Eedy; Chetrit, Angela; Kalter-Leibovici, Ofra; Kinori, Michael; Ben-Zion, Itay; Wygnanski-Jaffe, Tamara

    2015-06-01

    To describe trends in the incidence and causes of legal childhood blindness in Israel, one of the few countries worldwide that maintain a national registry of the blind. We performed a historical cohort study of annual reports of the National Registry of the Blind (NRB) between 1999 and 2013. All data regarding demographic information, year of registration and cause of blindness of children 0-18 years of age registered for blind certification were obtained from the annual reports of the NRB. Causes of legal blindness analyzed were optic atrophy, retinitis pigmentosa, retinopathy of prematurity (ROP), albinism, other retinal disorders, cataract, and glaucoma. The main outcome measure was the incidence of new cases of certified legal blindness. The incidence of newly registered legally blind children in Israel almost halved from 7.7 per 100,000 in 1999 to 3.1 per 100,000 in 2013. The decline was mainly attributable to a decreased incidence of blindness resulting from retinitis pigmentosa and ROP. The incidence of registered cases due to cerebral visual impairment increased. During the past decade the incidence of severe childhood visual impairment and blindness declined in Israel. A continuous decline in consanguineous marriages among the Jewish and Arab populations in Israel may have contributed to the decrease in the rate of vision loss due to retinitis pigmentosa in children. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  2. The loss of efficiency caused by agents' uncoordinated routing in transport networks.

    Directory of Open Access Journals (Sweden)

    Zhongzhi Xu

    Full Text Available Large-scale daily commuting data were combined with detailed geographical information system (GIS data to analyze the loss of transport efficiency caused by drivers' uncoordinated routing in urban road networks. We used Price of Anarchy (POA to quantify the loss of transport efficiency and found that both volume and distribution of human mobility demand determine the POA. In order to reduce POA, a small number of highways require considerable decreases in traffic, and their neighboring arterial roads need to attract more traffic. The magnitude of the adjustment in traffic flow can be estimated using the fundamental measure traffic flow only, which is widely available and easy to collect. Surprisingly, the most congested roads or the roads with largest traffic flow were not those requiring the most reduction of traffic. This study can offer guidance for the optimal control of urban traffic and facilitate improvements in the efficiency of transport networks.

  3. Attitudes towards and perceptions of visual loss and its causes among Hong Kong Chinese adults.

    Science.gov (United States)

    Lau, Joseph Tak Fai; Lee, Vincent; Fan, Dorothy; Lau, Mason; Michon, John

    2004-06-01

    As part of a study of visual function among Hong Kong Chinese adults, their attitudes and perceptions related to visual loss were examined. These included fear of visual loss, negative functional impacts of visual loss, the relationship between ageing and visual loss and help-seeking behaviours related to visual loss. Demographic factors associated with these variables were also studied. The study population were people aged 40 and above randomly selected from the Shatin district of Hong Kong. The participants underwent eye examinations that included visual acuity, intraocular pressure measurement, visual field, slit-lamp biomicroscopy and ophthalmoscopy. The primary cause of visual disability was recorded. The participants were also asked about their attitudes and perceptions regarding visual loss using a structured questionnaire. The prevalence of bilateral visual disability was 2.2% among adults aged 40 or above and 6.4% among adults aged 60 or above. Nearly 36% of the participants selected blindness as the most feared disabling medical condition, which was substantially higher than conditions such as dementia, loss of limbs, deafness or aphasia. Inability to take care of oneself (21.0%), inconvenience related to mobility (20.2%) and inability to work (14.8%) were the three most commonly mentioned 'worst impact' effects of visual loss. Fully 68% of the participants believed that loss of vision is related to ageing. A majority of participants would seek help and advice from family members in case of visual loss. Visual function is perceived to be very important by Hong Kong Chinese adults. The fear of visual loss is widespread and particularly affects self-care and functional abilities. Visual loss is commonly seen as related to ageing. Attitudes and perceptions in this population may be modified by educational and outreach efforts in order to take advantage of preventive measures.

  4. Vitamin C reverses hypogonadal bone loss

    Science.gov (United States)

    Epidemiologic studies correlate low vitamin C intake with bone loss. The genetic deletion of enzymes involved in de novo vitamin C synthesis in mice, likewise, causes severe osteoporosis. However, very few studies have evaluated a protective role of this dietary supplement on the skeleton. Here, ...

  5. A Novel Mutation in the EDAR Gene Causes Severe Autosomal Recessive Hypohidrotic Ectodermal Dysplasia

    DEFF Research Database (Denmark)

    Henningsen, Emil; Svendsen, Mathias Tiedemann; Lildballe, D. L.

    2014-01-01

    We report on a 2-year-old girl presenting with a severe form of hypohidrotic ectodermal dysplasia (HED). The patient presented with hypotrichosis, anodontia, hypohidrosis, frontal bossing, prominent lips and ears, dry, pale skin, and dermatitis. The patient had chronic rhinitis with malodorous......-mediated NF-kB signalling. This complete loss-of-function mutation likely accounts for the severe clinical abnormalities in ectodermal structures in the described patient. (C) 2014 Wiley Periodicals, Inc....

  6. ["Loss of breath" as a cause of postoperative hypoxia and bradycardia in children submitted to tonsillectomy].

    Science.gov (United States)

    Moro, Eduardo Toshiyuki; Goulart, Alexandre Palmeira

    2015-01-01

    The "shortness of breath" or "breathing interruption" crisis can be considered a cause of hypoxia in childhood. It is characterized by the presence of a triggering factor followed by weeping and apnea in expiration accompanied by cyanosis or pallor. The sequence of events may include bradycardia, loss of consciousness, abnormal postural toneand even asystole. A review of the literature revealed only two reports of postoperative apneacaused by "shortness of breath". This article describes the case of a child with a history of "shortness of breath" undiagnosed before the adenotonsillectomy, but that represented the cause of episodes of hypoxemia and bradycardia in the postoperative period. the "shortness of breath" crisis should be considered as a possible cause of perioperative hypoxia in children, especially when there is a history suggestive of this problem. As some events may be accompanied by bradycardia, loss of consciousness, abnormal postural tone and even asystole, observation in a hospital setting should be considered. Copyright © 2013 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  7. Does job loss cause ill health?

    NARCIS (Netherlands)

    Salm, M.

    2009-01-01

    This study estimates the effect of job loss on health for near elderly employees based on longitudinal data from the Health and Retirement Study. Previous studies find a strong negative correlation between unemployment and health. To control for possible reverse causality, this study focuses on

  8. Attribution of Causes of Weight Loss and Weight Gain to 3-Year Mortality in Older Adults: Results From the Longitudinal Aging Study Amsterdam

    NARCIS (Netherlands)

    Wijnhoven, H.A.H.; van Zon, S.K.R.; Twisk, J.; Visser, M.

    2014-01-01

    Background: Weight loss is associated with a higher mortality risk in old age, but the underlying cause may impact this association. We examined associations between causes of intentional and unintentional weight loss and weight gain and mortality. Methods: We used data of five triannual examination

  9. Attribution of Causes of Weight Loss and Weight Gain to 3-Year Mortality in Older Adults : Results From the Longitudinal Aging Study Amsterdam

    NARCIS (Netherlands)

    Wijnhoven, Hanneke A. H.; van Zon, Sander K. R.; Twisk, Jos; Visser, Marjolein

    2014-01-01

    Background. Weight loss is associated with a higher mortality risk in old age, but the underlying cause may impact this association. We examined associations between causes of intentional and unintentional weight loss and weight gain and mortality. Methods. We used data of five triannual examination

  10. Loss of VPS13C Function in Autosomal-Recessive Parkinsonism Causes Mitochondrial Dysfunction and Increases PINK1/Parkin-Dependent Mitophagy.

    Science.gov (United States)

    Lesage, Suzanne; Drouet, Valérie; Majounie, Elisa; Deramecourt, Vincent; Jacoupy, Maxime; Nicolas, Aude; Cormier-Dequaire, Florence; Hassoun, Sidi Mohamed; Pujol, Claire; Ciura, Sorana; Erpapazoglou, Zoi; Usenko, Tatiana; Maurage, Claude-Alain; Sahbatou, Mourad; Liebau, Stefan; Ding, Jinhui; Bilgic, Basar; Emre, Murat; Erginel-Unaltuna, Nihan; Guven, Gamze; Tison, François; Tranchant, Christine; Vidailhet, Marie; Corvol, Jean-Christophe; Krack, Paul; Leutenegger, Anne-Louise; Nalls, Michael A; Hernandez, Dena G; Heutink, Peter; Gibbs, J Raphael; Hardy, John; Wood, Nicholas W; Gasser, Thomas; Durr, Alexandra; Deleuze, Jean-François; Tazir, Meriem; Destée, Alain; Lohmann, Ebba; Kabashi, Edor; Singleton, Andrew; Corti, Olga; Brice, Alexis

    2016-03-03

    Autosomal-recessive early-onset parkinsonism is clinically and genetically heterogeneous. The genetic causes of approximately 50% of autosomal-recessive early-onset forms of Parkinson disease (PD) remain to be elucidated. Homozygozity mapping and exome sequencing in 62 isolated individuals with early-onset parkinsonism and confirmed consanguinity followed by data mining in the exomes of 1,348 PD-affected individuals identified, in three isolated subjects, homozygous or compound heterozygous truncating mutations in vacuolar protein sorting 13C (VPS13C). VPS13C mutations are associated with a distinct form of early-onset parkinsonism characterized by rapid and severe disease progression and early cognitive decline; the pathological features were striking and reminiscent of diffuse Lewy body disease. In cell models, VPS13C partly localized to the outer membrane of mitochondria. Silencing of VPS13C was associated with lower mitochondrial membrane potential, mitochondrial fragmentation, increased respiration rates, exacerbated PINK1/Parkin-dependent mitophagy, and transcriptional upregulation of PARK2 in response to mitochondrial damage. This work suggests that loss of function of VPS13C is a cause of autosomal-recessive early-onset parkinsonism with a distinctive phenotype of rapid and severe progression. Copyright © 2016 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  11. Root causes and impacts of severe accidents at large nuclear power plants.

    Science.gov (United States)

    Högberg, Lars

    2013-04-01

    The root causes and impacts of three severe accidents at large civilian nuclear power plants are reviewed: the Three Mile Island accident in 1979, the Chernobyl accident in 1986, and the Fukushima Daiichi accident in 2011. Impacts include health effects, evacuation of contaminated areas as well as cost estimates and impacts on energy policies and nuclear safety work in various countries. It is concluded that essential objectives for reactor safety work must be: (1) to prevent accidents from developing into severe core damage, even if they are initiated by very unlikely natural or man-made events, and, recognizing that accidents with severe core damage may nevertheless occur; (2) to prevent large-scale and long-lived ground contamination by limiting releases of radioactive nuclides such as cesium to less than about 100 TBq. To achieve these objectives the importance of maintaining high global standards of safety management and safety culture cannot be emphasized enough. All three severe accidents discussed in this paper had their root causes in system deficiencies indicative of poor safety management and poor safety culture in both the nuclear industry and government authorities.

  12. Root Causes and Impacts of Severe Accidents at Large Nuclear Power Plants

    International Nuclear Information System (INIS)

    Hoegberg, Lars

    2013-01-01

    The root causes and impacts of three severe accidents at large civilian nuclear power plants are reviewed: the Three Mile Island accident in 1979, the Chernobyl accident in 1986, and the Fukushima Daiichi accident in 2011. Impacts include health effects, evacuation of contaminated areas as well as cost estimates and impacts on energy policies and nuclear safety work in various countries. It is concluded that essential objectives for reactor safety work must be: (1) to prevent accidents from developing into severe core damage, even if they are initiated by very unlikely natural or man-made events, and, recognizing that accidents with severe core damage may nevertheless occur; (2) to prevent large-scale and long lived ground contamination by limiting releases of radioactive nuclides such as cesium to less than about 100 TBq. To achieve these objectives the importance of maintaining high global standards of safety management and safety culture cannot be emphasized enough. All three severe accidents discussed in this paper had their root causes in system deficiencies indicative of poor safety management and poor safety culture in both the nuclear industry and government authorities

  13. Root Causes and Impacts of Severe Accidents at Large Nuclear Power Plants

    Energy Technology Data Exchange (ETDEWEB)

    Hoegberg, Lars

    2013-04-15

    The root causes and impacts of three severe accidents at large civilian nuclear power plants are reviewed: the Three Mile Island accident in 1979, the Chernobyl accident in 1986, and the Fukushima Daiichi accident in 2011. Impacts include health effects, evacuation of contaminated areas as well as cost estimates and impacts on energy policies and nuclear safety work in various countries. It is concluded that essential objectives for reactor safety work must be: (1) to prevent accidents from developing into severe core damage, even if they are initiated by very unlikely natural or man-made events, and, recognizing that accidents with severe core damage may nevertheless occur; (2) to prevent large-scale and long lived ground contamination by limiting releases of radioactive nuclides such as cesium to less than about 100 TBq. To achieve these objectives the importance of maintaining high global standards of safety management and safety culture cannot be emphasized enough. All three severe accidents discussed in this paper had their root causes in system deficiencies indicative of poor safety management and poor safety culture in both the nuclear industry and government authorities.

  14. Frequency, severity and causes of unexpected allergic reactions to food: a systematic literature review.

    Science.gov (United States)

    Versluis, A; Knulst, A C; Kruizinga, A G; Michelsen, A; Houben, G F; Baumert, J L; van Os-Medendorp, H

    2015-02-01

    Food allergic patients have to deal with an avoidance diet. Confusing labelling terms or precautionary labels can result in misinterpretation and risk-taking behaviour. Even those patients that strictly adhere to their diet experience (sometimes severe) unexpected allergic reactions to food. The frequency, severity and causes of such reactions are unknown. The objective of this review was to describe the frequency, severity and causes of unexpected allergic reactions to food in food allergic patients aged > 12 years, in order to develop improved strategies to deal with their allergy. A systematic review was carried out by two researchers, in six electronic databases (CINAHL, Cochrane, EMBASE, Medline, Psychinfo and Scopus). The search was performed with keywords relating to the frequency, severity and causes of unexpected allergic reactions to food. This resulted in 24 studies which met the inclusion criteria; 18 observational and six qualitative studies. This review shows that knowledge about the frequency of unexpected reactions is limited. Peanut, nuts, egg, fruit/vegetables and milk are the main causal foods. Severe reactions and even fatalities occur. Most reactions take place at home, but a significant number also take place when eating at friends' houses or in restaurants. Labelling issues, but also attitude and risky behaviour of patients can attribute to unexpected reactions. We conclude that prospective studies are needed to get more insight in the frequency, severity, quantity of unintended allergen ingested and causes of unexpected allergic reactions to food, to be able to optimize strategies to support patients in dealing with their food allergy. Although the exact frequency is not known, unexpected reactions to food occur in a significant number of patients and can be severe. For clinical practice, this means that patient education and dietary instructions are necessary. © 2014 John Wiley & Sons Ltd.

  15. Loss of Sfpq Causes Long-Gene Transcriptopathy in the Brain

    Directory of Open Access Journals (Sweden)

    Akihide Takeuchi

    2018-05-01

    Full Text Available Summary: Genes specifically expressed in neurons contain members with extended long introns. Longer genes present a problem with respect to fulfilment of gene length transcription, and evidence suggests that dysregulation of long genes is a mechanism underlying neurodegenerative and psychiatric disorders. Here, we report the discovery that RNA-binding protein Sfpq is a critical factor for maintaining transcriptional elongation of long genes. We demonstrate that Sfpq co-transcriptionally binds to long introns and is required for sustaining long-gene transcription by RNA polymerase II through mediating the interaction of cyclin-dependent kinase 9 with the elongation complex. Phenotypically, Sfpq disruption caused neuronal apoptosis in developing mouse brains. Expression analysis of Sfpq-regulated genes revealed specific downregulation of developmentally essential neuronal genes longer than 100 kb in Sfpq-disrupted brains; those genes are enriched in associations with neurodegenerative and psychiatric diseases. The identified molecular machinery yields directions for targeted investigations of the association between long-gene transcriptopathy and neuronal diseases. : It has been a long-standing question how mammalian neuronal cells achieve full gene length transcription of extra-long genes. Takeuchi et al. show that RNA-binding protein Sfpq sustains long-gene transcription through Pol II-CTD activation. Loss of Sfpq caused long-gene transcriptopathy, which could be the cause of neurodegenerative and psychiatric disorders. Keywords: RNA-binding protein, transcriptional regulation, RNA polymerase II, cyclin-dependent kinase 9, RBP/transcript-dependent elongation, long-gene transcriptotherapy, neuronal development, neurological and psychiatric diseases, long-gene diseases, long genopathies

  16. Loss of the smallest subunit of cytochrome c oxidase, COX8A, causes Leigh-like syndrome and epilepsy.

    Science.gov (United States)

    Hallmann, Kerstin; Kudin, Alexei P; Zsurka, Gábor; Kornblum, Cornelia; Reimann, Jens; Stüve, Burkhard; Waltz, Stephan; Hattingen, Elke; Thiele, Holger; Nürnberg, Peter; Rüb, Cornelia; Voos, Wolfgang; Kopatz, Jens; Neumann, Harald; Kunz, Wolfram S

    2016-02-01

    Isolated cytochrome c oxidase (complex IV) deficiency is one of the most frequent respiratory chain defects in humans and is usually caused by mutations in proteins required for assembly of the complex. Mutations in nuclear-encoded structural subunits are very rare. In a patient with Leigh-like syndrome presenting with leukodystrophy and severe epilepsy, we identified a homozygous splice site mutation in COX8A, which codes for the ubiquitously expressed isoform of subunit VIII, the smallest nuclear-encoded subunit of complex IV. The mutation, affecting the last nucleotide of intron 1, leads to aberrant splicing, a frame-shift in the highly conserved exon 2, and decreased amount of the COX8A transcript. The loss of the wild-type COX8A protein severely impairs the stability of the entire cytochrome c oxidase enzyme complex and manifests in isolated complex IV deficiency in skeletal muscle and fibroblasts, similar to the frequent c.845_846delCT mutation in the assembly factor SURF1 gene. Stability and activity of complex IV could be rescued in the patient's fibroblasts by lentiviral expression of wild-type COX8A. Our findings demonstrate that COX8A is indispensable for function of human complex IV and its mutation causes human disease. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  17. Technical and economic assessment of losses caused by rock swelling in mines of the Western Donbass

    Energy Technology Data Exchange (ETDEWEB)

    Pirskii, A A; Stovpnik, S N; Shmigol' , A V

    1988-07-01

    Describes mining geologic conditions in mines of the Pavlovgradugol' association where production increased by 1.7 times during the last 10 years. Floor swelling of 1.1 m/a occurs at depths over 300 m and with rock strength in side walls below 20 MPa. Annually 22 km of workings must undergo repair and 10.4% of crews does the repair work. Technical and economic consequences of floor swelling are analyzed. Annual outlays on maintenance of 1 m of roadways increased to 160-200 rubles. Annual losses on maintenance of workings per coal mining association lie within 300,000-500,000 rubles. At the im. Geroev Kosmosa and im. Leninskogo Komsomola Ukrainy mines these losses amount to 520,000-730,000 and 840,000-845,000 rubles respectively. A comprehensive analysis of production losses and delays in development workings caused by floor swelling is presented. Formulae for loss calculation are given.

  18. Application of the Finite Element Method to Reveal the Causes of Loss of Planeness of Hot-Rolled Steel Sheets during Laser Cutting

    Science.gov (United States)

    Garber, E. A.; Bolobanova, N. L.; Trusov, K. A.

    2018-01-01

    A finite element technique is developed to simulate the stresses and the strains during strip flattening to reveal the causes of the cutting-assisted loss of planeness of hot-rolled steel sheets processed in roller levelers. The loss of planeness is found to be caused by a nonuniform distribution of the flattening-induced longitudinal tensile stresses over the strip thickness and width. The application of tensile forces to a strip in a roller leveler decreases this nonuniformity and prevents loss of planeness in cutting.

  19. [Severe, subacute axonal polyneuropathy due to hypophosphatemia].

    NARCIS (Netherlands)

    Eijk, J.J.J. van; Abdo, W.F.; Deurwaarder, E. den; Zwarts, M.J.; Warrenburg, B.P.C. van de

    2010-01-01

    A 46-year-old man receiving tube feeding because of anorexia and weight loss developed progressive neurological symptoms initially resembling Guillain-Barre syndrome. Eventually axonal neuropathy due to severe hypophosphatemia was diagnosed. Hypophosphatemia can be caused by the so-called refeeding

  20. Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation

    DEFF Research Database (Denmark)

    Clayton, Emma L.; Mancuso, Renzo; Nielsen, Troels Tolstrup

    2017-01-01

    Frontotemporal dementia (FTD)-causing mutations in the CHMP2B gene lead to the generation of mutant C-terminally truncated CHMP2B. We report that transgenic mice expressing endogenous levels of mutant CHMP2B developed late-onset brain volume loss associated with frank neuronal loss and FTD-like c...

  1. Hepatopulmonary syndrome causing severe hypoxaemia

    DEFF Research Database (Denmark)

    Lyngsøe, Bente Kjær; Andersen, Mette Winther; Eriksen, Jan

    2014-01-01

    Dyspnoea is a common complaint in patients with chronic liver disease. Hepatopulmonary syndrome (HPS) is an important cause to be aware of in the setting of liver disease, dyspnoea and hypoxaemia. HPS causes microvascular dilatation, angiogenesis and arteriovenous bypassing. The patients suffer f...... from hypoxaemia in upright position and even during minimal psychical activity. Contrast echocardiography, using micro-bubbles as the contrast, is required to establish the diagnosis. No medical therapy is available, only liver transplantation can cure the disease....

  2. Retinopathy of prematurity as a major cause of severe visual impairment and blindness in children in schools for the blind in Guadalajara city, Mexico.

    Science.gov (United States)

    Zepeda-Romero, L C; Barrera-de-Leon, J C; Camacho-Choza, C; Gonzalez Bernal, C; Camarena-Garcia, E; Diaz-Alatorre, C; Gutierrez-Padilla, J A; Gilbert, C

    2011-11-01

    To determine the causes of blindness in students attending schools for the blind in Guadalajara city, Mexico and to assess the availability of screening for retinopathy of prematurity (ROP) in local neonatal intensive care units. Information on causes of blindness was obtained by interview with parents and teachers, review of records and examination. Causes of visual loss in children with a distance visual acuity of blind) were determined and classified according to the WHO's classification system for children. Of 153 children in the two participating schools, 144 were severely visual impaired or blind. Their ages ranged from 4 months to 15 years and 58% were female. ROP was the most common cause of visual loss (34.7%), followed by optic nerve lesions (17.4%) and glaucoma (14.6%). 25/59 (42.3%) children aged 0-4 years were blind from ROP compared with 6/32 (18.8%) children aged 10-15 years. 78% of children blind from ROP had psychomotor delay and less than half (46%) had not received treatment for ROP. All five privately funded neonatal intensive care units in the city regularly screen for ROP compared with only four of the 12 units in the public sector. ROP is the leading cause of blindness in children in Mexico despite national guidelines being in place. Health policies promoting primary prevention through improved neonatal care need to be implemented. Advocacy is required so that the time ophthalmologists spend screening and treating ROP is included in their job description and hence salaried.

  3. Morphosyntactic correctness of written language production in adults with moderate to severe congenital hearing loss

    NARCIS (Netherlands)

    Huysmans, Elke; de Jong, Jan; Festen, Joost M.; Coene, Martine M.R.; Goverts, S. Theo

    2017-01-01

    Objective To examine whether moderate to severe congenital hearing loss (MSCHL) leads to persistent morphosyntactic problems in the written language production of adults, as it does in their spoken language production. Design Samples of written language in Dutch were analysed for morphosyntactic

  4. Understanding yield loss and pathogen biology to improve disease management: Stagonospora nodorum blotch - a case study in wheat

    Science.gov (United States)

    The estimated potential yield losses caused by plant pathogens are up to 16% globally, and most research in plant pathology aims to reduce yield loss in crops directly or indirectly. Yield losses caused by a certain disease depend not only on disease severity, but also on weather factors, the pathog...

  5. Homozygous loss of function BRCA1 variant causing a Fanconi-anemia-like phenotype, a clinical report and review of previous patients.

    Science.gov (United States)

    Freire, Bruna L; Homma, Thais K; Funari, Mariana F A; Lerario, Antônio M; Leal, Aline M; Velloso, Elvira D R P; Malaquias, Alexsandra C; Jorge, Alexander A L

    2018-03-01

    Fanconi Anemia (FA) is a rare and heterogeneous genetic syndrome. It is associated with short stature, bone marrow failure, high predisposition to cancer, microcephaly and congenital malformation. Many genes have been associated with FA. Previously, two adult patients with biallelic pathogenic variant in Breast Cancer 1 gene (BRCA1) had been identified in Fanconi Anemia-like condition. The proband was a 2.5 year-old girl with severe short stature, microcephaly, neurodevelopmental delay, congenital heart disease and dysmorphic features. Her parents were third degree cousins. Routine screening tests for short stature was normal. We conducted whole exome sequencing (WES) of the proband and used an analysis pipeline to identify rare nonsynonymous genetic variants that cause short stature. We identified a homozygous loss-of-function BRCA1 mutation (c.2709T > A; p. Cys903*), which promotes the loss of critical domains of the protein. Cytogenetic study with DEB showed an increased chromosomal breakage. We screened heterozygous parents of the index case for cancer and we detected, in her mother, a metastatic adenocarcinoma in an axillar lymph node with probable primary site in the breast. It is possible to consolidate the FA-like phenotype associated with biallelic loss-of-function BRCA1, characterized by microcephaly, short stature, developmental delay, dysmorphic face features and cancer predisposition. In our case, the WES allowed to establish the genetic cause of short stature in the context of a chromosome instability syndrome. An identification of BRCA1 mutations in our patient allowed precise genetic counseling and also triggered cancer screening for the patient and her family members. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  6. Two Cases of Severe Combined Immunodeficiency Caused By Adenosine Deaminase Deficiency

    Directory of Open Access Journals (Sweden)

    Turkan Patiroglu

    2014-08-01

    Full Text Available Severe Combined Immune Deficiency (SCID is a primary immune deficiency disorder manifested with severe infections upon first months of life, which is characterized by diverse genetic defects in T and B lymphocyte functions and occasionally in NK cells. ADA deficiency is a form of SCID progressing with severe lymphopenia and immune deficiency caused by toxic metabolites of ADA. Bone marrow transplantation (BMT is the only curative treatment although prophylactic anti-microbial therapy, intravenous immunoglobulin (IVIG and enzyme replacement can achieve transient improvements. Early diagnosis before development of severe infections and organ injury and referral to pediatric immunology clinics will make considerable contributions to prognosis. Here, we presented 2 cousins with SCID who had positive family history with deceased sibling; presented with tanning at skin, severe neonatal infections and Q246X (c736C>T non-sense mutation in exon 8 in ADA gene  in order to emphasize this rare mutation and pediatric emergencies associated with this disorder.

  7. Edge-related loss of tree phylogenetic diversity in the severely fragmented Brazilian Atlantic forest.

    Directory of Open Access Journals (Sweden)

    Bráulio A Santos

    Full Text Available Deforestation and forest fragmentation are known major causes of nonrandom extinction, but there is no information about their impact on the phylogenetic diversity of the remaining species assemblages. Using a large vegetation dataset from an old hyper-fragmented landscape in the Brazilian Atlantic rainforest we assess whether the local extirpation of tree species and functional impoverishment of tree assemblages reduce the phylogenetic diversity of the remaining tree assemblages. We detected a significant loss of tree phylogenetic diversity in forest edges, but not in core areas of small (<80 ha forest fragments. This was attributed to a reduction of 11% in the average phylogenetic distance between any two randomly chosen individuals from forest edges; an increase of 17% in the average phylogenetic distance to closest non-conspecific relative for each individual in forest edges; and to the potential manifestation of late edge effects in the core areas of small forest remnants. We found no evidence supporting fragmentation-induced phylogenetic clustering or evenness. This could be explained by the low phylogenetic conservatism of key life-history traits corresponding to vulnerable species. Edge effects must be reduced to effectively protect tree phylogenetic diversity in the severely fragmented Brazilian Atlantic forest.

  8. Traumatic brain injury causes an FK506-sensitive loss and an overgrowth of dendritic spines in rat forebrain.

    Science.gov (United States)

    Campbell, John N; Register, David; Churn, Severn B

    2012-01-20

    Traumatic brain injury (TBI) causes both an acute loss of tissue and a progressive injury through reactive processes such as excitotoxicity and inflammation. These processes may worsen neural dysfunction by altering neuronal circuitry beyond the focally-damaged tissue. One means of circuit alteration may involve dendritic spines, micron-sized protuberances of dendritic membrane that support most of the excitatory synapses in the brain. This study used a modified Golgi-Cox technique to track changes in spine density on the proximal dendrites of principal cells in rat forebrain regions. Spine density was assessed at 1 h, 24 h, and 1 week after a lateral fluid percussion TBI of moderate severity. At 1 h after TBI, no changes in spine density were observed in any of the brain regions examined. By 24 h after TBI, however, spine density had decreased in ipsilateral neocortex in layer II and III and dorsal dentate gyrus (dDG). This apparent loss of spines was prevented by a single, post-injury administration of the calcineurin inhibitor FK506. These results, together with those of a companion study, indicate an FK506-sensitive mechanism of dendritic spine loss in the TBI model. Furthermore, by 1 week after TBI, spine density had increased substantially above control levels, bilaterally in CA1 and CA3 and ipsilaterally in dDG. The apparent overgrowth of spines in CA1 is of particular interest, as it may explain previous reports of abnormal and potentially epileptogenic activity in this brain region.

  9. PREGNANCY LOSS IN THE F344 RAT CAUSED BY BROMODICHLOROMETHANE: EFFECTS ON SERUM LUTEINIZING HORMONE LEVELS

    Science.gov (United States)

    PREGNANCY LOSS IN THE F344 RAT CAUSED BY BROMODICHLOROMETHANE: EFFECTS ON SERUM LUTEINIZING HORMONE LEVELS Bielmeier1, S.R., D.S. Best2, and M.G. Narotsky2; 1University of North Carolina at Chapel Hill, Curriculum in Toxicology, 2Reproductive Toxicology Division, U.S. Enviro...

  10. ALDH1A3 loss of function causes bilateral anophthalmia/microphthalmia and hypoplasia of the optic nerve and optic chiasm.

    Science.gov (United States)

    Yahyavi, Mani; Abouzeid, Hana; Gawdat, Ghada; de Preux, Anne-Sophie; Xiao, Tong; Bardakjian, Tanya; Schneider, Adele; Choi, Alex; Jorgenson, Eric; Baier, Herwig; El Sada, Mohamad; Schorderet, Daniel F; Slavotinek, Anne M

    2013-08-15

    The major active retinoid, all-trans retinoic acid, has long been recognized as critical for the development of several organs, including the eye. Mutations in STRA6, the gene encoding the cellular receptor for vitamin A, in patients with Matthew-Wood syndrome and anophthalmia/microphthalmia (A/M), have previously demonstrated the importance of retinol metabolism in human eye disease. We used homozygosity mapping combined with next-generation sequencing to interrogate patients with anophthalmia and microphthalmia for new causative genes. We used whole-exome and whole-genome sequencing to study a family with two affected brothers with bilateral A/M and a simplex case with bilateral anophthalmia and hypoplasia of the optic nerve and optic chiasm. Analysis of novel sequence variants revealed homozygosity for two nonsense mutations in ALDH1A3, c.568A>G, predicting p.Lys190*, in the familial cases, and c.1165A>T, predicting p.Lys389*, in the simplex case. Both mutations predict nonsense-mediated decay and complete loss of function. We performed antisense morpholino (MO) studies in Danio rerio to characterize the developmental effects of loss of Aldh1a3 function. MO-injected larvae showed a significant reduction in eye size, and aberrant axonal projections to the tectum were noted. We conclude that ALDH1A3 loss of function causes anophthalmia and aberrant eye development in humans and in animal model systems.

  11. Causes, Patterns, and Severity of Androgen Excess in 1205 Consecutively Recruited Women.

    Science.gov (United States)

    Elhassan, Yasir S; Idkowiak, Jan; Smith, Karen; Asia, Miriam; Gleeson, Helena; Webster, Rachel; Arlt, Wiebke; O'Reilly, Michael W

    2018-03-01

    Androgen excess in women is predominantly due to underlying polycystic ovary syndrome (PCOS). However, there is a lack of clarity regarding patterns and severity of androgen excess that should be considered predictive of non-PCOS pathology. We examined the diagnostic utility of simultaneous measurement of serum dehydroepiandrosterone sulfate (DHEAS), androstenedione (A4), and testosterone (T) to delineate biochemical signatures and cutoffs predictive of non-PCOS disorders in women with androgen excess. Retrospective review of all women undergoing serum androgen measurement at a large tertiary referral center between 2012 and 2016. Serum A4 and T were measured by tandem mass spectrometry and DHEAS by immunoassay. Patients with at least one increased serum androgen underwent phenotyping by clinical notes review. In 1205 women, DHEAS, A4, and T were measured simultaneously. PCOS was the most common diagnosis in premenopausal (89%) and postmenopausal women (29%). A4 was increased in all adrenocortical carcinoma (ACC) cases (n = 15) and T in all ovarian hyperthecosis (OHT) cases (n = 7); all but one case of congenital adrenal hyperplasia (CAH; n = 18) were identified by increased levels of A4 and/or T. In premenopausal women, CAH was a prevalent cause of severe A4 (59%) and T (43%) excess; severe DHEAS excess was predominantly due to PCOS (80%). In postmenopausal women, all cases of severe DHEAS and A4 excess were caused by ACC and severe T excess equally by ACC and OHT. Pattern and severity of androgen excess are important predictors of non-PCOS pathology and may be used to guide further investigations as appropriate.

  12. Diabetic macular oedema and visual loss: relationship to location, severity and duration

    DEFF Research Database (Denmark)

    Gardner, Thomas W; Larsen, Michael; Girach, Aniz

    2009-01-01

    Abstract. Purpose: To assess the relationship between visual acuity (VA) and diabetic macular oedema (DMO) in relation to the location of retinal thickening and the severity and duration of central macular thickening. Methods: Data from 584 eyes in 340 placebo-treated patients in the 3-years...... (Snellen equivalent = 20/125). Diabetic retinopathy and DMO status were assessed using stereo photographs. Results: Nearly one third of study eyes had foveal centre-involving DMO at the start of the trial. Sustained moderate visual loss was found in 36 eyes, most commonly associated with DMO at the centre...

  13. Value loss from weevil-caused defects in eastern white pine lumber

    Science.gov (United States)

    Myron D. Ostrander; Carl H. Stoltenberg

    1957-01-01

    Owners of eastern white pine stands suffer financially in several ways from attacks by the white-pine weevil (Pissodes strobi). Crooks, forks, and other weevil-caused tree-bole deformities increase bucking, logging, and sawing costs, and they reduce recoverable volumes. The injuries also reduce the average value of the lumber recovered. It is only with this reduction...

  14. Craniopharyngioma causing bilateral vision loss 4 months after unremarkable magnetic resonance imaging of the brain

    Directory of Open Access Journals (Sweden)

    Rainy Betts

    2015-01-01

    Full Text Available A 65-year-old man developed bilateral vision loss 4 months after magnetic resonance imaging demonstrated no lesion in the vicinity of the optic chiasm, hypothalamus, and suprasellar tissues. Repeat computed tomography 3 months later showed a predominantly cystic mass of the suprasellar cistern with extension into the anterior third ventricle, which histologically was a craniopharyngioma. The clinical course of this case fuels the controversy whether craniopharyngiomas arise from embryonic rests or can be acquired. From a clinical perspective, it raises questions about when to obtain imaging studies dedicated to the chiasm and the appropriate interval in which a scan should be repeated to exclude structural causes of bilateral vision loss.

  15. Progressive sensorineural hearing loss, subjective tinnitus and vertigo caused by elevated blood lipids.

    Science.gov (United States)

    Pulec, J L; Pulec, M B; Mendoza, I

    1997-10-01

    The otologist frequently sees patients with progressive sensorineural hearing loss, subjective aural tinnitus and vertigo with no apparent cause. Elevated blood lipids may be a cause of inner ear malfunction on a biochemical basis. To establish the true incidence of this condition, all new patients (4,251) seen during an eight-year period were evaluated; of these, 2,332 patients had complaints of inner ear disease. All had a complete neurotologic examination, appropriate audiometric and vestibular studies and imaging, and blood tests including lipid phenotype studies. Hyperlipoproteinemia was found in 120 patients (5.1%). Most patients were found to be overweight and had additional coexisting conditions such as diabetes mellitus. Treatment with vasodilators and a 500-calorie, high-protein, low-carbohydrate diet yielded improvement of symptoms in 83% of patients within five months of initiation of treatment.

  16. [Presbycusis - Age Related Hearing Loss].

    Science.gov (United States)

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

    Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. © Georg Thieme Verlag KG Stuttgart · New York.

  17. Cobalamin C Deficiency Shows a Rapidly Progressing Maculopathy With Severe Photoreceptor and Ganglion Cell Loss

    Science.gov (United States)

    Bonafede, Lucas; Ficicioglu, Can H.; Serrano, Leona; Han, Grace; Morgan, Jessica I. W.; Mills, Monte D.; Forbes, Brian J.; Davidson, Stefanie L.; Binenbaum, Gil; Kaplan, Paige B.; Nichols, Charles W.; Verloo, Patrick; Leroy, Bart P.; Maguire, Albert M.; Aleman, Tomas S.

    2015-01-01

    Purpose To describe in detail the retinal structure and function of a group of patients with cobalamin C (cblC) disease. Methods Patients (n = 11, age 4 months to 15 years) with cblC disease (9/11, early onset) diagnosed by newborn screening underwent complete ophthalmic examinations, fundus photography, near-infrared reflectance imaging, and spectral-domain optical coherence tomography (SD-OCT). Electroretinograms (ERGs) were performed in a subset of patients. Results Patients carried homozygous or compound heterozygote mutations in the methylmalonic aciduria and homocystinuria type C (MMACHC) gene. Late-onset patients had a normal exam. All early-onset patients showed a maculopathy; older subjects had a retina-wide degeneration (n = 4; >7 years of age). In general, retinal changes were first observed before 1 year of age and progressed within months to a well-established maculopathy. Pseudocolobomas were documented in three patients. Measurable visual acuities ranged from 20/200 to 20/540. Nystagmus was present in 8/11 patients; 5/6 patients had normal ERGs; 1/6 had reduced rod-mediated responses. Spectral-domain OCT showed macular thinning, with severe ganglion cell layer (GCL) and outer nuclear layer (ONL) loss. Inner retinal thickening was observed in areas of total GCL/ONL loss. A normal lamination pattern in the peripapillary nasal retina was often seen despite severe central and/or retina-wide disease. Conclusions Patients with early-onset cblC and MMACHC mutations showed an early-onset, unusually fast-progressing maculopathy with severe central ONL and GCL loss. An abnormally thickened inner retina supports a remodeling response to both photoreceptor and ganglion cell degeneration and/or an interference with normal development in early-onset cblC. PMID:26658511

  18. Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice.

    Science.gov (United States)

    Petkau, Terri L; Blanco, Jake; Leavitt, Blair R

    2017-10-01

    Progranulin deficiency due to heterozygous null mutations in the GRN gene is a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous loss-of-function GRN mutations cause neuronal ceroid lipofuscinosis (NCL). Aged progranulin-knockout mice display highly exaggerated lipofuscinosis, microgliosis, and astrogliosis, as well as mild cell loss in specific brain regions. Progranulin is a secreted glycoprotein expressed in both neurons and microglia, but not astrocytes, in the brain. We generated conditional progranulin-knockout mice that lack progranulin in nestin-expressing cells (Nes-cKO mice), which include most neurons as well as astrocytes. We confirmed near complete knockout of progranulin in neurons in Nes-cKO mice, while microglial progranulin levels remained similar to that of wild-type animals. Overall brain progranulin levels were reduced by about 50% in Nes-cKO, and no Grn was detected in primary Nes-cKO neurons. Nes-cKO mice aged to 12months did not display any increase in lipofuscin deposition, microgliosis, or astrogliosis in the four brain regions examined, though increases were observed for most of these measures in Grn-null animals. We conclude that neuron-specific loss of progranulin is not sufficient to cause similar neuropathological changes to those seen in constitutive Grn-null animals. Our results suggest that increased lipofuscinosis and gliosis in Grn-null animals are not caused by intrinsic progranulin deficiency in neurons, and that microglia-derived progranulin may be sufficient to maintain neuronal health and homeostasis in the brain. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. A tradeoff between the losses caused by computer viruses and the risk of the manpower shortage

    NARCIS (Netherlands)

    Bi, Jichao; Yang, L.; Yang, Xiaofan; Wu, Yingbo; Tang, Yuan Yan

    2018-01-01

    This article addresses the tradeoff between the losses caused by a new virus and the size of the team for developing an antivirus against the virus. First, an individual-level virus spreading model is proposed to capture the spreading process of the virus before the appearance of its natural

  20. Pathogenic Features of Dysuria in Young Women with Secondary Amenorrhea Caused by Body Weight Loss.

    Science.gov (United States)

    Shelkovnikova, N V; Neimark, A I; Taranina, T S; Pichigina, A K; Molodyh, O P; Lushnikova, E L

    2016-12-01

    We examined 11 women aged 19-26 years (mean age 22.5±3.5 years) with secondary amenorrhea complaining frequent urination over 1.5 years and repeatedly, but unsuccessful treated for overactive bladder and chronic cystitis. The rare cause of sustained urination disorders in young female patients of reproductive age was established: development of secondary amenorrhea caused by weight loss ("cosmetic" amenorrhea) with subsequent estrogene deficit and urogenital atrophy. Morphological examination of the bladder mucosa, an important clue to the diagnosis, helps to identify the true cause of dysuria, urogenital atrophy of the bladder mucosa, in secondary ("cosmetic") amenorrhea, and determine future course of etiopathogenic treatment of sustained dysuria in young women. The treatment is often effective in case of proper and timely diagnosis and the absence of irreversible changes.

  1. Severe structural and functional visual system damage leads to profound loss of vision-related quality of life in patients with neuromyelitis optica spectrum disorders.

    Science.gov (United States)

    Schmidt, Felix; Zimmermann, Hanna; Mikolajczak, Janine; Oertel, Frederike C; Pache, Florence; Weinhold, Maria; Schinzel, Johann; Bellmann-Strobl, Judith; Ruprecht, Klemens; Paul, Friedemann; Brandt, Alexander U

    2017-01-01

    Neuromyelitis optica spectrum disorders (NMOSD) are characterized by devastating optic neuritis attacks causing more structural damage and visual impairment than in multiple sclerosis (MS). The objective of this study was to compare vision-related quality of life in NMOSD and MS patients and correlate it to structural retinal damage and visual function. Thirty-one NMOSD and 31 matched MS patients were included. Vision-related quality of life was assessed with the 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ). All patients underwent retinal optical coherence tomography and visual acuity and contrast sensitivity measurements. Vision-related quality of life was reduced in NMOSD compared to MS patients. This difference was driven by a higher incidence of bilateral and more severe optic neuritis in the NMOSD group. Retinal thinning and visual impairment were significantly greater in the NMOSD cohort. Lower vision-related quality of life was associated with more retinal damage and reduced visual function as assessed by visual acuity and contrast sensitivity. NMOSD-related bilateral ON-attacks cause severe structural damage and visual impairment that lead to severe loss of vision-related quality of life. The NEI-VFQ is a helpful tool to monitor vision-related quality of life in NMOSD patients. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. Electroconvulsive therapy and memory loss: a personal journey.

    Science.gov (United States)

    Donahue, A B

    2000-06-01

    The cause for the significant gap between research and anecdotal evidence regarding the extent of some memory loss after electroconvulsive therapy (ECT) has never been adequately explained. A patient's development of awareness and self-education about her severe side effects from ECT raises questions regarding many current assumptions about memory loss. ECT-specific studies, which conclude that side effects are short term and narrow in scope, have serious limitations, including the fact that they do not take into account broader scientific knowledge about memory function. Because of the potential for devastating and permanent memory loss with ECT, informed consent needs significant enhancement until advancing research on both improved techniques and on better predictive knowledge regarding memory loss progresses to making a greater impact on clinical applications. Follow-up care and education in coping skills need to be a regular part of ECT practice when patients do experience severe effects.

  3. Management of Conductive Hearing Loss in Children.

    Science.gov (United States)

    Dougherty, William; Kesser, Bradley W

    2015-12-01

    Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can be caused by a simple cerumen impaction, middle ear fluid, or complex middle ear abnormalities with or without the absence of the ear canal (congenital aural atresia). This article presents evidence-based recommendations for the evaluation and management of the child with both acquired and congenital CHL. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Loss of keratin K2 expression causes aberrant aggregation of K10, hyperkeratosis, and inflammation.

    Science.gov (United States)

    Fischer, Heinz; Langbein, Lutz; Reichelt, Julia; Praetzel-Wunder, Silke; Buchberger, Maria; Ghannadan, Minoo; Tschachler, Erwin; Eckhart, Leopold

    2014-10-01

    Keratin K2 is one of the most abundant structural proteins of the epidermis; however, its biological significance has remained elusive. Here we show that suprabasal type II keratins, K1 and K2, are expressed in a mutually exclusive manner at different body sites of the mouse, with K2 being confined to the ear, sole, and tail skin. Deletion of K2 caused acanthosis and hyperkeratosis of the ear and the tail epidermis, corneocyte fragility, increased transepidermal water loss, and local inflammation in the ear skin. The loss of K2 was partially compensated by upregulation of K1 expression. However, a significant portion of K2-deficient suprabasal keratinocytes lacked a regular cytoskeleton and developed massive aggregates of the type I keratin, K10. Aggregate formation, but not hyperkeratosis, was suppressed by the deletion of both K2 and K10, whereas deletion of K10 alone caused clumping of K2 in ear skin. Taken together, this study demonstrates that K2 is a necessary and sufficient binding partner of K10 at distinct body sites of the mouse and that unbalanced expression of these keratins results in aggregate formation.

  5. Nonsyndromic Hearing Loss Caused by USH1G Mutations: Widening the USH1G Disease Spectrum

    NARCIS (Netherlands)

    Oonk, A.M.M.; Huet, R.A.C. van; Leijendeckers, J.M.; Oostrik, J.; Venselaar, H.; WIjk, E. van; Beynon, A.J.; Kunst, H.P.M.; Hoyng, C.B.; Kremer, H.; Schraders, M.; Pennings, R.J.E.

    2015-01-01

    OBJECTIVE: Currently, six genes are known to be associated with Usher syndrome type I, and mutations in most of these genes can also cause nonsyndromic hearing loss. The one exception is USH1G, which is currently only known to be involved in Usher syndrome type I and atypical Usher syndrome. DESIGN:

  6. Advances in the Understanding of the Genetic Causes of Hearing Loss in Children Inform a Rational Approach to Evaluation.

    Science.gov (United States)

    Carey, John C; Palumbos, Janice C

    2016-10-01

    Hearing loss represents the most common sensory disability of children. Remarkable advances in the identification of genes underlying nonsyndromic and syndromic hearing loss in just the last 2 decades have led to the ability to determine the specific genetic cause of hearing loss in many children. Surprisingly one gene, GJB2, encoding the protein connexin-26, accounts for about 20 % of sensorineural hearing loss (including in India) and is considered the first tier test in evaluating an infant with unexplained congenital hearing loss. Using the knowledge of the etiology of hearing loss, the authors propose a diagnostic reasoning process for the assessment of a child in the pediatric setting. Second tier testing consists of the multiple gene panels using whole exome sequencing strategies, and is becoming available in some regions of the world including the US. Referral to medical genetics is always a consideration in a child with no explanation for the hearing loss and in families with questions about recurrence risk.

  7. [Severe Eyeball and Facial Skeletal Injuries Caused by Firefighting Sport].

    Science.gov (United States)

    Rusňák, Š; Maranová, Z; Kasl, Z; Hecová, L; Voigt, E; Raiskup, F

    2017-07-01

    The aim of this work is to draw attention to possible injuries of the eye and the facial skeleton caused by firefighting sport. There was a group of 9 patients presented who were treated from 2006 to 2015 in the Department of Ophthalmology at the University Hospital in Pilsen and diagnosed with severe eyeball contusion after being hit by a jet of water and/or a water pipe. Three cases are presented in detail. Georg Thieme Verlag KG Stuttgart · New York.

  8. Weight loss and severe jaundice in a patient with hyperthyroidism.

    Science.gov (United States)

    Breidert, M; Offensperger, S; Blum, H E; Fischer, R

    2011-09-01

    Thyrotoxicosis may significantly alter hepatic function and is associated with autoimmune disorders of the liver. We report the case of a thyrotoxic patient with Graves' disease and histologically established cholestatic hepatitis. Medical treatment of hyperthyroidism normalized liver function tests. In patients with elevated liver function parameters and jaundice of unknown origin, thyroid function should generally be tested. Moreover, medical treatment of hyperthyroidism with thyrostatics may cause severe hepatitis whereas untreated hyperthyroid patients are at risk of developing chronic liver failure. © Georg Thieme Verlag KG Stuttgart · New York.

  9. Hypercortisolemia Is Associated with Severity of Bone Loss and Depression in Hypothalamic Amenorrhea and Anorexia Nervosa

    OpenAIRE

    Lawson, Elizabeth A.; Donoho, Daniel; Miller, Karen K.; Misra, Madhusmita; Meenaghan, Erinne; Lydecker, Janet; Wexler, Tamara; Herzog, David B.; Klibanski, Anne

    2009-01-01

    Context: Anorexia nervosa (AN) and functional hypothalamic amenorrhea (HA) are associated with low bone density, anxiety, and depression. Women with AN and HA have elevated cortisol levels. Significant hypercortisolemia, as in Cushing’s disease, causes bone loss. It is unknown whether anxiety and depression and/or cortisol dysregulation contribute to low bone density in AN or HA.

  10. Biodiversity loss, emerging infectious diseases and impact on human and crops

    International Nuclear Information System (INIS)

    Shinwari, Z.K.; Gilani, S.A.; Khan, A.L.

    2012-01-01

    We are losing biodiversity through several factors ranging from global warming, climatic change, unsustainable use of natural resources, human settlements, demand for food, medicine etc. Consequently, the biodiversity losses are causing emergence of infectious diseases (EIDs) which are making them more virulent than the past. Both biodiversity loss and emergence of diseases significantly impact the human derived benefits in-terms of economy and food. Ecological stability, productivity and food-web interactions are indirectly correlated with biodiversity and any change in these will cause losses in biodiversity that would certainly influence the human derived benefits and crops. The current article reviews the biodiversity losses and emerging infectious diseases at various levels reported by recent literature which will help in current status of EIDs and future recommendations. (author)

  11. Severe extremity amputations in surviving Palestinian civilians caused by explosives fired from drones during the Gaza War.

    Science.gov (United States)

    Heszlein-Lossius, Hanne; Al-Borno, Yahya; Shaqoura, Samar; Skaik, Nashwa; Giil, Lasse Melvær; Gilbert, Mads

    2018-02-21

    operations (p=0·0001). Weapons fired on the Gaza Strip from Israeli drones caused severe injuries in surviving Palestinian civilians. Drone-fired missiles resulted in major amputations in almost all victims who had limb losses. Substantially more severe injuries were inflicted by the drone-launched explosives than by other weapons used during the Gaza War. Traumatic amputations caused by drones were often immediately complete. One limitation of our study is that it does not elucidate injury patterns in victims with fatal injuries. None. Copyright © 2018 Elsevier Ltd. All rights reserved.

  12. Modeling farm nutrient flows in the North China Plain to reduce nutrient losses

    NARCIS (Netherlands)

    Zhao, Zhanqing; Bai, Zhaohai; Wei, Sha; Ma, Wenqi; Wang, Mengru; Kroeze, Carolien; Ma, Lin

    2017-01-01

    Years of poor nutrient management practices in the agriculture industry in the North China Plain have led to large losses of nutrients to the environment, causing severe ecological consequences. Analyzing farm nutrient flows is urgently needed in order to reduce nutrient losses. A farm-level

  13. PREGNANCY LOSS IN MARES

    Directory of Open Access Journals (Sweden)

    Tibary A

    2015-12-01

    Full Text Available Pregnancy loss is an important aspect of equine practice due to the economic and emotional loss that it engenders. Pregnancy loss is often divided in two categories: early pregnancy loss (EPL or embryonic death (ED (first 42 days and fetal losses (after 42 days. Diagnosis of the causes of pregnancy loss is often very challenging. Many of the causes of EPL remain poorly documented but studies on embryo development and embryo-uterine interaction have been able to shed some light on predisposing factors. Fetal losses or abortions are dominated by infectious causes and particularly bacterial placentitis. Detailed reviews of pregnancy loss were recently published by the authors (Tibary et al., 2012; Tibary and Pearson, 2012; Tibary et al., 2014. The objective of this paper is to provide an overview of the epidemiology, etiology, diagnosis and prevention of pregnancy loss in the mare.

  14. Causes and consequences of mid–21st-century rapid ice loss events simulated by the Rossby centre regional atmosphere-ocean model

    Directory of Open Access Journals (Sweden)

    Jean-Philippe Paquin

    2013-08-01

    Full Text Available Recent observations and modelling studies suggest that the Arctic climate is undergoing important transition. One manifestation of this change is seen in the rapid sea-ice cover decrease as experienced in 2007 and 2012. Although most numerical climate models cannot adequately reproduce the recent changes, some models produce similar Rapid Ice Loss Events (RILEs during the mid–21st-century. This study presents an analysis of four specific RILEs clustered around 2040 in three transient climate projections performed with the coupled Rossby Centre regional Atmosphere-Ocean model (RCAO. The analysis shows that long-term thinning causes increased vulnerability of the Arctic Ocean sea-ice cover. In the Atlantic sector, pre-conditioning (thinning of sea ice combined with anomalous atmospheric and oceanic heat transport causes large ice loss, while in the Pacific sector of the Arctic Ocean sea-ice albedo feedback appears important, particularly along the retreating sea-ice margin. Although maximum sea-ice loss occurs in the autumn, response in surface air temperature occurs in early winter, caused by strong increase in ocean-atmosphere surface energy fluxes, mainly the turbulent fluxes. Synchronicity of the events around 2040 in the projections is caused by a strong large-scale atmospheric circulation anomaly at the Atlantic lateral boundary of the regional model. The limited impact on land is caused by vertical propagation of the surface heat anomaly rather than horizontal, caused by the absence of low-level temperature inversion over the ocean.

  15. Mutations in CEP78 Cause Cone-Rod Dystrophy and Hearing Loss Associated with Primary-Cilia Defects.

    Science.gov (United States)

    Nikopoulos, Konstantinos; Farinelli, Pietro; Giangreco, Basilio; Tsika, Chrysanthi; Royer-Bertrand, Beryl; Mbefo, Martial K; Bedoni, Nicola; Kjellström, Ulrika; El Zaoui, Ikram; Di Gioia, Silvio Alessandro; Balzano, Sara; Cisarova, Katarina; Messina, Andrea; Decembrini, Sarah; Plainis, Sotiris; Blazaki, Styliani V; Khan, Muhammad Imran; Micheal, Shazia; Boldt, Karsten; Ueffing, Marius; Moulin, Alexandre P; Cremers, Frans P M; Roepman, Ronald; Arsenijevic, Yvan; Tsilimbaris, Miltiadis K; Andréasson, Sten; Rivolta, Carlo

    2016-09-01

    Cone-rod degeneration (CRD) belongs to the disease spectrum of retinal degenerations, a group of hereditary disorders characterized by an extreme clinical and genetic heterogeneity. It mainly differentiates from other retinal dystrophies, and in particular from the more frequent disease retinitis pigmentosa, because cone photoreceptors degenerate at a higher rate than rod photoreceptors, causing severe deficiency of central vision. After exome analysis of a cohort of individuals with CRD, we identified biallelic mutations in the orphan gene CEP78 in three subjects from two families: one from Greece and another from Sweden. The Greek subject, from the island of Crete, was homozygous for the c.499+1G>T (IVS3+1G>T) mutation in intron 3. The Swedish subjects, two siblings, were compound heterozygotes for the nearby mutation c.499+5G>A (IVS3+5G>A) and for the frameshift-causing variant c.633delC (p.Trp212Glyfs(∗)18). In addition to CRD, these three individuals had hearing loss or hearing deficit. Immunostaining highlighted the presence of CEP78 in the inner segments of retinal photoreceptors, predominantly of cones, and at the base of the primary cilium of fibroblasts. Interaction studies also showed that CEP78 binds to FAM161A, another ciliary protein associated with retinal degeneration. Finally, analysis of skin fibroblasts derived from affected individuals revealed abnormal ciliary morphology, as compared to that of control cells. Altogether, our data strongly suggest that mutations in CEP78 cause a previously undescribed clinical entity of a ciliary nature characterized by blindness and deafness but clearly distinct from Usher syndrome, a condition for which visual impairment is due to retinitis pigmentosa. Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  16. Trends and causes of historical wetland loss in coastal Louisiana

    Science.gov (United States)

    Bernier, Julie

    2013-01-01

    Wetland losses in the northern Gulf Coast region of the United States are so extensive that they represent critical concerns to government environmental agencies and natural resource managers. In Louisiana, almost 3,000 square kilometers (km2) of low-lying wetlands converted to open water between 1956 and 2004, and billions of dollars in State and Federal funding have been allocated for coastal restoration projects intended to compensate for some of those wetland losses. Recent research at the St. Petersburg Coastal and Marine Science Center (SPCMSC) focused on understanding the physical processes and human activities that contributed to historical wetland loss in coastal Louisiana and the spatial and temporal trends of that loss. The physical processes (land-surface subsidence and sediment erosion) responsible for historical wetland loss were quantified by comparing marsh-surface elevations, water depths, and vertical displacements of stratigraphic contacts at 10 study areas in the Mississippi River delta plain and 6 sites at Sabine National Wildlife Refuge (SNWR) in the western chenier plain. The timing and extent of land loss at the study areas was determined by comparing historical maps, aerial photographs, and satellite imagery; the temporal and spatial trends of those losses were compared with historical subsidence rates and hydrocarbon production trends.

  17. Tau causes synapse loss without disrupting calcium homeostasis in the rTg4510 model of tauopathy.

    Directory of Open Access Journals (Sweden)

    Katherine J Kopeikina

    Full Text Available Neurofibrillary tangles (NFTs of tau are one of the defining hallmarks of Alzheimer's disease (AD, and are closely associated with neuronal degeneration. Although it has been suggested that calcium dysregulation is important to AD pathogenesis, few studies have probed the link between calcium homeostasis, synapse loss and pathological changes in tau. Here we test the hypothesis that pathological changes in tau are associated with changes in calcium by utilizing in vivo calcium imaging in adult rTg4510 mice that exhibit severe tau pathology due to over-expression of human mutant P301L tau. We observe prominent dendritic spine loss without disruptions in calcium homeostasis, indicating that tangles do not disrupt this fundamental feature of neuronal health, and that tau likely induces spine loss in a calcium-independent manner.

  18. Loss of normal anagen hair in pemphigus vulgaris.

    Science.gov (United States)

    Daneshpazhooh, M; Mahmoudi, H R; Rezakhani, S; Valikhani, M; Naraghi, Z S; Mohammadi, Y; Habibi, A; Chams-Davatchi, C

    2015-07-01

    Pemphigus vulgaris (PV) is a known cause of loss of 'normal' anagen hair; that is, shedding of intact anagen hairs covered by root sheaths. However, studies on this subject are limited. To investigate anagen hair shedding in patients with PV, and ascertain its association with disease severity. In total, 96 consecutive patients with PV (new patients or patients in relapse) who were admitted to the dermatology wards of a tertiary hospital were enrolled in this study. Demographic data, PV phenotype, disease severity and presence of scalp lesions were recorded. A group of 10-20 hairs were pulled gently from different areas of the scalp (lesional and nonlesional skin) in all patients, and anagen hairs were counted. Disease severity was graded according to Harman score. Anagen hair was obtained by pull test in 59 of the 96 patients (61.5%), of whom 2 had normal scalp. The mean ± SD anagen hair count was 5.9 ± 7.6 (range 0-31). In univariate analysis, anagen hair loss (P hair count was significantly higher in the severe (mean 6.83 ± 7.89) than the moderate (mean 1.06 ± 1.94) subgroup (P hair loss (OR = 1.16, 95% CI = 1.05-1.28, P hair loss was an independent predictor of the disease severity. © 2015 British Association of Dermatologists.

  19. Increased steroidogenesis promotes early-onset and severe vision loss in females with OPA1 dominant optic atrophy

    DEFF Research Database (Denmark)

    Sarzi, Emmanuelle; Seveno, Marie; Angebault, Claire

    2016-01-01

    levels of steroid precursor pregnenolone in females, causing an early-onset vision loss, abolished by ovariectomy. In addition, steroid production in retina is also increased which, in conjunction with high circulating levels, impairs estrogen receptor expression and mitochondrial respiratory complex IV...... tested the hypothesis of deregulated steroid production in retina due to a disease-causing OPA1 mutation and its contribution to the visual phenotypic variations. Using the mouse model carrying the human recurrent OPA1 mutation, we disclosed that Opa1 haploinsufficiency leads to very high circulating...

  20. A model on CME/Flare initiation: Loss of Equilibrium caused by mass loss of quiescent prominences

    Science.gov (United States)

    Miley, George; Chon Nam, Sok; Kim, Mun Song; Kim, Jik Su

    2015-08-01

    Coronal Mass Ejections (CMEs) model should give an answer to enough energy storage for giant bulk plasma into interplanetary space to escape against the sun’s gravitation and its explosive eruption. Advocates of ‘Mass Loading’ model (e.g. Low, B. 1996, SP, 167, 217) suggested a simple mechanism of CME initiation, the loss of mass from a prominence anchoring magnetic flux rope, but they did not associate the mass loss with the loss of equilibrium. The catastrophic loss of equilibrium model is considered as to be a prospective CME/Flare model to explain sudden eruption of magnetic flux systems. Isenberg, P. A., et al (1993, ApJ, 417, 368)developed ideal magnetohydrodynamic theory of the magnetic flux rope to show occurrence of catastrophic loss of equilibrium according to increasing magnetic flux transported into corona.We begin with extending their study including gravity on prominence’s material to obtain equilibrium curves in case of given mass parameters, which are the strengths of the gravitational force compared with the characteristic magnetic force. Furthermore, we study quasi-static evolution of the system including massive prominence flux rope and current sheet below it to obtain equilibrium curves of prominence’s height according to decreasing mass parameter in a properly fixed magnetic environment. The curves show equilibrium loss behaviors to imply that mass loss result in equilibrium loss. Released fractions of magnetic energy are greater than corresponding zero-mass case. This eruption mechanism is expected to be able to apply to the eruptions of quiescent prominences, which is located in relatively weak magnetic environment with 105 km of scale length and 10G of photospheric magnetic field.

  1. [Severe hypertriglyceridemia--an important cause of pancreatitis].

    Science.gov (United States)

    Graesdal, Asgeir

    2008-05-01

    Moderate hypertriglyceridaemia is a risk factor for cardiovascular disease and serious hypertriglyceridaemia, with triglyceride values above 10 mmol/L, increases the risk of pancreatitis. Gallstones and alcohol abuse are regarded as the two most important causes of acute pancreatitis, but the considerable risk posed by hypertriglyceridaemia has probably been underrated. It is therefore crucial to acquire updated knowledge and awareness of the fact that high levels of triglycerides can cause pancreatitis. This article is based on current literature retrieved though a search on the topic and clinical experience. Serious hypertriglyceridaemia is a relatively rare condition and its usual cause is genetic predisposition combined with obesity, diabetes or alcohol abuse. Certain types of medication, as well as pregnancy, are also well known causes. Current literature suggests that hypertriglyceridaemia is the cause of pancreatitis in 1-38% of the cases--a substantial variation. The condition is often accompanied by low amylase values and may therefore be underrated as a cause. Our case reports illustrate that the etiology is complex. Plasmapheresis or LDL-apheresis may be indicated when conservative treatment proves insufficient.

  2. Rapid intestinal transit as a primary cause of severe chronic diarrhea in patients with amyloidosis.

    Science.gov (United States)

    Guirl, Michael J; Högenauer, Christoph; Santa Ana, Carol A; Porter, Jack L; Little, Katherine H; Stone, Marvin J; Fordtran, John S

    2003-10-01

    The cause of severe diarrhea in patients with systemic amyloidosis is obscure. We therefore performed pathophysiological studies in three such patients in an effort to determine the mechanism of amyloid diarrhea. Epithelial cell absorption rate of electrolytes was measured during steady state GI perfusion of a saline-mannitol solution. GI transit time of PEG and absorption of radiolabeled bile acid were measured simultaneously while subjects ingested three meals per day. To obtain a diarrhea control group for transit time and bile acid absorption, normal subjects were studied when they had diarrhea caused by ingestion of Milk of Magnesia (MOM). Diarrhea could not be explained by malabsorption of ingested nutrients, bacterial overgrowth, bile acid malabsorption, or epithelial cell malabsorption of electrolytes. However, 25% of polyethylene glycol (PEG) ingested with a standard meal was recovered in stool in 45 min, which is 10 times faster than in normal subjects with equally severe diarrhea caused by ingestion of MOM. All of the patients had autonomic neuropathy that remained unrecognized for 15-36 months after onset of chronic diarrhea; it seems likely that this was the cause of rapid transit. Severe chronic diarrhea in three patients with systemic amyloidosis was mediated by extremely rapid transit of chyme and digestive secretions through the intestine.

  3. Energy losses in switches

    International Nuclear Information System (INIS)

    Martin, T.H.; Seamen, J.F.; Jobe, D.O.

    1993-01-01

    The authors experiments show energy losses between 2 and 10 times that of the resistive time predictions. The experiments used hydrogen, helium, air, nitrogen, SF 6 polyethylene, and water for the switching dielectric. Previously underestimated switch losses have caused over predicting the accelerator outputs. Accurate estimation of these losses is now necessary for new high-efficiency pulsed power devices where the switching losses constitute the major portion of the total energy loss. They found that the switch energy losses scale as (V peak I peak ) 1.1846 . When using this scaling, the energy losses in any of the tested dielectrics are almost the same. This relationship is valid for several orders of magnitude and suggested a theoretical basis for these results. Currents up to .65 MA, with voltages to 3 MV were applied to various gaps during these experiments. The authors data and the developed theory indicates that the switch power loss continues for a much longer time than the resistive time, with peak power loss generally occurring at peak current in a ranging discharge instead of the early current time. All of the experiments were circuit code modeled after developing a new switch loss version based on the theory. The circuit code predicts switch energy loss and peak currents as a function of time. During analysis of the data they noticed slight constant offsets between the theory and data that depended on the dielectric. They modified the plasma conductivity for each tested dielectric to lessen this offset

  4. [Cause analysis and management of severe dislocated or subluxated intraocular lenses in the capsular bag].

    Science.gov (United States)

    Wang, Wen-qing; Jia, Li-Li; Lu, Bin; Fang, Jian; Chen, Ying

    2006-05-01

    To analyze the causes of severe dislocated and subluxated intraocular lenses (IOL) in capsular bag and the relevancy with abnormal capsular constitution. The abnormality of capsular and the position of IOL were retrospectively analyzed by slit lamp and microscope in dilated pupil. Surgeries were performed in all of 31 cases in order to exchange or reposit the dislocated or subluxated lOL. Among 31 cases, there were 20 cases with the dislocated or subluxated IOL in the earlier postoperative period and 11 cases in the later postoperative period. The subluxated IOL in 5 cases (16.1%) were caused by the large rupture of the posterior capsular, and by the rupture of the posterior capsular in 9 cases (29.0%). A upward subluxated IOL developed in one case (3.2%) as the result of small rupture in the equater capsular. The subluxatd IOL in the anterior chamber in two cases (6.5%) was induced by the radial rupture of residual anterior capsular. The size of the IOLs in 2 cases were not matched to the diameter of the capsule bag. The dislocated IOL into the vitreous in 2 cases was caused by the rupture of the superior capsular. The subluxated IOL in 2 cases (6.5%) was caused by the zonal finers rupture due to trauma. The severe disfiguration of the IOL haptic was found in one case. The dislocated IOL in 6 cases (19.4%) was caused by asymmetric capsular shrinkage. A subluxated IOL in one case was resulted from the zonular defects with pseudoexfoliation syndrome. The postoperative corrected visual acuity was 0.5 - 1.0 in 24 eyes, 0.1 - 0.4 in 6 eyes, and 0.05 in one eye. There are several causes for the dislocated or subluxated IOL. The correction of the IOL position should depend on the cause for the dislocated or subluxated IOL.

  5. Causes of non-adherence to therapeutic guidelines in severe community-acquired pneumonia

    Science.gov (United States)

    Gattarello, Simone; Ramírez, Sergio; Almarales, José Rafael; Borgatta, Bárbara; Lagunes, Leonel; Encina, Belén; Rello, Jordi

    2015-01-01

    Objective To assess the adherence to Infectious Disease Society of America/American Thoracic Society guidelines and the causes of lack of adherence during empirical antibiotic prescription in severe pneumonia in Latin America. Methods A clinical questionnaire was submitted to 36 physicians from Latin America; they were asked to indicate the empirical treatment in two fictitious cases of severe respiratory infection: community-acquired pneumonia and nosocomial pneumonia. Results In the case of communityacquired pneumonia, 11 prescriptions of 36 (30.6%) were compliant with international guidelines. The causes for non-compliant treatment were monotherapy (16.0%), the unnecessary prescription of broad-spectrum antibiotics (40.0%) and the use of non-recommended antibiotics (44.0%). In the case of nosocomial pneumonia, the rate of adherence to the Infectious Disease Society of America/American Thoracic Society guidelines was 2.8% (1 patient of 36). The reasons for lack of compliance were monotherapy (14.3%) and a lack of dual antibiotic coverage against Pseudomonas aeruginosa (85.7%). If monotherapy with an antipseudomonal antibiotic was considered adequate, the antibiotic treatment would be adequate in 100% of the total prescriptions. Conclusion The compliance rate with the Infectious Disease Society of America/American Thoracic Society guidelines in the community-acquired pneumonia scenario was 30.6%; the most frequent cause of lack of compliance was the indication of monotherapy. In the case of nosocomial pneumonia, the compliance rate with the guidelines was 2.8%, and the most important cause of non-adherence was lack of combined antipseudomonal therapy. If the use of monotherapy with an antipseudomonal antibiotic was considered the correct option, the treatment would be adequate in 100% of the prescriptions. PMID:25909312

  6. Successful Immunoglobulin Treatment in Severe Cryptogenic Organizing Pneumonia Caused by Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Dong Hoon Lee

    2015-08-01

    Full Text Available In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.

  7. Hereditary Hearing Loss.

    Science.gov (United States)

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

    This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

  8. Ocular firework trauma: a systematic review on incidence, severity, outcome and prevention.

    Science.gov (United States)

    Wisse, R P L; Bijlsma, W R; Stilma, J S

    2010-12-01

    To provide a systematic review on ocular firework trauma with emphasis on incidence and patient demographics, the extent of ocular trauma and visual function loss, and firework regulation effects on injury rates. A literature search was performed using predetermined inclusion and exclusion criteria. Demographic characteristics of ocular firework casualties were obtained and incidence rates of sustained trauma and vision loss calculated. Twenty-six relevant articles were suitable for calculation of trauma incidence and patient demographics, of which 17 articles could be used for calculating trauma severity and vision loss. Victims were male (77%), young (82%) and often bystander (47%). Most of the trauma was mild and temporary. Penetrating eye trauma, globe contusions and burns accounted for 18.2%, with a 3.9% enucleation rate. Mean visual acuity was >10/20 in 56.8%, with severe vision loss (firework legislation show 87% less eye trauma (pfirework traumas show severe vision loss, mostly in young males. Bystanders are as frequently injured. Firework traumas are a preventable cause of severe ocular injury and blindness because countries using restrictive firework legislation have remarkable lower trauma incidence rates.

  9. SEVERITY CLASSIFICATION OF MICROANEURYSMS USING NEURAL NETWORK

    Directory of Open Access Journals (Sweden)

    Shree Divya R

    2014-01-01

    Full Text Available Diabetic Retinopathy is one of the most common causes of blindness that leads to the loss of vision to the human eye. Several methods have been proposed to detect several defects of the human eye like hemorrhages, exudates etc. which are to be considered as the major symptoms. Among them, Microaneurysms should be considered as one of the severe condition for the early blindness. Several techniques have been proposed based on this, but they have certain drawbacks. A new technique called neural network taken for presentation, helps to detect and determine the severity of Microaneurysms which would be able to give a better performance than the existing techniques.

  10. Extracorporeal membrane oxygenation causes loss of intestinal epithelial barrier in the newborn piglet.

    Science.gov (United States)

    Kurundkar, Ashish R; Killingsworth, Cheryl R; McIlwain, R Britt; Timpa, Joseph G; Hartman, Yolanda E; He, Dongning; Karnatak, Rajendra K; Neel, Mary L; Clancy, John P; Anantharamaiah, G M; Maheshwari, Akhil

    2010-08-01

    Extracorporeal membrane oxygenation (ECMO) is an important life-support system used in neonates and young children with intractable cardiorespiratory failure. In this study, we used our porcine neonatal model of venoarterial ECMO to investigate whether ECMO causes gut barrier dysfunction. We subjected 3-wk-old previously healthy piglets to venoarterial ECMO for up to 8 h and evaluated gut mucosal permeability, bacterial translocation, plasma levels of bacterial products, and ultrastructural changes in gut epithelium. We also measured plasma lipopolysaccharide (LPS) levels in a small cohort of human neonates receiving ECMO. In our porcine model, ECMO caused a rapid increase in gut mucosal permeability within the first 2 h of treatment, leading to a 6- to 10-fold rise in circulating bacterial products. These changes in barrier function were associated with cytoskeletal condensation in epithelial cells, which was explained by phosphorylation of a myosin II regulatory light chain. In support of these findings, we also detected elevated plasma LPS levels in human neonates receiving ECMO, indicating a similar loss of gut barrier function in these infants. On the basis of these data, we conclude that ECMO is an independent cause of gut barrier dysfunction and bacterial translocation may be an important contributor to ECMO-related inflammation.

  11. The loss rates of O+ in the inner magnetosphere caused by both magnetic field line curvature scattering and charge exchange reactions

    Science.gov (United States)

    Ji, Y.; Shen, C.

    2014-03-01

    With consideration of magnetic field line curvature (FLC) pitch angle scattering and charge exchange reactions, the O+ (>300 keV) in the inner magnetosphere loss rates are investigated by using an eigenfunction analysis. The FLC scattering provides a mechanism for the ring current O+ to enter the loss cone and influence the loss rates caused by charge exchange reactions. Assuming that the pitch angle change is small for each scattering event, the diffusion equation including a charge exchange term is constructed and solved; the eigenvalues of the equation are identified. The resultant loss rates of O+ are approximately equal to the linear superposition of the loss rate without considering the charge exchange reactions and the loss rate associated with charge exchange reactions alone. The loss time is consistent with the observations from the early recovery phases of magnetic storms.

  12. Regional Variations in the Risk and Severity of Ciguatera Caused by Eating Moray Eels.

    Science.gov (United States)

    Chan, Thomas Y K

    2017-06-26

    Moray eels ( Gymnothorax species) from tropical waters have long been known to be high-risk species, and the consumption of particularly the viscera or ungutted eels can result in severe ciguatera (known as Gymnothorax or moray eel poisoning), characterized by prominent neurological features. In this review, the main objective was to describe the risk and severity of ciguatera caused by eating moray eels in different parts of the world. Moray eels can accumulate very high ciguatoxin (CTX) levels in the flesh and particularly the liver. Therefore, even the smaller ones can be toxic and the consumption of an average portion (particularly liver) can result in severe or fatal ciguatera. Moray eels (particularly when ungutted) must never be served in gatherings since they can cause mass poisoning because of their large sizes and high CTX levels. Apart from regulatory measures restricting or excluding access, the public should be repeatedly warned to avoid eating moray eels.

  13. Regional Variations in the Risk and Severity of Ciguatera Caused by Eating Moray Eels

    Directory of Open Access Journals (Sweden)

    Thomas Y. K. Chan

    2017-06-01

    Full Text Available Moray eels (Gymnothorax species from tropical waters have long been known to be high-risk species, and the consumption of particularly the viscera or ungutted eels can result in severe ciguatera (known as Gymnothorax or moray eel poisoning, characterized by prominent neurological features. In this review, the main objective was to describe the risk and severity of ciguatera caused by eating moray eels in different parts of the world. Moray eels can accumulate very high ciguatoxin (CTX levels in the flesh and particularly the liver. Therefore, even the smaller ones can be toxic and the consumption of an average portion (particularly liver can result in severe or fatal ciguatera. Moray eels (particularly when ungutted must never be served in gatherings since they can cause mass poisoning because of their large sizes and high CTX levels. Apart from regulatory measures restricting or excluding access, the public should be repeatedly warned to avoid eating moray eels.

  14. Regional Variations in the Risk and Severity of Ciguatera Caused by Eating Moray Eels

    Science.gov (United States)

    Chan, Thomas Y. K.

    2017-01-01

    Moray eels (Gymnothorax species) from tropical waters have long been known to be high-risk species, and the consumption of particularly the viscera or ungutted eels can result in severe ciguatera (known as Gymnothorax or moray eel poisoning), characterized by prominent neurological features. In this review, the main objective was to describe the risk and severity of ciguatera caused by eating moray eels in different parts of the world. Moray eels can accumulate very high ciguatoxin (CTX) levels in the flesh and particularly the liver. Therefore, even the smaller ones can be toxic and the consumption of an average portion (particularly liver) can result in severe or fatal ciguatera. Moray eels (particularly when ungutted) must never be served in gatherings since they can cause mass poisoning because of their large sizes and high CTX levels. Apart from regulatory measures restricting or excluding access, the public should be repeatedly warned to avoid eating moray eels. PMID:28672845

  15. Process analysis using the concept of intrinsic and extrinsic exergy losses

    International Nuclear Information System (INIS)

    Chang Hsuan; Chuang, S.-C.

    2003-01-01

    This paper introduces a two-level idealization concept and decomposes the exergy losses of processing operations into the intrinsic part and the extrinsic part. The first level idealization is the reversible operation and the second level idealization is the thermodynamic equilibrium operation. The exergy losses arising from the deviations from the first level idealization only, caused by configuration constraints, are defined as the intrinsic exergy losses. The extra exergy losses which arise from further deviations from the second level idealization, caused by transport rate limitations, are defined as the extrinsic exergy losses. Demonstrated by several example cases of different complex levels, the analysis results can pinpoint what and where to focus on for improvements: (1) design configurations or transport rate limitations, and (2) the specific locations within the operations or processes. As an example, for a de-ethanizer, the improvement measures on configuration-related and transport rate-related design conditions result in a 11.42% reduction of overall column intrinsic exergy loss and a 81.74% reduction of total individual stage extrinsic exergy loss

  16. Pigeon fancier’s lung – An under-diagnosed cause of severely debilitating and chronic breathlessness

    Directory of Open Access Journals (Sweden)

    Vishal Chopra

    2017-07-01

    Full Text Available Pigeon fanciers lung or Bird fanciers lung (BFL is one of the common and preventable causes of hypersensitivity pneumonitis. It is an under diagnosed cause of severe incapacitating breathlessness and can be acute, sub-acute or chronic. We report a case of 53 year old female who presented with severe chronic breathlessness due to regular exposure to pigeons for last 35 years. Clinicians should take a detailed history of exposure in patients with unexplained breathlessness as the avoidance of exposure to the antigens can reverse the disease preventing the morbidity and mortality of the patient.

  17. Loss of hfe function reverses impaired recognition memory caused by olfactory manganese exposure in mice.

    Science.gov (United States)

    Ye, Qi; Kim, Jonghan

    2015-03-01

    Excessive manganese (Mn) in the brain promotes a variety of abnormal behaviors, including memory deficits, decreased motor skills and psychotic behavior resembling Parkinson's disease. Hereditary hemochromatosis (HH) is a prevalent genetic iron overload disorder worldwide. Dysfunction in HFE gene is the major cause of HH. Our previous study has demonstrated that olfactory Mn uptake is altered by HFE deficiency, suggesting that loss of HFE function could alter manganese-associated neurotoxicity. To test this hypothesis, Hfe-knockout (Hfe (-/-)) and wild-type (Hfe (+/+)) mice mice were intranasally-instilled with manganese chloride (MnCl2 5 mg/kg) or water daily for 3 weeks and examined for memory function. Olfactory Mn diminished both short-term recognition and spatial memory in Hfe (+/+) mice, as examined by novel object recognition task and Barnes maze test, respectively. Interestingly, Hfe (-/-) mice did not show impaired recognition memory caused by Mn exposure, suggesting a potential protective effect of Hfe deficiency against Mn-induced memory deficits. Since many of the neurotoxic effects of manganese are thought to result from increased oxidative stress, we quantified activities of anti-oxidant enzymes in the prefrontal cortex (PFC). Mn instillation decreased superoxide dismutase 1 (SOD1) activity in Hfe (+/+) mice, but not in Hfe (-/-) mice. In addition, Hfe deficiency up-regulated SOD1 and glutathione peroxidase activities. These results suggest a beneficial role of Hfe deficiency in attenuating Mn-induced oxidative stress in the PFC. Furthermore, Mn exposure reduced nicotinic acetylcholine receptor levels in the PFC, indicating that blunted acetylcholine signaling could contribute to impaired memory associated with intranasal manganese. Together, our model suggests that disrupted cholinergic system in the brain is involved in airborne Mn-induced memory deficits and loss of HFE function could in part prevent memory loss via a potential up-regulation of

  18. Hospital Management of Severe Hypertriglyceridemia in Children.

    Science.gov (United States)

    Valaiyapathi, Badhma; Ashraf, Ambika P

    2017-01-01

    Severe Hypertriglyceridemia (HTG), i.e., plasma triglyceride levels exceeding 1000 mg/dL, is one of the established causes of acute pancreatitis and severe abdominal pain. There are no established pediatric guidelines regarding treatment of children and adolescents with severe HTG. To review the pathophysiology and etiology of severe HTG in the pediatric age group, and to discuss management options. Severe HTG is usually due to deficient or absent Lipoprotein Lipase (LPL) activity, which can be due to primary genetic etiology or secondary causes triggering HTG in those with underlying genetic susceptibility. Hospitalization is indicated for patients with severe HTG who are symptomatic with abdominal pain or pancreatitis, in those with uncontrolled diabetes requiring insulin, or, in those with substantial elevations of plasma TG. Fasting followed by fat free diet until plasma TG declines to severe pancreatitis, shock or multi-organ failure. Medications such as fibrates and omega-3 fatty acids are not effective if LPL activity is absent or when plasma TG is >1800 mg/dL. Medications only have an adjunct role in the management. Low fat diet, lifestyle changes, weight loss, control of secondary causes, and patient education form the mainstay of management once the patient is discharged. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  19. Causes of Childhood Vision Impairment in the School for the Blind in Eritrea.

    Science.gov (United States)

    Gyawali, Rajendra; Moodley, Vanessa R

    2017-12-01

    Our study provides the much-needed evidence on causes of childhood blindness in Eritrea. This will assist authorities to plan appropriate strategies and implement preventive, curative, and rehabilitative services to address these causes of vision loss in children in this resource-limited country. This study aims to identify the causes of severe vision impairment and blindness in children attending the only school for the blind in Eritrea. All children enrolled in the school were examined, and the World Health Organization form for the examination of visually impaired children was used to record the data. Examination included visual acuity, refraction, anterior segment, and fundus assessment. Causes of vision loss for children with severe vision impairment (visual acuity blindness (visual acuity blindness. The major causes of vision loss were corneal scars (16.9%), cataract (12.7%), phthisis bulbi (11.3%), congenital eye deformities (11.3%), optic atrophy (9.3%), and presumed chorioretinal Toxoplasma scars (7.0%). Hereditary factors were the major known etiological category (15.5%) followed by the sequel of eye injuries (12.7%). Blindness due to vitamin A deficiency was not found, whereas infectious causes such as measles and ophthalmia neonatorum were relatively absent (one case each). Potentially avoidable causes of vision impairment were accounted for in 47.9% of children. This study provides the first direct evidence on childhood vision impairment in Eritrea. Despite the limitations, it is clearly shown that nearly half of the vision loss is due to avoidable causes. Thus, preventive public health strategies, specialist pediatric eye care, and rehabilitative services are recommended to address childhood vision impairment in Eritrea.

  20. [Etiological and exacerbation factors for COPD. Body weight loss].

    Science.gov (United States)

    Yokoyama, Akihito

    2016-05-01

    Hunger or malnutrition is not only a historical issue but also a current problem worldwide. Biological responses to hunger are evolutionary prepared in our body, including energy generation by degradation of body proteins. Extreme weight loss (malnutrition) can cause air space enlargement in human and rodents. However, the changes in rodents could be reversible, since refeeding could repair the pathology. On the other hand, weight loss is a common feature in patients with more severe COPD. Complex factors, such as increased energy consumption, decreased food uptake by low grade inflammation, socio-economic factors and so on, are involved in weight loss. Weight loss in patients with COPD also increases the risk of exacerbation, hospitalization, and death.

  1. Sleep Loss in Resident Physicians: The Cause of Medical Errors?

    Directory of Open Access Journals (Sweden)

    Milton eKramer

    2010-10-01

    Full Text Available This review begins with the history of the events starting with the death of Libby Zion that lead to the Bell Commission, that the studied her death and made recommendations for improvement that were codified into law in New York state as the 405 law that the ACGME essentially adopted in putting a cap on work hours and establishing the level of staff supervision that must be available to residents in clinical situations particularly the emergency room and acute care units. A summary is then provided of the findings of the laboratory effects of total sleep deprivation including acute total sleep loss and the consequent widespread physiologic alterations, and of the effects of selective and chronic sleep loss. Generally the sequence of responses to increasing sleep loss goes from mood changes to cognitive effects to performance deficits. In the laboratory situation, deficits resulting from sleep deprivation are clearly and definitively demonstrable. Sleep loss in the clinical situation is usually sleep deprivation superimposed on chronic sleep loss. An examination of questionnaire studies, the literature on reports of sleep loss, studies of the reduction of work hours on performance as well as observational and a few interventional studies have yielded contradictory and often equivocal results. The residents generally find they feel better working fewer hours but improvements in patient care are often not reported or do not occur. A change in the attitude of the resident toward his role and his patient has not been salutary. Decreasing sleep loss should have had a positive effect on patient care in reducing medical error, but this remains to be unequivocally demonstrated.

  2. Indirect causes of severe adverse maternal outcomes: a secondary analysis of the WHO Multicountry Survey on Maternal and Newborn Health.

    Science.gov (United States)

    Lumbiganon, P; Laopaiboon, M; Intarut, N; Vogel, J P; Souza, J P; Gülmezoglu, A M; Mori, R

    2014-03-01

    To assess the proportion of severe maternal outcomes resulting from indirect causes, and to determine pregnancy outcomes of women with indirect causes. Secondary analysis of the WHO Multicountry Survey on Maternal and Newborn Health. A total of 359 health facilities in 29 countries in Africa, Asia, Latin America, and the Middle East. A total of 314 623 pregnant women admitted to the participating facilities. We identified the percentage of women with severe maternal outcomes arising from indirect causes. We evaluated the risk of severe maternal and perinatal outcomes in women with, versus without, underlying indirect causes, using adjusted odds ratios and 95% confidence intervals, by a multilevel, multivariate logistic regression model, accounting for clustering effects within countries and health facilities. Severe maternal outcomes and preterm birth, fetal mortality, early neonatal mortality, perinatal mortality, low birthweight, and neonatal intensive care unit admission. Amongst 314 623 included women, 2822 were reported to suffer from severe maternal outcomes, out of which 20.9% (589/2822; 95% CI 20.1-21.6%) were associated with indirect causes. The most common indirect cause was anaemia (50%). Women with underlying indirect causes showed significantly higher risk of obstetric complications (adjusted odds ratio, aOR, 7.0; 95% CI 6.6-7.4), severe maternal outcomes (aOR 27.9; 95% CI 24.7-31.6), and perinatal mortality (aOR 3.8; 95% CI 3.5-4.1). Indirect causes were responsible for about one-fifth of severe maternal outcomes. Women with underlying indirect causes had significantly increased risks of severe maternal and perinatal outcomes. © 2014 RCOG The World Health Organization retains copyright and all other rights in the manuscript of this article as submitted for publication.

  3. Suppression of decidual cell response induced by tributyltin chloride in pseudopregnant rats: a cause of early embryonic loss

    Energy Technology Data Exchange (ETDEWEB)

    Harazono, A.; Ema, M. [National Inst. of Health Sciences, Osaka Branch (Japan)

    2000-12-01

    In our previous studies, tributyltin chloride (TBTC1) at doses of 16.3 mg/kg and above caused implantation failure (preimplantation embryonic loss) and postimplantation embryonic loss in rats following administration on gestational day (GD) 0 through GD 3 and GD 4 through GD 7, respectively. This study was designed to assess the effects of TBTC1 on uterine function as a cause of early embryonic loss in pseudopregnant rats. TBTC1 was given orally to pseudopregnant rats at doses of 4.1, 8.1, 16.3 and 32.5 mg/kg on pseudopregnant day (PPD) 0 to PPD 3 or 8.1, 16.3, 32.5 and 65.1 mg/kg on PPD 4 to PPD 7. The decidual cell response was induced by bilateral scratch trauma on PPD 4. The uterine weight on PPD 9 served as an index of uterine decidualization. Uterine weight and serum progesterone levels on PPD 9 were significantly decreased after administration of TBTC1 at doses of 16.3 mg/kg and above on PPD 0 to PPD 3 or PPD 4 to PPD 7. Administration of TBTC1 at doses of 8.1 mg/kg and above on PPD 0 to 3 also significantly decreased serum progesterone levels on PPD 4. TBTC1 had no effect on ovarian weight and number of corpora lutea. It can be concluded that TBTC1 suppresses the uterine decidual cell response and decreases progesterone levels, and these effects are responsible for early embryonic loss due to TBTC1 exposure. (orig.)

  4. Severe Hand Injuries Caused by a Mole Gun

    Directory of Open Access Journals (Sweden)

    Serdar Düzgün

    2017-10-01

    Full Text Available Objective: Injuries by mole guns differ from other firearm injuries primarily because they are close-range, low-energy injuries that are highly contaminated owing to contact with contaminated surfaces and the presence of numerous residual foreign bodies within the wound. The aim of this article is to share our surgical experience regarding the repair of severe hand injuries caused by mole guns. Material and Methods: This retrospective study included 11 patients with hand injuries. Data obtained about the patients, including age, gender, dominant or non-dominant hand, injuries to all vital structures, and reconstructive procedures were assessed, categorized, and recorded. Results: Ten patients had defects in one or several common digital branches of the median nerve in areas ranging from the distal part of the carpal tunnel to the distal palmar crease. All patients had flexor and extensor tendon injuries in all fingers except for the first finger. Reconstruction of soft tissue and skin defects was carried out with an interpolation flap planned from a random-based subpectoral- paraumbilical region in five patients, a SCIA-based groin flap in four patients, a reverse-radial forearm flap in one patient, and an adipofascial flap planned from the forearm in one patient. Conclusion: Mole gun injuries typically include all structures of the hand, and repair procedures involve every anatomic structure of the hand. An early and effective surgical operation followed by prolonged and effective physical therapy protocols is vital for regaining the full spectrum of hand functions.

  5. The relationship between extreme weather events and crop losses in central Taiwan

    Science.gov (United States)

    Lai, Li-Wei

    2017-09-01

    The frequency of extreme weather events, which cause severe crop losses, is increasing. This study investigates the relationship between crop losses and extreme weather events in central Taiwan from 2003 to 2015 and determines the main factors influencing crop losses. Data regarding the crop loss area and meteorological information were obtained from government agencies. The crops were categorised into the following five groups: `grains', `vegetables', `fruits', `flowers' and `other crops'. The extreme weather events and their synoptic weather patterns were categorised into six and five groups, respectively. The data were analysed using the z score, correlation coefficient and stepwise regression model. The results show that typhoons had the highest frequency of all extreme weather events (58.3%). The largest crop loss area (4.09%) was caused by two typhoons and foehn wind in succession. Extreme wind speed coupled with heavy rainfall is an important factor affecting the losses in the grain and vegetable groups. Extreme wind speed is a common variable that affects the loss of `grains', `vegetables', `fruits' and `flowers'. Consecutive extreme weather events caused greater crop losses than individual events. Crops with long production times suffered greater losses than those with short production times. This suggests that crops with physical structures that can be easily damaged and long production times would benefit from protected cultivation to maintain food security.

  6. Celiac Disease Presenting as Profound Diarrhea and Weight Loss ? A Celiac Crisis

    OpenAIRE

    Bul, Vadim; Sleesman, Brett; Boulay, Brian

    2016-01-01

    Patient: Male, 46 Final Diagnosis: Celiac crisis Symptoms: Abdominal pain ? chronic diarrhea ? lightheadedness ? weakness ? weight loss Medication: ? Clinical Procedure: ? Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Celiac disease is a hypersensitivity enteropathy that can have various presentations in adults. Rarely, patients can present with severe lab abnormalities, dehydration and weight loss caused by celiac disease ? a celiac crisis. Case Report: A 46-...

  7. Receptor tyrosine kinase inhibition causes simultaneous bone loss and excess bone formation within growing bone in rats

    International Nuclear Information System (INIS)

    Nurmio, Mirja; Joki, Henna; Kallio, Jenny; Maeaettae, Jorma A.; Vaeaenaenen, H. Kalervo; Toppari, Jorma; Jahnukainen, Kirsi; Laitala-Leinonen, Tiina

    2011-01-01

    During postnatal skeletal growth, adaptation to mechanical loading leads to cellular activities at the growth plate. It has recently become evident that bone forming and bone resorbing cells are affected by the receptor tyrosine kinase (RTK) inhibitor imatinib mesylate (STI571, Gleevec (registered) ). Imatinib targets PDGF, ABL-related gene, c-Abl, c-Kit and c-Fms receptors, many of which have multiple functions in the bone microenvironment. We therefore studied the effects of imatinib in growing bone. Young rats were exposed to imatinib (150 mg/kg on postnatal days 5-7, or 100 mg/kg on postnatal days 5-13), and the effects of RTK inhibition on bone physiology were studied after 8 and 70 days (3-day treatment), or after 14 days (9-day treatment). X-ray imaging, computer tomography, histomorphometry, RNA analysis and immunohistochemistry were used to evaluate bone modeling and remodeling in vivo. Imatinib treatment eliminated osteoclasts from the metaphyseal osteochondral junction at 8 and 14 days. This led to a resorption arrest at the growth plate, but also increased bone apposition by osteoblasts, thus resulting in local osteopetrosis at the osteochondral junction. The impaired bone remodelation observed on day 8 remained significant until adulthood. Within the same bone, increased osteoclast activity, leading to bone loss, was observed at distal bone trabeculae on days 8 and 14. Peripheral quantitative computer tomography (pQCT) and micro-CT analysis confirmed that, at the osteochondral junction, imatinib shifted the balance from bone resorption towards bone formation, thereby altering bone modeling. At distal trabecular bone, in turn, the balance was turned towards bone resorption, leading to bone loss. - Research highlights: → 3-Day imatinib treatment. → Causes growth plate anomalies in young rats. → Causes biomechanical changes and significant bone loss at distal trabecular bone. → Results in loss of osteoclasts at osteochondral junction.

  8. Starvation Ketoacidosis: A Cause of Severe Anion Gap Metabolic Acidosis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Nupur Sinha

    2014-01-01

    Full Text Available Pregnancy is a diabetogenic state characterized by relative insulin resistance, enhanced lipolysis, elevated free fatty acids and increased ketogenesis. In this setting, short period of starvation can precipitate ketoacidosis. This sequence of events is recognized as “accelerated starvation.” Metabolic acidosis during pregnancy may have adverse impact on fetal neural development including impaired intelligence and fetal demise. Short periods of starvation during pregnancy may present as severe anion gap metabolic acidosis (AGMA. We present a 41-year-old female in her 32nd week of pregnancy, admitted with severe AGMA with pH 7.16, anion gap 31, and bicarbonate of 5 mg/dL with normal lactate levels. She was intubated and accepted to medical intensive care unit. Urine and serum acetone were positive. Evaluation for all causes of AGMA was negative. The diagnosis of starvation ketoacidosis was established in absence of other causes of AGMA. Intravenous fluids, dextrose, thiamine, and folic acid were administered with resolution of acidosis, early extubation, and subsequent normal delivery of a healthy baby at full term. Rapid reversal of acidosis and favorable outcome are achieved with early administration of dextrose containing fluids.

  9. LPIN1 gene mutations: a major cause of severe rhabdomyolysis in early childhood

    NARCIS (Netherlands)

    Michot, Caroline; Hubert, Laurence; Brivet, Michèle; de Meirleir, Linda; Valayannopoulos, Vassili; Müller-Felber, Wolfgang; Venkateswaran, Ramesh; Ogier, Hélène; Desguerre, Isabelle; Altuzarra, Cécilia; Thompson, Elizabeth; Smitka, Martin; Huebner, Angela; Husson, Marie; Horvath, Rita; Chinnery, Patrick; Vaz, Frederic M.; Munnich, Arnold; Elpeleg, Orly; Delahodde, Agnès; de Keyzer, Yves; de Lonlay, Pascale

    2010-01-01

    Autosomal recessive LPIN1 mutations have been recently described as a novel cause of rhabdomyolysis in a few families. The purpose of the study was to evaluate the prevalence of LPIN1 mutations in patients exhibiting severe episodes of rhabdomyolysis in infancy. After exclusion of primary fatty acid

  10. First report of mango malformation disease caused by Fusarium pseudocircinatum in Mexico

    Science.gov (United States)

    Mango (Mangifera indica L.) malformation disease (MMD) is one of the most important diseases affecting this crop worldwide, causing severe economic loss due to reduction of yield. Subsequent to the first report in India in 1891 (3), MMD has spread worldwide to most mango-growing regions. Several spe...

  11. Severe hypoglycaemia associated with ingesting counterfeit medication.

    Science.gov (United States)

    Chaubey, Santosh K; Sangla, Kunwarjit S; Suthaharan, Emershia N; Tan, Yong M

    2010-06-21

    Cross-border importation of traditional and prescription medications is common, and many of these drugs are not approved by the Australian Therapeutic Goods Administration. Furthermore, counterfeit versions of prescription medications are also available (eg, weight-loss medications, anabolic steroids, and medications to enhance sexual performance). We describe a 54-year-old man with the first Australian case of severe hypoglycaemia induced by imported, laboratory-confirmed counterfeit Cialis. This serves to remind medical practitioners that counterfeit medication may be the cause of severe hypoglycaemia (or other unexplained illness).

  12. Mutations in LPL, APOC2, APOA5, GPIHBP1 and LMF1 in patients with severe hypertriglyceridaemia

    NARCIS (Netherlands)

    Surendran, R P; Visser, M E; Heemelaar, S; Wang, J; Peter, J; Defesche, J C; Kuivenhoven, J A; Hosseini, M; Péterfy, M; Kastelein, J J P; Johansen, C T; Hegele, R A; Stroes, E S G; Dallinga-Thie, G M

    OBJECTIVES: The severe forms of hypertriglyceridaemia (HTG) are caused by mutations in genes that lead to the loss of function of lipoprotein lipase (LPL). In most patients with severe HTG (TG > 10 mmol L(-1) ), it is a challenge to define the underlying cause. We investigated the molecular basis of

  13. Traumatic retropharyngeal emphysema as a cause for severe respiratory distress in a newborn

    International Nuclear Information System (INIS)

    Barlev, Dan M.; Nagourney, Beth A.; Saintonge, Ronald

    2003-01-01

    Traumatic injury to the pharynx or esophagus in a newborn from intubation or tube suctioning may have various presentations. Difficulty passing a gastric tube or feeding problems may erroneously suggest the diagnosis of esophageal atresia. Associated respiratory distress may be caused by pneumothorax or pleural effusion if the pleural space is entered. We report the case of a full-term newborn presenting with severe respiratory distress caused by a large retropharyngeal air collection resulting from hypopharyngeal perforation from prior intubation and suctioning. Chest abnormality, sufficient to account for the degree of respiratory distress, was not demonstrated. (orig.)

  14. A Case Report of Unilateral Severe Visual Loss Along with Bilateral Optic Disc Cupping Secondary to Metastatic Brain Tumor

    Directory of Open Access Journals (Sweden)

    M Mahdavi

    2006-07-01

    Full Text Available Purpose: To report a case of unilateral severe visual loss and bilateral optic disc cupping secondary to brain metastasis of bronchogenic carcinoma Patient and findings: A 48 year-old woman presented with severe visual loss of left eye without redness or pain or any systemic findings .Clinical findings included decreased visual acuity of left eye to 4 m CF and (+3 positive Marcus-Gunn reflex .There was asymmetric optic disc cupping associated with visual field defect in left eye The neurologic investigations showed a secondary metastatic tumor in the brain from bronchogenic carcinoma. Conclusion: Before making a diagnosis of normal -tension glaucoma in asymmetric optic disc cupping and normal intraocular pressure, ophthalmologists should rule out neurologic defects and brain tumors.

  15. The loss rates of O{sup +} in the inner magnetosphere caused by both magnetic field line curvature scattering and charge exchange reactions

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Y., E-mail: yji@spaceweather.ac.cn [State Key Laboratory of Space Weather, Center for Space Science and Applied Research, Chinese Academy of Sciences, Beijing 100190 (China); College of Earth Science, University of Chinese Academy of Sciences, Beijing 100049 (China); Shen, C. [State Key Laboratory of Space Weather, Center for Space Science and Applied Research, Chinese Academy of Sciences, Beijing 100190 (China)

    2014-03-15

    With consideration of magnetic field line curvature (FLC) pitch angle scattering and charge exchange reactions, the O{sup +} (>300 keV) in the inner magnetosphere loss rates are investigated by using an eigenfunction analysis. The FLC scattering provides a mechanism for the ring current O{sup +} to enter the loss cone and influence the loss rates caused by charge exchange reactions. Assuming that the pitch angle change is small for each scattering event, the diffusion equation including a charge exchange term is constructed and solved; the eigenvalues of the equation are identified. The resultant loss rates of O{sup +} are approximately equal to the linear superposition of the loss rate without considering the charge exchange reactions and the loss rate associated with charge exchange reactions alone. The loss time is consistent with the observations from the early recovery phases of magnetic storms.

  16. Near total penile amputation caused by hair tourniquet, managed ...

    African Journals Online (AJOL)

    The hair tourniquet syndrome is an unusual phenomenon which can cause severe damage to the affected organ, sometimes even its loss. This problem requires urgent attention and prompt management to ensure anatomical and functional preservation. Herein we report a case of penile hair tourniquet which was ...

  17. An improved iron loss estimation for permanent magnet brushless machines

    CERN Document Server

    Fang, D

    1999-01-01

    This paper presents an improved approach for predicting iron losses in permanent magnet brushless machines. The new approach is based on the fundamental concept that eddy current losses are proportional to the square of the time rate of change of flux density. Expressions are derived for predicting hysteresis and eddy current losses in the stator teeth and yoke. The so-called anomalous or excess losses, caused by the induced eddy current concentration around moving magnetic domain walls and neglected in the conventional core loss calculation, are also included in the proposed approach. In addition, the model is also capable of accounting for the stator skewing, if present. The core losses obtained from the proposed approach are compared with those measured on an existing PM motor at several operating speeds, showing very good agreement. (14 refs).

  18. Severe Hypothyroidism due to the Loss of Therapeutic Efficacy of l-Thyroxine in a Patient with Esophageal Complication Associated with Systemic Sclerosis.

    Science.gov (United States)

    Lobasso, Antonio; Nappi, Liliana; Barbieri, Letizia; Peirce, Carmela; Ippolito, Serena; Arpaia, Debora; Rossi, Francesca Wanda; de Paulis, Amato; Biondi, Bernadette

    2017-01-01

    Thyroid function abnormalities and thyroid autoantibodies have been frequently described in patients with systemic autoimmune diseases as systemic sclerosis (SSc). Serum TSH levels are higher in SSc patients with more severe skin diseases and a worse modified Rodnan skin score. Asymptomatic esophageal involvement due to SSc has never been described as a cause of severe hypothyroidism due to l-thyroxine (l-T4) malabsorption in patients with Hashimoto's thyroiditis (HT) and SSc. Here, we report a case of a 56-year-old female affected by both SSc and HT who developed severe hypothyroidism due to the loss of therapeutic efficacy of l-T4. Therapeutic failure resulted from the altered l-T4 absorption because of SSc esophageal complications. Clinical findings improved after the administration of oral liquid l-T4. Thyroid function completely normalized with a full clinical recovery, the disappearance of the pericardial effusion and the improvement of the pulmonary pressure. A recognition of a poor absorption is crucial in patients with hypothyroidism and SSc to reduce the risk of the subsequent adverse events. This case suggests the importance of clinical and laboratory surveillance in patients with SSc and HT because the systemic complications of these dysfunctions may worsen the prognosis of hypothyroid SSc/HT patients.

  19. De novo loss- or gain-of-function mutations in KCNA2 cause epileptic encephalopathy

    DEFF Research Database (Denmark)

    Syrbe, Steffen; Hedrich, Ulrike B S; Riesch, Erik

    2015-01-01

    disability, delayed speech development and sometimes ataxia. Functional studies of the two mutations associated with this phenotype showed almost complete loss of function with a dominant-negative effect. Two further individuals presented with a different and more severe epileptic encephalopathy phenotype....... They carried mutations inducing a drastic gain-of-function effect leading to permanently open channels. These results establish KCNA2 as a new gene involved in human neurodevelopmental disorders through two different mechanisms, predicting either hyperexcitability or electrical silencing of KV1.2-expressing...

  20. Near total penile amputation caused by hair tourniquet, managed ...

    African Journals Online (AJOL)

    V.K. Dikshit

    2015-12-02

    Dec 2, 2015 ... Abstract. The hair tourniquet syndrome is an unusual phenomenon which can cause severe damage to the affected organ, sometimes even its loss. This problem requires urgent attention and prompt management to ensure anatomical and functional preservation. Herein we report a case of penile hair ...

  1. A case of severe anorexia, excessive weight loss and high peptide YY levels after sleeve gastrectomy.

    Science.gov (United States)

    Pucci, Andrea; Cheung, Wui Hang; Jones, Jenny; Manning, Sean; Kingett, Helen; Adamo, Marco; Elkalaawy, Mohamed; Jenkinson, Andrew; Finer, Nicholas; Doyle, Jacqueline; Hashemi, Majid; Batterham, Rachel L

    2015-01-01

    Sleeve gastrectomy (SG) is the second most commonly performed bariatric procedure worldwide. Altered circulating gut hormones have been suggested to contribute post-operatively to appetite suppression, decreased caloric intake and weight reduction. In the present study, we report a 22-year-old woman who underwent laparoscopic SG for obesity (BMI 46 kg/m(2)). Post-operatively, she reported marked appetite reduction, which resulted in excessive weight loss (1-year post-SG: BMI 22 kg/m(2), weight loss 52%, >99th centile of 1-year percentage of weight loss from 453 SG patients). Gastrointestinal (GI) imaging, GI physiology/motility studies and endoscopy revealed no anatomical cause for her symptoms, and psychological assessments excluded an eating disorder. Despite nutritional supplements and anti-emetics, her weight loss continued (BMI 19 kg/m(2)), and she required nasogastric feeding. A random gut hormone assessment revealed high plasma peptide YY (PYY) levels. She underwent a 3 h meal study following an overnight fast to assess her subjective appetite and circulating gut hormone levels. Her fasted nausea scores were high, with low hunger, and these worsened with nutrient ingestion. Compared to ten other post-SG female patients, her fasted circulating PYY and nutrient-stimulated PYY and active glucagon-like peptide 1 (GLP1) levels were markedly elevated. Octreotide treatment was associated with suppressed circulating PYY and GLP1 levels, increased appetite, increased caloric intake and weight gain (BMI 22 kg/m(2) after 6 months). The present case highlights the value of measuring gut hormones in patients following bariatric surgery who present with anorexia and excessive weight loss and suggests that octreotide treatment can produce symptomatic relief and weight regain in this setting. Roux-en-Y gastric bypass and SG produce marked sustained weight reduction. However, there is a marked individual variability in this reduction, and post-operative weight loss

  2. Auditory agnosia due to long-term severe hydrocephalus caused by spina bifida - specific auditory pathway versus nonspecific auditory pathway.

    Science.gov (United States)

    Zhang, Qing; Kaga, Kimitaka; Hayashi, Akimasa

    2011-07-01

    A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear. Her bilateral auditory cortices were preserved, as shown by neuroimaging, whereas her auditory radiations were severely damaged owing to progressive hydrocephalus. Although she had a complete bilateral hearing loss, she felt great pleasure when exposed to music. After years of self-training to read lips, she regained fluent ability to communicate. Clinical manifestations of this patient indicate that auditory agnosia can occur after long-term hydrocephalus due to spina bifida; the secondary auditory pathway may play a role in both auditory perception and hearing rehabilitation.

  3. Treatment of Obesity: Weight Loss and Bariatric Surgery

    Science.gov (United States)

    Wolfe, Bruce M.; Kvach, Elizaveta; Eckel, Robert H.

    2016-01-01

    This review focuses on the mechanisms underlying, and indications for, bariatric surgery in the reduction of cardiovascular disease (CVD) as well as other expected benefits of this intervention. The fundamental basis for bariatric surgery for the purpose of accomplishing weight loss is the determination that severe obesity is a disease associated with multiple adverse effects on health which can be reversed or improved by successful weight loss in patients who have been unable to sustain weight loss by non-surgical means. An explanation of possible indications for weight loss surgery as well as specific bariatric surgical procedures is presented, along with review of the safety literature of such procedures. Procedures that are less invasive or those that involve less gastrointestinal rearrangement accomplish considerably less weight loss but have substantially lower perioperative and longer-term risk. The ultimate benefit of weight reduction relates to the reduction of the co-morbidities, quality of life and all-cause mortality. With weight loss being the underlying justification for bariatric surgery in ameliorating CVD risk, current evidence-based research is discussed concerning body fat distribution, dyslipidemia, hypertension, diabetes, inflammation, obstructive sleep apnea and others. The rationale for bariatric surgery reducing CVD events is discussed and juxtaposed with impacts on all-cause mortalities. Given the improvement of established obesity-related CVD risk factors following weight loss, it is reasonable to expect a reduction of CVD events and related mortality following weight loss in populations with obesity. The quality of the current evidence is reviewed and future research opportunities and summaries are stated. PMID:27230645

  4. No upward trend in normalised windstorm losses in Europe: 1970-2008

    Science.gov (United States)

    Barredo, J. I.

    2010-01-01

    On 18 January 2007, windstorm Kyrill battered Europe with hurricane-force winds killing 47 people and causing 10 billion US in damage. Kyrill poses several questions: is Kyrill an isolated or exceptional case? Have there been events costing as much in the past? This paper attempts to put Kyrill into an historical context by examining large historical windstorm event losses in Europe for the period 1970-2008 across 29 European countries. It asks the question what economic losses would these historical events cause if they were to recur under 2008 societal conditions? Loss data were sourced from reinsurance firms and augmented with historical reports, peer-reviewed articles and other ancillary sources. Following the same conceptual approach outlined in previous studies, the data were then adjusted for changes in population, wealth, and inflation at the country level and for inter-country price differences using purchasing power parity. The analyses reveal no trend in the normalised windstorm losses and confirm increasing disaster losses are driven by societal factors and increasing exposure.

  5. Evidence of increasing drought severity caused by temperature rise in southern Europe

    International Nuclear Information System (INIS)

    Vicente-Serrano, Sergio M; Lopez-Moreno, Juan-I; Lorenzo-Lacruz, Jorge; García-Ruiz, José M; Azorin-Molina, Cesar; Morán-Tejeda, Enrique; Revuelto, Jesús; Beguería, Santiago; Sanchez-Lorenzo, Arturo; Trigo, Ricardo; Coelho, Fatima; Espejo, Francisco

    2014-01-01

    We use high quality climate data from ground meteorological stations in the Iberian Peninsula (IP) and robust drought indices to confirm that drought severity has increased in the past five decades, as a consequence of greater atmospheric evaporative demand resulting from temperature rise. Increased drought severity is independent of the model used to quantify the reference evapotranspiration. We have also focused on drought impacts to drought-sensitive systems, such as river discharge, by analyzing streamflow data for 287 rivers in the IP, and found that hydrological drought frequency and severity have also increased in the past five decades in natural, regulated and highly regulated basins. Recent positive trend in the atmospheric water demand has had a direct influence on the temporal evolution of streamflows, clearly identified during the warm season, in which higher evapotranspiration rates are recorded. This pattern of increase in evaporative demand and greater drought severity is probably applicable to other semiarid regions of the world, including other Mediterranean areas, the Sahel, southern Australia and South Africa, and can be expected to increasingly compromise water supplies and cause political, social and economic tensions among regions in the near future. (paper)

  6. Seroprevalence of Bovine Anaplasmosis Caused by Anaplasma Marginale in Malaysia

    OpenAIRE

    Pong, Samantha; Nik-Him, Nik-Ahmad I. I

    2012-01-01

    Anaplasmosis, also known as yellow fever, is an infectious parasitic disease of cattle caused by the protozoan Anaplasma marginale. Anaplasma marginale infects the erythrocytes and causes severe anaemia, weakness, loss of appetite, fever, depression, abortion, decreased milk production, constipation, jaundice and sometimes death. In Malaysia, data on A. marginale infection is still behind compared to other parasites such as nematodes. Anaplasmosis in livestock has received little attention in...

  7. Aetiology and treatment of severe postpartum haemorrhage

    DEFF Research Database (Denmark)

    Edwards, Hellen

    2017-01-01

    This thesis is comprised of three studies focusing on severe postpartum haemorrhage (PPH). PPH is a major cause of maternal morbidity and mortality worldwide. Risk factors include retained placenta, prolonged duration of the third stage of labour, previous caesarean section, and operative vaginal...... delivery. Occurrence and development of PPH are, however, unpredictable and can sometimes give rise to massive haemorrhage or even hysterectomy and maternal death. Severe haemorrhage can lead to coagulopathy causing further haemorrhage and requiring substitution with blood transfusions. The aim...... had a cardiac arrest, and a total of 128 women (52%) required a hysterectomy. Hysterectomy was associated with increased blood loss, increased number of blood transfusions, a higher fresh frozen plasma to red blood cell ratio (p=0.010), and an increased number of red blood cells before first platelet...

  8. Hormonal causes of recurrent pregnancy loss (RPL).

    Science.gov (United States)

    Pluchino, Nicola; Drakopoulos, Panagiotis; Wenger, Jean Marie; Petignat, Patrick; Streuli, Isabelle; Genazzani, Andrea Riccardo

    2014-01-01

    Endocrine disorders play a major role in approximately 8% to 12% of recurrent pregnancy loss (RPL). Indeed, the local hormonal milieu is crucial in both embryo attachment and early pregnancy. Endocrine abnormalities, including thyroid disorders, luteal phase defects, polycystic ovary syndrome, hyperprolactinaemia and diabetes have to be evaluated in any case of RPL. Moreover, elevated androgen levels and some endocrinological aspects of endometriosis are also factors contributing to RPL. In the present article, we review the significance of endocrine disease on RPL.

  9. HYPERTENSIVE CHOROIDOPATHY AS AN UNCOMMON CAUSE S OF VISUAL LOSS IN PREGNANCY

    Directory of Open Access Journals (Sweden)

    Bijnya Birajita

    2015-08-01

    Full Text Available INTRODUCTION: HISTORY: A 30 year old lady in her 32 weeks gestation period was referred to our ophthalmology clinic with chief complaints of diminution of vision in both eyes for 4 months duration. Her medical history was suggestive of uncontrolled hypertension. On Ophthalmic evaluation she had best corrected visual acuity of 20/200 in both eyes with normal color vision. Her pupils were bilaterally equal in size and normally reacting to light. The intraocular pressure was within normal range and anterior segment evaluation was within normal limits. Dilated fundus evaluation revealed only arterio - venous crossing changes, mottled retina and resolving hard exudates in both eyes [Fig 1a, & 1 b]. The fundus picture could not explain the severity of vision loss in the patient which prompted us for further workup.

  10. Management of Children with Mild, Moderate, and Moderately Severe Sensorineural Hearing Loss.

    Science.gov (United States)

    Tharpe, Anne Marie; Gustafson, Samantha

    2015-12-01

    Any degree of hearing loss can have a negative impact on child development. The amount of impact is largely determined by the type, quality, and timeliness of intervention. Early identification and management of hearing loss is essential for minimizing the impact of hearing loss and ensuring that children can reach their cognitive, linguistic, educational, and social potential. Advances in hearing technology and broadening of candidacy for same, have resulted in improved outcomes for many children with hearing loss. Through ongoing hearing monitoring throughout childhood, children with congenital, late-onset, or progressive losses can receive timely management from interprofessional, collaborative teams. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Root rot symptoms in sugar beet lines caused by Fusarium oxysporum f. sp. betae

    Science.gov (United States)

    The soil-borne fungus Fusarium oxysporum may cause both Fusarium yellows and Fusarium root rot diseases with severe yield losses in cultivated sugar beet worldwide. These two diseases cause similar foliar symptoms but different root response and have been proposed to be due to two distinct F. oxyspo...

  12. A mutation in the centriole-associated protein centrin causes genomic instability via increased chromosome loss in Chlamydomonas reinhardtii

    Directory of Open Access Journals (Sweden)

    Marshall Wallace F

    2005-05-01

    Full Text Available Abstract Background The role of centrioles in mitotic spindle function remains unclear. One approach to investigate mitotic centriole function is to ask whether mutation of centriole-associated proteins can cause genomic instability. Results We addressed the role of the centriole-associated EF-hand protein centrin in genomic stability using a Chlamydomonas reinhardtii centrin mutant that forms acentriolar bipolar spindles and lacks the centrin-based rhizoplast structures that join centrioles to the nucleus. Using a genetic assay for loss of heterozygosity, we found that this centrin mutant showed increased genomic instability compared to wild-type cells, and we determined that the increase in genomic instability was due to a 100-fold increase in chromosome loss rates compared to wild type. Live cell imaging reveals an increased rate in cell death during G1 in haploid cells that is consistent with an elevated rate of chromosome loss, and analysis of cell death versus centriole copy number argues against a role for multipolar spindles in this process. Conclusion The increased chromosome loss rates observed in a centrin mutant that forms acentriolar spindles suggests a role for centrin protein, and possibly centrioles, in mitotic fidelity.

  13. Surgical correction of severe enophthalmos caused by bullet injury

    Directory of Open Access Journals (Sweden)

    S M Balaji

    2016-01-01

    Full Text Available Ballistic injuries of oral and maxillofacial region are usually fatal due to close propinquity with the vital structures. The severity of injury depends on the caliber of the weapon used and distance from which the patient is shot. The preliminary care of facial ballistic wounds strictly adheres to the basics of trauma resuscitation. Early and appropriate surgical management has proved to be influential on the final outcome and esthetic result. Treatment of facial gunshot wounds should be planned and carried out carefully to avoid esthetic complications. It takes even multiple-staged corrections to achieve the targeted functional and esthetic treatment plan. Prevention and control of infection is one of the most important goals to achieve the success of the treatment. Herewith, we present a case of facial gunshot injury with fractures in the orbital floor, medial wall maxillary sinus, and buttress of the zygomatic bone causing deficit, which was successfully managed by surgical reconstruction.

  14. Anomalous delayed loss of trapped D-D fusion products in TFTR

    International Nuclear Information System (INIS)

    Zweben, S.J.; Darrow, D.S.; Fredrickson, E.D.; Mynick, H.E.

    1993-02-01

    A new anomalous delayed loss of D-D fusion products has been measured at the bottom of the TFRR vessel. This loss is delayed by ∼ 0.2 sec with respect to the usual prompt first-orbit loss, and has a correspondingly lower energy, i.e. about half the fusion product birth energy. This loss process dominates the total fusion product loss measured 90 degrees below the midplane for plasma currents. I≥ 1.8 MA and major radii near R=2.45 m, e.g. for recent TFTR supershots. This delayed feature can occur without large coherent MED activity, although it can be strongly modulated by such activity. Several possible causes for this phenomenon are discussed, but no clear explanation for this delayed loss has yet been found

  15. Transiently increasing cAMP levels selectively in hippocampal excitatory neurons during sleep deprivation prevents memory deficits caused by sleep loss

    NARCIS (Netherlands)

    Havekes, Robbert; Bruinenberg, Vibeke M.; Tudor, Jennifer C.; Ferri, Sarah L.; Baumann, Arnd; Meerlo, Peter; Abel, Ted

    2014-01-01

    The hippocampus is particularly sensitive to sleep loss. Although previous work has indicated that sleep deprivation impairs hippocampal cAMP signaling, it remains to be determined whether the cognitive deficits associated with sleep deprivation are caused by attenuated cAMP signaling in the

  16. Loss-of-function mutation in RUSC2 causes intellectual disability and secondary microcephaly.

    Science.gov (United States)

    Alwadei, Ali H; Benini, Ruba; Mahmoud, Adel; Alasmari, Ali; Kamsteeg, Erik-Jan; Alfadhel, Majid

    2016-12-01

    Inherited aberrancies in intracellular vesicular transport are associated with a variety of neurological and non-neurological diseases. RUSC2 is a gene found on chromosome 9p13.3 that codes for iporin, a ubiquitous protein with high expression in the brain that interacts with Rab proteins (GTPases implicated in intracellular protein trafficking). Although mutations in Rab proteins have been described as causing brain abnormalities and intellectual disability, until now no disease-causing mutations in RUSC2 have ever been reported in humans. We describe, to our knowledge for the first time, three patients with inherited homozygous nonsense mutations identified in RUSC2 on whole-exome sequencing. All three patients had central hypotonia, microcephaly, and moderate to severe intellectual disability. Two patients had additional features of early-onset epilepsy and absence of the splenium. This report adds to the ever-expanding landscape of genetic causes of intellectual disability and increases our understanding of the cellular processes underlying this important neurological entity. © 2016 Mac Keith Press.

  17. Honeybee immunity and colony losses

    Directory of Open Access Journals (Sweden)

    F. Nazzi

    2014-10-01

    Full Text Available The decline of honeybee colonies and their eventual collapse is a widespread phenomenon in the Northern hemisphere of the globe, which severely limits the beekeeping industry. This dramatic event is associated with an enhanced impact of parasites and pathogens on honeybees, which is indicative of reduced immunocompetence. The parasitic mite Varroa destructor and the vectored viral pathogens appear to play a key-role in the induction of this complex syndrome. In particular, the Deformed Wing Virus (DWV is widespread and is now considered, along with Varroa, one of the major causes of bee colony losses. Several lines of evidence indicate that this mite/DWV association severely affects the immune system of honeybees and makes them more sensitive to the action of other stress factors. The molecular mechanisms underpinning these complex interactions are currently being investigated and the emerging information has allowed the development of a new functional model, describing how different stress factors may synergistically concur in the induction of bee immune alteration and health decline. This provides a new logical framework in which to interpret the proposed multifactorial origin of bee colony losses and sets the stage for a more comprehensive and integrated analysis of the effect that multiple stress agents may have on honeybees.

  18. Comprehensive Treatment of Severe Periodontal and Periimplant Bone Destruction Caused by Iatrogenic Factors

    Directory of Open Access Journals (Sweden)

    Gregor-Georg Zafiropoulos

    2018-01-01

    Full Text Available Dental implant success requires placement after periodontal therapy, with adequate bone volume, plaque control, primary stability, control of risk factors, and use of well-designed prostheses. This report describes the surgical and prosthetic management of a patient with severe iatrogenic periodontal/periimplant bone destruction. Methods. A 55-year-old female smoker with fixed partial dentures (FPDs supported on teeth and implants presented with oral pain, swelling, bleeding, and a 10-year history of multiple implant placements and implants/prosthesis failures/replacements. Radiographs showed severe bone loss, subgingival caries, and periapical lesions. All implants and teeth were removed except implants #4 and #10 which served to retain an interim maxillary restoration. Bone defects were covered with nonresorbable dPTFE membranes. In the mandible, three new implants were placed and loaded immediately with a bar-retained temporary denture. Results. Seven months postoperatively, the bone defects were regenerated, and three additional mandibular implants were placed. All mandibular implants were splinted and loaded with a removable overdenture. Conclusions. In this case, periimplant infection and tissue destruction resulted from the lack of periodontal treatment/maintenance and failure to use evidence-based surgical and loading protocols. Combination therapy resolved the disease and the patient's severe discomfort while providing immediate function and an aesthetic solution.

  19. Dental erosion caused by gastroesophageal reflux disease: a case report

    OpenAIRE

    Cengiz, Seda; Cengiz, M ?nan?; Sara?, Y ?inasi

    2009-01-01

    Introduction Chronic regurgitation of gastric acids in patients with gastroesophageal reflux disease may cause dental erosion, which can lead in combination with attrition or bruxism to extensive loss of coronal tooth tissue. Case presentation This clinical report describes treatment of severe tooth wear of a gastroesophageal reflux disease patient who is 54-year-old Turkish male patient. After his medical treatment, severe tooth wear, bruxism and decreased vertical dimensions were determined...

  20. [From gene to disease; genetic causes of hearing loss and visual impairment sometimes accompanied by vestibular problems (Usher syndrome)].

    Science.gov (United States)

    Pennings, R J E; Kremer, H; Deutman, A F; Kimberling, W J; Cremers, C W R J

    2002-12-07

    Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their own genetic subtypes (Usher 1A-Usher IG, Usher 2A-Usher 2C and Usher 3). The majority of the Usher type I cases are caused by mutations in the MYO7A gene (Usher 1B) while mutations in the USH2A gene (Usher 2A) are the cause of most cases of type II. Usher syndrome type III, caused by mutations in the USH3 gene, is frequently seen only in Finland.

  1. De novo loss-of-function mutations in WAC cause a recognizable intellectual disability syndrome and learning deficits in Drosophila.

    Science.gov (United States)

    Lugtenberg, Dorien; Reijnders, Margot R F; Fenckova, Michaela; Bijlsma, Emilia K; Bernier, Raphael; van Bon, Bregje W M; Smeets, Eric; Vulto-van Silfhout, Anneke T; Bosch, Danielle; Eichler, Evan E; Mefford, Heather C; Carvill, Gemma L; Bongers, Ernie M H F; Schuurs-Hoeijmakers, Janneke Hm; Ruivenkamp, Claudia A; Santen, Gijs W E; van den Maagdenberg, Arn M J M; Peeters-Scholte, Cacha M P C D; Kuenen, Sabine; Verstreken, Patrik; Pfundt, Rolph; Yntema, Helger G; de Vries, Petra F; Veltman, Joris A; Hoischen, Alexander; Gilissen, Christian; de Vries, Bert B A; Schenck, Annette; Kleefstra, Tjitske; Vissers, Lisenka E L M

    2016-08-01

    Recently WAC was reported as a candidate gene for intellectual disability (ID) based on the identification of a de novo mutation in an individual with severe ID. WAC regulates transcription-coupled histone H2B ubiquitination and has previously been implicated in the 10p12p11 contiguous gene deletion syndrome. In this study, we report on 10 individuals with de novo WAC mutations which we identified through routine (diagnostic) exome sequencing and targeted resequencing of WAC in 2326 individuals with unexplained ID. All but one mutation was expected to lead to a loss-of-function of WAC. Clinical evaluation of all individuals revealed phenotypic overlap for mild ID, hypotonia, behavioral problems and distinctive facial dysmorphisms, including a square-shaped face, deep set eyes, long palpebral fissures, and a broad mouth and chin. These clinical features were also previously reported in individuals with 10p12p11 microdeletion syndrome. To investigate the role of WAC in ID, we studied the importance of the Drosophila WAC orthologue (CG8949) in habituation, a non-associative learning paradigm. Neuronal knockdown of Drosophila CG8949 resulted in impaired learning, suggesting that WAC is required in neurons for normal cognitive performance. In conclusion, we defined a clinically recognizable ID syndrome, caused by de novo loss-of-function mutations in WAC. Independent functional evidence in Drosophila further supported the role of WAC in ID. On the basis of our data WAC can be added to the list of ID genes with a role in transcription regulation through histone modification.

  2. Abrupt fire regime change may cause landscape-wide loss of mature obligate seeder forests.

    Science.gov (United States)

    Bowman, David M J S; Murphy, Brett P; Neyland, Dominic L J; Williamson, Grant J; Prior, Lynda D

    2014-03-01

    Obligate seeder trees requiring high-severity fires to regenerate may be vulnerable to population collapse if fire frequency increases abruptly. We tested this proposition using a long-lived obligate seeding forest tree, alpine ash (Eucalyptus delegatensis), in the Australian Alps. Since 2002, 85% of the Alps bioregion has been burnt by several very large fires, tracking the regional trend of more frequent extreme fire weather. High-severity fires removed 25% of aboveground tree biomass, and switched fuel arrays from low loads of herbaceous and litter fuels to high loads of flammable shrubs and juvenile trees, priming regenerating stands for subsequent fires. Single high-severity fires caused adult mortality and triggered mass regeneration, but a second fire in quick succession killed 97% of the regenerating alpine ash. Our results indicate that without interventions to reduce fire severity, interactions between flammability of regenerating stands and increased extreme fire weather will eliminate much of the remaining mature alpine ash forest. © 2013 John Wiley & Sons Ltd.

  3. Study on severe accident induced by large break loss of coolant accident for pressureized water reactor

    International Nuclear Information System (INIS)

    Zhang Longfei; Zhang Dafa; Wang Shaoming

    2007-01-01

    Using the best estimate computer code SCDAP/RELAP5/MOD3.2 and taking US Westinghouse corporation Surry nuclear power plant as the reference object, a typical three-loop pressurized water reactor severe accident calculation model was established and 25 cm large break loss of coolant accident (LBLOCA) in cold and hot leg of primary loop induced core melt accident was analyzed. The calculated results show that core melt progression is fast and most of the core material melt and relocated to the lower plenum. The lower head of reactor pressure vessel failed at an early time and the cold leg break is more severe than the hot leg break in primary loop during LBLOCA. (authors)

  4. Help! It's Hair Loss!

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Hair Loss KidsHealth / For Kids / Hair Loss What's in this ... head are in the resting phase. What Causes Hair Loss? Men, especially older men, are the ones who ...

  5. Severe Hypothyroidism due to the Loss of Therapeutic Efficacy of l-Thyroxine in a Patient with Esophageal Complication Associated with Systemic Sclerosis

    Directory of Open Access Journals (Sweden)

    Antonio Lobasso

    2017-09-01

    Full Text Available BackgroundThyroid function abnormalities and thyroid autoantibodies have been frequently described in patients with systemic autoimmune diseases as systemic sclerosis (SSc. Serum TSH levels are higher in SSc patients with more severe skin diseases and a worse modified Rodnan skin score. Asymptomatic esophageal involvement due to SSc has never been described as a cause of severe hypothyroidism due to l-thyroxine (l-T4 malabsorption in patients with Hashimoto’s thyroiditis (HT and SSc.Case reportHere, we report a case of a 56-year-old female affected by both SSc and HT who developed severe hypothyroidism due to the loss of therapeutic efficacy of l-T4. Therapeutic failure resulted from the altered l-T4 absorption because of SSc esophageal complications. Clinical findings improved after the administration of oral liquid l-T4. Thyroid function completely normalized with a full clinical recovery, the disappearance of the pericardial effusion and the improvement of the pulmonary pressure.ConclusionA recognition of a poor absorption is crucial in patients with hypothyroidism and SSc to reduce the risk of the subsequent adverse events. This case suggests the importance of clinical and laboratory surveillance in patients with SSc and HT because the systemic complications of these dysfunctions may worsen the prognosis of hypothyroid SSc/HT patients.

  6. Young Women's Phenomenological Sense of Father and Parental Marital Relationship and Their Relation to Paternal Loss.

    Science.gov (United States)

    Darlington, Susan

    The meaning fathers have in the lives of women as they are growing up was investigated. Women's perceptions of their fathers on several factors were measured to determine how these perceptions differed with father loss, cause and time of loss, perception of parental marital relationships, and presence of an older brother. Questionnaires were…

  7. Two novel mutations in thymidine kinase-2 cause early onset fatal encephalomyopathy and severe mtDNA depletion.

    Science.gov (United States)

    Lesko, Nicole; Naess, Karin; Wibom, Rolf; Solaroli, Nicola; Nennesmo, Inger; von Döbeln, Ulrika; Karlsson, Anna; Larsson, Nils-Göran

    2010-03-01

    Deficiency of thymidine kinase-2 (TK2) has been described in children with early onset fatal skeletal myopathy. TK2 is a mitochondrial deoxyribonucleoside kinase required for the phosphorylation of deoxycytidine and deoxythymidine and hence is vital for the maintenance of a balanced mitochondrial dNTP pool in post-mitotic tissues. We describe a patient with two novel TK2 mutations, which caused disease onset shortly after birth and death at the age of three months. One mutation (219insCG) generated an early stop codon, thus preventing the synthesis of a functional protein. The second mutation (R130W) resulted in an amino acid substitution, which caused a severe reduction (TK2 enzyme activity. These two novel TK2 mutations cause an extremely severe phenotype with overwhelming central nervous system symptoms not commonly seen in patients with TK2-deficiency. We conclude that the severe clinical presentation in this patient was due to a virtual lack of mitochondrial TK2 activity. Copyright 2009 Elsevier B.V. All rights reserved.

  8. Protein loss in human hair from combination straightening and coloring treatments.

    Science.gov (United States)

    França-Stefoni, Simone Aparecida; Dario, Michelli Ferrera; Sá-Dias, Tânia Cristina; Bedin, Valcinir; de Almeida, Adriano José; Baby, André Rolim; Velasco, Maria Valéria R

    2015-09-01

    Hair chemical treatments, such as dyeing and straightening products, are known to cause damage that can be assessed by protein loss. The aim of this study was to evaluate the hair protein loss caused by combined chemical treatments (dye and relaxer) using the validated bicinchoninic acid (BCA) method. Three kinds of straighteners, based on ammonium thioglycolate, guanidine hydroxide and sodium hydroxide, were evaluated and the least harmful combination indicated. Caucasian virgin dark brown hair tresses were treated with developed natural brown color oxidative hair dyeing and/or straightening commercial products based on ammonium thioglycolate, sodium hydroxide, or guanidine hydroxide. Protein loss quantification was assessed by the validated BCA method which has several advantages for quantifying protein loss in chemically treated hair. When both treatments (straightening and dyeing) were combined, a higher negative effect was observed, particularly for dyed hair treated with sodium hydroxide. In this case, a 356% increase in protein loss relative to virgin hair was observed and 208% in relation to only dyed hair. The combination of dying and relaxers based on ammonium thioglycolate or guanidine hydroxide caused a small increase in protein loss, suggesting that these straightening products could be the best alternatives for individuals wishing to combine both treatments. These results indicated that when application of both types of products is desired, ammonium thioglycolate or guanidine hydroxide should be chosen for the straightening process. © 2015 Wiley Periodicals, Inc.

  9. Staphylococcus aureus seroproteomes discriminate ruminant isolates causing mild or severe mastitis

    Directory of Open Access Journals (Sweden)

    Le Maréchal Caroline

    2011-02-01

    Full Text Available Abstract Staphylococcus aureus is a major cause of mastitis in ruminants. In ewe mastitis, symptoms range from subclinical to gangrenous mastitis. S. aureus factors or host-factors contributing to the different outcomes are not completely elucidated. In this study, experimental mastitis was induced on primiparous ewes using two S. aureus strains, isolated from gangrenous (strain O11 or subclinical (strain O46 mastitis. Strains induced drastically distinct clinical symptoms when tested in ewe and mice experimental mastitis. Notably, they reproduced mild (O46 or severe (O11 mastitis in ewes. Ewe sera were used to identify staphylococcal immunoreactive proteins commonly or differentially produced during infections of variable severity and to define core and accessory seroproteomes. Such SERological Proteome Analysis (SERPA allowed the identification of 89 immunoreactive proteins, of which only 52 (58.4% were previously identified as immunogenic proteins in other staphylococcal infections. Among the 89 proteins identified, 74 appear to constitute the core seroproteome. Among the 15 remaining proteins defining the accessory seroproteome, 12 were specific for strain O11, 3 were specific for O46. Distribution of one protein specific for each mastitis severity was investigated in ten other strains isolated from subclinical or clinical mastitis. We report here for the first time the identification of staphylococcal immunogenic proteins common or specific to S. aureus strains responsible for mild or severe mastitis. These findings open avenues in S. aureus mastitis studies as some of these proteins, expressed in vivo, are likely to account for the success of S. aureus as a pathogen of the ruminant mammary gland.

  10. Loss-of-Function Mutation in the Dioxygenase-Encoding FTO Gene Causes Severe Growth Retardation and Multiple Malformations

    Science.gov (United States)

    Boissel, Sarah; Reish, Orit; Proulx, Karine; Kawagoe-Takaki, Hiroko; Sedgwick, Barbara; Yeo, Giles S.H.; Meyre, David; Golzio, Christelle; Molinari, Florence; Kadhom, Noman; Etchevers, Heather C.; Saudek, Vladimir; Farooqi, I. Sadaf; Froguel, Philippe; Lindahl, Tomas; O'Rahilly, Stephen; Munnich, Arnold; Colleaux, Laurence

    2009-01-01

    FTO is a nuclear protein belonging to the AlkB-related non-haem iron- and 2-oxoglutarate-dependent dioxygenase family. Although polymorphisms within the first intron of the FTO gene have been associated with obesity, the physiological role of FTO remains unknown. Here we show that a R316Q mutation, inactivating FTO enzymatic activity, is responsible for an autosomal-recessive lethal syndrome. Cultured skin fibroblasts from affected subjects showed impaired proliferation and accelerated senescence. These findings indicate that FTO is essential for normal development of the central nervous and cardiovascular systems in human and establish that a mutation in a human member of the AlkB-related dioxygenase family results in a severe polymalformation syndrome. PMID:19559399

  11. OI Issues: Hearing Loss

    Science.gov (United States)

    ... signals normally to the brain. In addition, hearing losses are classified according to the degree of severity: • Mild, • Moderate, • Severe, • Profound. Hearing losses are also classified according to the sound frequency ...

  12. All-Cause and Acute Pancreatitis Health Care Costs in Patients With Severe Hypertriglyceridemia.

    Science.gov (United States)

    Rashid, Nazia; Sharma, Puza P; Scott, Ronald D; Lin, Kathy J; Toth, Peter P

    2017-01-01

    The aim of this study was to assess health care utilization and costs related to acute pancreatitis (AP) in patients with severe hypertriglyceridemia (sHTG) levels. Patients with sHTG levels 1000 mg/dL or higher were identified from January 1, 2007, to June 30, 2013. The first identified incident triglyceride level was labeled as index date. All-cause, AP-related health care visits, and mean total all-cause costs in patients with and without AP were compared during 12 months postindex. A generalized linear model regression was used to compare costs while controlling for patient characteristics and comorbidities. Five thousand five hundred fifty sHTG patients were identified, and 5.4% of these patients developed AP during postindex. Patients with AP had significantly (P < 0.05) more all-cause outpatient visits, hospitalizations, longer length of stays during the hospital visits, and emergency department visits versus patients without AP. Mean (SD) unadjusted all-cause health care costs in the 12 months postindex were $25,343 ($33,139) for patients with AP compared with $15,195 ($24,040) for patients with no AP. The regression showed annual all-cause costs were 49.9% higher (P < 0.01) for patients with AP versus without AP. Patients who developed AP were associated with higher costs; managing patients with sHTG at risk of developing AP may help reduce unnecessary costs.

  13. Fungicides reduce Rhododendron root rot and mortality caused by Phytophthora cinnamomi, but not by P. plurivora

    Science.gov (United States)

    Rhododendron root rot, caused by several Phytophthora species, can cause devastating losses in nursery-grown plants. Most research on chemical control of root rot has focused on Phytophthora cinnamomi. However, it is unknown whether treatments recommended for P. cinnamomi are also effective for othe...

  14. Oxidants and not alkylating agents induce rapid mtDNA loss and mitochondrial dysfunction

    Science.gov (United States)

    Furda, Amy M.; Marrangoni, Adele M.; Lokshin, Anna; Van Houten, Bennett

    2013-01-01

    Mitochondrial DNA (mtDNA) is essential for proper mitochondrial function and encodes 22 tRNAs, 2 rRNAs and 13 polypeptides that make up subunits of complex I, III, IV, in the electron transport chain and complex V, the ATP synthase. Although mitochondrial dysfunction has been implicated in processes such as premature aging, neurodegeneration, and cancer, it has not been shown whether persistent mtDNA damage causes a loss of oxidative phosphorylation. We addressed this question by treating mouse embryonic fibroblasts with either hydrogen peroxide (H2O2) or the alkylating agent methyl methanesulfonate (MMS) and measuring several endpoints, including mtDNA damage and repair rates using QPCR, levels of mitochondrial- and nuclear-encoded proteins using antibody analysis, and a pharmacologic profile of mitochondria using the Seahorse Extracellular Flux Analyzer. We show that a 60 min treatment with H2O2 causes persistent mtDNA lesions, mtDNA loss, decreased levels of a nuclear-encoded mitochondrial subunit, a loss of ATP-linked oxidative phosphorylation and a loss of total reserve capacity. Conversely, a 60 min treatment with 2 mM MMS causes persistent mtDNA lesions but no mtDNA loss, no decrease in levels of a nuclear-encoded mitochondrial subunit, and no mitochondrial dysfunction. These results suggest that persistent mtDNA damage is not sufficient to cause mitochondrial dysfunction. PMID:22766155

  15. Factors influencing to earthquake caused economical losses on urban territories

    Science.gov (United States)

    Nurtaev, B.; Khakimov, S.

    2005-12-01

    Questions of assessment of earthquake economical losses on urban territories of Uzbekistan, taking into account damage forming factors, which are increqasing or reducing economical losses were discussed in the paper. Buildings and facilities vulnerability factors were classified. From total value (equal to 50) were selected most important ones. Factors ranging by level of impact and weight function in loss assessment were ranged. One group of damage forming factors includs seismic hazard assessment, design, construction and maintenance of building and facilities. Other one is formed by city planning characteristics and includes : density of constructions and population, area of soft soils, existence of liquefaction susceptible soils and etc. To all these factors has been given weight functions and interval values by groups. Methodical recomendations for loss asessment taking into account above mentioned factors were developed. It gives possibility to carry out preventive measures for protection of vulnerable territories, to differentiate cost assessment of each region in relation with territory peculiarity and damage value. Using developed method we have ranged cities by risk level. It has allowed to establish ratings of the general vulnerability of urban territories of cities and on their basis to make optimum decisions, oriented to loss mitigation and increase of safety of population. Besides the technique can be used by insurance companies for estimated zoning of territory, development of effective utilization schema of land resources, rational town-planning, an economic estimation of used territory for supply with information of the various works connected to an estimation of seismic hazard. Further improvement of technique of establishment of rating of cities by level of damage from earthquakes will allow to increase quality of construction, rationality of accommodation of buildings, will be an economic stimulator for increasing of seismic resistance of

  16. Two year reduction in sleep apnea symptoms and associated diabetes incidence after weight loss in severe obesity.

    Science.gov (United States)

    Grunstein, Ronald R; Stenlöf, Kaj; Hedner, Jan A; Peltonen, Markku; Karason, Kristjan; Sjöström, Lars

    2007-06-01

    To evaluate the effect of bariatric surgery on sleep apnea symptoms and obesity-associated morbidity in patients with severe obesity. Prospective study. University hospitals and community centers in Sweden. We investigated the influence of weight loss surgery (n=1729) on sleep apnea symptoms and obesity-related morbidity using a conservatively treated group (n=1748) as a control. Baseline BMI in surgical group (42.2+/-4.4 kg/m(2)) and control group (40.1+/-4.6 kg/m(2)) changed -9.7+/-5 kg/m(2) and 0+/-3 kg/m(2), respectively, at 2-year follow-up. In the surgery group, there was a marked improvement in all obstructive sleep apnea (OSA) symptoms compared with the control group (P sleep apnea symptoms at 2 years. Despite adjustment for weight change and baseline central obesity, subjects reporting loss of OSA symptoms had a lower 2-year incidence of diabetes and hypertriglyceridemia. Improvement in OSA in patients losing weight may provide health benefits in addition to weight loss alone.

  17. Economic Estimation of the Losses Caused by Surface Water Pollution Accidents in China From the Perspective of Water Bodies’ Functions

    Science.gov (United States)

    Yao, Hong; You, Zhen; Liu, Bo

    2016-01-01

    The number of surface water pollution accidents (abbreviated as SWPAs) has increased substantially in China in recent years. Estimation of economic losses due to SWPAs has been one of the focuses in China and is mentioned many times in the Environmental Protection Law of China promulgated in 2014. From the perspective of water bodies’ functions, pollution accident damages can be divided into eight types: damage to human health, water supply suspension, fishery, recreational functions, biological diversity, environmental property loss, the accident’s origin and other indirect losses. In the valuation of damage to people’s life, the procedure for compensation of traffic accidents in China was used. The functional replacement cost method was used in economic estimation of the losses due to water supply suspension and loss of water’s recreational functions. Damage to biological diversity was estimated by recovery cost analysis and damage to environmental property losses were calculated using pollutant removal costs. As a case study, using the proposed calculation procedure the economic losses caused by the major Songhuajiang River pollution accident that happened in China in 2005 have been estimated at 2263 billion CNY. The estimated economic losses for real accidents can sometimes be influenced by social and political factors, such as data authenticity and accuracy. Besides, one or more aspects in the method might be overestimated, underrated or even ignored. The proposed procedure may be used by decision makers for the economic estimation of losses in SWPAs. Estimates of the economic losses of pollution accidents could help quantify potential costs associated with increased risk sources along lakes/rivers but more importantly, highlight the value of clean water to society as a whole. PMID:26805869

  18. Economic Estimation of the Losses Caused by Surface Water Pollution Accidents in China From the Perspective of Water Bodies' Functions.

    Science.gov (United States)

    Yao, Hong; You, Zhen; Liu, Bo

    2016-01-22

    The number of surface water pollution accidents (abbreviated as SWPAs) has increased substantially in China in recent years. Estimation of economic losses due to SWPAs has been one of the focuses in China and is mentioned many times in the Environmental Protection Law of China promulgated in 2014. From the perspective of water bodies' functions, pollution accident damages can be divided into eight types: damage to human health, water supply suspension, fishery, recreational functions, biological diversity, environmental property loss, the accident's origin and other indirect losses. In the valuation of damage to people's life, the procedure for compensation of traffic accidents in China was used. The functional replacement cost method was used in economic estimation of the losses due to water supply suspension and loss of water's recreational functions. Damage to biological diversity was estimated by recovery cost analysis and damage to environmental property losses were calculated using pollutant removal costs. As a case study, using the proposed calculation procedure the economic losses caused by the major Songhuajiang River pollution accident that happened in China in 2005 have been estimated at 2263 billion CNY. The estimated economic losses for real accidents can sometimes be influenced by social and political factors, such as data authenticity and accuracy. Besides, one or more aspects in the method might be overestimated, underrated or even ignored. The proposed procedure may be used by decision makers for the economic estimation of losses in SWPAs. Estimates of the economic losses of pollution accidents could help quantify potential costs associated with increased risk sources along lakes/rivers but more importantly, highlight the value of clean water to society as a whole.

  19. Economic Estimation of the Losses Caused by Surface Water Pollution Accidents in China From the Perspective of Water Bodies’ Functions

    Directory of Open Access Journals (Sweden)

    Hong Yao

    2016-01-01

    Full Text Available The number of surface water pollution accidents (abbreviated as SWPAs has increased substantially in China in recent years. Estimation of economic losses due to SWPAs has been one of the focuses in China and is mentioned many times in the Environmental Protection Law of China promulgated in 2014. From the perspective of water bodies’ functions, pollution accident damages can be divided into eight types: damage to human health, water supply suspension, fishery, recreational functions, biological diversity, environmental property loss, the accident’s origin and other indirect losses. In the valuation of damage to people’s life, the procedure for compensation of traffic accidents in China was used. The functional replacement cost method was used in economic estimation of the losses due to water supply suspension and loss of water’s recreational functions. Damage to biological diversity was estimated by recovery cost analysis and damage to environmental property losses were calculated using pollutant removal costs. As a case study, using the proposed calculation procedure the economic losses caused by the major Songhuajiang River pollution accident that happened in China in 2005 have been estimated at 2263 billion CNY. The estimated economic losses for real accidents can sometimes be influenced by social and political factors, such as data authenticity and accuracy. Besides, one or more aspects in the method might be overestimated, underrated or even ignored. The proposed procedure may be used by decision makers for the economic estimation of losses in SWPAs. Estimates of the economic losses of pollution accidents could help quantify potential costs associated with increased risk sources along lakes/rivers but more importantly, highlight the value of clean water to society as a whole.

  20. Impalement of an unusual foreign body on the temporomandibular joint causing severe trismus.

    Science.gov (United States)

    Ito, Ryohei; Kubota, Kosei; Furudate, Ken; Nakagawa, Hiroshi; Kon, Takao; Tamura, Yoshihiro; Kobayashi, Wataru

    2016-12-01

    A penetrating injury by a foreign body is comparatively common in the oral and maxillofacial region. On the other hand, injury to the temporomandibular joint (TMJ) by a foreign object is very rare. The TMJ is an anatomically narrow space surrounded by hard bony processes. An unusual case of trauma with severe trismus caused by a foreign body that impaled the TMJ is reported. A 55-year-old man presented with a 5 × 1-cm laceration to the right cheek caused by a flying object propelled during the use of a lawn mower. The edge of the foreign body had a metallic wire, which became imbedded in the wound. His jaw opening was severely limited. Computed tomography revealed that the foreign body was 3 mm in diameter and was impaled on the articular capsule. The object was successfully removed, and the wound and interior of the TMJ were irrigated. Rehabilitation of mouth opening was started on postoperative day 3. On day 9, mouth opening had improved to 35 mm, and he was discharged. After 1 year, mouth opening was 45 mm with no sign of any TMJ disorders. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Lacrimal punctum occlusion in the treatment of severe keratoconjunctivitis sicca caused by Sjogren syndrome - A uniocular evaluation

    NARCIS (Netherlands)

    Mansour, Khaled; Leonhardt, Carolien J.; Kalk, Wouter W.; Bootsma, Hendrika; Bruin, Klaas J.; Blanksma, Lieuwe J.

    Purpose: A controlled uniocular study to evaluate the short-term efficacy of lacrimal punctum occlusion in the treatment of severe dry eye caused by Sjogren syndrome. Methods: Uniocular punctum occlusion by punctum plug in the upper and lower puncta in 1 eye was performed in 20 patients with severe

  2. Loss-of-function mutations in CAST cause peeling skin, leukonychia, acral punctate keratoses, cheilitis, and knuckle pads.

    Science.gov (United States)

    Lin, Zhimiao; Zhao, Jiahui; Nitoiu, Daniela; Scott, Claire A; Plagnol, Vincent; Smith, Frances J D; Wilson, Neil J; Cole, Christian; Schwartz, Mary E; McLean, W H Irwin; Wang, Huijun; Feng, Cheng; Duo, Lina; Zhou, Eray Yihui; Ren, Yali; Dai, Lanlan; Chen, Yulan; Zhang, Jianguo; Xu, Xun; O'Toole, Edel A; Kelsell, David P; Yang, Yong

    2015-03-05

    Calpastatin is an endogenous specific inhibitor of calpain, a calcium-dependent cysteine protease. Here we show that loss-of-function mutations in calpastatin (CAST) are the genetic causes of an autosomal-recessive condition characterized by generalized peeling skin, leukonychia, acral punctate keratoses, cheilitis, and knuckle pads, which we propose to be given the acronym PLACK syndrome. In affected individuals with PLACK syndrome from three families of different ethnicities, we identified homozygous mutations (c.607dup, c.424A>T, and c.1750delG) in CAST, all of which were predicted to encode truncated proteins (p.Ile203Asnfs∗8, p.Lys142∗, and p.Val584Trpfs∗37). Immunohistochemistry shows that staining of calpastatin is reduced in skin from affected individuals. Transmission electron microscopy revealed widening of intercellular spaces with chromatin condensation and margination in the upper stratum spinosum in lesional skin, suggesting impaired intercellular adhesion as well as keratinocyte apoptosis. A significant increase of apoptotic keratinocytes was also observed in TUNEL assays. In vitro studies utilizing siRNA-mediated CAST knockdown revealed a role for calpastatin in keratinocyte adhesion. In summary, we describe PLACK syndrome, as a clinical entity of defective epidermal adhesion, caused by loss-of-function mutations in CAST. Copyright © 2015 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  3. Hair Loss Following The Topiramate Treatment

    Directory of Open Access Journals (Sweden)

    I Ghafoor

    2017-02-01

    Full Text Available BACKGROUND AND OBJECTIVE: Genetics, hormone profiles and other physiologic factors can cause hair loss. Medication induced hair loss is an occasional side effect of many psychopharmaceuticals. It can reduce medication compliance if not discovered and treated. We present a 18 year old female with migraine headache who developed hair loss after 3 months of receiving topiramate treatment. CASE REPORT: 18 year old female had been suffering from headache visited in psychiatric clinic. She agreed to a treatment with topiramate (50mg per day for Migraine headache. 3 months later, the patient complained of significant hair loss. Topiramate tapered to 25 mg/day and stopped. Hair loss stopped after topiramate withdrawal. Two weeks after reintroduction of topiramate, hair loss developed again. The medication was stopped and hair loss stopped again. CONCLUSION: Topiramate can cause hair loss. Although the condition is not life-threatening, a decrease in medication compliance can cause recurrence of the underlying disease. It is necessary to ask the patient at visits about it.

  4. Mutations of CDKL5 Cause a Severe Neurodevelopmental Disorder with Infantile Spasms and Mental Retardation

    Science.gov (United States)

    Weaving, Linda S.; Christodoulou, John; Williamson, Sarah L.; Friend, Kathie L.; McKenzie, Olivia L. D.; Archer, Hayley; Evans, Julie; Clarke, Angus; Pelka, Gregory J.; Tam, Patrick P. L.; Watson, Catherine; Lahooti, Hooshang; Ellaway, Carolyn J.; Bennetts, Bruce; Leonard, Helen; Gécz, Jozef

    2004-01-01

    Rett syndrome (RTT) is a severe neurodevelopmental disorder caused, in most classic cases, by mutations in the X-linked methyl-CpG-binding protein 2 gene (MECP2). A large degree of phenotypic variation has been observed in patients with RTT, both those with and without MECP2 mutations. We describe a family consisting of a proband with a phenotype that showed considerable overlap with that of RTT, her identical twin sister with autistic disorder and mild-to-moderate intellectual disability, and a brother with profound intellectual disability and seizures. No pathogenic MECP2 mutations were found in this family, and the Xq28 region that contains the MECP2 gene was not shared by the affected siblings. Three other candidate regions were identified by microsatellite mapping, including 10.3 Mb at Xp22.31-pter between Xpter and DXS1135, 19.7 Mb at Xp22.12-p22.11 between DXS1135 and DXS1214, and 16.4 Mb at Xq21.33 between DXS1196 and DXS1191. The ARX and CDKL5 genes, both of which are located within the Xp22 region, were sequenced in the affected family members, and a deletion of nucleotide 183 of the coding sequence (c.183delT) was identified in CDKL5 in the affected family members. In a screen of 44 RTT cases, a single splice-site mutation, IVS13-1G→A, was identified in a girl with a severe phenotype overlapping RTT. In the mouse brain, Cdkl5 expression overlaps—but is not identical to—that of Mecp2, and its expression is unaffected by the loss of Mecp2. These findings confirm CDKL5 as another locus associated with epilepsy and X-linked mental retardation. These results also suggest that mutations in CDKL5 can lead to a clinical phenotype that overlaps RTT. However, it remains to be determined whether CDKL5 mutations are more prevalent in specific clinical subgroups of RTT or in other clinical presentations. PMID:15492925

  5. Ionization Chambers for the LHC Beam Loss Detection

    CERN Document Server

    Assmann, R W; Ferioli, G; Gschwendtner, E; Kain, V

    2003-01-01

    At the Large Hadron Collider (LHC) a beam loss system will be used to prevent and protect superconducting magnets against coil quenches and coil damages. Ionisation chambers will be mounted outside the cryostat to measure the secondary shower particles caused by lost beam particles. Since the stored particle beam intensity is eight orders of magnitude larger than the lowest quench level and the losses should be detected with a relative error of two, the design and the location of the detectors have to be optimised. For that purpose a two-fold simulation was carried out. The longitudinal loss locations of the tertiary halo is investigated by tracking the halo through several magnet elements. These loss distributions are combined with simulations of the particle fluence outside the cryostat, which is induced by lost protons at the vacuum pipe. The base-line ionisation chamber has been tested at the PS Booster in order to determine the detector response at the high end of the dynamic range.

  6. Causes and consequences of the loss of serotonergic presynapses elicited by the consumption of 3,4-methylenedioxymethamphetamine (MDMA, "ecstasy") and its congeners.

    Science.gov (United States)

    Huether, G; Zhou, D; Rüther, E

    1997-01-01

    The massive and prolonged stimulation of serotonin (5-HT)-release and the increased dopaminergic activity are responsible for the acute psychomimetic and psychostimulatory effects of 3,4-methylenedioxy-methamphetamine (MDMA, "ecstasy") and its congeners. In vulnerable subjects, at high doses or repeated use, and under certain unfavorable conditions (crowding, high ambient temperature), severe, in some cases fatal, averse systemic reactions (hyperthermia, serotonin-syndrome) may occur during the first few hours. Animal experiments revealed the existence of similar differences in vulnerability and similar dose- and context-related influences on a similar sequence of acute responses. The severity of these acute systemic responses is closely related to the severity of the long-term damage to 5-HT axon terminals caused by the administration of substituted amphetamines. Attempts to identify the mechanisms involved in this selective degeneration of 5-HT presynapses brought to light a multitude of different factors and conditions which either attenuate or potentiate the loss of 5-HT terminals caused by MDMA and related amphetamine derivatives. These puzzling observations suggest that the degeneration of 5-HT presynapses represents only the final step in a sequence of events which compromise the ability of 5-HT terminals to maintain their functional and structural integrity. Substituted amphetamines selectively tax energy metabolism in 5-HT presynapses through their ability to exchange with 5-HT and to dissipate transmembrane ion gradients. The active carrier systems in the vesicular and presynaptic membrane operate at a permanently activated state. The resulting energy deficit can no longer adequately restored by the 5-HT presynapses when their availability of substrates for ATP production is additionally reduced by the hyperthermic and other energy consuming reactions which are elicited by the systemic administration of substituted amphetamines. The exhaustion of energy

  7. Increasing severity of damage caused by floods in the Spanish Mediterranean coast (1960-2014), climate change or vulnerability?

    Science.gov (United States)

    Perez, Alfredo; Gil, Salvador; Lopez, Francisco; Barriendos, Mariano

    2016-04-01

    In recent decades, there has been an increase in physical and economic losses (WMO, CRED and UCL, 2014) that raises serious concerns in society. Climate change projections may explain the rise in flood losses; however, these shouldn't be considered yet (Bouwer, 2011). According to IPCC (2014), there is low confidence in anthropogenic climate change affecting the frequency and magnitude of fluvial floods on a global scale. In other words, this increase in flood events is not completely related to the higher frequency of heavy rainfall. To illustrate the aforementioned, a spatial example can be seen in the study area. In the Spanish Mediterranean coast, we see an increase in economic losses within the last 50 years due to flood events (Gil et al., 2014). It seems that the socio-economic growth and the rise of housing construction (Gaja, 2008) have led to an increase in vulnerability and exposure which are mainly responsible for those losses and the increase in severity of flood events (Pérez et al., 2015). Furthermore, this situation will probably become more precarious if some climate forecasts are met [IPCC, 2014; AEMET, 2015], and if the economic model fails to adopt efficient adaptive measures. Therefore, it is interesting to focus attention on social factors either within the present or future scenario in order to minimise the potential consequences and improve the adaptation. The main objective of this work focuses on the study of the evolution of the severity of the floods in the Spanish Mediterranean coast for the period (1960-2015). To do that, a statistical analysis of the data base [Gil et al., 2014; extended to the entire Spanish Mediterranean coast (MEDIFLOOD)] and a multiscale mapping (local, provincial and regional level) of the frequency of these events will take place in order to make comparisons and show spatiotemporal patterns according to the severity events evolution. Preliminary results show some interesting statistically significant

  8. Retrograde nail for tibiotalocalcaneal arthrodesis as a limb salvage procedure for open distal tibia and talus fractures with severe bone loss.

    Science.gov (United States)

    Ochman, Sabine; Evers, Julia; Raschke, Michael J; Vordemvenne, Thomas

    2012-01-01

    The treatment of complex fractures of the distal tibia, ankle, and talus with soft tissue damage, bone loss, and nonreconstructable joints for which the optimal timing for reduction and fixation has been missed is challenging. In such cases primary arthrodesis might be a treatment option. We report a series of multi-injured patients with severe soft tissue damage and bone loss, who were treated with a retrograde tibiotalocalcaneal arthrodesis nail as a minimally invasive treatment option for limb salvage. After a median follow-up of 5.4 years, all patients returned to their former profession. The ankle and bone fusion was complete, with moderate functional results and quality of life. Calcaneotibial arthrodesis using a retrograde nail is a good treatment option for nonreconstructable fractures of the ankle joint with severe bone loss and poor soft tissue quality in selected patients with multiple injuries, in particular, those involving both lower extremities, as a salvage procedure. Copyright © 2012 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Loss of Melanopsin-Expressing Retinal Ganglion Cells in Severely Staged Glaucoma Patients

    DEFF Research Database (Denmark)

    Obara, Elisabeth Anne; Hannibal, Jens; Heegaard, Steffen

    2016-01-01

    Purpose: Multiple studies have shown overwhelming evidence supporting the impairment of melanopsin function due to glaucoma. However, few studies have been carried out in humans analyzing the histology of melanopsin-expressing retinal ganglion cells (mRGCs) in retinas with glaucoma. The aim...... of this study was to analyze the pattern of expression of mRGCs relative to RGCs in the normal retina and retinas harboring varying stages of glaucoma. Methods: Paraffin-embedded human donor eyes with glaucoma (n = 11) and age-matched controls (n = 10) were obtained from Department of Pathology at Rigshospital...... difference was observed in mRGC expression in the normal retinas and mild-staged retinas with glaucoma; the densities of mRGCs were 3.08 ± 0.47 and 3.00 ± 0.13 cell counts/mm2, respectively. However, the severely staged retinas with glaucoma showed a significant loss in mRGCs density, 1.09 ± 0.35 cell counts...

  10. Hair loss in women.

    Science.gov (United States)

    Harfmann, Katya L; Bechtel, Mark A

    2015-03-01

    Hair loss is a common cause of morbidity for many women. As a key member of the woman's health care team, the obstetrician/gynecologist may be the first person to evaluate the complaint of hair loss. Common types of nonscarring hair loss, including female pattern hair loss and telogen effluvium, may be diagnosed and managed by the obstetrician/gynecologist. A systematic approach to diagnosis and management of these common forms of hair loss is presented.

  11. Human SOD1 ALS Mutations in a Drosophila Knock-In Model Cause Severe Phenotypes and Reveal Dosage-Sensitive Gain- and Loss-of-Function Components.

    Science.gov (United States)

    Şahin, Aslı; Held, Aaron; Bredvik, Kirsten; Major, Paxton; Achilli, Toni-Marie; Kerson, Abigail G; Wharton, Kristi; Stilwell, Geoff; Reenan, Robert

    2017-02-01

    Amyotrophic Lateral Sclerosis (ALS) is the most common adult-onset motor neuron disease and familial forms can be caused by numerous dominant mutations of the copper-zinc superoxide dismutase 1 (SOD1) gene. Substantial efforts have been invested in studying SOD1-ALS transgenic animal models; yet, the molecular mechanisms by which ALS-mutant SOD1 protein acquires toxicity are not well understood. ALS-like phenotypes in animal models are highly dependent on transgene dosage. Thus, issues of whether the ALS-like phenotypes of these models stem from overexpression of mutant alleles or from aspects of the SOD1 mutation itself are not easily deconvolved. To address concerns about levels of mutant SOD1 in disease pathogenesis, we have genetically engineered four human ALS-causing SOD1 point mutations (G37R, H48R, H71Y, and G85R) into the endogenous locus of Drosophila SOD1 (dsod) via ends-out homologous recombination and analyzed the resulting molecular, biochemical, and behavioral phenotypes. Contrary to previous transgenic models, we have recapitulated ALS-like phenotypes without overexpression of the mutant protein. Drosophila carrying homozygous mutations rendering SOD1 protein enzymatically inactive (G85R, H48R, and H71Y) exhibited neurodegeneration, locomotor deficits, and shortened life span. The mutation retaining enzymatic activity (G37R) was phenotypically indistinguishable from controls. While the observed mutant dsod phenotypes were recessive, a gain-of-function component was uncovered through dosage studies and comparisons with age-matched dsod null animals, which failed to show severe locomotor defects or nerve degeneration. We conclude that the Drosophila knock-in model captures important aspects of human SOD1-based ALS and provides a powerful and useful tool for further genetic studies. Copyright © 2017 by the Genetics Society of America.

  12. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

    Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl

    2013-01-01

    Carbon monoxide poisoning is one of the rare causes of hearing loss which may cause reversible or irreversible, unilateral or bilateral hearing loss after acute or chronic exposure. In this report, we present a case of bilateral sensorineural hearing loss in a secondary smelting workshop worker a...

  13. Severe pain as a possible cause of dropped head syndrome that was attenuated after amputation of an ischemic lower limb.

    Science.gov (United States)

    Maki, Satoshi; Koda, Masao; Furuya, Takeo; Takahashi, Kazuhisa; Yamazaki, Masashi

    2016-03-02

    Dropped head syndrome (DHS) is defined as weakness of the neck extensor muscles causing a correctable chin-on-the-chest deformity. Here we report the case of a patient with severe pain from lower leg ischemia showing DHS whose symptoms were attenuated by pain relief after amputation of the severely ischemic lower leg. To our knowledge this is the first report indicating that severe pain can cause DHS. A 64-year-old Asian woman was referred to our department with a 1-month history of DHS. She also suffered from severe right foot pain because of limb ischemia. She began to complain of DHS as her gangrenous foot pain worsened. She had neck pain and difficulty with forward gaze. We found no clinical or laboratory findings of neuromuscular disorder or isolated neck extensor myopathy. We amputated her leg below the knee because of progressive foot gangrene. Her severe foot pain resolved after the surgery and her DHS was attenuated. Severe pain can cause DHS. If a patient with DHS has severe pain in another part of the body, we recommend considering aggressive pain relief as a treatment option.

  14. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease.

    Science.gov (United States)

    Oji, Vinzenz; Eckl, Katja-Martina; Aufenvenne, Karin; Nätebus, Marc; Tarinski, Tatjana; Ackermann, Katharina; Seller, Natalia; Metze, Dieter; Nürnberg, Gudrun; Fölster-Holst, Regina; Schäfer-Korting, Monika; Hausser, Ingrid; Traupe, Heiko; Hennies, Hans Christian

    2010-08-13

    Generalized peeling skin disease is an autosomal-recessive ichthyosiform erythroderma characterized by lifelong patchy peeling of the skin. After genome-wide linkage analysis, we have identified a homozygous nonsense mutation in CDSN in a large consanguineous family with generalized peeling skin, pruritus, and food allergies, which leads to a complete loss of corneodesmosin. In contrast to hypotrichosis simplex, which can be associated with specific dominant CDSN mutations, peeling skin disease is characterized by a complete loss of CDSN expression. The skin phenotype is consistent with a recent murine Cdsn knockout model. Using three-dimensional human skin models, we demonstrate that lack of corneodesmosin causes an epidermal barrier defect supposed to account for the predisposition to atopic diseases, and we confirm the role of corneodesmosin as a decisive epidermal adhesion molecule. Therefore, peeling skin disease will represent a new model disorder for atopic diseases, similarly to Netherton syndrome and ichthyosis vulgaris in the recent past.

  15. Folliculin mutations are not associated with severe COPD

    Directory of Open Access Journals (Sweden)

    Litonjua Augusto A

    2008-12-01

    Full Text Available Abstract Background Rare loss-of-function folliculin (FLCN mutations are the genetic cause of Birt-Hogg-Dubé syndrome, a monogenic disorder characterized by spontaneous pneumothorax, fibrofolliculomas, and kidney tumors. Loss-of-function folliculin mutations have also been described in pedigrees with familial spontaneous pneumothorax. Because the majority of patients with folliculin mutations have radiographic evidence of pulmonary cysts, folliculin has been hypothesized to contribute to the development of emphysema. To determine whether folliculin sequence variants are risk factors for severe COPD, we genotyped seven previously reported Birt-Hogg-Dubé or familial spontaneous pneumothorax associated folliculin mutations in 152 severe COPD probands participating in the Boston Early-Onset COPD Study. We performed bidirectional resequencing of all 14 folliculin exons in a subset of 41 probands and subsequently genotyped four identified variants in an independent sample of345 COPD subjects from the National Emphysema Treatment Trial (cases and 420 male smokers with normal lung function from the Normative Aging Study (controls. Results None of the seven previously reported Birt-Hogg-Dubé or familial spontaneous pneumothorax mutations were observed in the 152 severe, early-onset COPD probands. Exon resequencing identified 31 variants, including two non-synonymous polymorphisms and two common non-coding polymorphisms. No significant association was observed for any of these four variants with presence of COPD or emphysema-related phenotypes. Conclusion Genetic variation in folliculin does not appear to be a major risk factor for severe COPD. These data suggest that familial spontaneous pneumothorax and COPD have distinct genetic causes, despite some overlap in radiographic characteristics.

  16. High dietary sodium chloride causes further protein loss during head-down tilt bed rest (HDBR)

    Science.gov (United States)

    Buehlmeier, Judith; Frings-Meuthen, Petra; Baecker, Natalie; Stehle, Peter; Heer, Martina

    Human spaceflight is associated with a loss of body protein most likely caused by muscle degradation. Additionally astronauts tend towards a high dietary intake of sodium chloride (NaCl), which has recently been shown to induce low grade metabolic acidosis (Frings-Meuthen et al. JBMR, Epub 2007). In several patterns, e.g. chronical renal failure, metabolic acidosis is associated with protein catabolism. We therefore hypothesized that high dietary intake of NaCl enforces protein losses in HDBR, a model for physiological changes in microgravity (µG). Eight healthy male subjects (mean age 26.25 ± 3.5; mean body weight: 78.5 ± 4.1 kg) participated in a 14-day bed rest study in the metabolic ward of the DLR - Institute of Aerospace Medicine, Cologne, Germany. The study was carried out in a cross over design, consisting of two phases, each lasting 22 days (5 days adaptation, 14 days 6° HDBR and 3 days recovery). Both study phases were identical with respect to environmental conditions and study protocol. Subjects received an individually tailored, weight-maintaining diet containing 1.3 g protein/kg/day. The diet was identical in both study phases with the exception of NaClintake: Every subject received a low NaCl diet (0.7 mmol/kg/day) in one phase and a high NaCl diet (7.7 mmol/kg/day) in another one. Blood gas for analysis of acid-base balance was implemented at days 4 and 5 of adaptation, days 2, 5, 7, 10, 12, 14 of HDBR and days 2, 3 of recovery. Continuous urine collection started on the first day in the metabolic ward to analyze nitrogen excretion. Nitrogen balance was calculated from the difference between protein intake and urinary nitrogen excretion, determined by use of chemiluminescence (Grimble et al. JPEN, 1988). Plasma pH did not change significantly (p=0.285), but plasma bicarbonate and base excess decreased (p=0.0175; p=0.0093) with high NaCl intake in HDBR compared to the low NaCl diet. Nitrogen balance in HDBR was negative, as expected in

  17. Loss-of-Function FANCL Mutations Associate with Severe Fanconi Anemia Overlapping the VACTERL Association.

    Science.gov (United States)

    Vetro, Annalisa; Iascone, Maria; Limongelli, Ivan; Ameziane, Najim; Gana, Simone; Della Mina, Erika; Giussani, Ursula; Ciccone, Roberto; Forlino, Antonella; Pezzoli, Laura; Rooimans, Martin A; van Essen, Antoni J; Messa, Jole; Rizzuti, Tommaso; Bianchi, Paolo; Dorsman, Josephine; de Winter, Johan P; Lalatta, Faustina; Zuffardi, Orsetta

    2015-05-01

    The diagnosis of VACTERL syndrome can be elusive, especially in the prenatal life, due to the presence of malformations that overlap those present in other genetic conditions, including the Fanconi anemia (FA). We report on three VACTERL cases within two families, where the two who arrived to be born died shortly after birth due to severe organs' malformations. The suspicion of VACTERL association was based on prenatal ultrasound assessment and postnatal features. Subsequent chromosome breakage analysis suggested the diagnosis of FA. Finally, by next-generation sequencing based on the analysis of the exome in one family and of a panel of Fanconi genes in the second one, we identified novel FANCL truncating mutations in both families. We used ectopic expression of wild-type FANCL to functionally correct the cellular FA phenotype for both mutations. Our study emphasizes that the diagnosis of FA should be considered when VACTERL association is suspected. Furthermore, we show that loss-of-function mutations in FANCL result in a severe clinical phenotype characterized by early postnatal death. © 2015 WILEY PERIODICALS, INC.

  18. The willingness to pay of parties to traffic accidents for loss of productivity and consolation compensation.

    Science.gov (United States)

    Jou, Rong-Chang; Chen, Tzu-Ying

    2015-12-01

    In this study, willingness to pay (WTP) for loss of productivity and consolation compensation by parties to traffic accidents is investigated using the Tobit model. In addition, WTP is compared to compensation determined by Taiwanese courts. The modelling results showed that variables such as education, average individual monthly income, traffic accident history, past experience of severe traffic accident injuries, the number of working days lost due to a traffic accident, past experience of accepting compensation for traffic accident-caused productivity loss and past experience of accepting consolation compensation caused by traffic accidents have a positive impact on WTP. In addition, average WTP for these two accident costs were obtained. We found that parties to traffic accidents were willing to pay more than 90% of the compensation determined by the court in the scenario of minor and moderate injuries. Parties were willing to pay approximately 80% of the compensation determined by the court for severe injuries, disability and fatality. Therefore, related agencies can use our study findings as the basis for determining the compensation that parties should pay for productivity losses caused by traffic accidents of different types. Copyright © 2015 Elsevier Ltd. All rights reserved.

  19. Two α1-Globin Gene Point Mutations Causing Severe Hb H Disease.

    Science.gov (United States)

    Jiang, Hua; Huang, Lv-Yin; Zhen, Li; Jiang, Fan; Li, Dong-Zhi

    Hb H disease is generally a moderate form of α-thalassemia (α-thal) that rarely requires regular blood transfusions. In this study, two Chinese families with members carrying transfusion-dependent Hb H disease were investigated for rare mutations on the α-globin genes (HBA1, HBA2). In one family, Hb Zürich-Albisrieden [α59(E8)Gly→Arg; HBA1: c.178G>C] in combination with the Southeast Asian (- - SEA ) deletion was the defect responsible for the severe phenotype. In another family, a novel hemoglobin (Hb) variant named Hb Sichuan (HBA1: c.393_394insT), causes α-thal and a severe phenotype when associated with the - - SEA deletion. As these two HBA1 mutations can present as continuous blood transfusion-dependent α-thal, it is important to take this point into account for detecting the carriers, especially in couples in which one partner is already a known α 0 -thal carrier.

  20. Cigarette smoke inhalation increases the alveolar bone loss caused by primary occlusal trauma in a rat model.

    Science.gov (United States)

    Campos, M L G; Corrêa, M G; Júnior, F H N; Casati, M Z; Sallum, E A; Sallum, A W

    2014-04-01

    Occlusal trauma (OT) and smoking are both factors that alter alveolar bone metabolism and therefore could synergistically act on alveolar bone loss. The aim of this experimental study was to evaluate the influence of short-term cigarette smoke inhalation (CSI) on inter-radicular alveolar bone loss promoted by primary OT in a rat model. Forty-eight animals were randomly assigned to one of three groups based on treatment type: OT + CSI (n = 16), animals were exposed to CSI three times per day, for 8 min per exposure, and they concomitantly received unilateral vertical augmentation creating an occlusal interference inducing experimental OT; OT (n = 16), animals received only unilateral vertical augmentation; negative control (NC; n = 16), animals maintained for equal periods to achieve periodontal baseline values of periodontal ligament dimension. Each group was divided into two subgroups (n = 8) based on treatment length: 7 or 14 d. After 7 d, the OT + CSI group exhibited significantly higher bone loss compared to the NC group (p = 0.0022). After 14 d, the OT (p < 0.0001) and OT + CSI (p < 0.0001) groups presented significantly higher bone loss compared to the NC group, and OT + CSI resulted in significantly higher bone loss than OT alone (p = 0.0241). The number of tartrate-resistant acid phosphatase-positive cells on the linear surface of the bone crest after 7 d was significantly higher in the OT + CSI group as compared to the NC and OT groups (p < 0.0001 and p = 0.0045, respectively) and remained significantly higher in the OT + CSI group after 14 d, compared to the OT group (p < 0.0001). Short-term CSI increases early bone loss in association with OT after 7 d, and this worsens in severity after 14 d of exposure. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Use of low-dose UV-C irradiation to control powdery mildew caused by Podosphaera aphanis on strawberry plants

    Science.gov (United States)

    Powdery mildew of strawberry plants, caused by Podoshaera aphanis, can cause severe losses by reducing fruit yield, quality and predisposing fruit to other diseases. Fungicides have been routinely used to control this disease. However, limitations mainly related to their effectiveness, re-entry pe...

  2. Neutrophil Extracellular Trap-Related Extracellular Histones Cause Vascular Necrosis in Severe GN.

    Science.gov (United States)

    Kumar, Santhosh V R; Kulkarni, Onkar P; Mulay, Shrikant R; Darisipudi, Murthy N; Romoli, Simone; Thomasova, Dana; Scherbaum, Christina R; Hohenstein, Bernd; Hugo, Christian; Müller, Susanna; Liapis, Helen; Anders, Hans-Joachim

    2015-10-01

    Severe GN involves local neutrophil extracellular trap (NET) formation. We hypothesized a local cytotoxic effect of NET-related histone release in necrotizing GN. In vitro, histones from calf thymus or histones released by neutrophils undergoing NETosis killed glomerular endothelial cells, podocytes, and parietal epithelial cells in a dose-dependent manner. Histone-neutralizing agents such as antihistone IgG, activated protein C, or heparin prevented this effect. Histone toxicity on glomeruli ex vivo was Toll-like receptor 2/4 dependent, and lack of TLR2/4 attenuated histone-induced renal thrombotic microangiopathy and glomerular necrosis in mice. Anti-glomerular basement membrane GN involved NET formation and vascular necrosis, whereas blocking NET formation by peptidylarginine inhibition or preemptive anti-histone IgG injection significantly reduced all aspects of GN (i.e., vascular necrosis, podocyte loss, albuminuria, cytokine induction, recruitment or activation of glomerular leukocytes, and glomerular crescent formation). To evaluate histones as a therapeutic target, mice with established GN were treated with three different histone-neutralizing agents. Anti-histone IgG, recombinant activated protein C, and heparin were equally effective in abrogating severe GN, whereas combination therapy had no additive effects. Together, these results indicate that NET-related histone release during GN elicits cytotoxic and immunostimulatory effects. Furthermore, neutralizing extracellular histones is still therapeutic when initiated in established GN. Copyright © 2015 by the American Society of Nephrology.

  3. Core loss during a severe accident (COLOSS)

    International Nuclear Information System (INIS)

    Adroguer, B.; Bertrand, F.; Chatelard, P.; Cocuaud, N.; Van Dorsselaere, J.P.; Bellenfant, L.; Knocke, D.; Bottomley, D.; Vrtilkova, V.; Belovsky, L.; Mueller, K.; Hering, W.; Homann, C.; Krauss, W.; Miassoedov, A.; Schanz, G.; Steinbrueck, M.; Stuckert, J.; Hozer, Z.; Bandini, G.; Birchley, J.; Berlepsch, T. von; Kleinhietpass, I.; Buck, M.; Benitez, J.A.F.; Virtanen, E.; Marguet, S.; Azarian, G.; Caillaux, A.; Plank, H.; Boldyrev, A.; Veshchunov, M.; Kobzar, V.; Zvonarev, Y.; Goryachev, A.

    2005-01-01

    The COLOSS project was a 3-year shared-cost action, which started in February 2000. The work-programme performed by 19 partners was shaped around complementary activities aimed at improving severe accident codes. Unresolved risk-relevant issues regarding H 2 production, melt generation and the source term were studied through a large number of experiments such as (a) dissolution of fresh and high burn-up UO 2 and MOX by molten Zircaloy (b) simultaneous dissolution of UO 2 and ZrO 2 (c) oxidation of U-O-Zr mixtures (d) degradation-oxidation of B 4 C control rods. Corresponding models were developed and implemented in severe accident computer codes. Upgraded codes were then used to apply results in plant calculations and evaluate their consequences on key severe accident sequences in different plants involving B 4 C control rods and in the TMI-2 accident. Significant results have been produced from separate-effects, semi-global and large-scale tests on COLOSS topics enabling the development and validation of models and the improvement of some severe accident codes. Breakthroughs were achieved on some issues for which more data are needed for consolidation of the modelling in particular on burn-up effects on UO 2 and MOX dissolution and oxidation of U-O-Zr and B 4 C-metal mixtures. There was experimental evidence that the oxidation of these mixtures can contribute significantly to the large H 2 production observed during the reflooding of degraded cores under severe accident conditions. The plant calculation activity enabled (a) the assessment of codes to calculate core degradation with the identification of main uncertainties and needs for short-term developments and (b) the identification of safety implications of new results. Main results and recommendations for future R and D activities are summarized in this paper

  4. Dental erosion caused by gastroesophageal reflux disease: a case report.

    Science.gov (United States)

    Cengiz, Seda; Cengiz, M Inanç; Saraç, Y Sinasi

    2009-07-22

    Chronic regurgitation of gastric acids in patients with gastroesophageal reflux disease may cause dental erosion, which can lead in combination with attrition or bruxism to extensive loss of coronal tooth tissue. This clinical report describes treatment of severe tooth wear of a gastroesophageal reflux disease patient who is 54-year-old Turkish male patient. After his medical treatment, severe tooth wear, bruxism and decreased vertical dimensions were determined. The vertical dimension was re-established and maxillary and mandibular anterior and posterior teeth were prepared for metal-ceramic restorations. Metal-ceramic fixed partial dentures were fabricated as full mouth restorations for both maxillary and mandibular arches because of splinting all teeth. And then maxillary stabilization splint was fabricated for his bruxism history. Significant loss of coronal tooth structure must taken into consideration. Gastroesophageal reflux disease by itself or in combination with attrition, abrasion or bruxism may be responsible for the loss. An extensive diagnostic evaluation is essential for the medical and dental effects of the problem.

  5. Coping with Memory Loss

    Science.gov (United States)

    ... Consumers Home For Consumers Consumer Updates Coping With Memory Loss Share Tweet Linkedin Pin it More sharing ... be evaluated by a health professional. What Causes Memory Loss? Anything that affects cognition—the process of ...

  6. Abutment tooth loss in patients with overdentures.

    Science.gov (United States)

    Ettinger, Ronald L; Qian, Fang

    2004-06-01

    Since the 1960s, the use of natural teeth as overdenture abutments has become part of accepted clinical practice. Several longitudinal studies have been conducted, but tooth loss has not been reported to be a significant problem. The aim of this study was to identify the incidence and causes of tooth loss in a prospective cohort study of subjects wearing overdentures. The study, conducted between 1973 and 1994, evaluated 273 subjects (62.3 percent male) with a mean age of 59.6 years. Of the 273 subjects with 666 abutments, 74 lost 133 abutments. The most common cause of tooth loss was periodontal disease (29.3 percent) followed by periapical lesions (18.8 percent) and caries (16.5 percent). Through logistic regression, the authors found that subjects who lost teeth were more likely to have medical problems that could cause soft-tissue lesions of the oral mucosa, were less likely to use fluoride daily and were less likely to return for yearly recall visits. The authors found 22 vertical fractures in 17 subjects. Chi2 analysis revealed that overdenture teeth in the maxillary arch that were opposed by natural teeth were more likely to experience vertical fractures. In a study that followed up some patients for as long as 22 years, the rate of tooth loss was 20.0 percent. Many of these failures could have been prevented if patients had practiced better oral hygiene. The findings suggest that if a dentist recommends overdenture therapy, the patient needs to be examined regularly to reduce the risk of experiencing caries and periodontal disease. Also, if the abutments are in the maxilla and are opposed by natural teeth, the dentist should consider using thimble crowns to reduce the risk of vertical fractures.

  7. Pathophysiological mechanisms of severe anaemia in Malawian children.

    Directory of Open Access Journals (Sweden)

    Michaël Boele van Hensbroek

    2010-09-01

    Full Text Available Severe anaemia is a major cause of morbidity and mortality in African children. The aetiology is multi-factorial, but interventions have often targeted only one or a few causal factors, with limited success.We assessed the contribution of different pathophysiological mechanisms (red cell production failure [RCPF], haemolysis and blood loss to severe anaemia in Malawian children in whom etiological factors have been described previously. More complex associations between etiological factors and the mechanisms were explored using structural equation modelling. In 235 children with severe anaemia (haemoglobin<3.2 mMol/L [5.0 g/dl] studied, RCPF, haemolysis and blood loss were found in 48.1%, 21.7% and 6.9%, respectively. The RCPF figure increased to 86% when a less stringent definition of RCPF was applied. RCPF was the most common mechanism in each of the major etiological subgroups (39.7-59.7%. Multiple aetiologies were common in children with severe anaemia. In the final model, nutritional and infectious factors, including malaria, were directly or indirectly associated with RCPF, but not with haemolysis.RCPF was the most common pathway leading to severe anaemia, from a variety of etiological factors, often found in combination. Unlike haemolysis or blood loss, RCPF is a defect that is likely to persist to a significant degree unless all of its contributing aetiologies are corrected. This provides a further explanation for the limited success of the single factor interventions that have commonly been applied to the prevention or treatment of severe anaemia. Our findings underline the need for a package of measures directed against all of the local aetiologies of this often fatal paediatric syndrome.

  8. A SEVERE CASE OF ACUTE NECROTIC PANCREATITIS CAUSED BY DUODENAL CYST DUPLICATION

    Directory of Open Access Journals (Sweden)

    Gabriel Constantinescu

    2018-03-01

    Full Text Available Introduction. Duodenal duplication cyst (DDC is a rare congenital malformation that appears in the embryonic development of the digestive tract. It is a benign condition usually diagnosed in infancy and early childhood, being a rare and difficult diagnosis in adult population. DDC is a recognized cause of duodenal obstruction, acute pancreatitis, obstructive jaundice and even digestive hemorrhage. Case presentation. We report the case of a young adult male with abdominal pain history, who presents with recurrent episodes of acute severe necrotic pancreatitis. The abdominal computed tomography scan revealed a Balthazar C necrotic pancreatitis with partial thrombosis of the splenic vein and a cystic mass in the second part of the duodenum. The endoscopic ultrasonography (EUS established that the duodenal cystic lesion came from the second layer, meaning the submucosa. We performed endoscopic cystotomy with complete evacuation of the fluid content into the duodenum, with favorable clinical outcome. Conclusions. The particularity of the case is represented by the low incidence of this pathology and the rare form of presentation, meaning acute pancreatitis probably from pancreatic ductular hypertension caused by the DDC.

  9. Oil sands mining and reclamation cause massive loss of peatland and stored carbon

    Science.gov (United States)

    Rooney, Rebecca C.; Bayley, Suzanne E.; Schindler, David W.

    2012-01-01

    We quantified the wholesale transformation of the boreal landscape by open-pit oil sands mining in Alberta, Canada to evaluate its effect on carbon storage and sequestration. Contrary to claims made in the media, peatland destroyed by open-pit mining will not be restored. Current plans dictate its replacement with upland forest and tailings storage lakes, amounting to the destruction of over 29,500 ha of peatland habitat. Landscape changes caused by currently approved mines will release between 11.4 and 47.3 million metric tons of stored carbon and will reduce carbon sequestration potential by 5,734–7,241 metric tons C/y. These losses have not previously been quantified, and should be included with the already high estimates of carbon emissions from oil sands mining and bitumen upgrading. A fair evaluation of the costs and benefits of oil sands mining requires a rigorous assessment of impacts on natural capital and ecosystem services. PMID:22411786

  10. Radiotherapy on the neck nodes predicts severe weight loss in patients with early stage laryngeal cancer

    International Nuclear Information System (INIS)

    Langius, Jacqueline A.E.; Doornaert, Patricia; Spreeuwenberg, Marieke D.; Langendijk, Johannes A.; Leemans, C. Rene; Schueren, Marian A.E. van Bokhorst-de van der

    2010-01-01

    Background and purpose: Although patients with early stage (T1/T2) laryngeal cancer (LC) are thought to have a low incidence of malnutrition, severe weight loss is observed in a subgroup of these patients during radiotherapy (RT). The objective of this study was to evaluate weight loss and nutrition-related symptoms in patients with T1/T2 LC during RT and to select predictive factors for early identification of malnourished patients. Methods: Of all patients with T1/T2 LC, who received primary RT between 1999 and 2007, the following characteristics were recorded: sex, age, TNM classification, tumour location, radiation schedule, performance status, quality of life, weight loss, and nutrition-related symptoms. The association between baseline characteristics and malnutrition (>5% weight loss during RT) was investigated by Cox regression analysis. Results: The study population consisted of 238 patients. During RT, 44% of patients developed malnutrition. Tumour location, TNM classification, RT on the neck nodes, RT dose, nausea/vomiting, pain, swallowing, senses problems, trouble with social eating, dry mouth and the use of painkillers were all significantly associated with malnutrition. In the multivariate analysis, RTs on both the neck nodes (HR 4.16, 95% CI 2.62-6.60) and dry mouth (HR 1.72, 95% CI 1.14-2.60) remained predictive. Nevertheless, RT on the neck nodes alone resulted in the best predictive model for malnutrition scores. Conclusions: Patients with early stage laryngeal cancer are at risk of malnutrition during radiotherapy. Radiotherapy on the neck nodes is the best predictor of malnutrition during radiotherapy. Therefore, we suggest to offer nutritional counselling to all the patients who receive nodal irradiation.

  11. Frequent complications and severe bone loss associated with the repiphysis expandable distal femoral prosthesis.

    Science.gov (United States)

    Cipriano, Cara A; Gruzinova, Irina S; Frank, Rachel M; Gitelis, Steven; Virkus, Walter W

    2015-03-01

    The treatment of choice for distal femur malignancies in skeletally immature patients remains controversial. An expandable endoprosthesis device (Repiphysis Limb Salvage System; Wright Medical Technology, Arlington, TN, USA) allows for limb preservation and noninvasive lengthening but has been associated with significant complications; however, the extent and implications of bone loss associated with this implant have not been reported. Our goals were to report (1) the 2-year minimum clinical outcomes after placement of the Repiphysis expandable prosthesis for pediatric distal femur malignancies; (2) the complications associated with this prosthesis; (3) the failure rate of this prosthesis; and (4) the revision alternatives available for salvage procedures. Between 2002 and 2010, one surgeon (SG) treated all skeletally immature patients (mean age, 10.1 years; range, 4.7-13.6 years) with distal femoral osteosarcoma using a Repiphysis expandable prosthesis. Of the 12 patients who met these criteria, two were excluded for death from disease before 2 years, and mean followup for the remaining 10 was 72 months (range, 26-119 months). Medical records were retrospectively reviewed for complications and clinical outcomes, as assessed by the Musculoskeletal Tumor Society (MSTS) scoring system. Radiographs at final followup were reviewed for bone loss and analyzed by the two senior authors (SG, WWV) to determine reconstruction options available for future revisions. MSTS scores averaged 67%, and we observed 37 implant-related complications requiring a total of 15 reoperations. Six patients underwent implant revisions with aseptic loosening being the predominant mode of failure; ultimately, four of these were converted to adult modular oncology prostheses, and two underwent total femoral replacements. Bone loss in this series was severe in terms of femoral length, cortical thinning, and metadiaphyseal compromise, and most patients will not have sufficient bone stock to permit

  12. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability

    OpenAIRE

    Houge, G.; Rasmussen, I.H.; Hovland, R.

    2011-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the pos...

  13. World offshore energy loss statistics

    International Nuclear Information System (INIS)

    Kaiser, Mark J.

    2007-01-01

    Offshore operations present a unique set of environmental conditions and adverse exposure not observed in a land environment taking place in a confined space in a hostile environment under the constant danger of catastrophe and loss. It is possible to engineer some risks to a very low threshold of probability, but losses and unforeseen events can never be entirely eliminated because of cost considerations, the human factor, and environmental uncertainty. Risk events occur infrequently but have the potential of generating large losses, as evident by the 2005 hurricane season in the Gulf of Mexico, which was the most destructive and costliest natural disaster in the history of offshore production. The purpose of this paper is to provide a statistical assessment of energy losses in offshore basins using the Willis Energy Loss database. A description of the loss categories and causes of property damage are provided, followed by a statistical assessment of damage and loss broken out by region, cause, and loss category for the time horizon 1970-2004. The impact of the 2004-2005 hurricane season in the Gulf of Mexico is summarized

  14. Causes of diffuse alopecia in women

    Directory of Open Access Journals (Sweden)

    Rustom Aziza

    1994-01-01

    Full Text Available Fifty female patients ranging in age between 14-45 years, complaining of a diffuse loss of hair for periods varying from 1-22 months were thoroughly interrogated to look for the factors leading to the hair loss. Laboratory investigations undertaken included a routine estimation of haemoglobin, ESR, urinalysis, examination of the stools, and any other test indicated by the history or examination. The hair loss was quantitated by asking the patient to collect all the fallen hairs during a 24 hours period for 7 consecutive days, and pack them into appropriately labelled polythene bags. Each collection was weighed to calculated the average daily loss. Such collections were repeated once a month for a period of 4-5 months to monitor the variations in the hair loss and study the effect for treatment. Ten hairs randomly picked from each collection were examined microscopically to look for any shaft abnormalities in addition to the examination of the root end and the distal end. The probable causes of hair loss in these patients were found to be psychologic stress in 21 cases, fever in 11 cases, child-birth/abortion in 5 cases, and a surgical operation in 3 cases. Some of these cases had more than one factor, while in 15 cases there was no clinical evidence of any of the known causes of hair loss. Routine laboratory investigations however revealed a haemoglobin level of less than 12 gm% in 25 cases, an ESR higher than 20 mm in 18 cases, asymptomatic urinary tract infection in 13 cases and gastro-intestinal parasites in stools in 12 cases. Thus every patient had at least one of the causative factors, while several patients had more than one factor which could be responsible for the hair loss. Almost all the cases in this group seemed to have telogen effluvium. Examination of the root end in most of the hairs showed a club hair, while the hair shaft revealed trichorrhexis in some of the hairs in 10 cases and trichoptilosis in 3 cases. Appropriate

  15. Causes of childhood blindness in the northeastern states of India

    Directory of Open Access Journals (Sweden)

    Bhattacharjee Harsha

    2008-01-01

    Full Text Available Background: The northeastern region (NER of India is geographically isolated and ethno-culturally different from the rest of the country. There is lacuna regarding the data on causes of blindness and severe visual impairment in children from this region. Aim: To determine the causes of severe visual impairment and blindness amongst children from schools for the blind in the four states of NER of India. Design and Setting: Survey of children attending special education schools for the blind in the NER. Materials and Methods: Blind and severely visually impaired children (best corrected visual acuity < 20/200 in the better eye, aged up to 16 years underwent visual acuity estimation, external ocular examination, retinoscopy and fundoscopy. Refraction and low vision workup was done where indicated. World Health Organization′s reporting form was used to code anatomical and etiological causes of visual loss. Statistical Analysis: Microsoft Excel Windows software with SPSS. Results: A total of 376 students were examined of whom 258 fulfilled the eligibility criteria. The major anatomical causes of visual loss amongst the 258 were congenital anomalies (anophthalmos, microphthalmos 93 (36.1%; corneal conditions (scarring, vitamin A deficiency 94 (36.7%; cataract or aphakia 28 (10.9%, retinal disorders 15 (5.8% and optic atrophy 14 (5.3%. Nearly half of the children were blind from conditions which were either preventable or treatable (48.5%. Conclusion: Nearly half the childhood blindness in the NER states of India is avoidable and Vitamin A deficiency forms an important component unlike other Indian states. More research and multisectorial effort is needed to tackle congenital anomalies.

  16. AC power losses in Bi-2223/Ag HTS tapes

    International Nuclear Information System (INIS)

    Savvides, N.; Reilly, D.; Mueller, K.-H.; Herrmann, J.

    1998-01-01

    Full text: We report measurements at 77 K of the transport ac losses of Bi-2223/Ag composite tapes. The investigated tapes vary from single filament to multifilament construction and include both conventional tapes and other conductor shapes with twisted filaments. The self-field ac losses were determined at 77 K and 60 Hz as a function of ac current amplitude (0 - 100 A). We observe different behaviour among tapes depending on their quality and strain history. For 'good' virgin tapes the experimental data are well described by the Norris equations for the dependence of power loss P on the amplitude I m of the transport current. The data of good monofilament tapes are fitted to the Norris equation P ∼ I m n for an elliptical cross section (ie. n = 3) and the data of good multifilament tapes are fitted to the Norris equation for a rectangular strip (ie. n = 4). Many specimens, however, show a range of behaviour with lower values of n. Based on our work on the effect of strain on the dc transport properties of tapes, we carried out detailed investigations of the effect of controlled applied bend strain on the ac loss. Our results show that irreversible damage to superconducting filaments (ie. cracks) cause the ac loss to rise and n to decrease with increasing strain. In addition, applied strains much greater than the irreversible strain limit cause the ac loss to increase by several orders of magnitude and become ohmic in character with n = 2. Theoretical work is in progress to model the observed behaviour

  17. The cost of vision loss in Canada. 1. Methodology.

    Science.gov (United States)

    Gordon, Keith D; Cruess, Alan F; Bellan, Lorne; Mitchell, Scott; Pezzullo, M Lynne

    2011-08-01

    This paper outlines the methodology used to estimate the cost of vision loss in Canada. The results of this study will be presented in a second paper. The cost of vision loss (VL) in Canada was estimated using a prevalence-based approach. This was done by estimating the number of people with VL in a base period (2007) and the costs associated with treating them. The cost estimates included direct health system expenditures on eye conditions that cause VL, as well as other indirect financial costs such as productivity losses. Estimates were also made of the value of the loss of healthy life, measured in Disability Adjusted Life Years or DALY's. To estimate the number of cases of VL in the population, epidemiological data on prevalence rates were applied to population data. The number of cases of VL was stratified by gender, age, ethnicity, severity and cause. The following sources were used for estimating prevalence: Population-based eye studies; Canadian Surveys; Canadian journal articles and research studies; and International Population Based Eye Studies. Direct health costs were obtained primarily from Health Canada and Canadian Institute for Health Information (CIHI) sources, while costs associated with productivity losses were based on employment information compiled by Statistics Canada and on economic theory of productivity loss. Costs related to vision rehabilitation (VR) were obtained from Canadian VR organizations. This study shows that it is possible to estimate the costs for VL for a country in the absence of ongoing local epidemiological studies. Copyright © 2011 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.

  18. Female pattern hair loss: Current treatment concepts

    Directory of Open Access Journals (Sweden)

    Quan Q Dinh

    2007-07-01

    Full Text Available Quan Q Dinh, Rodney SinclairDepartment of Dermatology, St Vincent’s Hospital, Fitzroy, Victoria, AustraliaAbstract: Fewer than 45% of women go through life with a full head of hair. Female pattern hair loss is the commonest cause of hair loss in women and prevalence increases with advancing age. Affected women may experience psychological distress and impaired social functioning. In most cases the diagnosis can be made clinically and the condition treated medically. While many women using oral antiandrogens and topical minoxidil will regrow some hair, early diagnosis and initiation of treatment is desirable as these treatments are more effective at arresting progression of hair loss than stimulating regrowth. Adjunctive nonpharmacological treatment modalities such as counseling, cosmetic camouflage and hair transplantation are important measures for some patients. The histology of female pattern hair loss is identical to that of male androgenetic alopecia. While the clinical pattern of the hair loss differs between men, the response to oral antiandrogens suggests that female pattern hair loss is an androgen dependant condition, at least in the majority of cases. Female pattern hair loss is a chronic progressive condition. All treatments need to be continued to maintain the effect. An initial therapeutic response often takes 12 or even 24 months. Given this delay, monitoring for treatment effect through clinical photography or standardized clinical severity scales is helpful.Keywords: female pattern hair loss, androgenetic alopecia

  19. Hypercortisolemia is associated with severity of bone loss and depression in hypothalamic amenorrhea and anorexia nervosa.

    Science.gov (United States)

    Lawson, Elizabeth A; Donoho, Daniel; Miller, Karen K; Misra, Madhusmita; Meenaghan, Erinne; Lydecker, Janet; Wexler, Tamara; Herzog, David B; Klibanski, Anne

    2009-12-01

    Anorexia nervosa (AN) and functional hypothalamic amenorrhea (HA) are associated with low bone density, anxiety, and depression. Women with AN and HA have elevated cortisol levels. Significant hypercortisolemia, as in Cushing's disease, causes bone loss. It is unknown whether anxiety and depression and/or cortisol dysregulation contribute to low bone density in AN or HA. Our objective was to investigate whether hypercortisolemia is associated with bone loss and mood disturbance in women with HA and AN. We conducted a cross-sectional study in a clinical research center. We studied 52 women [21 healthy controls (HC), 13 normal-weight women with functional HA, and 18 amenorrheic women with AN]. Serum samples were measured every 20 min for 12 h overnight and pooled for average cortisol levels. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry (DXA) at anteroposterior and lateral spine and hip. Hamilton Rating Scales for Anxiety (HAM-A) and Depression (HAM-D) were administered. BMD was lower in AN and HA than HC at all sites and lower in AN than HA at the spine. On the HAM-D and HAM-A, AN scored higher than HA, and HA scored higher than HC. Cortisol levels were highest in AN, intermediate in HA, and lowest in HC. HAM-A and HAM-D scores were associated with decreased BMD. Cortisol levels were positively associated with HAM-A and HAM-D scores and negatively associated with BMD. Hypercortisolemia is a potential mediator of bone loss and mood disturbance in these disorders.

  20. PPIB mutations cause severe osteogenesis imperfecta

    NARCIS (Netherlands)

    van Dijk, Fleur S.; Nesbitt, Isabel M.; Zwikstra, Eline H.; Nikkels, Peter G. J.; Piersma, Sander R.; Fratantoni, Silvina A.; Jimenez, Connie R.; Huizer, Margriet; Morsman, Alice C.; Cobben, Jan M.; van Roij, Mirjam H. H.; Elting, Mariet W.; Verbeke, Jonathan I. M. L.; Wijnaendts, Liliane C. D.; Shaw, Nick J.; Högler, Wolfgang; McKeown, Carole; Sistermans, Erik A.; Dalton, Ann; Meijers-Heijboer, Hanne; Pals, Gerard

    2009-01-01

    Deficiency of cartilage-associated protein (CRTAP) or prolyl 3-hydroxylase 1(P3H1) has been reported in autosomal-recessive lethal or severe osteogenesis imperfecta (OI). CRTAP, P3H1, and cyclophilin B (CyPB) form an intracellular collagen-modifying complex that 3-hydroxylates proline at position

  1. Corona helps curb losses

    Energy Technology Data Exchange (ETDEWEB)

    Laasonen, M.; Lahtinen, M.; Lustre, L.

    1996-11-01

    The greatest power losses in electricity transmission arise through a phenomenon called load losses. Corona losses caused by the surface discharge of electricity also constitute a considerable cost item. IVS, the nationwide network company, is investigating corona- induced losses, and has also commissioned similar research from IVO International, the Technical Research Centre of Finland (VTT) and from Tampere University of Technology. The research work strives to gain more in-depth knowledge on the phenomenon of frosting and its impact on corona losses. The correct prediction of frost helps reduce corona losses, while also cutting costs considerably. (orig.)

  2. Increasing mass loss from Greenland's Mittivakkat Gletscher

    DEFF Research Database (Denmark)

    Hasholt, Bent; Mernild, S.H.; Knudsen, N.T.

    2011-01-01

    Warming in the Arctic during the past several decades has caused glaciers to thin and retreat, and recent mass loss from the Greenland Ice Sheet is well documented. Local glaciers peripheral to the ice sheet are also retreating, but few mass-balance observations are available to quantify that ret...... a local phenomenon, but are indicative of glacier changes in the broader region. Mass-balance observations for the MG therefore provide unique documentation of the general retreat of Southeast Greenland's local glaciers under ongoing climate warming....

  3. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

    Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl

    2013-01-01

    Carbon monoxide poisoning is one of the rare causes of hearing loss which may cause reversible or irreversible, unilateral or bilateral hearing loss after acute or chronic exposure. In this report, we present a case of bilateral sensorineural hearing loss in a secondary smelting workshop worker...... after an acute exposure to carbon monoxide. This complication was diagnosed by pure-tone audiometry and confirmed by transient evoked otoacoustic emissions. Hearing loss has not improved after 3 months of followup....

  4. [Current aspects of harmonization of classification of occupational hearing loss].

    Science.gov (United States)

    Pankova, V B; Sinëva, E L; Tavartkiladze, G A; Fedina, I N; Preobrazhenskaia, E A; Mukhamedova, G R

    2013-01-01

    The objective of the present work was to develop unified criteria for the evaluation of the severity of noise-induced hearing loss. Current approaches to taking expert decisions based on the results of medical examination of the patients with impaired hearing are substantially different due to the considerable difference between the criteria for the estimation of hearing envisaged by the international classification, occupational pathology classification, and the national system of medico-social expertise. We undertook an attempt to correct and harmonize the existing criteria for the estimation of severity of occupational hearing loss based on the integration of all the three classification in order to "reduce them to a common denominator" and thereby to ensure the basis for the unified diagnostic and expert decisions for the cases of hearing impairment of any etiology. The project proposed in this paper makes it possible to use unified criteria for the assessment of the degree of hearing loss caused by occupational noises for diagnostic purposes and expertise compatible with the internationally accepted approaches.

  5. Recent progress on MHD-induced loss of D-D fusion products in TFTR

    International Nuclear Information System (INIS)

    Zweben, S.J.; Darrow, D.S.; Budny, R.V.; Cheng, C.Z.; Fredrickson, E.D.; Herrmann, H.; Mynick, H.E.; Schivell, J.

    1993-08-01

    This paper reviews the recent progress made toward understanding the MHD-induced loss of D-D fusion products which has been seen on TFTR since 1988. These measurements have been made using the ''lost alpha'' diagnostic, which is described briefly. The largest MHD- induced loss occurs with coherent 3/2 or 2/1 MHD activity (kink/tearing modes), which can cause up to ∼3--5 times the first-orbit loss at I∼1.6--1.8 MA, roughly a ∼20--30% global los of D-D fusion products. Modeling of these MHD-induced losses has progressed to the point where the basic loss mechanism can be accounted for qualitatively, but the experimental results can not yet be understood quantitatively. Several alpha loss codes are being developed to improve the quantitative comparison between experiment and theory

  6. An overview of hereditary hearing loss.

    Science.gov (United States)

    Bayazit, Yildirim A; Yilmaz, Metin

    2006-01-01

    Understanding the genetic basis of hearing loss is important because almost 50% of profound hearing loss are caused by genetic factors and more than 120 independent genes have been identified. In this review, after a brief explanation of some genetic terms (allele, heterozygosis, homozygosis, polymorphism, genotype and phenotype), classification of genetic hearing loss (syndromic versus nonsyndromic, and recessive dominant, X-linked and mitochondrial) was performed. Some of the most common syndromes (Usher, Pendred, Jervell and Lange-Nielsen, Waardenburg, branchio-oto-renal, Stickler, Treacher Collins and Alport syndromes, biotinidase deficiency and Norrie disease) causing genetic hearing loss were also explained briefly. The genes involved in hearing loss and genetic heterogeneity were presented. Copyright 2006 S. Karger AG, Basel.

  7. Chronic plus binge ethanol exposure causes more severe pancreatic injury and inflammation

    Energy Technology Data Exchange (ETDEWEB)

    Ren, Zhenhua [Department of Anatomy, School of Basic Medicine, Anhui Medical University, Hefei, Anhui, China 230032 (China); Department of Pharmacology and Nutritional Sciences, University of Kentucky College of Medicine, Lexington, KY 40536 (United States); Yang, Fanmuyi; Wang, Xin; Wang, Yongchao; Xu, Mei; Frank, Jacqueline A. [Department of Pharmacology and Nutritional Sciences, University of Kentucky College of Medicine, Lexington, KY 40536 (United States); Ke, Zun-ji [Department of Biochemistry, Shanghai University of Traditional Chinese Medicine, Shanghai 201203 (China); Zhang, Zhuo; Shi, Xianglin [Department of Toxicology and Cancer Biology, University of Kentucky College of Medicine, Lexington, KY 40536 (United States); Luo, Jia, E-mail: jialuo888@uky.edu [Department of Pharmacology and Nutritional Sciences, University of Kentucky College of Medicine, Lexington, KY 40536 (United States)

    2016-10-01

    Alcohol abuse increases the risk for pancreatitis. The pattern of alcohol drinking may impact its effect. We tested a hypothesis that chronic ethanol consumption in combination with binge exposure imposes more severe damage to the pancreas. C57BL/6 mice were divided into four groups: control, chronic ethanol exposure, binge ethanol exposure and chronic plus binge ethanol exposure. For the control group, mice were fed with a liquid diet for two weeks. For the chronic ethanol exposure group, mice were fed with a liquid diet containing 5% ethanol for two weeks. In the binge ethanol exposure group, mice were treated with ethanol by gavage (5 g/kg, 25% ethanol w/v) daily for 3 days. For the chronic plus binge exposure group, mice were fed with a liquid diet containing 5% ethanol for two weeks and exposed to ethanol by gavage during the last 3 days. Chronic and binge exposure alone caused minimal pancreatic injury. However, chronic plus binge ethanol exposure induced significant apoptotic cell death. Chronic plus binge ethanol exposure altered the levels of alpha-amylase, glucose and insulin. Chronic plus binge ethanol exposure caused pancreatic inflammation which was shown by the macrophages infiltration and the increase of cytokines and chemokines. Chronic plus binge ethanol exposure increased the expression of ADH1 and CYP2E1. It also induced endoplasmic reticulum stress which was demonstrated by the unfolded protein response. In addition, chronic plus binge ethanol exposure increased protein oxidation and lipid peroxidation, indicating oxidative stress. Therefore, chronic plus binge ethanol exposure is more detrimental to the pancreas. - Highlights: • Chronic plus binge alcohol drinking causes more pancreatic injury. • Chronic plus binge alcohol drinking induces more pancreatic inflammation. • Chronic plus binge alcohol causes more endoplasmic reticulum stress and oxidative stress.

  8. Chronic plus binge ethanol exposure causes more severe pancreatic injury and inflammation

    International Nuclear Information System (INIS)

    Ren, Zhenhua; Yang, Fanmuyi; Wang, Xin; Wang, Yongchao; Xu, Mei; Frank, Jacqueline A.; Ke, Zun-ji; Zhang, Zhuo; Shi, Xianglin; Luo, Jia

    2016-01-01

    Alcohol abuse increases the risk for pancreatitis. The pattern of alcohol drinking may impact its effect. We tested a hypothesis that chronic ethanol consumption in combination with binge exposure imposes more severe damage to the pancreas. C57BL/6 mice were divided into four groups: control, chronic ethanol exposure, binge ethanol exposure and chronic plus binge ethanol exposure. For the control group, mice were fed with a liquid diet for two weeks. For the chronic ethanol exposure group, mice were fed with a liquid diet containing 5% ethanol for two weeks. In the binge ethanol exposure group, mice were treated with ethanol by gavage (5 g/kg, 25% ethanol w/v) daily for 3 days. For the chronic plus binge exposure group, mice were fed with a liquid diet containing 5% ethanol for two weeks and exposed to ethanol by gavage during the last 3 days. Chronic and binge exposure alone caused minimal pancreatic injury. However, chronic plus binge ethanol exposure induced significant apoptotic cell death. Chronic plus binge ethanol exposure altered the levels of alpha-amylase, glucose and insulin. Chronic plus binge ethanol exposure caused pancreatic inflammation which was shown by the macrophages infiltration and the increase of cytokines and chemokines. Chronic plus binge ethanol exposure increased the expression of ADH1 and CYP2E1. It also induced endoplasmic reticulum stress which was demonstrated by the unfolded protein response. In addition, chronic plus binge ethanol exposure increased protein oxidation and lipid peroxidation, indicating oxidative stress. Therefore, chronic plus binge ethanol exposure is more detrimental to the pancreas. - Highlights: • Chronic plus binge alcohol drinking causes more pancreatic injury. • Chronic plus binge alcohol drinking induces more pancreatic inflammation. • Chronic plus binge alcohol causes more endoplasmic reticulum stress and oxidative stress.

  9. Severe signal loss in diamond beam loss monitors in high particle rate environments by charge trapping in radiation-induced defects

    Energy Technology Data Exchange (ETDEWEB)

    Kassel, Florian; Boer, Wim de [Institute for Experimental Nuclear Physics (IEKP), KIT, Karlsruhe (Germany); Guthoff, Moritz; Dabrowski, Anne [CERN, Meyrin (Switzerland)

    2016-10-15

    The beam condition monitoring leakage (BCML) system is a beam monitoring device in the compact muon solenoid (CMS) experiment at the large hadron collider (LHC). As detectors 32 poly-crystalline (pCVD) diamond sensors are positioned in rings around the beam pipe. Here, high particle rates occur from the colliding beams scattering particles outside the beam pipe. These particles cause defects, which act as traps for the ionization, thus reducing the charge collection efficiency (CCE). However, the loss in CCE was much more severe than expected from low rate laboratory measurements and simulations, especially in single-crystalline (sCVD) diamonds, which have a low initial concentration of defects. After an integrated luminosity of a few fb{sup -1} corresponding to a few weeks of LHC operation, the CCE of the sCVD diamonds dropped by a factor of five or more and quickly approached the poor CCE of pCVD diamonds. The reason why in real experiments the CCE is much worse than in laboratory experiments is related to the ionization rate. At high particle rates the trapping rate of the ionization is so high compared with the detrapping rate, that space charge builds up. This space charge reduces locally the internal electric field, which in turn increases the trapping rate and recombination and hence reduces the CCE in a strongly non-linear way. A diamond irradiation campaign was started to investigate the rate-dependent electrical field deformation with respect to the radiation damage. Besides the electrical field measurements via the transient current technique (TCT), the CCE was measured. The experimental results were used to create an effective deep trap model that takes the radiation damage into account. Using this trap model, the rate-dependent electrical field deformation and the CCE were simulated with the software SILVACO TCAD. The simulation, tuned to rate-dependent measurements from a strong radioactive source, was able to predict the non-linear decrease of the

  10. Severe signal loss in diamond beam loss monitors in high particle rate environments by charge trapping in radiation-induced defects

    International Nuclear Information System (INIS)

    Kassel, Florian; Boer, Wim de; Guthoff, Moritz; Dabrowski, Anne

    2016-01-01

    The beam condition monitoring leakage (BCML) system is a beam monitoring device in the compact muon solenoid (CMS) experiment at the large hadron collider (LHC). As detectors 32 poly-crystalline (pCVD) diamond sensors are positioned in rings around the beam pipe. Here, high particle rates occur from the colliding beams scattering particles outside the beam pipe. These particles cause defects, which act as traps for the ionization, thus reducing the charge collection efficiency (CCE). However, the loss in CCE was much more severe than expected from low rate laboratory measurements and simulations, especially in single-crystalline (sCVD) diamonds, which have a low initial concentration of defects. After an integrated luminosity of a few fb -1 corresponding to a few weeks of LHC operation, the CCE of the sCVD diamonds dropped by a factor of five or more and quickly approached the poor CCE of pCVD diamonds. The reason why in real experiments the CCE is much worse than in laboratory experiments is related to the ionization rate. At high particle rates the trapping rate of the ionization is so high compared with the detrapping rate, that space charge builds up. This space charge reduces locally the internal electric field, which in turn increases the trapping rate and recombination and hence reduces the CCE in a strongly non-linear way. A diamond irradiation campaign was started to investigate the rate-dependent electrical field deformation with respect to the radiation damage. Besides the electrical field measurements via the transient current technique (TCT), the CCE was measured. The experimental results were used to create an effective deep trap model that takes the radiation damage into account. Using this trap model, the rate-dependent electrical field deformation and the CCE were simulated with the software SILVACO TCAD. The simulation, tuned to rate-dependent measurements from a strong radioactive source, was able to predict the non-linear decrease of the CCE in

  11. Natural Disasters under the Form of Severe Storms in Europe: the Cause-Effect Analysis

    Directory of Open Access Journals (Sweden)

    Virginia Câmpeanu

    2009-07-01

    Full Text Available For more than 100 years, from 1900 to 2008, there were almost 400 storms natural disasters in Europe, 40% of which occurred in the 1990s. The international prognoses for the world weather suggest a tendency toward increasing in frequency and intensity of the severe storms as the climate warms. In these circumstances, for a researcher in the field of Environmental Economics, a natural question occurs, on whether people can contribute to reducing the frequency and the magnitude of severe storms that produce disastreous social and economic effects, by acting on their causes. In researching an answer to support the public policies in the field, a cause-effect analysis applied to Europe might make a contribution to the literature in the field. This especially considering the fact that international literature regarding the factors influencing global warming contains certainties in regard to the natural factors of influence, but declared incertitudes or skepticism in regard to anthropogenic ones. Skepticism, and even tension arised during the international negotiations in Copenhagen (December 2009 in regard to the agreement for limiting global warming, with doubts being raised about the methods used by experts of the International Climate Experts Group (GIEC, and thus the results obtained, which served as a basis for the negotiations. The object of critics was in regard to the form, and at times in regard to the content. It was not about contesting the phenomenon of Global warming during the negotiations, but the methods of calculation. The methodology relies on qualitative (type top down and quantitative (type correlations bottom up cause-effect analysis of the storm disasters in Europe. Based on the instruments used, we proposed a dynamic model of association of the evolution of storm disasters in Europe with anthropogenic factors, with 3 variants. Results: The diagram cause-effect (Ishikawa or fishbone diagram and quantitative correlation of sub

  12. Sudden visual loss after cardiac resynchronization therapy device implantation.

    Science.gov (United States)

    De Vitis, Luigi A; Marchese, Alessandro; Giuffrè, Chiara; Carnevali, Adriano; Querques, Lea; Tomasso, Livia; Baldin, Giovanni; Maestranzi, Gisella; Lattanzio, Rosangela; Querques, Giuseppe; Bandello, Francesco

    2017-03-10

    To report a case of sudden decrease in visual acuity possibly due to a cardiogenic embolism in a patient who underwent cardiac resynchronization therapy (CRT) device implantation. A 62-year-old man with severe left ventricular systolic dysfunction and a left bundle branch block was referred to our department because of a sudden decrease in visual acuity. Nine days earlier, he had undergone cardiac transapical implantation of a CRT device, which was followed, 2 days later, by an inflammatory reaction. The patient underwent several general and ophthalmologic examinations, including multimodal imaging. At presentation, right eye (RE) best-corrected visual acuity (BCVA) was counting fingers and RE pupil was hyporeactive. Fundus examination revealed white-centered hemorrhagic dots suggestive of Roth spots. Fluorescein angiography showed delay in vascular perfusion during early stage, late hyperfluorescence of the macula and optic disk, and peripheral perivascular leakage. The first visual field test showed complete loss of vision RE and a normal left eye. Due to suspected giant cell arteritis, temporal artery biopsy was performed. Thirty minutes after the procedure, an ischemic stroke with right hemisyndrome and aphasia occurred. The RE BCVA worsened to hands motion. Four months later, RE BCVA did not improve, despite improvement in fluorescein angiography inflammatory sign. We report a possible cardiogenic embolism secondary to undiagnosed infective endocarditis causing monocular visual loss after CRT device implantation. It remains unclear how the embolus caused severe functional damage without altering the retinal anatomical structure.

  13. Beam loss caused by edge focusing of injection bump magnets and its mitigation in the 3-GeV rapid cycling synchrotron of the Japan Proton Accelerator Research Complex

    Directory of Open Access Journals (Sweden)

    H. Hotchi

    2016-01-01

    Full Text Available In the 3-GeV rapid cycling synchrotron of the Japan Proton Accelerator Research Complex, transverse injection painting is utilized not only to suppress space-charge induced beam loss in the low energy region but also to mitigate foil scattering beam loss during charge-exchange injection. The space-charge induced beam loss is well minimized by the combination of modest transverse painting and full longitudinal painting. But, for sufficiently mitigating the foil scattering part of beam loss, the transverse painting area has to be further expanded. However, such a wide-ranging transverse painting had not been realized until recently due to beta function beating caused by edge focusing of pulsed injection bump magnets during injection. This beta function beating additionally excites random betatron resonances through a distortion of the lattice superperiodicity, and its resultant deterioration of the betatron motion stability causes significant extra beam loss when expanding the transverse painting area. To solve this issue, we newly installed pulse-type quadrupole correctors to compensate the beta function beating. This paper presents recent experimental results on this correction scheme for suppressing the extra beam loss, while discussing the beam loss and its mitigation mechanisms with the corresponding numerical simulations.

  14. Missing Strands? Dealing with Hair Loss

    Science.gov (United States)

    ... 2017 Print this issue Missing Strands? Dealing with Hair Loss En español Send us your comments Hair loss is often associated with men and aging, but ... or their treatments, and many other things cause hair loss. The most common type of hair loss is ...

  15. Choline Deficiency Causes Colonic Type II Natural Killer T (NKT) Cell Loss and Alleviates Murine Colitis under Type I NKT Cell Deficiency.

    Science.gov (United States)

    Sagami, Shintaro; Ueno, Yoshitaka; Tanaka, Shinji; Fujita, Akira; Niitsu, Hiroaki; Hayashi, Ryohei; Hyogo, Hideyuki; Hinoi, Takao; Kitadai, Yasuhiko; Chayama, Kazuaki

    2017-01-01

    Serum levels of choline and its derivatives are lower in patients with inflammatory bowel disease (IBD) than in healthy individuals. However, the effect of choline deficiency on the severity of colitis has not been investigated. In the present study, we investigated the role of choline deficiency in dextran sulfate sodium (DSS)-induced colitis in mice. Methionine-choline-deficient (MCD) diet lowered the levels of type II natural killer T (NKT) cells in the colonic lamina propria, peritoneal cavity, and mesenteric lymph nodes, and increased the levels of type II NKT cells in the livers of wild-type B6 mice compared with that in mice fed a control (CTR) diet. The gene expression pattern of the chemokine receptor CXCR6, which promotes NKT cell accumulation, varied between colon and liver in a manner dependent on the changes in the type II NKT cell levels. To examine the role of type II NKT cells in colitis under choline-deficient conditions, we assessed the severity of DSS-induced colitis in type I NKT cell-deficient (Jα18-/-) or type I and type II NKT cell-deficient (CD1d-/-) mice fed the MCD or CTR diets. The MCD diet led to amelioration of inflammation, decreases in interferon (IFN)-γ and interleukin (IL)-4 secretion, and a decrease in the number of IFN-γ and IL-4-producing NKT cells in Jα18-/- mice but not in CD1d-/- mice. Finally, adaptive transfer of lymphocytes with type II NKT cells exacerbated DSS-induced colitis in Jα18-/- mice with MCD diet. These results suggest that choline deficiency causes proinflammatory type II NKT cell loss and alleviates DSS-induced colitis. Thus, inflammation in DSS-induced colitis under choline deficiency is caused by type II NKT cell-dependent mechanisms, including decreased type II NKT cell and proinflammatory cytokine levels.

  16. Factors contributing to tooth loss among the elderly: A cross sectional study.

    Science.gov (United States)

    Natto, Zuhair S; Aladmawy, Majdi; Alasqah, Mohammed; Papas, Athena

    2014-12-01

    The present study evaluates the influence of several demographic, health, personal, and clinical factors on the number of missing teeth in old age sample. The number of patients included was 259; they received a full mouth examination and answered a questionnaire provided by one examiner. All the variables related to teeth loss based on the literature were included. These variables focused on age, gender, race, marital status, clinical attachment level, pocket depth, year of smoking, number of cigarettes smoked per day, number of medications, root decay, coronal decay, health status, and year of education. Statistical analysis involved stepwise multivariate linear regression. Teeth loss was statistically associated with clinical attachment level (CAL)(p value 0.0001), pocket depth (PD) (0.0007) and education level (0.0048). When smoking was included in the model, age was significantly associated with teeth loss (0.0037). At least one of these four factors was also related to teeth loss in several specific groups such as diabetes mellitus, male, and White. The multiple linear regressions for all the proposed variables showed that they contributed to teeth loss by about 23%. It can be concluded that less education or increased clinical attachment level loss may increase number of missing teeth. Additionally, age may cause teeth loss in the presence of smoking. Copyright © 2014. Published by Elsevier B.V.

  17. Causes and pattern of tooth loss in children and adolescents in a ...

    African Journals Online (AJOL)

    Information obtained included age, gender, missing and extracted teeth, and reasons for extraction/tooth loss. Exfoliated primary teeth and congenitally missing teeth were excluded. Data was analysed using Epi info 2007 statistical software. Chi-square test was used to compare proportions of tooth loss among age groups.

  18. Streptozotocin, Type I Diabetes Severity and Bone

    Directory of Open Access Journals (Sweden)

    Motyl Katherine

    2009-01-01

    Full Text Available Abstract As many as 50% of adults with type I (T1 diabetes exhibit bone loss and are at increased risk for fractures. Therapeutic development to prevent bone loss and/or restore lost bone in T1 diabetic patients requires knowledge of the molecular mechanisms accounting for the bone pathology. Because cell culture models alone cannot fully address the systemic/metabolic complexity of T1 diabetes, animal models are critical. A variety of models exist including spontaneous and pharmacologically induced T1 diabetic rodents. In this paper, we discuss the streptozotocin (STZ-induced T1 diabetic mouse model and examine dose-dependent effects on disease severity and bone. Five daily injections of either 40 or 60 mg/kg STZ induce bone pathologies similar to spontaneously diabetic mouse and rat models and to human T1 diabetic bone pathology. Specifically, bone volume, mineral apposition rate, and osteocalcin serum and tibia messenger RNA levels are decreased. In contrast, bone marrow adiposity and aP2 expression are increased with either dose. However, high-dose STZ caused a more rapid elevation of blood glucose levels and a greater magnitude of change in body mass, fat pad mass, and bone gene expression (osteocalcin, aP2. An increase in cathepsin K and in the ratio of RANKL/OPG was noted in high-dose STZ mice, suggesting the possibility that severe diabetes could increase osteoclast activity, something not seen with lower doses. This may contribute to some of the disparity between existing studies regarding the role of osteoclasts in diabetic bone pathology. Examination of kidney and liver toxicity indicate that the high STZ dose causes some liver inflammation. In summary, the multiple low-dose STZ mouse model exhibits a similar bone phenotype to spontaneous models, has low toxicity, and serves as a useful tool for examining mechanisms of T1 diabetic bone loss.

  19. Streptozotocin, Type I Diabetes Severity and Bone

    Directory of Open Access Journals (Sweden)

    Motyl Katherine

    2009-03-01

    Full Text Available Abstract As many as 50% of adults with type I (T1 diabetes exhibit bone loss and are at increased risk for fractures. Therapeutic development to prevent bone loss and/or restore lost bone in T1 diabetic patients requires knowledge of the molecular mechanisms accounting for the bone pathology. Because cell culture models alone cannot fully address the systemic/metabolic complexity of T1 diabetes, animal models are critical. A variety of models exist including spontaneous and pharmacologically induced T1 diabetic rodents. In this paper, we discuss the streptozotocin (STZ-induced T1 diabetic mouse model and examine dose-dependent effects on disease severity and bone. Five daily injections of either 40 or 60 mg/kg STZ induce bone pathologies similar to spontaneously diabetic mouse and rat models and to human T1 diabetic bone pathology. Specifically, bone volume, mineral apposition rate, and osteocalcin serum and tibia messenger RNA levels are decreased. In contrast, bone marrow adiposity and aP2 expression are increased with either dose. However, high-dose STZ caused a more rapid elevation of blood glucose levels and a greater magnitude of change in body mass, fat pad mass, and bone gene expression (osteocalcin, aP2. An increase in cathepsin K and in the ratio of RANKL/OPG was noted in high-dose STZ mice, suggesting the possibility that severe diabetes could increase osteoclast activity, something not seen with lower doses. This may contribute to some of the disparity between existing studies regarding the role of osteoclasts in diabetic bone pathology. Examination of kidney and liver toxicity indicate that the high STZ dose causes some liver inflammation. In summary, the multiple low-dose STZ mouse model exhibits a similar bone phenotype to spontaneous models, has low toxicity, and serves as a useful tool for examining mechanisms of T1 diabetic bone loss.

  20. Characteristics and Impacts of the severe Hailstorm on 28 July 2013

    Science.gov (United States)

    Kunz, Michael; Jürgen Punge, Heinz; Fluck, Elody; Schmidberger, Manuel; Blahak, Ulrich; Handwerker, Jan; Mohr, Susanna; Mühr, Bernhard

    2015-04-01

    On 27/28 July, two severe supercell thunderstorms in Germany caused unexpected extreme losses of 3.1 bn EUR(insured) and 4.0 bn EUR(economic), respectively. According to the recently published damage statistics of Munich Re for the year 2013, these hail events were the costliest natural catastrophe in worldwide for that year ranked by insured losses. This example exemplifies the large damage potential related to hail events, which is still underestimated both by the public and the insurance industry. On 27 July, the first supercell moved over the federal states of North Rhine-Westphalia and Lower Saxony. Large hail with diameters of up to 7.5 cm according to observations archived in the European Severe Weather Database (ESWD) caused severe damage especially over the Volkswagen factory in Wolfsburg. One day later, on 28 July, another supercell formed upstream of the Black Forest Mountains and moved almost parallel over the Swabian Jura and Bavaria. Hail with diameters of up to 8 cm fell over a heavily populated region between the cities of Reutlingen and Tübingen. In this area, exposed assets are extremely high, which partly explain the high total loss. Approximately 100,000 buildings and 50,000 automobiles (not considered are the damaged automobiles at the parking lot in Wolfsburg) were severely damaged by these two events. Considering the single event definition over a 72-hr period, which is usually applied in the insurance industry, these hailstorms were one of the most expensive loss events in Germany. In this paper, we investigate the severe hailstorm on 28 July from different views. By using and combining available observational data sets, the objective is to reconstruct the whole events at a very high resolution and to examine the conditions that are most relevant for convective initiation and the further development of the organized convective cell. Using a series of photos of damaged objects the aim is to relate different object classes and hail stone

  1. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability.

    Science.gov (United States)

    Houge, G; Rasmussen, I H; Hovland, R

    2012-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the post-synaptic density, and a role in RAS/MAPK-dependent signal transduction.

  2. Understanding Loss Deductions for Timber

    Science.gov (United States)

    John Greene; Michael Jacobson

    1998-01-01

    Forestland owners whose timber has been destroyed may be eligible to take a deduction for the loss on their federal income tax. The loss must be physical in nature and caused by an identifiable event or combination of events that has run its course. There are two types of losses from natural events. Casualty losses are sudden, unexpected, and unusual - as from a fire...

  3. Phenomenological uncertainty analysis of containment building pressure load caused by severe accident sequences

    International Nuclear Information System (INIS)

    Park, S.Y.; Ahn, K.I.

    2014-01-01

    Highlights: • Phenomenological uncertainty analysis has been applied to level 2 PSA. • The methodology provides an alternative to simple deterministic analyses and sensitivity studies. • A realistic evaluation provides a more complete characterization of risks. • Uncertain parameters of MAAP code for the early containment failure were identified. - Abstract: This paper illustrates an application of a severe accident analysis code, MAAP, to the uncertainty evaluation of early containment failure scenarios employed in the containment event tree (CET) model of a reference plant. An uncertainty analysis of containment pressure behavior during severe accidents has been performed for an optimum assessment of an early containment failure model. The present application is mainly focused on determining an estimate of the containment building pressure load caused by severe accident sequences of a nuclear power plant. Key modeling parameters and phenomenological models employed for the present uncertainty analysis are closely related to the in-vessel hydrogen generation, direct containment heating, and gas combustion. The basic approach of this methodology is to (1) develop severe accident scenarios for which containment pressure loads should be performed based on a level 2 PSA, (2) identify severe accident phenomena relevant to an early containment failure, (3) identify the MAAP input parameters, sensitivity coefficients, and modeling options that describe or influence the early containment failure phenomena, (4) prescribe the likelihood descriptions of the potential range of these parameters, and (5) evaluate the code predictions using a number of random combinations of parameter inputs sampled from the likelihood distributions

  4. Clonal Clusters and Virulence Factors of Group C and G Streptococcus Causing Severe Infections, Manitoba, Canada, 2012-2014.

    Science.gov (United States)

    Lother, Sylvain A; Demczuk, Walter; Martin, Irene; Mulvey, Michael; Dufault, Brenden; Lagacé-Wiens, Philippe; Keynan, Yoav

    2017-07-01

    The incidence of group C and G Streptococcus (GCGS) bacteremia, which is associated with severe disease and death, is increasing. We characterized clinical features, outcomes, and genetic determinants of GCGS bacteremia for 89 patients in Winnipeg, Manitoba, Canada, who had GCGS bacteremia during 2012-2014. Of the 89 patients, 51% had bacteremia from skin and soft tissue, 70% had severe disease features, and 20% died. Whole-genome sequencing analysis was performed on isolates derived from 89 blood samples and 33 respiratory sample controls: 5 closely related genetic lineages were identified as being more likely to cause invasive disease than non-clade isolates (83% vs. 57%, p = 0.002). Virulence factors cbp, fbp, speG, sicG, gfbA, and bca clustered clonally into these clades. A clonal distribution of virulence factors may account for severe and fatal cases of bacteremia caused by invasive GCGS.

  5. First report of leaf rust of blueberry caused by Thekopsora minima in Mexico

    Science.gov (United States)

    Blueberry (Vaccinium corymbosum L.) is becoming an important crop in the states of Jalisco and Michoacan in Mexico. As the area under blueberry cultivation increases, new diseases causing severe losses are appearing. Leaf rust is one of the most destructive diseases of blueberry in Mexico. Sori on t...

  6. Changes in biodiversity and functioning of reef fish assemblages following coral bleaching and coral loss

    KAUST Repository

    Pratchett, M.S.; Hoey, A.S.; Wilson, S.K.; Messmer, V.; Graham, N.A.J.

    2011-01-01

    Coral reef ecosystems are increasingly subject to severe, large-scale disturbances caused by climate change (e.g., coral bleaching) and other more direct anthropogenic impacts. Many of these disturbances cause coral loss and corresponding changes in habitat structure, which has further important effects on abundance and diversity of coral reef fishes. Declines in the abundance and diversity of coral reef fishes are of considerable concern, given the potential loss of ecosystem function. This study explored the effects of coral loss, recorded in studies conducted throughout the world, on the diversity of fishes and also on individual responses of fishes within different functional groups. Extensive (>60%) coral loss almost invariably led to declines in fish diversity. Moreover, most fishes declined in abundance following acute disturbances that caused >10% declines in local coral cover. Response diversity, which is considered critical in maintaining ecosystem function and promoting resilience, was very low for corallivores, but was much higher for herbivores, omnivores and carnivores. Sustained and ongoing climate change thus poses a significant threat to coral reef ecosystems and diversity hotspots are no less susceptible to projected changes in diversity and function.

  7. Changes in biodiversity and functioning of reef fish assemblages following coral bleaching and coral loss

    KAUST Repository

    Pratchett, M.S.

    2011-08-12

    Coral reef ecosystems are increasingly subject to severe, large-scale disturbances caused by climate change (e.g., coral bleaching) and other more direct anthropogenic impacts. Many of these disturbances cause coral loss and corresponding changes in habitat structure, which has further important effects on abundance and diversity of coral reef fishes. Declines in the abundance and diversity of coral reef fishes are of considerable concern, given the potential loss of ecosystem function. This study explored the effects of coral loss, recorded in studies conducted throughout the world, on the diversity of fishes and also on individual responses of fishes within different functional groups. Extensive (>60%) coral loss almost invariably led to declines in fish diversity. Moreover, most fishes declined in abundance following acute disturbances that caused >10% declines in local coral cover. Response diversity, which is considered critical in maintaining ecosystem function and promoting resilience, was very low for corallivores, but was much higher for herbivores, omnivores and carnivores. Sustained and ongoing climate change thus poses a significant threat to coral reef ecosystems and diversity hotspots are no less susceptible to projected changes in diversity and function.

  8. Trends, productivity losses, and associated medical conditions among toxoplasmosis deaths in the United States, 2000-2010.

    Science.gov (United States)

    Cummings, Patricia L; Kuo, Tony; Javanbakht, Marjan; Sorvillo, Frank

    2014-11-01

    Few studies have quantified toxoplasmosis mortality, associated medical conditions, and productivity losses in the United States. We examined national multiple cause of death data and estimated productivity losses caused by toxoplasmosis during 2000-2010. A matched case-control analysis examined associations between comorbid medical conditions and toxoplasmosis deaths. In total, 789 toxoplasmosis deaths were identified during the 11-year study period. Blacks and Hispanics had the highest toxoplasmosis mortality compared with whites. Several medical conditions were associated with toxoplasmosis deaths, including human immunodeficiency virus (HIV), lymphoma, leukemia, and connective tissue disorders. The number of toxoplasmosis deaths with an HIV codiagnosis declined from 2000 to 2010; the numbers without such a codiagnosis remained static. Cumulative disease-related productivity losses for the 11-year period were nearly $815 million. Although toxoplasmosis mortality has declined in the last decade, the infection remains costly and is an important cause of preventable death among non-HIV subgroups. © The American Society of Tropical Medicine and Hygiene.

  9. Beliefs about causes, symptoms, and stigma associated with severe mental illness among 'highly acculturated' Chinese-American patients.

    Science.gov (United States)

    Lin, Susan Y

    2013-12-01

    Literature about experiences of mental illness among ethnic minority has tended to focus on first-generation migrants. This study fills that gap by exploring experiences among highly acculturated Chinese-American patients with mental illness. Twenty-nine participants completed semi-structured interviews based on Kleinman's explanatory model, which were audio-taped, transcribed and coded for qualitative analysis. Beliefs about the causes of mental illness included biological factors, head trauma and personal losses. Issues relating to stigma and shame were also discussed. Highly acculturated ethnic minority patients may ascribe to a biomedical model at the same time as ascribing to culture-specific beliefs.

  10. Impact of socioeconomic deprivation on rate and cause of death in severe mental illness.

    Science.gov (United States)

    Martin, Julie Langan; McLean, Gary; Park, John; Martin, Daniel J; Connolly, Moira; Mercer, Stewart W; Smith, Daniel J

    2014-09-12

    Socioeconomic status has important associations with disease-specific mortality in the general population. Although individuals with Severe Mental Illnesses (SMI) experience significant premature mortality, the relationship between socioeconomic status and mortality in this group remains under investigated. We aimed to assess the impact of socioeconomic status on rate and cause of death in individuals with SMI (schizophrenia and bipolar disorder) relative to the local (Glasgow) and wider (Scottish) populations. Cause and age of death during 2006-2010 inclusive for individuals with schizophrenia or bipolar disorder registered on the Glasgow Psychosis Clinical Information System (PsyCIS) were obtained by linkage to the Scottish General Register Office (GRO). Rate and cause of death by socioeconomic status, measured by Scottish Index of Multiple Deprivation (SIMD), were compared to the Glasgow and Scottish populations. Death rates were higher in people with SMI across all socioeconomic quintiles compared to the Glasgow and Scottish populations, and persisted when suicide was excluded. Differences were largest in the most deprived quintile (794.6 per 10,000 population vs. 274.7 and 252.4 for Glasgow and Scotland respectively). Cause of death varied by socioeconomic status. For those living in the most deprived quintile, higher drug-related deaths occurred in those with SMI compared to local Glasgow and wider Scottish population rates (12.3% vs. 5.9%, p = socioeconomic quintiles compared to the Glasgow and Scottish populations but was most marked in the most deprived quintiles when suicide was excluded as a cause of death. Further work assessing the impact of socioeconomic status on specific causes of premature mortality in SMI is needed.

  11. [Acute visual loss in pregnancy caused by craniopharyngioma].

    Science.gov (United States)

    Grillo-Mallo, E; Jiménez-Benito, J; Diéz-Feijóo, E; Alonso Alonso, I; Ferrero Collado, A; Muñoz Quiñones, S

    2014-04-01

    A 38-year-old female, at 20-weeks gestation, experienced a sudden visual loss and visual-field abnormalities. The neuroimaging tests showed a craniopharyngioma. Surgical removal was performed with a successful outcome as regards the pregnancy and visual function. It is known that pituitary adenomas may grow during pregnancy; however this is unusual in craniopharyngiomas. They usually present with visual problems due to their suprasellar topography. Surgery is the treatment of choice, the outcome essentially depending on its complete resection. Copyright © 2010 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  12. Severe Hemorrhage from the Umbilical Cord at Birth: A Preventable Cause of Neonatal Shock

    OpenAIRE

    Singh, Neetu; Suresh, Gautham

    2013-01-01

    Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe...

  13. Mutations in CHD7 in patients with CHARGE syndrome cause T-B + natural killer cell + severe combined immune deficiency and may cause Omenn-like syndrome.

    NARCIS (Netherlands)

    Gennery, A.R.; Slatter, M.A.; Rice, J.; Hoefsloot, L.H.; Barge, D.; McLean-Tooke, A.; Montgomery, T.; Goodship, J.A.; Burt, A.D.; Flood, T.J.; Abinun, M.; Cant, A.J.; Johnson, D.

    2008-01-01

    More than 11 genetic causes of severe combined immunodeficiency (SCID) have been identified, affecting development and/or function of T lymphocytes, and sometimes B lymphocytes and natural killer (NK) cells. Deletion of 22q11.2 is associated with immunodeficiency, although less than 1% of cases are

  14. Relationship between Affective Symptoms and Malnutrition Severity in Severe Anorexia Nervosa

    Science.gov (United States)

    Mattar, Lama; Huas, Caroline; group, EVHAN; Godart, Nathalie

    2012-01-01

    Background Very few studies have investigated the relationship between malnutrition and psychological symptoms in Anorexia Nervosa (AN). They have used only body weight or body mass index (BMI) for the nutritional assessment and did not always report on medication, or if they did, it was not included in the analysis of results, and they did not include confounding factors such as duration of illness, AN subtype or age. The present study investigates this relationship using indicators other than BMI/weight, among which body composition and biological markers, also considering potential confounders related to depression and anxiety. Methods 155 AN patients, (DSM-IV) were included consecutively upon admission to inpatient treatment. Depression, anxiety, obsessive behaviours and social functioning were measured using various scales. Nutritional status was measured using BMI, severity of weight loss, body composition, and albumin and prealbumin levels. Results No correlation was found between BMI at inclusion, fat-free mass index, fat mass index, and severity of weight loss and any of the psychometric scores. Age and medication are the only factors that affect the psychological scores. None of the psychological scores were explained by the nutritional indicators with the exception of albumin levels which was negatively linked to the LSAS fear score (p = 0.024; beta = −0.225). Only the use of antidepressants explained the variability in BDI scores (p = 0.029; beta = 0.228) and anxiolytic use explained the variability in HADs depression scores (p = 0.037; beta = 0.216). Conclusion The present study is a pioneer investigation of various nutritional markers in relation to psychological symptoms in severely malnourished AN patients. The clinical hypothesis that malnutrition partly causes depression and anxiety symptoms in AN in acute phase is not confirmed, and future studies are needed to back up our results. PMID:23185320

  15. Medicines and Bone Loss

    Science.gov (United States)

    ... The doses of thyroid hormone used to treat hypothyroidism (underactive thyroid) don’t harm bone and shouldn’t be cause for concern. Only high doses, used for thyroid cancer treatment, can cause bone loss. High doses or long- ...

  16. Climatology of damage-causing hailstorms over Germany

    Science.gov (United States)

    Kunz, M.; Puskeiler, M.; Schmidberger, M.

    2012-04-01

    In several regions of Central Europe, such as southern Germany, Austria, Switzerland, and northern Italy, hailstorms often cause substantial damage to buildings, crops, or automobiles on the order of several million EUR. In the federal state of Baden-Württemberg, for example, most of the insured damage to buildings is caused by large hailstones. Due to both their local-scale extent and insufficient direct monitoring systems, hail swaths are not captured accurately and uniquely by a single observation system. Remote-sensing systems such as radars are able to detect convection signals in a basic way, but they lack the ability to discern a clear relation between measured intensity and hail on the ground. These shortcomings hamper statistical analysis on the hail probability and intensity. Hail modelling thus is a big challenge for the insurance industry. Within the project HARIS-CC (Hail Risk and Climate Change), different meteorological observations are combined (3D / 2D radar, lightning, satellite and radiosounding data) to obtain a comprehensive picture of the hail climatology over Germany. The various approaches were tested and calibrated with loss data from different insurance companies between 2005 and 2011. Best results are obtained by considering the vertical distance between the 0°C level of the atmosphere and the echo top height estimated from 3D reflectivity data from the radar network of German Weather Service (DWD). Additionally, frequency, intensity, width, and length of hail swaths are determined by applying a cell tracking algorithm to the 3D radar data (TRACE3D; Handwerker, 2002). The hailstorm tracks identified are merged with loss data using a geographical information system (GIS) to verify damage-causing hail on the ground. Evaluating the hailstorm climatology revealed that hail probability exhibits high spatial variability even over short distances. An important issue is the spatial pattern of hail occurrence that is considered to be due to

  17. Cause-Specific Mortality after Stroke: Relation to Age, Sex, Stroke Severity, and Risk Factors in a 10-Year Follow-Up Study

    DEFF Research Database (Denmark)

    Mogensen, UB; Olsen, TS; Andersen, KK

    2013-01-01

    We investigated cause-specific mortality in relation to age, sex, stroke severity, and cardiovascular risk factor profile in the Copenhagen Stroke Study cohort with 10 years of follow-up. In a Copenhagen community, all patients admitted to the hospital with stroke during 1992-1993 (n = 988) were.......2% for nonvascular disease. Death after stroke was associated with older age, male sex, greater stroke severity, and diabetes regardless of the cause of death. Previous stroke and hemorrhagic stroke were associated with death by stroke, ischemic heart disease was associated with death by heart/arterial disease...... registered on admission. Evaluation included stroke severity, computed tomography scan, and a cardiovascular risk profile. Cause of death within 10 years according to death certificate information was classified as stroke, heart/arterial disease, or nonvascular disease. Competing-risks analyses were...

  18. Impact of weight loss achieved through a multidisciplinary intervention on appetite in patients with severe obesity

    DEFF Research Database (Denmark)

    Coutinho, Silvia R; Rehfeld, Jens F; Holst, Jens J

    2018-01-01

    The impact of lifestyle-induced weight loss (WL) on appetite in patients with obesity remains controversial. This study aimed was to assess the short- and long-term impact of WL achieved by diet and exercise, on appetite in patients with obesity. Thirty-five (22 females) adults with severe obesity......), in the fasting and postprandial states, were measured at baseline (B), week 4 (W4), 1 and 2-years (and average values for all fasting and postprandial time points computed). BW was significantly reduced and VO2max (ml/kg/min) increased at all time points compared with B (3.5, 8.1 and 8.4 % WL and 7, 11 and 8...... compared with B. Average GLP-1 was reduced at W4 and CCK increased at 2y. After lifestyle-induced WL, patients with severe obesity will, therefore, have to deal with increased hunger in the long-term. In conclusion, sustained WL at 2y achieved with diet and exercise is associated with increased hunger...

  19. Case of severe intestinal complications caused by high dose-rate intracavitary irradiation for cervical cancer

    Energy Technology Data Exchange (ETDEWEB)

    Koga, Kenji; Nishikawa, Kiyoshi; Matsuki, Kazuhiko; Watanabe, Katsushi

    1987-02-01

    A 46-year-old woman with severe intestinal complication caused by high dose-rate intracavitary irradiation is reported. She received radiation treatment of stage IIb cervical cancer between July 24 and September 26, 1984: a dose of 2400 rad to a point A concurrently with 2000 rad to the parametrium following 4000 rad to the whole pelvis. Eight months later she developed diarrhea and bloody stool. Barium enema study revealed a stenosis at 20 to 25 cm from the anal ring and romanoscopy oozing coagula at the same site. On November 29, 1985 transverse colostomy was performed because of continuing bloody stool and abdominal pain. On January 30, 1986 resection of the ileum and ileostomy were done because of the ileum perforation located 26 cm apart from the ileum end. Some discussion on the causes of this complication are made, suggesting that short length of a tandem and deep location of ovoids influence its cause.

  20. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    Science.gov (United States)

    Hardies, Katia; May, Patrick; Djémié, Tania; Tarta-Arsene, Oana; Deconinck, Tine; Craiu, Dana; Helbig, Ingo; Suls, Arvid; Balling, Rudy; Weckhuysen, Sarah; De Jonghe, Peter; Hirst, Jennifer; Afawi, Zaid; Barisic, Nina; Baulac, Stéphanie; Caglayan, Hande; Depienne, Christel; De Kovel, Carolien G.F.; Dimova, Petia; Guerrero-López, Rosa; Guerrini, Renzo; Hjalgrim, Helle; Hoffman-Zacharska, Dorota; Jahn, Johanna; Klein, Karl Martin; Koeleman, Bobby P.C.; Leguern, Eric; Lehesjoki, Anna-Elina; Lemke, Johannes; Lerche, Holger; Marini, Carla; Muhle, Hiltrud; Rosenow, Felix; Serratosa, Jose M.; Møller, Rikke S.; Stephani, Ulrich; Striano, Pasquale; Talvik, Tiina; Von Spiczak, Sarah; Weber, Yvonne; Zara, Federico

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-function variants (p.Gln46Profs*9 and p.Arg97*) was further investigated in a patient's fibroblast cell line. We show that the premature stop mutations in AP4S1 result in a reduction of all AP-4 subunits and loss of AP-4 complex assembly. Recruitment of the AP-4 accessory protein tepsin, to the membrane was also abolished. In retrospect, the clinical phenotype in the family is consistent with previous reports of the AP-4 deficiency syndrome. Our study reports the second family with mutations in AP4S1 and describes the first two patients with loss of AP4S1 and seizures. We further discuss seizure phenotypes in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies. PMID:25552650

  1. Did Eucalyptus contribute to environment degradation? Implications from a dispute on causes of severe drought in Yunnan and Guizhou, China

    OpenAIRE

    WenJun Zhang

    2012-01-01

    Various viewpoints were proposed to explain the causes of recent years' severe drought occurred in Yunnan and Guizhou, China. In general there are two parties of viewpoints, the Eucalyptus cause and climate change cause. I think Yunnan-Guizhou drought has been mainly caused by abnormal climate change. Eucalyptus was not significant in the formation of Yunnan-Guizhou drought. However, the forestation effect of Eucalyptus in China was not good. Environment quality and biodiversity in Eucalyptus...

  2. Habitat loss as the main cause of the slow recovery of fish faunas of regulated large rivers in Europe: The transversal floodplain gradient

    NARCIS (Netherlands)

    Aarts, B.G.W.; Van den Brink, F.W.B.; Nienhuis, P.H.

    2004-01-01

    In large European rivers the chemical water quality has improved markedly in recent decades, yet the recovery of the fish fauna is not proceeding accordingly. Important causes are the loss of habitats in the main river channels and their floodplains, and the diminished hydrological connectivity

  3. Drug-induced hair loss.

    Science.gov (United States)

    2016-05-01

    Hair loss can have major psychological consequences. It can be due to a wide variety of causes, including hormonal disorders, dietary factors, infections, inflammation, trauma, emotional factors, and cancer. Drugs can also induce hair loss, by interacting with the hair growth cycle. Drug-induced hair loss may be immediate or delayed, sudden or gradual, and diffuse or localised. It is usually reversible after drug discontinuation. The drugs most often implicated in hair loss are anticancer agents, interferon, azole antifungals, lithium, immunosuppressants, and many other drugs belonging to a variety of pharmacological classes.

  4. Effect of weight loss on the severity of psoriasis

    DEFF Research Database (Denmark)

    Jensen, P; Zachariae, Claus; Christensen, R

    2013-01-01

    Psoriasis is associated with adiposity and weight gain increases the severity of psoriasis and the risk of incident psoriasis. Therefore, we aimed to measure the effect of weight reduction on the severity of psoriasis in obese patients with psoriasis.......Psoriasis is associated with adiposity and weight gain increases the severity of psoriasis and the risk of incident psoriasis. Therefore, we aimed to measure the effect of weight reduction on the severity of psoriasis in obese patients with psoriasis....

  5. Duplication and Loss of Function of Genes Encoding RNA Polymerase III Subunit C4 Causes Hybrid Incompatibility in Rice

    Directory of Open Access Journals (Sweden)

    Giao Ngoc Nguyen

    2017-08-01

    Full Text Available Reproductive barriers are commonly observed in both animals and plants, in which they maintain species integrity and contribute to speciation. This report shows that a combination of loss-of-function alleles at two duplicated loci, DUPLICATED GAMETOPHYTIC STERILITY 1 (DGS1 on chromosome 4 and DGS2 on chromosome 7, causes pollen sterility in hybrid progeny derived from an interspecific cross between cultivated rice, Oryza sativa, and an Asian annual wild rice, O. nivara. Male gametes carrying the DGS1 allele from O. nivara (DGS1-nivaras and the DGS2 allele from O. sativa (DGS2-T65s were sterile, but female gametes carrying the same genotype were fertile. We isolated the causal gene, which encodes a protein homologous to DNA-dependent RNA polymerase (RNAP III subunit C4 (RPC4. RPC4 facilitates the transcription of 5S rRNAs and tRNAs. The loss-of-function alleles at DGS1-nivaras and DGS2-T65s were caused by weak or nonexpression of RPC4 and an absence of RPC4, respectively. Phylogenetic analysis demonstrated that gene duplication of RPC4 at DGS1 and DGS2 was a recent event that occurred after divergence of the ancestral population of Oryza from other Poaceae or during diversification of AA-genome species.

  6. Loss of renal function causes premature aging of the immune system.

    Science.gov (United States)

    Betjes, Michiel G H; Meijers, Ruud W J; Litjens, Nicolle H R

    2013-01-01

    Uremia-associated immune deficiency is a well-known complication of loss of renal function and contributes significantly to the overall mortality and morbidity of patients with end-stage renal disease. Chronic inflammation and increased oxidative stress are underlying the uremia-associated immune deficiency. In this review, the differential impact of uremia on the cellular immune system is summarized. Virtually all immune cells studied show a combination of an activated status and loss of function. However, uremia preferentially decreases the number and function of lymphoid cells while myeloid cells show normal and/or elevated cell numbers with increased production of inflammatory cytokines and reactive oxygen species. These particular changes are compatible with immunological aging, which is characterized by loss of thymic function, attrition of telomeres and an expanded memory T cell population. Similar to aging in healthy individuals, the proinflammatory and potential cardiotoxic subsets of CD28(null) T cells and CD16(+) monocytes are increased. Epigenetically changed hematopoietic stem cells may be involved in immunological aging as specific DNA regions become hypermethylated. Proinflammatory T cells and monocytes persist after kidney transplantation, which constitutes a persistent cardiovascular risk factor. Possible therapeutic options to reverse or halt uremia-associated immunological aging are discussed. Premature aging of the immune system is a dominant feature in patients with end-stage renal failure, which corresponds to immunological aging in elderly healthy individuals, which is characterized by preferential loss of cells belonging to the lymphoid cell lineage, inflammation and expansion of proinflammatory immune cells. © 2013 S. Karger AG, Basel.

  7. Sensorineural hearing loss in hemorrhagic dengue?

    Directory of Open Access Journals (Sweden)

    Bruna Natália Freire Ribeiro

    2015-01-01

    Discussion and conclusion: This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss?

  8. Bee moth (Galleria mellonella) allergic reactions are caused by several thermolabile antigens.

    Science.gov (United States)

    Villalta, D; Martelli, P; Mistrello, G; Roncarolo, D; Zanoni, D

    2004-09-01

    Exposure and contact with bee moth (Galleria mellonella) larvae (Gm) can cause an allergic reaction both in anglers and breeders. We described the case of an amateur fisherman who experienced an allergic reaction using Gm but not using heat-treated Gm (h-Gm) (mummies). The aim of this study was to demonstrate by immunoblotting and radioallergosorbent test (RAST)-inhibition experiments the loss of allergenic epitopes in h-Gm extracts. Galleria mellonella larvae and h-Gm were homogenized and extracted at 10% (w/v) in 0.5 M phosphate-buffered saline, pH 7.4 containing 0.5% NaN(3) for 16 h at 4 degrees C. Gm and h-Gm extracts were electrophoresed in a 10% polyacrylamide precast Nupage Bis-Tris gel at 180 mA for 1 h and the resolved proteins stained with 0.1% Coomassie brilliant blue and the molecular weight calculated. For the immunoblotting detection of allergenic components the resolved extracts were transferred onto a nitrocellulose membrane and incubated with the patient's serum. Bound specific-IgE was detected by peroxidase-conjugated anti-human IgE. RAST inhibition experiments were performed according to the Ceska method. The protein profile of Gm and h-Gm extracts resulted markedly different in number, intensity and the position of bands, indicating that heat-treatment modifies the chemical-physical characteristics of the protein contents. The Gm extract showed a strong-coloured band at 73 kDa and more than 20 components ranging from 12 to 133 kDa; h-Gm showed two main band at 77 and 38 kDa and about 15 faint bands between 20 and 133 kDa apparently without any correspondence to the bands present in the Gm extract. Immunoblotting with the patient's serum demonstrated several bands of reactivity with the Gm extract ranging from 20 to 100 kDa and no recognizable bands, but only a diffuse smear with h-Gm. When used in a RAST inhibition experiment the h-Gm extract demonstrated an inability to compete with the Gm one for the binding to patient's IgE serum. The h

  9. A Simple Method for Estimating the Economic Cost of Productivity Loss Due to Blindness and Moderate to Severe Visual Impairment.

    Science.gov (United States)

    Eckert, Kristen A; Carter, Marissa J; Lansingh, Van C; Wilson, David A; Furtado, João M; Frick, Kevin D; Resnikoff, Serge

    2015-01-01

    To estimate the annual loss of productivity from blindness and moderate to severe visual impairment (MSVI) using simple models (analogous to how a rapid assessment model relates to a comprehensive model) based on minimum wage (MW) and gross national income (GNI) per capita (US$, 2011). Cost of blindness (COB) was calculated for the age group ≥50 years in nine sample countries by assuming the loss of current MW and loss of GNI per capita. It was assumed that all individuals work until 65 years old and that half of visual impairment prevalent in the ≥50 years age group is prevalent in the 50-64 years age group. For cost of MSVI (COMSVI), individual wage and GNI loss of 30% was assumed. Results were compared with the values of the uncorrected refractive error (URE) model of productivity loss. COB (MW method) ranged from $0.1 billion in Honduras to $2.5 billion in the United States, and COMSVI ranged from $0.1 billion in Honduras to $5.3 billion in the US. COB (GNI method) ranged from $0.1 million in Honduras to $7.8 billion in the US, and COMSVI ranged from $0.1 billion in Honduras to $16.5 billion in the US. Most GNI method values were near equivalent to those of the URE model. Although most people with blindness and MSVI live in developing countries, the highest productivity losses are in high income countries. The global economy could improve if eye care were made more accessible and more affordable to all.

  10. An investigation of two dimensions of impulsivity as predictors of loss-of-control eating severity and frequency.

    Science.gov (United States)

    Espel, Hallie M; Muratore, Alexandra F; Lowe, Michael R

    2017-10-01

    Loss-of-control (LOC) eating episodes represent one form of dysregulated eating common to full- and sub-threshold eating disorders. Extensive evidence suggests that impulsivity, particularly in the context of negative affect and/or depression, may play an important etiological role in the development and maintenance of LOC eating. However, most prior studies have considered LOC eating as a dichotomous rather than dimensional construct, and few studies have considered the interaction of multiple dimensions of impulsivity while also accounting for the role of depressive symptoms. The present study examined the independent and interacting effects of two facets of impulsivity-response inhibition and negative urgency-on LOC eating episode severity and frequency among college women (N = 102). Depressive symptom severity was included as a covariate. Results indicated that greater negative urgency was associated with greater LOC severity; this effect was moderated by response inhibition, such that the effect of urgency was particularly pronounced for individuals with higher response inhibition capacity. Negative urgency was the only significant predictor of LOC frequency. Depression had no significant effect on either LOC severity or frequency (ps ≥ 0.16). Results support the importance of considering multiple facets of impulsivity in predicting LOC eating behavior, and further indicate that factors influencing subjective severity and frequency of LOC may be distinct. Copyright © 2017 Elsevier Ltd. All rights reserved.

  11. [Ocular myositis as a rare cause of vision loss].

    Science.gov (United States)

    Rollnik, J D; Requadt, H

    2017-04-01

    Ocular myositis is a rare disease characterized by painful diplopia but loss of vision rarely occurs. The article reviews the literature focusing on the differential diagnostics. We report the case of an 80-year-old women suffering from slowly progressive loss of vision in the left eye. Diplopia was only present at the beginning and there was only moderate pain. Computed tomography and magnetic resonance imaging revealed a swelling of the left medial, lateral and inferior rectus muscles of the orbit leading to compression of the optic nerve in the orbital cone. An intravenous prednisolone stoss therapy (1000 mg per day for 3 consecutive days) was initiated, followed by oral medication of 100 mg per day then tapering over 10 weeks. Vision improved and no relapses were observed. Physicians should be aware of this rare disease to ensure quick diagnosis and treatment of ocular myositis.

  12. Trends, Productivity Losses, and Associated Medical Conditions Among Toxoplasmosis Deaths in the United States, 2000–2010

    Science.gov (United States)

    Cummings, Patricia L.; Kuo, Tony; Javanbakht, Marjan; Sorvillo, Frank

    2014-01-01

    Few studies have quantified toxoplasmosis mortality, associated medical conditions, and productivity losses in the United States. We examined national multiple cause of death data and estimated productivity losses caused by toxoplasmosis during 2000–2010. A matched case–control analysis examined associations between comorbid medical conditions and toxoplasmosis deaths. In total, 789 toxoplasmosis deaths were identified during the 11-year study period. Blacks and Hispanics had the highest toxoplasmosis mortality compared with whites. Several medical conditions were associated with toxoplasmosis deaths, including human immunodeficiency virus (HIV), lymphoma, leukemia, and connective tissue disorders. The number of toxoplasmosis deaths with an HIV codiagnosis declined from 2000 to 2010; the numbers without such a codiagnosis remained static. Cumulative disease-related productivity losses for the 11-year period were nearly $815 million. Although toxoplasmosis mortality has declined in the last decade, the infection remains costly and is an important cause of preventable death among non-HIV subgroups. PMID:25200264

  13. Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation

    Directory of Open Access Journals (Sweden)

    Shuang Xia

    2017-01-01

    Full Text Available Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF and regional homogeneity (ReHo of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45 was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age.

  14. Injury Severity and Causes of Death from Operation Iraqi Freedom and Operation Enduring Freedom: 2003-2004 Versus 2006

    National Research Council Canada - National Science Library

    Kelly, Joseph F; Ritenour, Amber E; McLaughlin, Daniel F; Bagg, Karen A; Apodaca, Amy N; Mallak, Craig T; Pearse, Lisa; Lawnick, Mary M; Champion, Howard R; Wade, Charles E

    2008-01-01

    .... The authors hypothesized that the severity of wounds has increased over time. In this study, they examined cause of death looking for opportunities to improve clinical research and training for the battlefield...

  15. Biallelic loss-of-function variants in DOCK3 cause muscle hypotonia, ataxia, and intellectual disability.

    Science.gov (United States)

    Helbig, K L; Mroske, C; Moorthy, D; Sajan, S A; Velinov, M

    2017-10-01

    DOCK3 encodes the dedicator of cytokinesis 3 protein, a member of the DOCK180 family of proteins that are characterized by guanine-nucleotide exchange factor activity. DOCK3 is expressed exclusively in the central nervous system and plays an important role in axonal outgrowth and cytoskeleton reorganization. Dock3 knockout mice exhibit motor deficiencies with abnormal ataxic gait and impaired learning. We report 2 siblings with biallelic loss-of-function variants in DOCK3. Diagnostic whole-exome sequencing (WES) and chromosomal microarray were performed on a proband with severe developmental disability, hypotonia, and ataxic gait. Testing was also performed on the proband's similarly affected brother. A paternally inherited 458 kb deletion in chromosomal region 3p21.2 disrupting the DOCK3 gene was identified in both affected siblings. WES identified a nonsense variant c.382C>G (p.Gln128*) in the DOCK3 gene (NM_004947) on the maternal allele in both siblings. Common features in both affected individuals include severe developmental disability, ataxic gait, and severe hypotonia, which recapitulates the Dock3 knockout mouse phenotype. We show that complete DOCK3 deficiency in humans leads to developmental disability with significant hypotonia and gait ataxia, probably due to abnormal axonal development. © 2017 The Authors. Clinical Genetics published by John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Heterogeneity in phenotype of usher-congenital hyperinsulinism syndrome: hearing loss, retinitis pigmentosa, and hyperinsulinemic hypoglycemia ranging from severe to mild with conversion to diabetes.

    Science.gov (United States)

    Al Mutair, Angham N; Brusgaard, Klaus; Bin-Abbas, Bassam; Hussain, Khalid; Felimban, Naila; Al Shaikh, Adnan; Christesen, Henrik T

    2013-03-01

    To evaluate the phenotype of 15 children with congenital hyperinsulinism (CHI) and profound hearing loss, known as Homozygous 11p15-p14 Deletion syndrome (MIM #606528). Prospective clinical follow-up and genetic analysis by direct sequencing, multiplex ligation-dependent probe amplification, and microsatellite markers. Genetic testing identified the previous described homozygous deletion in 11p15, USH1C:c.(90+592)_ABCC8:c.(2694-528)del. Fourteen patients had severe CHI demanding near-total pancreatectomy. In one patient with mild, transient neonatal hypoglycemia and nonautoimmune diabetes at age 11 years, no additional mutations were found in HNF1A, HNF4A, GCK, INS, and INSR. Retinitis pigmentosa was found in two patients aged 9 and 13 years. No patients had enteropathy or renal tubular defects. Neuromotor development ranged from normal to severe delay with epilepsy. The phenotype of Homozygous 11p15-p14 Deletion syndrome, or Usher-CHI syndrome, includes any severity of neonatal-onset CHI and severe, sensorineural hearing loss. Retinitis pigmentosa and nonautoimmune diabetes may occur in adolescence.

  17. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    DEFF Research Database (Denmark)

    Hardies, Katia; May, Patrick; Djémié, Tania

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-...... in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies....

  18. Olive anthracnose: a yield- and oil quality-degrading disease caused by several species of Colletotrichum that differ in virulence, host preference and geographical distribution.

    Science.gov (United States)

    Talhinhas, Pedro; Loureiro, Andreia; Oliveira, Helena

    2018-03-08

    Olive anthracnose causes fruit rot leading to its drop or mummification, resulting in yield losses and the degradation of oil quality. The disease is caused by diverse species of Colletotrichum, mostly clustering in the C. acutatum species complex. Colletotrichum nymphaeae and C. godetiae are the prevalent species in the Northern Hemisphere, whereas C. acutatum sensu stricto is the most frequent species in the Southern Hemisphere, although it is recently and quickly emerging in the Northern Hemisphere. The disease has been reported from all continents, but it attains higher incidence and severity in the west of the Mediterranean Basin, where it is endemic in traditional orchards of susceptible cultivars. The pathogens are able to survive on vegetative organs. On the fruit surface, infections remain quiescent until fruit maturity, when typical anthracnose symptoms develop. Under severe epidemics, defoliation and death of branches can also occur. Pathogen species differ in virulence, although this depends on the cultivar. The selection of resistant cultivars depends strongly on pathogen diversity and environmental conditions, posing added difficulties to breeding efforts. Chemical disease control is normally achieved with copper-based fungicides, although this may be insufficient under highly favourable disease conditions and causes concern because of the presence of fungicide residues in the oil. In areas in which the incidence is high, farmers tend to anticipate harvest, with consequences in yield and oil characteristics. Olive production systems, harvest and post-harvest processing have experienced profound changes in recent years, namely new training systems using specific cultivars, new harvest and processing techniques and new organoleptic market requests. Changes are also occurring in both the geographical distribution of pathogen populations and the taxonomic framework. In addition, stricter rules concerning pesticide use are likely to have a strong impact

  19. Prevalence and causes of vision loss in Central and South Asia: 1990-2010.

    Science.gov (United States)

    Jonas, Jost B; George, Ronnie; Asokan, Rashima; Flaxman, Seth R; Keeffe, Jill; Leasher, Janet; Naidoo, Kovin; Pesudovs, Konrad; Price, Holly; Vijaya, Lingam; White, Richard A; Wong, Tien Y; Resnikoff, Serge; Taylor, Hugh R; Bourne, Rupert R A

    2014-05-01

    To examine the prevalence, patterns and trends of vision impairment and its causes from 1990 to 2010 in Central and South Asia. Based on the Global Burden of Diseases Study 2010 and ongoing literature searches, we examined prevalence and causes of moderate and severe vision impairment (MSVI; presenting visual acuity Central Asia, the estimated age-standardised prevalence of blindness decreased from 0.4% (95% CI 0.3% to 0.6%) to 0.2% (95% CI 0.2% to 0.3%) and of MSVI from 3.0% (95% CI 1.9% to 4.7%) to 1.9% (95% CI 1.2% to 3.2%), and in South Asia blindness decreased from 1.7% (95% CI 1.4% to 2.1%) to 1.1% (95% CI 0.9% to 1.3%) and MSVI from 8.9% (95% CI 6.9% to 10.9%) to 6.4% (95% CI 5.2% to 8.2%). In 2010, 135 000 (95% CI 99,000 to 194,000) people were blind in Central Asia and 10,600,000 (95% CI 8,397,000 to 12,500,000) people in South Asia. MSVI was present in 1,178,000 (95% CI 772,000 to 2,243,000) people in the Central Asia, and in 71,600,000 (95% CI 57,600,000 to 92,600,000) people in South Asia. Women were generally more often affected than men. The leading causes of blindness (cataract) and MSVI (undercorrected refractive error) did not change from 1990 to 2010. The prevalence of blindness and MSVI in South Asia is still three times higher than in Central Asia and globally, with women generally more often affected than women. In both regions, cataract and undercorrected refractive error were major causes of blindness and MSVI.

  20. Coping proactively with economic stress: career adaptability in the face of job insecurity, job loss, unemployment, and underemployment

    NARCIS (Netherlands)

    Klehe, U.-C.; Zikic, J.; van Vianen, A.E.M.; Koen, J.; Buyken, M.; Perrewé, P.L; Halbesleben, J.R.B.; Rosen, C.C.

    2012-01-01

    Economic stressors such as job insecurity, job loss, unemployment, and underemployment cause severe difficulties for the workers affected, their families, organizations, and societies overall. Consequently, most past research has taken a thoroughly negative perspective on economic stress, addressing

  1. Restoration of Motor Defects Caused by Loss of Drosophila TDP-43 by Expression of the Voltage-Gated Calcium Channel, Cacophony, in Central Neurons.

    Science.gov (United States)

    Lembke, Kayly M; Scudder, Charles; Morton, David B

    2017-09-27

    Defects in the RNA-binding protein, TDP-43, are known to cause a variety of neurodegenerative diseases, including amyotrophic lateral sclerosis and frontotemporal lobar dementia. A variety of experimental systems have shown that neurons are sensitive to TDP-43 expression levels, yet the specific functional defects resulting from TDP-43 dysregulation have not been well described. Using the Drosophila TDP-43 ortholog TBPH, we previously showed that TBPH-null animals display locomotion defects as third instar larvae. Furthermore, loss of TBPH caused a reduction in cacophony , a Type II voltage-gated calcium channel, expression and that genetically restoring cacophony in motor neurons in TBPH mutant animals was sufficient to rescue the locomotion defects. In the present study, we examined the relative contributions of neuromuscular junction physiology and the motor program to the locomotion defects and identified subsets of neurons that require cacophony expression to rescue the defects. At the neuromuscular junction, we showed mEPP amplitudes and frequency require TBPH. Cacophony expression in motor neurons rescued mEPP frequency but not mEPP amplitude. We also showed that TBPH mutants displayed reduced motor neuron bursting and coordination during crawling and restoring cacophony selectively in two pairs of cells located in the brain, the AVM001b/2b neurons, also rescued the locomotion and motor defects, but not the defects in neuromuscular junction physiology. These results suggest that the behavioral defects associated with loss of TBPH throughout the nervous system can be associated with defects in a small number of genes in a limited number of central neurons, rather than peripheral defects. SIGNIFICANCE STATEMENT TDP-43 dysfunction is a common feature in neurodegenerative diseases, including amyotrophic lateral sclerosis, frontotemporal lobar dementia, and Alzheimer's disease. Loss- and gain-of-function models have shown that neurons are sensitive to TDP-43

  2. Prevalence and causes of severe visual impairment and blindness among children in the lorestan province of iran, using the key informant method.

    Science.gov (United States)

    Razavi, Hessom; Kuper, Hannah; Rezvan, Farhad; Amelie, Khatere; Mahboobi-Pur, Hassan; Oladi, Mohammad Reza; Muhit, Mohammad; Hashemi, Hassan

    2010-03-01

    To estimate the prevalence and causes of severe visual impairment and blindness among children in Lorestan province of Iran, and to assess the feasibility of the Key Informant Method in this setting. Potential cases were identified using the Key Informant Method, in 3 counties of Lorestan province during June through August 2008, and referred for examination. Causes of severe visual impairment/blindness were determined and categorized using standard World Health Organization methods. Of 123 children referred for examination, 27 children were confirmed to have severe visual impairment/blindness or blindness. The median age was11 years (interquartile range 6-13), and 59% were girls. After adjusting for non-attenders, the estimated prevalence of severe visual impairment/blindness was 0.04% (0.03-0.05). The main site of abnormality was retina (44%), followed by disorders of the whole eye (33%). The majority of causes had a hereditary etiology (70%), which was associated with a family history of blindness (P = 0.002). Potentially avoidable causes of severe visual impairment/blindness were found in 14 children (52%). Almost all children with severe visual impairment/blindness had a history of parental consanguinity (93%). Our findings suggest a moderate prevalence of childhood blindness in the Lorestan province of Iran, a high proportion of which may be avoidable, given improved access to ophthalmic and genetic counselling services in rural areas. The Key Informant Method is feasible in Iran; future research is discussed.

  3. Incidence, causes, severity and treatment of throat discomfort: a four-region online questionnaire survey.

    Science.gov (United States)

    Addey, Dilys; Shephard, Adrian

    2012-08-10

    Acute sore throat is commonly associated with viral infections. Consumers typically rely on over-the-counter treatments and other remedies to treat symptoms; however, limited information is available regarding consumer perceptions of sore throat or treatment needs. The aim of this study was to investigate perceptions of throat discomfort and how these influence attitudes and consumer behaviour with regard to treatment. Online consumer surveys were completed by participants invited by email between 2003 and 2004 in four markets: the UK, France, Poland, and Malaysia. The questionnaire consisted of 24 questions that covered key issues surrounding throat discomfort including incidence in the past 12 months, causes, severity, effects on functionality and quality of life, actions taken to relieve throat discomfort, the efficacy of these approaches and the reasons behind using specific products. In total, 6465 men and women aged ≥18 years were surveyed, identifying 3514 participants who had suffered throat discomfort/irritation in the past 12 months (response rate of 54%). These participants completed the full survey. The breakdown of throat discomfort sufferers was: UK, 912; France, 899; Poland, 871; Malaysia, 832. A high proportion of respondents experienced one or more instances of throat discomfort in the previous 12 months, with an overall incidence of 54%. Infections including the common cold/influenza and other bacteria/viruses were commonly perceived causes of throat discomfort (72% and 46%, respectively). Physical and environmental factors were also perceived to be causative, including airborne pollution (28%), smoking (23%), and air conditioning (31%). Symptoms perceived to be caused by an infection were associated with a higher degree of suffering (mean degree of suffering for bacteria/virus and common cold/influenza; 3.4 and 3.0, respectively). Medicinal products were used for all perceived causes, but more commonly for sore throats thought to be caused by

  4. Incidence, causes, severity and treatment of throat discomfort: a four-region online questionnaire survey

    Directory of Open Access Journals (Sweden)

    Addey Dilys

    2012-08-01

    Full Text Available Abstract Background Acute sore throat is commonly associated with viral infections. Consumers typically rely on over-the-counter treatments and other remedies to treat symptoms; however, limited information is available regarding consumer perceptions of sore throat or treatment needs. The aim of this study was to investigate perceptions of throat discomfort and how these influence attitudes and consumer behaviour with regard to treatment. Methods Online consumer surveys were completed by participants invited by email between 2003 and 2004 in four markets: the UK, France, Poland, and Malaysia. The questionnaire consisted of 24 questions that covered key issues surrounding throat discomfort including incidence in the past 12 months, causes, severity, effects on functionality and quality of life, actions taken to relieve throat discomfort, the efficacy of these approaches and the reasons behind using specific products. Results In total, 6465 men and women aged ≥18 years were surveyed, identifying 3514 participants who had suffered throat discomfort/irritation in the past 12 months (response rate of 54%. These participants completed the full survey. The breakdown of throat discomfort sufferers was: UK, 912; France, 899; Poland, 871; Malaysia, 832. A high proportion of respondents experienced one or more instances of throat discomfort in the previous 12 months, with an overall incidence of 54%. Infections including the common cold/influenza and other bacteria/viruses were commonly perceived causes of throat discomfort (72% and 46%, respectively. Physical and environmental factors were also perceived to be causative, including airborne pollution (28%, smoking (23%, and air conditioning (31%. Symptoms perceived to be caused by an infection were associated with a higher degree of suffering (mean degree of suffering for bacteria/virus and common cold/influenza; 3.4 and 3.0, respectively. Medicinal products were used for all perceived causes, but more

  5. Chronic central serous choroidopathy with severe visual loss in hyperopic microphthalmic identical twins

    Directory of Open Access Journals (Sweden)

    Fabry, Annelies

    2011-01-01

    Full Text Available Objective: To report chronic central serous choroidopathy with severe visual loss in hyperopic microphthalmic identical twins. Methods: The index patient was first examined in 1994, at age 31, and has been followed up closely for 17 years. He had repeated fluorescein and indocyanine green angiograms, OCT, ultrasound biometry, and recently also autofluorescence and EDI OCT. His twin brother was first examined in 2010, at age 47, and had a similar extensive exploration. Results: The index patient had a mean refractive error of +6 D OU and VA was 20/32++ in the RE and 20/200 in the LE in 1994. Vision slowly went down to 20/800+ in the RE and 20/600 in the LE in 2011. His twin brother has a mean refractive error of +6 D OU and VA 20/400 OU. Both have a short axial length of the eye, a thick choroid with dilated vessels, and central serous choroidopathy with cystic degeneration of the macula and retina in the posterior pole. Conclusions: We add to the reported complications of microphthalmos, chronic central serous choroidopathy.

  6. A homozygous missense mutation in human KLOTHO causes severe tumoral calcinosis

    Science.gov (United States)

    Ichikawa, Shoji; Imel, Erik A.; Kreiter, Mary L.; Yu, Xijie; Mackenzie, Donald S.; Sorenson, Andrea H.; Goetz, Regina; Mohammadi, Moosa; White, Kenneth E.; Econs, Michael J.

    2007-01-01

    Familial tumoral calcinosis is characterized by ectopic calcifications and hyperphosphatemia due to inactivating mutations in FGF23 or UDP-N-acetyl-α-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 3 (GALNT3). Herein we report a homozygous missense mutation (H193R) in the KLOTHO (KL) gene of a 13-year-old girl who presented with severe tumoral calcinosis with dural and carotid artery calcifications. This patient exhibited defects in mineral ion homeostasis with marked hyperphosphatemia and hypercalcemia as well as elevated serum levels of parathyroid hormone and FGF23. Mapping of H193R mutation onto the crystal structure of myrosinase, a plant homolog of KL, revealed that this histidine residue was at the base of the deep catalytic cleft and mutation of this histidine to arginine should destabilize the putative glycosidase domain (KL1) of KL, thereby attenuating production of membrane-bound and secreted KL. Indeed, compared with wild-type KL, expression and secretion of H193R KL were markedly reduced in vitro, resulting in diminished ability of FGF23 to signal via its cognate FGF receptors. Taken together, our findings provide what we believe to be the first evidence that loss-of-function mutations in human KL impair FGF23 bioactivity, underscoring the essential role of KL in FGF23-mediated phosphate and vitamin D homeostasis in humans. PMID:17710231

  7. Motor unit number estimation in the quantitative assessment of severity and progression of motor unit loss in Hirayama disease.

    Science.gov (United States)

    Zheng, Chaojun; Zhu, Yu; Zhu, Dongqing; Lu, Feizhou; Xia, Xinlei; Jiang, Jianyuan; Ma, Xiaosheng

    2017-06-01

    To investigate motor unit number estimation (MUNE) as a method to quantitatively evaluate severity and progression of motor unit loss in Hirayama disease (HD). Multipoint incremental MUNE was performed bilaterally on both abductor digiti minimi and abductor pollicis brevis muscles in 46 patients with HD and 32 controls, along with handgrip strength examination. MUNE was re-evaluated approximately 1year after initial examination in 17 patients with HD. The MUNE values were significantly lower in all the tested muscles in the HD group (Pdisease duration (Pmotor unit loss in patients with HD within approximately 1year (P4years. A reduction in the functioning motor units was found in patients with HD compared with that in controls, even in the early asymptomatic stages. Moreover, the motor unit loss in HD progresses gradually as the disease advances. These results have provided evidence for the application of MUNE in estimating the reduction of motor unit in HD and confirming the validity of MUNE for tracking the progression of HD in a clinical setting. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

  8. Characteristics of possible beam losses in superconducting cyclotron

    International Nuclear Information System (INIS)

    Pradhan, J.; Paul, Santanu; Debnath, Jayanta; Dutta, Atanu; Bhunia, Uttam; Naser, Md. Zamal Abdul; Singh, Vinay; Agrawal, Ankur; Dey, Malay Kanti

    2015-01-01

    In a compact superconducting cyclotron large coherent oscillation and off-centering of the beam may cause large amount of beam loss. The off-centered beam may hit the beam chamber wall prohibiting extraction of the beam. Or it may hit the RF liner surfaces due to vertical blow-up across various resonances during acceleration. The vertical shift of beam caused by the mis-alignment gradually moves the beam out of geometrical median plane eventually leading to internal beam losses. The loss of isochronisms results the reduction of beam intensity depending on the particle phase history. Small field perturbations generated by trim coils have been used to identify the beam loss mechanisms in the superconducting cyclotron at out centre. Besides, the beam loss due to interaction of accelerating ions with residual gases is also discussed. The beam profile obtained from differential and three finger probes gives a clear insight of the loss-mechanism. The paper describes different beam losses observed in the cyclotron with corresponding beam profiles under different field perturbations, Special emphasis is given on characteristics features of beam-current profile to identify the cause of beam loss. (author)

  9. Cellular mechanisms of cyclophosphamide-induced taste loss in mice.

    Science.gov (United States)

    Mukherjee, Nabanita; Pal Choudhuri, Shreoshi; Delay, Rona J; Delay, Eugene R

    2017-01-01

    Many commonly prescribed chemotherapy drugs such as cyclophosphamide (CYP) have adverse side effects including disruptions in taste which can result in loss of appetite, malnutrition, poorer recovery and reduced quality of life. Previous studies in mice found evidence that CYP has a two-phase disturbance in taste behavior: a disturbance immediately following drug administration and a second which emerges several days later. In this study, we examined the processes by which CYP disturbs the taste system by examining the effects of the drug on taste buds and cells responsible for taste cell renewal using immunohistochemical assays. Data reported here suggest CYP has direct cytotoxic effects on lingual epithelium immediately following administration, causing an early loss of taste sensory cells. Types II and III cells in fungiform taste buds appear to be more susceptible to this effect than circumvallate cells. In addition, CYP disrupts the population of rapidly dividing cells in the basal layer of taste epithelium responsible for taste cell renewal, manifesting a disturbance days later. The loss of these cells temporarily retards the system's capacity to replace Type II and Type III taste sensory cells that survived the cytotoxic effects of CYP and died at the end of their natural lifespan. The timing of an immediate, direct loss of taste cells and a delayed, indirect loss without replacement of taste sensory cells are broadly congruent with previously published behavioral data reporting two periods of elevated detection thresholds for umami and sucrose stimuli. These findings suggest that chemotherapeutic disturbances in the peripheral mechanisms of the taste system may cause dietary challenges at a time when the cancer patient has significant need for well balanced, high energy nutritional intake.

  10. Early determinants of long-term T-cell reconstitution after hematopoietic stem cell transplantation for severe combined immunodeficiency

    NARCIS (Netherlands)

    Borghans, José A.; Bredius, Robbert G.; Hazenberg, Mette D.; Roelofs, Helene; Jol-van der Zijde, Els C.; Heidt, Jeroen; Otto, Sigrid A.; Kuijpers, Taco W.; Fibbe, Willem E.; Vossen, Jaak M.; Miedema, Frank; van Tol, Maarten J.

    2006-01-01

    The immune system of patients with severe combined immunodeficiency (SCID) reconstitutes to a large extent during the first years after hematopoietic stem cell transplantation (HSCT). It was suggested, however, that accelerated loss of thymus output may cause impaired immune function at the long

  11. Myopic loss aversion revisited

    OpenAIRE

    Blavatskyy, Pavlo; Pogrebna, Ganna

    2009-01-01

    In this paper we reexamine several experimental papers on myopic loss aversion by analyzing individual rather than aggregate choice patterns. We find that the behavior of the majority of subjects is inconsistent with the hypothesis of myopic loss aversion.

  12. Severe hemolytic disease of fetus and newborn caused by red blood cell antibodies undetected at first-trimester screening (CME).

    Science.gov (United States)

    Dajak, Slavica; Stefanović, Vedran; Capkun, Vesna

    2011-07-01

    The objective was to determine clinical consequences of anti-D and non-D antibodies undetected at first-trimester screening for infant or fetus. This retrospective cohort study included all pregnant women with red blood cell (RBC) antibodies who were tested between 1993 and 2008. Data were obtained from the forms for tracking immunization at the transfusion department. Each form was analyzed for three data sets: the order of screening at which the antibodies were detected (initial or repeated screening), the order of pregnancy (first pregnancy or higher), and whether the antibodies caused severe hemolytic disease of fetus and newborn (HDFN). In D- women, anti-D was detected in 1.3% of cases. The anti-D was undetected in 72 (37%) cases on the first-trimester screening, of which eight cases were complicated by severe HDFN. In this group, three patients were primigravidae. An overall non-D incidence of 0.2% was observed. In 16 cases, non-D were undetected on the first-trimester screening (10 anti-c, two anti-E, two anti-C, one anti-S, and one case of anti-Rh17). Non-D antibodies undetected on initial screening caused 11 cases of severe HDFN (27% of all severe non-D HDFN). Ten of them were in multiparous women. Seven of 11 cases with severe HDFN that were missed were caused by anti-c. The third-trimester screening may detect RBC antibodies that were not present or detected on the first-trimester screening. Such screening may be especially relevant in D+ multiparous women due to the risk of HDFN. © 2010 American Association of Blood Banks.

  13. Systemic Steroid Application Caused Sudden Death of a Patient with Sudden Deafness

    Directory of Open Access Journals (Sweden)

    Eriko Ogino-Nishimura

    2013-01-01

    Full Text Available A 63-year-old man, who was diagnosed with sudden sensorineural hearing loss (SSHL, showed severe hypertension 10 hours after prednisolone administration. Subsequently, the patient suddenly died due to pulmonary edema. The autopsy indicated a pheochromocytoma in the right adrenal gland, and the cause of death was determined to be a pheochromocytoma crisis induced by systemic administration of prednisolone. Pheochromocytoma crisis is a life-threatening condition and can result from the use of corticosteroids. Physicians should consider the risk of a pheochromocytoma crisis due to systemic corticosteroids in the treatment of patients with sudden sensorineural hearing loss.

  14. Loss-of-Function of Gli3 in Mice Causes Abnormal Frontal Bone Morphology and Premature Synostosis of the Interfrontal Suture

    OpenAIRE

    Veistinen, Lotta; Takatalo, Maarit; Tanimoto, Yukiho; Kesper, Dörthe A.; Vortkamp, Andrea; Rice, David P. C.

    2012-01-01

    Greig cephalopolysyndactyly syndrome (GCPS) is an autosomal dominant disorder with polydactyly and syndactyly of the limbs and a broad spectrum of craniofacial abnormalities. Craniosynostosis of the metopic suture (interfrontal suture in mice) is an important but rare feature associated with GCPS. GCPS is caused by mutations in the transcription factor GLI3, which regulates Hedgehog signaling. The Gli3 loss-of-function (Gli3Xt-J/Xt-J) mouse largely phenocopies the human syndrome with the mice...

  15. Insurability and mitigation of flood losses in private households in Germany.

    Science.gov (United States)

    Thieken, Annegret H; Petrow, Theresia; Kreibich, Heidi; Merz, Bruno

    2006-04-01

    In Germany, flood insurance is provided by private insurers as a supplement to building or contents insurance. This article presents the results of a survey of insurance companies with regard to eligibility conditions for flood insurance changes after August 2002, when a severe flood caused 1.8 billion euro of insured losses in the Elbe and the Danube catchment areas, and the general role of insurance in flood risk management in Germany. Besides insurance coverage, governmental funding and public donations played an important role in loss compensation after the August 2002 flood. Therefore, this article also analyzes flood loss compensation, risk awareness, and mitigation in insured and uninsured private households. Insured households received loss compensation earlier. They also showed slightly better risk awareness and mitigation strategies. Appropriate incentives should be combined with flood insurance in order to strengthen future private flood loss mitigation. However, there is some evidence that the surveyed insurance companies do little to encourage precautionary measures. To overcome this problem, flood hazards and mitigation strategies should be better communicated to both insurance companies and property owners.

  16. Exposure to acute severe hypoxia leads to increased urea loss and disruptions in acid-base and ionoregulatory balance in dogfish sharks (Squalus acanthias).

    Science.gov (United States)

    Zimmer, Alex M; Wood, Chris M

    2014-01-01

    The effects of acute moderate (20% air O2 saturation; 6-h exposure) and severe (5% air O2 saturation; 4-h exposure) hypoxia on N-waste, acid-base, and ion balance in dogfish sharks (Squalus acanthias suckleyi) were evaluated. We predicted that the synthesis and/or retention of urea, which are active processes, would be inhibited by hypoxia. Exposure to moderate hypoxia had negligible effects on N-waste fluxes or systemic physiology, except for a modest rise in plasma lactate. Exposure to severe hypoxia led to a significant increase in urea excretion (Jurea), while plasma, liver, and muscle urea concentrations were unchanged, suggesting a loss of urea retention. Ammonia excretion (Jamm) was elevated during normoxic recovery. Moreover, severe hypoxia led to disruptions in acid-base balance, indicated by a large increase in plasma [lactate] and substantial decreases in arterial pHa and plasma [Formula: see text], as well as loss of ionic homeostasis, indicated by increases in plasma [Mg(2+)], [Ca(2+)], and [Na(+)]. We suggest that severe hypoxia in dogfish sharks leads to a reduction in active gill homeostatic processes, such as urea retention, acid-base regulation and ionoregulation, and/or an osmoregulatory compromise due to increased functional gill surface area. Overall, the results provide a comprehensive picture of the physiological responses to a severe degree of hypoxia in an ancient fish species.

  17. Severe Heterotopic Ossification following Total Knee Replacement

    Directory of Open Access Journals (Sweden)

    Alexander L. Dodds

    2014-01-01

    Full Text Available Although the incidence of minor heterotopic ossification is probably higher than what is usually expected, severe heterotopic ossification (HO is an extremely rare event following total knee replacement surgery. We present the case of a 66-year-old woman who initially had achieved an excellent range of motion following bilateral uncemented rotating platform total knee replacement, before presenting with pain and loss of range of motion at 2 months after surgery. Severe HO was diagnosed on X-rays. Treatment consisted of nonoperative measures only, including physiotherapy with hydrotherapy and anti-inflammatories. She eventually regained her range of motion when seen at 8 months after operation. This case illustrates that nonoperative treatment without the use of radiotherapy or surgery can be used to safely resolve stiffness caused by HO after total knee replacement.

  18. Loss-of-coolant and loss-of-flow accident in the ITER-EDA first wall/blanket cooling system

    Energy Technology Data Exchange (ETDEWEB)

    Komen, E.M.J.; Koning, H.

    1995-05-01

    This report presents the analysis of the transient thermal-hydraulic system behaviour inside the first wall/blanket cooling system and the resulting temperature response inside the first wall and blanket of the ITER-EDA (International Thermonuclear Experimental Reactor - Engineering Design Activities) reactor design during a: - Loss-of-coolant accident caused by a reputure of the pump suction pipe; - loss-of-flow accident caused by a trip of the recirculation pump. (orig.).

  19. Loss-of-coolant and loss-of-flow accident in the ITER-EDA first wall/blanket cooling system

    International Nuclear Information System (INIS)

    Komen, E.M.J.; Koning, H.

    1995-05-01

    This report presents the analysis of the transient thermal-hydraulic system behaviour inside the first wall/blanket cooling system and the resulting temperature response inside the first wall and blanket of the ITER-EDA (International Thermonuclear Experimental Reactor - Engineering Design Activities) reactor design during a: - Loss-of-coolant accident caused by a reputure of the pump suction pipe; - loss-of-flow accident caused by a trip of the recirculation pump. (orig.)

  20. Are open-fit hearing aids a possible alternative to bone-anchored hearing devices in patients with mild to severe hearing loss? A preliminary trial

    Directory of Open Access Journals (Sweden)

    Amberley V. Ostevik

    2013-08-01

    Full Text Available Open-fit hearing aids (OFHAs may be of benefit for some individuals with chronic outer and middle ear conditions for which boneanchored hearing devices (BAHDs are normally recommended. The purpose of this study was to compare performance between OFHAs and BAHDs. A Starkey Destiny 800 OFHA was fit on eight adult BAHD users and speech perception measures in quiet and in background noise were compared under two different test conditions: i BAHD only and ii OFHA only. Equivalent outcome measure performance between these two conditions suggests that the OFHA was able to provide sufficient amplification for mild to moderate degrees of hearing loss (pure-tone averages (PTAs less than 47 dB HL. The improved speech perception performances and increased loudness ratings observed for several of the participants with moderately-severe to severe degrees of hearing loss (PTAs of 47 dB HL or greater in the BAHD only condition suggest that the OFHA did not provide sufficient amplification for these individuals. Therefore, OFHAs may be a successful alternative to the BAHD for individuals with no more than a moderate conductive hearing loss who are unable or unwilling to undergo implant surgery or unable to wear conventional hearing aids due to allergies, irritation, or chronic infection associated with the ear being blocked with a shell or earmold.

  1. Bilateral idiopathic optic perineuritis with severe vision loss: A case report

    Directory of Open Access Journals (Sweden)

    Wee-Min Teh

    2015-07-01

    Full Text Available Optic perineuritis is an orbital inflammatory disorder that is either idiopathic or secondary to other conditions such as infection or systemic inflammatory disorders. This condition is very similar to demyelinating optic neuritis, but certain features of the history and magnetic resonance imaging findings are characteristic for and aid in the diagnosis of optic perineuritis. Vision loss varies greatly, from minimal clouding of vision up to only light perception. We report a case of a 44-year-old female with idiopathic bilateral optic perineuritis with vision loss of up to no light perception in both eyes. Radio imaging studies were typical of optic perineuritis and she was started on systemic corticosteroids. She responded very well to steroid therapy and achieved nearly complete visual recovery. There had been no relapse despite cessation of therapy.

  2. Preservation of beam loss induced quenches, beam lifetime and beam loss measurements with the HERA-p beam-loss-monitor system

    International Nuclear Information System (INIS)

    Wittenburg, K.

    1994-01-01

    The beam-loss-monitors (BLMs) in the HERA-Proton-ring (HERAp) must fulfil the following requirements: They have to measure losses sensitive and fast enough to prevent the superconducting magnets from beam loss induced quenching; the dynamic range of the monitors must exceed several decades in order to measure losses during beam lifetimes of hundreds of hours as well as the much stronger losses that may quench superconducting magnets; they have to be insensitive to the synchrotron radiation of the adjacent electron-ring (HERAe); and their radiation hardness must allow a monitor-lifetime of a few years of HERA operation. These requirements are well satisfied by the HERAp-BLM-System. (orig.)

  3. Primary cellular meningeal defects cause neocortical dysplasia and dyslamination

    Science.gov (United States)

    Hecht, Jonathan H.; Siegenthaler, Julie A.; Patterson, Katelin P.; Pleasure, Samuel J.

    2010-01-01

    Objective Cortical malformations are important causes of neurological morbidity, but in many cases their etiology is poorly understood. Mice with Foxc1 mutations have cellular defects in meningeal development. We use hypomorphic and null alleles of Foxc1 to study the effect of meningeal defects on neocortical organization. Methods Embryos with loss of Foxc1 activity were generated using the hypomorphic Foxc1hith allele and the null Foxc1lacZ allele. Immunohistologic analysis was used to assess cerebral basement membrane integrity, marginal zone heterotopia formation, neuronal overmigration, meningeal defects, and changes in basement membrane composition. Dysplasia severity was quantified using two measures. Results Cortical dysplasia resembling cobblestone cortex, with basement membrane breakdown and lamination defects, is seen in Foxc1 mutants. As Foxc1 activity was reduced, abnormalities in basement membrane integrity, heterotopia formation, neuronal overmigration, and meningeal development appeared earlier in gestation and were more severe. Surprisingly, the basement membrane appeared intact at early stages of development in the face of severe deficits in meningeal development. Prominent defects in basement membrane integrity appeared as development proceeded. Molecular analysis of basement membrane laminin subunits demonstrated that loss of the meninges led to changes in basement membrane composition. Interpretation Cortical dysplasia can be caused by cellular defects in the meninges. The meninges are not required for basement membrane establishment but are needed for remodeling as the brain expands. Specific changes in basement membrane composition may contribute to subsequent breakdown. Our study raises the possibility that primary meningeal defects may cortical dysplasia in some cases. PMID:20976766

  4. Genetic Causes of Rickets

    Science.gov (United States)

    Acar, Sezer; Demir, Korcan; Shi, Yufei

    2017-01-01

    Rickets is a metabolic bone disease that develops as a result of inadequate mineralization of growing bone due to disruption of calcium, phosphorus and/or vitamin D metabolism. Nutritional rickets remains a significant child health problem in developing countries. In addition, several rare genetic causes of rickets have also been described, which can be divided into two groups. The first group consists of genetic disorders of vitamin D biosynthesis and action, such as vitamin D-dependent rickets type 1A (VDDR1A), vitamin D-dependent rickets type 1B (VDDR1B), vitamin D-dependent rickets type 2A (VDDR2A), and vitamin D-dependent rickets type 2B (VDDR2B). The second group involves genetic disorders of excessive renal phosphate loss (hereditary hypophosphatemic rickets) due to impairment in renal tubular phosphate reabsorption as a result of FGF23-related or FGF23-independent causes. In this review, we focus on clinical, laboratory and genetic characteristics of various types of hereditary rickets as well as differential diagnosis and treatment approaches. PMID:29280738

  5. Does occupational noise cause asymmetric hearing loss?

    Science.gov (United States)

    Dobie, Robert A

    2014-01-01

    Determine whether occupational noise exposure increases audiometric asymmetry. Audiograms were performed on 2044 men from the Occupational Noise and Hearing Survey, representing four groups based on preliminary screening (for previous noise exposure, otologic history, and otoscopy) and current occupational noise exposure. The effects of current noise exposure on audiometric asymmetry were tested using ANCOVA, with binaural average thresholds as covariates. There were no significant differences in asymmetry attributable to current occupational noise exposure. Occupational noise exposure does not usually cause or exacerbate audiometric asymmetry.

  6. Disease severity, self-reported experience of workplace discrimination and employment loss during the course of chronic HIV disease: differences according to gender and education.

    Science.gov (United States)

    Dray-Spira, R; Gueguen, A; Lert, F

    2008-02-01

    Evidence for the existence of a harmful effect of chronic disease on employment status has been provided. Although this effect of chronic illness on employment has been reported to be higher among the groups with the lowest position on the labour market, the mechanisms of such inequalities are poorly understood. The present study aimed at investigating social inequalities in the chances of maintaining employment during the course of HIV infection and at examining the correlates of such inequalities. The authors used data from a national representative sample of people living with HIV in France (ANRS-EN12-VESPA survey). Retrospective information on social trajectory and disease characteristics from the time of HIV diagnosis was available. The risk of employment loss associated with indicators of disease severity and HIV-related workplace discrimination was computed over time since HIV diagnosis according to sociodemographic and occupational factors, using Cox proportional hazards models. Among the 478 working-age participants diagnosed as being HIV-infected in the era of multitherapies and employed at the time of HIV diagnosis, 149 experienced employment loss. After adjusting for sociodemographic and occupational factors, disease severity and self-reported HIV-related discrimination at work were significantly associated with the risk of employment loss in a socially-differentiated manner: advancement in HIV disease was associated with an increased risk of employment loss among women (HR 4.45, 95% CI 2.10 to 9.43) but not among men; self-reported experience of HIV-related discrimination at work was associated with an increased risk of employment loss among individuals with a primary/secondary educational level (HR 8.85, 95% CI 3.68 to 21.30) but not among those more educated. Chronic HIV disease affects the chances of maintaining employment in a socially-differentiated manner, resulting in increasing inequalities regarding workforce participation. Disease severity

  7. 27 CFR 24.236 - Losses of spirits.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 1 2010-04-01 2010-04-01 false Losses of spirits. 24.236... OF THE TREASURY LIQUORS WINE Spirits § 24.236 Losses of spirits. Losses by theft or any other cause... the losses are discovered. A physical inventory of the spirits storage tanks will be taken at the...

  8. Etiology of hearing loss in children.

    Directory of Open Access Journals (Sweden)

    José Ignacio BENITO-OREJAS

    2017-06-01

    Full Text Available Introduction and objective: The neonatal hearing loss is one of the most common disabilities, with lifelong implications for the child and his family. The implementation of the universal newborn hearing screening and the development in molecular medicine, genetic and integrative neuroscience has perfected the early diagnosis of the hearing loss children and consequently its intervention. With this work, we want to clarify the audiological aspects and causes of the permanent hearing loss diagnosed during the past 20 years. Method: We reviewed retrospectively the records of the children diagnosed with less than 3 years of age of permanent hearing loss, during the period 1994-2015, in a tertiary center. Evaluate the time of home, laterality, type and degree of hearing loss. Depending on the background, genetic testing and other complementary explorations, we present the results of our diagnostic study. Results: In the study-population (n = 183, 71% of the permanent hearing loss > 30 dB HL was diagnosed at birth (congenital. Its main features are the bilaterality (81%, the predominance sensorineural (85% and the grade profound (42% or moderate (30%, more prevalent in the unilateral forms. About the etiologic diagnosis, a 47% of the cases are of origin genetic (29% of which are syndromic, a 25% of cause environmental and a 28% unknown. Discussion: Our results are consistent for the generally accepted distribution of causes, but there are discrepancies in the literature. Despite the different tests used, we had to infer the etiology in 62% of children with hearing loss, finally unknown by 28%. Conclusions: We consider fundamental the monitoring for a consensus standardized etiological protocol that orient in the diagnostic process of hearing loss in children.

  9. High honey bee losses in the winter of 2016-2017 in Timiș county, Romania

    Directory of Open Access Journals (Sweden)

    Daniela Marina Mot

    2017-05-01

    Full Text Available Beginning with November 2016 beekeepers from Timiș county reported bee colonies losses. During the winter months the situation became more the situation has become more severe, increasing losses of bee families. From November 2016, untill February 2017 were been received samples of dead bees from 23 different apiaries in Timiș county. The dead honey bees were analized in the way to discover what kind of causes produced all these losses. Was also proceeded to analyze the bacterial flora from bees intestine. Bacteriological examination of bees intestine and the cultivation on special medium of culture emphasized rich cultures of Paenibacillus larvae subsp. larvae (Bacillus larvae, etiologic agent for American foulbrood. These bacteria were been isolated from bee intestine in samples provided from 18 apiaries (78.26%. There are many causes of high degree of bee mortality in winter 2016-2017 but the isolation of Paenibacillus larvae subsp. larvae maybe was one of them.

  10. Xeroderma Pigmentosum/De Sanctis-Cacchione Syndrome: Unusual Cause of Ataxia

    Directory of Open Access Journals (Sweden)

    Robert Fekete

    2014-03-01

    Full Text Available Introduction: Xeroderma pigmentosum (XP is a rare autosomal recessive disorder of DNA repair, with a prevalence of 1 in 1 million. It may also be a cause of neurological symptoms including sensorineural hearing loss, peripheral neuropathy, ataxia, and chorea. Severe neurological symptoms including mental retardation, short stature, and hypogonadism invoke De Sanctis-Cacchione syndrome (DCS. Case Report: The patient was a 55-year-old woman with a history of mental retardation who developed chorea at age 32 and ataxia at age 37. She had numerous facial scars from 10 prior basal cell carcinoma excisions as well as diminished deep tendon reflexes, bilateral hearing loss, dysphagia, and skin freckling. Brain MRI revealed severe cortical, cerebellar, and brainstem atrophy. Supportive treatment and prevention of further damage from UV light is the mainstay of treatment in XP and DCS. Conclusion: XP and related disorders should be considered in the setting of neurological disorder and multiple cutaneous cancers.

  11. Loss-of-feedwater transients in PWRs

    International Nuclear Information System (INIS)

    Burns, R.D. III.

    1980-01-01

    Recent severe accident sequence analysis (SASA) work in LASL's Multifault Accident Analysis Section has focused on loss-of-feedwater (LOFW) transients at a 4-loop Westinghouse nuclear power reactor. In all transients studied, the initiator was loss of main feedwater and reactor coolant pump (RCP) trip, caused by temporary loss of off-site power. Subsequent automatic actions included reactor scram, closure of the main steam isolation valves, and initiation of auxiliary feedwater (AFW) flow. TRAC-PD2 calculations were designed to study the consequences of AFW delivery rates below the minimum specified in the emergency operating procedures (EOPs) for the reference 4-loop plant. Six types of LOFW scenarios have been studied, including (1) zero AFW availability (nominal case), (2) initially zero AFW but full recovery after 2 h, (3) zero AFW with steam generator (SG) atmospheric relief valve (ARV) malfunction, (4) zero AFW with high pressure charging flow initiated after 2 h, and (5) zero AFW with delay in reactor scram. Additional cases were considered to study the effects of uncertainties in pressurizer heater/spray operation, operator manual initiation of high pressure charging flow, reactor initial conditions, and RCP and power coastdown characteristics. Nominal case results, rationale for selections of other cases, and lessons learned are summarized

  12. Evaluation of Treatment Thresholds for Unconjugated Hyperbilirubinemia in Preterm Infants : Effects on Serum Bilirubin and on Hearing Loss?

    NARCIS (Netherlands)

    Hulzebos, Christian V.; van Dommelen, Paula; Verkerk, Paul H.; Dijk, Peter H.; Van Straaten, Henrica L. M.

    2013-01-01

    Background: Severe unconjugated hyperbilirubinemia may cause deafness. In the Netherlands, 25% lower total serum bilirubin (TSB) treatment thresholds were recently implemented for preterm infants. Objective: To determine the rate of hearing loss in jaundiced preterms treated at high or at low TSB

  13. Evaluation of Treatment Thresholds for Unconjugated Hyperbilirubinemia in Preterm Infants: Effects on Serum Bilirubin and on Hearing Loss?

    NARCIS (Netherlands)

    Hulzebos, C.V.; Dommelen, P. van; Verkerk, P.H.; Dijk, P.H.; Straaten, H.L.M. van

    2013-01-01

    Background:Severe unconjugated hyperbilirubinemia may cause deafness. In the Netherlands, 25% lower total serum bilirubin (TSB) treatment thresholds were recently implemented for preterm infants.Objective:To determine the rate of hearing loss in jaundiced preterms treated at high or at low TSB

  14. Loss of PTEN causes SHP2 activation, making lung cancer cells unresponsive to IFN-γ

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Chia-Ling [Translational Research Center, Taipei Medical University, Taipei 110, Taiwan (China); Chiang, Tzu-Hui; Tseng, Po-Chun [Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan 701, Taiwan (China); Wang, Yu-Chih [Department of Microbiology and Immunology, School of Medicine, College of Medicine, Taipei Medical University, Taipei 110, Taiwan (China); Lin, Chiou-Feng, E-mail: cflin2014@tmu.edu.tw [Department of Microbiology and Immunology, School of Medicine, College of Medicine, Taipei Medical University, Taipei 110, Taiwan (China); Graduate Institute of Medical Sciences, College of Medicine, Taipei Medical University, Taipei 110, Taiwan (China)

    2015-10-23

    Src homology-2 domain-containing phosphatase (SHP) 2, an oncogenic phosphatase, inhibits type II immune interferon (IFN)-γ signaling by subverting signal transducers and activators of transcription 1 tyrosine phosphorylation and activation. For cancer immunoediting, this study aimed to investigate the decrease of phosphatase and tensin homolog deleted on chromosome 10 (PTEN), a tumor suppressor protein, leading to cellular impairment of IFN-γ signaling. In comparison with human lung adenocarcinoma A549 cells, the natural PTEN loss in another human lung adenocarcinoma line, PC14PE6/AS2 cells, presents reduced responsiveness in IFN-γ-induced IFN regulatory factor 1 activation and CD54 expression. Artificially silencing PTEN expression in A549 cells also caused cells to be unresponsive to IFN-γ without affecting IFN-γ receptor expression. IFN-γ-induced inhibition of cell proliferation and cytotoxicity were demonstrated in A549 cells but were defective in PC14PE6/AS2 cells and in PTEN-deficient A549 cells. Aberrant activation of SHP2 by ROS was specifically shown in PC14PE6/AS2 cells and PTEN-deficient A549 cells. Inhibiting ROS and SHP2 rescued cellular responses to IFN-γ-induced cytotoxicity and inhibition of cell proliferation in PC14PE6/AS2 cells. These results demonstrate that a decrease in PTEN facilitates ROS/SHP2 signaling, causing lung cancer cells to become unresponsive to IFN-γ. - Highlights: • This study demonstrates that PTEN decrease causes cellular unresponsive to IFN-γ. • Lung cancer cells with PTEN deficiency show unresponsive to IFN-γ signaling. • PTEN decrease inhibits IFN-γ-induced CD54, cell proliferation inhibition, and cytotoxicity. • ROS-mediated SHP2 activation makes PTEN-deficient cells unresponsive to IFN-γ.

  15. Types and severity of operated supraclavicular brachial plexus injuries caused by traffic accidents.

    Science.gov (United States)

    Kaiser, Radek; Waldauf, Petr; Haninec, Pavel

    2012-07-01

    Brachial plexus injuries occur in up to 5% of polytrauma cases involving motorcycle accidents and in approximately 4% of severe winter sports injuries. One of the criteria for a successful operative therapy is the type of lesion. Upper plexus palsy has the best prognosis, whereas lower plexus palsy is surgically untreatable. The aim of this study was to evaluate a group of patients with brachial plexus injury caused by traffic accidents, categorize the injuries according to type of accident, and look for correlations between type of palsy (injury) and specific accidents. A total of 441 brachial plexus reconstruction patients from our department were evaluated retrospectively(1993 to 2011). Sex, age, neurological status, and the type and cause of injury were recorded for each case. Patients with BPI caused by a traffic accident were assessed in detail. Traffic accidents were the cause of brachial plexus injury in most cases (80.7%). The most common type of injury was avulsion of upper root(s) (45.7%) followed by rupture (28.2%), complete avulsion (16.9%) and avulsion of lower root(s) (9.2%). Of the patients, 73.9% had an upper,22.7% had a complete and only 3.4% had a lower brachial plexus palsy. The main cause was motorcycle accidents(63.2%) followed by car accidents (23.5%), bicycle accidents(10.7%) and pedestrian collisions (3.1%) (paccidents had a higher percentage of lower avulsion (22.7%) and a lower percentage of upper avulsion (29.3%), whereas cyclists had a higher percentage of upper avulsion (68.6%) based on the data from the entire group of patients (paccidents (9.3%,paccidents),significantly more upper and fewer lower palsies were present. In the bicycle accident group, upper palsy was the most common (89%). Study results indicate that the most common injury was an upper plexus palsy. It was characteristic of bicycle accidents, and significantly more common in car and motorcycle accidents. The results also indicate that it is important to consider the

  16. Study on the methodology for allocating costs of grid losses

    International Nuclear Information System (INIS)

    Aalberts, A.; Dekker, G.; Jaarsma, S.; Tieben, B.; Vlug, N.

    2011-01-01

    The research question in this report: 'Where are grid losses caused and in which (objective) manner can the costs of these grid losses be allocated to the buyers. The key questions answered in the research are: (1) In which locations of the electricity grid are losses caused and what is the volume of these losses?; (2) How are the costs of grid losses allocated to the various groups of end users?; and (3) What are alternative way(s) to allocate the costs of grid losses to the buyers?. [nl

  17. Hair Loss Myths.

    Science.gov (United States)

    DiMarco, Gabriella; McMichael, Amy

    2017-07-01

    INTRODUCTION: Hair loss is a common complaint seen in dermatology clinics. From frustration and attempts at self-help, patients with hair loss may present to the dermatologist with false beliefs, or myths, about the causes of their condition and what treatments are effective. METHODS: We identified 12 common myths about hair loss, categorized as myths about minoxidil treatment, vitamin and mineral supplements, natural topical treatments, and hair care practices. We performed a PubMed search to find evidence to support or refute each myth. RESULTS: We found that there is little evidence to support many of these common hair loss myths. In some cases, randomized controlled trials have investigated the effects of particular therapies and point to the effectiveness of certain hair loss treatments. DISCUSSION: In many cases, there have not been sufficient randomized controlled trials to evaluate the effect of different therapies and hair care practices on hair loss. It is best to guide patients toward treatments with a long track record of efficacy and away from those where little is known scientifically. J Drugs Dermatol. 2017;16(7):690-694..

  18. Brd2 disruption in mice causes severe obesity without Type 2 diabetes.

    Science.gov (United States)

    Wang, Fangnian; Liu, Hongsheng; Blanton, Wanda P; Belkina, Anna; Lebrasseur, Nathan K; Denis, Gerald V

    2009-12-14

    Certain human subpopulations are metabolically healthy but obese, or metabolically obese but normal weight; such mutations uncouple obesity from glucose intolerance, revealing pathways implicated in Type 2 diabetes. Current searches for relevant genes consume significant effort. We have reported previously a novel double bromodomain protein called Brd2, which is a transcriptional co-activator/co-repressor with SWI/SNF (switch mating type/sucrose non-fermenting)-like functions that regulates chromatin. In the present study, we show that wholebody disruption of Brd2, an unusual MHC gene, causes lifelong severe obesity in mice with pancreatic islet expansion, hyperinsulinaemia, hepatosteatosis and elevated pro-inflammatory cytokines, but, surprisingly, enhanced glucose tolerance, elevated adiponectin, increased weight of brown adipose tissue, heat production and expression of mitochondrial uncoupling proteins in brown adipose tissue, reduced macrophage infiltration in white adipose tissue, and lowered blood glucose, leading to an improved metabolic profile and avoiding eventual Type 2 diabetes. Brd2 is highly expressed in pancreatic beta-cells, where it normally inhibits beta-cell mitosis and insulin transcription. In 3T3-L1 pre-adipocytes, Brd2 normally co-represses PPAR-gamma (peroxisome-proliferator-activated receptor-gamma) and inhibits adipogenesis. Brd2 knockdown protects 3T3-L1 adipocytes from TNF-alpha (tumour necrosis factor-alpha)-induced insulin resistance, thereby decoupling inflammation from insulin resistance. Thus hypomorphic Brd2 shifts energy balance toward storage without causing glucose intolerance and may provide a novel model for obese metabolically healthy humans.

  19. Severe rickets in a young girl caused by celiac disease: the tragedy of delayed diagnosis: a case report.

    Science.gov (United States)

    Al-Sharafi, Butheinah A; Al-Imad, Shafiq A; Shamshair, Amani M; Al-Faqeeh, Derhim H

    2014-10-08

    Celiac disease is a systemic immune mediated disease which usually presents with gastrointestinal symptoms, but it may present with extra gastrointestinal manifestations such as metabolic bone disease and failure to thrive. This may lead to a delay in the diagnosis. We present a 13 year old female from the middle east with an 8 year history of severe rickets causing multiple bone deformities leaving the child crippled with bowing of both of her arms and legs. The patient was also found to have growth failure, anemia and on further workup she was found to have celiac disease. We are presenting this case because it shows a severe case of rickets after malabsorption for many years. Celiac disease should be kept in mind as a cause of rickets in patients not responding to usual forms of treatment or when associated with other manifestations of malabsorption.

  20. Severe hearing impairment among military veterans--United States, 2010.

    Science.gov (United States)

    2011-07-22

    A substantial proportion of hearing loss in the United States is attributable to employment-related exposure to noise. Among military veterans, the most common service-connected disabilities are hearing impairments, suggesting that occupational noise exposure during military service might cause more veterans to have hearing loss than nonveterans. However, a recent analysis of data from the 1993-1995 Epidemiology of Hearing Loss Study did not find significant differences between the two groups. To further investigate hearing loss among veterans, specifically the prevalence of severe hearing impairment (SHI), data from the 2010 Annual Social and Economic Supplement (ASEC) to the Current Population Survey (CPS) were analyzed. This report describes the results of those analyses, which indicated that the prevalence of SHI among veterans was significantly greater than among nonveterans. Veterans were 30% more likely to have SHI than nonveterans after adjusting for age and current occupation, and veterans who served in the United States or overseas during September 2001-March 2010, the era of overseas contingency operations (including Operations Enduring Freedom and Iraqi Freedom), were four times more likely than nonveterans to have SHI. These findings suggest a need for increased emphasis on improving military hearing conservation programs (HCPs) and on hearing loss surveillance in military and veterans' health systems.

  1. Farmers’ perceptions of crop pest severity in Nigeria are associated with landscape, agronomic and socio-economic factors

    NARCIS (Netherlands)

    Zhang, Wei; Kato, Edward; Bianchi, Felix; Bhandary, Prapti; Gort, Gerrit; Werf, van der Wopke

    2018-01-01

    Insect pests are a major cause of crop yield losses around the world and pest management plays a critical role in providing food security and farming income. This study links Nigerian farmers’ perceptions of pest severity to the landscape, agronomic, biophysical, and socio-economic context in which

  2. Medical surveillance of occupational Noise Induced Hearing Loss (NIHL) at RAPS hospital

    International Nuclear Information System (INIS)

    Manuwal, A.K.; Ganesan, S.

    2009-01-01

    Full text: One of the most challenging roles of a Certifying Surgeon is to act as catalyst for change. This paper is a presentation of the impact of such an effort. Noise is the insidious of all industrial pollutants, involving every industry and causing severe hearing loss. Exposure to excessive noise is the major avoidable cause of permanent hearing impairment. Noise - induced hearing loss (NIHL) is bilateral and symmetrical, usually affecting the higher frequencies 3 k, 4 k or 6 kHz ) and then spreading to the lower frequencies (0.5 k,1 k or 2 kHz). The major health effects are lack of concentration, irritation, fatigue, headache, sleep disturbances etc. Hearing protectors should be used when engineering controls and work practices are not feasible for reducing noise exposure to safe levels. Ear muffs, ear plugs and ear canal caps are the main types of hearing protectors. Awareness should be created among workers about the harmful effects of noise on hearing and other body systems by implementing compulsory education noise conservation programmes. The practice followed at RAPS Hospital for medical surveillance of Occupational Noise Induced Hearing Loss is being briefed

  3. Effect of curtovirus species competitiveness in host plants on transmission and incidence of Beet severe curly top virus and Beet mild curly top virus

    Science.gov (United States)

    Curly top disease, caused by viruses in the genus Curtovirus, causes significant economic losses for sugarbeet and other crops throughout the western United States. Recent studies demonstrated the two most abundant curtovirus species in the US are Beet severe curly top virus (BSCTV) and Beet mild c...

  4. Gewichtsverlies, serumnatriumconcentratie en restverschijnselen bij patiëntjes met hypertone dehydratie door onvoldoende borstvoeding [Weight loss, serum sodium concentration and residual symptoms in patients with hypernatremic dehydration caused by insufficient breastfeeding

    NARCIS (Netherlands)

    Breuning-Boers, J.M.; Dommelen, P. van; Wouwe, J.P. van; Verkerk, P.H.

    2006-01-01

    Objective. To determine the relationship between serum sodium concentration and weight loss as well as residual symptoms in newborns with hypernatremic dehydration caused by insufficient breastfeeding; and to determine the sensitivity of the following rule of thumb 'if weight loss is less than 10%,

  5. Quality change and mass loss of paddy during airtight storage in a ferro-cement bin in Sri Lanka

    NARCIS (Netherlands)

    Adhikarinayake, T.B.; Palipane, K.B.; Müller, J.

    2006-01-01

    In Sri Lanka, prices for paddy fluctuate severely showing a minimum price at harvest. To benefit from higher prices, farmers strive to store paddy, but lack of facilities and poor storage management cause quantitative and qualitative losses by rodents, insects and microbial deterioration. To

  6. Identification of the cause of severe skin infection by Fournier transform infrared spectroscopy: a case of Fournier's gangrene caused by fish bone.

    Science.gov (United States)

    Shimizu, Takae; Harada, Kazutoshi; Akazawa, Satoshi; Yamaguchi, Miyuki; Inozume, Takashi; Kawamura, Tatsuyoshi; Shibagaki, Naotaka; Momosawa, Akira; Shimada, Shinji

    2014-06-01

    Fournier's gangrene (FG) is an infrequent but highly lethal infection. Here we report a 74-year-old man who presented with genital swelling and severe malaise. Based on the physical and imaging examination results, the diagnosis of FG was confirmed. Intraoperative findings showed dirty necrosis of soft tissue, and a splinter-shaped foreign body was found in the perirectal region. The foreign body was thought to be the cause of the condition, and it was analyzed using Fourier transform infrared spectroscopy. We found that the foreign body was a mixture of calcium phosphate and protein, suggesting that the splinter was a bone. Moreover, during the medical interview, the patient mentioned about intake of fish around the time of onset of symptoms. Therefore, to confirm the results of the analysis, DNA was extracted from the foreign body, and genomic PCR with subsequent sequence analysis was performed. The DNA sequence was identical to that of Oncorhynchus kisutch, a salmon that is a very popular food in Japan. On the basis of these findings, we concluded that FG in this case was caused by the penetration into the rectum of an accidentally ingested fish bone. Although some cases of intra-abdominal abscess due to accidental ingestion of fish bone have been reported, FG caused by fish bone is extremely rare. © 2014 Japanese Dermatological Association.

  7. Cellular mechanisms of cyclophosphamide-induced taste loss in mice

    Science.gov (United States)

    Mukherjee, Nabanita; Pal Choudhuri, Shreoshi; Delay, Rona J.

    2017-01-01

    Many commonly prescribed chemotherapy drugs such as cyclophosphamide (CYP) have adverse side effects including disruptions in taste which can result in loss of appetite, malnutrition, poorer recovery and reduced quality of life. Previous studies in mice found evidence that CYP has a two-phase disturbance in taste behavior: a disturbance immediately following drug administration and a second which emerges several days later. In this study, we examined the processes by which CYP disturbs the taste system by examining the effects of the drug on taste buds and cells responsible for taste cell renewal using immunohistochemical assays. Data reported here suggest CYP has direct cytotoxic effects on lingual epithelium immediately following administration, causing an early loss of taste sensory cells. Types II and III cells in fungiform taste buds appear to be more susceptible to this effect than circumvallate cells. In addition, CYP disrupts the population of rapidly dividing cells in the basal layer of taste epithelium responsible for taste cell renewal, manifesting a disturbance days later. The loss of these cells temporarily retards the system’s capacity to replace Type II and Type III taste sensory cells that survived the cytotoxic effects of CYP and died at the end of their natural lifespan. The timing of an immediate, direct loss of taste cells and a delayed, indirect loss without replacement of taste sensory cells are broadly congruent with previously published behavioral data reporting two periods of elevated detection thresholds for umami and sucrose stimuli. These findings suggest that chemotherapeutic disturbances in the peripheral mechanisms of the taste system may cause dietary challenges at a time when the cancer patient has significant need for well balanced, high energy nutritional intake. PMID:28950008

  8. Cellular mechanisms of cyclophosphamide-induced taste loss in mice.

    Directory of Open Access Journals (Sweden)

    Nabanita Mukherjee

    Full Text Available Many commonly prescribed chemotherapy drugs such as cyclophosphamide (CYP have adverse side effects including disruptions in taste which can result in loss of appetite, malnutrition, poorer recovery and reduced quality of life. Previous studies in mice found evidence that CYP has a two-phase disturbance in taste behavior: a disturbance immediately following drug administration and a second which emerges several days later. In this study, we examined the processes by which CYP disturbs the taste system by examining the effects of the drug on taste buds and cells responsible for taste cell renewal using immunohistochemical assays. Data reported here suggest CYP has direct cytotoxic effects on lingual epithelium immediately following administration, causing an early loss of taste sensory cells. Types II and III cells in fungiform taste buds appear to be more susceptible to this effect than circumvallate cells. In addition, CYP disrupts the population of rapidly dividing cells in the basal layer of taste epithelium responsible for taste cell renewal, manifesting a disturbance days later. The loss of these cells temporarily retards the system's capacity to replace Type II and Type III taste sensory cells that survived the cytotoxic effects of CYP and died at the end of their natural lifespan. The timing of an immediate, direct loss of taste cells and a delayed, indirect loss without replacement of taste sensory cells are broadly congruent with previously published behavioral data reporting two periods of elevated detection thresholds for umami and sucrose stimuli. These findings suggest that chemotherapeutic disturbances in the peripheral mechanisms of the taste system may cause dietary challenges at a time when the cancer patient has significant need for well balanced, high energy nutritional intake.

  9. North-south differences in Chinese agricultural losses due to climate-change-influenced droughts

    Science.gov (United States)

    Qiang, Zhang; Lanying, Han; Jingjing, Lin; Qingyan, Cheng

    2018-01-01

    One of the effects of global climate change is increase in the frequency and severity of drought, which strongly affects the Chinese agricultural production. In order to cope these changes more effectively, it is important to document and analyze the agricultural losses caused by drought. We collected and analyzed conventional meteorological data and agricultural statistics data, in order to outline trends in drought occurrence and decline in agricultural yield. Data were assembled for the period 1960-2010. The study pays particular attention to regional differences between northern and southern China. Our results show the drought-caused agricultural loss rates (DCALR) in China have increased by approximately 0.5% per decade in the past 50 years. The study area in this paper is for the whole of the People's Republic of China, minus the Qinghai-Tibetan Plateau; when we analyzed regional differences, we found that losses increased by approximately 0.6% per decade in northern China, close to twice the increase in southern China. Moreover, drought risks and agricultural losses are rising faster in northern China. Our results also indicate that the agriculture in northern China is more sensitive to changes in precipitation, whereas the agriculture in southern China is more sensitive to temperature changes.

  10. A treatable cause of myelopathy and vision loss mimicking neuromyelitis optica spectrum disorder: late-onset biotinidase deficiency.

    Science.gov (United States)

    Yilmaz, Sanem; Serin, Mine; Canda, Ebru; Eraslan, Cenk; Tekin, Hande; Ucar, Sema Kalkan; Gokben, Sarenur; Tekgul, Hasan; Serdaroglu, Gul

    2017-06-01

    Biotinidase deficiency is characterized by severe neurological manifestations as hypotonia, lethargy, ataxia, hearing loss, seizures and developmental retardation in its classical form. Late-onset biotinidase deficiency presents distinctly from the classical form such as limb weakness and vision problems. A 14-year-old boy presented with progressive vision loss and upper limb weakness. The patient was initiated steroid therapy with a preliminary diagnosis of neuromyelitis optica spectrum disorder due to the craniospinal imaging findings demonstrating optic nerve, brainstem and longitudinally extensive spinal cord involvement. Although the patient exhibited partial clinical improvement after pulse steroid therapy, craniocervical imaging performed one month after the initiation of steroid therapy did not show any regression. The CSF IgG index was <0.8 (normal: <0.8), oligoclonal band and aquaporin-4 antibodies were negative. Metabolic investigations revealed a low biotinidase enzyme activity 8% (0.58 nmoL/min/mL; normal range: 4.4 to 12). Genetic testing showed c.98-104delinsTCC and p.V457 M mutations in biotinidase (BTD) gene. At the third month of biotin replacement therapy, control craniospinal MRI demonstrated a complete regression of the lesions. The muscle strength of the case returned to normal. His visual acuity was 7/10 in the left eye and 9/10 in the right. The late-onset form of the biotinidase deficiency should be kept in mind in all patients with myelopathy with or without vision loss, particularly in those with inadequate response to steroid therapy. The family screening is important to identify asymptomatic individuals and timely treatment.

  11. Conditional Deletion of PDK1 in the Forebrain Causes Neuron Loss and Increased Apoptosis during Cortical Development

    Directory of Open Access Journals (Sweden)

    Congyu Xu

    2017-10-01

    Full Text Available Decreased expression but increased activity of PDK1 has been observed in neurodegenerative disease. To study in vivo function of PDK1 in neuron survival during cortical development, we generate forebrain-specific PDK1 conditional knockout (cKO mice. We demonstrate that PDK1 cKO mice display striking neuron loss and increased apoptosis. We report that PDK1 cKO mice exhibit deficits on several behavioral tasks. Moreover, PDK1 cKO mice show decreased activities for Akt and mTOR. These results highlight an essential role of endogenous PDK1 in the maintenance of neuronal survival during cortical development.

  12. Monitoring severe accidents using AI techniques

    Energy Technology Data Exchange (ETDEWEB)

    No, Young Gyu; Ahn, Kwang Il [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of); Kim, Ju Hyun; Na, Man Gyun [Dept. of Nuclear Engineering, Chosun University, Gwangju (Korea, Republic of); Lim, Dong Hyuk [Korea Institute of Nuclear Nonproliferation and Control, Daejon (Korea, Republic of)

    2012-05-15

    After the Fukushima nuclear accident in 2011, there has been increasing concern regarding severe accidents in nuclear facilities. Severe accident scenarios are difficult for operators to monitor and identify. Therefore, accurate prediction of a severe accident is important in order to manage it appropriately in the unfavorable conditions. In this study, artificial intelligence (AI) techniques, such as support vector classification (SVC), probabilistic neural network (PNN), group method of data handling (GMDH), and fuzzy neural network (FNN), were used to monitor the major transient scenarios of a severe accident caused by three different initiating events, the hot-leg loss of coolant accident (LOCA), the cold-leg LOCA, and the steam generator tube rupture in pressurized water reactors (PWRs). The SVC and PNN models were used for the event classification. The GMDH and FNN models were employed to accurately predict the important timing representing severe accident scenarios. In addition, in order to verify the proposed algorithm, data from a number of numerical simulations were required in order to train the AI techniques due to the shortage of real LOCA data. The data was acquired by performing simulations using the MAAP4 code. The prediction accuracy of the three types of initiating events was sufficiently high to predict severe accident scenarios. Therefore, the AI techniques can be applied successfully in the identification and monitoring of severe accident scenarios in real PWRs.

  13. Monitoring severe accidents using AI techniques

    International Nuclear Information System (INIS)

    No, Young Gyu; Ahn, Kwang Il; Kim, Ju Hyun; Na, Man Gyun; Lim, Dong Hyuk

    2012-01-01

    After the Fukushima nuclear accident in 2011, there has been increasing concern regarding severe accidents in nuclear facilities. Severe accident scenarios are difficult for operators to monitor and identify. Therefore, accurate prediction of a severe accident is important in order to manage it appropriately in the unfavorable conditions. In this study, artificial intelligence (AI) techniques, such as support vector classification (SVC), probabilistic neural network (PNN), group method of data handling (GMDH), and fuzzy neural network (FNN), were used to monitor the major transient scenarios of a severe accident caused by three different initiating events, the hot-leg loss of coolant accident (LOCA), the cold-leg LOCA, and the steam generator tube rupture in pressurized water reactors (PWRs). The SVC and PNN models were used for the event classification. The GMDH and FNN models were employed to accurately predict the important timing representing severe accident scenarios. In addition, in order to verify the proposed algorithm, data from a number of numerical simulations were required in order to train the AI techniques due to the shortage of real LOCA data. The data was acquired by performing simulations using the MAAP4 code. The prediction accuracy of the three types of initiating events was sufficiently high to predict severe accident scenarios. Therefore, the AI techniques can be applied successfully in the identification and monitoring of severe accident scenarios in real PWRs.

  14. Epidemiology of dark leaf spot caused by Alternaria brassicicola and Alternaria brassicae in organic seed production of cauliflower

    NARCIS (Netherlands)

    Köhl, J.; Tongeren, van C.A.M.; Groenenboom-de Haas, B.H.; Hoof, van R.A.; Driessen, R.; Heijden, van der L.

    2010-01-01

    In organic seed production of Brassica vegetables, infections by Alternaria brassicicola and A. brassicae can cause severe losses of yield and seed quality. Four field experiments with or without artificial inoculation with A. brassicicola were conducted in organically managed seed-production crops

  15. Severe musculoskeletal time-loss injuries and symptoms of common mental disorders in professional soccer: a longitudinal analysis of 12-month follow-up data

    NARCIS (Netherlands)

    Kiliç, Ö; Aoki, H.; Goedhart, E.; Hägglund, M.; Kerkhoffs, G. M. M. J.; Kuijer, P. P. F. M.; Waldén, M.; Gouttebarge, V.

    2018-01-01

    Psychological factors have shown to be predictors of injury in professional football. However, it seems that this is a two-way relationship, as severe musculoskeletal time-loss injuries have shown to be associated with the onset of symptoms of common mental disorders (CMD). There is no longitudinal

  16. A new loss mechanism in graphene nanoresonators due to the synthetic electric fields caused by inherent out-of-plane membrane corrugations

    International Nuclear Information System (INIS)

    Firsova, N E; Firsov, Yu A

    2012-01-01

    For the first time the influence of out-of-plane deformations, which always exist in graphene, on the non-stationary processes is considered for the case of a monolayer graphene nanoresonator. A new loss mechanism for this device caused by dissipative intra-valley currents stipulated by synthetic electric fields is studied. These fields are generated by time-dependent gauge fields arising in a graphene membrane due to its intrinsic out-of-plane distortions and the influence of the external periodic electromotive force. The corresponding formula for the quality factor has a quantum mechanical origin and includes quantum mechanical parameters. This loss mechanism accounts for an essential part (about 40%) of losses in a graphene nanoresonator and it is specific just for graphene. The ways of minimization of this kind of dissipation (an increase in the quality factor of the electromechanical system) are discussed. It is explained why one can enhance the quality factor by correctly choosing a combination of strains (by strain engineering). In addition, it is shown that the quality factor can be increased by switching on a magnetic field perpendicular to the graphene membrane.

  17. Causes of iron overload in blood donors - a clinical study

    DEFF Research Database (Denmark)

    Laursen, A H; Bjerrum, O W; Friis-Hansen, L

    2018-01-01

    BACKGROUND AND OBJECTIVES: Despite the obligate iron loss from blood donation, some donors present with hyperferritinaemia that can result from a wide range of acute and chronic conditions including hereditary haemochromatosis (HH). The objective of our study was to investigate the causes...... of hyperferritinaemia in the blood donor population and explore the value of extensive HH mutational analyses. MATERIALS AND METHODS: Forty-nine consecutive donors (f = 6, m = 43) were included prospectively from the Capital Regional Blood Center. Inclusion criteria were a single ferritin value >1000 μg/l or repeated...... four donors had apparent alternative causes of hyperferritinaemia. CONCLUSION: HH-related mutations were the most frequent cause of hyperferritinaemia in a Danish blood donor population, and it appears that several different HH-genotypes can contribute to hyperferritinaemia. HH screening in blood...

  18. ECT and memory loss.

    Science.gov (United States)

    Squire, L R

    1977-09-01

    The author reviews several studies that clarify the nature of the memory loss associated with ECT. Bilateral ECT produced greater anterograde memory loss than right unilateral ECT and more extensive retrograde amnesia than unilateral ECT. Reactivating memories just before ECT did not produce amnesia. Capacity for new learning recovered substantially by several months after ECT, but memory complaints were common in individuals who had received bilateral ECT. Other things being equal, right unilateral ECT seems preferable to bilateral ECT because the risks to memory associated with unilateral ECT are smaller.

  19. [Labor productivity losses attributable to premature deaths due to traffic injuries between 2002 and 2012].

    Science.gov (United States)

    Cubí-Mollá, Patricia; Peña-Longobardo, Luz María; Casal, Bruno; Rivera, Berta; Oliva-Moreno, Juan

    2015-09-01

    To estimate the years of potential life lost, years of potential productive life lost and the labor productivity losses attributable to premature deaths due to traffic injuries between 2002 and 2012 in Spain. Several statistical sources were combined (Spanish Registry of Deaths, Labor Force Survey and Wage Structure Survey) to develop a simulation model based on the human capital approach. This model allowed us to estimate the loss of labor productivity caused by premature deaths following traffic injuries from 2002 to 2012. In addition, mortality tables with life expectancy estimates were used to compute years of potential life lost and years of potential productive life lost. The estimated loss of labour productivity caused by fatal traffic injuries between 2002 and 2012 in Spain amounted to 9,521 million euros (baseline year 2012). The aggregate number of years of potential life lost in the period amounted to 1,433,103, whereas the years of potential productive life lost amounted to 875,729. Throughout the period analyzed, labor productivity losses and years of life lost diminished substantially. Labor productivity losses due to fatal traffic injuries decreased throughout the period analyzed. Nevertheless, the cumulative loss was alarmingly high. Estimation of the economic impact of health problems can complement conventional indicators of distinct dimensions and be used to support public policy making. Copyright © 2014 SESPAS. Published by Elsevier Espana. All rights reserved.

  20. Assessment of patient perception of glaucomatous visual field loss and its association with disease severity using Amsler grid.

    Directory of Open Access Journals (Sweden)

    Kenji Fujitani

    Full Text Available To investigate patients' perception of glaucomatous VF loss and its association with glaucoma severity using the Amsler grid test.In this prospective cross-sectional study, glaucoma patients with abnormal 10-2 Humphrey Swedish Interactive Threshold Algorithm-standard VF tests were enrolled consecutively. All patients underwent a black-on-white Amsler grid test for each eligible eye. They were asked to outline any perceived scotomas (areas with abnormal grid lines on the grid and then describe verbally their perception of the scotomas. Examiners asked patients to clarify their descriptions. All descriptions used by patients were recorded in their own words, which were then sorted into descriptor categories according to similar themes. The number of descriptor categories was counted for each eye. 10-2 VF mean deviation (MD was compared among eyes that reported different number of descriptor categories. The mean 10-2 VF MD values were compared among different descriptor categories.Fifty glaucoma patients (88 eyes were included. Patients used a total of 44 different descriptors for their scotomas. Patients' descriptors were classified into categories that incorporated similar themes, resulting in 4 overarching descriptor categories: Missing/White, Blurry/Gray, Black, and Not Aware. Fifty-two eyes reported one descriptor category and 19 eyes reported two descriptor categories (mean number of descriptor categories = 1.27±0.45. Eyes that reported two descriptor categories had worse VF MD than those that reported one (-17.86±10.31 dB vs. -12.08±7.53 dB; p = 0.012. When eyes were organized according to its combination of descriptor categories, each eye naturally sorted into one of the following 5 groups, in frequency order: Missing/White (27 eyes; 31%, Blurry/Gray (21 eyes; 24%, combined Missing/White and Blurry/Gray (19 eyes; 21%, Not Aware (17 eyes; 19%, and Black (4 eyes; 5%. The mean 10-2 VF MD severity order was Black (-21.18±10.59 dB, combined

  1. Ablation of whirlin long isoform disrupts the USH2 protein complex and causes vision and hearing loss.

    Directory of Open Access Journals (Sweden)

    Jun Yang

    2010-05-01

    Full Text Available Mutations in whirlin cause either Usher syndrome type II (USH2, a deafness-blindness disorder, or nonsyndromic deafness. The molecular basis for the variable disease expression is unknown. We show here that only the whirlin long isoform, distinct from a short isoform by virtue of having two N-terminal PDZ domains, is expressed in the retina. Both long and short isoforms are expressed in the inner ear. The N-terminal PDZ domains of the long whirlin isoform mediates the formation of a multi-protein complex that includes usherin and VLGR1, both of which are also implicated in USH2. We localized this USH2 protein complex to the periciliary membrane complex (PMC in mouse photoreceptors that appears analogous to the frog periciliary ridge complex. The latter is proposed to play a role in photoreceptor protein trafficking through the connecting cilium. Mice carrying a targeted disruption near the N-terminus of whirlin manifest retinal and inner ear defects, reproducing the clinical features of human USH2 disease. This is in contrast to mice with mutations affecting the C-terminal portion of whirlin in which the phenotype is restricted to the inner ear. In mice lacking any one of the USH2 proteins, the normal localization of all USH2 proteins is disrupted, and there is evidence of protein destabilization. Taken together, our findings provide new insights into the pathogenic mechanism of Usher syndrome. First, the three USH2 proteins exist as an obligatory functional complex in vivo, and loss of one USH2 protein is functionally close to loss of all three. Second, defects in the three USH2 proteins share a common pathogenic process, i.e., disruption of the PMC. Third, whirlin mutations that ablate the N-terminal PDZ domains lead to Usher syndrome, but non-syndromic hearing loss will result if they are spared.

  2. Considerations for surviving the loss of a main feedwater pump at full power

    International Nuclear Information System (INIS)

    Gaydos, K.A.; Calvo, R.; Conroy, P.W.; Klein, C.M.; Mellers, J.E.

    1990-01-01

    Today's economics dictate that nuclear power operational costs be contained by addressing frequently-occurring trips that might be minimized or avoided via specific upgrades. Much recent attention has focused on the significant percentage of plant trips related to feedwater flow regulation; however, another frequent feedwater-related trip stems from the loss of a single main feedwater pump while operating at high power levels, causing a plant trip on low steam generator water-level. This paper summarizes the results of several plant-specific studies that evaluate a unit's capabilities to consistently survive the loss of a main feedwater pump from full power, and outlines a methodology for analyzing this capability

  3. The impact of hearing loss on the quality of life of elderly adults

    Science.gov (United States)

    Ciorba, Andrea; Bianchini, Chiara; Pelucchi, Stefano; Pastore, Antonio

    2012-01-01

    Hearing loss is the most common sensory deficit in the elderly, and it is becoming a severe social and health problem. Especially in the elderly, hearing loss can impair the exchange of information, thus significantly impacting everyday life, causing loneliness, isolation, dependence, and frustration, as well as communication disorders. Due to the aging of the population in the developed world, presbycusis is a growing problem that has been reported to reduce quality of life (QoL). Progression of presbycusis cannot be remediated; therefore, optimal management of this condition not only requires early recognition and rehabilitation, but it also should include an evaluation of QoL status and its assessment. PMID:22791988

  4. Loss experience from natural phenomena hazards in the Department of Energy (50 years of natural phenomena hazard losses)

    International Nuclear Information System (INIS)

    Hill, J.R.

    1993-01-01

    This paper presents a historical prespective on losses due to natural hazard incidents (1943-1993) at Department of Energy (DOE) and predecessor agencies including the Atomic Energy Commission (AEC) and the Energy Research and Development Agency (ERDA). This paper also demonstrates how an existing DOE resource can be used to gain valuable insight into injury or property damage incidents. That resource is the Computerized Accident/Incident Reporting System (CAIRS) module of DOE's Safety Performance Measurement System. CAIRS data selected the 1981-1991 DOE injury/illness reports, from all the accident reports of the AEC that cited a natural phenomena hazard as either the direct or indirect cause of the injury/property damage. Specifically, injury or property damage reports were selected for analysis if they had a causal factor link to severe weather or natural phenomena hazard categories. Natural phenomena hazard categories are injury/property damage caused by hurricane/tornado, earthquake, lightning, or flood. Severe weather categories are injury/property damage associated with other than normal weather conditions. The lessons learned, as a result of reviewing case histories, are presented, as are suggestions on how to reduce the likelihood of future injuries/property damage as a result of similar events. A significant finding, is that most injuries and property damage were the result of an indirect causal link to a natural phenomena hazard and thus, may be more preventable than previously thought possible. The primary message, however, is that CAIRS and other incident data bases are valuable resources and should be considered for use by those interested in identifying new ways of protecting the health and safety of the worker and for reducing building losses due to the effects of natural phenomena hazards

  5. The 1421 St.Elisabeth flooding ‘event’ and the loss of “De Groote Waard”, the Netherlands

    NARCIS (Netherlands)

    Weerts, H.J.T.; Cohen, K.M.; Kleinhans, M.G.

    2012-01-01

    According to legend, a very severe storm in November 1421 caused catastrophic flooding and overnight loss of the larger part of “De Groote Waard”, a prosperous polder area in medieval The Netherlands. Allegedly, more than 25 villages were lost and thousands of people were killed. But what had really

  6. An unusual cause of severe dyspnea: A laryngeal live leech: Case report.

    Science.gov (United States)

    Anajar, Said; Ansari, Rachid; Hassnaoui, Jawad; Abada, Reda; Roubal, Mohammed; Mahtar, Mohammed

    2017-01-01

    Foreign bodies in the upper airways are one of the most challenging otolaryngology emergencies, leeches present a very rare cause of airway foreign bodies around the world. A 6-year-old girl was referred to our otolaryngology department at a tertiary university hospital with a severe dyspnea and hemoptysis. Nasofibroscopy revealed a dark living leech in the supraglottic area which extends to the glottis. The patient was urgently admitted to the operating room, the leech was grasped and removed with a foreign body forceps with a full length of more than 6cm. All symptoms were relieved post operatively and she was discharged one day later. Leeches should be suspected as an airway foreign body in patients with a recent history of drinking from stream water. Prevention remains the best treatment for such cases based simply on hygiene measures like not drinking stream water directly and filtering drinking water before it is used. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  7. Early-Onset Severe Encephalopathy with Epilepsy: The BRAT1 Gene Should Be Added to the List of Causes

    NARCIS (Netherlands)

    van de Pol, L.A.; Wolf, N.I.; van Weissenbruch, M.M.; Stam, C.J.; Weiss, M.M.; Waisfisz, Q.; Kevelam, S.H.; Bugiani, M.; van de Kamp, J.M.; Knaap, M.

    2015-01-01

    A variety of pathologies can underlie early-onset severe encephalopathy with epilepsy. To aid the diagnostic process in such patients we present an overview of causes, including the rapidly expanding list of genes involved. When no explanation is found, whole-exome sequencing (WES) can be used in an

  8. Loss of function of C9orf72 causes motor deficits in a zebrafish model of amyotrophic lateral sclerosis.

    Science.gov (United States)

    Ciura, Sorana; Lattante, Serena; Le Ber, Isabelle; Latouche, Morwena; Tostivint, Hervé; Brice, Alexis; Kabashi, Edor

    2013-08-01

    To define the role that repeat expansions of a GGGGCC hexanucleotide sequence of the C9orf72 gene play in the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). A genetic model for ALS was developed to determine whether loss of function of the zebrafish orthologue of C9orf72 (zC9orf72) leads to abnormalities in neuronal development. C9orf72 mRNA levels were quantified in brain and lymphoblasts derived from FTLD and ALS/FTLD patients and in zebrafish. Knockdown of the zC9orf72 was performed using 2 specific antisense morpholino oligonucleotides to block transcription. Quantifications of spontaneous swimming and tactile escape response, as well as measurements of axonal projections from the spinal cord, were performed. Significantly decreased expression of C9orf72 transcripts in brain and lymphoblasts was found in sporadic FTLD and ALS/FTLD patients with normal-size or expanded hexanucleotide repeats. The zC9orf72 is selectively expressed in the developing nervous system at developmental stages. Loss of function of the zC9orf72 transcripts causes both behavioral and cellular deficits related to locomotion without major morphological abnormalities. These deficits were rescued upon overexpression of human C9orf72 mRNA transcripts. Our results indicate C9orf72 haploinsufficiency could be a contributing factor in the spectrum of ALS/FTLD neurodegenerative disorders. Loss of function of the zebrafish orthologue of zC9orf72 expression in zebrafish is associated with axonal degeneration of motor neurons that can be rescued by expressing human C9orf72 mRNA, highlighting the specificity of the induced phenotype. These results reveal a pathogenic consequence of decreased C9orf72 levels, supporting a loss of function mechanism of disease. © 2013 American Neurological Association.

  9. Correlation between audiovestibular function tests and hearing outcomes in severe to profound sudden sensorineural hearing loss.

    Science.gov (United States)

    Wang, Chi-Te; Huang, Tsung-Wei; Kuo, Shih-Wei; Cheng, Po-Wen

    2009-02-01

    This study investigated whether audiovestibular function tests, namely auditory brain stem response (ABR) and vestibular-evoked myogenic potential (VEMP) tests were correlated to hearing outcomes after controlling the effects of other potential confounding factors in severe to profound sudden sensorineural hearing loss (SSHL). Eighty-eight patients with severe to profound SSHL were enrolled in this study. Pretreatment hearing levels, results of audiovestibular function tests, and final hearing outcomes were recorded from retrospective chart reviews. Other factors, including age, gender, delay of treatment, vertigo, diabetes mellitus, and hypertension, were collected as well. Comparative analysis between multiple variables and hearing outcomes was conducted using the cumulative logits model in overall subjects. Further, multivariate analysis of prognostic factors was conducted in the stratified groups of severe (70 dB HL 90 dB HL) SSHL. Multivariate analysis showed that pretreatment hearing levels, presence of vertigo, and results of ABR and VEMP testing were significant outcome predictors in the overall subjects. Stratification analysis demonstrated that both the presence of ABR and VEMP waveforms were significantly correlated with better hearing outcomes in the group of severe SSHL [ABR: adjusted odds ratio (aOR) = 14.7, 95% confidence interval (CI) = 1.78 to 122, p = 0.01; VEMP: aOR = 5.91, 95% CI = 1.18 to 29.5, p = 0.03], whereas the presence of vertigo was the only significant negative prognostic factor in the group of profound SSHL (aOR = 0.24, 95% CI = 0.06 to 0.95, p = 0.04). Other variables, including age, gender, diabetes mellitus, hypertension, and delay of treatment, were not significantly related to hearing outcomes in both groups (p > 0.05). A predictive hearing recovery table with the combined ABR and VEMP results was proposed for the group of severe SSHL. ABR and VEMP tests should be included in the battery of neurootological examinations in

  10. DNA damage and apoptosis of endometrial cells cause loss of the early embryo in mice exposed to carbon disulfide

    International Nuclear Information System (INIS)

    Zhang, Bingzhen; Shen, Chunzi; Yang, Liu; Li, Chunhui; Yi, Anji; Wang, Zhiping

    2013-01-01

    Carbon disulfide (CS 2 ) may lead to spontaneous abortion and very early pregnancy loss in women exposed in the workplace, but the mechanism remains unclear. We designed an animal model in which gestating Kunming strain mice were exposed to CS 2 via i.p. on gestational day 4 (GD4). We found that the number of implanted blastocysts on GD8 was significantly reduced by each dose of 0.1 LD 50 (157.85 mg/kg), 0.2 LD 50 (315.7 mg/kg) and 0.4 LD 50 (631.4 mg/kg). In addition, both the level of DNA damage and apoptosis rates of endometrial cells on GD4.5 were increased, showed definite dose–response relationships, and inversely related to the number of implanted blastocysts. The expressions of mRNA and protein for the Bax and caspase-3 genes in the uterine tissues on GD4.5 were up-regulated, while the expressions of mRNA and protein for the Bcl-2 gene were dose-dependently down-regulated. Our results indicated that DNA damage and apoptosis of endometrial cells were important reasons for the loss of implanted blastocysts induced by CS 2 . - Highlights: • We built an animal model of CS2 exposure during blastocyst implantation. • Endometrial cells were used in the comet assay to detect DNA damage. • CS2 exposure caused DNA damage and endometrial cell apoptosis. • DNA damage and endometrial cell apoptosis were responsible for embryo loss

  11. Severe ionosphere disturbances caused by the sudden response of evening subequatorial ionospheres to geomagnetic storms

    International Nuclear Information System (INIS)

    Tanaka, T.

    1981-01-01

    By monitoring C band beacon signals from geostationary satellites in Japan, we have observed anomalously strong ionospheric scintillations several times during three years from 1978 to 1980. These severe scinitillations occur associated with geomagnetic storms and accompany sudden and intense ionospheric perturbations in the low-latiude region. Through the analysis of these phenomena we have identified a new type of ionospheric disturbances characterized by intensifications of equatorial anomalies and successive severe ionospheric scintillations that extend to the C band range. The events occur only during a limited local time interval after the sunset, when storm time decreases of midlatitude geomagnetic fields in the same meridan take place during the same time interval. From the viewpoint of ionospheric storms, these disturbances precede the occurrence of midlatitude negative phases and storm time depressions of equatorial anomalies to indicate that the cause of the events is different from distrubed thermospheric circulations. The timing and magnitude of substorms at high-latitudes not always correlate with the events. We have concluded that the phenomena are closely related with penetrations toward low-latitudes of electric fields owing to the partial closure of asymmetrical ring currents

  12. Mutations in Cancer Cause Gain of Cysteine, Histidine, and Tryptophan at the Expense of a Net Loss of Arginine on the Proteome Level

    Directory of Open Access Journals (Sweden)

    Viktoriia Tsuber

    2017-07-01

    Full Text Available Accumulation of somatic mutations is critical for the transition of a normal cell to become cancerous. Mutations cause amino acid substitutions that change properties of proteins. However, it has not been studied as to what extent the composition and accordingly chemical properties of the cell proteome is altered as a result of the increased mutation load in cancer. Here, we analyzed data on amino acid substitutions caused by mutations in about 2000 protein coding genes from the Cancer Cell Line Encyclopedia that contains information on nucleotide and amino acid alterations in 782 cancer cell lines, and validated the analysis with information on amino acid substitutions for the same set of proteins in the Catalogue of Somatic Mutations in Cancer (COSMIC; v78 in circa 18,000 tumor samples. We found that nonsynonymous single nucleotide substitutions in the analyzed proteome subset ultimately result in a net gain of cysteine, histidine, and tryptophan at the expense of a net loss of arginine. The extraordinary loss of arginine may be attributed to some extent to composition of its codons as well as to the importance of arginine in the functioning of prominent tumor suppressor proteins like p53.

  13. Signal loss in magnetic resonance imaging caused by intraoral anchored dental magnetic materials

    International Nuclear Information System (INIS)

    Blankenstein, F.H.; Naumann, M.; Truong, B.; Thomas, A.; Schroeder, R.J.

    2006-01-01

    Purpose: to measure the maximum extent of the signal loss areas in the center of the susceptibility artifacts generated by ferromagnetic dental magnet attachments using three different sequences in the 1.5 and 3.0 Tesla MRI. Materials and methods: five different pieces of standard dental magnet attachments with volumes of 6.5 to 31.4 mm 3 were used: a NdFeB magnet with an open magnetic field, a NdFeB magnet with a closed magnetic field, a SmCo magnet with an open magnetic field, a stainless steel keeper (AUM-20) and a PdCo piece. The attachments were placed between two cylindrical phantoms and examined in 1.5 and 3.0 Tesla MRI using gradient echo and T1- and T2-weighted spin echoes. We measured the maximum extent of the generated signal loss areas parallel and perpendicular to the direction of B O . Results: in gradient echoes the artifacts were substantially larger and symmetrically adjusted around the object. The areas with total signal loss were mushroom-like with a maximum extent of 7.4 to 9.7 cm parallel to the direction of B O and 6.7 to 7.4 cm perpendicular to B O . In spin echoes the signal loss areas were obviously smaller, but not centered. The maximum values ranged between 4.9 and 7.2 cm (parallel B O ) and 3.6 and 7.0 cm (perpendicular B O ). The different ferromagnetic attachments had no clinically relevant influence on the signal loss neither in 1.5 T nor 3.0 T MRI. Conclusions: ferromagnetic materials used in dentistry are not intraorally standardized. To ensure, that the area of interest is not affected by the described artifacts, the maximum extent of the signal loss area should be assumed: a radius of up to 7 cm in 1.5 and 3.0 T MRI by T1 and T2 sequences, and a radius of up to 10 cm in T2* sequences. To decide whether magnet attachments have to be removed before MR imaging, physicians should consider both the intact retention of the keepers and the safety distance between the ferromagnetic objects and the area of interest. (orig.)

  14. Winter in the Ouachitas--a severe winter storm signature in Pinus echinata in the Ouachita Mountains of Oklahoma and Arkansas, USA

    Science.gov (United States)

    Douglas J. Stevenson; Thomas B. Lynch; Pradip Saud; Robert Heineman; Randal Holeman; Dennis Wilson; Keith Anderson; Chris Cerny; James M. Guldin

    2016-01-01

    Each year severe winter storms (≈ice storms) damage trees throughout the southern USA. Arkansas and Oklahoma have a history of severe winter storms. To extend that history back beyond the reach of written records, a distinctive tree ring pattern or signature is needed. Storm-caused breakage, branch loss and bending stress provide that signature. We found a severe storm...

  15. Acute quadriplegia caused by necrotizing myopathy in a renal transplant recipient with severe pneumonia: acute onset and complete recovery.

    Science.gov (United States)

    Tu, Guo-Wei; Song, Jie-Qiong; Ting, Simon Kang Seng; Ju, Min-Jie; He, Hong-Yu; Dong, Ji-Hong; Luo, Zhe

    2015-02-03

    Critical illness polyneuropathy and myopathy are multifaceted complications that follow severe illnesses involving the sensorimotor axons and proximal skeletal muscles. These syndromes have rarely been reported among renal transplant recipients. In this paper, we report a case of acute quadriplegia caused by necrotizing myopathy in a renal transplant recipient with severe pneumonia. The muscle strength in the patient's extremities improved gradually after four weeks of comprehensive treatment, and his daily life activities were normal a year after being discharged.

  16. A peptidomimetic targeting white fat causes weight loss and improved insulin resistance in obese monkeys.

    Science.gov (United States)

    Barnhart, Kirstin F; Christianson, Dawn R; Hanley, Patrick W; Driessen, Wouter H P; Bernacky, Bruce J; Baze, Wallace B; Wen, Sijin; Tian, Mei; Ma, Jingfei; Kolonin, Mikhail G; Saha, Pradip K; Do, Kim-Anh; Hulvat, James F; Gelovani, Juri G; Chan, Lawrence; Arap, Wadih; Pasqualini, Renata

    2011-11-09

    Obesity, defined as body mass index greater than 30, is a leading cause of morbidity and mortality and a financial burden worldwide. Despite significant efforts in the past decade, very few drugs have been successfully developed for the treatment of obese patients. Biological differences between rodents and primates are a major hurdle for translation of anti-obesity strategies either discovered or developed in rodents into effective human therapeutics. Here, we evaluate the ligand-directed peptidomimetic CKGGRAKDC-GG-(D)(KLAKLAK)(2) (henceforth termed adipotide) in obese Old World monkeys. Treatment with adipotide induced targeted apoptosis within blood vessels of white adipose tissue and resulted in rapid weight loss and improved insulin resistance in obese monkeys. Magnetic resonance imaging and dual-energy x-ray absorptiometry confirmed a marked reduction in white adipose tissue. At experimentally determined optimal doses, monkeys from three different species displayed predictable and reversible changes in renal proximal tubule function. Together, these data in primates establish adipotide as a prototype in a new class of candidate drugs that may be useful for treating obesity in humans.

  17. Magnitude and causes of loss to follow-up among patients with viral hepatitis at a tertiary care hospital in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Hanan H. Balkhy

    2017-07-01

    Full Text Available Background: Non-adherence with recommended follow-up visits is a major barrier for completing treatment of viral hepatitis and is consequently associated with unfavorable outcomes of health services. Objectives: To estimate the magnitude and identify perceived reasons and patient characteristics associated with loss to follow-up in a tertiary care setting. Methods: A two-step cross-sectional study design was used, including a chart review (2011 followed by phone survey (2012. Loss to follow-up was recorded among those who were diagnosed with hepatitis B (HBV or C (HCV during 2009–2010 but never returned for recommended/scheduled follow-up appointment(s. Results: A total of 328 patients (202 HBV and 126 HCV were included in the current analysis. The average age was 49.6 ± 17.9 years, and 57% were males. Out of 328, 131 (40% were not advised to do follow-up, and 98 (30% were not doing follow-up. Perceived reasons for loss to follow-up were as follows: unaware that a follow-up appointment was scheduled (69%, never informed of need for follow-up by healthcare provider (15%, personal belief that follow-up was not necessary (9%, logistical reasons (3% and other reasons (5%. Loss to follow-up was higher among those who had been diagnosed with HBV, referred by non-liver-related specialty, never advised to follow-up, unaware of their diagnosis, incorrectly identified their type of hepatitis, lacking hepatitis complications, having full medical coverage, pregnant, and those with low knowledge or negative attitude towards hepatitis. Conclusions: Loss to follow-up is a significant problem among patients with hepatitis in a tertiary care center, with several patient and system failures being implicated. Keywords: Viral hepatitis, Loss to follow-up, Tertiary care, Management, Saudi Arabia

  18. Study of the Subjective Visibility of Packet Loss Artifacts in Decoded Video Sequences

    DEFF Research Database (Denmark)

    Korhonen, Jari

    2018-01-01

    Packet loss is a significant cause of visual impairments in video broadcasting over packet-switched networks. There are several subjective and objective video quality assessment methods focused on the overall perception of video quality. However, less attention has been paid on the visibility...... of packet loss artifacts appearing in spatially and temporally limited regions of a video sequence. In this paper, we present the results of a subjective study, using a methodology where a video sequence is displayed on a touchscreen and the users tap it in the positions where they observe artifacts. We...... also analyze the objective features derived from those artifacts, and propose different models for combining those features into an objective metric for assessing the noticeability of the artifacts. The practical results show that the proposed metric predicts visibility of packet loss impairments...

  19. The impact of hearing loss on the quality of life of elderly adults

    Directory of Open Access Journals (Sweden)

    Ciorba A

    2012-06-01

    Full Text Available Andrea Ciorba, Chiara Bianchini, Stefano Pelucchi, Antonio PastoreENT and Audiology Department, University Hospital of Ferrara, Ferrara, ItalyAbstract: Hearing loss is the most common sensory deficit in the elderly, and it is becoming a severe social and health problem. Especially in the elderly, hearing loss can impair the exchange of information, thus significantly impacting everyday life, causing loneliness, isolation, dependence, and frustration, as well as communication disorders. Due to the aging of the population in the developed world, presbycusis is a growing problem that has been reported to reduce quality of life (QoL. Progression of presbycusis cannot be remediated; therefore, optimal management of this condition not only requires early recognition and rehabilitation, but it also should include an evaluation of QoL status and its assessment.Keywords: hearing loss, presbycusis, quality of life, elderly

  20. Intrinsic rotation produced by ion orbit loss and X-loss

    Energy Technology Data Exchange (ETDEWEB)

    Stacey, W. M. [Georgia Institute of Technology, Atlanta, Georgia 30332 (United States); Boedo, J. A. [University of California - San Diego, San Diego, California 92093 (United States); Evans, T. E.; Groebner, R. J. [General Atomics, San Diego, California 92186 (United States); Grierson, B. A. [Princeton Plasma Physics Laboratory, Princeton, New Jersey 08453 (United States)

    2012-11-15

    A practical calculation model for the intrinsic rotation imparted to the edge plasma by the directionally preferential loss of ions on orbits that cross the last closed flux surface is presented and applied to calculate intrinsic rotation in several DIII-D [J. Luxon, Nucl. Fusion 42, 614 (2002)] discharges. The intrinsic rotation produced by ion loss is found to be sensitive to the edge temperature and radial electric field profiles, which has implications for driving intrinsic rotation in future large tokamaks.