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Sample records for glutaric acidemia type

  1. Genetics Home Reference: glutaric acidemia type I

    Science.gov (United States)

    ... Glutaric acidemia type 1 in patients of Lumbee heritage from North Carolina. Mol Genet Metab. 2006 May; ... 825-8. Review. Citation on PubMed More from Genetics Home Reference Bulletins Genetics Home Reference Celebrates Its ...

  2. Magnetic Resonance Imaging Findings of Adult-Onset Glutaric Aciduria Type

    Energy Technology Data Exchange (ETDEWEB)

    Sonmez, G.; Mutlu, H.; Ozturk, E.; Sildiroglu, H.O.; Keskin, A.T.; Basekim, C.C.; Kizilkaya, E. [Dept. of Radiology, GATA Haydarpasa Teaching Hospital, Istanbul (Turkey)

    2007-07-15

    Glutaric aciduria or glutaric acidemia type I, an autosomal recessive disease, usually presents with an acute encephalopathic crisis in young children. We report the magnetic resonance (MR) and proton MR spectroscopy (MRS) imaging findings of a previously healthy 20-year-old man who presented with recurrent headaches. Organic acids from the patient's urine contained large amounts of adipate, glutarate, and 3-hydroxyglutarate consistent with glutaric aciduria type I.

  3. Magnetic Resonance Imaging Findings of Adult-Onset Glutaric Aciduria Type

    International Nuclear Information System (INIS)

    Sonmez, G.; Mutlu, H.; Ozturk, E.; Sildiroglu, H.O.; Keskin, A.T.; Basekim, C.C.; Kizilkaya, E.

    2007-01-01

    Glutaric aciduria or glutaric acidemia type I, an autosomal recessive disease, usually presents with an acute encephalopathic crisis in young children. We report the magnetic resonance (MR) and proton MR spectroscopy (MRS) imaging findings of a previously healthy 20-year-old man who presented with recurrent headaches. Organic acids from the patient's urine contained large amounts of adipate, glutarate, and 3-hydroxyglutarate consistent with glutaric aciduria type I

  4. Magnetic Resonance Imaging Findings of Adult-Onset Glutaric Aciduria Type I

    Energy Technology Data Exchange (ETDEWEB)

    Sonmez, G.; Mutlu, H.; Ozturk, E.; Sildiroglu, H.O.; Keskin, A.T.; Basekim, C.C.; Kizilkaya, E. [Dept. of Radiology, GATA Haydarpasa Teaching Hospital, Istanbul (Turkey)

    2007-07-15

    Glutaric aciduria or glutaric acidemia type I, an autosomal recessive disease, usually presents with an acute encephalopathic crisis in young children. We report the magnetic resonance (MR) and proton MR spectroscopy (MRS) imaging findings of a previously healthy 20-year-old man who presented with recurrent headaches. Organic acids from the patient's urine contained large amounts of adipate, glutarate, and 3-hydroxyglutarate consistent with glutaric aciduria type I.

  5. Glutaric acidemia type II: gene structure and mutations of the electron transfer flavoprotein:ubiquinone oxidoreductase (ETF:QO) gene.

    Science.gov (United States)

    Goodman, Stephen I; Binard, Robert J; Woontner, Michael R; Frerman, Frank E

    2002-01-01

    Glutaric acidemia type II is a human inborn error of metabolism which can be due to defects in either subunit of electron transfer flavoprotein (ETF) or in ETF:ubiquinone oxidoreductase (ETF:QO), but few disease-causing mutations have been described. The ETF:QO gene is located on 4q33, and contains 13 exons. Primers to amplify these exons are presented, together with mutations identified by molecular analysis of 20 ETF:QO-deficient patients. Twenty-one different disease-causing mutations were identified on 36 of the 40 chromosomes.

  6. Recurrent rhabdomyolysis and glutaric aciduria type I: a case report and literature review.

    Science.gov (United States)

    Qian, Gu-Ling; Hong, Fang; Tong, Fan; Fu, Hai-Dong; Liu, Ai-Min

    2016-08-01

    Glutaric acidemia type I (GA-I) is a rare metabolic disorder caused by mutation of the glutaryl- CoA dehydrogenase (GCDH) gene. The occurrence of rhabdomyolysis with GA-I is extremely rare. We reported a child with recurrent rhabdomyolysis and undiagnosed glutaric acidemia type I (GA-I). And a literature review was performed. A 4.5-year-old girl was admitted to our hospital due to recurrent rhabdomyolysis for 3 times within three years. At the third admission, she was diagnosed with GA-I by biochemical testing and mutation analysis. The girl was found to have a serine to leucine replacement mutation of the GCDH gene in exon 8 at position 764. Other three patients with rhabdomyolysis and GA-I were discovered by literature searching. This report highlights that patients with GA-I may have an increased risk of rhabdomyolysis.

  7. Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish.

    OpenAIRE

    Biery, B. J.; Stein, D. E.; Morton, D. H.; Goodman, S. I.

    1996-01-01

    The structure of the human glutaryl coenzyme A dehydrogenase (GCD) gene was determined to contain 11 exons and to span approximately 7 kb. Fibroblast DNA from 64 unrelated glutaric acidemia type I (GA1) patients was screened for mutations by PCR amplification and analysis of SSCP. Fragments with altered electrophoretic mobility were subcloned and sequenced to detect mutations that caused GA1. This report describes the structure of the GCD gene, as well as point mutations and polymorphisms fou...

  8. Glutaric Aciduria type I and acute renal failure — Coincidence or causality?

    Directory of Open Access Journals (Sweden)

    Ben Pode-Shakked

    2014-01-01

    Full Text Available Glutaric Aciduria type I (GA-I is a rare organic acidemia, caused by mutations in the GCDH gene, and characterized by encephalopathic crises with neurological sequelae. We report herein a patient with GA-I who presented with severe acute renal failure requiring dialysis, following an acute diarrheal illness. Histopathological evaluation demonstrated acute tubular necrosis, and molecular diagnosis revealed the patient to be homozygous for a previously unreported mutation, p.E64D. As renal impairment is not part of the clinical spectrum typical to GA-I, possible associations of renal failure and the underlying inborn error of metabolism are discussed, including recent advancements made in the understanding of the renal transport of glutaric acid and its derivatives during metabolic disturbance in GA-I.

  9. Pharmacological evidence for GABAergic and glutamatergic involvement in the convulsant and behavioral effects of glutaric acid.

    Science.gov (United States)

    Lima, T T; Begnini, J; de Bastiani, J; Fialho, D B; Jurach, A; Ribeiro, M C; Wajner, M; de Mello, C F

    1998-08-17

    The effect of intrastriatal administration of glutaric acid (GTR), a metabolite that accumulates in glutaric acidemia type I (GA-I), on the behavior of adult male rats was investigated. After cannula placing, rats received unilateral intrastriatal injections of GTR buffered to pH 7.4 with NaOH or NaCl. GTR induced rotational behavior toward the contralateral side of injection and clonic convulsions in a dose-dependent manner. Rotational behavior was prevented by intrastriatal preadministration of DNQX and muscimol, but not by the preadministration of MK-801. Convulsions were prevented by intrastriatal preinjection of muscimol. This study provides evidence for a participation of glutamatergic non-NMDA and GABAergic mechanisms in the GTR-induced behavioral alterations. These findings may be of value in understanding the physiopathology of the neurological dysfunction in glutaric acidemia.

  10. A fast and simple solid phase microextraction coupled with gas chromatography-triple quadrupole mass spectrometry method for the assay of urinary markers of glutaric acidemias.

    Science.gov (United States)

    Naccarato, Attilio; Gionfriddo, Emanuela; Elliani, Rosangela; Sindona, Giovanni; Tagarelli, Antonio

    2014-10-30

    The analysis of characteristic urinary acidic markers such as glutaric, 3-hydroxyglutaric, 2-hydroxyglutaric, adipic, suberic, sebacic, ethylmalonic, 3-hydroxyisovaleric and isobutyric acid constitutes the recommended follow-up testing procedure for glutaric acidemia type 1 (GA-1) and type 2 (GA-2). The goal of the work herein presented is the development of a fast and simple method for the quantification of these biomarkers in human urine. The proposed analytical approach is based on the use of solid phase microextraction (SPME) combined with gas chromatography-triple quadrupole mass spectrometry (GC-QqQ-MS) afterward a rapid derivatization of acidic moieties by propyl chloroformate, propanol and pyridine. Trueness and precision of the proposed protocol, tested at 5, 30 and 80mgl -1 , provided satisfactory values: recoveries were in the range between 72% and 116% and the relative standard deviations (RSD%) were between 0.9% and 18% (except for isobutyric acid at 5mgl -1 ). The LOD values achieved by the proposed method ranged between 1.0 and 473μgl -1 . Copyright © 2014 Elsevier B.V. All rights reserved.

  11. Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish.

    Science.gov (United States)

    Biery, B J; Stein, D E; Morton, D H; Goodman, S I

    1996-11-01

    The structure of the human glutaryl coenzyme A dehydrogenase (GCD) gene was determined to contain 11 exons and to span approximately 7 kb. Fibroblast DNA from 64 unrelated glutaric acidemia type I (GA1) patients was screened for mutations by PCR amplification and analysis of SSCP. Fragments with altered electrophoretic mobility were subcloned and sequenced to detect mutations that caused GA1. This report describes the structure of the GCD gene, as well as point mutations and polymorphisms found in 7 of its 11 exons. Several mutations were found in more than one patient, but no one prevalent mutation was detected in the general population. As expected from pedigree analysis, a single mutant allele causes GA1 in the Old Order Amish of Lancaster County, Pennsylvania. Several mutations have been expressed in Escherichia coli, and all produce diminished enzyme activity. Reduced activity in GCD encoded by the A421V mutation in the Amish may be due to impaired association of enzyme subunits.

  12. Glutaric aciduria type 1--importance of early diagnosis and treatment.

    Science.gov (United States)

    Afroze, Bushra; Yunus, Zabedah Mohammad

    2014-05-01

    Glutaric aciduria type 1 is a rare inherited organic academia. Untreated patients characteristically develop dystonia secondary to striatal injury during early childhood, which results in high morbidity and mortality. In patients diagnosed during neonatal period, striatal injury can be prevented by metabolic treatment including low lysine diet, carnitine supplementation and aggressive emergency treatment during acute episode of inter current illnesses. However, after the onset of neurological damage initiation of treatment is generally not effective. Therefore; glutaric aciduria type 1 is included in newborn screening panel for inherited metabolic diseases in many countries. We describe two children in a family with glutaric aciduria type 1 and their different long term outcomes. The first child was diagnosed late leading to severe neurological damage. The second child was diagnosed in the neonatal period as a result of selective high-risk screening and was treated appropriately giving a normal growth.

  13. Glutaric aciduria type 1: neuroimaging features with clinical correlation

    Energy Technology Data Exchange (ETDEWEB)

    Mohammad, Shaimaa Abdelsattar; Ahmed, Khaled A. [Ain-Shams University, Department of Radiodiagnosis, Faculty of Medicine, Cairo (Egypt); Abdelkhalek, Heba Salah; Zaki, Osama K. [Ain-Shams University, Medical Genetics Unit, Pediatric Department, Faculty of Medicine, Cairo (Egypt)

    2015-10-15

    Glutaric aciduria type 1 is a rare neurometabolic disease with high morbidity. To describe the MR imaging abnormalities in glutaric aciduria type 1 and to identify any association between the clinical and imaging features. MRI scans of 29 children (mean age: 16.9 months) with confirmed diagnosis of glutaric aciduria type 1 were retrospectively reviewed. Gray matter and white matter scores were calculated based on a previously published pattern-recognition approach of assessing leukoencephalopathies. Hippocampal formation and opercular topography were assessed in relation to the known embryological basis. MRI scores were correlated with morbidity score. The most consistent MRI abnormality was widened operculum with dilatation of the subarachnoid spaces surrounding underdeveloped frontotemporal lobes. Incomplete hippocampal inversion was also seen. The globus pallidus was the most frequently involved gray matter structure (86%). In addition to the central tegmental tract, white matter abnormalities preferentially involved the central and periventricular regions. The morbidity score correlated with the gray matter abnormality score (P = 0.004). Patients with dystonia had higher gray matter and morbidity scores. Morbidity is significantly correlated with abnormality of gray matter, rather than white matter, whether secondary to acute encephalopathic crisis or insidious onset disease. (orig.)

  14. Glutaric aciduria type I

    International Nuclear Information System (INIS)

    Mandel, H.; Berant, M.; Braun, J.; Technion-Israel Inst. of Tech., Haifa; El-Peleg, O.; Christensen, E.

    1991-01-01

    Serial CT findings in an infant with glutaric aciduria type I (GA-I) are reported. The major CT features were dilatation of the insular cisterns, regression of the temporal lobes, with 'bat wings' dilatation of the Sylvian fissures and hypodensity of the lenticular nuclei. CT changes preceded the onset of symptoms by 3 months. An improvement in the temporal lobe atrophy was seen after a period of treatment, coinciding with marked clinical improvement. A peculiar feature was the presence of external hydrocephalus, which diverted the attention from manifestations of the primary disease and thus constituted a diagnostic pitfall. The delineation and recognition of the characteristic radiologic manifestations of GA-I are essential for allowing an adequate radiologist/clinician interaction in diagnosing this inborn error of metabolism. (orig.)

  15. Occurrence of subdural hematomas in Dutch glutaric aciduria type 1 patients

    NARCIS (Netherlands)

    Vester, Marloes E M; Visser, Gepke; Wijburg, Frits A.; van Spronsen, Francjan J.; Williams, Monique; van Rijn, Rick R.

    2016-01-01

    Patients with glutaric aciduria type 1 (GA1), a rare inherited metabolic disorder, have an increased risk for subdural hematomas (SDHs). GA1 is therefore generally included in the differential diagnosis of children presenting with SDHs. This retrospective cohort study reviews all 25 registered, in

  16. Occurrence of subdural hematomas in Dutch glutaric aciduria type 1 patients

    NARCIS (Netherlands)

    Vester, Marloes E. M.; Visser, Gepke; Wijburg, Frits A.; van Spronsen, Francjan J.; Williams, Monique; van Rijn, Rick R.

    Patients with glutaric aciduria type 1 (GA1), a rare inherited metabolic disorder, have an increased risk for subdural hematomas (SDHs). GA1 is therefore generally included in the differential diagnosis of children presenting with SDHs. This retrospective cohort study reviews all 25 registered, in

  17. Occurrence of subdural hematomas in Dutch glutaric aciduria type 1 patients

    NARCIS (Netherlands)

    Vester, M.E.M. (Marloes E.M.); G. Visser (G.); F.A. Wijburg (Frits); F.J. van Spronsen; M. Williams (Martine); R.R. van Rijn (Rick)

    2016-01-01

    textabstractPatients with glutaric aciduria type 1 (GA1), a rare inherited metabolic disorder, have an increased risk for subdural hematomas (SDHs). GA1 is therefore generally included in the differential diagnosis of children presenting with SDHs. This retrospective cohort study reviews all 25

  18. Novel contiguous gene deletion in peruvian girl with Trichothiodystrophy type 4 and glutaric aciduria type 3.

    Science.gov (United States)

    La Serna-Infantes, Jorge; Pastor, Miguel Chávez; Trubnykova, Milana; Velásquez, Félix Chavesta; Sotomayor, Flor Vásquez; Barriga, Hugo Abarca

    2018-02-05

    Trichothiodystrophy type 4 is a rare autosomal recessive and ectodermal disorder, characterized by dry, brittle, sparse and sulfur-deficient hair and other features like intellectual disability, ichthyotic skin and short stature, caused by a homozygous mutation in MPLKIP gene. Glutaric aciduria type 3 is caused by a homozygous mutation in SUGCT gene with no distinctive phenotype. Both genes are localized on chromosome 7 (7p14). We report an 8-year-old female with short stature, microcephaly, development delay, intellectual disability and hair characterized for dark, short, coarse, sparse and brittle associated to classical trichorrhexis microscopy pattern. Chromosome microarray analysis showed a 125 kb homozygous pathogenic deletion, which includes genes MPLKIP and SUGCT, not described before. This is the first case described in Peru of a novel contiguous gene deletion of Trichothiodystrophy type 4 and Glutaric aciduria type 3 performed by chromosome microarray analysis, highlighting the contribution and importance of molecular technologies on diagnosis of rare genetic conditions. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

  19. A review of patients with glutaric aciduria type 1 at Inkosi Albert ...

    African Journals Online (AJOL)

    Glutaric aciduria type 1 (GA1) is a rare, autosomal-recessive organic acidaemia. It is caused by deficiency of glutaryl-co-enzyme A (CoA) dehydrogenase (GCDH) resulting from a mutation in the GCDH gene on chromosome 19p13.2. There are 108 known disease-causing mutations in the Human Gene Mutation Database.

  20. Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

    NARCIS (Netherlands)

    Vester, Marloes E. M.; Bilo, Rob A. C.; Karst, Wouter A.; Daams, Joost G.; Duijst, Wilma L. J. M.; van Rijn, Rick R.

    2015-01-01

    Glutaric aciduria type 1 (GA1) is a rare metabolic disorder of glutaryl-CoA-dehydrogenase enzyme deficiency. Children with GA1 are reported to be predisposed to subdural hematoma (SDH) development due to stretching of cortical veins secondary to cerebral atrophy and expansion of CSF spaces.

  1. Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

    NARCIS (Netherlands)

    Vester, M.E.; Bilo, R.A.; Karst, W.A.; Daams, J.G.; Duijst, W.L.J.M.; Rijn, R.R. van

    2015-01-01

    PURPOSE: Glutaric aciduria type 1 (GA1) is a rare metabolic disorder of glutaryl-CoA-dehydrogenase enzyme deficiency. Children with GA1 are reported to be predisposed to subdural hematoma (SDH) development due to stretching of cortical veins secondary to cerebral atrophy and expansion of CSF spaces.

  2. Cerebral H-1 MR spectroscopy revealing white matter NAA decreases in glutaric aciduria type I

    NARCIS (Netherlands)

    Sijens, P. E.; Smit, G. P. A.; Meiners, L. C.; Oudkerk, M.; van Spronsen, F. J.

    MR spectroscopy in two patients with glutaric aciduria type I revealed reductions in the white matter N-acetylaspartate signal, in the more severe case accompanied by a loss of glutamate and the appearance of lactate signals. (c) 2006 Elsevier Inc. All rights reserved.

  3. Screening of a healthy newborn identifies three adult family members with symptomatic glutaric aciduria type I

    Directory of Open Access Journals (Sweden)

    MCH Janssen

    2014-06-01

    Full Text Available We report three adult sibs (one female, two males with symptomatic glutaric acidura type I, who were diagnosed after a low carnitine level was found by newborn screening in a healthy newborn of the women. All three adults had low plasma carnitine, elevated glutaric acid levels and pronounced 3-hydroxyglutaric aciduria. The diagnosis was confirmed by undetectable glutaryl-CoA dehydrogenase activity in lymphocytes and two pathogenic heterozygous mutations in the GCDH gene (c.1060A>G, c.1154C>T. These results reinforce the notion that abnormal metabolite levels in newborns may lead to the diagnosis of adult metabolic disease in the mother and potentially other family members.

  4. Acute Pancreatitis with Rapid Clinical Improvement in a Child with Isovaleric Acidemia

    Directory of Open Access Journals (Sweden)

    Elpis Mantadakis

    2013-01-01

    Full Text Available Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancreatitis should be ruled out in the context of vomiting in any patient with isovaleric acidemia. Conversely, branched-chain organic acidemias should be included in the differential diagnosis of any child with pancreatitis of unknown origin.

  5. Glutaric aciduria type I: a common cause of episodic encephalopathy and spastic paralysis in the Amish of Lancaster County, Pennsylvania.

    Science.gov (United States)

    Morton, D H; Bennett, M J; Seargeant, L E; Nichter, C A; Kelley, R I

    1991-10-01

    We have diagnosed type I glutaric aciduria (GA-I) in 14 children from 7 Old Order Amish families in Lancaster County, Pennsylvania. An otherwise rare disorder, GA-I appears to be a common cause of acute encephalopathy and cerebral palsy among the Amish. The natural history of the disease, which was previously unrecognized in this population, is remarkably variable and ranges from acute infantile encephalopathy and sudden death to static extrapyramidal cerebral palsy to normal adult. Ten patients first manifested the disease between 3 and 18 months at the time of an acute infectious illness. Four of these children died in early childhood, also during acute illnesses. However, there has been little progression of the neurological disease after age 5 years in the surviving children and intellect usually has been preserved, even in children with severe spastic paralysis. When well, patients have plasma glutaric acid concentrations ranging from 4.8 to 14.2 mumol/liter (nl 0-5.6 mumol/liter) and urinary glutaric acid concentrations from 12.5 to 196 mg/g creatinine (nl 0.5-8.4 mg/g creatinine). We have found that GA-I can be diagnosed in the Amish by measurement of urinary glutaric acid concentrations using isotope-dilution gas chromatography/mass spectrometry, whereas the diagnosis can easily be missed by routine urine organic acid gas chromatography.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. Assignment of electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO) to human chromosome 4q33 by fluorescence in situ hybridization and somatic cell hybridization.

    Science.gov (United States)

    Spector, E B; Seltzer, W K; Goodman, S I

    1999-08-01

    Electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO) is a nuclear-encoded protein located in the inner mitochondrial membrane. Inherited defects of ETF-QO cause glutaric acidemia type II. We here describe the localization of the ETF-QO gene to human chromosome 4q33 by somatic cell hybridization and fluorescence in situ hybridization. Copyright 1999 Academic Press.

  7. A case of glutaric aciduria type I with unique abnormalities in the cerebral CT findings

    International Nuclear Information System (INIS)

    Yamaguchi, Seiji; Orii, Tadao; Yasuda, Kanji; Kohno, Yoshinori

    1987-01-01

    A first Japanese case of glutaric aciduria type I (GA-I) was described. She was a 7-month-old girl presenting with poor head control, irritability and sleeplessness. The profile of urinary organic acids by gas chromatography mass spectrometry (GC/MS) suggesting GA-I were confirmed by no activity of glutaryl-CoA dehydrogenase in the fibroblasts. The cerebral computer tomography (CT) showed marked changes such as large fluid collections on bilateral frontotemporal regions and a slight enlargement of bilateral ventricles. The amounts of urinary glutarate excretion decreased after restriction of lysine and tryptophan in her diet and administration of carnitine improved the carnitine levels in blood and urine, while these were less effective for the neurological symptoms. On the other hand, oral administration of lioresal, an analogue of gamma-aminobutyrate (GABA), cleared her symptoms such as ill temper, irritability and sleeplessness dramatically, and the abnormalities of the CT examinations were not more deteriorative until 2 years of her age at least. The neurological manifestations of GA-I seemed to be affected by the unusual metabolism of GABA in the central nervous system. (author)

  8. Severe metabolic or mixed acidemia on intensive care unit admission: incidence, prognosis and administration of buffer therapy. A prospective, multiple-center study.

    Science.gov (United States)

    Jung, Boris; Rimmele, Thomas; Le Goff, Charlotte; Chanques, Gérald; Corne, Philippe; Jonquet, Olivier; Muller, Laurent; Lefrant, Jean-Yves; Guervilly, Christophe; Papazian, Laurent; Allaouchiche, Bernard; Jaber, Samir

    2011-01-01

    In this study, we sought describe the incidence and outcomes of severe metabolic or mixed acidemia in critically ill patients as well as the use of sodium bicarbonate therapy to treat these illnesses. We conducted a prospective, observational, multiple-center study. Consecutive patients who presented with severe acidemia, defined herein as plasma pH below 7.20, were screened. The incidence, sodium bicarbonate prescription and outcomes of either metabolic or mixed severe acidemia were analyzed. Among 2, 550 critically ill patients, 200 (8%) presented with severe acidemia, and 155 (6% of the total admissions) met the inclusion criteria. Almost all patients needed mechanical ventilation and vasopressors during their ICU stay, and 20% of them required renal replacement therapy within the first 24 hours of their ICU stay. Severe metabolic or mixed acidemia was associated with a mortality rate of 57% in the ICU. Delay of acidemia recovery as opposed to initial pH value was associated with increased mortality in the ICU. The type of acidemia did not influence the decision to administer sodium bicarbonate. The incidence of severe metabolic or mixed acidemia in critically ill patients was 6% in the present study, and it was associated with a 57% mortality rate in the ICU. In contradistinction with the initial acid-base parameters, the rapidity of acidemia recovery was an independent risk factor for mortality. Sodium bicarbonate prescription was very heterogeneous between ICUs. Further studies assessing specific treatments may be of interest in this population.

  9. Acute Pancreatitis with Rapid Clinical Improvement in a Child with Isovaleric Acidemia

    OpenAIRE

    Mantadakis, Elpis; Chrysafis, Ioannis; Tsouvala, Emmanouela; Evangeliou, Athanassios; Chatzimichael, Athanassios

    2013-01-01

    Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L...

  10. Severe metabolic or mixed acidemia on intensive care unit admission: incidence, prognosis and administration of buffer therapy. a prospective, multiple-center study

    Science.gov (United States)

    2011-01-01

    Introduction In this study, we sought describe the incidence and outcomes of severe metabolic or mixed acidemia in critically ill patients as well as the use of sodium bicarbonate therapy to treat these illnesses. Methods We conducted a prospective, observational, multiple-center study. Consecutive patients who presented with severe acidemia, defined herein as plasma pH below 7.20, were screened. The incidence, sodium bicarbonate prescription and outcomes of either metabolic or mixed severe acidemia were analyzed. Results Among 2, 550 critically ill patients, 200 (8%) presented with severe acidemia, and 155 (6% of the total admissions) met the inclusion criteria. Almost all patients needed mechanical ventilation and vasopressors during their ICU stay, and 20% of them required renal replacement therapy within the first 24 hours of their ICU stay. Severe metabolic or mixed acidemia was associated with a mortality rate of 57% in the ICU. Delay of acidemia recovery as opposed to initial pH value was associated with increased mortality in the ICU. The type of acidemia did not influence the decision to administer sodium bicarbonate. Conclusions The incidence of severe metabolic or mixed acidemia in critically ill patients was 6% in the present study, and it was associated with a 57% mortality rate in the ICU. In contradistinction with the initial acid-base parameters, the rapidity of acidemia recovery was an independent risk factor for mortality. Sodium bicarbonate prescription was very heterogeneous between ICUs. Further studies assessing specific treatments may be of interest in this population. PMID:21995879

  11. Disease: H01400 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available cts [DS:H00525]; Glutaric acidemia [DS:H00178]; Lysinuric protein intolerance [DS:H00899]; Pyruvate carboxyl...aired by substrate deficiencies assumed cause in various disorders including lysinuric protein intolerance,

  12. Autism in patients with propionic acidemia

    NARCIS (Netherlands)

    Witters, P.; Debbold, E.; Crivelly, K.; Kerckhove, K. Vande; Corthouts, K.; Debbold, B.; Andersson, H.; Vannieuwenborg, L.; Geuens, S.; Baumgartner, M.; Kozicz, L.T.; Settles, L.; Morava, E.

    2016-01-01

    Certain inborn errors of metabolism have been suggested to increase the risk of autistic behavior. In an animal model, propionic acid ingestion triggered abnormal behavior resembling autism. So far only a few cases were reported with propionic acidemia and autistic features. From a series of twelve

  13. Demographic and clinical features of glutaric acidemia type 1; a high ...

    African Journals Online (AJOL)

    Osama K. Zaki

    2014-02-01

    Feb 1, 2014 ... onset of symptoms, macrocephaly (85%) was the commonest feature of GAI followed by ... Health in Egypt for management of patients with metabolic dis- orders ..... in the national screening program for hypothyroidism may be.

  14. Stabilization of blood methylmalonic acid level in methylmalonic acidemia after liver transplantation.

    Science.gov (United States)

    Chen, P W; Hwu, W L; Ho, M C; Lee, N C; Chien, Y H; Ni, Y H; Lee, P H

    2010-05-01

    Methylmalonic acidemia with complete mutase deficiency (mut(0) type) is an inborn error of metabolism with high mortality and morbidity. LT has been suggested to be a solution to this disease, but elevation of urinary and blood MMA was still observed after LT. In this study, we measured dry blood spot MMA and its precursor propionyl-carnitine (C3-carnitine) for mut(0) patients. The results revealed that when C3-carnitine rose during metabolic stress, MMA rose exponentially (up to 1000 micromol/L) in patients who did not undergo LT. In patients who underwent LT, MMA rose to 100-200 micromol/L when C3-carnitine reached 10-20 micromol/L. However, when C3-carnitine rose further to 40-50 micromol/L, MMA levels just stayed put. Therefore, LT stabilized blood MMA level, though there might be a threshold for blood MMA clearance by the donor liver. This finding should be critical to understand the long-term outcome for LT in methylmalonic acidemia.

  15. The long-term treatment of a patient with type 1 diabetes mellitus and glutaric aciduria type 1: the effect of insulin.

    Science.gov (United States)

    Del Rizzo, Monica; Galderisi, Alfonso; Celato, Andrea; Furlan, Francesca; Giordano, Laura; Cazzorla, Chiara; Fasan, Ilaria; Moretti, Carlo; Zschocke, Johannes; Burlina, Alberto B

    2016-08-01

    The coexistence of two diseases associated with different metabolic disorders is a very rare event. Some associations, although sporadic, can be particularly challenging both in terms of diagnostic and therapeutic management and in terms of theoretical perspective. Here, we report a child affected by type 1 diabetes mellitus (T1DM) and glutaric aciduria type 1 (GA1). The child was diagnosed with classical T1DM at 15 months of age, with a tendency toward hypoglycemia. A few months later, during an acute intercurrent infective episode, the child displayed acute hypotonia of the lower limbs and limbs dystonia. A brain MRI showed bilateral striatal necrosis, suggesting GA1 diagnosis. Treatment with a low-lysine dietary regimen and carnitine supplementation was started and resulted in an improvement in metabolic control and a reduction of hypoglycemic episodes along with an increasing in insulin daily dose. After 2 years, the neurological outcome consisted of a reduction in dystonic movements and a metabolic stability of both diseases. This case provides some insight into the reciprocal interconnections between the two metabolic disorders. Similar pathogenic mechanisms responsible for the neuronal injury might have impacted each other, and a strict relationship between a specific aspect of GA1-impaired metabolism and glucose homeostasis might explain how the tailored management of GA1 was not only effective in controlling the disease, but it also resulted in an improvement in the control of the glycemic profile. What in known: • Glutaric aciduria type 1 (GA1) usually presents in childhood with severe and possibly irreversible neuronal damage, triggered by a catabolic stress • The association of GA1 with other diseases, including type 1 diabetes mellitus (T1DM), is a rare event, complicating the treatment management What is new: • Insulin treatment has a role in preventing GA1 metabolic decompensation, even in the catabolic condition of hypoglycemia • Promoting

  16. Long-term continuous N-carbamylglutamate treatment in frequently decompensated propionic acidemia: a case report.

    Science.gov (United States)

    Tummolo, Albina; Melpignano, Livio; Carella, Antonella; Di Mauro, Anna Maria; Piccinno, Elvira; Vendemiale, Marcella; Ortolani, Federica; Fedele, Stefania; Masciopinto, Maristella; Papadia, Francesco

    2018-04-22

    Propionic acidemia is a rare autosomal recessive inherited metabolic disorder that can inhibit the synthesis of N-acetylglutamate, the obligatory activator in urea synthesis, leading to hyperammonemia. N-carbamylglutamate ameliorates hyperammonemia in decompensated propionic acidemia. The effects of long-term continuous N-acetylglutamate administration in such patients are unknown. We report our clinical experience with continuous administration of N-acetylglutamate for 6 years in a patient with propionic acidemia frequently presenting with hyperammonemia. A male Caucasian patient with frequently decompensated propionic acidemia and hyperammonemia was admitted 78 times for acute attacks during the first 9 years of his life. Continuous daily treatment with oral N-carbamylglutamate 100 mg/kg (50 mg/kg after 6 months) was initiated. During 6 years of treatment, he had a significant decrease in his mean plasma ammonia levels (75.7 μmol/L vs. 140.3 μmol/L before N-carbamylglutamate therapy, p N-acetylglutamate administration outside the emergency setting. If this observation is confirmed, future studies should aim to optimize the dosage and explore effects of the dosage requirements on other drugs and on protein tolerance.

  17. Simultaneous analysis of amino acid and organic acid by NMR spectrometry, 2. Diagnostic aids for inborn error of metabolism

    Energy Technology Data Exchange (ETDEWEB)

    Koda, Naoya; Yamaguchi, Shuichi; Mori, Takeshi.

    1987-09-01

    Analysis of urine from patients with inborn error of metabolism were studied by /sup 1/H-nuclear magnetic resonance (NMR) spectrometry. Diseases studied were as follows; phenylketonuria, biotin responsive multiple carboxylase deficiency, non-ketotic hyperglycinemia, 3-ketothiolase deficiency, alkaptonuria, methylmalonic acidemia, isovaleric acidemia, glutaric aciduria, argininosuccinic aciduria and hyperornithinemia. In each disease, specific metabolites in urine were recognized by NMR spectrometry. This method is accomplished within 10 minutes with non-treated small volume of urine and will be successfully available for the screening andor diagnosis of inherited metabolic diseases of amino acid and organic acid.

  18. Compound heterozygous mutations in electron transfer flavoprotein dehydrogenase identified in a young Chinese woman with late-onset glutaric aciduria type II

    OpenAIRE

    Xue, Ying; Zhou, Yun; Zhang, Keqin; Li, Ling; Kayoumu, Abudurexiti; Chen, Liye; Wang, Yuhui; Lu, Zhiqiang

    2017-01-01

    Background Glutaric aciduria type II (GA II) is an autosomal recessive disorder affecting fatty acid and amino acid metabolism. The late-onset form of GA II disorder is almost exclusively associated with mutations in the electron transfer flavoprotein dehydrogenase (ETFDH) gene. Till now, the clinical features of late-onset GA II vary widely and pose a great challenge for diagnosis. The aim of the current study is to characterize the clinical phenotypes and genetic basis of a late-onset GAII ...

  19. Structural and vibrational spectral investigations of melaminium glutarate monohydrate by FTIR, FT-Raman and DFT methods

    Science.gov (United States)

    Arjunan, V.; Marchewka, M. K.; Raj, Arushma; Yang, Haifeng; Mohan, S.

    2015-01-01

    Melaminium glutarate monohydrate has been synthesised and FTIR and FT-Raman spectral investigations are carried out. The molecular geometry and vibrational frequencies of melaminium glutarate monohydrate in the ground state have been determined by using B3LYP method with 6-31++G**, 6-31++G and cc-pVDZ basis sets. The stability of the system, inter molecular hydrogen bonding and the electron donor-acceptor interactions of the complex have been investigated by using natural bonding orbital analysis. It reveals that the Nsbnd H⋯O and Osbnd H⋯O intermolecular interactions significantly influence crystal packing of this molecular complex. The glutarate anion forms hydrogen bonds to the melaminium cation as the proton donor of the type Nsbnd H⋯O with a distance (N⋯O) = 2.51 Å. It is also linked by other hydrogen bonds to the water molecule of the type Osbnd H⋯O with (O⋯O) = 2.82 Å and to the amino (sbnd NH2) group of melaminium cation of the type Nsbnd H⋯O with (N⋯O) = 2.82 Å as the proton acceptor. The electrostatic potential of the complex is in the range +1.892e × 10-2 to -1.892e × 10-2. The limits of total electron density of the complex is +6.679e × 10-2 to -6.679e × 10-2.

  20. Structural and vibrational spectral investigations of melaminium glutarate monohydrate by FTIR, FT-Raman and DFT methods.

    Science.gov (United States)

    Arjunan, V; Marchewka, M K; Raj, Arushma; Yang, Haifeng; Mohan, S

    2015-01-25

    Melaminium glutarate monohydrate has been synthesised and FTIR and FT-Raman spectral investigations are carried out. The molecular geometry and vibrational frequencies of melaminium glutarate monohydrate in the ground state have been determined by using B3LYP method with 6-31++G(**), 6-31++G and cc-pVDZ basis sets. The stability of the system, inter molecular hydrogen bonding and the electron donor-acceptor interactions of the complex have been investigated by using natural bonding orbital analysis. It reveals that the N-H⋯O and O-H⋯O intermolecular interactions significantly influence crystal packing of this molecular complex. The glutarate anion forms hydrogen bonds to the melaminium cation as the proton donor of the type N-H⋯O with a distance (N⋯O)=2.51 Å. It is also linked by other hydrogen bonds to the water molecule of the type O-H⋯O with (O⋯O)=2.82 Å and to the amino (NH2) group of melaminium cation of the type N-H⋯O with (N⋯O)=2.82 Å as the proton acceptor. The electrostatic potential of the complex is in the range +1.892e×10(-2) to -1.892e×10(-2). The limits of total electron density of the complex is +6.679e×10(-2) to -6.679e×10(-2). Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Simultaneous analysis of amino acid and organic acid by NMR spectrometry, 2

    International Nuclear Information System (INIS)

    Koda, Naoya; Yamaguchi, Shuichi; Mori, Takeshi.

    1987-01-01

    Analysis of urine from patients with inborn error of metabolism were studied by 1 H-nuclear magnetic resonance (NMR) spectrometry. Diseases studied were as follows; phenylketonuria, biotin responsive multiple carboxylase deficiency, non-ketotic hyperglycinemia, 3-ketothiolase deficiency, alkaptonuria, methylmalonic acidemia, isovaleric acidemia, glutaric aciduria, argininosuccinic aciduria and hyperornithinemia. In each disease, specific metabolites in urine were recognized by NMR spectrometry. This method is accomplished within 10 minutes with non-treated small volume of urine and will be successfully available for the screening and/or diagnosis of inherited metabolic diseases of amino acid and organic acid. (author)

  2. Isovaleric acidemia: an integrated approach toward predictive laboratory medicine

    NARCIS (Netherlands)

    Dercksen, M.

    2014-01-01

    Isovaleric acidemia (IVA) is an autosomal recessive disorder caused by isovaleryl-CoA dehydrogenase (IVD) deficiency (E.C.1.2.99.10) and patients present with a heterogeneous phenotype, ranging from metabolically mild and/or intermittent to metabolically severe. A cohort of ten IVA patients with a

  3. Evaporation of methyl- and dimethyl-substituted malonic, succinic, glutaric and adipic acid particles at ambient temperatures

    DEFF Research Database (Denmark)

    Mønster, Jacob Garbrecht; Rosenørn, Thomas; Svenningsson, Birgitta

    2004-01-01

    Evaporation; organic aerosols; vapor pressure; dicarboxylic acid; maonic acid; succinic acid; glutaric acid; adipic acid......Evaporation; organic aerosols; vapor pressure; dicarboxylic acid; maonic acid; succinic acid; glutaric acid; adipic acid...

  4. De Novo Biosynthesis of Glutarate via α-Keto Acid Carbon Chain Extension and Decarboxylation Pathway in Escherichia coli.

    Science.gov (United States)

    Wang, Jian; Wu, Yifei; Sun, Xinxiao; Yuan, Qipeng; Yan, Yajun

    2017-10-20

    Microbial based bioplastics are promising alternatives to petroleum based synthetic plastics due to their renewability and economic feasibility. Glutarate is one of the most potential building blocks for bioplastics. The recent biosynthetic routes for glutarate were mostly based on the l-lysine degradation pathway from Pseudomonas putida that required lysine either by feeding or lysine overproduction via genetic manipulations. Herein, we established a novel glutarate biosynthetic pathway by incorporation of a "+1" carbon chain extension pathway from α-ketoglutarate (α-KG) in combination with α-keto acid decarboxylation pathway in Escherichia coli. Introduction of homocitrate synthase (HCS), homoaconitase (HA) and homoisocitrate dehydrogenase (HICDH) from Saccharomyces cerevisiae into E. coli enabled "+1" carbon extension from α-KG to α-ketoadipate (α-KA), which was subsequently converted into glutarate by a promiscuous α-keto acid decarboxylase (KivD) and a succinate semialdehyde dehydrogenase (GabD). The recombinant E. coli coexpressing all five genes produced 0.3 g/L glutarate from glucose. To further improve the titers, α-KG was rechanneled into carbon chain extension pathway via the clustered regularly interspersed palindromic repeats system mediated interference (CRISPRi) of essential genes sucA and sucB in tricarboxylic acid (TCA) cycle. The final strain could produce 0.42 g/L glutarate, which was increased by 40% compared with the parental strain.

  5. Interaction of glutaric aciduria type 1-related glutaryl-CoA dehydrogenase with mitochondrial matrix proteins.

    Directory of Open Access Journals (Sweden)

    Jessica Schmiesing

    Full Text Available Glutaric aciduria type 1 (GA1 is an inherited neurometabolic disorder caused by mutations in the GCDH gene encoding glutaryl-CoA dehydrogenase (GCDH, which forms homo- and heteromeric complexes in the mitochondrial matrix. GA1 patients are prone to the development of encephalopathic crises which lead to an irreversible disabling dystonic movement disorder. The clinical and biochemical manifestations of GA1 vary considerably and lack correlations to the genotype. Using an affinity chromatography approach we report here for the first time on the identification of mitochondrial proteins interacting directly with GCDH. Among others, dihydrolipoamide S-succinyltransferase (DLST involved in the formation of glutaryl-CoA, and the β-subunit of the electron transfer flavoprotein (ETFB serving as electron acceptor, were identified as GCDH binding partners. We have adapted the yellow fluorescent protein-based fragment complementation assay and visualized the oligomerization of GCDH as well as its direct interaction with DLST and ETFB in mitochondria of living cells. These data suggest that GCDH is a constituent of multimeric mitochondrial dehydrogenase complexes, and the characterization of their interrelated functions may provide new insights into the regulation of lysine oxidation and the pathophysiology of GA1.

  6. Postnatal and antenatal laboratory diagnosis of glutaric aciduria II in a South African family

    International Nuclear Information System (INIS)

    Henderson, H.E.; Balla, R.; De Jong, G.; Piek, C.J.; Mienie, L.J.; Erasmus, E.

    1987-01-01

    Glutaric aciduria type II (Ga II) was proved in a neonate who presented shortly after birth with respiratory distress, metabolic acidosis, non-ketotic hypoglycaemia and a sweaty-feet-like odour. The diagnosis was based on elevated levels of glutaric and other acids in the urine and on studies on cultured skin fibroblasts where defective metabolism of fatty acids of varying chain length was demonstrated. Antenatal diagnosis was performed on a subsequent pregnancy in this family where an abnormal amniotic fluid organic acid profile together with defective fatty acid oxidation in cultured amnion cells was indicative of GA II in the fetus. A measure of efficency of butyrate oxidation was obtained by analysis of the amount of 14 C label incorporated into trichloro-acetic acid precipitable macromolecules. The incorporation of ( 3 H) leucine was taken as a general indicator of metabolism in the cell population. This is the first report of this genetic disorder in a South African family and it should be considered in suspected organic acidaemia in the neonatal period

  7. Thermal analysis, X-ray powder diffraction and electron microscopy data related with the production of 1:1 Caffeine:Glutaric Acid cocrystals

    Directory of Open Access Journals (Sweden)

    Íris Duarte

    2016-09-01

    Full Text Available The data presented in this article are related to the production of 1:1 Caffeine:Glutaric Acid cocrystals as part of the research article entitled “Green production of cocrystals using a new solvent-free approach by spray congealing” (Duarte et al., 2016 [1]. More specifically, here we present the thermal analysis and the X-ray powder diffraction data for pure Glutaric Acid, used as a raw material in [1]. We also include the X-ray powder diffraction and electron microscopy data obtained for the 1:1 Caffeine:Glutaric Acid cocrystal (form II produced using the cooling crystallization method reported in “Operating Regions in Cooling Cocrystallization of Caffeine and Glutaric Acid in Acetonitrile” (Yu et al., 2010 [2]. Lastly, we show the X-ray powder diffraction data obtained for assessing the purity of the 1:1 Caffeine:Glutaric cocrystals produced in [1].

  8. Could glutaric acid (GA) replace glutaraldehyde in the preparation of ...

    Indian Academy of Sciences (India)

    folds received wide attention due to low cost, ease of processing and biocompatibility .... ference in the absorbance divided by the absorbance of the native material ..... The present study explicitly demonstrated that glutaric acid acts as suitable ...

  9. Anesthetic management of comprehensive dental restoration in a child with glutaric aciduria type 1 using volatile sevoflurane

    Directory of Open Access Journals (Sweden)

    Wei-Nung Teng

    2014-10-01

    Full Text Available Glutaric aciduria type 1 (GA1 is a rare, inherited mitochondrial disorder that results from deficiency of mitochondrial glutaryl-CoA dehydrogenase. Most patients develop neurological dysfunction early in life, which leads to severe disabilities. We present a 37-month-old girl with GA1 manifested as macrocephaly and hypotonia who received comprehensive dental restoration surgery under general anesthesia with sevoflurane. She was placed on specialized fluid management during a preoperative fasting period and anesthesia was administered without complications. All the physiological parameters, including glucose and lactate blood levels and arterial blood gas were carefully monitored and maintained within normal range perioperatively. Strategies for anesthetic management should include prevention of pulmonary aspiration, dehydration, hyperthermia and catabolic state, adequate analgesia to minimize surgical stress, and avoidance of prolonged neuromuscular blockade. We administered general anesthesia with sevoflurane uneventfully, which was well tolerated by our patient with GA1. Additionally, communication with a pediatric geneticist and surgeons should be undertaken to formulate a comprehensive anesthetic strategy in these patients.

  10. Concurrent non-ketotic hyperglycinemia and propionic acidemia in an eight year old boy

    Directory of Open Access Journals (Sweden)

    Paul S. Kruszka

    2014-01-01

    Full Text Available This is the first reported case of a patient with both non-ketotic hyperglycinemia and propionic acidemia. At 2 years of age, the patient was diagnosed with non-ketotic hyperglycinemia by elevated glycine levels and mutations in the GLDC gene (paternal allele: c.1576_1577insC delT and c.1580delGinsCAA; p.S527Tfs*13, and maternal allele: c.1819G>A; p.G607S. At 8 years of age after having been placed on ketogenic diet, he became lethargic and had severe metabolic acidosis with ketonuria. Urine organic acid analysis and plasma acylcarnitine profile were consistent with propionic acidemia. He was found to have an apparently homozygous mutation in the PCCB gene: c.49C>A; p.Leu17Met. The patient was also treated with natural protein restriction, carnitine, biotin, and thiamine and had subjective and biochemical improvement.

  11. Diagnosis of propionic acidemia by gas chromatography coupled to mass spectrometry in a case analysis

    International Nuclear Information System (INIS)

    Camayd Viera, Ivette; Robaina Jimenez, Zoe; Contreras Roura, Jiovanna

    2011-01-01

    Propionic acidemia is an inherited metabolic disease caused by a deficiency in the propionyl-CoA carboxilase, a biotin-dependent mitochondrial enzyme. The disorder is a clinically heterogeneous disease and one of the most frequently occurring organic acidurias. We report the first Cuban case with a severe form of propionic acidemia followed by acidosis and death. The diagnosis was carried out by gas chromatography coupled to mass spectrometry. Our aim is to highlight the importance of organic acids urine analysis as part of the first laboratory tests in undiagnosed seriously ill children. The definitive diagnosis is important as it serves as a clear guideline to establish a suitable treatment and allows geneticists to provide patients with a proper genetic counseling

  12. Hypoattenuation of the basal nuclei as a sign of propionic acidemia

    International Nuclear Information System (INIS)

    Asenjo, M.; Moron, A.; Marti, M.; Dominguez, F.

    1999-01-01

    The hypoattenuation of the basal ganglia is an uncommon radiological finding that suggests a metabolic or hypoxic disorder. We report a case of propionic acidemia in a five-year-old boy, presenting as a symmetric hypoattenuation of the basal neclei. We discuss this and other causes of this radiological finding, as well as the possible mechanism and underlying pathology. (Author) 17 refs

  13. Salt and cocrystals of sildenafil with dicarboxylic acids: solubility and pharmacokinetic advantage of the glutarate salt.

    Science.gov (United States)

    Sanphui, Palash; Tothadi, Srinu; Ganguly, Somnath; Desiraju, Gautam R

    2013-12-02

    Sildenafil is a drug used to treat erectile dysfunction and pulmonary arterial hypertension. Because of poor aqueous solubility of the drug, the citrate salt, with improved solubility and pharmacokinetics, has been marketed. However, the citrate salt requires an hour to reach its peak plasma concentration. Thus, to improve solubility and bioavailability characteristics, cocrystals and salts of the drug have been prepared by treating aliphatic dicarboxylic acids with sildenafil; the N-methylated piperazine of the drug molecule interacts with the carboxyl group of the acid to form a heterosynthon. Salts are formed with oxalic and fumaric acid; salt monoanions are formed with succinic and glutaric acid. Sildenafil forms cocrystals with longer chain dicarboxylic acids such as adipic, pimelic, suberic, and sebacic acids. Auxiliary stabilization via C-H···O interactions is also present in these cocrystals and salts. Solubility experiments of sildenafil cocrystal/salts were carried out in 0.1N HCl aqueous medium and compared with the solubility of the citrate salt. The glutarate salt and pimelic acid cocrystal dissolve faster than the citrate salt in a two hour dissolution experiment. The glutarate salt exhibits improved solubility (3.2-fold) compared to the citrate salt in water. Solubilities of the binary salts follow an inverse correlation with their melting points, while the solubilities of the cocrystals follow solubilities of the coformer. Pharmacokinetic studies on rats showed that the glutarate salt exhibits doubled plasma AUC values in a single dose within an hour compared to the citrate salt. The high solubility of glutaric acid, in part originating from the strained conformation of the molecule and its high permeability, may be the reason for higher plasma levels of the drug.

  14. Efficiency of acidemia correction on intermittent versus continuous hemodialysis in acute methanol poisoning

    Czech Academy of Sciences Publication Activity Database

    Zakharov, S.; Pelclová, D.; Navrátil, Tomáš; Běláček, J.; Latta, J.; Písař, M.; Rulíšek, J.; Lepš, J.; Zídek, P.; Kučera, C.; Boček, R.; Mazur, M.; Belik, Z.; Chalupa, J.; Talafa, V.; Kodras, K.; Nalos, D.; Sedlak, C.; Šenkyřík, M.; Šmíd, J.; Šálek, T.; Roberts, D. M.; Hovda, K. E.

    2017-01-01

    Roč. 55, č. 2 (2017), s. 123-132 ISSN 1556-3650 Institutional support: RVO:61388955 Keywords : Acidemia * continuous veno-venous hemodialysis * extended daily hemodialysis * intermittent hemodialysis * metabolic acidosis * methanol poisoning Subject RIV: CF - Physical ; Theoretical Chemistry OBOR OECD: Physical chemistry Impact factor: 3.677, year: 2016

  15. Growth behavior of anodic oxide formed by aluminum anodizing in glutaric and its derivative acid electrolytes

    Science.gov (United States)

    Nakajima, Daiki; Kikuchi, Tatsuya; Natsui, Shungo; Suzuki, Ryosuke O.

    2014-12-01

    The growth behavior of anodic oxide films formed via anodizing in glutaric and its derivative acid solutions was investigated based on the acid dissociation constants of electrolytes. High-purity aluminum foils were anodized in glutaric, ketoglutaric, and acetonedicarboxylic acid solutions under various electrochemical conditions. A thin barrier anodic oxide film grew uniformly on the aluminum substrate by glutaric acid anodizing, and further anodizing caused the film to breakdown due to a high electric field. In contrast, an anodic porous alumina film with a submicrometer-scale cell diameter was successfully formed by ketoglutaric acid anodizing at 293 K. However, the increase and decrease in the temperature of the ketoglutaric acid resulted in non-uniform oxide growth and localized pitting corrosion of the aluminum substrate. An anodic porous alumina film could also be fabricated by acetonedicarboxylic acid anodizing due to the relatively low dissociation constants associated with the acid. Acid dissociation constants are an important factor for the fabrication of anodic porous alumina films.

  16. Volumetric properties of aqueous solutions of glutaric acid

    International Nuclear Information System (INIS)

    Ben-Hamo, Meyrav; Apelblat, Alexander; Manzurola, Emanuel

    2007-01-01

    Densities of aqueous solutions with molalities up to 6 mol . kg -1 were determined at 5 K temperature intervals, from T = 288.15 K to T = 333.15 K. Densities served to evaluate the apparent molar volumes, V 2,φ (m, T), the cubic expansion coefficients, α(m, T), and the changes of isobaric heat capacities with respect to pressure, (∂C P /∂P) T,m . They were qualitatively correlated with the changes in the structure of water when glutaric acid is dissolved in it

  17. Renal responses to prolonged (48 h) hypoxemia without acidemia in the late-gestation ovine fetus

    NARCIS (Netherlands)

    Dassel, ACM; Aarnoudse, JG

    The effect of sustained moderate hypoxia on renal blood flow and renal function was studied in the ovine fetus (123-129 days). The experiments consisted of 48 h of isocapnic hypoxia, not resulting in acidemia, but sufficient to produce redistribution of blood flow in favor of the brain at the

  18. Volumetric properties of aqueous solutions of glutaric acid

    Energy Technology Data Exchange (ETDEWEB)

    Ben-Hamo, Meyrav [Department of Chemical Engineering, Ben Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105 (Israel); Apelblat, Alexander [Department of Chemical Engineering, Ben Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105 (Israel)]. E-mail: apelblat@bgu.ac.il; Manzurola, Emanuel [Department of Chemical Engineering, Ben Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105 (Israel)

    2007-07-15

    Densities of aqueous solutions with molalities up to 6 mol . kg{sup -1} were determined at 5 K temperature intervals, from T = 288.15 K to T = 333.15 K. Densities served to evaluate the apparent molar volumes, V {sub 2,{phi}}(m, T), the cubic expansion coefficients, {alpha}(m, T), and the changes of isobaric heat capacities with respect to pressure, ({partial_derivative}C {sub P}/{partial_derivative}P) {sub T,m}. They were qualitatively correlated with the changes in the structure of water when glutaric acid is dissolved in it.

  19. An unusual cause of high anion gap metabolic acidosis: pyroglutamic acidemia. A case report.

    Science.gov (United States)

    Romero, Jorge E; Htyte, Nay

    2013-01-01

    Pyroglutamic acidemia is an uncommon metabolic disorder, which is usually diagnosed at early ages. The mechanism of action is thought to be glutathione depletion, and its clinical manifestations consist of hemolytic anemia, mental retardation, ataxia, and chronic metabolic acidosis. However, an acquired form has been described in adult patients, who usually present with confusion, respiratory distress, and high anion gap metabolic acidosis (HAGMA). It is also associated with many conditions, including chronic acetaminophen consumption. A 68-year-old white male, with chronic acetaminophen use presented to our service on multiple occasions with severe HAGMA. The patient was admitted to the intensive care unit and required mechanical ventilation and aggressive supportive measures. After ruling out the most frequent etiologies for his acid-base disorder and considering the long history of Tylenol ingestion, his 5-oxiproline (pyroglutamic acid) levels were sent to diagnose pyroglutamic acidemia. Clinicians need to be aware of this cause for metabolic acidosis since it might be a more common metabolic disturbance in compromised patients than would be expected. Subjects with HAGMA that cannot be explained by common causes should be tested for the presence of 5-oxoproline. Discontinuation of the offending drug is therapeutic.

  20. Thermodynamics of Dissolution for Crystalline Racemic Tartaric and Glutaric Acids and Isatin in KOH Aqueous Solutions at 298.15 K

    Science.gov (United States)

    Lytkin, A. I.; Chernikov, V. V.; Krutova, O. N.; Litvinenko, V. E.; Volkov, A. V.; Bychkova, S. A.; Skvortsov, I. A.

    2018-01-01

    Enthalpies of dissolution are found for crystalline racemic tartaric and glutaric acids and isatin in water and in potassium hydroxide solutions at 298.15 K via direct calorimetry. The protolytic equilibria in isatin aqueous solutions are studied at 298.15 K and ionic strengths of 0.5 (relative to potassium nitrate) by potentiometric means. Standard enthalpies of formation are calculated for racemic tartaric and glutaric acids, isatin, and the products of their dissociation in aqueous solutions.

  1. The implementation of neonatal peritoneal dialysis in a clinical setting.

    Science.gov (United States)

    Unal, Sevim; Bilgin, Leyla; Gunduz, Mehmet; Uncu, Nermin; Azili, Mujdem Nur; Tiryaki, Tugrul

    2012-10-01

    To investigate etiology, outcome and complications related to neonatal peritoneal dialysis (PD). Neonates treated with PD in our neonatal intensive care unit during 2007-2010 were analyzed retrospectively. Among 4036 hospitalized neonates; 20 neonates (0.5%) who underwent 21 cycles of PD [7 preterm, 13 term; 13 female, 7 male] were included. The mean birth weight was 2930.2 ± 720.6 g (1120-4570), mean gestational age was 37.5 ± 3.5 weeks (27-41). The etiologic disorders included inborn errors of metabolism (propionic acidemia, methylmalonic acidemia, citrullinemia, glutaric aciduria type 2, maple syrup urine disease, 10), or acute renal failure secondary to perinatal asphyxia (4), sepsis (2), prematurity (2), hypoplastic left heart syndrome (1), kernicterus (1). The complications included peritonitis (2), early leakage (4), hemorrhage (1), catheter removal (3) and occlusion (2). The mortality rate was 50%. The gestational ages and birth weights of surviving neonates were higher (p neonates, chronic renal failure (1), severe (4) and moderate neuromotor impairment (2) developed within 4-43 months. PD, although invasive, is an effective therapy in neonates. The complexity and invasiveness of the procedure is probably responsible for high rate of complications and mortality. If appropriate catheter selection and technique in the placement should be done, PD might improve outcome.

  2. An experimental and theoretical investigation of loperamide hydrochloride-glutaric acid cocrystals.

    Science.gov (United States)

    Bruni, Giovanna; Maietta, Mariarosa; Maggi, Lauretta; Mustarelli, Piercarlo; Ferrara, Chiara; Berbenni, Vittorio; Freccero, Mauro; Scotti, Federico; Milanese, Chiara; Girella, Alessandro; Marini, Amedeo

    2013-07-11

    Cocrystallization is a powerful method to improve the physicochemical properties of drugs. Loperamide hydrochloride is a topical analgesic for the gastrointestinal tract showing low and pH-dependent solubility; for this reason, an enhancement of its solubility or dissolution rate, particularly at the pH of the intestinal tract, could improve its local efficacy. Here we prepared cocrystals of this active principle with glutaric acid and so obtained a new crystalline solid representing a viable alternative to improve the physicochemical properties and thus the pharmaceutical behavior of the drug. Differential scanning calorimetry, X-ray powder diffraction, Fourier infrared spectroscopy, solid-state NMR, and scanning electron microscopy coupled to the energy-dispersive X-ray spectrometry were used to investigate the new solid-phase formation. DFT calculations at B3LYP/6-31G(d) level of theory, in the gas phase, including frequencies computation, provided a rationale for the interaction between loperamide hydrochloride and glutaric acid. The cocrystals showed improved water solubility in comparison with loperamide HCl, and the pharmaceutical formulation proposed was able to release the drug more rapidly in comparison with three reference commercial products when tested at neutral pH values.

  3. Hypercapnea and Acidemia despite Hyperventilation following Endotracheal Intubation in a Case of Unknown Severe Salicylate Poisoning

    Directory of Open Access Journals (Sweden)

    Shannon M. Fernando

    2017-01-01

    Full Text Available Salicylates are common substances for deliberate self-harm. Acute salicylate toxicity is classically associated with an initial respiratory alkalosis, followed by an anion gap metabolic acidosis. The respiratory alkalosis is achieved through hyperventilation, driven by direct stimulation on the respiratory centers in the medulla and considered as a compensatory mechanism to avoid acidemia. However, in later stages of severe salicylate toxicity, patients become increasingly obtunded, with subsequent loss of airway reflexes, and therefore intubation may be necessary. Mechanical ventilation has been recommended against in acute salicylate poisoning, as it is believed to take away the compensatory hyperpnea and tachypnea. Despite the intuitive physiological basis for this recommendation, there is a paucity of evidence to support it. We describe a case of a 59-year-old male presenting with decreased level of consciousness and no known history of ingestion. He was intubated and experienced profound hypercarbia and acidemia despite mechanical ventilation with high minute ventilation and tidal volumes. This case illustrates the deleterious effects of intubation in severe salicylate toxicity.

  4. Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive

    NARCIS (Netherlands)

    Baertling, F.; Al-Murshedi, F.; Sanchez Caballero, L.M.; Al-Senaidi, K.; Joshi, N.P.; Venselaar, H.; Brand, M.A.M. van den; Nijtmans, L.G.J.; Rodenburg, R.J.T.

    2017-01-01

    COX5A is a nuclear-encoded subunit of mitochondrial respiratory chain complex IV (cytochrome c oxidase). We present patients with a homozygous pathogenic variant in the COX5A gene. Clinical details of two affected siblings suffering from early-onset pulmonary arterial hypertension, lactic acidemia,

  5. Free radicals in dicarboxylic acids: an e.s.r. study of radical conversions in γ-irradiated single crystals of glutaric acid and glutaric-2,2,4,4-d4 acid

    International Nuclear Information System (INIS)

    Bergene, R.; Minegishi, A.; Riesz, P.

    1980-01-01

    The γ-radiation-induced free radicals in single crystals of glutaric acid and glutaric-2,2,4,4-d 4 acid were studied in the temperature range 77-300 K by e.s.r. techniques. At 77 K the decarboxylation radical and the anion are stabilized. At higher temperatures the decarboxylation radical is found to be converted into a hydrogen abstraction radical with an activation energy of 6.3 +- 0.5 kcal/mole for the non-deuterated crystal. This radical is stable at room temperature. The anion seems to be converted to a unidentified intermediate radical which in turn is converted to the σ-acyl radical. An analysis of the g-value anisotropy and of the 13 C hyperfine splitting variation for this radical in the deuterated crystal is consistent with the assigned radical structure. By heat treatment the σ-acyl radical is converted to another form of the hydrogen abstraction radical with an activation energy of 9.6 +- 0.6 kcal/mole in the deuterated crystal. U.V.-light (lambda= 254 nm) transforms one of the room temperature radicals into the other. (author)

  6. Central Venous Catheter-Related Bloodstream Infection with Kocuria kristinae in a Patient with Propionic Acidemia

    OpenAIRE

    Kimura, Masato; Kawai, Eichiro; Yaoita, Hisao; Ichinoi, Natsuko; Sakamoto, Osamu; Kure, Shigeo

    2017-01-01

    Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The patient had a central venous catheter implanted for total parenteral nutrition approximately 6 mont...

  7. Neurodevelopmental profiles of children with glutaric aciduria type I diagnosed by newborn screening: a follow-up case series.

    Science.gov (United States)

    Brown, Amy; Crowe, Louise; Beauchamp, Miriam H; Anderson, Vicki; Boneh, Avihu

    2015-01-01

    Glutaric aciduria type I (GA-I) is an inherited metabolic disorder that may lead to severe motor disorder and cognitive impairment. GA-I is now included in the newborn screening programme in many countries as early detection allows for prompt treatment and effectively reduces the risk of poor developmental outcome. Information regarding the long-term neurodevelopmental outcome of children with GA-I treated early is sparse.We recruited children with a confirmed diagnosis of GA-I diagnosed via newborn screening, treated in our centre and >3 years of age (n = 6). Children were assessed at two time points using a comprehensive neuropsychological test battery. Four of these had been the subject of a previous report. All participants were male, 3-6 years at the initial assessment and 6-12 years of age at the follow-up assessment.Fine motor skills were below average in all patients. Speech, which was affected in all four patients reported previously, improved following speech therapy. IQ scores remained generally stable within the normal range. Executive functioning was average to high average in four patients. Behaviour, as assessed through parental questionnaires, was problematic in two patients. Compounding factors included child neglect, family history of autism and multiple admissions to hospital (n = 1 in each).GA-I affects fine motor skills and speech, regardless of early treatment, but not IQ scores. Patients with GA-I should be referred for assessment and appropriate early intervention. Further research is needed to correlate specific neuropsychological deficits with neuroimaging.

  8. Stability constants of glutarate complexes of copper(II), zinc(II), cobalt(II) and uranyl(II) by paper electrophoresis

    International Nuclear Information System (INIS)

    Singh, R.K.P.; Yadava, J.R.; Yadava, K.L.

    1981-01-01

    Stability constants of Copper(II), Zinc(II), Cobalt(II) and Uranyl(II) glutarates have been determined by paper electrophoresis. Glutaric acid (0.005 mol dmsup(-3)) was added to the background electrolyte : 0.1 mol dmsup(-3) HClO 4 . The proportions of (CH 2 ) 3 COOH COO - and (CH 2 ) 3 C 2 O 4 2- were varied by changing the pH of the electrolyte. These anions yielded the complexes Cu(CH 2 ) 3 C 2 O 4 , [Zn(CH 2 ) 3 COOH COO] + [Co(CH 2 ) 3 COOH COO] + and UO 2 (CH 2 ) 3 C 2 O 4 whose stability constants are found to be 10sup(3.9), 10sup(2.9), 10sup(2.7) and 10sup(13.5) respectively. (author)

  9. Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

    DEFF Research Database (Denmark)

    Baumgartner, Matthias R; Hörster, Friederike; Dionisi-Vici, Carlo

    2014-01-01

    Methylmalonic and propionic acidemia (MMA/PA) are inborn errors of metabolism characterized by accumulation of propionic acid and/or methylmalonic acid due to deficiency of methylmalonyl-CoA mutase (MUT) or propionyl-CoA carboxylase (PCC). MMA has an estimated incidence of ~ 1: 50,000 and PA of ~...... recognition and delayed diagnosis due to nonspecific clinical presentation and insufficient awareness of health care professionals because of disease rarity.......:100'000 -150,000. Patients present either shortly after birth with acute deterioration, metabolic acidosis and hyperammonemia or later at any age with a more heterogeneous clinical picture, leading to early death or to severe neurological handicap in many survivors. Mental outcome tends to be worse in PA...

  10. Synthesis, crystal structure, and magnetic properties of two-dimensional divalent metal glutarate/dipyridylamine coordination polymers, with a single crystal-to-single crystal transformation in the copper derivative

    International Nuclear Information System (INIS)

    Montney, Matthew R.; Supkowski, Ronald M.; Staples, Richard J.; LaDuca, Robert L.

    2009-01-01

    Hydrothermal reaction of divalent metal chlorides with glutaric acid and 4,4'-dipyridylamine (dpa) has afforded an isostructural family of coordination polymers with formulation [M(glu)(dpa)] n (M=Co (1), Ni (2), Cu (3); glu=glutarate). Square pyramidal coordination is seen in 1-3, with semi-ligation of a sixth donor to produce a '5+1' extended coordination sphere. Neighboring metal atoms are linked into 1D [M(glu)] n neutral chains through chelating/monodentate bridging glutarate moieties with a syn-anti binding mode, and semi-chelation of the pendant carboxylate oxygen. These chains further connect into 2D layers through dipodal dpa ligands. Neighboring layers stack into the pseudo 3D crystal structure of 1-3 through supramolecular hydrogen bonding between dpa amine units and the semi-chelated glutarate oxygen atoms. The variable temperature magnetic behavior of 1-3 was explored and modeled as infinite 1D Heisenberg chains. Notably, complex 3 undergoes a thermally induced single crystal-to-single crystal transformation between centric and acentric space groups, with a conformationally disordered unilayer structure at 293 K and an ordered bilayer structure at 173 K. All materials were further characterized via infrared spectroscopy and elemental and thermogravimetric analyses. - Graphical abstract: The coordination polymers [M(glu)(dpa)] n (M=Co (1), Ni (2), Cu (3); glu=glutarate, dpa=4,4'-dipyridylamine) exhibit 2D layer structures based on 1D [M(glu)] n chains linked through dpa tethers. Antiferromagnetic coupling is observed for 2 and 3, while ferromagnetism is predominant in 1. Compound 3 undergoes a thermally induced single crystal-to-single crystal transformation from an acentric to a centrosymmetric space group

  11. /sup 1/H-NMR urinalysis. Simultaneous screening of inborn errors of metabolism of amino acid and organic acid disorders

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Hideaki; Yamaguchi, Shuichi

    1988-02-01

    In an effort to examine the usefulness of /sup 1/H-nuclear magnetic resonance (NMR) urinalysis in the diagnosis of congenital metabolic disorders, 70 kinds of urinary metabolites were analysed in relation to the diagnosis of inborn errors of amino acid and organic acid disorders. Homogated decoupling (HMG) method failed to analyze six metabolites within the undetectable range. When non-decoupling method (NON), in which the materials are dissolved in dimethyl sulfoxide, was used, the identification of signals became possible. The combination of HMG and NON methods was, therefore, considered to identify all of the metabolites. When the urine samples, which were obtained from patients with hyperglycerolemia, hyperornithinemia, glutaric acidemia type II, or glycerol kinase deficiency, were analysed by using both HMG and NON methods, abnormally increased urinary metabolites were detected. /sup 1/H-NMR urinalysis, if used in the combination of HMG and NON methods, may allow simultanenous screening of inborn errors of metabolism of amino acid and organic acid disorders. (Namekawa, K.).

  12. 1H-NMR urinalysis

    International Nuclear Information System (INIS)

    Yamamoto, Hideaki; Yamaguchi, Shuichi

    1988-01-01

    In an effort to examine the usefulness of 1 H-nuclear magnetic resonance (NMR) urinalysis in the diagnosis of congenital metabolic disorders, 70 kinds of urinary metabolites were analysed in relation to the diagnosis of inborn errors of amino acid and organic acid disorders. Homogated decoupling (HMG) method failed to analyze six metabolites within the undetectable range. When non-decoupling method (NON), in which the materials are dissolved in dimethyl sulfoxide, was used, the identification of signals became possible. The combination of HMG and NON methods was, therefore, considered to identify all of the metabolites. When the urine samples, which were obtained from patients with hyperglycerolemia, hyperornithinemia, glutaric acidemia type II, or glycerol kinase deficiency, were analysed by using both HMG and NON methods, abnormally increased urinary metabolites were detected. 1 H-NMR urinalysis, if used in the combination of HMG and NON methods, may allow simultanenous screening of inborn errors of metabolism of amino acid and organic acid disorders. (Namekawa, K.)

  13. Probing conformational states of glutaryl-CoA dehydrogenase by fragment screening

    Energy Technology Data Exchange (ETDEWEB)

    Begley, Darren W.; Davies, Douglas R.; Hartley, Robert C.; Hewitt, Stephen N.; Rychel, Amanda L.; Myler, Peter J.; Van Voorhis, Wesley C.; Staker, Bart L.; Stewart, Lance J. (Emerald)

    2014-10-02

    Glutaric acidemia type 1 is an inherited metabolic disorder which can cause macrocephaly, muscular rigidity, spastic paralysis and other progressive movement disorders in humans. The defects in glutaryl-CoA dehydrogenase (GCDH) associated with this disease are thought to increase holoenzyme instability and reduce cofactor binding. Here, the first structural analysis of a GCDH enzyme in the absence of the cofactor flavin adenine dinucleotide (FAD) is reported. The apo structure of GCDH from Burkholderia pseudomallei reveals a loss of secondary structure and increased disorder in the FAD-binding pocket relative to the ternary complex of the highly homologous human GCDH. After conducting a fragment-based screen, four small molecules were identified which bind to GCDH from B. pseudomallei. Complex structures were determined for these fragments, which cause backbone and side-chain perturbations to key active-site residues. Structural insights from this investigation highlight differences from apo GCDH and the utility of small-molecular fragments as chemical probes for capturing alternative conformational states of preformed protein crystals.

  14. Early Infantile Epileptic Encephalopathy in an STXBP1 Patient with Lactic Acidemia and Normal Mitochondrial Respiratory Chain Function

    Directory of Open Access Journals (Sweden)

    Dong Li

    2016-01-01

    Full Text Available A wide range of clinical findings have been associated with mutations in Syntaxin Binding Protein 1 (STXBP1, including multiple forms of epilepsy, nonsyndromic intellectual disability, and movement disorders. STXBP1 mutations have recently been associated with mitochondrial pathology, although it remains unclear if this phenotype is a part of the core feature for this gene disorder. We report a 7-year-old boy who presented for diagnostic evaluation of intractable epilepsy, episodic ataxia, resting tremor, and speech regression following a period of apparently normal early development. Mild lactic acidemia was detected on one occasion at the time of an intercurrent illness. Due to the concern for mitochondrial disease, ophthalmologic evaluation was performed that revealed bilateral midperiphery pigmentary mottling. Optical coherence tomography (OCT testing demonstrated a bilaterally thickened ganglion cell layer in the perifovea. Skeletal muscle biopsy analysis showed no mitochondrial abnormalities or respiratory chain dysfunction. Exome sequencing identified a de novo c.1651C>T (p.R551C mutation in STXBP1. Although mitochondrial dysfunction has been reported in some individuals, our proband had only mild lactic acidemia and no skeletal muscle tissue evidence of mitochondrial disease pathology. Thus, mitochondrial dysfunction is not an obligate feature of STXBP1 disease.

  15. Fetal pancreatic beta-cell function in pregnancies complicated by maternal diabetes mellitus: relationship to fetal acidemia and macrosomia.

    Science.gov (United States)

    Salvesen, D R; Brudenell, J M; Proudler, A J; Crook, D; Nicolaides, K H

    1993-05-01

    Our purpose was to investigate the relationship between fetal pancreatic beta-cell function and fetal acidemia and macrosomia in pregnancies complicated by maternal diabetes mellitus. A cross-sectional study at the Harris Birthright Research Centre for Fetal Medicine, London, was performed. In 32 pregnancies complicated by maternal diabetes mellitus cordocentesis was performed at 36 to 39 weeks' gestation for the measurement of umbilical venous blood pH, PO2, PCO2, lactate, and glucose concentration; plasma insulin immunoreactivity; and insulin/glucose ratio. A reference range for plasma insulin and insulin/glucose ratio was constructed by studying fetal blood samples from 80 women who did not have diabetes mellitus. Mean umbilical venous blood pH was significantly lower and plasma insulin immunoreactivity and insulin/glucose ratio were significantly higher than the appropriate normal mean for gestation. There were significant associations between (1) maternal and fetal blood glucose concentrations (r = 0.95, p < 0.0001), (2) fetal blood glucose and plasma insulin immunoreactivity (r = 0.57, p < 0.01), (3) fetal plasma insulin immunoreactivity and blood pH (r = -0.39, p < 0.05), and (4) fetal insulin/glucose ratio and degree of macrosomia (r = 0.76, p < 0.0001). Fetal pancreatic beta-cell hyperplasia is implicated in the pathogenesis of both fetal acidemia and macrosomia.

  16. Novel ETF dehydrogenase mutations in a patient with mild glutaric aciduria type II and complex II-III deficiency in liver and muscle.

    Science.gov (United States)

    Wolfe, Lynne A; He, Miao; Vockley, Jerry; Payne, Nicole; Rhead, William; Hoppel, Charles; Spector, Elaine; Gernert, Kim; Gibson, K Michael

    2010-12-01

    We describe a 22-year-old male who developed severe hypoglycemia and lethargy during an acute illness at 4 months of age and subsequently grew and developed normally. At age 4 years he developed recurrent vomiting with mild hyperammonemia and dehydration requiring frequent hospitalizations. Glutaric aciduria Type II was suspected based upon biochemical findings and managed with cornstarch, carnitine and riboflavin supplements. He did not experience metabolic crises between ages 4-12 years. He experienced recurrent vomiting, mild hyperammonemia, and generalized weakness associated with acute illnesses and growth spurts. At age 18 years, he developed exercise intolerance and proximal muscle weakness leading to the identification of multiple acyl-CoA dehydrogenase and complex II/III deficiencies in both skeletal muscle and liver. Subsequent molecular characterization of the ETFDH gene revealed novel heterozygous mutations, p.G274X:c.820 G > T (exon 7) and p.P534L: c.1601 C > T (exon 12), the latter within the iron sulfur-cluster and predicted to affect ubiquinone reductase activity of ETFDH and the docking of ETF to ETFDH. Our case supports the concept of a structural interaction between ETFDH and other enzyme partners, and suggests that the conformational change upon ETF binding to ETFDH may play a key role in linking ETFDH to II/III super-complex formation.

  17. The effect of citrulline and arginine supplementation on lactic acidemia in MELAS syndrome☆

    Science.gov (United States)

    El-Hattab, Ayman W.; Emrick, Lisa T.; Williamson, Kaitlin C.; Craigen, William J.; Scaglia, Fernando

    2013-01-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a mitochondrial disorder in which nitric oxide (NO) deficiency may play a role in the pathogenesis of several complications including stroke-like episodes and lactic acidosis. Supplementing the NO precursors arginine and citrulline restores NO production in MELAS syndrome. In this study we evaluated the effect of arginine or citrulline on lactic acidemia in adults with MELAS syndrome. Plasma lactate decreased significantly after citrulline supplementation, whereas the effect of arginine supplementation did not reach statistical significance. These results support the potential therapeutic utility of arginine and citrulline in MELAS syndrome and suggest that citrulline supplementation may be more efficacious. However, therapeutic efficacy of these compounds should be further evaluated in clinical trials. PMID:25411654

  18. The effect of citrulline and arginine supplementation on lactic acidemia in MELAS syndrome.

    Science.gov (United States)

    El-Hattab, Ayman W; Emrick, Lisa T; Williamson, Kaitlin C; Craigen, William J; Scaglia, Fernando

    2013-12-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a mitochondrial disorder in which nitric oxide (NO) deficiency may play a role in the pathogenesis of several complications including stroke-like episodes and lactic acidosis. Supplementing the NO precursors arginine and citrulline restores NO production in MELAS syndrome. In this study we evaluated the effect of arginine or citrulline on lactic acidemia in adults with MELAS syndrome. Plasma lactate decreased significantly after citrulline supplementation, whereas the effect of arginine supplementation did not reach statistical significance. These results support the potential therapeutic utility of arginine and citrulline in MELAS syndrome and suggest that citrulline supplementation may be more efficacious. However, therapeutic efficacy of these compounds should be further evaluated in clinical trials.

  19. Methylmalonic and propionic acidemias: lipid profiles of normal and affected human skin fibroblasts incubated with [1-14C]propionate

    International Nuclear Information System (INIS)

    Giudici, T.A.; Chen, R.G.; Oizumi, J.; Shaw, K.N.; Ng, W.G.; Donnell, G.N.

    1986-01-01

    Normal human skin fibroblasts and those from methylmalonic acidemia and propionic acidemia patients were grown in culture. Following incubation with [1- 14 C]propionate, the major lipid classes in the cells were separated by thin layer chromatography and isolated fractions analyzed by radio gas chromatography for the presence of odd-numbered long-chain fatty acids; the pattern of even-numbered long-chain fatty acids was obtained also. Normal fibroblasts incorporated a small percentage of propionate into odd-numbered fatty acids which were present in all lipids studied. The abnormal cells incorporated a larger amount while maintaining the characteristic ratios of odd-numbered fatty acids found in the normal line. Most of the radioactivity was associated with phospholipids which are the predominant constituents of cell membranes. A characteristic C15/C17 ratio was found for different phospholipids and the triglyceride fraction; pentadecanoic acid was the principal odd-numbered fatty acid utilized in the assembly of complex lipids. Compared to even-numbered long-chain fatty acids the absolute amount of odd-numbered fatty acids was low (1-2%), even in affected cells. An unusual polar lipid fraction was isolated in the course of the study. In the normal cell it contained several unlabeled eicosanoids which were missing from the same fraction of both affected cell lines

  20. Heptadecanoylcarnitine (C17) a novel candidate biomarker for newborn screening of propionic and methylmalonic acidemias.

    Science.gov (United States)

    Malvagia, Sabrina; Haynes, Christopher A; Grisotto, Laura; Ombrone, Daniela; Funghini, Silvia; Moretti, Elisa; McGreevy, Kathleen S; Biggeri, Annibale; Guerrini, Renzo; Yahyaoui, Raquel; Garg, Uttam; Seeterlin, Mary; Chace, Donald; De Jesus, Victor R; la Marca, Giancarlo

    2015-10-23

    3-Hydroxypalmitoleoyl-carnitine (C16:1-OH) has recently been reported to be elevated in acylcarnitine profiles of patients with propionic acidemia (PA) or methylmalonic acidemia (MMA) during expanded newborn screening (NBS). High levels of C16:1-OH, combined with other hydroxylated long chain acylcarnitines are related to long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) and trifunctional protein (TFP) deficiency. The acylcarnitine profile of two LCHADD patients was evaluated using liquid chromatography-tandem mass spectrometric method. A specific retention time was determined for each hydroxylated long chain acylcarnitine. The same method was applied to some neonatal dried blood spots (DBSs) from PA and MMA patients presenting abnormal C16:1-OH concentrations. The retention time of the peak corresponding to C16:1-OH in LCHADD patients differed from those in MMA and PA patients. Heptadecanoylcarnitine (C17) has been identified as the novel biomarker specific for PA and MMA patients through high resolution mass spectrometry (Orbitrap) experiments. We found that 21 out of 23 neonates (22 MMA, and 1PA) diagnosed through the Tuscany region NBS program exhibited significantly higher levels of C17 compared to controls. Twenty-three maternal deficiency (21 vitamin B12 deficiency, 1 homocystinuria and 1 gastrin deficiency) samples and 82 false positive for elevated propionylcarnitine (C3) were also analyzed. We have characterized a novel biomarker able to detect propionate disorders during expanded newborn screening (NBS). The use of this new biomarker may improve the analytical performances of NBS programs especially in laboratories where second tier tests are not performed. Copyright © 2015 Elsevier B.V. All rights reserved.

  1. Predictive value of late decelerations for fetal acidemia in unselective low-risk pregnancies.

    Science.gov (United States)

    Sameshima, Hiroshi; Ikenoue, Tsuyomu

    2005-01-01

    We evaluated the clinical significance of late decelerations (LD) of intrapartum fetal heart rate (FHR) monitoring to detect low pH (LD (occasional, 50%; recurrent, > or = 50%) and severity (reduced baseline FHR accelerations and variability) of LD, and low pH (test, and one-way analysis of variance with the Bonferroni/Dunn test. In the 5522 low-risk pregnancies, 301 showed occasional LD and 99 showed recurrent LD. Blood gases and pH values deteriorated as the incidence of LD increased and as baseline accelerations or variability was decreased. Positive predictive value for low pH (LD, and > 50% in recurrent LD with no baseline FHR accelerations and reduced variability. In low-risk pregnancies, information on LD combined with acceleration and baseline variability enables us to predict the potential incidence of fetal acidemia.

  2. Alkyl group dependence on structure and magnetic properties in layered cobalt coordination polymers containing substituted glutarate ligands and 4,4'-bipyridine

    International Nuclear Information System (INIS)

    Nettleman, Joseph H.; Supkowski, Ronald M.; LaDuca, Robert L.

    2010-01-01

    Five two-dimensional divalent cobalt coordination polymers containing 4,4'-bipyridine (bpy) and substituted or unsubstituted glutarate ligands have been prepared hydrothermally and structurally characterized by single-crystal X-ray diffraction. [Co(mg)(bpy)] n (1, mg=3-methylglutarate) forms a (4,4) rhomboid grid structure based on the connection of {Co 2 (CO 2 ) 2 } dimeric units. Using the more sterically encumbered ligands 3,3-dimethylglutarate (dmg) and 3-ethyl, 3-methylglutarate (emg) generated {[Co(dmg)(bpy)(H 2 O)].2H 2 O} n (2) and {[Co(emg)(bpy)(H 2 O)].H 2 O} n (3), respectively. These complexes manifest {Co(CO 2 )} n chains linked into 2-D by aliphatic dicarboxylate and bpy ligands. The 'tied-back' substituted glutarate ligand 1,1-cyclopentanediacetate (cda) afforded [Co(cda)(bpy)] n (4), and the unsubstituted glutarate (glu) generated [Co(glu)(bpy)] n (5), both of which exhibit a topology similar to that of 1. The magnetic properties of complexes 1-4 were analyzed successfully with a recently developed phenomenological chain model accounting for both magnetic coupling (J) and zero-field splitting effects (D), even though 1 and 4 contain isolated, discrete {Co 2 (CO 2 ) 2 } dimers. The D parameter in this series varied between 21.8(8) and 48.0(9) cm -1 . However weak antiferromagnetic coupling was observed in 1 (J=-2.43(4) cm -1 ) and 4 (J=-0.89(2) cm -1 ), while weak ferromagnetic coupling appears to be operative in both 2 (J=0.324(5) cm -1 ) and 3 (J=0.24(1) cm -1 ). - Five two-dimensional divalent cobalt coordination polymers containing 4,4'-bipyridine (bpy) and substituted or unsubstituted glutarate ligands have been prepared and structurally characterized by single-crystal X-ray diffraction. Three contain dimeric {Co 2 (CO 2 ) 2 } units, while two manifest {Co(CO 2 )} n chains, depending on the steric bulk of the substituent. The magnetic properties of the complexes were analyzed successfully with a recently developed phenomenological chain model

  3. Synthesis of Dimethyl Glutarate from Cyclobutanone and Dimethyl Carbonate over Solid Base Catalysts

    International Nuclear Information System (INIS)

    Zhi, Chen; Dudu, Wu

    2012-01-01

    A facile route for the synthesis of dimethyl glutarate (DMG) from cyclobutanone and dimethyl carbonate (DMC) in the presence of solid base catalysts has been developed. It was found that the intermediate carbomethoxycyclobutanone (CMCB) was produced from cyclobutanone with DMC in the first step, and then CMCB was further converted to DMG by reacting with a methoxide group. The role of the basic catalysts can be mainly ascribed to the activation of cyclobutanone via the abstraction of a proton in the α-position by base sites, and solid bases with moderate strength, such as MgO, favor the formation of DMG

  4. Synthesis of Dimethyl Glutarate from Cyclobutanone and Dimethyl Carbonate over Solid Base Catalysts

    Energy Technology Data Exchange (ETDEWEB)

    Zhi, Chen; Dudu, Wu [Guangdong Medical College, Dongguan (China)

    2012-06-15

    A facile route for the synthesis of dimethyl glutarate (DMG) from cyclobutanone and dimethyl carbonate (DMC) in the presence of solid base catalysts has been developed. It was found that the intermediate carbomethoxycyclobutanone (CMCB) was produced from cyclobutanone with DMC in the first step, and then CMCB was further converted to DMG by reacting with a methoxide group. The role of the basic catalysts can be mainly ascribed to the activation of cyclobutanone via the abstraction of a proton in the {alpha}-position by base sites, and solid bases with moderate strength, such as MgO, favor the formation of DMG

  5. Heptadecanoylcarnitine (C17) a novel candidate biomarker for propionic and methylmalonic acidemias during expanded newborn screening

    Science.gov (United States)

    Malvagia, Sabrina; Haynes, Christopher A.; Grisotto, Laura; Ombrone, Daniela; Funghini, Silvia; Moretti, Elisa; McGreevy, Kathleen; Buggeri, Annibale; Guerrini, Renzo; Yahyaoui, Raquel; Garg, Uttam; Seeterlin, Mary; Chace, Donald; De Jesus, Victor; la Marca, Giancarlo

    2017-01-01

    Background 3-hydroxypalmitoleoyl-carnitine (C16:1-OH) was recently reported to be elevated in acylcarnitine profile of propionic acidemia (PA) or methylmalonic acidemia (MMA) patients during expanded newborn screening (NBS). High levels of C16:1-OH, combined with other hydroxylated long chain acylcarnitines are related to long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). Methods The acylcarnitine profile of two LCHADD patients was evaluated using liquid chromatography-tandem mass spectrometric method. A specific retention time was reported for each hydroxylated long chain acylcarnitine. The same method was applied to some neonatal dried blood spots (DBS) from PA and MMA patients presenting abnormal C16:1-OH concentrations. Results The final retention time of the peak corresponding to C16:1-OH in LCHADD patients differed from those in MMA and PA patients. Heptadecanoylcarnitine (C17) has been identified as the novel biomarker specific for PA and MMA patients through high resolution mass spectrometry (Orbitrap) experiments. We found that 21 out of 23 neonates (22 MMA, and 1PA) diagnosed through the Tuscany region NBS program had significantly higher levels of C17 compared to levels detected in controls. Twenty-three maternal deficiencies (21 vitamin B12 deficiency, 1 homocystinuria and 1 gastrin deficiency) and 82 false positive for propionylcarnitine (C3) results were also analyzed. Conclusions This paper reports on the characterization of a novel biomarker able to detect propionate disorders during expanded newborn screening (NBS). The use of this new biomarker may improve the analytical performances of NBS programs especially in laboratories where second tier tests are not performed. PMID:26368264

  6. A male case with CDKL5-associated encephalopathy manifesting transient methylmalonic acidemia.

    Science.gov (United States)

    Akamine, Satoshi; Ishizaki, Yoshito; Sakai, Yasunari; Torisu, Hiroyuki; Fukai, Ryoko; Miyake, Noriko; Ohkubo, Kazuhiro; Koga, Hiroshi; Sanefuji, Masafumi; Sakata, Ayumi; Kimura, Masahiko; Yamaguchi, Seiji; Sakamoto, Osamu; Hara, Toshiro; Saitsu, Hirotomo; Matsumoto, Naomichi; Ohga, Shouichi

    2018-03-03

    Mutations in the X-linked gene CDKL5 cause early-onset epileptic encephalopathy and severe developmental delay. Because this disorder predominantly affects females, the full clinical spectrum of male patients remains elusive. We herein report a 16-year-old boy, who suffered from intractable seizures 20 days after birth. Serial electroencephalograms detected recurrent focal epileptiform discharges from age 4 months, which evolved to hypsarrhythmia later in infancy. Mass-spectrometric analyses revealed increase in urinary excretion of methylmalonic acid without perturbed concentrations of propionic acid, homocystein and methionine. Whole-exome sequencing identified a de novo, truncating mutation in CDKL5 (NM_003159.2:c.419dupA, p.Asn140Lysfs*8). Targeted sequencing excluded concomitant mutations in methylmalonic academia-associated genes. No methylmalonic acidemia has been reported in children with CDKL5 disorder. Extensive analyses on organic acid metabolism for males with CDKL5 mutations will gain more insight into their biochemical profiles in infancy. Copyright © 2018. Published by Elsevier Masson SAS.

  7. N-acetylcysteine prevents spatial memory impairment induced by chronic early postnatal glutaric acid and lipopolysaccharide in rat pups.

    Directory of Open Access Journals (Sweden)

    Fernanda S Rodrigues

    Full Text Available BACKGROUND AND AIMS: Glutaric aciduria type I (GA-I is characterized by accumulation of glutaric acid (GA and neurological symptoms, such as cognitive impairment. Although this disease is related to oxidative stress and inflammation, it is not known whether these processes facilitate the memory impairment. Our objective was to investigate the performance of rat pups chronically injected with GA and lipopolysaccharide (LPS in spatial memory test, antioxidant defenses, cytokines levels, Na+, K+-ATPase activity, and hippocampal volume. We also evaluated the effect of N-acetylcysteine (NAC on theses markers. METHODS: Rat pups were injected with GA (5 umol g of body weight-1, subcutaneously; twice per day; from 5th to 28th day of life, and were supplemented with NAC (150 mg/kg/day; intragastric gavage; for the same period. LPS (2 mg/kg; E.coli 055 B5 or vehicle (saline 0.9% was injected intraperitoneally, once per day, from 25th to 28th day of life. Oxidative stress and inflammatory biomarkers as well as hippocampal volume were assessed. RESULTS: GA caused spatial learning deficit in the Barnes maze and LPS potentiated this effect. GA and LPS increased TNF-α and IL-1β levels. The co-administration of these compounds potentiated the increase of IL-1β levels but not TNF-α levels in the hippocampus. GA and LPS increased TBARS (thiobarbituric acid-reactive substance content, reduced antioxidant defenses and inhibited Na+, K+-ATPase activity. GA and LPS co-administration did not have additive effect on oxidative stress markers and Na+, K+ pump. The hippocampal volume did not change after GA or LPS administration. NAC protected against impairment of spatial learning and increase of cytokines levels. NAC Also protected against inhibition of Na+,K+-ATPase activity and oxidative markers. CONCLUSIONS: These results suggest that inflammatory and oxidative markers may underlie at least in part of the neuropathology of GA-I in this model. Thus, NAC could

  8. Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I.

    Science.gov (United States)

    Rodrigues, Marília Danyelle Nunes; Seminotti, Bianca; Zanatta, Ângela; de Mello Gonçalves, Aline; Bellaver, Bruna; Amaral, Alexandre Umpierrez; Quincozes-Santos, André; Goodman, Stephen Irwin; Woontner, Michael; Souza, Diogo Onofre; Wajner, Moacir

    2017-08-01

    Patients affected by glutaric aciduria type I (GA-I) show progressive cortical leukoencephalopathy whose pathogenesis is poorly known. In the present work, we exposed cortical astrocytes of wild-type (Gcdh +/+ ) and glutaryl-CoA dehydrogenase knockout (Gcdh -/- ) mice to the oxidative stress inducer menadione and measured mitochondrial bioenergetics, redox homeostasis, and cell viability. Mitochondrial function (MTT and JC1-mitochondrial membrane potential assays), redox homeostasis (DCFH oxidation, nitrate and nitrite production, GSH concentrations and activities of the antioxidant enzymes SOD and GPx), and cell death (propidium iodide incorporation) were evaluated in primary cortical astrocyte cultures of Gcdh +/+ and Gcdh -/- mice unstimulated and stimulated by menadione. We also measured the pro-inflammatory response (TNFα levels, IL1-β and NF-ƙB) in unstimulated astrocytes obtained from these mice. Gcdh -/- mice astrocytes were more vulnerable to menadione-induced oxidative stress (decreased GSH concentrations and altered activities of the antioxidant enzymes), mitochondrial dysfunction (decrease of MTT reduction and JC1 values), and cell death as compared with Gcdh +/+ astrocytes. A higher inflammatory response (TNFα, IL1-β and NF-ƙB) was also observed in Gcdh -/- mice astrocytes. These data indicate a higher susceptibility of Gcdh -/- cortical astrocytes to oxidative stress and mitochondrial dysfunction, probably leading to cell death. It is presumed that these pathomechanisms may contribute to the cortical leukodystrophy observed in GA-I patients.

  9. Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.

    Science.gov (United States)

    Westermann, C M; Dorland, L; Votion, D M; de Sain-van der Velden, M G M; Wijnberg, I D; Wanders, R J A; Spliet, W G M; Testerink, N; Berger, R; Ruiter, J P N; van der Kolk, J H

    2008-05-01

    The aim of the current study was to assess lipid metabolism in horses with atypical myopathy. Urine samples from 10 cases were subjected to analysis of organic acids, glycine conjugates, and acylcarnitines revealing increased mean excretion of lactic acid, ethylmalonic acid, 2-methylsuccinic acid, butyrylglycine, (iso)valerylglycine, hexanoylglycine, free carnitine, C2-, C3-, C4-, C5-, C6-, C8-, C8:1-, C10:1-, and C10:2-carnitine as compared with 15 control horses (12 healthy and three with acute myopathy due to other causes). Analysis of plasma revealed similar results for these predominantly short-chain acylcarnitines. Furthermore, measurement of dehydrogenase activities in lateral vastus muscle from one horse with atypical myopathy indeed showed deficiencies of short-chain acyl-CoA dehydrogenase (0.66 as compared with 2.27 and 2.48 in two controls), medium-chain acyl-CoA dehydrogenase (0.36 as compared with 4.31 and 4.82 in two controls) and isovaleryl-CoA dehydrogenase (0.74 as compared with 1.43 and 1.61 nmol min(-1) mg(-1) in two controls). A deficiency of several mitochondrial dehydrogenases that utilize flavin adenine dinucleotide as cofactor including the acyl-CoA dehydrogenases of fatty acid beta-oxidation, and enzymes that degrade the CoA-esters of glutaric acid, isovaleric acid, 2-methylbutyric acid, isobutyric acid, and sarcosine was suspected in 10 out of 10 cases as the possible etiology for a highly fatal and prevalent toxic equine muscle disease similar to the combined metabolic derangements seen in human multiple acyl-CoA dehydrogenase deficiency also known as glutaric acidemia type II.

  10. Clinical and neurocognitive outcome in symptomatic isovaleric acidemia

    Directory of Open Access Journals (Sweden)

    Grünert Sarah C

    2012-01-01

    Full Text Available Abstract Background Despite its first description over 40 years ago, knowledge of the clinical course of isovaleric acidemia (IVA, a disorder predisposing to severe acidotic episodes during catabolic stress, is still anecdotal. We aimed to investigate the phenotypic presentation and factors determining the neurological and neurocognitive outcomes of patients diagnosed with IVA following clinical manifestation. Methods Retrospective data on 21 children and adults with symptomatic IVA diagnosed from 1976 to 1999 were analyzed for outcome determinants including age at diagnosis and number of catabolic episodes. Sixteen of 21 patients were evaluated cross-sectionally focusing on the neurological and neurocognitive status. Additionally, 155 cases of patients with IVA published in the international literature were reviewed and analyzed for outcome parameters including mortality. Results 57% of study patients (12/21 were diagnosed within the first weeks of life and 43% (9/21 in childhood. An acute metabolic attack was the main cause of diagnostic work-up. 44% of investigated study patients (7/16 showed mild motor dysfunction and only 19% (3/16 had cognitive deficits. No other organ complications were found. The patients' intelligence quotient was not related to the number of catabolic episodes but was inversely related to age at diagnosis. In published cases, mortality was high (33% if associated with neonatal diagnosis, following manifestation at an average age of 7 days. Conclusions Within the group of "classical" organic acidurias, IVA appears to be exceptional considering its milder neuropathologic implications. The potential to avoid neonatal mortality and to improve neurologic and cognitive outcome under early treatment reinforces IVA to be qualified for newborn screening.

  11. Central Venous Catheter-Related Bloodstream Infection with Kocuria kristinae in a Patient with Propionic Acidemia.

    Science.gov (United States)

    Kimura, Masato; Kawai, Eichiro; Yaoita, Hisao; Ichinoi, Natsuko; Sakamoto, Osamu; Kure, Shigeo

    2017-01-01

    Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The patient had a central venous catheter implanted for total parenteral nutrition approximately 6 months prior to the onset of symptoms because of repeated acute pancreatitis. K. kristinae was isolated from two sets of blood cultures collected from the catheter. Vancomycin followed by cefazolin for 16 days and 5-day ethanol lock therapy successfully eradicated the K. kristinae bacteremia. Although human infections with this organism appear to be rare and are sometimes considered to result from contamination, physicians should not underestimate its significance when it is isolated in clinical specimens.

  12. Central Venous Catheter-Related Bloodstream Infection with Kocuria kristinae in a Patient with Propionic Acidemia

    Directory of Open Access Journals (Sweden)

    Masato Kimura

    2017-01-01

    Full Text Available Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The patient had a central venous catheter implanted for total parenteral nutrition approximately 6 months prior to the onset of symptoms because of repeated acute pancreatitis. K. kristinae was isolated from two sets of blood cultures collected from the catheter. Vancomycin followed by cefazolin for 16 days and 5-day ethanol lock therapy successfully eradicated the K. kristinae bacteremia. Although human infections with this organism appear to be rare and are sometimes considered to result from contamination, physicians should not underestimate its significance when it is isolated in clinical specimens.

  13. Towards the development of an enzyme replacement therapy for the metabolic disorder propionic acidemia

    Directory of Open Access Journals (Sweden)

    Mahnaz Darvish-Damavandi

    2016-09-01

    Full Text Available Propionic acidemia (PA is a life-threatening disease caused by the deficiency of a mitochondrial biotin-dependent enzyme known as propionyl coenzyme-A carboxylase (PCC. This enzyme is responsible for degrading the metabolic intermediate, propionyl coenzyme-A (PP-CoA, derived from multiple metabolic pathways. Currently, except for drastic surgical and dietary intervention that can only provide partial symptomatic relief, no other form of therapeutic option is available for this genetic disorder. Here, we examine a novel approach in protein delivery by specifically targeting and localizing our protein candidate of interest into the mitochondrial matrix of the cells. In order to test this concept of delivery, we have utilized cell penetrating peptides (CPPs and mitochondria targeting sequences (MTS to form specific fusion PCC protein, capable of translocating and localizing across cell membranes. In vitro delivery of our candidate fusion proteins, evaluated by confocal images and enzymatic activity assay, indicated effectiveness of this strategy. Therefore, it holds immense potential in creating a new paradigm in site-specific protein delivery and enzyme replacement therapeutic for PA.

  14. The M405V allele of the glutaryl-CoA dehydrogenase gene is an important marker for glutaric aciduria type I (GA-I) low excretors.

    Science.gov (United States)

    Schillaci, Lori-Anne P; Greene, Carol L; Strovel, Erin; Rispoli-Joines, Jessica; Spector, Elaine; Woontner, Michael; Scharer, Gunter; Enns, Gregory M; Gallagher, Renata; Zinn, Arthur B; McCandless, Shawn E; Hoppel, Charles L; Goodman, Stephen I; Bedoyan, Jirair K

    2016-09-01

    Glutaric aciduria type I (GA-I) is an autosomal recessive organic aciduria resulting from a functional deficiency of glutaryl-CoA dehydrogenase, encoded by GCDH. Two clinically indistinguishable diagnostic subgroups of GA-I are known; low and high excretors (LEs and HEs, respectively). Early medical and dietary interventions can result in significantly better outcomes and improved quality of life for patients with GA-I. We report on nine cases of GA-I LE patients all sharing the M405V allele with two cases missed by newborn screening (NBS) using tandem mass spectrometry (MS/MS). We describe a novel case with the known pathogenic M405V variant and a novel V133L variant, and present updated and previously unreported clinical, biochemical, functional and molecular data on eight other patients all sharing the M405V allele. Three of the nine patients are of African American ancestry, with two as siblings. GCDH activity was assayed in six of the nine patients and varied from 4 to 25% of the control mean. We support the use of urine glutarylcarnitine as a biochemical marker of GA-I by demonstrating that glutarylcarnitine is efficiently cleared by the kidney (50-90%) and that plasma and urine glutarylcarnitine follow a linear relationship. We report the allele frequencies for three known GA-I LE GCDH variants (M405V, V400M and R227P) and note that both the M405V and V400M variants are significantly more common in the population of African ancestry compared to the general population. This report highlights the M405V allele as another important molecular marker in patients with the GA-I LE phenotype. Therefore, the incorporation into newborn screening of molecular screening for the M405V and V400M variants in conjunction with MS/MS could help identify asymptomatic at-risk GA-I LE patients that could potentially be missed by current NBS programs. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Functional characterization of novel genotypes and cellular oxidative stress studies in propionic acidemia.

    Science.gov (United States)

    Gallego-Villar, Lorena; Pérez-Cerdá, Celia; Pérez, Belén; Abia, David; Ugarte, Magdalena; Richard, Eva; Desviat, Lourdes R

    2013-09-01

    Propionic acidemia (PA), caused by a deficiency of the mitochondrial biotin dependent enzyme propionyl-CoA carboxylase (PCC) is one of the most frequent organic acidurias in humans. PA is caused by mutations in either the PCCA or PCCB genes encoding the α- and β-subunits of the PCC enzyme which are assembled as an α6β6 dodecamer. In this study we have investigated the molecular basis of the defect in ten fibroblast samples from PA patients. Using homology modeling with the recently solved crystal structure of the PCC holoenzyme and a eukaryotic expression system we have analyzed the structural and functional effect of novel point mutations, also revealing a novel splice defect by minigene analysis. In addition, we have investigated the contribution of oxidative stress to cellular damage measuring reactive oxygen species (ROS) levels and apoptosis parameters in patient fibroblasts, as recent studies point to a secondary mitochondrial dysfunction as pathophysiological mechanism in this disorder. The results show an increase in intracellular ROS content compared to controls, correlating with the activation of the JNK and p38 signaling pathways. Highest ROS levels were present in cells harboring functionally null mutations, including one severe missense mutation. This work provides molecular insight into the pathogenicity of PA variants and indicates that oxidative stress may be a major contributing factor to the cellular damage, supporting the proposal of antioxidant strategies as novel supplementary therapy in this rare disease.

  16. Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive.

    Science.gov (United States)

    Baertling, Fabian; Al-Murshedi, Fathiya; Sánchez-Caballero, Laura; Al-Senaidi, Khalfan; Joshi, Niranjan P; Venselaar, Hanka; van den Brand, Mariël Am; Nijtmans, Leo Gj; Rodenburg, Richard Jt

    2017-06-01

    COX5A is a nuclear-encoded subunit of mitochondrial respiratory chain complex IV (cytochrome c oxidase). We present patients with a homozygous pathogenic variant in the COX5A gene. Clinical details of two affected siblings suffering from early-onset pulmonary arterial hypertension, lactic acidemia, failure to thrive, and isolated complex IV deficiency are presented. We show that the variant lies within the evolutionarily conserved COX5A/COX4 interface domain, suggesting that it alters the interaction between these two subunits during complex IV biogenesis. In patient skin fibroblasts, the enzymatic activity and protein levels of complex IV and several of its subunits are reduced. Lentiviral complementation rescues complex IV deficiency. The monomeric COX1 assembly intermediate accumulates demonstrating a function of COX5A in complex IV biogenesis. A potential therapeutic lead is demonstrated by showing that copper supplementation leads to partial rescue of complex IV deficiency in patient fibroblasts. © 2017 Wiley Periodicals, Inc.

  17. Liquid-liquid phase separation in internally mixed magnesium sulfate/glutaric acid particles

    Science.gov (United States)

    Wu, Feng-Min; Wang, Xiao-Wei; Jing, Bo; Zhang, Yun-Hong; Ge, Mao-Fa

    2018-04-01

    The confocal Raman microscopy is utilized to investigate the liquid-liquid phase separation (LLPS) of mixed magnesium sulfate/glutaric acid (MgSO4/GA) droplets deposited on a hydrophobic polytetrafluoroethylene (PTFE) substrate and a hydrophilic quartz substrate. Raman spectra collected from different regions of the mixed droplets provide detailed information of component distributions for MgSO4 and GA. During the dehydration process, the MgSO4/GA mixed particles show the initial liquid-liquid phase separation between 85% and 80% relative humidity (RH) on both the hydrophobic and hydrophilic substrates. For the droplets deposited on the two substrates, the inner phase of droplets is dominated by aqueous MgSO4, which is surrounded by a rich GA organic layer due to the surface tension effects. In addition, the crystallization of GA could be observed in the organic aqueous phase while it is inhibited in the inner MgSO4 phase due to the effects of gel formation of MgSO4 at low RH. The Raman spectra reveal that with decreasing RH the morphology of the mixed droplet evolves from a uniform droplet to the structure of LLPS with the GA crystallizing in the outer layer and MgSO4 gel formed in the inner phase. These findings contribute to the further understanding of the role of interactions between inorganic salts and organic acids on the morphological evolution and environmental effects of atmospheric aerosols under ambient RH conditions.

  18. Development and Validation of a GC-FID Method for Diagnosis of Methylmalonic Acidemia

    Directory of Open Access Journals (Sweden)

    Fatemeh Keyfi

    2016-05-01

    Full Text Available Background: Urinary organic acids are water-soluble intermediates and end products of the metabolism of amino acids, carbohydrates, lipids, and a number of other metabolic processes. In the hereditary diseases known as organic acidurias, an enzyme or co-factor defect in a metabolic pathway leads to the accumulation and increased excretion of one or more of these acidic metabolites. Gas chromatography is the most commonly-used technology to separate and identify these metabolites. In this report the analytical conditions for the determination of methylmalonic acid using a gas chromatography/flame ionization detector (GC-FID are studied with the aim to establish a method to analyze organic acids in human urine. Methods: Studies included the GC-FID method development, the conditions of the derivatization (trimethylsilylation reaction, and the stability of the methylmalonic acid standard solution and trimethylsilyl derivatives during storage. Also, a systematic comparison between GC-FID and gas chromatography/mass spectrometry (GC-MS was performed. Results: The highest resolution and sensitivity were obtained at 60 oC with a 30 min reaction time. Standard solutions and derivatized samples were stable for 7 days at 4-8 oC. Relative standard deviations of within-day and day-to-day assay results were less than 5%. Methylmalonic acid was detected in thirty human urine samples by the proposed GC-FID, and the results were compared with gold standard technique GC-MS. The correlation coefficient between GC-MS and GC-FID was obtained with R2= 0.997. Conclusions: The developed method was applied to the quantitative analysis of methylmalonic acid in urine from hospitalized children with methylmalonic acidemia. With this method we aim to support pediatric clinics in Iran and assist in clinical diagnostics.

  19. Adult methylmalonic acidemia presented as neuromyelitis optica: one case report

    Directory of Open Access Journals (Sweden)

    Sheng-de LI

    2015-10-01

    Full Text Available A 26-year-old male was admitted to our department, complaining of cognitive impairment, urine incontinence for 3 months, blurred vision for one month and numbness of bilateral lower limbs for 20 days. Presumed as “depression” and “viral encephalitis”, antidepressant and dexamethasone had been given but had no response. Neurological examination demonstrated impaired orientation to time and place; hearing impairment of right ear; normal muscle force in upper limbs, proximal lower muscle force was 2 and distal was 0; normal tendon reflex in both upper limbs; diminished tendon reflex in both lower limbs; left palmomental reflex (+; bilateral Babinski sign (+. Below T10: diminished superficial, deep sensation and cortical sensory. Cranial MRI on admission revealed widened sulci in bilateral cleft and frontal, temporal and insular lobes, indicating brain atrophy. Spinal MRI revealed high-intensity signals of C3-7 level and T1-12 level. The patient was diagnosed as “neuromyelitis optica (NMO” at first, but cognitive impairment is really rare in NMO. It finally turned out to be “inherited metabolic diseases” with the negative results of aquaporin 4 (AQP4, NMO-IgG, GM1, voltage-gated potassium channel (VGKC from serum and cerebrospinal fluid (CSF. The elevated level of plasm homocysteine [30.79 mmol/L (5-20 mmol/L] and urine methylmalonic acid [0.40 mmol/L (0.001 mmol/L] ascertained the diagnosis of methylmalonic acidemia. The patient was given oral treatment of folate 5 mg (3 times a day, 13 days and levocarnitine 1 g (3 times a day, 8 days and intramuscular injection of mecobalamine 1mg (once a day, 4 days or 0.50 mg (once a day, 8 days and adenosylcobalamine 0.50 mg (once a day, 8 days. Sixteen days on discharge, the patient’s neurological examination revealed no obvious recovery of vision; lower muscle force: about Ⅳ, right sensory level: T12-L1, and left sensory level lowered to L3. Reexamination of MRI revealed brain atrophy

  20. Sodium bicarbonate treatment during transient or sustained lactic acidemia in normoxic and normotensive rats.

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    Franco Valenza

    Full Text Available INTRODUCTION: Lactic acidosis is a frequent cause of poor outcome in the intensive care settings. We set up an experimental model of lactic acid infusion in normoxic and normotensive rats to investigate the systemic effects of lactic acidemia per se without the confounding factor of an underlying organic cause of acidosis. METHODOLOGY: Sprague Dawley rats underwent a primed endovenous infusion of L(+ lactic acid during general anesthesia. Normoxic and normotensive animals were then randomized to the following study groups (n = 8 per group: S sustained infusion of lactic acid, S+B sustained infusion+sodium bicarbonate, T transient infusion, T+B transient infusion+sodium bicarbonate. Hemodynamic, respiratory and acid-base parameters were measured over time. Lactate pharmacokinetics and muscle phosphofructokinase enzyme's activity were also measured. PRINCIPAL FINDINGS: Following lactic acid infusion blood lactate rose (P<0.05, pH (P<0.05 and strong ion difference (P<0.05 drop. Some rats developed hemodynamic instability during the primed infusion of lactic acid. In the normoxic and normotensive animals bicarbonate treatment normalized pH during sustained infusion of lactic acid (from 7.22 ± 0.02 to 7.36 ± 0.04, P<0.05 while overshoot to alkalemic values when the infusion was transient (from 7.24 ± 0.01 to 7.53 ± 0.03, P<0.05. When acid load was interrupted bicarbonate infusion affected lactate wash-out kinetics (P<0.05 so that blood lactate was higher (2.9 ± 1 mmol/l vs. 1.0 ± 0.2, P<0.05, group T vs. T+B respectively. The activity of phosphofructokinase enzyme was correlated with blood pH (R2 = 0.475, P<0.05. CONCLUSIONS: pH decreased with acid infusion and rose with bicarbonate administration but the effects of bicarbonate infusion on pH differed under a persistent or transient acid load. Alkalization affected the rate of lactate disposal during the transient acid load.

  1. Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH)

    DEFF Research Database (Denmark)

    Keyser, B.; Muhlhausen, C.; Dickmanns, A.

    2008-01-01

    Glutaric aciduria type 1 (GA1) is an autosomal recessive neurometabolic disorder caused by mutations in the glutaryl-CoA dehydrogenase gene (GCDH), leading to an accumulation and high excretion of glutaric acid and 3-hydroxyglutaric acid. Considerable variation in severity of the clinical phenotype......Da GCDH complexes. Molecular modeling of mutant GCDH suggests that Met263 at the surface of the GCDH protein might be part of the contact interface to interacting proteins. These results indicate that reduced intramitochondrial stability as well as the impaired formation of homo- and heteromeric GCDH...

  2. Disorders of fatty acid oxidation and autosomal recessive polycystic kidney disease-different clinical entities and comparable perinatal renal abnormalities.

    Science.gov (United States)

    Hackl, Agnes; Mehler, Katrin; Gottschalk, Ingo; Vierzig, Anne; Eydam, Marcus; Hauke, Jan; Beck, Bodo B; Liebau, Max C; Ensenauer, Regina; Weber, Lutz T; Habbig, Sandra

    2017-05-01

    Differential diagnosis of prenatally detected hyperechogenic and enlarged kidneys can be challenging as there is a broad phenotypic overlap between several rare genetic and non-genetic disorders. Metabolic diseases are among the rarest underlying disorders, but they demand particular attention as their prognosis and postnatal management differ from those of other diseases. We report two cases of cystic, hyperechogenic and enlarged kidneys detected on prenatal ultrasound images, resulting in the suspected diagnosis of autosomal recessive polycystic kidney disease (ARPKD). Postnatal clinical course and work-up, however, revealed early, neonatal forms of disorders of fatty acid oxidation (DFAO) in both cases, namely, glutaric acidemia type II, based on identification of the novel, homozygous splice-site mutation c.1117-2A > G in the ETFDH gene, in one case and carnitine palmitoyltransferase II deficiency in the other case. Review of pre- and postnatal sonographic findings resulted in the identification of some important differences that might help to differentiate DFAO from ARPKD. In DFAO, kidneys are enlarged to a milder degree than in ARPKD, and the cysts are located ubiquitously, including also in the cortex and the subcapsular area. Interestingly, recent studies have pointed to a switch in metabolic homeostasis, referred to as the Warburg effect (aerobic glycolysis), as one of the underlying mechanisms of cell proliferation and cyst formation in cystic kidney disease. DFAO are characterized by the inhibition of oxidative phosphorylation, resulting in aerobic glycolysis, and thus they do resemble the Warburg effect. We therefore speculate that this inhibition might be one of the pathomechanisms of renal hyperproliferation and cyst formation in DFAO analogous to the reported findings in ARPKD. Neonatal forms of DFAO can be differentially diagnosed in neonates with cystic or hyperechogenic kidneys and necessitate immediate biochemical work-up to provide early

  3. An unusual insertion/deletion in the gene encoding the β-subunit of propionyl-CoA carboxylase is a frequent mutation in Caucasian propionic acidemia

    International Nuclear Information System (INIS)

    Tahara, T.; Kraus, J.P.; Rosenberg, L.E.

    1990-01-01

    Propionic acidemia is an inherited disorder of organic acid metabolism that is caused by deficiency of propionly-CoA carboxylase. Affected patients fall into two complementation groups, pccA and pccBC (subgroups B, C, and BC), resulting from deficiency of the nonidentical α and β subunits of PCC, respectively. The authors have detected an unusual insertion/deletion in the DNA of patients from the pccBC and pccC subgroups that replaces 14 nucleotides in the coding sequence of the β subunit with 12 nucleotides unrelated to this region of the gene. Among 14 unrelated Caucasian patients in the pccBc complementation group, this unique mutation was found in 8 of 28 mutant alleles examined. Mutant allele-specific oligonucleotide hybridization to amplified genomic DNAs revealed that the inserted 12 nucleotides do not originate in an ∼1000-bp region around the mutation. In the course of the investigation, they identified another mutation in the same exon: a 3-bp in-frame deletion that eliminates one of two isoleucine codons immediately preceding the Msp I site. Two unrelated patients were compound heterozygotes for this single-codon deletion and for the insertion/deletion described above. They conclude that either there is a propensity for the PCC β-subunit gene to undergo mutations of this sort at this position or, more likely, the mutations in all of the involved Caucasian patients have a common origin in preceding generations

  4. Identification of the human mitochondrial FAD transporter and its potential role in multiple acyl-CoA dehydrogenase deficiency

    NARCIS (Netherlands)

    Spaan, András N.; Ijlst, Lodewijk; van Roermund, Carlo W. T.; Wijburg, Frits A.; Wanders, Ronald J. A.; Waterham, Hans R.

    2005-01-01

    Multiple acyl-CoA dehydrogenase deficiency (MADD) or glutaric aciduria type II (GAII) is most often caused by mutations in the genes encoding the alpha- or beta-subunit of electron transfer flavoprotein (ETF) or electron transfer flavoprotein dehydrogenase (ETF-DH). Since not all patients have

  5. Feasibility of adjunct therapeutic hypothermia treatment for hyperammonemia and encephalopathy due to urea cycle disorders and organic acidemias.

    Science.gov (United States)

    Lichter-Konecki, Uta; Nadkarni, Vinay; Moudgil, Asha; Cook, Noah; Poeschl, Johannes; Meyer, Michael T; Dimmock, David; Baumgart, Stephen

    2013-08-01

    Children with urea cycle disorders (UCDs) or organic acidemias (OAs) and acute hyperammonemia and encephalopathy are at great risk for neurological injury, developmental delay, intellectual disability, and death. Nutritional support, intravenous alternative pathway therapy, and dialysis are used to treat severe hyperammonemia associated with UCDs and nutritional support and dialysis are used to treat severe hyperammonemia in OAs. Brain protective treatment while therapy is initiated may improve neurological and cognitive function for the lifetime of the child. Animal experiments and small clinical trials in hepatic encephalopathy caused by acute liver failure suggest that therapeutic hypothermia provides neuroprotection in hyperammonemia associated encephalopathy. We report results of an ongoing pilot study that assesses if whole body cooling during rescue treatment of neonates with acute hyperammonemia and encephalopathy is feasible and can be conducted safely. Adjunct whole body therapeutic hypothermia was conducted in addition to standard treatment in acutely encephalopathic, hyperammonemic neonates with UCDs and OAs requiring dialysis. Therapeutic hypothermia was initiated using cooling blankets as preparations for dialysis were underway. Similar to standard therapeutic hypothermia treatment for neonatal hypoxic ischemic encephalopathy, patients were maintained at 33.5°C±1°C for 72h, they were then slowly rewarmed by 0.5°C every 3h over 18h. In addition data of age-matched historic controls were collected for comparison. Seven patients were cooled using the pilot study protocol and data of seven historic controls were reviewed. All seven patients survived the initial rescue and cooling treatment, 6 patients were discharged home 2-4weeks after hospitalization, five of them feeding orally. The main complication observed in a majority of patients was hypotension. Adjunct therapeutic hypothermia for neonates with UCDs and OAs receiving standard treatment was

  6. Methylmalonic acidemia

    Science.gov (United States)

    ... to diagnose this condition include: Ammonia test Blood gases Complete blood count CT scan or MRI of ... 2018, A.D.A.M., Inc. Duplication for commercial use must be authorized in writing by ADAM ...

  7. Diagnosis and management of glutaric aciduria type I--revised recommendations

    DEFF Research Database (Denmark)

    Kölker, Stefan; Christensen, Ernst; Leonard, James V

    2011-01-01

    is often difficult to treat, and the efficacy of available drugs cannot be predicted precisely in individual patients. The major aim of this revision is to re-evaluate the previous diagnostic and therapeutic recommendations for patients with this disease and incorporate new research findings...

  8. Structural studies on actinides carboxylates. 3. Preparation, properties and crystal structure of lithium glutarate hydrogenglutarate dioxouranate(VI)tetrahydrate UO/sub 2/(C/sub 5/H/sub 6/O/sub 4/)Li(C/sub 5/H/sub 7/O/sub 4/). 4H/sub 2/O

    Energy Technology Data Exchange (ETDEWEB)

    Benetollo, F; Bombieri, G [Consiglio Nazionale delle Ricerche, Padua (Italy). Lab. di Chimica e Tecnologia dei Radioelementi; Herrero, J A; Rojas, R M

    1979-01-01

    The synthesis, thermal behaviour and crystal structure of lithium glutaratehydrogenglutaratedioxouranate(VI) tetrahydrate is described. The compound crystallizes in the monoclinic system, space group P2/sub 1//n. The two glutarato ligands behave differently; one is bridging the uranyl groups in infinite chains running approximately in the a axis direction, the second is bridging the uranyl and the lithium ions. The carboxylic groups are chelated on the uranium and monodentate on the lithium. The structure is linked through a network of hydrogen bonding involving water molecules and oxygen atoms from the carboxylato groups. The geometry around the uranium is approximately hexagonal bipyramidal while the lithium is tetrahedrally coordinated with one glutarate oxygen and 3 water oxygens.

  9. Isovalerianeacidæmi--en sjælden og alvorlig defekt i nedbrydningen af leucin

    DEFF Research Database (Denmark)

    Lund, Ann-Britt Kiholm; Lund, Allan

    2011-01-01

    Isovaleric acidaemia (IVA) is an organic acidemia caused by deficient metabolism of the essential amino acid leucine. We describe the biochemistry, diagnostics, and treatment of IVA, and present the known Danish patients.......Isovaleric acidaemia (IVA) is an organic acidemia caused by deficient metabolism of the essential amino acid leucine. We describe the biochemistry, diagnostics, and treatment of IVA, and present the known Danish patients....

  10. Bench-to-bedside review: Treating acid–base abnormalities in the intensive care unit – the role of renal replacement therapy

    OpenAIRE

    Naka, Toshio; Bellomo, Rinaldo

    2004-01-01

    Acid–base disorders are common in critically ill patients. Metabolic acid–base disorders are particularly common in patients who require acute renal replacement therapy. In these patients, metabolic acidosis is common and multifactorial in origin. Analysis of acid–base status using the Stewart–Figge methodology shows that these patients have greater acidemia despite the presence of hypoalbuminemic alkalosis. This acidemia is mostly secondary to hyperphosphatemia, hyperlactatemia, and the accu...

  11. Poly[μ4-glutarato-di-μ3-glutarato-bis(1,10-phenanthrolinediyttrium(III

    Directory of Open Access Journals (Sweden)

    Ling Jin

    2011-08-01

    Full Text Available In the title complex, [Y2(C5H6O43(C12H8N22]n, three glutarate groups and two 1,10-phenanthroline molecules surround the two YIII ions. Both YIII ions are coordinated by two N atoms from the 1,10-phenanthroline, seven O atoms from five glutarate groups in a distorted tricapped trigonal–prismatic geometry. The YIII ions are bridged by glutarate ligands in three modes, forming a layered, polymeric structure. The resulting layers are assembled by π–π stacking interactions [centroid–centroid distances = 3.740 (3 and 3.571 (3 Å] into a three-dimensional supramolecular architecture.

  12. Methylmalonic Aciduria in Children: Clinical Recommendations

    Directory of Open Access Journals (Sweden)

    Alexander A. Baranov

    2017-01-01

    Full Text Available Methylmalonic acidemia (aciduria is an inherited metabolic disturbance from the group of organic acidemias (acidurias. The article presents etiopathogenetic, epidemiological, diagnostic, and therapeutic aspects of the problem. The possibilities of laboratory and instrumental diagnostic methods the tactics of dietary correction of metabolic disorders in acute and interstitial periods of the disease are described in details; features of drug treatment are outlined. The necessary information for clinical practice and patients’ everyday life is given in the article.

  13. Fatores de risco maternos associados à acidose fetal Maternal risk factors associated with fetal acidosis

    Directory of Open Access Journals (Sweden)

    José Mauro Madi

    2010-09-01

    Full Text Available OBJETIVOS: avaliar os fatores de risco maternos associados à acidose fetal. MÉTODOS: estudo tipo caso-controle composto por 188 recém-nascidos, sendo que 47 compuseram o grupo casos (pH de artéria umbilical OBJECTIVES: to assess maternal risk factors associated with fetal acidosis. METHODS: a case-control type study was conducted of 188 neonates, of whom 47 comprised the case group (umbilical arterial pH <7.0 and 141 the control (umbilical arterial pH E7.1 <7.3. The study included only single-gestation neonates without congenital malformations. Both maternal and fetal variables were taken into consideration. Statistical analysis involved the calculation of the raw and adjusted Odds Ratio, Student's t-test, the chi-squared test and multivariate analysis using Enter-method non-conditional logistic regression. The level of statistical significance was set at p<0.05. RESULTS: in the case group higher percentages of caesarian sections and pre-term births were observed, involving almost five times as much intensive care and twenty-five times more likelihood of Apgar in the 5th minute <7. No association was observed between the groups and fetal presentation, mother's age, history of miscarriage, years of schooling of mother or attendance at prenatal sessions. After multivariate analysis, the only risk factors that remained significant were complications relating to the placenta or the umbilical cord. Deliveries involving complications relating to the placenta or the umbilical cord were three times more likely to involve fetal acidemia. CONCLUSIONS: acidemia among neonates was associated with a higher percentage of caesarians, premature births, a need for intensive care and treatment and an Apgar index of <7 in the 5th minute. After multivariate analysis, complications relating to premature displacement of the placenta and the umbilical cord were the only remaining risk factors associated with fetal acidemia.

  14. Complexing Agents and pH Influence on Chemical Durability of Type I Molded Glass Containers.

    Science.gov (United States)

    Biavati, Alberto; Poncini, Michele; Ferrarini, Arianna; Favaro, Nicola; Scarpa, Martina; Vallotto, Marta

    2017-01-01

    Among the factors that affect the glass surface chemical durability, pH and complexing agents present in aqueous solution have the main role. Glass surface attack can be also related to the delamination issue causing glass particles' appearance in the pharmaceutical preparation. A few methods to check for glass containers delamination propensity and some control guidelines have been proposed. The present study emphasizes the possible synergy between a few complexing agents with pH on borosilicate glass chemical durability.Hydrolytic attack was performed in small-volume 23 mL type I glass containers autoclaved according to the European Pharmacopoeia or United States Pharmacopeia for 1 h at 121 °C, in order to enhance the chemical attack due to time, temperature, and the unfavorable surface/volume ratio. Solutions of 0.048 M or 0.024 M (M/L) of the acids citric, glutaric, acetic, EDTA (ethylenediaminetetraacetic acid), together with sodium phosphate with water for comparison, were used for the trials. The pH was adjusted ±0.05 units at fixed values 5.5, 6.6, 7, 7.4, 8, and 9 by LiOH diluted solution.Because silicon is the main glass network former, silicon release into the attack solutions was chosen as the main index of the glass surface attack and analysed by inductively coupled plasma atomic emission spectrophotometry. The work was completed by the analysis of the silicon release in the worst attack conditions of molded glass, soda lime type II glass, and tubing borosilicate glass vials to compare different glass compositions and forming technologies. Surface analysis by scanning electron microscopy was finally performed to check for the surface status after the worst chemical attack condition by citric acid. LAY ABSTRACT: Glass, like every packaging material, can have some usage limits, mainly in basic pH solutions. The issue of glass surface degradation particles that appear in vials (delamination) has forced a number of drug product recalls in recent years

  15. Hygroscopic properties of internally mixed particles composed of NaCl and water-soluble organic acids.

    Science.gov (United States)

    Ghorai, Suman; Wang, Bingbing; Tivanski, Alexei; Laskin, Alexander

    2014-02-18

    Atmospheric aging of naturally emitted marine aerosol often leads to formation of internally mixed particles composed of sea salts and water-soluble organic compounds of anthropogenic origin. Mixing of sea salt and organic components has profound effects on the evolving chemical composition and hygroscopic properties of the resulted particles, which are poorly understood. Here, we have studied chemical composition and hygroscopic properties of laboratory generated NaCl particles mixed with malonic acid (MA) and glutaric acid (GA) at different molar ratios using micro-FTIR spectroscopy, atomic force microscopy, and X-ray elemental microanalysis. Hygroscopic properties of internally mixed NaCl and organic acid particles were distinctly different from pure components and varied significantly with the type and amount of organic compound present. Experimental results were in a good agreement with the AIM modeling calculations of gas/liquid/solid partitioning in studied systems. X-ray elemental microanalysis of particles showed that Cl/Na ratio decreased with increasing organic acid component in the particles with MA yielding lower ratios relative to GA. We attribute the depletion of chloride to the formation of sodium malonate and sodium glutarate salts resulted by HCl evaporation from dehydrating particles.

  16. Complexing agents and pH influence on chemical durability of type I moulded glass containers.

    Science.gov (United States)

    Biavati, Alberto; Poncini, Michele; Ferrarini, Arianna; Favaro, Nicola; Scarpa, Martina; Vallotto, Marta

    2017-06-16

    Among the factors that affect the glass surface chemical durability, pH and complexing agents presence in aqueous solution have the main role (1). Glass surface attack can be also related to the delamination issue with glass particles appearance in the pharmaceutical preparation. A few methods to check for glass containers delamination propensity and some control guidelines have been proposed (2,3). The present study emphasizes the possible synergy between a few complexing agents with pH on the borosilicate glass chemical durability. Hydrolytic attack was performed in small volume 23 ml type I glass containers autoclaved according to EP or USP for 1 hour at 121°C, in order to enhance the chemical attack due to time, temperature and the unfavourable surface/volume ratio. 0,048 M or 0.024 M (moles/liter) solutions of the acids citric, glutaric, acetic, EDTA (ethylenediaminetetraacetic acid) and sodium phosphate with water for comparison, were used for the trials. The pH was adjusted ± 0,05 units at fixed values 5,5-6,6-7-7,4-8-9 by LiOH diluted solution. Since silicon is the main glass network former, silicon release into the attack solutions was chosen as the main index of the glass surface attack and analysed by ICPAES. The work was completed by the analysis of the silicon release in the worst attack conditions, of moulded glass, soda lime type II and tubing borosilicate glass vials to compare different glass compositions and forming technologies. Surface analysis by SEM was finally performed to check for the surface status after the worst chemical attack condition by citric acid. Copyright © 2017, Parenteral Drug Association.

  17. Inborn Errors of Intermediary Metabolism in Critically Ill Mexican Newborns

    Directory of Open Access Journals (Sweden)

    Ibarra-González Isabel MSc

    2014-04-01

    Full Text Available Inborn errors of intermediary metabolism (IEiM are complex diseases with high clinical heterogeneity, and some patients who have severe enzyme deficiencies or are subjected to stress (catabolism/infections actually decompensate in the neonatal period. In this study, we performed metabolic tests on 2025 newborns in Mexico admitted to 35 neonatal intensive care units or emergency wards (NICUs/EWs over a 6-year period, in whom a metabolic disorder was clinically suspected. Of these 2025 newborns with sickness, 11 had IEiM, revealing a prevalence of 1:184. Clinical characteristics and outcomes of the newborns with confirmed IEiM are shown. Of these 11 patients, 4 had isolated methylmalonic acidemia, 3 had maple syrup urine disease, 2 had urea cycle disorders, 1 had 3-hydroxy-3-methylglutaric acidemia, and 1 had isovaleric acidemia. During the first week of life (average 3 days, all of these newborns presented with impaired alertness, hypotonia, feeding difficulties, and vomiting along with metabolic acidosis and hyperammonemia. Of the 11 newborns with IEiM, 7 died, leading to a mortality rate of 64%. In conclusion, the differential diagnosis of newborns admitted to the NICU/EW must include IEiM, requiring systematic screening of this population.

  18. Genetics Home Reference: methylmalonic acidemia

    Science.gov (United States)

    ... Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens ... patients with the cblD inborn error of cobalamin metabolism. J Pediatr. 2009 Apr;154(4):551-6. ...

  19. Genetics Home Reference: propionic acidemia

    Science.gov (United States)

    ... Cerdá C, Scholl-Bürgi S, Skovby F, Wijburg F, MacDonald A, Martinelli D, Sass JO, Valayannopoulos V, Chakrapani ... Cerdá C, Scholl-Bürgi S, Skovby F, Wijburg F, MacDonald A, Martinelli D, Sass JO, Valayannopoulos V, Chakrapani ...

  20. [Intrapartum amnioinfusion in patients with meconium-stained amniotic fluid].

    Science.gov (United States)

    Engel, Karina; Samborska, Monika; Bilar, Marek; Sipak-Szmigiel, Olimpia; Ronin-Walknowska, Elzbieta

    2008-09-01

    The aim of the study was to evaluate the effect of intrapartum amnioinfusion in the presence of meconium stained amniotic fluid. 93 women with meconium-stained amniotic fluid were assigned to receive amnioinfusion or no amnioinfusion (128 women). The trials were evaluated for fetal distress syndrome, route of delivery, fetal acidemia, Apgar score at 1 and 5 min., meconium aspiration syndrome, postpartum endometritis and maternal hospital stays. Amnioinfusion in cases of meconium-stained fluid did not improve the number of fetal distress symptoms during fetal heart rate monitoring. Amnioinfusion was associated with a significant decrease of neonatal acidemia although it did not improve Apgar score. In our study amnioinfusion was not associated with reduction in the incidence of neonatal outcome and puerperial complications.

  1. Indirect coupling to Na+ of p-aminohippuric acid uptake into rat renal basolateral membrane vesicles

    International Nuclear Information System (INIS)

    Shimada, H.; Moewes, B.; Burckhardt, G.

    1987-01-01

    Experiments with basolateral membrane vesicles prepared from rat kidney cortex were performed to study the mechanism by which p-aminohippuric acid (PAH) is taken up across the contraluminal membrane and is concentrated in proximal tubule cells. An inward Na + gradient failed to stimulate [ 3 H]PAH uptake compared with K + or Li + and did not cause intravesicular PAH accumulation above equilibrium distribution. In the absence of Na + , the dicarboxylates glutarate and suberate cis-inhibited and trans-stimulated [ 3 H]PAH uptake, indicating a common transport system. In the presence of Na + , 10 μM glutarate in the incubation medium did not cis-inhibit, but rather stimulated [ 3 H]PAH uptake and caused PAH accumulation above equilibrium distribution (over-shoot). Li + diminished this stimulation, but was without effect on [ 3 H]PAH/PAH- and [ 3 H]PAH/glutarate exchange. The data indicate the coexistence of a Na + -sensitive transport system for dicarboxylates and a Li + -insensitive PAH/dicarboxylate exchanger in the basolateral membrane. The authors propose that dicarboxylates are cotransported with Na + into the cell and subsequently exchange for extracellular PAH at the basolateral membrane. PAH uptake is thereby indirectly coupled to Na + via the Na + /dicarboxylate cotransporter

  2. Congenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders.

    Science.gov (United States)

    Krishna, Shri H; McKinney, Alexander M; Lucato, Leandro T

    2014-04-01

    Numerous congenital-genetic inborn errors of metabolism (CIEMs) have been identified and characterized in detail within recent decades, with promising therapeutic options. Neuroimaging is becoming increasingly utilized in earlier stages of CIEMs, and even in asymptomatic relatives of patients with a CIEM, so as to monitor disease progress and treatment response. This review attempts to summarize in a concise fashion the neuroimaging findings of various CIEMs that may present in adulthood, as well as those that may persist into adulthood, whether because of beneficial therapy or a delay in diagnosis. Notably, some of these disorders have neuroimaging findings that differ from their classic infantile or early childhood forms, whereas others are identical to their early pediatric forms. The focus of this review is their appearance on routine magnetic resonance imaging sequences, with some basic attention to the findings of such CIEMs on specialized neuroimaging, based on recent or preliminary research. The general classes of disorders covered in this complex review are: peroxisomal disorders (adrenoleukodystrophy), lysosomal storage disorders (including metachromatic leukodystrophy, Krabbe or globoid cell leukodystrophy, Fabry, Niemann-Pick, GM1, GM2, Gaucher, mucopolysaccharidoses, and Salla diseases), mitochondrial disorders (including mitochondrial encephalomyopathy with lactic acidosis and strokelike episodes, myoclonic epilepsy with ragged red fibers, Leigh disease, and Kearns-Sayre syndrome), urea cycle disorders, several organic acidemias (including phenylketonuria, maple syrup urine disease, 3-hydroxy-3-methylglutaryl colyase deficiency, glutaric acidurias, methylmalonic academia, proprionic academia, 3-methylglucatonic aciduria, and 2-hydroxyglutaric acidurias), cytoskeletal or transporter molecule defects (including Alexander or fibrinoid leukodystrophy, proteolipid protein-1 defect or Pelizaeus Merzbacher, Wilson, and Huntington diseases), and several

  3. A chiral mixed carboxylate, [Nd4(H2O)2(OOC(CH2)3COO)4(C2O4)2], exhibiting NLO properties

    International Nuclear Information System (INIS)

    Vaidhyanathan, R.; Natarajan, Srinivasan; Rao, C.N.R.

    2004-01-01

    Reaction of a mixture of neodymium carbonate, HCl, oxamic acid and glutaric acid under hydrothermal conditions gives rise to a new mixed carboxylate of neodymium, [Nd 4 (H 2 O) 2 (OOC(CH 2 ) 3 COO) 4 (C 2 O 4 ) 2 ], I. The structure, determined using single crystal X-ray diffraction, comprises a helical column formed by the grafting of the oxalate unit on to helical NdO 9 chains, cross-linked by the glutarate anions. It is noteworthy that the pitch of the helix is equivalent to the length of the oxalate unit. Furthermore, I shows about 1.1 times the SHG activity of urea

  4. Decomposition of no-clean solder flux systems and their effects on the corrosion reliability of electronics

    DEFF Research Database (Denmark)

    Conseil, Helene; Verdingovas, Vadimas; Jellesen, Morten Stendahl

    2016-01-01

    No-clean flux systems are used today for the soldering of electronic printed circuit board assemblies assuming that all the aggressive substances of the flux will vanish during the soldering process i.e. evaporate, decompose or being enclosed safely in the residues. However this is not true in most...... that the fluxes do not decompose fully within the temperature regime of the soldering process, leaving behind significant level of weak organic acid residues. The residue depending on the type and amount can be can be very aggressive towards the corrosion on the printed circuit board assemblies. The glutaric acid...

  5. Expanded Newborn Screening for Inborn Errors of Metabolism and Genetic Characteristics in a Chinese Population

    Directory of Open Access Journals (Sweden)

    Kejian Guo

    2018-04-01

    Full Text Available The incidence of inborn errors of metabolisms (IEMs varies dramatically in different countries and regions. Expanded newborn screening for IEMs by tandem mass spectrometry (MS/MS is an efficient approach for early diagnosis and presymptomatic treatment to prevent severe permanent sequelae and death. To determine the characteristics of IEMs and IEMs-associated mutations in newborns in Jining area, China, 48,297 healthy neonates were recruited for expanded newborn screening by MS/MS. The incidence of IEMs was 1/1178 in Jining, while methylmalonic acidemia, phenylketonuria, and primary carnitine deficiency ranked the top 3 of all detected IEMs. Thirty mutations in nine IEMs-associated genes were identified in 28 confirmed cases. As 19 cases with the mutations in phenylalanine hydroxylase (PAH, solute carrier family 22 member 5 (SLC22A5, and methylmalonic aciduria (cobalamin deficiency cblC type with homocystinuria (MMACHC genes, respectively, it suggested that mutations in the PAH, SLC22A5, and MMACHC genes are the predominant causes of IEMs, leading to the high incidence of phenylketonuria, primary carnitine deficiency, and methylmalonic acidemia, respectively. Our work indicated that the overall incidence of IEMs is high and the mutations in PAH, SLC22A5, and MMACHC genes are the leading causes of IEMs in Jining area. Therefore, it is critical to increase the coverage of expanded newborn screening by MS/MS and prenatal genetic consulting in Jining area.

  6. Ethanol Induced Urine Acidification is Related with Early Acetaldehyde Concentration

    Directory of Open Access Journals (Sweden)

    Soon Kil Kwon

    2014-06-01

    Conclusion: In conclusion, urine acidification after ethanol ingestion is related with serum acetaldehyde concentration. Early elevation of acetaldhyde could induce urine acidification, but the urine pH was elevated after a few hours, that might make prolonged acidemia.

  7. Tandem Mass Neonatal Screening in Taiwan—Report from One Center

    Directory of Open Access Journals (Sweden)

    Hsiang-Po Huang

    2006-01-01

    Conclusion: We found that MS/MS neonatal screening was valuable in the early diagnosis of severe and treatable inborn errors of metabolism such as organic acidemias and urea cycle disorders. It also detected less severe disorders that required only observation.

  8. Uso de bicarbonato de sódio na acidose metabólica do paciente gravemente enfermo Sodium bicarbonate in the critically lll patient with metabolic acidosis

    Directory of Open Access Journals (Sweden)

    Paulo Novis Rocha

    2009-12-01

    Full Text Available A acidose lática é um distúrbio do equilíbrio ácido-base muito frequente em pacientes internados em unidades de terapia intensiva e está associado a um mau prognóstico. Embora exista um acúmulo substancial de evidências de que níveis críticos de acidemia provocam inúmeros efeitos adversos sobre o funcionamento celular, a utilização de bicarbonato de sódio para o tratamento da acidose lática em pacientes gravemente enfermos permanece alvo de controvérsias. Neste artigo, pretendemos: 1 analisar as principais diferenças entre as acidoses hiperclorêmicas e as acidoses orgânicas, com ânion gap (AG elevado, visando embasar a discussão sobre os fundamentos da terapia com bicarbonato de sódio nas acidoses metabólicas; 2 avaliar os riscos associados à persistência de níveis críticos de acidemia, contrastando-os com os riscos do uso de bicarbonato de sódio; 3 analisar criticamente as evidências da literatura sobre o uso de bicarbonato de sódio no tratamento da acidose lática do paciente crítico, com ênfase em ensaios clínicos randomizados em seres humanos; 4 fornecer um fundamento para a utilização judiciosa de bicarbonato de sódio nesta situação.Lactic acidosis is an acid-base imbalance frequently found in critically ill patients. It is associated with a poor prognosis. Despite the substantial body of evidence that critical levels of acidemia have several adverse effects on cell function, the use of sodium bicarbonate to treat lactic acidosis in critically ill patients remains highly controversial. This article aimed at: 1 analyzing the main differences between hyperchloremic and organic acidoses, with high anion gap; 2 comparing the risks associated with critical levels of acidemia with those associated with the use of sodium bicarbonate; 3 critically analyzing the literature evidence about the use of sodium bicarbonate for the treatment of lactic acidosis in critically ill patients, with an emphasis on randomized

  9. Structure of β- N-dimethylamino-4-dodecyloxypropiophenone complexes with di- and polycarboxylic acids

    Science.gov (United States)

    Lebedeva, Tamara L.; Shandryuk, George A.; Sycheva, Tatyana I.; Bezborodov, Vladimir S.; Talroze, Raissa V.; Platé, Nicolai A.

    1995-07-01

    The type of bonds responsible for the complexation of di- and polyacids with the tertiary amine β- N-dimethylamino-4-dodecyloxypropiophenone is studied by means of FTIR spectroscopy. The complexes are shown to be stable due to strong H-bonding with partial charge transfer. The characteristic composition for complexes of polyacrylic, polymethacrylic and malonic acids is calculated as 2:1 (number of carboxylic groups per number of amine molecules) whereas glutaric acid forms complexes of different composition including 1:1. The characteristic composition results from the structure of the initial acid. The structures of both the characteristic complex and "excess" acid are also discussed.

  10. The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia.

    Science.gov (United States)

    Lee, Young Mok; Pan, Chi-Jiunn; Koeberl, Dwight D; Mansfield, Brian C; Chou, Janice Y

    2013-11-01

    Glycogen storage disease type-Ia (GSD-Ia) patients deficient in glucose-6-phosphatase-α (G6Pase-α or G6PC) manifest impaired glucose homeostasis characterized by fasting hypoglycemia, growth retardation, hepatomegaly, nephromegaly, hyperlipidemia, hyperuricemia, and lactic acidemia. Two efficacious recombinant adeno-associated virus pseudotype 2/8 (rAAV8) vectors expressing human G6Pase-α have been independently developed. One is a single-stranded vector containing a 2864-bp of the G6PC promoter/enhancer (rAAV8-GPE) and the other is a double-stranded vector containing a shorter 382-bp minimal G6PC promoter/enhancer (rAAV8-miGPE). To identify the best construct, a direct comparison of the rAAV8-GPE and the rAAV8-miGPE vectors was initiated to determine the best vector to take forward into clinical trials. We show that the rAAV8-GPE vector directed significantly higher levels of hepatic G6Pase-α expression, achieved greater reduction in hepatic glycogen accumulation, and led to a better toleration of fasting in GSD-Ia mice than the rAAV8-miGPE vector. Our results indicated that additional control elements in the rAAV8-GPE vector outweigh the gains from the double-stranded rAAV8-miGPE transduction efficiency, and that the rAAV8-GPE vector is the current choice for clinical translation in human GSD-Ia. © 2013.

  11. Fetal blood gas values during fetoscopic myelomeningocele repair performed under carbon dioxide insufflation.

    Science.gov (United States)

    Baschat, Ahmet A; Ahn, Edward S; Murphy, Jamie; Miller, Jena L

    2018-05-10

    Fetoscopic myelomeningocele (MMC) repair is performed with intrauterine carbon dioxide (CO 2 ) insufflation. While lamb experiments have shown significant fetal acidemia following CO 2 insufflation corresponding information for human pregnancies is not available. We performed umbilical venous cord blood sampling in three patients during fetoscopic MMC repair at 25+1, 25+3 and 24+1 weeks gestation. Fetal venous pH at the beginning of CO 2 insufflation were 7.36, 7.46 and 7.37; repeat values were 7.28, 7.35, 7.36 after 181, 159 and 149 minutes respectively. The partial pressure of oxygen and carbon dioxide was maintained in the normal range at these times and pH decrease was less in patient 3 receiving humidified CO2 insufflation. Our observations suggest that in contrast to sheep experiments, CO2 insufflation during fetoscopic myelomeningocele repair does not cause fetal acidemia. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  12. Synthesis, mechanical, thermal and chemical properties of ...

    Indian Academy of Sciences (India)

    Unknown

    Cardanol, an excellent monomer for polymer production, has been isolated from CNSL and allowed to ... solution), and 1% glutaric acid catalyst in methanol were refluxed .... volving terminal isocyanate groups with active hydrogen present in ...

  13. [Interference for Various Quench Agents of Chemical Disinfectants on Detection of Endotoxin Activities in Water].

    Science.gov (United States)

    Zhang, Can; Liu, Wen-jun; Shi, Yun; An, Dai-zhi; Bai, Miao; Xu, Wen

    2015-05-01

    The quenching agents such as histidine, glycine, ascorbic acid, Tween-80, sodium sulfite and sodium hyposulfite are commonly used for quenching the residual disinfectant in water. In this paper, in order to select the optimal type and concentration range of quenching agents prior to the Limulus assays, the interference effects of each quenching agent at different concentrations on endotoxin detection were investigated by the Limulus assays of kinetic-turbidity. Our results identified that, as for 0-1.0% concentration of histidine, ascorbic acid, Tween-80, sodium sulfite (pH unadjusted and pH neutral), interference on the Limulus assays was existed. Hence, these quenching agents could not be applied as neutralizers prior to Limulus assays. Although, there was no interference on endotoxin detection for the glycine, a yellow color, developed by the quenching products of glycine and glutaric dialdehyde, contributed to false positive results. Hence, glycine should not be used as quenching agents in Limulus assays for samples containing glutaric dialdehyde. Compared with other quenching agents as histidine, glycine, ascorbic acid, Tween-80, sodium sulfite, 0-1.0% concentration of sodium hyposulfite elicited no obvious interference, while 1.0%-5.0% concentration of sodium hyposulfite illustrated exhibition effect for endotoxin detection. All in all, compared with other quenching agents as histidine, glycine, ascorbic acid, Tween-80 and sodium sulfite, sodium hyposulfite is suitable for quenching chemicals prior to endotoxin detection and less than 0.5% of concentration is allowable.

  14. An assessment of predictive value of the biophysical profile in women with preeclampsia using data from the fullPIERS database.

    Science.gov (United States)

    Payne, Beth A; Kyle, Phillipa M; Lim, Kenneth; Lisonkova, Sarka; Magee, Laura A; Pullar, Barbra; Qu, Ziguang; von Dadelszen, Peter

    2013-07-01

    Pre-eclampsia is associated with increased risk to both the mother and fetus. Effective monitoring of the fetal condition is essential to the management of women with pre-eclampsia. The biophysical profile (BPP) is one monitoring tool available to clinicians. To compare the BPP test with cardiotocography/non-stress test (CTG/NST) alone for their ability to predict fetal acidemia at birth or a composite adverse perinatal outcome among women with preeclampsia and to estimate the effect of BPP assessment on mode of delivery and birth outcome. Secondary analysis of a prospective cohort of women with preeclampsia. The predictive ability of the tests was assessed based on sensitivity, specificity, positive and negative likelihood ratios (LR+, LR-). Women assessed with the BPP were compared with matched controls not assessed with the BPP to determine the odds of Cesarean delivery or adverse perinatal outcomes after adjustment for potential confounders. Five out of 89 women (5.6%) had an abnormal BPP; 18 out of 89 (20.2%) had an abnormal CTG/NST. Fetal acidemia was diagnosed in 13 fetuses (14.6%); composite adverse perinatal outcome in 68 fetuses/infants (76.4%). Both tests had relatively poor predictive performance for both outcomes (LR+ between 2.50 and 3.90 and LR- between 0.64 and 0.93). Assessment with the BPP was positively associated with fetal acidemia (adjusted OR 4.84; 95% CI 1.33-17.66). The BPP and CTG/NST alone were poor predictors of perinatal outcome in this cohort; multiple tests should be considered when assessing fetal risk in women with preeclampsia. Crown Copyright © 2013. Published by Elsevier B.V. All rights reserved.

  15. Renal and cerebral responses to hypoxemia in the ovine fetus

    NARCIS (Netherlands)

    Mild to moderate hypoxemia without major changes in pH and pCO(2) does not reduce fetal renal blood flow and fetal urine production rate. Other factors such as acidemia, hypercapnia or changes in lung liquid production or fetal swallowing are candidates for the reduced amniotic fluid in the growth

  16. Adenoviral-mediated correction of methylmalonyl-CoA mutase deficiency in murine fibroblasts and human hepatocytes

    Directory of Open Access Journals (Sweden)

    Korson Mark

    2007-04-01

    Full Text Available Abstract Background Methylmalonic acidemia (MMA, a common organic aciduria, is caused by deficiency of the mitochondrial localized, 5'deoxyadenosylcobalamin dependent enzyme, methylmalonyl-CoA mutase (MUT. Liver transplantation in the absence of gross hepatic dysfunction provides supportive therapy and metabolic stability in severely affected patients, which invites the concept of using cell and gene delivery as future treatments for this condition. Methods To assess the effectiveness of gene delivery to restore the defective metabolism in this disorder, adenoviral correction experiments were performed using murine Mut embryonic fibroblasts and primary human methylmalonyl-CoA mutase deficient hepatocytes derived from a patient who harbored two early truncating mutations, E224X and R228X, in the MUT gene. Enzymatic and expression studies were used to assess the extent of functional correction. Results Primary hepatocytes, isolated from the native liver after removal subsequent to a combined liver-kidney transplantation procedure, or Mut murine fibroblasts were infected with a second generation recombinant adenoviral vector that expressed the murine methylmalonyl-CoA mutase as well as eGFP from distinct promoters. After transduction, [1-14C] propionate macromolecular incorporation studies and Western analysis demonstrated complete correction of the enzymatic defect in both cell types. Viral reconstitution of enzymatic expression in the human methylmalonyl-CoA mutase deficient hepatocytes exceeded that seen in fibroblasts or control hepatocytes. Conclusion These experiments provide proof of principle for viral correction in methylmalonic acidemia and suggest that hepatocyte-directed gene delivery will be an effective therapeutic treatment strategy in both murine models and in human patients. Primary hepatocytes from a liver that was unsuitable for transplantation provided an important resource for these studies.

  17. Hydroxycinnamic acids are ester-linked directly to glucosyl moieties within the lignan macromolecule from flaxseed hulls

    NARCIS (Netherlands)

    Struijs, K.; Vincken, J.P.; Verhoef, R.P.; Voragen, A.G.J.; Gruppen, H.

    2008-01-01

    In flaxseed hulls, lignans are present in an oligomeric structure. Secoisolariciresinol diglucoside (SDG), ester-linked to hydroxy-methyl-glutaric acid (HMGA), forms the backbone of this lignan macromolecule. The hydroxycinnamic acids p-coumaric acid glucoside (CouAG) and ferulic acid glucoside

  18. Selective screening in neonates suspected to have inborn errors of ...

    African Journals Online (AJOL)

    Results: 13 patients (32.5%) were diagnosed as having IEM, 7 of them (53.8%) had urea cycle defect, 2 (15.4%) had maple syrup urine disease, while methylmalonic acidemia, fatty acid oxidation defect, mitochondrial disease, and galactosemia were diagnosed in one patient each (7.7%). Out of these patients, 12 patients ...

  19. Genetics Home Reference: methylmalonic acidemia with homocystinuria

    Science.gov (United States)

    ... or Free article on PubMed Central Kräutler B. Biochemistry of B12-cofactors in human metabolism. Subcell Biochem. ... not be used as a substitute for professional medical care or advice. Users with questions about a ...

  20. Infusion of sodium bicarbonate in experimentally induced metabolic acidosis does not provoke cerebrospinal fluid (CSF) acidosis in calves.

    Science.gov (United States)

    Abeysekara, Saman; Zello, Gordon A; Lohmann, Katharina L; Alcorn, Jane; Hamilton, Don L; Naylor, Jonathan M

    2012-01-01

    In a crossover study, 5 calves were made acidotic by intermittent intravenous infusion of isotonic hydrochloric acid (HCl) over approximately 24 h. This was followed by rapid (4 h) or slow (24 h) correction of blood pH with isotonic sodium bicarbonate (NaHCO(3)) to determine if rapid correction of acidemia produced paradoxical cerebrospinal fluid (CSF) acidosis. Infusion of HCl produced a marked metabolic acidosis with respiratory compensation. Venous blood pH (mean ± S(x)) was 7.362 ± 0.021 and 7.116 ± 0.032, partial pressure of carbon dioxide (Pco(2), torr) 48.8 ± 1.3 and 34.8 ± 1.4, and bicarbonate (mmol/L), 27.2 ± 1.27 and 11 ± 0.96; CSF pH was 7.344 ± 0.031 and 7.240 ± 0.039, Pco(2) 42.8 ± 2.9 and 34.5 ± 1.4, and bicarbonate 23.5 ± 0.91 and 14.2 ± 1.09 for the period before the infusion of hydrochloric acid and immediately before the start of sodium bicarbonate correction, respectively. In calves treated with rapid infusion of sodium bicarbonate, correction of venous acidemia was significantly more rapid and increases in Pco(2) and bicarbonate in CSF were also more rapid. However, there was no significant difference in CSF pH. After 4 h of correction, CSF pH was 7.238 ± 0.040 and 7.256 ± 0.050, Pco(2) 44.4 ± 2.2 and 34.2 ± 2.1, and bicarbonate 17.8 ± 1.02 and 14.6 ± 1.4 for rapid and slow correction, respectively. Under the conditions of this experiment, rapid correction of acidemia did not provoke paradoxical CSF acidosis.

  1. Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.

    Science.gov (United States)

    Bindu, P S; Taly, A B; Sonam, K; Govindaraju, C; Arvinda, H R; Gayathri, N; Bharath, M M Srinivas; Ranjith, D; Nagappa, M; Sinha, S; Khan, N A; Thangaraj, K

    2014-02-01

    Bilateral hypertrophic olivary degeneration on brain MRI has been reported in a few metabolic, genetic and neurodegenerative disorders, including mitochondrial disorders. In this report, we sought to analyse whether bilateral symmetrical inferior olivary nucleus hypertrophy is specifically associated with mitochondrial disorders in children. This retrospective study included 125 children (mean age, 7.6 ± 5 years; male:female, 2.6:1) diagnosed with various metabolic and genetic disorders during 2005-2012. The routine MRI sequences (T1 weighted, T2 weighted and fluid-attenuated inversion-recovery sequences) were analysed for the presence of bilateral symmetrical olivary hypertrophy and central tegmental tract or dentate nuclei signal changes. The other imaging findings and the final diagnoses were noted. The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3). Bilateral inferior olivary nucleus hypertrophy was noted in 10 patients, all of whom belonged to the Leigh and Leigh-like syndrome group. Bilateral hypertrophic olivary degeneration on MRI is relatively often, but not routinely, seen in children with Leigh and Leigh-like syndrome. Early detection of this finding by radiologists and physicians may facilitate targeted metabolic testing in these children. This article highlights the occurrence of bilateral hypertrophic olivary nucleus degeneration on MRI in children with Leigh and Leigh-like syndrome, compared with other metabolic disorders.

  2. The flavonoid herbacetin diglucoside as a constituent of the lignan macromolecule from flaxseed hulls

    NARCIS (Netherlands)

    Struijs, K.; Vincken, J.P.; Verhoef, R.P.; Oostveen, van W.H.M.; Voragen, A.G.J.; Gruppen, H.

    2007-01-01

    Lignans in flaxseed are known to be part of a macromolecule in which they are connected through the linker-molecule hydroxy-methyl-glutaric acid (HMGA). In this study, the lignan macromolecule was extracted from flaxseed hulls and degraded to its monomeric constituents by complete saponification.

  3. Mechanisms underlying metabolic and neural defects in zebrafish and human multiple acyl-CoA dehydrogenase deficiency (MADD.

    Directory of Open Access Journals (Sweden)

    Yuanquan Song

    2009-12-01

    Full Text Available In humans, mutations in electron transfer flavoprotein (ETF or electron transfer flavoprotein dehydrogenase (ETFDH lead to MADD/glutaric aciduria type II, an autosomal recessively inherited disorder characterized by a broad spectrum of devastating neurological, systemic and metabolic symptoms. We show that a zebrafish mutant in ETFDH, xavier, and fibroblast cells from MADD patients demonstrate similar mitochondrial and metabolic abnormalities, including reduced oxidative phosphorylation, increased aerobic glycolysis, and upregulation of the PPARG-ERK pathway. This metabolic dysfunction is associated with aberrant neural proliferation in xav, in addition to other neural phenotypes and paralysis. Strikingly, a PPARG antagonist attenuates aberrant neural proliferation and alleviates paralysis in xav, while PPARG agonists increase neural proliferation in wild type embryos. These results show that mitochondrial dysfunction, leading to an increase in aerobic glycolysis, affects neurogenesis through the PPARG-ERK pathway, a potential target for therapeutic intervention.

  4. Electrospun single-walled carbon nanotube/polyvinyl alcohol composite nanofibers: structure-property relationships

    International Nuclear Information System (INIS)

    Naebe, Minoo; Lin Tong; Wang Xungai; Staiger, Mark P; Dai Liming

    2008-01-01

    Polyvinyl alcohol (PVA) nanofibers and single-walled carbon nanotube (SWNT)/PVA composite nanofibers have been produced by electrospinning. An apparent increase in the PVA crystallinity with a concomitant change in its main crystalline phase and a reduction in the crystalline domain size were observed in the SWNT/PVA composite nanofibers, indicating the occurrence of a SWNT-induced nucleation crystallization of the PVA phase. Both the pure PVA and SWNT/PVA composite nanofibers were subjected to the following post-electrospinning treatments: (i) soaking in methanol to increase the PVA crystallinity, and (ii) cross-linking with glutaric dialdehyde to control the PVA morphology. Effects of the PVA morphology on the tensile properties of the resultant electrospun nanofibers were examined. Dynamic mechanical thermal analyses of both pure PVA and SWNT/PVA composite electrospun nanofibers indicated that SWNT-polymer interaction facilitated the formation of crystalline domains, which can be further enhanced by soaking the nanofiber in methanol and/or cross-linking the polymer with glutaric dialdehyde

  5. Ethanol production with a flocculating mutant of Zymomonas mobilis and immobilized glycoside hydrolases. Ethanolgewinnung mit einer flockenden Mutante von Zymomonas mobilis und immobilisierten Glycosidhydrolasen

    Energy Technology Data Exchange (ETDEWEB)

    Tramm-Werner, S.

    1987-05-25

    A method to extend the substrate spectrum of Z. mobilis was developed. Higher ethanol yields were achieved by simultaneous use of hydrolases cross-linked with glutar aldehyde together with the flocculating Zymomonas cells (TW 602). Apart from the high product yields, the method is characterized by low susceptibility to infections.

  6. Amnioinfusion for relief of recurrent severe and moderate variable decelerations in labor.

    Science.gov (United States)

    Regi, Annie; Alexander, Nancy; Jose, Ruby; Lionel, Jessie; Varghese, Lilly; Peedicayil, Abraham

    2009-05-01

    To determine whether intrapartum amnioinfusion (AI) relieves recurrent moderate and severe variable decelerations in laboring women with clear or grade I meconium-stained amniotic fluid and reduces cesarean section rate for fetal distress. A randomized controlled trial was conducted in labor unit of Christian Medical College Hospital, Vellore, India, between October 2003 and September 2004. Women were randomized to receive AI (group I) and not to receive it (group II). A total of 150 women (75 in each group) were included in the study. There was significant relief of variable decelerations in group I and no difference in overall cesarean section rate but significant reduction in cesarean section rate for fetal distress in group I, and significant reduction in cesarean section rate for fetal distress in nulliparous women of group I. Neonatal acidemia was also significantly reduced in the nulliparous women receiving AI. The duration of maternal postpartum hospital stay was significantly reduced in group I. There were no adverse maternal or neonatal outcomes. AI was a beneficial therapeutic intervention in women patients showing fetal distress in first stage of labor, and it reduced cesarean section for fetal distress and neonatal acidemia.

  7. Dietary practices in isovaleric acidemia: A European survey

    Directory of Open Access Journals (Sweden)

    A. Pinto

    2017-09-01

    Conclusions: This survey demonstrates wide differences in dietary practice in the management of IVA across European centres. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability.

  8. Dietary practices in propionic acidemia: A European survey

    Directory of Open Access Journals (Sweden)

    A. Daly

    2017-12-01

    Conclusions: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007 safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

  9. A Methylmalonic Acidemia Case Presenting with Acrodermatitis Enteropathica

    Directory of Open Access Journals (Sweden)

    hesaneh izadyar

    2014-08-01

    Full Text Available We encountered a patient with methylmalonic aciduria associated with skin lesions resembling acrodermatitis enteropathica. This child was being fed with a low-protein diet when the skin disorder developed. A deficiency in plasma levels isoleucine, was confirmed. Supplementation of a high-caloric, protein-rich diet led to a prompt improvement of skin lesions. We assume that in our patient the skin lesions were the result of malnutrition, rather than being primarily associated with the underlying metabolic disease. To our knowledge, few reports are so far available concerning methylmalonic aciduria complicated by skin eruptions.

  10. The davDT operon of Pseudomonas putida, involved in lysine catabolism, is induced in response to the pathway intermediate delta-aminovaleric acid

    DEFF Research Database (Denmark)

    Revelles, O.; Espinosa-Urgel, M.; Molin, Søren

    2004-01-01

    -aminovaleric acid and then further degraded to glutaric acid via the action of the davDT gene products. We show that the davDT genes form an operon transcribed from a single sigma(70)-dependent promoter. The relatively high level of basal expression from the davD promoter increased about fourfold in response...

  11. [Diagnosis of neonatal metabolic acidosis by eucapnic pH determination].

    Science.gov (United States)

    Racinet, C; Richalet, G; Corne, C; Faure, P; Peresse, J-F; Leverve, X

    2013-09-01

    The identification of a metabolic acidosis is a key criterion for establishing a causal relationship between fetal perpartum asphyxia and neonatal encephalopathy and/or cerebral palsy. The diagnostic criteria currently used (pH and base deficit or lactatemia) are imprecise and non-specific. The study aimed to determine among a low-risk cohort of infants born at term (n = 867), the best diagnostic tool of metabolic acidosis in the cordonal from the following parameters: pH, blood gases and lactate values at birth. The data were obtained from arterial blood of the umbilical cord by a blood gas analyser. The parameter best predicting metabolic analysis was estimated from the partial correlations established between the most relevant parameters. The results showed a slight change in all parameters compared to adult values: acidemia (pH: 7.28 ± 0.01), hypercapnia (56.5 ± 1.59 mmHg) and hyperlactatemia (3.4 ± 0.05 mmol/L). From partial correlation analysis, pCO(2) emerged to be the main contributor of acidemia, while lactatemia was shown to be non-specific for metabolic acidosis. Seven cases (0.81 %) showed a pH less than 7.00 with marked hypercapnia. The correction of this respiratory component by EISENBERG's method led to the eucapnic pH, classifying six out of seven cases as exclusive respiratory acidosis. It has been demonstrated that the criteria from ACOG-AAP for defining a metabolic acidosis are incomplete, imprecise and generating errors in excess. The same is true for lactatemia, whose physiological significance has been completely revised, challenging the misconception of lactic acidosis as a specific marker of hypoxia. It appeared that eucapnic pH was the best way for obtaining a reliable diagnosis of metabolic acidosis. We proposed to adopt a simple decision scheme for determining whether a metabolic acidosis has occurred in case of acidemia less than 7.00. Copyright © 2013. Published by Elsevier SAS.

  12. Molecular characterisation of a recombinant bovine glycine N-acyltransferase / Christoffel Petrus Stephanus Badenhorst

    OpenAIRE

    Badenhorst, Christoffel Petrus Stephanus

    2010-01-01

    Conjugation of glycine to organic acids is an important detoxification mechanism. Metabolites of aspirin and industrial solvents, benzoic acid found in plant material and many endogenous metabolites are detoxified by conjugation to glycine. The enzyme responsible for glycine conjugation, glycine N-acyltransferase (GL YAT), is investigated in this study. The enzyme is also important for the management of organic acidemias which are inherited metabolic diseases. However, not all ...

  13. Walter Reed Army Medical Center, Washington, D. C. - Annual Progress Report FY-89. Volume 1

    Science.gov (United States)

    1990-01-02

    Enemas Cause Hyperphosphatemia and Acidemia in Patients with Ulcerative Colitis After Routine5 Colonic Cleansing." 4. LCDR Michael S. Opsahl, MC...Cholestyramine Treatment of 101 Thyrotoxicosis (3/88) 1303-88 Burman, Kenneth COL MC. The Clinical Application of In Situ 102 Hybridization to Detect Viral...Absorption of Commercial 144 (Oral) Calcium Carbonate Supplements (10/87) 1398-87 Solomon, Barbara DNSc. The Management of Hyperthyroidism Due 145 to

  14. Basicity and ionization constants of some molybdenum(6) complex acids

    International Nuclear Information System (INIS)

    Fedorov, A.A.

    1975-01-01

    Coordination of the Mo(6) ions of the malic, trioxyglutaric, glucaric and mucic acids changes their acidity. Complex Mo(6) acids are stronger then corresponding oxyacids. It was demonstrated that complexes of Mo(6) with malic, trioxyglutaric, glutaric, mucic acids and mannitol were behaving as polybasic acids during titration. Sequential ionization constants of the complex acids were determined

  15. Acidose metabólica na infância: por que, quando e como tratá-la? Metabolic acidosis in childhood: why, when and how to treat

    Directory of Open Access Journals (Sweden)

    Olberes V. B. Andrade

    2007-05-01

    Full Text Available OBJETIVO: Apresentar uma revisão atualizada e crítica sobre os mecanismos das principais patologias associadas e o tratamento da acidose metabólica, discutindo aspectos controversos quanto aos benefícios e riscos da utilização do bicarbonato de sódio e outras formas de terapia. FONTES DOS DADOS: Revisão da literatura publicada, obtida através de busca eletrônica com as palavras-chave acidose metabólica, acidose láctica, cetoacidose diabética, ressuscitação cardiopulmonar, bicarbonato de sódio e terapêutica nas bases de dados PubMed/MEDLINE, LILACS e Cochrane Library, entre 1996 e 2006, além de publicações clássicas referentes ao tema, sendo selecionadas as mais atuais e representativas, buscando-se consensos e diretrizes. SÍNTESE DOS DADOS: A utilização de bicarbonato de sódio não demonstra benefícios no quadro hemodinâmico, evolução clínica, morbidade e mortalidade nos quadros de acidose metabólica de anion gap elevado, relacionados à acidose láctica, cetoacidose diabética e ressuscitação cardiorrespiratória. Assim, a sua utilização rotineira não é indicada. Devem ser considerados os potenciais efeitos colaterais. O tratamento da doença de base é fundamental para reversão do processo. Outras terapias alternativas não demonstram efetividade comprovada em grande escala. CONCLUSÕES: Apesar dos efeitos conhecidos da acidemia em situações críticas no organismo, discute-se o papel protetor da acidemia nas células sob hipoxemia e os riscos da alcalemia secundária à intervenção medicamentosa. Existe consenso na reposição de álcalis e bicarbonato de sódio nos casos de acidose de anion gap normal; entretanto, nos casos de acidose de anion gap elevado, particularmente na acidose láctica, cetoacidose diabética e na ressuscitação cardiorrespiratória, o uso de bicarbonato de sódio não demonstra benefícios, além dos potenciais efeitos adversos, o que torna restrita sua indicação. Apesar da

  16. Materials for the 21st Century. Can the carbon come from CO{sub 2}

    Energy Technology Data Exchange (ETDEWEB)

    Kissling, S.; Altenbuchner, P.T.; Rieger, B. [Technische Univ. Muenchen (Germany). WACKER Lehrstuhl fuer Makromolekulare Chemie

    2012-07-01

    The major amount of synthetic polymers depend on petroleum feedstock. The topic of biodegradable plastics has drawn much attention since the discovery of poly(propylene carbonate) by Inoue, over 40 years ago. Coupling of carbon dioxide with epoxides leads to polycarbonates, which are temperature stable, biodegradable, firm, elastic and highly transparent. These properties open up a wide range of applications. The most preferable epoxide in the copolymerization is propylene oxide. Up to date, there are three different catalyst systems (zinc glutarates, zinc phenoxides and {beta}-diketiminato-zinc-complexes) containing zinc as a metal center. Due to the fact that heterogeneous zinc glutarates show low activities, our work focuses on homogenous catalyst systems. Herein it is a big challenge to avoid the formation of byproducts, namely the back-biting reaction and the consecutive insertion of epoxides. Therefore a catalyst must be designed to suppress these reactions. Furthermore, most of the zinc based catalysts are only active for the copolymerization of CO{sub 2} and cyclohexene oxide and not propylene oxide. The presentation will give a detailed insight into new catalyst structures and especially mechanistic aspects concerned with electronical and sterical influences of the polymerization catalyst. (orig.)

  17. L-arabinose metabolism in Herbaspirillum seropedicae.

    Science.gov (United States)

    Mathias, A L; Rigo, L U; Funayama, S; Pedrosa, F O

    1989-01-01

    The pathway for L-arabinose metabolism in Herbaspirillum seropedicae was shown to involve nonphosphorylated intermediates and to produce alpha-ketoglutarate. The activities of the enzymes and the natures of several intermediates were determined. The pathway was inducible by L-arabinose, and two key enzymes, L-arabinose dehydrogenase and 2-keto-glutarate semialdehyde dehydrogenase, were present in all strains of H. seropedicae tested. PMID:2768202

  18. Two-dimensional structure of poly[[[μ2-1,4-bis(pyridin-4-ylbutane]bis(μ4-pentanedioatodicopper(II] acetonitrile disolvate

    Directory of Open Access Journals (Sweden)

    Do Nam Lee

    2017-10-01

    Full Text Available In the title compound, {[Cu2(μ4-C5H6O42(μ2-C14H16N2]·2CH3CN}n, the Cu2 dinuclear units are connected by glutartate ligands, forming one-dimensional double chains. These chains, are in turn bridged by 1,4-bis(pyridin-4-ylbutane ligands to form a two-dimensional layer structure parallel to (112. The carboxylate groups of the glutarate ligand bridge two copper(II ions, forming a paddle-wheel-type Cu2(CO24 dinuclear secondary building unit. A crystallographic inversion centre is located midway between two CuII ions, with a Cu...Cu distance of 2.639 (3 Å. The coordination geometry of the unique CuII ion is slightly disorted square pyramidal, formed by four equatorial carboxylate O atoms and an axial pyridyl N atom.

  19. Nationwide survey of rotavirus-associated encephalopathy and sudden unexpected death in Japan.

    Science.gov (United States)

    Kawamura, Yoshiki; Ohashi, Masahiro; Ihira, Masaru; Hashimoto, Shuji; Taniguchi, Koki; Yoshikawa, Tetsushi

    2014-08-01

    Rotavirus can cause severe complications such as encephalopathy/encephalitis and sudden unexpected death. The incidence of rotavirus-associated encephalopathy/encephalitis or sudden unexpected death remains unknown. To clarify the clinical features of rotavirus-associated encephalitis/encephalopathy and sudden unexpected death, we conducted a nationwide survey in Japan. A two-part questionnaire was designed to determine the number of the cases and the clinical features of severe cases of rotavirus infection, including encephalitis/encephalopathy and sudden unexpected death, between 2009 and 2011. Of the 1365 questionnaires sent to hospitals, 963 (70.5%) were returned and eligible for analysis. We determined 58 cases of rotavirus-associated encephalitis/encephalopathy and 7 cases of sudden unexpected death. These patients were diagnosed with rotavirus infection by immunochromatography. Although 36/58 (62.1%) encephalitis/encephalopathy patients had no sequelae, 15/58 (25.9%) patients had neurological sequelae, and 7/58 (12.1%) patients had fatal outcomes. Pleocytosis was observed in 9/40 (22.5%) patients and cerebrospinal fluid protein levels were elevated in only 4/40 (10%) patients. Elevated lactate dehydrogenase (LDH) (>500 IU/L) or acidemia (pHdeath were 44.0 and 4.9 cases in Japan, respectively. Elevated LDH (>500 IU/L) or acidemia (pH<7.15) were related to a poor prognosis of the encephalitis/encephalopathy. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  20. Spectrum analysis of common inherited metabolic diseases in Chinese patients screened and diagnosed by tandem mass spectrometry.

    Science.gov (United States)

    Han, Lianshu; Han, Feng; Ye, Jun; Qiu, Wenjuan; Zhang, Huiwen; Gao, Xiaolan; Wang, Yu; Ji, Wenjun; Gu, Xuefan

    2015-03-01

    Information concerning inherited metabolic diseases in China is scarce. We investigated the prevalence and age distributions of amino acid, organic acid, and fatty acid oxidation disorders in Chinese patients. Blood levels of amino acids and acylcarnitines (tandem mass spectrometry) were measured in 18,303 patients with suspected inherited metabolic diseases. Diagnosis was based on clinical features, blood levels of amino acids or acylcarnitines, urinary organic acid levels (gas chromatography-mass spectrometry), and (in some) gene mutation tests. Inherited metabolic diseases were confirmed in 1,135 patients (739 males, 396 females). Median age was 12 months (1 day to 59 years). There were 28 diseases: 12 amino acid disorders (580 patients, 51.1%), with hyperphenylalaninemia (HPA) being the most common; nine organic acidemias (408 patients, 35.9%), with methylmalonic acidemia (MMA) as the most common; and seven fatty acid oxidation defects (147 patients, 13.0%), with multiple acyl-coenzyme A dehydrogenase deficiency (MADD) being the most common. Onset was mainly at 1-6 months for citrin deficiency, 0-6 months for MMA, and in newborns for ornithine transcarbamylase deficiency (OTCD). HPA was common in patients aged 1-3 years, and MADD was common in patients >18 years. In China, HPA, citrin deficiency, MMA, and MADD are the most common inherited disorders, particularly in newborns/infants. © 2014 Wiley Periodicals, Inc.

  1. Neuroimaging of nonaccidental head trauma: pitfalls and controversies

    Energy Technology Data Exchange (ETDEWEB)

    Fernando, Sujan [University of Missouri-Kansas School of Medicine, Department of Medicine, Kansas City, MO (United States); Obaldo, Ruby E. [The University of Kansas Medical Center, Department of Radiology, Kansas City, MO (United States); Walsh, Irene R. [The University of Missouri-Kansas City, Children' s Mercy Hospitals and Clinics, Department of Emergency Medicine, Kansas City, MO (United States); Lowe, Lisa H. [The University of Missouri-Kansas City, Children' s Mercy Hospitals and Clinics, Department of Radiology, Kansas City, MO (United States)

    2008-08-15

    Although certain neuroimaging appearances are highly suggestive of abuse, radiological findings are often nonspecific. The objective of this review is to discuss pitfalls, controversies, and mimics occurring in neuroimaging of nonaccidental head trauma in order to allow the reader to establish an increased level of comfort in distinguishing between nonaccidental and accidental head trauma. Specific topics discussed include risk factors, general biomechanics and imaging strategies in nonaccidental head trauma, followed by the characteristics of skull fractures, normal prominent tentorium and falx versus subdural hematoma, birth trauma versus nonaccidental head trauma, hyperacute versus acute on chronic subdural hematomas, expanded subarachnoid space versus subdural hemorrhage, controversy regarding subdural hematomas associated with benign enlarged subarachnoid spaces, controversy regarding hypoxia as a cause of subdural hematoma and/or retinal hemorrhages without trauma, controversy regarding the significance of retinal hemorrhages related to nonaccidental head trauma, controversy regarding the significance of subdural hematomas in general, and pitfalls of glutaric aciduria type 1 and hemophagocytic lymphohistiocytosis mimicking nonaccidental head trauma. (orig.)

  2. Late-onset form of beta-electron transfer flavoprotein deficiency

    DEFF Research Database (Denmark)

    Curcoy, A; Olsen, Rikke Katrine Jentoft; Ribes, A

    2003-01-01

    Multiple acyl-CoA-dehydrogenase deficiency (MADD) or glutaric aciduria type II (GAII) are a group of metabolic disorders due to deficiency of either electron transfer flavoprotein (ETF) or electron transfer flavoprotein ubiquinone oxidoreductase (ETF-QO). We report the clinical features...... and biochemical and molecular genetic analyses of a patient with a mild late-onset form of GAII due to beta-ETF deficiency. Biochemical data showed an abnormal urine organic acid profile, low levels of free carnitine, increased levels of C(10:1n-6), and C(14:1n-9) in plasma, and decreased oxidation of [9,10-3H......]palmitate and [9,10-3H]myristate in fibroblasts, suggesting MAD deficiency. In agreement with these findings, mutational analysis of the ETF/ETFDH genes demonstrated an ETFB missense mutation 124T>C in exon 2 leading to replacement of cysteine-42 with arginine (C42R), and a 604_606AAG deletion in exon 6...

  3. Neuroimaging of nonaccidental head trauma: pitfalls and controversies

    International Nuclear Information System (INIS)

    Fernando, Sujan; Obaldo, Ruby E.; Walsh, Irene R.; Lowe, Lisa H.

    2008-01-01

    Although certain neuroimaging appearances are highly suggestive of abuse, radiological findings are often nonspecific. The objective of this review is to discuss pitfalls, controversies, and mimics occurring in neuroimaging of nonaccidental head trauma in order to allow the reader to establish an increased level of comfort in distinguishing between nonaccidental and accidental head trauma. Specific topics discussed include risk factors, general biomechanics and imaging strategies in nonaccidental head trauma, followed by the characteristics of skull fractures, normal prominent tentorium and falx versus subdural hematoma, birth trauma versus nonaccidental head trauma, hyperacute versus acute on chronic subdural hematomas, expanded subarachnoid space versus subdural hemorrhage, controversy regarding subdural hematomas associated with benign enlarged subarachnoid spaces, controversy regarding hypoxia as a cause of subdural hematoma and/or retinal hemorrhages without trauma, controversy regarding the significance of retinal hemorrhages related to nonaccidental head trauma, controversy regarding the significance of subdural hematomas in general, and pitfalls of glutaric aciduria type 1 and hemophagocytic lymphohistiocytosis mimicking nonaccidental head trauma. (orig.)

  4. Clinical neurogenetics: neurologic presentations of metabolic disorders.

    Science.gov (United States)

    Kwon, Jennifer M; D'Aco, Kristin E

    2013-11-01

    This article reviews aspects of the neurologic presentations of selected treatable inborn errors of metabolism within the category of small molecule disorders caused by defects in pathways of intermediary metabolism. Disorders that are particularly likely to be seen by neurologists include those associated with defects in amino acid metabolism (organic acidemias, aminoacidopathies, urea cycle defects). Other disorders of small molecule metabolism are discussed as additional examples in which early treatments have the potential for better outcomes. Copyright © 2013 Elsevier Inc. All rights reserved.

  5. Anesthetic Management of A Parturient With Diabetic Keto-Acidosis

    OpenAIRE

    Rupasinghe M N; Hemmad A R

    2013-01-01

    Diabetic ketoacidosis (DKA) in pregnancy is a life threatening medical emergency. It can compromise both the fetus and the mother profoundly. The incidence of DKA during pregnancy ranges between 2 to 3%, and carries a 10–20% risk of fetal death.1 DKA is characterized by a biochemical triad of ketonemia, hyperglycemia and acidemia. We present a case of a parturient with mismanaged DKA that was brought to the operating room for a STAT cesarean section (C/S) due to fetal distress and discuss the...

  6. Effect of atmospheric organic complexation on iron-bearing dust solubility

    OpenAIRE

    Paris , R.; Desboeufs , K. V.

    2013-01-01

    International audience; Recent studies reported that the effect of organic complexation may be a potentially important process to be considered by models estimating atmospheric iron flux to the ocean. In this study, we investigated this process effect by a series of dissolution experiments on iron-bearing dust in the presence or the absence of various organic compounds (acetate, formate, oxalate, malonate, succinate, glutarate, glycolate, lactate, tartrate and humic acid as an analogue of hum...

  7. Propionic acidemia as a cause of adult-onset dilated cardiomyopathy

    NARCIS (Netherlands)

    Riemersma, M.; Hazebroek, M.R.; Helderman-van den Enden, A.T.; Salomons, G.S.; Ferdinandusse, S.; Brouwers, M.; Ploeg, L. van der; Heymans, S.; Glatz, J.F.C.; Wijngaard, A. van den; Krapels, I.P.C.; Bierau, J.; Brunner, H.G.

    2017-01-01

    Dilated cardiomyopathy (DCM) is extremely heterogeneous with a large proportion due to dominantly inherited disease-causing variants in sarcomeric genes. Recessive metabolic diseases may cause DCM, usually with onset in childhood, and in the context of systemic disease. Whether metabolic defects can

  8. Capnography in the Emergency Department: A Review of Uses, Waveforms, and Limitations.

    Science.gov (United States)

    Long, Brit; Koyfman, Alex; Vivirito, Michael A

    2017-12-01

    Capnography has many uses in the emergency department (ED) and critical care setting, most commonly cardiac arrest and procedural sedation. This review evaluates several indications concerning capnography beyond cardiac arrest and procedural sedation in the ED, as well as limitations and specific waveforms. Capnography includes the noninvasive measurement of CO 2 , providing information on ventilation, perfusion, and metabolism in intubated and spontaneously breathing patients. Since the 1990s, capnography has been utilized extensively for cardiac arrest and procedural sedation. Qualitative capnography includes a colorimetric device, changing color on the amount of CO 2 present. Quantitative capnography provides a numeric value (end-tidal CO 2 ), and capnography most commonly includes a waveform as a function of time. Conditions in which capnography is informative include cardiac arrest, procedural sedation, mechanically ventilated patients, and patients with metabolic acidemia. Patients with seizure, trauma, and respiratory conditions, such as pulmonary embolism and obstructive airway disease, can benefit from capnography, but further study is needed. Limitations include use of capnography in conditions with mixed pathophysiology, patients with low tidal volumes, and equipment malfunction. Capnography should be used in conjunction with clinical assessment. Capnography demonstrates benefit in cardiac arrest, procedural sedation, mechanically ventilated patients, and patients with metabolic acidemia. Further study is required in patients with seizure, trauma, and respiratory conditions. It should only be used in conjunction with other patient factors and clinical assessment. Published by Elsevier Inc.

  9. Biochemical signatures mimicking multiple carboxylase deficiency in children with mutations in MT-ATP6.

    Science.gov (United States)

    Larson, Austin A; Balasubramaniam, Shanti; Christodoulou, John; Burrage, Lindsay C; Marom, Ronit; Graham, Brett H; Diaz, George A; Glamuzina, Emma; Hauser, Natalie; Heese, Bryce; Horvath, Gabriella; Mattman, Andre; van Karnebeek, Clara; Lane Rutledge, S; Williamson, Amy; Estrella, Lissette; Van Hove, Johan K L; Weisfeld-Adams, James D

    2018-01-04

    Elevations of specific acylcarnitines in blood reflect carboxylase deficiencies, and have utility in newborn screening for life-threatening organic acidemias and other inherited metabolic diseases. In this report, we describe a newly-identified association of biochemical features of multiple carboxylase deficiency in individuals harboring mitochondrial DNA (mtDNA) mutations in MT-ATP6 and in whom organic acidemias and multiple carboxylase deficiencies were excluded. Using retrospective chart review, we identified eleven individuals with abnormally elevated propionylcarnitine (C3) or hydroxyisovalerylcarnitine (C5OH) with mutations in MT-ATP6, most commonly m.8993T>G in high heteroplasmy or homoplasmy. Most patients were ascertained on newborn screening; most had normal enzymatic or molecular genetic testing to exclude biotinidase and holocarboxylase synthetase deficiencies. MT-ATP6 is associated with some cases of Leigh disease; clinical outcomes in our cohort ranged from death from neurodegenerative disease in early childhood to clinically and developmentally normal after several years of follow-up. These cases expand the biochemical phenotype associated with MT-ATP6 mutations, especially m.8993T>G, to include acylcarnitine abnormalities mimicking carboxylase deficiency states. Clinicians should be aware of this association and its implications for newborn screening, and consider mtDNA sequencing in patients exhibiting similar acylcarnitine abnormalities that are biotin-unresponsive and in whom other enzymatic deficiencies have been excluded. Copyright © 2018 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

  10. Diagnosis of glutaric aciduria type 1 by measuring 3-hydroxyglutaric acid in dried urine spots by liquid chromatography tandem mass spectrometry

    DEFF Research Database (Denmark)

    Al-Dirbashi, Osama Y; Kölker, Stefan; Ng, Dione

    2011-01-01

    completing a parallel comparative study with the standard screening method (i.e., molecular testing). In addition, follow-up DUS GA and 3HGA testing of babies with elevated dried blood spot C5DC acylcarnitines will be useful as a first-tier diagnostic test, thus reducing the number of cases requiring...

  11. Research into esterification of mixture of lower dicarboxylic acids by 2-ethylhexan-1-ol in the presence of p-toluensulfonic acid

    OpenAIRE

    Melnyk, Stepan; Melnyk, Yuriy; Nykulyshyn, Irena; Shevchuk, Liliya

    2017-01-01

    Regularities of esterification of the mixture of lower dicarboxylic acids (succinic, glutaric, adipic) by 2-ethylhexan-1-ol in the presence of catalysts – p-toluensulfonic and sulfuric acids under non-stationary conditions were studied. It was found that in the presence of mineral acid, the reaction flows at a lower rate. Application of benzene as a substance that facilitates separation of water, formed in the esterification reaction, makes it possible, due to a lower reaction temperature, to...

  12. Intrapartum amnioinfusion for meconium-stained fluid: meta-analysis of prospective clinical trials.

    Science.gov (United States)

    Pierce, J; Gaudier, F L; Sanchez-Ramos, L

    2000-06-01

    To evaluate the effectiveness of intrapartum prophylactic amnioinfusion in pregnancies complicated by meconium-stained amniotic fluid. We identified prospective clinical trials of amnioinfusion in pregnancies complicated by meconium-stained amniotic fluid (AF) published in English by using computerized databases, references in published studies, and index reviews. We analyzed prospective studies of intrapartum amnioinfusion for meconium-stained AF. In every case, group allocation was based exclusively on meconium in AF. Only published studies with clearly documented outcome data were included. The trials were evaluated for meconium below the vocal cords, meconium aspiration syndrome, fetal acidemia, cesarean delivery, and postpartum endometritis. Each trial was evaluated for the quality of its methodology, inclusion and exclusion criteria, adequacy of randomization, amnioinfusion protocols, definition of outcomes, and statistical analyses. Thirteen studies met inclusion criteria for our systematic review. Odds ratios (ORs) with their 95% confidence intervals (CIs) were calculated for each outcome. Estimates of ORs and risk differences for dichotomous outcomes were calculated using random and fixed-effects models. We tested for homogeneity across the studies. We found that intrapartum amnioinfusion significantly reduced the frequency of meconium aspiration syndrome (OR 0.30; 95% CI 0.19, 0. 46), of meconium below the vocal cords, and neonatal acidemia. Subjects allocated to receive amnioinfusion also had a significantly lower overall cesarean rate (OR 0.74, 95% CI 0.59, 0.93) without increased postpartum endometritis. Amnioinfusion in cases of meconium-stained fluid significantly improves neonatal outcome, lowers the cesarean delivery rate, and does not increase the postpartum endometritis rate.

  13. Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.

    Science.gov (United States)

    Saad, Laura O; Mirandola, Sandra R; Maciel, Evelise N; Castilho, Roger F

    2006-04-01

    Methylmalonic acidemia (MMAemia) is an inherited metabolic disorder of branched amino acid and odd-chain fatty acid metabolism, involving a defect in the conversion of methylmalonyl-coenzyme A to succinyl-coenzyme A. Systemic and neurological manifestations in this disease are thought to be associated with the accumulation of methylmalonate (MMA) in tissues and biological fluids with consequent impairment of energy metabolism and oxidative stress. In the present work we studied the effect of MMA and two other inhibitors of mitochondrial respiratory chain complex II (malonate and 3-nitropropionate) on the activity of lactate dehydrogenase (LDH) in tissue homogenates from adult rats. MMA potently inhibited LDH-catalyzed conversion of lactate to pyruvate in liver and brain homogenates as well as in a purified bovine heart LDH preparation. LDH was about one order of magnitude less sensitive to inhibition by MMA when catalyzing the conversion of pyruvate to lactate. Kinetic studies on the inhibition of brain LDH indicated that MMA inhibits this enzyme competitively with lactate as a substrate (K (i)=3.02+/-0.59 mM). Malonate and 3-nitropropionate also strongly inhibited LDH-catalyzed conversion of lactate to pyruvate in brain homogenates, while no inhibition was observed by succinate or propionate, when present in concentrations of up to 25 mM. We propose that inhibition of the lactate/pyruvate conversion by MMA contributes to lactate accumulation in blood, metabolic acidemia and inhibition of gluconeogenesis observed in patients with MMAemia. Moreover, the inhibition of LDH in the central nervous system may also impair the lactate shuttle between astrocytes and neurons, compromising neuronal energy metabolism.

  14. Refinement Types for TypeScript

    OpenAIRE

    Vekris, Panagiotis; Cosman, Benjamin; Jhala, Ranjit

    2016-01-01

    We present Refined TypeScript (RSC), a lightweight refinement type system for TypeScript, that enables static verification of higher-order, imperative programs. We develop a formal core of RSC that delineates the interaction between refinement types and mutability. Next, we extend the core to account for the imperative and dynamic features of TypeScript. Finally, we evaluate RSC on a set of real world benchmarks, including parts of the Octane benchmarks, D3, Transducers, and the TypeScript co...

  15. The structure and binding mode of citrate in the stabilization of gold nanoparticles

    KAUST Repository

    Al-Johani, Hind; Abou-Hamad, Edy; Jedidi, Abdesslem; Widdifield, Cory M.; Viger-Gravel, Jasmine; Sangaru, Shiv; Gajan, David; Anjum, Dalaver H.; Ould-Chikh, Samy; Hedhili, Mohamed N.; Gurinov, Andrei; Kelly, Michael J.; El Eter, Mohamad; Cavallo, Luigi; Basset, Jean-Marie; Basset, Jean-Marie

    2017-01-01

    Elucidating the binding mode of carboxylate-containing ligands to gold nanoparticles (AuNPs) is crucial to understand their stabilizing role. A detailed picture of the three-dimensional structure and coordination modes of citrate, acetate, succinate and glutarate to AuNPs is obtained by 13C and 23Na solid-state NMR in combination with computational modelling and electron microscopy. The binding between the carboxylates and the AuNP surface is found to occur in three different modes. These three modes are simultaneously present at low citrate to gold ratios, while a monocarboxylate monodentate (1κO1) mode is favoured at high citrate:gold ratios. The surface AuNP atoms are found to be predominantly in the zero oxidation state after citrate coordination, although trace amounts of Auδ+ are observed. 23Na NMR experiments show that Na+ ions are present near the gold surface, indicating that carboxylate binding occurs as a 2e− L-type interaction for each oxygen atom involved. This approach has broad potential to probe the binding of a variety of ligands to metal nanoparticles.

  16. The structure and binding mode of citrate in the stabilization of gold nanoparticles

    KAUST Repository

    Al-Johani, Hind

    2017-03-27

    Elucidating the binding mode of carboxylate-containing ligands to gold nanoparticles (AuNPs) is crucial to understand their stabilizing role. A detailed picture of the three-dimensional structure and coordination modes of citrate, acetate, succinate and glutarate to AuNPs is obtained by 13C and 23Na solid-state NMR in combination with computational modelling and electron microscopy. The binding between the carboxylates and the AuNP surface is found to occur in three different modes. These three modes are simultaneously present at low citrate to gold ratios, while a monocarboxylate monodentate (1κO1) mode is favoured at high citrate:gold ratios. The surface AuNP atoms are found to be predominantly in the zero oxidation state after citrate coordination, although trace amounts of Auδ+ are observed. 23Na NMR experiments show that Na+ ions are present near the gold surface, indicating that carboxylate binding occurs as a 2e− L-type interaction for each oxygen atom involved. This approach has broad potential to probe the binding of a variety of ligands to metal nanoparticles.

  17. Novel routes to 1,2,3,4-tetrahydropyrrolo[1,2-a]pyrazines and 5,6,9,10,11,11a-hexahydro-8H-pyrido[1,2-a]pyrrolo[2,1-c]pyrazines.

    Science.gov (United States)

    Katritzky, Alan R; Jain, Ritu; Xu, Yong-Jiang; Steel, Peter J

    2002-11-15

    Condensation reactions of benzotriazole and 2-(pyrrol-1-yl)-1-ethylamine (1) with formaldehyde and glutaric dialdehyde, respectively, afforded intermediates 2 and 6. Subsequent nucleophilic substitutions of the benzotriazole group in 2 and 6 with Grignard reagents, sodium cyanide, and sodium borohydride gave 1,2,3,4-tetrahydropyrrolo[1,2-a]pyrazines 3a-e, 4, 5 and 5,6,9,10,11,11a-hexahydro-8H-pyrido[1,2-a]pyrrolo[2,1-c]pyrazines 7a-c, 8, 9, respectively, in good yields.

  18. Refining types using type guards in TypeScript

    NARCIS (Netherlands)

    de Wolff, Ivo Gabe; Hage, J.

    2017-01-01

    We discuss two adaptations of the implementation of type guards and narrowing in the TypeScript compiler. The first is an improvement on the original syntax-directed implementation, and has now replaced the original one in the TypeScript compiler. It is specifically suited for the scenario in which

  19. Type Classes for Lightweight Substructural Types

    Directory of Open Access Journals (Sweden)

    Edward Gan

    2015-02-01

    Full Text Available Linear and substructural types are powerful tools, but adding them to standard functional programming languages often means introducing extra annotations and typing machinery. We propose a lightweight substructural type system design that recasts the structural rules of weakening and contraction as type classes; we demonstrate this design in a prototype language, Clamp. Clamp supports polymorphic substructural types as well as an expressive system of mutable references. At the same time, it adds little additional overhead to a standard Damas-Hindley-Milner type system enriched with type classes. We have established type safety for the core model and implemented a type checker with type inference in Haskell.

  20. Pulse radiolysis in model studies toward radiation processing

    Energy Technology Data Exchange (ETDEWEB)

    Sonntag, C Von; Bothe, E; Ulanski, P; Deeble, D J [Max-Planck-Institut fuer Strahlenchemie, Muelheim an der Ruhr (Germany)

    1995-10-01

    Using the pulse radiolysis technique, the OH-radical-induced reactions of poly(vinyl alcohol) PVAL, poly(acrylic acid) PAA, poly(methyacrylic acid) PMA, and hyaluronic acid have been investigated in dilute aqueos solution. The reactions of the free-radical intermediates were followed by UV-spectroscopy and low-angle laser light-scattering; the scission of the charged polymers was also monitored by conductometry. For more detailed product studies, model systems such as 2,4-dihydroxypentane (for PVAL) and 2,4-dimethyl glutaric acid (for PAA) was also investigated. (author).

  1. The peculiarity of aerobic energy supply of rat tissues of different age upon prolonged ionizing and thermal exposure

    International Nuclear Information System (INIS)

    Tsyhun, G.F.

    1998-01-01

    Energy-producing functions of brain, myocardium, and hepatic mitochondria in mature and immature rats in remote period after prolonged ionizing X-ray at total dose 12,9 m C/kg and thermal exposure (4 hours, 37 degrees centigrade, 25 times) were studied. Dehydrogenase activities (pyruvate-, isocitrate-, 2-oxy glutarate-, succinate- and malate dehydrogenases) were reduced in mitochondria of different tissues of adult rats and it was especially considerable after combined influence. A higher resistance of young rats, as compared to adult ones, to combined radiation-thermal treatments was established

  2. Type inference for correspondence types

    DEFF Research Database (Denmark)

    Hüttel, Hans; Gordon, Andy; Hansen, Rene Rydhof

    2009-01-01

    We present a correspondence type/effect system for authenticity in a π-calculus with polarized channels, dependent pair types and effect terms and show how one may, given a process P and an a priori type environment E, generate constraints that are formulae in the Alternating Least Fixed......-Point (ALFP) logic. We then show how a reasonable model of the generated constraints yields a type/effect assignment such that P becomes well-typed with respect to E if and only if this is possible. The formulae generated satisfy a finite model property; a system of constraints is satisfiable if and only...... if it has a finite model. As a consequence, we obtain the result that type/effect inference in our system is polynomial-time decidable....

  3. 1H MR spectroscopy of the basal ganglia in childhood: a semiquantitative analysis

    International Nuclear Information System (INIS)

    Lam, W.W.M.; Zhao, H.; Berry, G.T.; Kaplan, P.; Gibson, J.; Kaplan, B.S.

    1998-01-01

    Proton MR spectra of the basal ganglia were obtained from 28 patients, 24 male and 14 female, median age 16.3 months (5 weeks to 31 years). They included 17 patients with normal MRI of the basal ganglia without metabolic disturbance (control group) and 11 patients with various metabolic diseases: one case each of high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease, Galloway-Mowat syndrome, Pelizaeus-Merzbacher disease, hemolytic-uremic syndrome and Wilson disease and two cases of Alagille syndrome and methylmalonic acidemia with abnormal MRI of the basal ganglia or blood or urine analysis (abnormal group). The MR spectrum was measured by using STEAM. The MR-visible water content of the region of interest was obtained. Levels of myoinositol, choline, creatine and N -acetylaspartate were measured using a semiquantitative approach, with absolute reference calibration. In the control group, there was a gradual drop of water content over the first year of life; N -acetylaspartate, creatine and myoinositol levels showed no significant change with age, in contrast to the occipital, parietal and cerebellar regions. Choline showed a gradual decrease for the first 2 years of life and then remained fairly constant. In the abnormal group the water content was not significantly different. N -Acetylaspartate was decreased in patients with high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease and one case of methylmalonic acidemia. Decreased creatine was also found in Leigh disease, and decreased choline in Galloway-Mowat syndrome and Wilson disease. Myoinositol was elevated in the patient with abnormally high serum sodium, and decreased in the hemolytic-uremic syndrome. (orig.)

  4. Guarded dependent type theory with coinductive types

    DEFF Research Database (Denmark)

    Bizjak, Aleš; Grathwohl, Hans Bugge; Clouston, Ranald

    2016-01-01

    We present guarded dependent type theory, gDTT, an extensional dependent type theory with a later' modality and clock quantifiers for programming and proving with guarded recursive and coinductive types. The later modality is used to ensure the productivity of recursive definitions in a modular......, type based, way. Clock quantifiers are used for controlled elimination of the later modality and for encoding coinductive types using guarded recursive types. Key to the development of gDTT are novel type and term formers involving what we call delayed substitutions’. These generalise the applicative...... functor rules for the later modality considered in earlier work, and are crucial for programming and proving with dependent types. We show soundness of the type theory with respect to a denotational model....

  5. Type Families with Class, Type Classes with Family

    DEFF Research Database (Denmark)

    Serrano, Alejandro; Hage, Jurriaan; Bahr, Patrick

    2015-01-01

    Type classes and type families are key ingredients in Haskell programming. Type classes were introduced to deal with ad-hoc polymorphism, although with the introduction of functional dependencies, their use expanded to type-level programming. Type families also allow encoding type-level functions......, now as rewrite rules. This paper looks at the interplay of type classes and type families, and how to deal with shortcomings in both of them. Furthermore, we show how to use families to simulate classes at the type level. However, type families alone are not enough for simulating a central feature...... of type classes: elaboration, that is, generating code from the derivation of a rewriting. We look at ways to solve this problem in current Haskell, and propose an extension to allow elaboration during the rewriting phase....

  6. A study on complex formation of cadmium (II) ions, 9

    International Nuclear Information System (INIS)

    Matsui, Haruo

    1984-01-01

    Formation constants of cadmium (11) complexes with dicarboxylic acids such as oxalic, malonic, methylmalonic, succinic, and glutaric acids were determined in aqueous solutions containing 3 mol.dm -3 LiClO 4 as a constan ionic medium at 25 0 C by potentiometric titrations. It was reported in the previous works that cadmium (11)- aspartic acid complexes contained two chelate rings. However, a problem remained whether the second chelate ring could be formed by six membered-ring containing -O-Cd-N- bond or by seven membered-ring containing -O-Cd-O- bond. The results of the present work suggested that it would be formed by a six membered ring. Cadmium (11) ions were coordinated with a carboxylic group of the dicarboxylic acids studied, and formed no chelate ring within the complexes. The white precipitate appeared in the solution containing cadmium (11) ion and oxalic acid, in the pH range below 3.0, therefore, the chelate formation was not ascertained in this case. The formation constants, log βsub(pr)= log([Cdsub(p)Lsub(r)sup((2p-2r)+)]/([Cd 2+ ]sup(p)[L 2- ]sup(r))), of the complexes were: log β 11 = 1.98, log β 12 = 3.05 for cadmium (11)-malonic acid; log β 11 = 2.28, log β 12 = 3.06 for cadmium (11)-methylmalonic acid; log β 11 = 1.78, log β 12 = 3.08 for cadmium (11)-succinic acid; log β 11 = 1.85, log β 12 = 3.28 for cadmium (11)-glutaric acid complexes. (author)

  7. Concrete Types for TypeScript

    OpenAIRE

    Richards, Gregor; Zappa Nardelli, Francesco; Vitek, Jan

    2015-01-01

    Typescript extends JavaScript with optional type annotations that are, by design, unsound and, that the Typescript compiler discards as it emits code. This design point preserves programming idioms developers are familiar with, and allows them to leave their legacy code unchanged, while offering a measure of static error checking in parts of the program that have type annotations. We present an alternative design for TypeScript, one where it is possible to support the same degree of dynamism,...

  8. Early onset diabetes-genetic and hormonal analysis in pakistan population

    International Nuclear Information System (INIS)

    Wahid, M.; Kamran, M.

    2016-01-01

    Background: Mitochondrial DNA mutation and hormonal imbalance is involved in the pathogenesis of early onset diabetes but data is lacking in Pakistani population. The study was planned to delineate the clinical presentation of early onset diabetes with possible hormonal and genetic etiological factors and aascertain the possible etiological role of insulin and glucagon in these patients either on oral hypoglycaemic or subcutaneous insulin therapy. Methods: Retrospective, analytical case control study with conventional sampling technique carried at Centre for Research in Experimental and Applied Medicine (CREAM) affiliated with the department of Biochemistry and Molecular Biology, Army Medical College Rawalpindi from Dec 2006 to July 2011. Study included the patients (20-35 years of age) with early onset diabetes on oral hypoglycemic (n=240), insulin therapy (n=280), and compared with non-diabetic healthy controls (n=150). A fragment surrounding tRNALeu (UUR) gene was amplified by AmpliTaq from mtDNA which was extracted from peripheral blood leucocytes. Then it was subjected to restriction endonucleases, ApaI for A3242G mutation and HaeIII for G3316A mutation detection. Plasma glucose, glycosylated Hb, osmolality, insulin and glucagon levels along with ABGs analysis was also done. Results: Non diabetic controls comprised of 51% males and 49% females, diabetics on oral hypoglycemic 60% males and 40 % females and on insulin therapy 54% males and 46% females. Insulin dependent diabetics had statistically significant hyperglucagonemia, acidemia and bicarbonate deficit. MtDNA A3242G and G3316A mutations were not detected. Conclusion: relative hyperglucagonemia and acidemia in Insulin dependent diabetics was a potent threat leading to DKA. The absence of two mtDNA mutations in ND1 gene rules out the possibility of involvement of these mutations in early onset diabetes in Pakistani population. (author)

  9. Effects of L-glutamine supplementation on maternal and fetal hemodynamics in gestating ewes exposed to alcohol.

    Science.gov (United States)

    Sawant, Onkar B; Ramadoss, Jayanth; Hankins, Gary D; Wu, Guoyao; Washburn, Shannon E

    2014-08-01

    Not much is known about effects of gestational alcohol exposure on maternal and fetal cardiovascular adaptations. This study determined whether maternal binge alcohol exposure and L-glutamine supplementation could affect maternal-fetal hemodynamics and fetal regional brain blood flow during the brain growth spurt period. Pregnant sheep were randomly assigned to one of four groups: saline control, alcohol (1.75-2.5 g/kg body weight), glutamine (100 mg/kg body weight) or alcohol + glutamine. A chronic weekend binge drinking paradigm between gestational days (GD) 99 and 115 was utilized. Fetuses were surgically instrumented on GD 117 ± 1 and studied on GD 120 ± 1. Binge alcohol exposure caused maternal acidemia, hypercapnea, and hypoxemia. Fetuses were acidemic and hypercapnic, but not hypoxemic. Alcohol exposure increased fetal mean arterial pressure, whereas fetal heart rate was unaltered. Alcohol exposure resulted in ~40 % reduction in maternal uterine artery blood flow. Labeled microsphere analyses showed that alcohol induced >2-fold increases in fetal whole brain blood flow. The elevation in fetal brain blood flow was region-specific, particularly affecting the developing cerebellum, brain stem, and olfactory bulb. Maternal L-glutamine supplementation attenuated alcohol-induced maternal hypercapnea, fetal acidemia and increases in fetal brain blood flow. L-Glutamine supplementation did not affect uterine blood flow. Collectively, alcohol exposure alters maternal and fetal acid-base balance, decreases uterine blood flow, and alters fetal regional brain blood flow. Importantly, L-glutamine supplementation mitigates alcohol-induced acid-base imbalances and alterations in fetal regional brain blood flow. Further studies are warranted to elucidate mechanisms responsible for alcohol-induced programming of maternal uterine artery and fetal circulation adaptations in pregnancy.

  10. Sertoli cell index and spermatic reserves in adult captive African lions (Panthera leo, Linnaeus, 1758).

    Science.gov (United States)

    de Barros, João Bosco Gonçalves; de Paula, Tarcízio Antônio Rego; da Matta, Sérgio Luis Pinto; Fonseca, Cláudio César; Leite, Flaviana Lima Guião; Rossi, João Luiz; de Oliveira, Priscila Carvalho; da Costa, Eduardo Paulino

    2007-12-01

    The intrinsic yield of spermatogenesis and the supporting indexes of the Sertoli cells are the best indicators for the spermatic production capacity in a species. The aim of the present study was to quantify the intrinsic yield of the spermatogenetic process, as well as the Sertoli cell index and spermatic reserves. Testicular fragments of five adult African lions was fixed in 4% glutaric aldehyde, dehydrated at increasing alcohol concentrations, included into hydroxyethyl methacrylate, and were cut into 4 microm thickness. In the seminiferous epithelium of the African lions, 10.3 primary spermatocytes at pre-leptotene phase are produced by the type-A spermatogonia. During meiotic divisions, only 2.7 spermatids were produced from the primary spermatocytes. The general spermatogenesis production in the African lions was approximately 22.1 cells, and each Sertoli cell was able to sustain and maintain approximately 14.9 cells of the germinative line, from which 7.9 are round spermatids. A total of 103x10(6) spermatozoa are produced by each testis gram at each cycle of the seminiferous epithelium. The spermatic reserve of lion is below the amplitude observed in mammals.

  11. Klebsiella Typing

    DEFF Research Database (Denmark)

    Hansen, D S; Skov, R; Benedí, J.V.

    2002-01-01

    OBJECTIVE: To compare pulsed-field gel electrophoresis (PFGE) typing and O:K-serotyping of Klebsiella in two different epidemiological settings. METHODS: One hundred and four bacteremia isolates without known epidemiological relation and 47 isolates from an outbreak in a neonatal intensive care...... unit (NICU) were K-typed by countercurrent immunoelectrophoresis (CCIE), O-typed by an inhibition enzyme-linked immunosorbent assay method, and typed by pulsed-field gel electrophoresis (PFGE) using the restriction enzyme XbaI. RESULTS: Typing data for the 104 bacteremia isolates were compared...... with regard to typability, number of types, maximum number of isolates per type, and the Discriminative Index (DI). O-typing combined with K-typing (DI 0.98) as O:K-serotyping (DI 0.99) gave a very discriminative typing system, whereas O-typing alone was not very discriminative (DI 0.76). PFGE (DI 1...

  12. Typing is writing: Linguistic properties modulate typing execution.

    Science.gov (United States)

    Pinet, Svetlana; Ziegler, Johannes C; Alario, F-Xavier

    2016-12-01

    Typing is becoming our preferred way of writing. Perhaps because of the relative recency of this change, very few studies have investigated typing from a psycholinguistic perspective. In addition, and despite obvious similarities between typing and handwriting, typing research has remained rather disconnected from handwriting research. The current study aimed at bridging this gap by evaluating how typing is affected by a number of psycholinguistic variables defined at the word, syllable, and letter levels. In a writing-to-dictation task, we assessed typing performance by measuring response accuracy, onset latencies - an index of response preparation and initiation - and interkeystroke intervals (IKIs) - an index of response execution processes. The lexical and sublexical factors revealed a composite pattern of effects. Lexical frequency improved response latencies and accuracy, while bigram frequency speeded up IKIs. Sound-spelling consistency improved latencies, but had an inhibitory effect on IKI. IKIs were also longer at syllable boundaries. Together, our findings can be fit within a framework for typed production that combines the previously developed theories of spelling and typing execution. At their interface, we highlight the need for an intermediate hierarchical stage, perhaps in the form of a graphemic buffer for typing.

  13. Type Inference for Session Types in the Pi-Calculus

    DEFF Research Database (Denmark)

    Graversen, Eva Fajstrup; Harbo, Jacob Buchreitz; Huttel, Hans

    2014-01-01

    In this paper we present a direct algorithm for session type inference for the π-calculus. Type inference for session types has previously been achieved by either imposing limitations and restriction on the π-calculus, or by reducing the type inference problem to that for linear types. Our approach...

  14. Combined untargeted and targeted fingerprinting by comprehensive two-dimensional gas chromatography: revealing fructose-induced changes in mice urinary metabolic signatures.

    Science.gov (United States)

    Bressanello, Davide; Liberto, Erica; Collino, Massimo; Chiazza, Fausto; Mastrocola, Raffaella; Reichenbach, Stephen E; Bicchi, Carlo; Cordero, Chiara

    2018-04-01

    This study exploits the information potential of comprehensive two-dimensional gas chromatography configured with a parallel dual secondary column-dual detection by mass spectrometry and flame ionization (GC×2GC-MS/FID) to study changes in urinary metabolic signatures of mice subjected to high-fructose diets. Samples are taken from mice fed with normal or fructose-enriched diets provided either in aqueous solution or in solid form and analyzed at three stages of the dietary intervention (1, 6, and 12 weeks). Automated Untargeted and Targeted fingerprinting for 2D data elaboration is adopted for the most inclusive data mining of GC×GC patterns. The UT fingerprinting strategy performs a fully automated peak-region features fingerprinting and combines results from pre-targeted compounds and unknowns across the sample-set. The most informative metabolites, with statistically relevant differences between sample groups, are obtained by unsupervised multivariate analysis (MVA) and cross-validated by multi-factor analysis (MFA) with external standard quantitation by GC-MS. Results indicate coherent clustering of mice urine signatures according to dietary manipulation. Notably, the metabolite fingerprints of mice fed with liquid fructose exhibited greater derangement in fructose, glucose, citric, pyruvic, malic, malonic, gluconic, cis-aconitic, succinic and 2-keto glutaric acids, glycine acyl derivatives (N-carboxy glycine, N-butyrylglycine, N-isovaleroylglycine, N-phenylacetylglycine), and hippuric acid. Untargeted fingerprinting indicates some analytes which were not a priori pre-targeted which provide additional insights: N-acetyl glucosamine, N-acetyl glutamine, malonyl glycine, methyl malonyl glycine, and glutaric acid. Visual features fingerprinting is used to track individual variations during experiments, thereby extending the panorama of possible data elaboration tools. Graphical abstract ᅟ.

  15. [Influence of exogenous gamma-aminobutyric acid (GABA) on GABA metabolism and amino acid contents in roots of melon seedling under hypoxia stress].

    Science.gov (United States)

    Wang, Chun-Yan; Li, Jing-Rui; Xia, Qing-Ping; Wu, Xiao-Lei; Gao, Hong-Bo

    2014-07-01

    This paper investigated the influence of gamma-aminobutyric acid (GABA) on GABA metabolism and amino acid content under hypoxia stress by accurately controlling the level of dissolved oxygen in hydroponics, using the roots of melon 'Xiyu 1' seedlings as the test material. The results showed that compared with the control, the growth of roots was inhibited seriously under hypoxia stress. Meanwhile, the hypoxia-treated roots had significantly higher activities of glutamate decarboxylase (GAD), glutamate dehydrogenase (GDH), glutamate synthase (GOGAT), glutamine synthetase (GS), alanine aminotransferase (ALT), aspartate aminotransferase (AST) as well as the contents of GABA, pyruvic acid, alanine (Ala) and aspartic acid (Asp). But the contents of glutamic acid (Glu) and alpha-keto glutaric acid in roots under hypoxia stress was obviously lower than those of the control. Exogenous treatment with GABA alleviated the inhibition effect of hypoxia stress on root growth, which was accompanied by an increase in the contents of endogenous GABA, Glu, alpha-keto glutaric acid and Asp. Furthermore, under hypoxia stress, the activities of GAD, GDH, GOGAT, GS, ALT, AST as well as the contents of pyruvic acid and Ala significantly decreased in roots treated with GABA. However, adding GABA and viny-gamma-aminobutyric acid (VGB) reduced the alleviation effect of GABA on melon seedlings under hypoxia stress. The results suggested that absorption of GABA by roots could alleviate the injury of hypoxia stress to melon seedlings. This meant that GABA treatment allows the normal physiological metabolism under hypoxia by inhibiting the GAD activity through feedback and maintaining higher Glu content as well as the bal- ance of carbon and nitrogen.

  16. 常用英文缩略语名词解释

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    AMD:年龄相关性黄斑变性(age—related macular degeneration)DR:糖尿病视网膜病变(diabetic retinopathy)OIR:氧诱导视网膜病变(oxygen—induced retinopathy)Nrf2:核因子E2相关因子2(nuclear factor—E2-related factor 2)ARE:抗氧化反应元件(antioxidant response element)Hrd1:羟甲基戊二酰辅酶A还原酶降解蛋白1(hydroxymethyl glutaric acyl coenzyme A reductase degradation protein 1)。

  17. Type checking with open type functions

    DEFF Research Database (Denmark)

    Schrijvers, Tom; Jones, Simon Peyton; Chakravarty, Manual

    2008-01-01

    We report on an extension of Haskell with open type-level functions and equality constraints that unifies earlier work on GADTs, functional dependencies, and associated types. The contribution of the paper is that we identify and characterise the key technical challenge of entailment checking; an...

  18. Updating signal typing in voice: addition of type 4 signals.

    Science.gov (United States)

    Sprecher, Alicia; Olszewski, Aleksandra; Jiang, Jack J; Zhang, Yu

    2010-06-01

    The addition of a fourth type of voice to Titze's voice classification scheme is proposed. This fourth voice type is characterized by primarily stochastic noise behavior and is therefore unsuitable for both perturbation and correlation dimension analysis. Forty voice samples were classified into the proposed four types using narrowband spectrograms. Acoustic, perceptual, and correlation dimension analyses were completed for all voice samples. Perturbation measures tended to increase with voice type. Based on reliability cutoffs, the type 1 and type 2 voices were considered suitable for perturbation analysis. Measures of unreliability were higher for type 3 and 4 voices. Correlation dimension analyses increased significantly with signal type as indicated by a one-way analysis of variance. Notably, correlation dimension analysis could not quantify the type 4 voices. The proposed fourth voice type represents a subset of voices dominated by noise behavior. Current measures capable of evaluating type 4 voices provide only qualitative data (spectrograms, perceptual analysis, and an infinite correlation dimension). Type 4 voices are highly complex and the development of objective measures capable of analyzing these voices remains a topic of future investigation.

  19. Is type-D personality trait(s or state? An examination of type-D temporal stability in older Israeli adults in the community

    Directory of Open Access Journals (Sweden)

    Ada H. Zohar

    2016-02-01

    Full Text Available Background. Type D personality was suggested as a marker of poorer prognosis for patients of cardiovascular disease. It is defined by having a score of 10 or more on both sub-scales of the DS14 questionnaire, Social Inhibition (SI and Negative Affectivity (NA. As Type D was designed to predict risk, its temporal stability is of prime importance. Methods. Participants in the current study were 285 community volunteers, who completed the DS14, and other personality scales, at a mean interval of six years. Results. The prevalence of Type D did not change. The component traits of Type D showed rank order stability. Type D caseness temporal stability was improved by using the sub-scales product as a criterion. Logistic hierarchical regression predicting Type D classification from Time1 demonstrated that the best predictors were Time1 scores on NA and SI, with the character trait of Cooperation, and the alexithymia score adding some predictive power. Conclusions. The temporal stability of the component traits, and of the prevalence of Type D were excellent. Temporal stability of Type D caseness may be improved by using a product threshold, rather than the current rule. Research is required in order to formulate the optimal timing for Type D measurement for predictive purposes.

  20. Respiratory muscle strength and muscle endurance are not affected by acute metabolic acidemia.

    NARCIS (Netherlands)

    Nizet, T.A.C.; Heijdra, Y.F.; Elshout, F.J.J. van den; Ven, M.J.T. van de; Bosch, F.H.; Mulder, P.H.M. de; Folgering, H.T.M.

    2009-01-01

    Respiratory muscle fatigue in asthma and chronic obstructive lung disease (COPD) contributes to respiratory failure with hypercapnia, and subsequent respiratory acidosis. Therapeutic induction of acute metabolic acidosis further increases the respiratory drive and, therefore, may diminish

  1. Blood typing

    Science.gov (United States)

    ... detect these minor antigens. It is done before transfusions, except in emergency situations. Alternative Names Cross matching; Rh typing; ABO blood typing; Blood group; Anemia - immune hemolytic blood type; ...

  2. Molecular typing of methicillin-resistant Staphylococcus aureus: Comparison of PCR-based open reading frame typing, multilocus sequence typing, and Staphylococcus protein A gene typing.

    Science.gov (United States)

    Ogihara, Shinji; Saito, Ryoichi; Sawabe, Etsuko; Kozakai, Takahiro; Shima, Mari; Aiso, Yoshibumi; Fujie, Toshihide; Nukui, Yoko; Koike, Ryuji; Hagihara, Michio; Tohda, Shuji

    2018-04-01

    The recently developed PCR-based open reading frame typing (POT) method is a useful molecular typing tool. Here, we evaluated the performance of POT for molecular typing of methicillin-resistant Staphylococcus aureus (MRSA) isolates and compared its performance to those of multilocus sequence typing (MLST) and Staphylococcus protein A gene typing (spa typing). Thirty-seven MRSA isolates were collected between July 2012 and May 2015. MLST, spa typing, and POT were performed, and their discriminatory powers were evaluated using Simpson's index analysis. The MRSA isolates were classified into 11, 18, and 33 types by MLST, spa typing, and POT, respectively. The predominant strains identified by MLST, spa typing, and POT were ST8 and ST764, t002, and 93-191-127, respectively. The discriminatory power of MLST, spa typing, and POT was 0.853, 0.875, and 0.992, respectively, indicating that POT had the highest discriminatory power. Moreover, the results of MLST and spa were available after 2 days, whereas that of POT was available in 5 h. Furthermore, POT is rapid and easy to perform and interpret. Therefore, POT is a superior molecular typing tool for monitoring nosocomial transmission of MRSA. Copyright © 2017 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

  3. Type classes for mathematics in type theory

    OpenAIRE

    Spitters, Bas; Van der Weegen, Eelis

    2011-01-01

    The introduction of first-class type classes in the Coq system calls for re-examination of the basic interfaces used for mathematical formalization in type theory. We present a new set of type classes for mathematics and take full advantage of their unique features to make practical a particularly flexible approach formerly thought infeasible. Thus, we address both traditional proof engineering challenges as well as new ones resulting from our ambition to build upon this development a library...

  4. Luminescence dynamics in type-II GaAs/AlAs superlattices near the type-I to type-II crossover

    DEFF Research Database (Denmark)

    Langbein, Wolfgang Werner; Kalt, H.; Hvam, Jørn Märcher

    1996-01-01

    We report on a study of the time-resolved luminescence of type-II GaAs/AlAs superlattices near the type-I to type-II crossover. In spite of the slight type-II band alignment, the luminescence is dominated by the type-I transition. This is due to the inhomogeneous broadening of the type-I transiti...

  5. Several types of types in programming languages

    OpenAIRE

    Martini, Simone

    2015-01-01

    Part 2: Regular Submissions; International audience; Types are an important part of any modern programming language, but we often forget that the concept of type we understand nowadays is not the same it was perceived in the sixties. Moreover, we conflate the concept of " type " in programming languages with the concept of the same name in mathematical logic, an identification that is only the result of the convergence of two different paths, which started apart with different aims. The paper...

  6. Types and Automata

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Meineche Smidt, Erik

    A hierarchical type system for imperative programming languages gives rise to various computational problems, such as type equivalence, type ordering, etc. We present a particular class of finite automata which are shown to be isomorphic to type equations. All the relevant type concepts turn out...... to have well-known automata analogues, such as language equality, language inclusion, etc. This provides optimal or best known algorithms for the type system, by a process of translating type equations to automata, solving the analogous problem, and translating the result back to type equations. Apart...

  7. IL-10 dependent suppression of type 1, type 2 and type 17 cytokines in active pulmonary tuberculosis.

    Directory of Open Access Journals (Sweden)

    Nathella Pavan Kumar

    Full Text Available Although Type 1 cytokine responses are considered protective in pulmonary tuberculosis (PTB, their role as well as those of Type 2, 17 and immunoregulatory cytokines in tuberculous lymphadenitis (TBL and latent tuberculosis (LTB have not been well studied.To identify cytokine responses associated with pulmonary tuberculosis (TB, TB lymphadenitits and latent TB, we examined mycobacterial antigen-specific immune responses of PTB, TBL and LTB individuals. More specifically, we examined ESAT-6 and CFP-10 induced Type 1, Type 2 and Type 17 cytokine production and their regulation using multiplex ELISA.PTB individuals exhibited a significantly lower baseline as well as antigen-specific production of Type 1 (IFNγ, TNFα and IL-2; Type 2 (IL-4 and Type 17 (IL-17A and IL-17F cytokines in comparison to both TBL and LTB individuals. TBL individuals exhibited significantly lower antigen-specific IFNγ responses alone in comparison to LTB individuals. Although, IL-10 levels were not significantly higher, neutralization of IL-10 during antigen stimulation resulted in significantly enhanced production of IFNγ, IL-4 and IL-17A in PTB individuals, indicating that IL-10 mediates (at least partially the suppression of cytokine responses in PTB.Pulmonary TB is characterized by an IL-10 dependent antigen-specific suppression of Type 1, Type 2 and Type 17 cytokines, reflecting an important association of these cytokines in the pathogenesis of active TB.

  8. Hand functions in type 1 and type 2 diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Akpinar Pinar

    2017-01-01

    Full Text Available Introduction/Objective. Hand functions have an enormous impact on activities of daily living in patients with diabetes mellitus (DM, such as self-care, administering insulin injections, and preparing and eating meals. The aim of the study was to evaluate hand functions and grip strength in patients with type 1 and type 2 DM. Methods. This was an observational case-control study investigating the hand functions and grip strength in patients with type 1 and type 2 DM. The study comprised 41 patients with type 1 DM aged 25–50 years sex- and age-matched, 40 non-diabetic controls, and 91 patients with type 2 DM aged 40–65 years sex- and age-matched 60 non-diabetic controls. Patients with documented history of diabetic sensorimotor neuropathy and adhesive capsulitis were excluded. The Duruoz Hand Index was used to assess the functional hand disability. Grip strength was tested with a calibrated Jamar dynamometer. Results. The Duruoz Hand Index scores in patients with type 2 DM were significantly higher than in persons in the control group (p 0.05. Grip strength values of patients with type 1 DM were significantly lower compared to those in the control group (p < 0.05, whereas there was no significant difference between patients with type 2 DM and their control group. There was a negatively significant correlation between grip strength and the Duruoz Hand Index scores in patients with both type 1 and type 2 DM (p < 0.05. Conclusion. Patients with type 1 DM and type 2 DM have different degrees of hand disability as compared to healthy control groups.

  9. Directing polyallylamine adsorption on microlens array patterned silicon for microarray fabrication.

    Science.gov (United States)

    Saini, Gaurav; Gates, Richard; Asplund, Matthew C; Blair, Steve; Attavar, Sachin; Linford, Matthew R

    2009-06-21

    The selective adsorption of reagents is often essential for bioarray and lab-on-a-chip type devices. As the starting point for a bioarray, alkyl monolayer terminated silicon shards were photopatterned in a few nanoseconds with thousands of wells (spots) using an optical element, a microlens array. Polyallylamine (PAAm), a primary amine containing polymer, adsorbed with little selectivity to the spots, i.e., silicon oxide, over the hydrophobic background. However, at appropriate concentrations, addition of a cationic surfactant to the PAAm deposition solution, cetyltrimethylammonium chloride, prevented the nonspecific adsorption of PAAm onto the hydrophobic monolayer, while directing it effectively to the active spots on the device. A nonionic surfactant was less effective in preventing the nonspecific adsorption of PAAm onto the hydrophobic monolayer. The localized reactions/interactions of adsorbed PAAm with four species that are useful for bioconjugate chemistry: glutaric anhydride, phenylenediisothiocyanate, biotin NHS ester, and an oligonucleotide (DNA) were shown in the spots of an array. The reactivity of PAAm was further demonstrated with an isocyanate. Time-of-flight secondary ion mass spectrometry (ToF-SIMS) played an important role in confirming selective surface reactivity and adsorption. X-ray photoelectron spectroscopy (XPS), spectroscopic ellipsometry, and wetting confirmed PAAm reactivity on planar substrates.

  10. Phage typing or CRISPR typing for epidemiological surveillance of Salmonella Typhimurium?

    Science.gov (United States)

    Mohammed, Manal

    2017-11-07

    Salmonella Typhimurium is the most dominant Salmonella serovar around the world. It is associated with foodborne gastroenteritis outbreaks but has recently been associated with invasive illness and deaths. Characterization of S. Typhimurium is therefore very crucial for epidemiological surveillance. Phage typing has been used for decades for subtyping of S. Typhimurium to determine the epidemiological relation among isolates. Recent studies however have suggested that high throughput clustered regular interspaced short palindromic repeats (CRISPR) typing has the potential to replace phage typing. This study aimed to determine the efficacy of high-throughput CRISPR typing over conventional phage typing in epidemiological surveillance and outbreak investigation of S. Typhimurium. In silico analysis of whole genome sequences (WGS) of well-documented phage types of S. Typhimurium reveals the presence of different CRISPR type among strains belong to the same phage type. Furthermore, different phage types of S. Typhimurium share identical CRISPR type. Interestingly, identical spacers were detected among outbreak and non-outbreak associated DT8 strains of S. Typhimurium. Therefore, CRISPR typing is not useful for the epidemiological surveillance and outbreak investigation of S. Typhimurium and phage typing, until it is replaced by WGS, is still the gold standard method for epidemiological surveillance of S. Typhimurium.

  11. Solar Type II Radio Bursts and IP Type II Events

    Science.gov (United States)

    Cane, H. V.; Erickson, W. C.

    2005-01-01

    We have examined radio data from the WAVES experiment on the Wind spacecraft in conjunction with ground-based data in order to investigate the relationship between the shocks responsible for metric type II radio bursts and the shocks in front of coronal mass ejections (CMEs). The bow shocks of fast, large CMEs are strong interplanetary (IP) shocks, and the associated radio emissions often consist of single broad bands starting below approx. 4 MHz; such emissions were previously called IP type II events. In contrast, metric type II bursts are usually narrowbanded and display two harmonically related bands. In addition to displaying complete dynamic spectra for a number of events, we also analyze the 135 WAVES 1 - 14 MHz slow-drift time periods in 2001-2003. We find that most of the periods contain multiple phenomena, which we divide into three groups: metric type II extensions, IP type II events, and blobs and bands. About half of the WAVES listings include probable extensions of metric type II radio bursts, but in more than half of these events, there were also other slow-drift features. In the 3 yr study period, there were 31 IP type II events; these were associated with the very fastest CMEs. The most common form of activity in the WAVES events, blobs and bands in the frequency range between 1 and 8 MHz, fall below an envelope consistent with the early signatures of an IP type II event. However, most of this activity lasts only a few tens of minutes, whereas IP type II events last for many hours. In this study we find many examples in the radio data of two shock-like phenomena with different characteristics that occur simultaneously in the metric and decametric/hectometric bands, and no clear example of a metric type II burst that extends continuously down in frequency to become an IP type II event. The simplest interpretation is that metric type II bursts, unlike IP type II events, are not caused by shocks driven in front of CMEs.

  12. Tension Type Headache: Evaluation of Chronic Type

    Directory of Open Access Journals (Sweden)

    Ömer Karadaş

    2013-11-01

    Full Text Available Tension type headache(TTH which is a primary headache has episodic and chronic forms. Episodic TTH (ETTH can also be frequent-type and non-frequent-type. According to population-based studies, annual prevalence rates are 38.3% for ETTH and 2.2% for chronic TTH (CTTH. Patients can shift between the sub-groups of TTH. In particular, patients with ETTH are at risk of developing CTTH. Peripheral and central nociceptive mechanism are thought to be responsible in occurrence of TTH. Psychiatric disorders are frequently associated with TTH. Although basic and combined analgesics are used in acute treatment and antidepresants are used in prophylaxis, new treatment modalities are needed.

  13. Prophylactic amnioinfusion for intrapartum oligohydramnios: a meta-analysis of randomized controlled trials.

    Science.gov (United States)

    Pitt, C; Sanchez-Ramos, L; Kaunitz, A M; Gaudier, F

    2000-11-01

    To evaluate the effectiveness of intrapartum prophylactic amnioinfusion in pregnancies complicated by oligohydramnios. Randomized controlled trials of prophylactic amnioinfusion in women with oligohydramnios were identified using computerized databases, index reviews, and references cited in original studies and review articles. We evaluated, abstracted data from, and analyzed randomized studies of prophylactic intrapartum amnioinfusion in women with oligohydramnios. In every study the group allocation was based exclusively on presence of oligohydramnios. Only published studies with clearly documented outcome data were included. The quality of each trial was evaluated for methodology, inclusion and exclusion criteria, adequacy of randomization, amnioinfusion protocols, definition of outcomes, and statistical analyses. The trials were evaluated concerning cesarean deliveries for fetal heart rate (FHR) abnormalities, overall cesarean rates, acidemia at birth, intrapartum fetal heart rate abnormalities, Apgar scores under 7 at 5 minutes, and postpartum endometritis. Thirty-five studies were identified, of which 14 met the inclusion criteria for this systematic review. They included 1533 patients, 793 in the amnioinfusion group, and 740 controls. Odds ratios (OR) with their 95% confidence intervals (CI) for each outcome were calculated. We calculated an estimate of the OR and risk difference for dichotomous outcomes using random and fixed-effects models. A test of homogeneity was done across studies. Women with oligohydramnios who received intrapartum amnioinfusion had lower incidence of cesarean for FHR abnormalities (OR 0.23; 95% CI 0.15, 0.35). Intrapartum amnioinfusion also was associated with lower overall rates of cesarean deliveries (OR 0.52; 95% CI 0.40, 0. 68), acidemia at birth (OR 0.40; 95% CI 0.30, 0.55), FHR abnormalities during labor (OR 0.24; 95% CI 0.17, 0.34), and Apgar scores under 7 at 5 minutes (OR 0.52; 95% CI 0.29, 0.91). Postpartum endometritis

  14. Factors influencing immediate post-release survival of spectacled eiders following surgical implantation of transmitters with percutaneous antennae

    Science.gov (United States)

    Sexson, Matthew G.; Mulcahy, Daniel M.; Spriggs, Maria; Myers, Gwen E.

    2014-01-01

    Surgically implanted transmitters are a common method for tracking animal movements. Immediately following surgical implantation, animals pass through a critical recovery phase when behaviors may deviate from normal and the likelihood of individual survival may be reduced. Therefore, data collected during this period may be censored to minimize bias introduced by surgery-related behaviors or mortality. However, immediate post-release mortalities negate a sampling effort and reduce the amount of data potentially collected after the censoring period. Wildlife biologists should employ methods to support an animal’s survival through this period, but factors contributing to immediate post-release survival have not been formally assessed. We evaluated factors that potentially influenced the immediate post-release survival of 56 spectacled eiders (Somateria fischeri) marked with coelomically implanted satellite transmitters with percutaneous antennae in northern Alaska in 2010 and 2011. We modeled survival through the first 14 days following release and assessed the relative importance and effect of 15 covariates hypothesized to influence survival during this immediate post-release period. Estimated daily survival rate increased over the duration of the immediate post-release period; the probability of mortality was greatest within the first 5 days following release. Our top-ranking model included the effect of 2 blood analytes, pH and hematocrit, measured prior to surgical implantation of a transmitter. We found a positive response to pH; eiders exhibiting acidemia (low pH) prior to surgery were less likely to survive the immediate post-release period. We found a curvilinear response to hematocrit; eiders exhibiting extremely low or high pre-surgery hematocrit were also less likely to survive the immediate post-release period. In the interest of maximizing the survival of marked birds following release, hematological data obtained prior to surgical implantation of

  15. 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.

  16. Innate-Type and Acquired-Type Allergy Regulated by IL-33

    Directory of Open Access Journals (Sweden)

    Tomohiro Yoshimoto

    2014-01-01

    Full Text Available We propose two types of allergic response: IgE-dependent and IgE-independent, and designate these as 'acquired-type allergy' and 'innate-type allergy', respectively. IL-33 stimulates both innate (basophils, mast cells, or group 2 innate lymphoid cells and acquired (Th2 cells allergy-related cells to induce and/or augment Th2 cytokine production, which leads to eosinophilic inflammation in vivo. Thus, IL-33 is an essential regulator for both 'innate-type allergy' and 'acquired-type allergy', and might be an attractive therapeutic target for allergic diseases.

  17. Type 1 or Type 2 Diabetes and Pregnancy

    Science.gov (United States)

    ... and Pregnancy Articles Type 1 or Type 2 Diabetes and Pregnancy Language: English (US) Español (Spanish) Recommend on Facebook Tweet Share Compartir Problems of Diabetes in Pregnancy Blood sugar that is not well ...

  18. Discriminating the reaction types of plant type III polyketide synthases.

    Science.gov (United States)

    Shimizu, Yugo; Ogata, Hiroyuki; Goto, Susumu

    2017-07-01

    Functional prediction of paralogs is challenging in bioinformatics because of rapid functional diversification after gene duplication events combined with parallel acquisitions of similar functions by different paralogs. Plant type III polyketide synthases (PKSs), producing various secondary metabolites, represent a paralogous family that has undergone gene duplication and functional alteration. Currently, there is no computational method available for the functional prediction of type III PKSs. We developed a plant type III PKS reaction predictor, pPAP, based on the recently proposed classification of type III PKSs. pPAP combines two kinds of similarity measures: one calculated by profile hidden Markov models (pHMMs) built from functionally and structurally important partial sequence regions, and the other based on mutual information between residue positions. pPAP targets PKSs acting on ring-type starter substrates, and classifies their functions into four reaction types. The pHMM approach discriminated two reaction types with high accuracy (97.5%, 39/40), but its accuracy decreased when discriminating three reaction types (87.8%, 43/49). When combined with a correlation-based approach, all 49 PKSs were correctly discriminated, and pPAP was still highly accurate (91.4%, 64/70) even after adding other reaction types. These results suggest pPAP, which is based on linear discriminant analyses of similarity measures, is effective for plant type III PKS function prediction. pPAP is freely available at ftp://ftp.genome.jp/pub/tools/ppap/. goto@kuicr.kyoto-u.ac.jp. Supplementary data are available at Bioinformatics online. © The Author(s) 2017. Published by Oxford University Press.

  19. Greenberger-Horne-Zeilinger-type and W-type entangled coherent states: Generation and Bell-type inequality tests without photon counting

    International Nuclear Information System (INIS)

    Jeong, Hyunseok; Nguyen Ba An

    2006-01-01

    We study Greenberger-Horne-Zeilinger-type (GHZ-type) and W-type three-mode entangled coherent states. Both types of entangled coherent states violate Mermin's version of the Bell inequality with threshold photon detection (i.e., without photon counting). Such an experiment can be performed using linear optics elements and threshold detectors with significant Bell violations for GHZ-type entangled coherent states. However, to demonstrate Bell-type inequality violations for W-type entangled coherent states, additional nonlinear interactions are needed. We also propose an optical scheme to generate W-type entangled coherent states in free-traveling optical fields. The required resources for the generation are a single-photon source, a coherent state source, beam splitters, phase shifters, photodetectors, and Kerr nonlinearities. Our scheme does not necessarily require strong Kerr nonlinear interactions; i.e., weak nonlinearities can be used for the generation of the W-type entangled coherent states. Furthermore, it is also robust against inefficiencies of the single-photon source and the photon detectors

  20. Selective screening of 650 high risk Iranian patients for detection of inborn error of metabolism

    Directory of Open Access Journals (Sweden)

    Narges Pishva

    2015-02-01

    Full Text Available Objective: Although metabolic diseases individually are rare ,but overall have an incidence of 1/2000 and can cause devastating and irreversible effect if not diagnosed early and treated promptly. selective screening is an acceptable method for detection of these multi presentation diseases.Method: using panel neonatal screening for detection of metabolic diseases in 650 high risk Iranian patients in Fars province. The following clinical features were used as inclusion criteria for investigation of the patients.Lethargy, poor feeding ,persistent vomiting, cholestasis, intractable seizure ,decreased level of consciousness ,persistent hypoglycemia, unexplained acid base disturbance and unexplained neonatal death.Result: Organic acidemia with 40 cases (42% was the most frequent disorder diagnosed in our high risk populations, followed by disorder of galactose metabolism(30%, 15 patient had classic galactosemia(GALT

  1. Selective screening of 650 high risk Iranian patients for detection of inborn error of metabolism

    Directory of Open Access Journals (Sweden)

    Narges Pishva

    2015-02-01

    Full Text Available Objective: Although metabolic diseases individually are rare ,but overall have an incidence of 1/2000 and can cause devastating and irreversible effect if not diagnosed early and treated promptly. selective screening is an acceptable method for detection of these multi presentation diseases. Method: using panel neonatal screening for detection of metabolic diseases in 650 high risk Iranian patients in Fars province. The following clinical features were used as inclusion criteria for investigation of the patients. Lethargy, poor feeding ,persistent vomiting, cholestasis, intractable seizure ,decreased level of consciousness ,persistent hypoglycemia, unexplained acid base disturbance and unexplained neonatal death. Result: Organic acidemia with 40 cases (42% was the most frequent disorder diagnosed in our high risk populations, followed by disorder of galactose metabolism(30%, 15 patient had classic galactosemia(GALT

  2. Aminoacidopatias de interesse neurológico

    Directory of Open Access Journals (Sweden)

    Aron J. Diament

    1974-06-01

    Full Text Available As aminoácidopatias constituem o grupo mais numeroso dos erros inatos do metabolismo, sendo crescente seu número em vista das inúmeras cadeias metabólicas envolvendo os aminoácidos na economia humana. É apresentada uma classificação atualizada das principais aminoacidopatias que determinam sintomatologia neurológica e/ou mental. São revistos os principais métodos de diagnóstico, apontando-se as falhas de algumas metodologias. São abordadas algumas particularidades da fenilcetonúria, leucinose e acidemia propiônica, principalmente no que concerne à variação genética. Finalmente, são apresentadas duas aminoacidopatias recentemente descritas: a aciduria piroglutâmica e a deficiência da beta-metil-crotonil-CoA-carboxilase.

  3. Types of intersections.

    NARCIS (Netherlands)

    2015-01-01

    There are many types of intersections in the Netherlands. In an inherently safe road traffic system, however, the number of intersection types needs to be limited, depending on the road types that intersect. The desired types of intersections do not always correspond with the recommendations in the

  4. Equational type logic

    NARCIS (Netherlands)

    Manca, V.; Salibra, A.; Scollo, Giuseppe

    1990-01-01

    Equational type logic is an extension of (conditional) equational logic, that enables one to deal in a single, unified framework with diverse phenomena such as partiality, type polymorphism and dependent types. In this logic, terms may denote types as well as elements, and atomic formulae are either

  5. CT findings of muscular dystrophy; Limb girdle type (LG), myotonic type (MYD) and Duchenne type (DMD)

    Energy Technology Data Exchange (ETDEWEB)

    Saitoh, Hiroshi (Tokushima Univ. (Japan). School of Medicine)

    1991-07-01

    CT scans of muscles in patients with limb girdle type (LG), myotonic type (MYD) and Duchenne type (DMD) dystrophies were obtained at five different body levels: the neck, L3 vertebral body, pelvic girdle, thigh and lower leg. CT numbers, cross sectional areas (CSA) and %CSA of muscle or fat were evaluated in each muscle. The characteristic CT patterns for each type of muscular dystrophy were obtained. Compared with DMD, the gracilis and soleus were more severely damaged in LG and the biceps femoris remained relatively preserved among the hamstrings. In addition, the multifidus of the neck and sternocleidomastoid also were more severely damaged in MYD. This study suggests that CT scan will be useful in the differential diagnosis of these types of muscular dystrophy as well as in planning appropriate rehabilitation and detecting damaged muscles. (author).

  6. Thinking about gender types: cognitive organization of female and male types.

    Science.gov (United States)

    Vonk, Roos; Ashmore, Richard D

    2003-06-01

    We examined the content and dimensional structure of a large and representative sample of gender types. In Study 1, using an open-ended procedure, participants generated 306 different labels for female types (e.g. housewife, feminist, femme fatale, secretary, slob) and 310 for male types (e.g. workaholic, family man, sissy, womanizer, labourer). In Study 2A, a multidimensional configuration of 229 of these male and female types was derived from a free sorting task among a new set of participants. In Study 2B, a subset of types was judged on several dimensions of meaning, which were then fitted into the configuration of types. The most important dimensions in describing the structure of gender types were: young-old, masculine-feminine and traditional-modern. The masculine-feminine dimension showed that the male and female types were largely separated from each other; within each gender category, the types were ordered by their position on the masculine-feminine dimension. Several other aspects of current thinking about men and women are discussed.

  7. Guarded Cubical Type Theory

    DEFF Research Database (Denmark)

    Birkedal, Lars; Bizjak, Aleš; Clouston, Ranald

    2016-01-01

    This paper improves the treatment of equality in guarded dependent type theory (GDTT), by combining it with cubical type theory (CTT). GDTT is an extensional type theory with guarded recursive types, which are useful for building models of program logics, and for programming and reasoning...... with coinductive types. We wish to implement GDTT with decidable type-checking, while still supporting non-trivial equality proofs that reason about the extensions of guarded recursive constructions. CTT is a variation of Martin-L\\"of type theory in which the identity type is replaced by abstract paths between...... terms. CTT provides a computational interpretation of functional extensionality, is conjectured to have decidable type checking, and has an implemented type-checker. Our new type theory, called guarded cubical type theory, provides a computational interpretation of extensionality for guarded recursive...

  8. Guarded Cubical Type Theory

    DEFF Research Database (Denmark)

    Birkedal, Lars; Bizjak, Aleš; Clouston, Ranald

    2016-01-01

    This paper improves the treatment of equality in guarded dependent type theory (GDTT), by combining it with cubical type theory (CTT). GDTT is an extensional type theory with guarded recursive types, which are useful for building models of program logics, and for programming and reasoning...... with coinductive types. We wish to implement GDTT with decidable type checking, while still supporting non-trivial equality proofs that reason about the extensions of guarded recursive constructions. CTT is a variation of Martin-L\\"of type theory in which the identity type is replaced by abstract paths between...... terms. CTT provides a computational interpretation of functional extensionality, enjoys canonicity for the natural numbers type, and is conjectured to support decidable type-checking. Our new type theory, guarded cubical type theory (GCTT), provides a computational interpretation of extensionality...

  9. A Type System For Certified Runtime Type Analysis

    Science.gov (United States)

    2002-12-01

    1999 ACM SIGPLAN International Conf. on Functional Pro- gramming (ICFP’99), pages 183–196. ACM Press, September 1999. [Min97] Yasuhiko Minamide. Full...lifting of type parameters. Technical report, RIMS, Kyoto University, 1997. [MMH96] Yasuhiko Minamide, Greg Morrisett, and Robert Harper. Typed

  10. Guarded Type Promotion

    DEFF Research Database (Denmark)

    Winther, Johnni

    2011-01-01

    conditional using the instanceof operator and thus the cast type is redundantly mentioned twice. We propose a new typing rule for Java called Guarded Type Promotion aimed at eliminating the need for the explicit casts when guarded. This new typing rule is backward compatible and has been fully implemented...... in a Java 6 compiler. Through our extensive testing of real-life code we show that guarded casts account for approximately one fourth of all casts and that Guarded Type Promotion can eliminate the need for 95 percent of these guarded casts....

  11. Facts about Type 2

    Medline Plus

    Full Text Available ... Alert Day Diabetes Basics Home Symptoms Diagnosis America's Diabetes Challenge Type 1 Type 2 Facts About Type 2 Enroll in ... Where Do I Begin With Type2? Living With Type 1 Diabetes Enroll in the Living WIth Type 2 Diabetes ...

  12. Type 2 diabetes

    Science.gov (United States)

    ... type 2 diabetes; Oral hypoglycemic - type 2 diabetes; High blood sugar - type 2 diabetes ... your kidneys are working well ( microalbuminuria and serum creatinine ). Visit your eye doctor at least once a ...

  13. Ceramide content is higher in type I compared to type II fibers in obesity and type 2 diabetes mellitus

    DEFF Research Database (Denmark)

    Kristensen, Ditte Bech; Prats Gavalda, Clara; Larsen, Steen

    2012-01-01

    This study investigated fiber-type-specific muscle ceramide content in obese subjects and type 2 diabetes patients. Two substudies, one which compared type 2 diabetes patients to both lean- and obese BMI-matched subjects and the other study which compared lean body-matched post-obese, obese......, and control subjects, were performed. A fasting blood sample was obtained and plasma insulin and glucose determined. A muscle biopsy was obtained from deltoideus and vastus lateralis, and fiber-type ceramide content was determined by fluorescence immunohistochemistry. Insulin sensitivity estimated by Quicki...... index was higher in lean compared to type 2 diabetes patients and obese controls. Also in control and post-obese subjects, a higher insulin sensitivity was observed compared to obese subjects. Ceramide content was consistently higher in type I than in type II muscle fibers and higher in deltoideus than...

  14. Hybrid type I-type II superconducting behavior in magnesium diboride

    International Nuclear Information System (INIS)

    Kunchur, M.N.; Saracila, G.; Arcos, D.A.; Cui, Y.; Pogrebnyakov, A.; Orgiani, P.; Xi, X.X.

    2006-01-01

    In traditional type-II superconductors, an applied magnetic field depresses the transition temperature and introduces magnetic flux vortices that cause resistive losses accompanied by a broadening of the transition. High-field high-pulsed-current measurements have revealed a new hybrid behavior in disordered magnesium diboride films: The superconductivity survives high magnetic fields by entering a mixed state with vortices (like a type II superconductor) but holds its vortices nearly motionless and avoids dissipation (like a type I superconductor). A study of this phenomenon in magnesium diboride films with varying degrees of scattering indicate that the hybrid type I-type II behavior arises from the two-band nature of the superconductivity and the different degrees of influence that disorder exerts on its different bands. (author)

  15. Blood Types

    Science.gov (United States)

    ... blood, safe blood transfusions depend on careful blood typing and cross-matching. There are four major blood ... cause exceptions to the above patterns. ABO blood typing is not sufficient to prove or disprove paternity ...

  16. Identification of type II and type III pyoverdine receptors from Pseudomonas aeruginosa.

    Science.gov (United States)

    de Chial, Magaly; Ghysels, Bart; Beatson, Scott A; Geoffroy, Valérie; Meyer, Jean Marie; Pattery, Theresa; Baysse, Christine; Chablain, Patrice; Parsons, Yasmin N; Winstanley, Craig; Cordwell, Stuart J; Cornelis, Pierre

    2003-04-01

    Pseudomonas aeruginosa produces, under conditions of iron limitation, a high-affinity siderophore, pyoverdine (PVD), which is recognized at the level of the outer membrane by a specific TonB-dependent receptor, FpvA. So far, for P. aeruginosa, three different PVDs, differing in their peptide chain, have been described (types I-III), but only the FpvA receptor for type I is known. Two PVD-producing P. aeruginosa strains, one type II and one type III, were mutagenized by a mini-TnphoA3 transposon. In each case, one mutant unable to grow in the presence of the strong iron chelator ethylenediaminedihydroxyphenylacetic acid (EDDHA) and the cognate PVD was selected. The first mutant, which had an insertion in the pvdE gene, upstream of fpvA, was unable to take up type II PVD and showed resistance to pyocin S3, which is known to use type II FpvA as receptor. The second mutant was unable to take up type III PVD and had the transposon insertion in fpvA. Cosmid libraries of the respective type II and type III PVD wild-type strains were constructed and screened for clones restoring the capacity to grow in the presence of PVD. From the respective complementing genomic fragments, type II and type III fpvA sequences were determined. When in trans, type II and type III fpvA restored PVD production, uptake, growth in the presence of EDDHA and, in the case of type II fpvA, pyocin S3 sensitivity. Complementation of fpvA mutants obtained by allelic exchange was achieved by the presence of cognate fpvA in trans. All three receptors posses an N-terminal extension of about 70 amino acids, similar to FecA of Escherichia coli, but only FpvAI has a TAT export sequence at its N-terminal end.

  17. Types of Diabetes

    Science.gov (United States)

    ... Diabetes, Sexual, & Bladder Problems Clinical Trials What is Diabetes? Diabetes is a disease that occurs when your ... is serious. What are the different types of diabetes? The most common types of diabetes are type ...

  18. Extending Dylan's type system for better type inference and error detection

    DEFF Research Database (Denmark)

    Mehnert, Hannes

    2010-01-01

    a dynamically typed language. Dylan poses several special challenges for gradual typing, such as multiple return values, variable-arity methods and generic functions (multiple dispatch). In this paper Dylan is extended with function types and parametric polymorphism. We implemented the type system...... and aunification-based type inference algorithm in the mainstream Dylan compiler. As case study we use the Dylan standard library (roughly 32000 lines of code), which witnesses that the implementation generates faster code with fewer errors. Some previously undiscovered errors in the Dylan library were revealed....

  19. Types of Dementia

    Science.gov (United States)

    ... Kids For Teens For Parents & Teachers Resolving Family Conflicts The Holidays and Alzheimer's Glossary Virtual Library Online ... Use Map Selector Search Alzheimer’s Association Alzheimer's & Dementia Types of Dementia Types of Dementia Types of Dementia ...

  20. Chemical-free n-type and p-type multilayer-graphene transistors

    Energy Technology Data Exchange (ETDEWEB)

    Dissanayake, D. M. N. M., E-mail: nandithad@voxtel-inc.com [Voxtel Inc, Lockey Laboratories, University of Oregon, Eugene Oregon 97402 (United States); Eisaman, M. D. [Sustainable Energy Technologies Department, Brookhaven National Laboratory, Upton, New York 11973 (United States); Department of Electrical and Computer Engineering, Stony Brook University, Stony Brook, New York 11794 (United States); Department of Physics and Astronomy, Stony Brook University, Stony Brook, New York 11794 (United States)

    2016-08-01

    A single-step doping method to fabricate n- and p-type multilayer graphene (MG) top-gate field effect transistors (GFETs) is demonstrated. The transistors are fabricated on soda-lime glass substrates, with the n-type doping of MG caused by the sodium in the substrate without the addition of external chemicals. Placing a hydrogen silsesquioxane (HSQ) barrier layer between the MG and the substrate blocks the n-doping, resulting in p-type doping of the MG above regions patterned with HSQ. The HSQ is deposited in a single fabrication step using electron beam lithography, allowing the patterning of arbitrary sub-micron spatial patterns of n- and p-type doping. When a MG channel is deposited partially on the barrier and partially on the glass substrate, a p-type and n-type doping profile is created, which is used for fabricating complementary transistors pairs. Unlike chemically doped GFETs in which the external dopants are typically introduced from the top, these substrate doped GFETs allow for a top gate which gives a stronger electrostatic coupling to the channel, reducing the operating gate bias. Overall, this method enables scalable fabrication of n- and p-type complementary top-gated GFETs with high spatial resolution for graphene microelectronic applications.

  1. Type 1 diabetes

    Science.gov (United States)

    Insulin-dependent diabetes; Juvenile onset diabetes; Diabetes - type 1; High blood sugar - type 1 diabetes ... Type 1 diabetes can occur at any age. It is most often diagnosed in children, adolescents, or young adults. Insulin is ...

  2. Type D (distressed) personality in primary care patients with type 2 diabetes

    DEFF Research Database (Denmark)

    Nefs, Giesje; Pouwer, Francois; Pop, Victor J M

    2012-01-01

    D Scale-14 (DS14) in type 2 diabetes patients. METHODS: 1553 primary care patients with type 2 diabetes were assessed for demographic, clinical, lifestyle and psychological characteristics in 2007. A subgroup (n=1012) completed the DS14 again 1 year later. RESULTS: The two-factor model of the Type D...... or physiological risk factors, but Type D women had a more sedentary lifestyle (p=.003). Type D patients experienced less social support and more stressful life events, loneliness, and more depressed mood, anhedonia and anxiety (p0......OBJECTIVE: In cardiovascular research, Type D personality (high negative affectivity and social inhibition) has been associated with a more than 3-fold increased risk of adverse health outcomes. This study examined the validity and clinical correlates of the Type D construct as assessed by the Type...

  3. Product shape selectivity of MFI-type, MEL-type, and BEA-type zeolites in the catalytic hydroconversion of heptane

    NARCIS (Netherlands)

    Poursaeidesfahani, A.; de Lange, M.F.; Khodadadian, F.; Dubbeldam, D.; Rigutto, Marcello; Nair, Nitish; Vlugt, T.J.H.

    2017-01-01

    The influence of product shape selectivity on the bifunctional conversion of n-C7 by zeolite catalysts is investigated. Three different zeolite catalysts with different pore sizes (MFI-type, MEL-type, and BEA-type zeolites) have been investigated experimentally. For all three

  4. Diabetes Type 2

    Science.gov (United States)

    Diabetes means your blood glucose, or blood sugar, levels are too high. With type 2 diabetes, the more common type, your body does not ... You have a higher risk of type 2 diabetes if you are older, have obesity, have a ...

  5. Role of type 1 and type 3 fimbriae in Klebsiella pneumoniae biofilm formation

    DEFF Research Database (Denmark)

    Schroll, C.; Barken, Kim Bundvig; Krogfelt, K.A.

    2010-01-01

    nosocomial infections. Most clinical K. pneumoniae isolates express two types of fimbrial adhesins, type 1 fimbriae and type 3 fimbriae. In this study, we characterized the role of type 1 and type 3 fimbriae in K. pneumoniae biofilm formation. Results: Isogenic fimbriae mutants of the clinical K. pneumoniae...... of planktonic cells. Type 1 fimbriae did not influence biofilm formation and the expression of type 1 fimbriae was found to be down-regulated in biofilm forming cells. In contrast, expression of type 3 fimbriae was found to strongly promote biofilm formation. Conclusion: By use of well defined isogenic mutants...... we found that type 3 fimbriae, but not type 1 fimbriae, strongly promote biofilm formation in K. pneumoniae C3091. As the vast majority of clinical K. pneumoniae isolates express type 3 fimbriae, this fimbrial adhesin may play a significant role in development of catheter associated K. pneumoniae...

  6. Type Inference with Inequalities

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff

    1991-01-01

    of (monotonic) inequalities on the types of variables and expressions. A general result about systems of inequalities over semilattices yields a solvable form. We distinguish between deciding typability (the existence of solutions) and type inference (the computation of a minimal solution). In our case, both......Type inference can be phrased as constraint-solving over types. We consider an implicitly typed language equipped with recursive types, multiple inheritance, 1st order parametric polymorphism, and assignments. Type correctness is expressed as satisfiability of a possibly infinite collection...

  7. Pattern of thyroid auto immunity in type 1 and type 2 diabetics

    International Nuclear Information System (INIS)

    Yasmin, T.

    2006-01-01

    To determine the level of thyroid auto immunity among clinically euthyroid patients of type 1 and type 2 diabetics and to correlate the levels with pattern of diabetes. Blood samples were collected from 163 subjects (51 of type 1, 61 of type 2 diabetics and 51 non- diabetic controls) and sera were separated. They were all clinically euthyroid and selected conveniently. Thyroid profile (FT4, TSH and anti- TPO) was assessed in the three groups and compared. It was observed that at a cut off value of less than 100 units of anti-TPO level was normal, raised anti-TPO antibodies were found in 12% of normal control group, 61% in type 1 and 42.3% in type 2 diabetics. In type 1 diabetics, the TSH was higher in the younger age patients with progressive rise in anti-TPO antibodies level by age. The TSH, anti-TPO antibodies and BSF were significantly higher in type 1 diabetics than in the controls. In type 2 diabetics, TSH was moderately low but anti-TPO antibodies and BSF were higher than in the controls (author)

  8. Multiparty Asynchronous Session Types

    DEFF Research Database (Denmark)

    Honda, Kohei; Yoshida, Nobuko; Carbone, Marco

    2016-01-01

    . This work extends the foregoing theories of binary session types to multiparty, asynchronous sessions, which often arise in practical communication-centered applications. Presented as a typed calculus for mobile processes, the theory introduces a new notion of types in which interactions involving multiple......Communication is a central elements in software development. As a potential typed foundation for structured communication-centered programming, session types have been studied over the past decade for a wide range of process calculi and programming languages, focusing on binary (two-party) sessions...... peers are directly abstracted as a global scenario. Global types retain the friendly type syntax of binary session types while specifying dependencies and capturing complex causal chains of multiparty asynchronous interactions. A global type plays the role of a shared agreement among communication peers...

  9. Multiparty symmetric sum types

    DEFF Research Database (Denmark)

    Nielsen, Lasse; Yoshida, Nobuko; Honda, Kohei

    2010-01-01

    This paper introduces a new theory of multiparty session types based on symmetric sum types, by which we can type non-deterministic orchestration choice behaviours. While the original branching type in session types can represent a choice made by a single participant and accepted by others...... determining how the session proceeds, the symmetric sum type represents a choice made by agreement among all the participants of a session. Such behaviour can be found in many practical systems, including collaborative workflow in healthcare systems for clinical practice guidelines (CPGs). Processes...... with the symmetric sums can be embedded into the original branching types using conductor processes. We show that this type-driven embedding preserves typability, satisfies semantic soundness and completeness, and meets the encodability criteria adapted to the typed setting. The theory leads to an efficient...

  10. Object-Oriented Type Systems

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Palsberg, Jens

    binding. Existing languages employ different type systems, and it can be difficult to compare, evaluate and improve them, since there is currently no uniform theory for such languages. This book provides such a theory. The authors review the type systems of Simula, Smalltalk, C++ and Eiffel and present......Object-Oriented Type Systems Jens Palsberg and Michael I. Schwartzbach Aarhus University, Denmark Type systems are required to ensure reliability and efficiency of software. For object-oriented languages, typing is an especially challenging problem because of inheritance, assignment, and late...... a type system that generalizes and explains them. The theory is based on an idealized object-oriented language called BOPL (Basic Object Programming Language), containing common features of the above languages. A type system, type inference algorithm, and typings of inheritance and genericity...

  11. Best Proximity Points of Contractive-type and Nonexpansive-type Mappings

    Directory of Open Access Journals (Sweden)

    R. Kavitha

    2018-02-01

    Full Text Available The purpose of this paper is to obtain best proximity point theorems for multivalued nonexpansive-type and contractive-type mappings on complete metric spaces and on certain closed convex subsets of Banach spaces. We obtain a convergence result under some assumptions and we prove the existence of common best proximity points for a sequence of multivalued contractive-type mappings.

  12. Diabetes, Type 1

    OpenAIRE

    Riazi, Afsane; Bradley, Clare

    2007-01-01

    This chapter provides an overview of the role of psychological stress in Type 1 diabetes. Studies relating to stress and Type 1 diabetes onset and control, as well as the evidence relating to stress management training in people with Type 1 diabetes are discussed.

  13. Liver/kidney microsomal antibody type 1 and liver cytosol antibody type 1 concentrations in type 2 autoimmune hepatitis

    OpenAIRE

    Muratori, L; Cataleta, M; Muratori, P; Lenzi, M; Bianchi, F

    1998-01-01

    Background—Liver/kidney microsomal antibody type 1 (LKM1) and liver cytosol antibody type 1 (LC1) are the serological markers of type 2 autoimmune hepatitis (AIH). 
Aims—Since LKM1 and LC1 react against two distinct liver specific autoantigens (cytochrome P450IID6 (CYP2D6) and a 58 kDa cytosolic polypeptide respectively), the aim was to see whether LKM1 and LC1 concentrations correlate with liver disease activity. 
Patients—Twenty one patients with type 2 AIH were studied. 
Methods—A...

  14. Qualification of Type IP-2, Type IP-3 and Type A packages for radioactive liquid shipments

    International Nuclear Information System (INIS)

    Marcu, L.; Sullivan, G.; Lo, K.K.

    2006-01-01

    Commercial products such as pails, drums or bulk containers can be used for radioactive materials transportation if they can be shown to meet the regulatory requirements. Ontario Power Generation (OPG) has successfully tested and qualified several off-the-shelf containers as Type IP-2, Type IP-3 and Type A packages for liquids in accordance with the International and Canadian Regulations. This paper describes the testing and qualification of these commercial products, and discusses the problems encountered and lessons learned during this process. (author)

  15. Relationships between hole type penetrameter sensitivities and wire type penetrameter sensitivities

    International Nuclear Information System (INIS)

    Iwahashi, Y.; Ebata, M.; Ooka, K.; Hirayama, K.; Senda, T.

    1985-01-01

    Radiographic investigations were conducted using the two types of penetrameters (hole and wire types) to obtain the relationships between hole type penetrameter sensitivities and wire type penetrameter sensitivities. Furthermore, the effects of radiation sources and kinds of X-ray films on the relationships were investigated. As a result, the quantitative relationship were defined. It was also clarified that the kinds of X-ray films and radiation sources have a relatively little effect on the relationship

  16. Theoretical models for Type I and Type II supernova

    International Nuclear Information System (INIS)

    Woosley, S.E.; Weaver, T.A.

    1985-01-01

    Recent theoretical progress in understanding the origin and nature of Type I and Type II supernovae is discussed. New Type II presupernova models characterized by a variety of iron core masses at the time of collapse are presented and the sensitivity to the reaction rate 12 C(α,γ) 16 O explained. Stars heavier than about 20 M/sub solar/ must explode by a ''delayed'' mechanism not directly related to the hydrodynamical core bounce and a subset is likely to leave black hole remnants. The isotopic nucleosynthesis expected from these massive stellar explosions is in striking agreement with the sun. Type I supernovae result when an accreting white dwarf undergoes a thermonuclear explosion. The critical role of the velocity of the deflagration front in determining the light curve, spectrum, and, especially, isotopic nucleosynthesis in these models is explored. 76 refs., 8 figs

  17. Typed ψ-calculi

    DEFF Research Database (Denmark)

    Hüttel, Hans

    2011-01-01

    A large variety of process calculi extend the pi-calculus with more general notions of messages. Bengtson et al. have shown that many of these pi-like calculi can be expressed as so-called psi-calculi. In this paper, we describe a simple type system for psi-calculi. The type system satisfies a su...... for the distributed pi-calculus of Hennessy and Riely and finally show how existing type systems for secrecy and authenticity in the spi calculus can be represented and shown to be safe.......A large variety of process calculi extend the pi-calculus with more general notions of messages. Bengtson et al. have shown that many of these pi-like calculi can be expressed as so-called psi-calculi. In this paper, we describe a simple type system for psi-calculi. The type system satisfies...... a subject reduction property and a general notion of channel safety. A number of existing systems are shown to be instances of our system, and other, new type systems can also be obtained. We first present a new type system for the calculus of explicit fusions by Wischik and Gardner, then one...

  18. A Kantorovich-Stancu Type Generalization of Szasz Operators including Brenke Type Polynomials

    Directory of Open Access Journals (Sweden)

    Rabia Aktaş

    2013-01-01

    Full Text Available We introduce a Kantorovich-Stancu type modification of a generalization of Szasz operators defined by means of the Brenke type polynomials and obtain approximation properties of these operators. Also, we give a Voronovskaya type theorem for Kantorovich-Stancu type operators including Gould-Hopper polynomials.

  19. Charting improvements in US registry HLA typing ambiguity using a typing resolution score.

    Science.gov (United States)

    Paunić, Vanja; Gragert, Loren; Schneider, Joel; Müller, Carlheinz; Maiers, Martin

    2016-07-01

    Unrelated stem cell registries have been collecting HLA typing of volunteer bone marrow donors for over 25years. Donor selection for hematopoietic stem cell transplantation is based primarily on matching the alleles of donors and patients at five polymorphic HLA loci. As HLA typing technologies have continually advanced since the beginnings of stem cell transplantation, registries have accrued typings of varied HLA typing ambiguity. We present a new typing resolution score (TRS), based on the likelihood of self-match, that allows the systematic comparison of HLA typings across different methods, data sets and populations. We apply the TRS to chart improvement in HLA typing within the Be The Match Registry of the United States from the initiation of DNA-based HLA typing to the current state of high-resolution typing using next-generation sequencing technologies. In addition, we present a publicly available online tool for evaluation of any given HLA typing. This TRS objectively evaluates HLA typing methods and can help define standards for acceptable recruitment HLA typing. Copyright © 2016 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.

  20. Linearly Refined Session Types

    Directory of Open Access Journals (Sweden)

    Pedro Baltazar

    2012-11-01

    Full Text Available Session types capture precise protocol structure in concurrent programming, but do not specify properties of the exchanged values beyond their basic type. Refinement types are a form of dependent types that can address this limitation, combining types with logical formulae that may refer to program values and can constrain types using arbitrary predicates. We present a pi calculus with assume and assert operations, typed using a session discipline that incorporates refinement formulae written in a fragment of Multiplicative Linear Logic. Our original combination of session and refinement types, together with the well established benefits of linearity, allows very fine-grained specifications of communication protocols in which refinement formulae are treated as logical resources rather than persistent truths.

  1. Object-Oriented Type Systems

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Palsberg, Jens

    a type system that generalizes and explains them. The theory is based on an idealized object-oriented language called BOPL (Basic Object Programming Language), containing common features of the above languages. A type system, type inference algorithm, and typings of inheritance and genericity......Object-Oriented Type Systems Jens Palsberg and Michael I. Schwartzbach Aarhus University, Denmark Type systems are required to ensure reliability and efficiency of software. For object-oriented languages, typing is an especially challenging problem because of inheritance, assignment, and late...... are provided for BOPL. Throughout, the results are related to the languages on which BOPL is based. This text offers advanced undergraduates and professional software developers a sound understanding of the key aspects of object-oriented type systems. All algorithms are implemented in a freely available...

  2. Thinking about gender types: Cognitive organization of female and male types

    NARCIS (Netherlands)

    Vonk, R.; Ashmore, R.D.

    2003-01-01

    We examined the content and dimensional structure of a large and representative sample of gender types. In Study 1, using an open-ended procedure, participants generated 306 different labels for female types (e.g. housewife, feminist, femme fatale, secretary, slob) and 310 for male types (e.g.

  3. Advances in Blood Typing.

    Science.gov (United States)

    Quraishy, N; Sapatnekar, S

    The clinical importance of blood group antigens relates to their ability to evoke immune antibodies that are capable of causing hemolysis. The most important antigens for safe transfusion are ABO and D (Rh), and typing for these antigens is routinely performed for patients awaiting transfusion, prenatal patients, and blood donors. Typing for other blood group antigens, typically of the Kell, Duffy, Kidd, and MNS blood groups, is sometimes necessary, for patients who have, or are likely to develop antibodies to these antigens. The most commonly used typing method is serological typing, based on hemagglutination reactions against specific antisera. This method is generally reliable and practical for routine use, but it has certain drawbacks. In recent years, molecular typing has emerged as an alternative or supplemental typing method. It is based on detecting the polymorphisms and mutations that control the expression of blood group antigens, and using this information to predict the probable antigen type. Molecular typing methods are useful when traditional serological typing methods cannot be used, as when a patient has been transfused and the sample is contaminated with red blood cells from the transfused blood component. Moreover, molecular typing methods can precisely identify clinically significant variant antigens that cannot be distinguished by serological typing; this capability has been exploited for the resolution of typing discrepancies and shows promise for the improved transfusion management of patients with sickle cell anemia. Despite its advantages, molecular typing has certain limitations, and it should be used in conjunction with serological methods. © 2016 Elsevier Inc. All rights reserved.

  4. Wild type measles virus attenuation independent of type I IFN

    Directory of Open Access Journals (Sweden)

    Horvat Branka

    2008-02-01

    Full Text Available Abstract Background Measles virus attenuation has been historically performed by adaptation to cell culture. The current dogma is that attenuated virus strains induce more type I IFN and are more resistant to IFN-induced protection than wild type (wt. Results The adaptation of a measles virus isolate (G954-PBL by 13 passages in Vero cells induced a strong attenuation of this strain in vivo. The adapted virus (G954-V13 differs from its parental strain by only 5 amino acids (4 in P/V/C and 1 in the M gene. While a vaccine strain, Edmonston Zagreb, could replicate equally well in various primate cells, both G954 strains exhibited restriction to the specific cell type used initially for their propagation. Surprisingly, we observed that both G954 strains induced type I IFN, the wt strain inducing even more than the attenuated ones, particularly in human plasmacytoid Dendritic Cells. Type I IFN-induced protection from the infection of both G954 strains depended on the cell type analyzed, being less efficient in the cells used to grow the viral strain. Conclusion Thus, mutations in M and P/V/C proteins can critically affect MV pathogenicity, cellular tropism and lead to virus attenuation without interfering with the α/β IFN system.

  5. Facts about Type 2

    Medline Plus

    Full Text Available ... Care of Type 2 Diabetes This two-page introduction to type 2 diabetes is in PDF format ... Basics Symptoms Type 1 Type 2 Gestational Myths Statistics Common Terms Genetics Living With Diabetes Recently Diagnosed ...

  6. Adherence issues in inherited metabolic disorders treated by low natural protein diets

    DEFF Research Database (Denmark)

    MaCdonald, A; van Rijn, M; Feillet, F

    2012-01-01

    Common inborn errors of metabolism treated by low natural protein diets [amino acid (AA) disorders, organic acidemias and urea cycle disorders] are responsible for a collection of diverse clinical symptoms, each condition presenting at different ages with variable severity. Precursor......-free or essential L-AAs are important in all these conditions. Optimal long-term outcome depends on early diagnosis and good metabolic control, but because of the rarity and severity of conditions, randomized controlled trials are scarce. In all of these disorders, it is commonly described that dietary adherence...... on their neuropsychological profile. There are little data about their ability to self-manage their own diet or the success of any formal educational programs that may have been implemented. Trials conducted in non-phenylketonuria (PKU) patients are rare, and the development of specialist L-AAs for non-PKU AA disorders has...

  7. Diagnosis and management of hypoaldosteronism without hypoadrenocorticism in an alpaca.

    Science.gov (United States)

    Butterworth, Kelly A; Pellegrini-Masini, Alessandra M; Barton, Michelle H

    2012-03-15

    Primary hypoaldosteronism without concurrent hypoadrenocorticism was diagnosed in an 8-year-old female alpaca with acute onset of weakness progressing to recumbency within 6 hours after onset. Hematologic testing at admission revealed profound hyponatremia, hypochloremia, and acidemia with a normal blood potassium concentration. Further diagnostic testing, including an ACTH stimulation test, led to a diagnosis of hypoaldosteronism in conjunction with normal cortisol production. The hembra responded well to i.v. polyionic fluid therapy with sodium supplementation and was managed successfully long term with free access to saline (0.9% NaCl) solution in addition to water ad libitum. To our knowledge, this is the first reported case of hypoaldosteronism in an alpaca. Hypoaldosteronism should be considered in alpacas as a possible differential diagnosis for refractory hyponatremia or for hyponatremia in which an underlying etiology is not determined.

  8. Mastering TypeScript

    CERN Document Server

    Rozentals, Nathan

    2015-01-01

    Whether you are a JavaScript developer aiming to learn TypeScript, or an experienced TypeScript developer wanting to take your skills to the next level, this book is for you. From basic to advanced language constructs, test-driven development, and object-oriented techniques, you will learn how to get the most out of the TypeScript language.

  9. Type Tricks

    DEFF Research Database (Denmark)

    Beier, Sofie

    2017-01-01

    Type Tricks’ is about typographical rules and the underlying structure of the work process in the design of new typefaces. In that way, it is both a reference book and a user manual. In an illustrative format, it presents the different stages of type design in an easily accessible manner. Being...

  10. TypeScript revealed

    CERN Document Server

    Maharry, Dan

    2013-01-01

    TypeScript Revealed is a quick 100-page guide to Anders Hejlsberg's new take on JavaScript. With this brief, fast-paced introduction to TypeScript, .NET, Web and Windows 8 application developers who are already familiar with JavaScript will easily get up to speed with TypeScript and decide whether or not to start incorporating it into their own development. TypeScript is 'JavaScript for Application-scale development'; a superset of JavaScript that brings to it an additional object-oriented-like syntax familiar to .NET programmers that compiles down into simple, clean JavaScript that any browse

  11. Potential biological pathways linking Type-D personality and poor health: A cross-sectional investigation.

    Directory of Open Access Journals (Sweden)

    Vera K Jandackova

    Full Text Available Type-D personality, defined as a combination of high negative affect and high social isolation, has been associated with poor health outcomes. However, pathways underlying this association are largely unknown. We investigated the relationship between Type-D personality and several biological and behavioral pathways including the autonomic nervous system, the immune system, glucose regulation and sleep in a large, apparently healthy sample.Data from a total of 646 respondents (age 41.6±11.5, 12,2% women were available for analysis. Persons with Type-D (negative affect and social isolation score ≥10 were contrasted with those without Type-D. Measures of plasma fibrinogen levels, white blood cell count, high sensitivity C-reactive protein, fasting plasma glucose (FPG, cholesterol, high-density and low-density lipoprotein, glycated hemoglobin (HbA1c, creatinine, triglycerides, and albumin were derived from fasting blood samples. Urine norepinephrine and free cortisol were determined by high-performance liquid chromatography. Time-domain heart rate variability (HRV measures were calculated for the 24hr recording period and for nighttime separately.Persons with Type-D had higher HbA1c, FPG, and fibrinogen, and lower nighttime HRV than those without Type-D, suggesting worse glycemic control, systemic inflammation and poorer autonomic nervous system modulation in Type-D persons. In addition, those with Type-D reported less social support and greater sleep difficulties while no group differences were observed for alcohol and cigarette consumption, physical activity and body mass index.Findings provide some of the first evidence for multiple possible biological and behavioral pathways between Type-D personality and increased morbidity and mortality.

  12. Interspecies radioimmunoassay for the major structural proteins of primate type-D retroviruses

    International Nuclear Information System (INIS)

    Colcher, D.; Teramoto, Y.A.; Schlom, J.

    1977-01-01

    A competition radioimmunoassay has been developed in which type-D retroviruses from three primate species compete. The assay utilizes the major structural protein (36,000 daltons) of the endogenous squirrel monkey retrovirus and antisera directed against the major structural protein (27,000 daltons) of the Mason-Pfizer monkey virus isolated from rhesus monkeys. Purified preparations of both viruses grown in heterologous cells, as well as extracts of heterologous cells infected with squirrel monkey retrovirus or Mason-Pfizer monkey virus, compete completely in the assay. Addition of an endogenous virus of the langur monkey also results in complete blocking. No blocking in the assay is observed with type-C baboon viruses, woolly monkey virus, and gibbon virus. Various other type-C and type-B viruses also showed no reactivity. An interspecies assay has thus been developed that recognizes the type-D retroviruses from both Old World monkey (rhesus and langur) and New World monkey (squirrel) species

  13. Undifferentiated-type gastric adenocarcinoma: prognostic impact of three histological types

    Directory of Open Access Journals (Sweden)

    Lee Han

    2012-11-01

    Full Text Available Abstract Background The prognostic value of the three constituents of undifferentiated-type gastric adenocarcinoma remains unclear. The present study assessed the clinicopathological characteristics and prognosis of undifferentiated-type mucinous adenocarcinoma (uMAC and signet ring cell carcinoma (SRC compared with those of poorly differentiated adenocarcinoma (PDAC. Methods In total, 1,376 patients with undifferentiated-type gastric adenocarcinoma were included, consisting of 1,002 patients diagnosed with PDAC, 54 with uMAC and 320 with SRC. Clinicopathological factors and survival rates were compared among the three histological types. Results Significant differences in the distribution of pathological stages were observed among the groups. Patients with SRC had a significantly better survival rate than those with PDAC or uMAC, in both the all patients including non-curative resected patients and curative-resected groups. In addition, there was significant difference in survival between the PDAC and uMAC groups. Multivariate analysis suggested that age, gender, tumor depth, lymph node metastasis and curability significantly affected survival. Histological type was not an independent prognostic factor. There was no significant difference in the pattern of recurrence among the three groups. Conclusions The uMAC and SRC had worse and favorable prognosis compared with PDCA, respectively. However, there were no differences in survival by pathological stage, thus histological type was not an independent predictor of prognosis.

  14. Web-based depression treatment for type 1 and type 2 diabetic patients

    DEFF Research Database (Denmark)

    van Bastelaar, Kim M P; Pouwer, Francois; Cuijpers, Pim

    2011-01-01

    intervention on a large scale at relatively low costs. This study evaluated the effectiveness of Web-based CBT for depression treatment in adults with type 1 or type 2 diabetes, with minimal guidance. RESEARCH DESIGN AND METHODS: A randomized controlled trial was conducted in the Netherlands in 255 adult...... no beneficial effect on glycemic control (P > 0.05). CONCLUSIONS: Web-based CBT depression treatment is effective in reducing depressive symptoms in adults with type 1 and type 2 diabetes. In addition, the intervention reduces diabetes-specific emotional distress in depressed patients.......OBJECTIVE: Comorbid depression is common in patients with type 1 and type 2 diabetes, adversely affecting quality of life, diabetes outcomes, and mortality. Depression can be effectively treated with cognitive behavior therapy (CBT). The Internet is a new and attractive method for delivering CBT...

  15. US healthcare costs attributable to type A and type B influenza.

    Science.gov (United States)

    Yan, Songkai; Weycker, Derek; Sokolowski, Stefania

    2017-09-02

    While the overall healthcare burden of seasonal influenza in the United States (US) has been well characterized, the proportion of influenza burden attributable to type A and type B illness warrants further elucidation. The aim of this study was to estimate numbers of healthcare encounters and healthcare costs attributable to influenza viral strains A and B in the US during the 2001/2002 - 2008/2009 seasons. Healthcare encounters and costs in the US during the 2001/2002 - 2008/2009 seasons for influenza type A and influenza type B were estimated separately and collectively, by season and age group, based on data from published literature and secondary sources for: rates of influenza-related encounters requiring formal healthcare, unit costs of influenza-related healthcare encounters, and estimates of population size. Across 8 seasons, projected annual numbers of influenza-related healthcare encounters ranged from 11.3-25.6 million, and healthcare costs, from $2.0-$5.8 billion. While the majority of influenza illness was attributable to type A strains, type B strains accounted for 37% of healthcare costs across all seasons, and as much as 66% in a single season. The outpatient burden of type B disease was considerable among persons aged 18-64 y while the hospital cost burden was highest in young children. Influenza viral strain B was associated with considerable health system burden each year during the period of interest. Increasing influenza vaccine coverage, especially with the recently approved quadrivalent products including an additional type B strain, could potentially reduce overall annual influenza burden in the US.

  16. Towards Practical Gradual Typing

    OpenAIRE

    Takikawa, Asumu; Feltey, Daniel; Dean, Earl; Flatt, Matthew; Findler, Robert Bruce; Tobin-Hochstadt, Sam; Felleisen, Matthias

    2015-01-01

    Over the past 20 years, programmers have embraced dynamically-typed programming languages. By now, they have also come to realize that programs in these languages lack reliable type information for software engineering purposes. Gradual typing addresses this problem; it empowers programmers to annotate an existing system with sound type information on a piecemeal basis. This paper presents an implementation of a gradual type system for a full-featured class-based language as well as a novel p...

  17. Weather types in the South Shetlands (Antarctica) using a circulation type approach

    Science.gov (United States)

    Mora, Carla; João Rocha, Maria; Dutra, Emanuel; Trigo, Isabel; Vieira, Gonçalo; Fragoso, Marcelo; Ramos, Miguel

    2010-05-01

    Weather types in the South Shetlands (Antarctica) were defined using an automated method based on the Lamb Weather Type classification scheme (Jones et al. 1993). This is an objective classification originally developed for the British Isles (Jones et al., 1993) and also applied to southeast (Goodess and Palutikof 1998) and northwest Spain (Lorenzo et al, 2009), Portugal (Trigo and DaCamara 2000) and Greece (Maheras et al. 2004) with good results. Daily atmospheric circulation in the South Shetlands region from 1989 to 2009 was classified using a 16-node grid of sea level pressure data from the ERA Interim. The classification is obtained through the comparison of the magnitudes of the directional and rotational components of the geostrophic flow. Basic circulation types were combined into 10 groups of weather types: four directional types (NW, N, S and SW), three anticyclonic types (A, ASW and ANW), and three cyclonic types (C, CSW and CNW). Westerly flow and cyclonic circulation are the most frequent events throughout the year. The sea level pressure field for each weather type is presented and the synoptic characteristics are described. The analysis is based on ERA-Interim fields, including mean sea level pressure, precipitation, cloud cover, humidity and air temperature. Snow thickess modelled using HTESSEL is also considered. Analysis of variance (anova) and multivariate analysis (principal component analysis) are applied to evaluate the characteristics of each weather type. This circulation-type approach showed good results in the past for the downscaling of precipitation in other regions, and we are interested in evaluating the possibilities that the classification offers for downscaling precipitation, but also for snow and air temperature. For this we will be using observational data at test sites in Livingston and Deception islands. We are also motivated by the possibility of using the circulation-type approach as a predictor in statistical downscaling

  18. TypingSuite: Integrated Software for Presenting Stimuli, and Collecting and Analyzing Typing Data

    Science.gov (United States)

    Mazerolle, Erin L.; Marchand, Yannick

    2015-01-01

    Research into typing patterns has broad applications in both psycholinguistics and biometrics (i.e., improving security of computer access via each user's unique typing patterns). We present a new software package, TypingSuite, which can be used for presenting visual and auditory stimuli, collecting typing data, and summarizing and analyzing the…

  19. ClusType: Effective Entity Recognition and Typing by Relation Phrase-Based Clustering

    Science.gov (United States)

    Ren, Xiang; El-Kishky, Ahmed; Wang, Chi; Tao, Fangbo; Voss, Clare R.; Ji, Heng; Han, Jiawei

    2015-01-01

    Entity recognition is an important but challenging research problem. In reality, many text collections are from specific, dynamic, or emerging domains, which poses significant new challenges for entity recognition with increase in name ambiguity and context sparsity, requiring entity detection without domain restriction. In this paper, we investigate entity recognition (ER) with distant-supervision and propose a novel relation phrase-based ER framework, called ClusType, that runs data-driven phrase mining to generate entity mention candidates and relation phrases, and enforces the principle that relation phrases should be softly clustered when propagating type information between their argument entities. Then we predict the type of each entity mention based on the type signatures of its co-occurring relation phrases and the type indicators of its surface name, as computed over the corpus. Specifically, we formulate a joint optimization problem for two tasks, type propagation with relation phrases and multi-view relation phrase clustering. Our experiments on multiple genres—news, Yelp reviews and tweets—demonstrate the effectiveness and robustness of ClusType, with an average of 37% improvement in F1 score over the best compared method. PMID:26705503

  20. Type B Drum packages

    International Nuclear Information System (INIS)

    Edwards, W.S.

    1995-11-01

    The Type B Drum package is a container in which a single drum containing Type B quantities of radioactive material will be packaged for shipment. The Type B Drum containers are being developed to fill a void in the packaging and transportation capabilities of the US Department of Energy (DOE), as no double containment packaging for single drums of Type B radioactive material is currently available. Several multiple-drum containers and shielded casks presently exist. However, the size and weight of these containers present multiple operational challenges for single-drum shipments. The Type B Drum containers will offer one unshielded version and, if needed, two shielded versions, and will provide for the option of either single or double containment. The primary users of the Type B Drum container will be any organization with a need to ship single drums of Type B radioactive material. Those users include laboratories, waste retrieval facilities, emergency response teams, and small facilities

  1. Comparison of diabetic ketoacidosis in patients with type-1 and type-2 diabetes mellitus.

    Science.gov (United States)

    Barski, Leonid; Nevzorov, Roman; Harman-Boehm, Ilana; Jotkowitz, Alan; Rabaev, Elena; Zektser, Miri; Zeller, Lior; Shleyfer, Elena; Almog, Yaniv

    2013-04-01

    Diabetic ketoacidosis (DKA) occurs most often in patients with type 1 diabetes, however patients with type 2 diabetes are also susceptible to DKA under stressful conditions. The aims of our study were to evaluate and compare the clinical and biochemical characteristics and outcomes of type 1 versus type 2 diabetes mellitus (DM) patients with DKA. A retrospective cohort study of adult patients hospitalized with DKA between January 1, 2003, and January 1, 2010. The clinical and biochemical characteristics of DKA patients with type-1 DM were compared with those of patients with type-2 DM. The primary outcome was in-hospital all-cause mortality. The study cohort included 201 consecutive patients for whom the admission diagnosis was DKA: 166 patients (82.6%) with type-1 DM and 35 patients (17.4%) with type-2 DM. The patients with DKA and type-2 DM were significantly older than patients with type-1 DM (64.3 versus 37.3, P ventilation and bed-ridden state were independent predictors of 30-day mortality.

  2. Honesty by Typing

    OpenAIRE

    Bartoletti , Massimo; Scalas , Alceste; Tuosto , Emilio; Zunino , Roberto

    2013-01-01

    We propose a type system for a calculus of contracting processes. Processes can establish sessions by stipulating contracts, and then can interact either by keeping the promises made, or not. Type safety guarantees that a typeable process is honest - that is, it abides by the contracts it has stipulated in all possible contexts, even in presence of dishonest adversaries. Type inference is decidable, and it allows to safely approximate the honesty of processes using either synchronous or async...

  3. Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method

    International Nuclear Information System (INIS)

    Nagamachi, Shigeki; Inoue, Kenjiro; Jinnouchi, Seishi; Hoshi, Hiroaki; Ono, Seiji; Ohnishi, Takashi; Futami, Shigemi; Watanabe, Katsushi; Hayashi, Tohru

    1994-01-01

    Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested. (author)

  4. Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method

    Energy Technology Data Exchange (ETDEWEB)

    Nagamachi, Shigeki; Inoue, Kenjiro; Jinnouchi, Seishi; Hoshi, Hiroaki; Ono, Seiji; Ohnishi, Takashi; Futami, Shigemi; Watanabe, Katsushi; Hayashi, Tohru [Miyazaki Medical Coll., Kiyotake (Japan)

    1994-02-01

    Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested. (author).

  5. Improving Precision of Types

    DEFF Research Database (Denmark)

    Winther, Johnni

    Types in programming languages provide a powerful tool for the programmer to document the code so that a large aspect of the intent can not only be presented to fellow programmers but also be checked automatically by compilers. The precision with which types model the behavior of programs...... is crucial to the quality of these automated checks, and in this thesis we present three different improvements to the precision of types in three different aspects of the Java programming language. First we show how to extend the type system in Java with a new type which enables the detection of unintended...

  6. TypePad For Dummies

    CERN Document Server

    Nelson, Melanie

    2010-01-01

    TypePad for Dummies will be on guiding readers through the technical tasks necessary for starting, using and customizing a TypePad blog. It will comprehensively explain choosing the right TypePad membership plan, singing up, configuring, using and customizing a blog using the TypePad system. It will also include: Illustrations from real-world blogs with screenshots and specific examples that bring the book's concepts and ideas to life.; Step-by-step tutorials that will walk readers through the process of signing up for a new TypePad blog and getting down the business of publishing their conten

  7. Types of Blood Donations

    Science.gov (United States)

    ... Red Cell Plasma Platelets Red Cells What blood donation type is best for me? **If you do ... type, a whole blood donation is recommended** Blood Donation Types: Volunteer Donations The standard or most common ...

  8. Facts about Type 2

    Medline Plus

    Full Text Available ... En Español Type 1 Type 2 About Us Online Community Meal Planning Sign In Search: Search More ... Diagnosing Diabetes and Learning About Prediabetes Type 2 Diabetes Risk Test Lower ...

  9. Behavioral economics survey of patients with type 1 and type 2 diabetes

    Directory of Open Access Journals (Sweden)

    Emoto N

    2015-05-01

    Full Text Available Naoya Emoto,1 Fumitaka Okajima,1 Hitoshi Sugihara,2 Rei Goto3,4 1Division of Endocrinology, Department of Medicine, Nippon Medical School Chiba Hokusoh Hospital, Chiba, 2Department of Endocrinology, Diabetes and Metabolism, Graduate School of Medicine, Nippon Medical School, Tokyo, 3Hakubi Center of Advanced Research, Kyoto University, Kyoto, 4Graduate School of Economics, Kyoto University, Kyoto, Japan Background: Adherence to treatment and the metabolic control of diabetes are challenging in many patients with diabetes. The theory of neuroeconomics can provide important clues for understanding unreasonable human behavior concerning decisions between outcomes occurring at different time points.  Objective: We investigated patients with type 1 and type 2 diabetes to determine whether patients who are at a risk of developing complications are less risk averse. We also examined whether patients with type 1 and type 2 diabetes have different behavioral traits in decision making under risk.  Methods: We conducted a behavioral economics survey of 219 outpatients, 66 with type 1 diabetes and 153 with type 2 diabetes. All patients had been referred by general practitioners or other departments in the hospital. At the time of the survey, levels of hemoglobin A1c were not significantly different between patients with type 1 and type 2 diabetes.  Results: Patients with type 2 diabetes showed a lower response rate to the survey compared with patients with type 1 diabetes (71.9% vs 87.9%, P<0.01. Logistic regression analysis indicated that diabetic retinopathy was negatively associated with risk averse in pricing of hypothetical lotteries, myopic time preference, willingness to pay for preventive medicine, and levels of satisfaction with life. Diabetic nephropathy was also negatively associated with risk averse in pricing of hypothetical lotteries. Detailed analysis revealed that a lower proportion of patients with type 2 diabetes (22.7% were categorized

  10. Human muscle fiber type-specific insulin signaling: Impact of obesity and type 2 diabetes

    DEFF Research Database (Denmark)

    Albers, Peter Hjorth; Pedersen, Andreas J T; Birk, Jesper Bratz

    2015-01-01

    Skeletal muscle is a heterogeneous tissue composed of different fiber types. Studies suggest that insulin-mediated glucose metabolism is different between muscle fiber types. We hypothesized that differences are due to fiber-type specific expression/regulation of insulin signaling elements and....../or metabolic enzymes. Pools of type I and II fibers were prepared from biopsies of the vastus lateralis muscles from lean, obese and type 2 diabetic subjects before and after a hyperinsulinemic-euglycemic clamp. Type I fibers compared to type II fibers have higher protein levels of the insulin receptor, GLUT4......, hexokinase II, glycogen synthase (GS), pyruvate dehydrogenase (PDH-E1α) and a lower protein content of Akt2, TBC1D4 and TBC1D1. In type I fibers compared to type II fibers, the phosphorylation-response to insulin was similar (TBC1D4, TBC1D1 and GS) or decreased (Akt and PDH-E1α). Phosphorylation...

  11. Blood Type Puzzle.

    Science.gov (United States)

    Kelly, Janet

    1997-01-01

    Presents a blood type puzzle that provides a visual, hands-on mechanism by which students can examine blood group reactions. Offers students an opportunity to construct their own knowledge about blood types. (JRH)

  12. Facts about Type 2

    Medline Plus

    Full Text Available ... a Member Donate Now! One Time Monthly In Memory In Honor Become a Member En Español Type 1 Type ... Diagnosing Diabetes and Learning About Prediabetes Type 2 Diabetes Risk Test Lower ...

  13. Facts about Type 2

    Medline Plus

    Full Text Available ... Do I Begin With Type2? Living With Type 1 Diabetes Enroll in the Living WIth Type 2 Diabetes Program In It Together We Can Help ... Tips for Caregivers Health Insurance Health Insurance ...

  14. Involvement of the Cys-Tyr cofactor on iron binding in the active site of human cysteine dioxygenase.

    Science.gov (United States)

    Arjune, Sita; Schwarz, Guenter; Belaidi, Abdel A

    2015-01-01

    Sulfur metabolism has gained increasing medical interest over the last years. In particular, cysteine dioxygenase (CDO) has been recognized as a potential marker in oncology due to its altered gene expression in various cancer types. Human CDO is a non-heme iron-dependent enzyme, which catalyzes the irreversible oxidation of cysteine to cysteine sulfinic acid, which is further metabolized to taurine or pyruvate and sulfate. Several studies have reported a unique post-translational modification of human CDO consisting of a cross-link between cysteine 93 and tyrosine 157 (Cys-Tyr), which increases catalytic efficiency in a substrate-dependent manner. However, the reaction mechanism by which the Cys-Tyr cofactor increases catalytic efficiency remains unclear. In this study, steady-state kinetics were determined for wild type CDO and two different variants being either impaired or saturated with the Cys-Tyr cofactor. Cofactor formation in CDO resulted in an approximately fivefold increase in k cat and tenfold increase in k cat/K m over the cofactor-free CDO variant. Furthermore, iron titration experiments revealed an 18-fold decrease in K d of iron upon cross-link formation. This finding suggests a structural role of the Cys-Tyr cofactor in coordinating the ferrous iron in the active site of CDO in accordance with the previously postulated reaction mechanism of human CDO. Finally, we identified product-based inhibition and α-ketoglutarate and glutarate as CDO inhibitors using a simplified well plate-based activity assay. This assay can be used for high-throughput identification of additional inhibitors, which may contribute to understand the functional importance of CDO in sulfur amino acid metabolism and related diseases.

  15. Industrial systems biology of Saccharomyces cerevisiae enables novel succinic acid cell factory.

    Directory of Open Access Journals (Sweden)

    José Manuel Otero

    Full Text Available Saccharomyces cerevisiae is the most well characterized eukaryote, the preferred microbial cell factory for the largest industrial biotechnology product (bioethanol, and a robust commerically compatible scaffold to be exploitted for diverse chemical production. Succinic acid is a highly sought after added-value chemical for which there is no native pre-disposition for production and accmulation in S. cerevisiae. The genome-scale metabolic network reconstruction of S. cerevisiae enabled in silico gene deletion predictions using an evolutionary programming method to couple biomass and succinate production. Glycine and serine, both essential amino acids required for biomass formation, are formed from both glycolytic and TCA cycle intermediates. Succinate formation results from the isocitrate lyase catalyzed conversion of isocitrate, and from the α-keto-glutarate dehydrogenase catalyzed conversion of α-keto-glutarate. Succinate is subsequently depleted by the succinate dehydrogenase complex. The metabolic engineering strategy identified included deletion of the primary succinate consuming reaction, Sdh3p, and interruption of glycolysis derived serine by deletion of 3-phosphoglycerate dehydrogenase, Ser3p/Ser33p. Pursuing these targets, a multi-gene deletion strain was constructed, and directed evolution with selection used to identify a succinate producing mutant. Physiological characterization coupled with integrated data analysis of transcriptome data in the metabolically engineered strain were used to identify 2(nd-round metabolic engineering targets. The resulting strain represents a 30-fold improvement in succinate titer, and a 43-fold improvement in succinate yield on biomass, with only a 2.8-fold decrease in the specific growth rate compared to the reference strain. Intuitive genetic targets for either over-expression or interruption of succinate producing or consuming pathways, respectively, do not lead to increased succinate. Rather, we

  16. Comparative study T-type and I-type layout of PWR nuclear power plants

    International Nuclear Information System (INIS)

    Eko Rudi Iswanto and Siti Alimah

    2010-01-01

    Determining plant layout is one of the five major stages during the life time of a nuclear power plant. Some important factors that affect in the selecting of plant layout are availability of infrastructure, economic aspects, social aspects, public and environment safety, and also easy to do. Another factor to be considered is requirements as seismic design, which refers to the principles of good security workers, communities and the environment of radiological risks. There are many layout types of nuclear power plant, two of them are T-type layout and I-type layout. Each type of the plant layout has advantage and disadvantage, therefore this study is to understand them. Good layout is able to provide a high level of security against earthquakes. In term of earthquake design, I-type layout has a higher security level than T-type layout. Therefore, I-type layout can be a good choice for PWR nuclear power plants 1000 MWe that will be built in Indonesia. (author)

  17. Facts about Type 2

    Medline Plus

    Full Text Available ... Day Diabetes Basics Home Symptoms Diagnosis America's Diabetes Challenge Type 1 Type 2 Facts About Type 2 ... Planner, and tips from experts Let's Be Well: Products to help you thrive with diabetes - lets-be- ...

  18. Facts about Type 2

    Medline Plus

    Full Text Available ... August 1, 2013 Last Edited: October 27, 2015 Articles from Diabetes Forecast® magazine: lp-type-2, . In this section Diabetes Basics Type 2 Facts About Type 2 Recently Diagnosed Treatment and Care Blood ...

  19. Facts about Type 2

    Medline Plus

    Full Text Available ... Ways to Give Close Are You at Risk? Home Prevention Diagnosing Diabetes and Learning About Prediabetes Type ... To Know Your Risk Alert Day Diabetes Basics Home Symptoms Diagnosis America's Diabetes Challenge Type 1 Type ...

  20. Classification of Parameters Extracted from Cardiotocographic Signals for Early Detection of Metabolic Acidemia in Newborns

    Directory of Open Access Journals (Sweden)

    ROTARIU, C.

    2015-08-01

    Full Text Available Fetal acidosis is reflected by the values of umbilical cord pH and base deficit (BDecf: normal recordings (pH over 7.2 and BDecf under 8 mmol/l and abnormal recordings (pH under 7.2 and BDecf over 8 mmol/l. The purpose of this paper is to present the implementation of an automated system for detecting fetal acidosis in cardiotocographic recordings. The method uses spectral analysis of medium (0.07-0.13 Hz and high (0.13-1 Hz frequency spectrum. We implement the algorithm for segments of the recordings without signal loss for better classification. We determined the normalized medium and high frequency components and mid to high frequency ratio. The recordings in the database are divided into a control group (100 normal recordings and a test group (431 normal or abnormal recordings. A t-test with the p value under 0.05 between the two groups is used to classify the test group. The classification is improved by including the presence of late and prolonged decelerations in the classification process, obtaining the final results, which are comparable to the best ones in current literature.

  1. Types for DSP Assembler Programs

    DEFF Research Database (Denmark)

    Larsen, Ken

    2006-01-01

    for reuse, and a procedure that computes point-wise vector multiplication. The latter uses a common idiom of prefetching memory resulting in out-of-bounds reading from memory. I present two extensions to the baseline type system: The first extension is a simple modification of some type rules to allow out......-ofbounds reading from memory. The second extension is based on two major modifications of the baseline type system: • Abandoning the type-invariance principle of memory locations and using a variation of alias types instead. • Introducing aggregate types, making it possible to have different views of a block...... of memory, thus enabling type checking of programs that directly manage and reuse memory. I show that both the baseline type system and the extended type system can be used to give type annotations to handwritten DSP assembler code, and that these annotations precisely and succinctly describe...

  2. Types for BioAmbients

    Directory of Open Access Journals (Sweden)

    Sara Capecchi

    2010-02-01

    Full Text Available The BioAmbients calculus is a process algebra suitable for representing compartmentalization, molecular localization and movements between compartments. In this paper we enrich this calculus with a static type system classifying each ambient with group types specifying the kind of compartments in which the ambient can stay. The type system ensures that, in a well-typed process, ambients cannot be nested in a way that violates the type hierarchy. Exploiting the information given by the group types, we also extend the operational semantics of BioAmbients with rules signalling errors that may derive from undesired ambients' moves (i.e. merging incompatible tissues. Thus, the signal of errors can help the modeller to detect and locate unwanted situations that may arise in a biological system, and give practical hints on how to avoid the undesired behaviour.

  3. Critical Imperative for the Reform of British Interpretation of Fetal Heart Rate Decelerations: Analysis of FIGO and NICE Guidelines, Post-Truth Foundations, Cognitive Fallacies, Myths and Occam's Razor.

    Science.gov (United States)

    Sholapurkar, Shashikant L

    2017-04-01

    Cardiotocography (CTG) has disappointingly failed to show good predictability for fetal acidemia or neonatal outcomes in several large studies. A complete rethink of CTG interpretation will not be out of place. Fetal heart rate (FHR) decelerations are the most common deviations, benign as well as manifestation of impending fetal hypoxemia/acidemia, much more commonly than FHR baseline or variability. Their specific nomenclature is important (center-stage) because it provides the basic concepts and framework on which the complex "pattern recognition" of CTG interpretation by clinicians depends. Unfortunately, the discrimination of FHR decelerations seems to be muddled since the British obstetrics adopted the concept of vast majority of FHR decelerations being "variable" (cord-compression). With proliferation of confusing waveform criteria, "atypical variables" became the commonest cause of suspicious/pathological CTG. However, National Institute for Health and Care Excellence (NICE) (2014) had to disband the "typical" and "atypical" terminology because of flawed classifying criteria. This analytical review makes a strong case that there are major and fundamental framing and confirmation fallacies (not just biases) in interpretation of FHR decelerations by NICE (2014) and International Federation of Gynecology and Obstetrics (FIGO) (2015), probably the biggest in modern medicine. This "post-truth" approach is incompatible with scientific practice. Moreover, it amounts to setting oneself for failure. The inertia to change could be best described as "backfire effect". There is abundant evidence that head-compression (and other non-hypoxic mediators) causes rapid rather than shallow/gradual decelerations. Currently, the vast majority of decelerations are attributed to unproven cord compression underpinned by flawed disproven pathophysiological hypotheses. Their further discrimination based on abstract, random, trial and error criteria remains unresolved suggesting a

  4. Acceleration and deceleration capacity of fetal heart rate in an in-vivo sheep model.

    Directory of Open Access Journals (Sweden)

    Massimo W Rivolta

    Full Text Available BACKGROUND: Fetal heart rate (FHR variability is an indirect index of fetal autonomic nervous system (ANS integrity. FHR variability analysis in labor fails to detect early hypoxia and acidemia. Phase-rectified signal averaging (PRSA is a new method of complex biological signals analysis that is more resistant to non-stationarities, signal loss and artifacts. It quantifies the average cardiac acceleration and deceleration (AC/DC capacity. OBJECTIVE: The aims of the study were: (1 to investigate AC/DC in ovine fetuses exposed to acute hypoxic-acidemic insult; (2 to explore the relation between AC/DC and acid-base balance; and (3 to evaluate the influence of FHR decelerations and specific PRSA parameters on AC/DC computation. METHODS: Repetitive umbilical cord occlusions (UCOs were applied in 9 pregnant near-term sheep to obtain three phases of MILD, MODERATE, and SEVERE hypoxic-acidemic insult. Acid-base balance was sampled and fetal ECGs continuously recorded. AC/DC were calculated: (1 for a spectrum of T values (T = 1÷50 beats; the parameter limits the range of oscillations detected by PRSA; (2 on entire series of fetal RR intervals or on "stable" series that excluded FHR decelerations caused by UCOs. RESULTS: AC and DC progressively increased with UCOs phases (MILD vs. MODERATE and MODERATE vs. SEVERE, p<0.05 for DC [Formula: see text] = 2-5, and AC [Formula: see text] = 1-3. The time evolution of AC/DC correlated to acid-base balance (0.4<[Formula: see text]<0.9, p<0.05 with the highest [Formula: see text] for [Formula: see text]. PRSA was not independent from FHR decelerations caused by UCOs. CONCLUSIONS: This is the first in-vivo evaluation of PRSA on FHR analysis. In the presence of acute hypoxic-acidemia we found increasing values of AC/DC suggesting an activation of ANS. This correlation was strongest on time scale dominated by parasympathetic modulations. We identified the best performing [Formula: see text] parameters

  5. Facts about Type 2

    Medline Plus

    Full Text Available ... Home Prevention Diagnosing Diabetes and Learning About Prediabetes Type 2 Diabetes Risk Test Lower Your Risk Healthy Eating Overweight ... About Type 2 Enroll in the Living WIth Type 2 Diabetes Program Gestational What is Gestational Diabetes? How to ...

  6. Analog insulin detemir for patients with type 1 and type 2 diabetes: a review

    Directory of Open Access Journals (Sweden)

    Gregory E Peterson

    2009-05-01

    Full Text Available Gregory E PetersonDepartment of Internal Medicine, Des Moines University, USAObjective: To review insulin detemir for clinical use to better manage patients with type 1 and type 2 diabetes.Methods: A MEDLINE search, in English, from June 30, 2006 to December 1, 2008, using the terms “insulin analogs,” “insulin detemir” and “long-acting insulin analog.”Results: Insulin detemir improves glycemic control, based on HbA1C reduction and fasting glucose levels, without increasing the risk of hypoglycemia and weight gain. Insulin detemir has lower glycemic variability, with less intra-subject variability in blood glucose levels in patients with type 1 and type 2 diabetes, without increasing the risk of hypoglycemia. When added to oral anti-diabetes agents (OADs in type 2 diabetes, insulin detemir demonstrates superiority to other basal insulin options.Conclusion: Insulin detemir appears to provide better glycemic control with a lower risk of hypoglycemia and less weight gain in the treatment of patients with type 1 and type 2 diabetes.Keywords: type 1 diabetes, type 2 diabetes, insulin analogs, insulin detemir

  7. Types of chemotherapy

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/patientinstructions/000910.htm Types of chemotherapy To use the sharing features on this page, ... cancer.org/treatment/treatments-and-side-effects/treatment-types/chemotherapy/how-chemotherapy-drugs-work.html . Updated February 15, ...

  8. Capital cost evaluation of liquid metal reactor by plant type - comparison of modular type with monolithic type -

    International Nuclear Information System (INIS)

    Mun, K. H.; Seok, S. D.; Song, K. D.; Kim, I. C.

    1999-01-01

    A preliminary economic comparison study was performed for KALIMER(Korea Advanced LIquid MEtal Reactor)between a modular plant type with 8 150MWe modules and a 1200MWe monolithic plant type. In both cases of FOAK (First-Of-A-Kind) Plant and NOAK (Nth-Of-A-Kind) Plant, the result says that the economics of monolithic plant is superior to its modular plant. In case of NOAK plant comparison, however, the cost difference is not significant. It means that modular plant can compete with monolithic plant in capital cost if it makes efforts of cost reduction and technical progress on the assumption that the same type of NOAK plant will be constructed continuously

  9. The nurses role in preventing type 2 diabetes mellitus type 2

    OpenAIRE

    Kittilsen, Jannike Granum; Lerfaldet, Hanne Nyhus; Norhaug, Anikken Thomassen; Tangen, Emmy Helene

    2016-01-01

    Innledning: Diabetes mellitus type 2 er et økende problem både i Norge og internasjonalt. Forekomsten av diabetes mellitus type 2 er firedoblet fra 1980 til 2014. Hensikt: Undersøke hvordan livsstilsendringer kan forebygge utviklingen av diabetes mellitus type 2, og hva sykepleiers rolle er i forebyggingen. Metode: Det har blitt benyttet litteraturstudie som metode. Systematiske søk ble gjort i databasene: Cinahl Complete, Ovid Nursing Database og Medline. Resultat: Gjennom systematisk søk er...

  10. The Role of MIF in Type 1 and Type 2 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Yuriko I. Sánchez-Zamora

    2014-01-01

    Full Text Available Autoimmunity and chronic low-grade inflammation are hallmarks of diabetes mellitus type one (T1DM and type two (T2DM, respectively. Both processes are orchestrated by inflammatory cytokines, including the macrophage migration inhibitory factor (MIF. To date, MIF has been implicated in both types of diabetes; therefore, understanding the role of MIF could affect our understanding of the autoimmune or inflammatory responses that influence diabetic pathology. This review highlights our current knowledge about the involvement of MIF in both types of diabetes in the clinical environment and in experimental disease models.

  11. Harnessing type I and type III CRISPR-Cas systems for genome editing

    DEFF Research Database (Denmark)

    Li, Yingjun; Pan, Saifu; Zhang, Yan

    2016-01-01

    CRISPR-Cas (clustered regularly interspaced short palindromic repeats-CRISPR-associated) systems are widespread in archaea and bacteria, and research on their molecular mechanisms has led to the development of genome-editing techniques based on a few Type II systems. However, there has not been any...... report on harnessing a Type I or Type III system for genome editing. Here, a method was developed to repurpose both CRISPR-Cas systems for genetic manipulation in Sulfolobus islandicus, a thermophilic archaeon. A novel type of genome-editing plasmid (pGE) was constructed, carrying an artificial mini-CRISPR...... and selectively retained as transformants. Using this strategy, different types of mutation were generated, including deletion, insertion and point mutations. We envision this method is readily applicable to different bacteria and archaea that carry an active CRISPR-Cas system of DNA interference provided...

  12. The brass-type texture and its deviation from the copper-type texture

    DEFF Research Database (Denmark)

    Leffers, Torben; Ray, R.K.

    2009-01-01

    Our basic aim with the present review is to address the classical problem of the “fcc rolling texture transition” – the fact that fcc materials may, depending on material parameters and rolling conditions, develop two different types of rolling textures, the copper-type texture and the brass...... the subject and sketch our approach for dealing with it. We then recapitulate the decisive progress made during the nineteen sixties in the empirical description of the fcc rolling texture transition and in lining up a number of possible explanations. Then follows a section about experimental investigations...... of the brass-type texture after the nineteen sixties covering texture measurements and microstructural investigations. The main observations are: (1) The brass-type texture deviates from the copper-type texture from an early stage of texture development. (2) Deformation twinning has a decisive effect...

  13. Suicidal ideation reported by adults with Type 1 or Type 2 diabetes

    DEFF Research Database (Denmark)

    Handley, T E; Ventura, A D; Browne, Jessica L

    2016-01-01

    factor for a suicide attempt, the findings have implications for healthcare professionals, pointing to the importance of adequate screening and action plans for appropriate follow-up of those reporting depression. Our findings are also indicative of the psychological toll of diabetes more generally......AIMS: To examine the prevalence and correlates of suicidal ideation (SI) in a community-based sample of adults with Type 1 or Type 2 diabetes. METHODS: Participants were 3338 adults aged 18-70 years with Type 1 diabetes (n = 1376) or Type 2 diabetes (non-insulin: n = 1238; insulin: n = 724) from...... a national survey administered to a random sample registered with the National Diabetes Services Scheme. Depression and SI were assessed using the Patient Health Questionnaire, and diabetes-specific distress with the Problem Areas In Diabetes scale. Separate logistic regression analyses by diabetes type...

  14. Type 1 diabetes

    DEFF Research Database (Denmark)

    Green, Anders; Kyvik, Kirsten Ohm

    2001-01-01

    Prediction of Type 1 diabetes at individual level is relevant for any possible intervention before clinical disease develops. Currently available markers of Type 1 diabetes include genetic specificities and immune markers, in addition to a positive family history. This chapter reviews the measures...... and methods of importance in predicting Type 1 diabetes. Based on numerical examples it is demonstrated that available markers have a low level of performance, even when combined. Even so, combined marker information may allow for the identification of the large majority of the general population who...... is at very low disease risk. The impact at population level of predicting Type 1 diabetes varies between societies because the performance of markers depends on levels of disease risk and distribution of markers within a population. The incorporation of the influence of non-genetic etiological factors may...

  15. Maternal and Neonatal Outcomes in Korean Women with Type 1 and Type 2 Diabetes

    Directory of Open Access Journals (Sweden)

    Hee-Sook Kim

    2015-08-01

    Full Text Available BackgroundThe purpose of this study was to evaluate maternal and neonatal outcomes in Korean women with type 1 diabetes and type 2 diabetes.MethodsWe performed a retrospective survey of 163 pregnancies in women with type 1 diabetes (n=13 and type 2 diabetes (n=150 treated from 2003 to 2010 at Cheil General Hospital & Women's Healthcare Center, Korea. We compared maternal characteristics as well as maternal and neonatal outcomes between groups.ResultsDifferences in glycosylated hemoglobin between type 1 and type 2 diabetes were not significant. Birth weight (3,501±689.6 g vs. 3,366±531.4 g and rate of major congenital malformations (7.7% vs. 5.6% were not significantly different. However, women with type 1 diabetes had higher rates of preeclampsia (38.5% vs. 8.2%, P=0.006, large for gestational age (LGA; 46.2% vs. 20.4%, P=0.004, macrosomia (38.5% vs. 13.4%, P=0.032, and admission for neonatal care (41.7% vs. 14.8%, P=0.03 than women with type 2 diabetes.ConclusionMaternal and neonatal outcomes for women with type 1 diabetes were poorer than for women with type 2 diabetes, especially preeclampsia, LGA, macrosomia and admission to the neonatal intensive care unit.

  16. Scintigraphic Evaluation of Multiple Endocrine Neoplasia Type 2 ( MEN Type 2 )

    International Nuclear Information System (INIS)

    Lee, Jae Tae; Lee, Kyu Bo; Whang, Kee Suk; Kim, Bo Wan; Lee, In Kyu

    1991-01-01

    Multiple endocrine neoplasia type 2 (MEN type 2, Sipple's syndrome) is a rare disorder characterized by the association of medullary carcinoma of the thyroid, parathyroid hyperplasia and can be diagnosed in early stage of the disease by meticulous screening tests of the family. This case report describes the location and categorization of tumors using 99m Tc-pertechnetate, I -NaI, 99m Tc-pentavalent(V) DMSA, 131 I -MIBG scans in two cases of MEN type 2 occurred in a 32 year old women and her 29-year old brother. In MEN type 2, we think, combined use of 99m Tc-(V)-DMSA, 99m Tc-pertechnetate and 1 '3 1 I -MIBG may be useful for the categorization of tumor mass lesions and planning appropriate therapy.

  17. Single-channel L-type Ca2+ currents in chicken embryo semicircular canal type I and type II hair cells.

    Science.gov (United States)

    Zampini, Valeria; Valli, Paolo; Zucca, Giampiero; Masetto, Sergio

    2006-08-01

    Few data are available concerning single Ca channel properties in inner ear hair cells and particularly none in vestibular type I hair cells. By using the cell-attached configuration of the patch-clamp technique in combination with the semicircular canal crista slice preparation, we determined the elementary properties of voltage-dependent Ca channels in chicken embryo type I and type II hair cells. The pipette solutions included Bay K 8644. With 70 mM Ba(2+) in the patch pipette, Ca channel activity appeared as very brief openings at -60 mV. Ca channel properties were found to be similar in type I and type II hair cells; therefore data were pooled. The mean inward current amplitude was -1.3 +/- 0.1 (SD) pA at - 30 mV (n = 16). The average slope conductance was 21 pS (n = 20). With 5 mM Ba(2+) in the patch pipette, very brief openings were already detectable at -80 mV. The mean inward current amplitude was -0.7 +/- 0.2 pA at -40 mV (n = 9). The average slope conductance was 11 pS (n = 9). The mean open time and the open probability increased significantly with depolarization. Ca channel activity was still present and unaffected when omega-agatoxin IVA (2 microM) and omega-conotoxin GVIA (3.2 microM) were added to the pipette solution. Our results show that types I and II hair cells express L-type Ca channels with similar properties. Moreover, they suggest that in vivo Ca(2+) influx might occur at membrane voltages more negative than -60 mV.

  18. Types of Anesthesia

    Science.gov (United States)

    ... Videos for Educators Search English Español Types of Anesthesia KidsHealth / For Teens / Types of Anesthesia What's in ... Get? Print en español Tipos de anestesia About Anesthesia Anesthesia is broken down into three main categories: ...

  19. Evaluation of microwave oven heating for prediction of drug-excipient compatibilities and accelerated stability studies

    DEFF Research Database (Denmark)

    Schou-Pedersen, Anne Marie V; Østergaard, Jesper; Cornett, Claus

    2015-01-01

    , if a microwave oven is applicable for accelerated drug stability testing. Chemical interactions were investigated in three selected model formulations of drug and excipients regarding the formation of ester and amide reaction products. The accelerated stability studies performed in the microwave oven using...... a design of experiments (DoE) approach in order to be able to rank excipients regarding reactivity: Study A: cetirizine with PEG 400, sorbitol, glycerol and propylene glycol. Study B: 6-aminocaproic acid with citrate, acetate, tartrate and gluconate. Study C: atenolol with citric, tartaric, malic, glutaric......, and sorbic acid. The model formulations were representative for oral solutions (co-solvents), parenteral solutions (buffer species) and solid dosage forms (organic acids applicable for solubility enhancement). The DoE studies showed overall that the same impurities were generated by microwave oven heating...

  20. Facts about Type 2

    Medline Plus

    Full Text Available ... from Diabetes Forecast® magazine: lp-type-2, . In this section Diabetes Basics Type 2 Facts About Type ... ensureArray(data.submitSurveyResponse.errors); $.each(surveyErrors, function () { if (this.errorField) { $('input[name="' + this.errorField + '"]').closest('.form-group') . ...

  1. Facts about Type 2

    Medline Plus

    Full Text Available ... or insulin to help you meet your target blood glucose levels. Type 2 usually gets worse over time – even if ... About Type 2 Recently Diagnosed Treatment and Care Blood Glucose ... Living With Type 2 Diabetes Program donate en -- A Future Without ...

  2. Diabetes Type 1

    Science.gov (United States)

    Diabetes means your blood glucose, or blood sugar, levels are too high. With type 1 diabetes, your pancreas does not make insulin. Insulin is ... kidneys, nerves, and gums and teeth. Type 1 diabetes happens most often in children and young adults ...

  3. Liquid-type Botulinum Toxin Type A in Adductor Spasmodic Dysphonia: A Prospective Pilot Study.

    Science.gov (United States)

    Cha, Wonjae; Jang, Jeon Yeob; Wang, Soo-Geun; Kang, Ji-Heon; Jo, Min-Gyu

    2017-05-01

    Botulinum toxin (BTX) has been widely used to treat adductor spasmodic dysphonia (ADSD). Most commercially available forms of BTX require reconstitution before use, which may increase the risk of contamination and requires careful titration. Recently, a liquid-type BTX type A (BTX-A) has been developed, which should simplify the procedure and enhance its efficacy. Herein, we present a prospective pilot study to investigate the efficacy and safety of liquid-type BTX-A in the treatment of ADSD. Twenty-six consecutive liquid-type BTX-A injections were performed in 12 patients with ADSD. We included as a control group 34 consecutive patients with ADSD who had previously undergone 52 vocal fold injection procedures with freeze-dried-type BTX-A. All patients in both groups had improvement of symptoms related to ADSD and period of normal voice. Most patients experienced breathiness, and the onset time, the peak response time, and the duration of breathiness were similar in both groups. The duration of effect (days) was 96.96 ± 18.91 and 77.38 ± 18.97 in the freeze-dried-type and the liquid-type groups, and the duration of benefit (days) was 80.02 ± 18.24 and 62.69 ± 19.73 in the freeze-dried-type and the liquid-type groups. To compare the efficacy between the freeze-dried-type and the liquid-type BTX-A, the sessions of the unilateral vocal fold injection were included and were categorized as group A (1 ~ 2 units BTX-A) and group B (2 ~ 3 units BTX-A), according to the dose per vocal fold. There was no significant difference of effect time between freeze-dried-type and liquid-type BTX-A groups. No adverse events related to BTX or vocal fold injection were reported. Liquid-type BTX-A is safe and effective for the treatment of spasmodic dysphonia. With the advantages of simple preparation, storage, and reuse and animal protein-free constituents, liquid-type BTX-A may be a good option in the treatment of spasmodic dysphonia. Copyright © 2017 The

  4. Pediatric obesity & type 2 diabetes.

    Science.gov (United States)

    Dea, Tara L

    2011-01-01

    This article focuses on (a) identifying obesity and other risk factors for developing type 2 diabetes, (b) differentiating between pediatric type 1 diabetes and type 2 diabetes, and (c) treating pediatric type 2 diabetes. Obesity has significant implications on a child's health, including an increased risk for insulin resistance and progression to type 2 diabetes. Type 2 diabetes in children, characterized by insulin resistance and relative pancreatic b-cell failure due to the increased demand for insulin production, has now reached epidemic proportions. Longitudinal research on pediatric type 2 diabetes, however, is lacking because this epidemic is relatively new. Treatment of type 2 diabetes in children is focused on lifestyle modification with weight management/increased physical activity, and pharmacological management through oral medication or insulin therapy. Because children with type 2 diabetes are at risk for developing diabetes-related complications earlier in life, they need to be closely monitored for comorbidities.

  5. Subtypes of batterers in treatment: empirical support for a distinction between type I, type II and type III.

    Directory of Open Access Journals (Sweden)

    José Luis Graña

    Full Text Available This study explores the existence of different types of batterers in a sample of 266 men who had been court referred for intimate partner violence. The data collected in the assessment that have been used to perform a hierarchical and a two-step cluster analysis fall into three areas: aggression towards the partner, general aggression and presence of psychopathology and personality traits, more specifically, alcohol use, borderline and antisocial personality traits, psychopathy traits, state anger and trait anger, anger expression and control, anger, hostility, and, finally, impulsivity. The results show a typology consisting of 3 types of batterers on the basis of violence level and psychopathology: low (65%, moderate (27.8% and high (7.1%. This study provides empirical support for the development of batterer typologies. These typologies will help achieve early detection of different types of batterers, allowing us to tailor interventions on the basis of the needs of each of the types.

  6. Facts about Type 2

    Medline Plus

    Full Text Available ... or insulin to help you meet your target blood glucose levels. Type 2 usually gets worse over time – even if ... About Type 2 Recently Diagnosed Treatment and Care Blood Glucose ... the Living With Type 2 Diabetes Program donate en -- A Future Without ...

  7. Fetal plasma erythropoietin concentration in severe growth retardation.

    Science.gov (United States)

    Snijders, R J; Abbas, A; Melby, O; Ireland, R M; Nicolaides, K H

    1993-02-01

    The aim of this study was to determine whether hypoxemia induces an increase in plasma erythropoietin concentration in human fetal life and, if so, whether this response stimulates fetal erythropoiesis. The plasma erythropoietin concentration in blood samples from 33 small-for-gestational-age fetuses at 26 to 38 weeks' gestation was measured. Measurements were compared with the reference range for gestation, and associations with PO2, pH, and erythroblast and erythrocyte counts were examined. The mean plasma erythropoietin concentration in the small-for-gestational-age fetuses was significantly increased, and the degree of increase was significantly associated both with fetal acidemia and, more strongly, with fetal erythroblastosis. Erythropoietin production in response to tissue hypoxia occurs from at least 26 weeks' gestation with measurable physiologic effects on erythropoiesis. Furthermore, more accurate assessment of tissue oxygenation may be obtained by measuring the erythroblast count rather than the blood pH.

  8. Coevolution of patch-type dependent emigration and patch-type dependent immigration.

    Science.gov (United States)

    Weigang, Helene C

    2017-08-07

    The three phases of dispersal - emigration, transfer and immigration - are affecting each other and the former and latter decisions may depend on patch types. Despite the inevitable fact of the complexity of the dispersal process, patch-type dependencies of dispersal decisions modelled as emigration and immigration are usually missing in theoretical dispersal models. Here, I investigate the coevolution of patch-type dependent emigration and patch-type dependent immigration in an extended Hamilton-May model. The dispersing population inhabits a landscape structured into many patches of two types and disperses during a continuous-time season. The trait under consideration is a four dimensional vector consisting of two values for emigration probability from the patches and two values for immigration probability into the patches of each type. Using the adaptive dynamics approach I show that four qualitatively different dispersal strategies may evolve in different parameter regions, including a counterintuitive strategy, where patches of one type are fully dispersed from (emigration probability is one) but individuals nevertheless always immigrate into them during the dispersal season (immigration probability is one). I present examples of evolutionary branching in a wide parameter range, when the patches with high local death rate during the dispersal season guarantee a high expected disperser output. I find that two dispersal strategies can coexist after evolutionary branching: a strategy with full immigration only into the patches with high expected disperser output coexists with a strategy that immigrates into any patch. Stochastic simulations agree with the numerical predictions. Since evolutionary branching is also found when immigration evolves alone, the present study is adding coevolutionary constraints on the emigration traits and hence finds that the coevolution of a higher dimensional trait sometimes hinders evolutionary diversification. Copyright © 2017

  9. Comparison of fluoroscopy-guided pull-type percutaneous radiological gastrostomy (pull-type-PRG) with conventional percutaneous radiological gastrostomy (push-type-PRG): clinical results in 253 patients

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Yang; Schneider, J.; Dueber, C.; Pitton, M.B. [University Medical Center of the Johannes Gutenberg University Mainz, Department of Diagnostic and Interventional Radiology, Mainz (Germany)

    2011-11-15

    To analyze the clinical results and complications of fluoroscopy guided internal-external pull-type percutaneous radiological gastrostomy (pull-type-PRG) and conventional external-internal percutaneous radiological gastrostomy (push-type-PRG). A total of 253 patients underwent radiological gastrostomy between January 2002 and January 2010. Data were collected retrospectively from radiology reports, Chart review of clinical notes, procedure reports, discharge summaries and subsequent hospital visits. Statistical analysis was performed to compare the two methods for gastrostomy with respect to peri-interventional aspects and clinical results. 128 patients received the Pull-type-PRG whereas the other 125 patients were served with the Push-type-PRG. Indications for gastrostomy were similar in these two groups. The most frequent indications for the both methods were stenotic oesophageal tumors or head/neck tumors (54.7% in pull-type-PRG, 68% in push-type-PRG). Gastrostomy procedures were successful in 98.3% in pull-type-PRG compared to 92% in push-type-PRG. There was no procedure-related mortality. Compared to Push-type-PRG, the peri-interventional complication rate was significantly reduced in pull-type-PRG (14.8% versus 34.4%, P = 0.002). Compared to the external-internal push-type-PRG, the internal-external Pull-type-PRG showed a high primary success rate and a decreased incidence of peri-interventional complications. (orig.)

  10. Comparison of fluoroscopy-guided pull-type percutaneous radiological gastrostomy (pull-type-PRG) with conventional percutaneous radiological gastrostomy (push-type-PRG): clinical results in 253 patients

    International Nuclear Information System (INIS)

    Yang, Yang; Schneider, J.; Dueber, C.; Pitton, M.B.

    2011-01-01

    To analyze the clinical results and complications of fluoroscopy guided internal-external pull-type percutaneous radiological gastrostomy (pull-type-PRG) and conventional external-internal percutaneous radiological gastrostomy (push-type-PRG). A total of 253 patients underwent radiological gastrostomy between January 2002 and January 2010. Data were collected retrospectively from radiology reports, Chart review of clinical notes, procedure reports, discharge summaries and subsequent hospital visits. Statistical analysis was performed to compare the two methods for gastrostomy with respect to peri-interventional aspects and clinical results. 128 patients received the Pull-type-PRG whereas the other 125 patients were served with the Push-type-PRG. Indications for gastrostomy were similar in these two groups. The most frequent indications for the both methods were stenotic oesophageal tumors or head/neck tumors (54.7% in pull-type-PRG, 68% in push-type-PRG). Gastrostomy procedures were successful in 98.3% in pull-type-PRG compared to 92% in push-type-PRG. There was no procedure-related mortality. Compared to Push-type-PRG, the peri-interventional complication rate was significantly reduced in pull-type-PRG (14.8% versus 34.4%, P = 0.002). Compared to the external-internal push-type-PRG, the internal-external Pull-type-PRG showed a high primary success rate and a decreased incidence of peri-interventional complications. (orig.)

  11. Type Systems for Bigraphs

    DEFF Research Database (Denmark)

    Elsborg, Ebbe; Hildebrandt, Thomas; Sangiorgi, Davide

    of controls and a set of reaction rules, collectively a bigraphical reactive system (BRS). Possible advantages of developing bigraphical type systems include: a deeper understanding of a type system itself and its properties; transfer of the type systems to the concrete family of calculi that the BRS models...

  12. Session Types at the Mirror

    Directory of Open Access Journals (Sweden)

    Luca Padovani

    2009-12-01

    Full Text Available We (redefine session types as projections of process behaviors with respect to the communication channels they use. In this setting, we give session types a semantics based on fair testing. The outcome is a unified theory of behavioral types that shares common aspects with conversation types and that encompass features of both dyadic and multi-party session types. The point of view we provide sheds light on the nature of session types and gives us a chance to reason about them in a framework where every notion, from well-typedness to the subtyping relation between session types, is semantically -rather than syntactically- grounded.

  13. Personality types of entrepreneurs.

    Science.gov (United States)

    Müller, Günter F; Gappisch, Cathrin

    2005-06-01

    85 German entrepreneurs were psychometrically assessed on 12 primary trait characteristics. The sample consisted of 49 men and 36 women whose mean age was 45.6 yr. (SD= 10.3). Occupational domains were production (40%) and services (60%). The mean duration of entrepreneurship within these domains was 13.1 yr. (SD=9.3). By factor analysis five personality types of entrepreneurs could be identified: Creative Acquisitor, Controlled Perseverator, Distant Achiever, Rational Manager, and Egocentric Agitator. These types correspond with types found in research by Miner and with the Myer-Briggs Indicator. In addition, correlations between general type potential and both job and life satisfaction of entrepreneurs were found. The results are discussed with regard to intercultural stability of personality types and implications for research and application.

  14. TypeSan : Practical type confusion detection

    NARCIS (Netherlands)

    Haller, Istvan; Jeon, Yuseok; Peng, Hui; Payer, Mathias; Giuffrida, Cristiano; Bos, Herbert; Van Der Kouwe, Erik

    2016-01-01

    The low-level C++ programming language is ubiquitously used for its modularity and performance. Typecasting is a fundamental concept in C++ (and object-oriented programming in general) to convert a pointer from one object type into another. However, downcasting (converting a base class pointer to a

  15. A Kantorovich Type of Szasz Operators Including Brenke-Type Polynomials

    Directory of Open Access Journals (Sweden)

    Fatma Taşdelen

    2012-01-01

    convergence properties of these operators by using Korovkin's theorem. We also present the order of convergence with the help of a classical approach, the second modulus of continuity, and Peetre's -functional. Furthermore, an example of Kantorovich type of the operators including Gould-Hopper polynomials is presented and Voronovskaya-type result is given for these operators including Gould-Hopper polynomials.

  16. Electrothermally-Actuated Micromirrors with Bimorph Actuators—Bending-Type and Torsion-Type

    Directory of Open Access Journals (Sweden)

    Cheng-Hua Tsai

    2015-06-01

    Full Text Available Three different electrothermally-actuated MEMS micromirrors with Cr/Au-Si bimorph actuators are proposed. The devices are fabricated with the SOIMUMPs process developed by MEMSCAP, Inc. (Durham, NC, USA. A silicon-on-insulator MEMS process has been employed for the fabrication of these micromirrors. Electrothermal actuation has achieved a large angular movement in the micromirrors. Application of an external electric current 0.04 A to the bending-type, restricted-torsion-type, and free-torsion-type mirrors achieved rotation angles of 1.69°, 3.28°, and 3.64°, respectively.

  17. Parasitic helminths and their beneficial impact on type 1 and type 2 diabetes.

    Science.gov (United States)

    Berbudi, Afiat; Ajendra, Jesuthas; Wardani, Ajeng P F; Hoerauf, Achim; Hübner, Marc P

    2016-03-01

    It is estimated that by the year 2035 almost 600 million people will suffer from diabetes. In the case of type 2 diabetes, the strongest increase of diabetes incidence occurs in developing and newly industrialized countries. This increase correlates not only with a progressing sedentary lifestyle and nutritional changes, but also environmental changes. Similarly, the increase of type 1 diabetes incidence in industrialized countries over the past decades cannot be explained by genetic factors alone, suggesting that environmental changes are also involved. One such environmental change is a reduced exposure to pathogens because of improved hygiene. Parasitic helminths modulate the immune system of their hosts and induce type 2 as well as regulatory immune responses. As pro-inflammatory immune responses are crucial for the onset of both type 1 and type 2 diabetes, helminth-induced immunomodulation may prevent diabetes onset and ameliorate insulin sensitivity. Several epidemiological studies in human and experimental animal models support such a protective effect of helminths for autoimmune diabetes. Recent studies further suggest that helminths may also provide such a beneficial effect for type 2 diabetes. In this review we summarize studies that investigated parasitic helminths and helminth-derived products and their impact on both type 1 and type 2 diabetes highlighting potential protective mechanisms. Copyright © 2015 John Wiley & Sons, Ltd.

  18. Rust types from JSON samples - Approximating type providers with procedural macros in Rust

    OpenAIRE

    Vesteraas, Erik Andreas

    2017-01-01

    When programmers access external data in a statically typed programming language, they are often faced with a dilemma between convenient and type-safe access to the data. In the programming language F#, a concept called type providers has been proposed as a solution to this problem by having compiler support for libraries with the capability to generate types at compile time. This thesis presents "json_typegen", a project which aims to show the feasibility of similar solutions in the Rust pro...

  19. Diabetes mellitus type 1

    OpenAIRE

    Desta, Semere Tekeste

    2017-01-01

    Høgskulen på Vestlandet Avdeling for helsefag for sykepleiere Tittel: Diabetes type 1 Bakgrunn for val av tema: I 2000 var det ca. 130 000 personer i Norge med diabetes, av disse hadde ca. 20 000 diabetes type 1. I dag er det ca. 230 000 personer som har diabetes i Norge. Av disse ca. 28 000 type 1 diabetes. Tallet viser hvor alvorlig sykdommen er, fordi det har vært og fortsatt er, en økning av antall diabetikere i Norge. Type 1 diabetes kan komme i alle aldersgrupper, men vanligvis...

  20. Type Ia Supernova Cosmology

    Science.gov (United States)

    Leibundgut, B.; Sullivan, M.

    2018-03-01

    The primary agent for Type Ia supernova cosmology is the uniformity of their appearance. We present the current status, achievements and uncertainties. The Hubble constant and the expansion history of the universe are key measurements provided by Type Ia supernovae. They were also instrumental in showing time dilation, which is a direct observational signature of expansion. Connections to explosion physics are made in the context of potential improvements of the quality of Type Ia supernovae as distance indicators. The coming years will see large efforts to use Type Ia supernovae to characterise dark energy.

  1. Type-safe pattern combinators

    DEFF Research Database (Denmark)

    Rhiger, Morten

    2009-01-01

    Macros still haven't made their way into typed higher-order programming languages such as Haskell and Standard ML. Therefore, to extend the expressiveness of Haskell or Standard ML, one must express new linguistic features in terms of functions that fit within the static type systems of these lan...... of these languages. This is particularly challenging when introducing features that span across multiple types and that bind variables. We address this challenge by developing, in a step by step manner, mechanisms for encoding patterns and pattern matching in Haskell in a type-safe way....

  2. Type-D personality is a stable taxonomy in post-MI patients over an 18-month period

    DEFF Research Database (Denmark)

    Martens, Elisabeth J; Kupper, Nina; Pedersen, Susanne S.

    2007-01-01

    type-D personality comprises a risk factor for adverse prognosis in patients with cardiovascular disease (CVD). However, concerns that type-D personality may not be a stable personality taxonomy and that progression of CVD may contribute to the manifestation of type-D personality have been voiced...

  3. Subset selection from Type-I and Type-II generalized logistic populations

    NARCIS (Netherlands)

    Laan, van der M.J.; Laan, van der P.

    1996-01-01

    We give an introduction to the logistic and generalized logistic distributions. We obtain exact results for the probability of correct selection from Type-I and Type-II generalized logistic populations which only differ in their location parameter. Some open problems are formulated.

  4. No evidence for increased self-reported cognitive failure in Type 1 and Type 2 diabetes

    DEFF Research Database (Denmark)

    Wessels, A M; Pouwer, F; Geelhoed-Duijvestijn, P H L M

    2007-01-01

    in adult out-patients with Type 1 and Type 2 diabetes. METHODS: Subjective cognitive functioning was measured in 187 diabetic patients using the Cognitive Failures Questionnaire (CFQ). Demographic and clinical characteristics were retrieved from the medical records. The Patient Health Questionnaire 9 items...... (PHQ-9) was self-administered along with the CFQ to correct for the confounding effect of depression. RESULTS: Analyses were based on 55 patients with Type 1 diabetes and 100 patients with Type 2 diabetes. No difference in mean CFQ score was observed between Type 1 and Type 2 diabetic patients...... or between Type 1 diabetic patients and healthy control subjects. Female patients with Type 2 diabetes reported significantly fewer cognitive complaints compared with female healthy control subjects. None of the demographic variables and diabetes-related complications was associated with subjective cognitive...

  5. Comparison of osteoporosis in US adults with type 1 and type 2 diabetes mellitus.

    Science.gov (United States)

    DeShields, S C; Cunningham, T D

    2018-01-20

    We examined bone mineral density (BMD) and osteoporosis prevalence in those with type 1 compared to type 2 diabetes derived from a nationally representative sample from the civilian community in the United States. Data from the National Health and Nutrition Examination Survey (NHANES) for 2005-2006, 2007-2008, 2009-2010, and 2013-2014 were merged to obtain a large sample of diabetics at least 20 years of age with participation in the interview and medical examination. Osteoporosis status was defined by BMD at the total femur, femoral neck, or total lumbar spine. Self-reported diabetics that were prescribed insulin within the first year of diagnosis, are currently taking insulin, and reported no prescriptions for any diabetic pills were classified as type 1. Remaining self-reported diabetics were deemed as having type 2. A total of 2050 diabetics were included in which 87 (4%) were classified as type 1. Type 1 diabetics were found to have a significantly lower BMD at the total femur and femoral neck, but not at the lumbar spine in the adjusted models. Diabetics with type 1 were 4.7 times more likely to have osteoporosis than those with type 2. There was no significant relationship between diabetes type and BMD or osteoporosis prior to adjustment for confounders. Although our results show an increased likelihood of osteoporosis among those with type 1 diabetes, future studies including a larger sample from a community population are needed. It may benefit diabetics, especially those with type 1, to initiate osteoporosis screening methods including evaluation of fracture risk, bone quality, and BMD measurements at multiple sites earlier than recommended.

  6. Osteogenesis imperfecta type V

    DEFF Research Database (Denmark)

    Rauch, Frank; Moffatt, Pierre; Cheung, Moira

    2013-01-01

    Osteogenesis imperfecta (OI) type V is an autosomal dominant bone fragility disorder that we had described a decade ago. Recent research has shown that OI type V is caused by a recurrent c.-14C>T mutation in IFITM5. In the present study, we assessed all patients diagnosed with OI type V at our...

  7. Flash-Type Discrimination

    Science.gov (United States)

    Koshak, William J.

    2010-01-01

    This viewgraph presentation describes the significant progress made in the flash-type discrimination algorithm development. The contents include: 1) Highlights of Progress for GLM-R3 Flash-Type discrimination Algorithm Development; 2) Maximum Group Area (MGA) Data; 3) Retrieval Errors from Simulations; and 4) Preliminary Global-scale Retrieval.

  8. Cardiovascular responses in type A and type B men to a series of stressors.

    Science.gov (United States)

    Ward, M M; Chesney, M A; Swan, G E; Black, G W; Parker, S D; Rosenman, R H

    1986-02-01

    Fifty-six healthy adult males were administered the Type A Structured Interview and assessed as exhibiting either Type A (N = 42) or Type B (N = 14) behavior pattern. They were monitored for systolic (SBP) and diastolic blood pressure (DBP) and heart rate (HR) responses during a series of six challenging tasks: Mental Arithmetic, Hypothesis Testing, Reaction Time, Video Game, Handgrip, and Cold Pressor. The results indicated that Type A subjects exhibited greater cardiovascular responses than did Type B subjects during some (Hypothesis Testing, Reaction Time, Video Game and Mental Arithmetic) but not all (Handgrip and Cold Pressor) of the tasks. These results are discussed in terms of previously reported findings on conditions that do and do not produce differences in Type A/B cardiovascular stress responses.

  9. Prospecting ideas for mesozoic granite-type, volcanics-type and exo-contact-type uranium deposits in South China. Pt.1

    International Nuclear Information System (INIS)

    Yu Dagan

    2001-01-01

    The Mesozoic uranium ore-formation process in South China resulted from the intense volcanic magmatism, the crust-mantle interaction and the fluidization in Yanshanian period, and there is great prospect for large-scale uranium concentration and ore-formation. Therefore, during the prospecting for Mesozoic granite-type, volcanics-type and exo-contact-type uranium deposits it is necessary to introduce deep-source metallogenic theory, to 'desalt' metallogenic theory of epithermal activation, to fully realize that uranium deposits may be formed not only at shallow depth, but also in conditions of deep-source, great depth and high temperature, as well as in environments of deep-source, shallow depth and median temperature, to give emphasis to the early-stage (130-95 Ma) uranium mineralization, to break the man-made boundary in prospecting for uranium only based on host rock type, to strengthen the research on Cretaceous magmatic system

  10. Delamination propensity of pharmaceutical glass containers by accelerated testing with different extraction media.

    Science.gov (United States)

    Guadagnino, Emanuel; Zuccato, Daniele

    2012-01-01

    The delamination of pharmaceutical glass is a serious issue, as it can cause glass particles to appear in vials, a problem that has forced a number of drug product recalls in recent years. In Type I pharmaceutical glass vials, delamination occurs generally at the bottom and shoulder, where extensive flaming during the conversion process can favor a strong evaporation of alkali and borate species and the formation of heavily enriched silica layers. The contact with parenteral preparations dissolved in an alkaline medium increases the rate of glass corrosion, while the differential hydration of these layers can cause the detachment of flakes. The purpose of this study was to investigate the effect of the pH and the composition of the extraction solutions on the propensity of different glass types to delaminate. Repeated autoclave extractions at 121 °C were carried out on different glass types with different extraction media, including organic extractants like citric and glutaric acid. When vials were in contact with alkaline solutions and similarly aggressive media, an increase in silica extraction values indicated glass corrosion and an increasing risk for further delamination. Under such conditions expansion 33 glass is extensively corroded, showing high silica concentration and heavy flaking as compared to other glass types. Sulfur-treated glass also showed early flaking, even if SiO(2) concentration was very low. A similar ranking was observed with extractions carried out with glutaric and citric acids, but at far much higher SiO(2) concentration levels. Extractions with 0.9% KCl solution can be used as an accelerated test to highlight the propensity of a glass to delaminate, but in no case it can be taken as a guarantee that the glass will not delaminate when exposed to the pharmaceutical drug, whose extraction ability requires case-by-case study. How can injectable drug manufacturers prevent glass delamination? The issue of delamination is a serious one, as it

  11. [Diagnostic values of serum type III procollagen N-terminal peptide in type IV gastric cancer].

    Science.gov (United States)

    Akazawa, S; Fujiki, T; Kanda, Y; Kumai, R; Yoshida, S

    1985-04-01

    Since increased synthesis of collagen has been demonstrated in tissue of type IV gastric cancer, we attempted to distinguish type IV gastric cancer from other cancers by measuring serum levels of type III procollagen N-terminal peptide (type III-N-peptide). Mean serum levels in type IV gastric cancer patients without metastasis were found to be elevated above normal values and developed a tendency to be higher than those in types I, II and III gastric cancer patients without metastasis. Highly positive ratios were found in patients with liver diseases including hepatoma and colon cancer, biliary tract cancer, and esophageal cancer patients with liver, lung or bone metastasis, but only 2 out of 14 of these cancer patients without such metastasis showed positive serum levels of type III-N-peptide. Positive cases in patients with type IV gastric cancer were obtained not only in the group with clinical stage IV but also in the groups with clinical stages II and III. In addition, high serum levels of type III-N-peptide in patients with type IV gastric cancer were seen not only in the cases with liver, lung or bone metastasis but also in cases with disseminated peritoneal metastasis alone. These results suggest that if the serum level of type III-N-peptide is elevated above normal values, type IV gastric cancer should be suspected after ruling out liver diseases, myelofibrosis and liver, lung or bone metastasis.

  12. Getting “Inside” Type I IFNs: Type I IFNs in Intracellular Bacterial Infections

    Directory of Open Access Journals (Sweden)

    Deann T. Snyder

    2017-01-01

    Full Text Available Type I interferons represent a unique and complex group of cytokines, serving many purposes during innate and adaptive immunity. Discovered in the context of viral infections, type I IFNs are now known to have myriad effects in infectious and autoimmune disease settings. Type I IFN signaling during bacterial infections is dependent on many factors including whether the infecting bacterium is intracellular or extracellular, as different signaling pathways are activated. As such, the repercussions of type I IFN induction can positively or negatively impact the disease outcome. This review focuses on type I IFN induction and downstream consequences during infection with the following intracellular bacteria: Chlamydia trachomatis, Listeria monocytogenes, Mycobacterium tuberculosis, Salmonella enterica serovar Typhimurium, Francisella tularensis, Brucella abortus, Legionella pneumophila, and Coxiella burnetii. Intracellular bacterial infections are unique because the bacteria must avoid, circumvent, and even co-opt microbial “sensing” mechanisms in order to reside and replicate within a host cell. Furthermore, life inside a host cell makes intracellular bacteria more difficult to target with antibiotics. Because type I IFNs are important immune effectors, modulating this pathway may improve disease outcomes. But first, it is critical to understand the context-dependent effects of the type I IFN pathway in intracellular bacterial infections.

  13. Two thumbs and one index: A comparison of manual coordination in touch-typing and mobile-typing.

    Science.gov (United States)

    Cerni, Tania; Longcamp, Marieke; Job, Remo

    2016-06-01

    It has been extensively demonstrated that in touch-typing, manual alternation is performed faster than manual repetition (see i.e. Rumelhart & Norman, 1982), due to parallel activation of successive keystrokes. In this experiment, we tested whether the manual coordination patterns typical of touch-typing can be observed in mobile-typing. We recruited skilled touch-typists and divided them into two groups depending on their typing habits on the mobile device. The "one-hand" group typed with one index finger on the mobile, and therefore produced words exclusively through manual repetition. The "two-hands" group used two thumbs, and therefore produced words through a combination of mobile-typing repetitions and alternations. The two groups were tested in a typing to dictation task with both a standard keyboard and a mobile keyboard. Results showed that manual alternation and manual repetition patterns are similar in touch-typing and in mobile-typing. For the "two-hands" group, the mean interkeystroke intervals (IKIs) for touch-typing decreased as manual alterations in words increased in both touch- and mobile-typing. The "one-hand" group showed an opposite pattern in mobile-typing. Bigram frequency was correlated with IKIs per bigrams in both tasks and groups, but the correlation for the "one-hand" group in mobile-typing was different. Our results suggest that manual coordination processes are the same in touch-typing and in mobile-typing despite different effectors, provided that both hands are used to type. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. Stack semantics of type theory

    DEFF Research Database (Denmark)

    Coquand, Thierry; Mannaa, Bassel; Ruch, Fabian

    2017-01-01

    We give a model of dependent type theory with one univalent universe and propositional truncation interpreting a type as a stack, generalizing the groupoid model of type theory. As an application, we show that countable choice cannot be proved in dependent type theory with one univalent universe...

  15. Type-II Weyl semimetals.

    Science.gov (United States)

    Soluyanov, Alexey A; Gresch, Dominik; Wang, Zhijun; Wu, QuanSheng; Troyer, Matthias; Dai, Xi; Bernevig, B Andrei

    2015-11-26

    Fermions--elementary particles such as electrons--are classified as Dirac, Majorana or Weyl. Majorana and Weyl fermions had not been observed experimentally until the recent discovery of condensed matter systems such as topological superconductors and semimetals, in which they arise as low-energy excitations. Here we propose the existence of a previously overlooked type of Weyl fermion that emerges at the boundary between electron and hole pockets in a new phase of matter. This particle was missed by Weyl because it breaks the stringent Lorentz symmetry in high-energy physics. Lorentz invariance, however, is not present in condensed matter physics, and by generalizing the Dirac equation, we find the new type of Weyl fermion. In particular, whereas Weyl semimetals--materials hosting Weyl fermions--were previously thought to have standard Weyl points with a point-like Fermi surface (which we refer to as type-I), we discover a type-II Weyl point, which is still a protected crossing, but appears at the contact of electron and hole pockets in type-II Weyl semimetals. We predict that WTe2 is an example of a topological semimetal hosting the new particle as a low-energy excitation around such a type-II Weyl point. The existence of type-II Weyl points in WTe2 means that many of its physical properties are very different to those of standard Weyl semimetals with point-like Fermi surfaces.

  16. Blood metals concentration in type 1 and type 2 diabetics.

    Science.gov (United States)

    Forte, Giovanni; Bocca, Beatrice; Peruzzu, Angela; Tolu, Francesco; Asara, Yolande; Farace, Cristiano; Oggiano, Riccardo; Madeddu, Roberto

    2013-12-01

    Mechanisms for the onset of diabetes and the development of diabetic complications remain under extensive investigations. One of these mechanisms is abnormal homeostasis of metals, as either deficiency or excess of metals, can contribute to certain diabetic outcomes. Therefore, this paper will report the blood levels of chromium (Cr), copper (Cu), iron (Fe), manganese (Mn), mercury (Hg), nickel (Ni), lead (Pb), selenium (Se), and zinc (Zn) in subjects with type 1 diabetes (n = 192, mean age 48.8 years, mean disease duration 20.6 years), type 2 diabetes (n = 68, mean age 68.4 years, mean disease duration 10.2 years), and in control subjects (n = 59, mean age 57.2 years), and discuss the results indicating their possible role in diabetes. The metal concentrations were measured by sector field inductively coupled plasma mass spectrometry after microwave-induced acid digestion of blood samples. The accuracy was checked using a blood-based certified reference material, and recoveries of all elements were in the range of 92-101 % of certified values. Type 1 diabetes was found to be associated with Cr (p = 0.02), Mn (p < 0.001), Ni (p < 0.001), Pb (p = 0.02), and Zn (p < 0.001) deficiency, and type 2 diabetes with Cr (p = 0.014), Mn (p < 0.001), and Ni (p < 0.001) deficiency. These deficiencies were appreciated also subdividing the understudied patients for gender and age groups. Furthermore, in type 1 diabetes, there was a positive correlation between Pb and age (p < 0.001, ρ = 0.400) and Pb and BMI (p < 0.001, ρ = 0.309), while a negative correlation between Fe and age (p = 0.002, ρ = -0.218). In type 2 diabetes, there was a negative correlation between Fe and age (p = 0.017, ρ = -0.294) and Fe and BMI (p = 0.026, ρ = -0.301). Thus, these elements may play a role in both forms of diabetes and combined mineral supplementations could have beneficial effects.

  17. Hardy type inequalities on time scales

    CERN Document Server

    Agarwal, Ravi P; Saker, Samir H

    2016-01-01

    The book is devoted to dynamic inequalities of Hardy type and extensions and generalizations via convexity on a time scale T. In particular, the book contains the time scale versions of classical Hardy type inequalities, Hardy and Littlewood type inequalities, Hardy-Knopp type inequalities via convexity, Copson type inequalities, Copson-Beesack type inequalities, Liendeler type inequalities, Levinson type inequalities and Pachpatte type inequalities, Bennett type inequalities, Chan type inequalities, and Hardy type inequalities with two different weight functions. These dynamic inequalities contain the classical continuous and discrete inequalities as special cases when T = R and T = N and can be extended to different types of inequalities on different time scales such as T = hN, h > 0, T = qN for q > 1, etc.In this book the authors followed the history and development of these inequalities. Each section in self-contained and one can see the relationship between the time scale versions of the inequalities and...

  18. A Type System for Tom

    Directory of Open Access Journals (Sweden)

    Claude Kirchner

    2010-03-01

    Full Text Available Extending a given language with new dedicated features is a general and quite used approach to make the programming language more adapted to problems. Being closer to the application, this leads to less programming flaws and easier maintenance. But of course one would still like to perform program analysis on these kinds of extended languages, in particular type checking and inference. In this case one has to make the typing of the extended features compatible with the ones in the starting language. The Tom programming language is a typical example of such a situation as it consists of an extension of Java that adds pattern matching, more particularly associative pattern matching, and reduction strategies. This paper presents a type system with subtyping for Tom, that is compatible with Java's type system, and that performs both type checking and type inference. We propose an algorithm that checks if all patterns of a Tom program are well-typed. In addition, we propose an algorithm based on equality and subtyping constraints that infers types of variables occurring in a pattern. Both algorithms are exemplified and the proposed type system is showed to be sound and complete.

  19. Types Of Christian Tourism

    Directory of Open Access Journals (Sweden)

    Cornelia Petroman

    2015-10-01

    Full Text Available Christian tourism is asub-type of tourism where pilgrims travel alone or in group as missionaries orpilgrims or to spend their free time to recollect; it attracts a large numberof travellers. Christian tourism can be practiced by religious young people,missionaries, participants to religious convents, amateurs of religiouscruises, religious camps, visitors of religious attractions, religiousadventurers. Christian tourism can be considered a sub-type of religioustourism because of the large number of people involved in this type of tourismworldwide. Christian tourism as a sub-category of religious tourism coversthree main sub-types: traditional pilgrimage practiced by most world religions,missionary travel to different areas in the world, and confessional voyage. Thestructure of hospitality industry is common to all types of tourism but, fortraditional pilgrimage, there must be special places for the pilgrims to sleep,eat and pray, because they belong, in most cases, to the medium-income touristcategory.

  20. Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Simon, Stéphanie; Lugan, Séverine; Bilheude, Jean-Marc; Perret-Liaudet, Armand; Ironside, James W.; Haik, Stéphane; Basset-Leobon, Christelle; Lacroux, Caroline; Peoch', Katell; Streichenberger, Nathalie; Langeveld, Jan; Head, Mark W.; Grassi, Jacques; Hauw, Jean-Jacques; Schelcher, Francois; Delisle, Marie Bernadette; Andréoletti, Olivier

    2008-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct PrPres types have been considered to represent potential distinct prion strains. However, since cases of CJD show co-occurrence of type 1 and type 2 PrPres in the brain, the basis of this classification system and its relationship to agent strain are under discussion. Different brain areas from 41 sCJD and 12 iatrogenic CJD (iCJD) cases were investigated, using Western blotting for PrPres and two other biochemical assays reflecting the behaviour of the disease-associated form of the prion protein (PrPSc) under variable PK digestion conditions. In 30% of cases, both type 1 and type 2 PrPres were identified. Despite this, the other two biochemical assays found that PrPSc from an individual patient demonstrated uniform biochemical properties. Moreover, in sCJD, four distinct biochemical PrPSc subgroups were identified that correlated with the current sCJD clinico-pathological classification. In iCJD, four similar biochemical clusters were observed, but these did not correlate to any particular PRNP 129 polymorphism or western blot PrPres pattern. The identification of four different PrPSc biochemical subgroups in sCJD and iCJD, irrespective of the PRNP polymorphism at codon 129 and the PrPres isoform provides an alternative biochemical definition of PrPSc diversity and new insight in the perception of Human TSE agents variability. PMID:18389084

  1. Rotating spacetimes of Goedel-type

    International Nuclear Information System (INIS)

    Reboucas, M.J.; Teixeira, A.F.F.

    1986-01-01

    The Goedel-type Riemannian manifolds are examined under two different assumptions on the algebraic structure of the energy-momentum tensor. All Goedel-type manifolds of either Segre type [1,(1,111)] or [(1,11)1] are shown to be spacetime-homogeneous. A generalization of Bampi-Zordan theorem is presented. All Goedel-type Riemannian manifolds of the algebric tachyon fluid type are shown to be conformally flat and isometric to Reboucas-Tiomno model. The conformal form of Reboucas-Tiomno is given. (Author) [pt

  2. Composing and decomposing data types

    DEFF Research Database (Denmark)

    Bahr, Patrick

    2014-01-01

    restrictive, disallowing subtypings that lead to more than one possible injection and should therefore be considered programming errors. Furthermore, from this refined subtyping constraint we derive a new constraint to express type isomorphism. We show how this isomorphism constraint allows us to decompose......Wouter Swierstra's data types à la carte is a technique to modularise data type definitions in Haskell. We give an alternative implementation of data types à la carte that offers more flexibility in composing and decomposing data types. To achieve this, we refine the subtyping constraint, which...... is at the centre of data types à la carte. On the one hand this refinement is more general, allowing subtypings that intuitively should hold but were not derivable beforehand. This aspect of our implementation removes previous restrictions on how data types can be combined. On the other hand our refinement is more...

  3. Improving self-management of type 1 and type 2 diabetes.

    Science.gov (United States)

    Phillips, Anne

    2016-01-06

    Diabetes is an increasingly common life-long condition, which has significant physical, psychological and behavioural implications for individuals. Self-management of type 1 and type 2 diabetes can be complex and challenging. A collaborative approach to care, between healthcare professionals and patients, is essential to promote self-management skills and knowledge to help patients engage in shared decision making and manage any difficulties associated with a diagnosis of diabetes.

  4. The Type 3 copper site is intact but labile in Type 2-depleted laccase

    DEFF Research Database (Denmark)

    Frank, P; Farver, O; Pecht, I

    1983-01-01

    We report results of experiments designed to characterize the Type 1 and Type 3 copper sites in Rhus laccase depleted of Type 2 copper (T2D). Use of the Lowry method for determining protein concentration yielded the value 5620 +/- 570 M-1 cm-1 for the extinction of the 615-nm absorption band...... as intensity perturbations at 280 and 615 nm. Comparison of difference spectra show that this 330-nm band derives from a Type 3 copper-bound peroxide and not from a reoxidized Type 3 site. Dioxygen reoxidation of ascorbate-reduced T2D laccase produced new difference bands at 330 nm (delta epsilon = 770 M-1 cm...

  5. Differences in typing forces, muscle activity, comfort, and typing performance among virtual, notebook, and desktop keyboards.

    Science.gov (United States)

    Kim, Jeong Ho; Aulck, Lovenoor; Bartha, Michael C; Harper, Christy A; Johnson, Peter W

    2014-11-01

    The present study investigated whether there were physical exposure and typing productivity differences between a virtual keyboard with no tactile feedback and two conventional keyboards where key travel and tactile feedback are provided by mechanical switches under the keys. The key size and layout were same across all the keyboards. Typing forces; finger and shoulder muscle activity; self-reported comfort; and typing productivity were measured from 19 subjects while typing on a virtual (0 mm key travel), notebook (1.8 mm key travel), and desktop keyboard (4 mm key travel). When typing on the virtual keyboard, subjects typed with less force (p's typing forces and finger muscle activity came at the expense of a 60% reduction in typing productivity (p typing sessions or when typing productivity is at a premium, conventional keyboards with tactile feedback may be more suitable interface. Copyright © 2014 Elsevier Ltd and The Ergonomics Society. All rights reserved.

  6. Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

    Science.gov (United States)

    Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

    2015-05-07

    Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Copyright 2015, SLACK Incorporated.

  7. Tension type headache

    Directory of Open Access Journals (Sweden)

    Debashish Chowdhury

    2012-01-01

    Full Text Available Tension type headaches are common in clinical practice. Earlier known by various names, the diagnosis has had psychological connotations. Recent evidence has helped clarify the neurobiological basis and the disorder is increasingly considered more in the preview of neurologists. The classification, clinical features, differential diagnosis and treatment of tension type headache are discussed in this paper.

  8. A Simple Solution to Type Specialization

    DEFF Research Database (Denmark)

    Danvy, Olivier

    1998-01-01

    Partial evaluation specializes terms, but traditionally this specialization does not apply to the type of these terms. As a result, specializing, e.g., an interpreter written in a typed language, which requires a “universal” type to encode expressible values, yields residual programs with type tags...... all over. Neil Jones has stated that getting rid of these type tags was an open problem, despite possible solutions such as Torben Mogensen's “constructor specialization.” To solve this problem, John Hughes has proposed a new paradigm for partial evaluation, “Type Specialization”, based on type...... inference instead of being based on symbolic interpretation. Type Specialization is very elegant in principle but it also appears non-trivial in practice. Stating the problem in terms of types instead of in terms of type encodings suggests a very simple type-directed solution, namely, to use a projection...

  9. Principal Typings in a Restricted Intersection Type System for Beta Normal Forms with De Bruijn Indices

    Directory of Open Access Journals (Sweden)

    Daniel Ventura

    2010-01-01

    Full Text Available The lambda-calculus with de Bruijn indices assembles each alpha-class of lambda-terms in a unique term, using indices instead of variable names. Intersection types provide finitary type polymorphism and can characterise normalisable lambda-terms through the property that a term is normalisable if and only if it is typeable. To be closer to computations and to simplify the formalisation of the atomic operations involved in beta-contractions, several calculi of explicit substitution were developed mostly with de Bruijn indices. Versions of explicit substitutions calculi without types and with simple type systems are well investigated in contrast to versions with more elaborate type systems such as intersection types. In previous work, we introduced a de Bruijn version of the lambda-calculus with an intersection type system and proved that it preserves subject reduction, a basic property of type systems. In this paper a version with de Bruijn indices of an intersection type system originally introduced to characterise principal typings for beta-normal forms is presented. We present the characterisation in this new system and the corresponding versions for the type inference and the reconstruction of normal forms from principal typings algorithms. We briefly discuss the failure of the subject reduction property and some possible solutions for it.

  10. A simple solution to type specialization

    DEFF Research Database (Denmark)

    Danvy, Olivier

    1998-01-01

    Partial evaluation specializes terms, but traditionally this specialization does not apply to the type of these terms. As a result, specializing, e.g., an interpreter written in a typed language, which requires a “universal” type to encode expressible values, yields residual programs with type tags...... all over. Neil Jones has stated that getting rid of these type tags was an open problem, despite possible solutions such as Torben Mogensen's “constructor specialization.” To solve this problem, John Hughes has proposed a new paradigm for partial evaluation, “Type Specialization”, based on type...... from the universal type to the specific type of the residual program. Standard partial evaluation then yields a residual program without type tags, simply and efficiently....

  11. Molecular Typing of Neisseria gonorrhoeae Isolates by Opa-Typing and Ribotyping in New Delhi, India

    Directory of Open Access Journals (Sweden)

    Pejvak Khaki

    2009-01-01

    Full Text Available Control and preventive measures for gonococcal infections are based on precise epidemiological characteristics of N. gonorrhoeae isolates. In the present study the potential utility of opa-typing and ribotyping for molecular epidemiological study of consecutive gonococcal strains was determined. Sixty gonococcal isolates were subjected to ribotyping with two restriction enzymes, AvaII and HincII, and opa-typing with TaqI and HpaII for epidemiological characterization of gonococcal population. Ribotyping with AvaII yielded 6 ribotype patterns while twelve RFLP patterns were observed with HincII. Opa-typing of the 60 isolates revealed a total 54 opa-types, which 48 were unique and 6 formed clusters. Fifty-two opa-types were observed with TaqI-digested PCR product while opa-typing with HpaII demonstrated 54 opa-types. The opa-types from isolates that were epidemiologically unrelated were distinct, whereas those from the sexual contacts were identical. The results showed that opa-typing is highly useful for characterizing gonococcal strains from sexual contacts and has more discriminatory than ribotyping that could differentiate between gonococci of the same ribotype. The technique even with a single restriction enzyme has a high level of discrimination (99.9% between epidemiologically unrelated isolates. In conclusion, the molecular methods such as opa-typing and ribotyping can be used for epidemiological characterization of gonococcal strains.

  12. Molecular Typing of Neisseria gonorrhoeae Isolates by Opa-Typing and Ribotyping in New Delhi, India

    Science.gov (United States)

    Khaki, Pejvak; Bhalla, Preena; Fayaz, Ahmad Mir; Moradi Bidhendi, Sohiela; Esmailzadeh, Majid; Sharma, Pawan

    2009-01-01

    Control and preventive measures for gonococcal infections are based on precise epidemiological characteristics of N. gonorrhoeae isolates. In the present study the potential utility of opa-typing and ribotyping for molecular epidemiological study of consecutive gonococcal strains was determined. Sixty gonococcal isolates were subjected to ribotyping with two restriction enzymes, AvaII and HincII, and opa-typing with TaqI and HpaII for epidemiological characterization of gonococcal population. Ribotyping with AvaII yielded 6 ribotype patterns while twelve RFLP patterns were observed with HincII. Opa-typing of the 60 isolates revealed a total 54 opa-types, which 48 were unique and 6 formed clusters. Fifty-two opa-types were observed with TaqI-digested PCR product while opa-typing with HpaII demonstrated 54 opa-types. The opa-types from isolates that were epidemiologically unrelated were distinct, whereas those from the sexual contacts were identical. The results showed that opa-typing is highly useful for characterizing gonococcal strains from sexual contacts and has more discriminatory than ribotyping that could differentiate between gonococci of the same ribotype. The technique even with a single restriction enzyme has a high level of discrimination (99.9%) between epidemiologically unrelated isolates. In conclusion, the molecular methods such as opa-typing and ribotyping can be used for epidemiological characterization of gonococcal strains. PMID:20016674

  13. The Difference between Flux Spectrums of WH-type Assembly and CANDU-type Lattice

    International Nuclear Information System (INIS)

    Ryu, Eun Hyun; Song, Yong Mann

    2014-01-01

    The nuclear reactors are categorized by the material of the moderator because of its importance. The representative materials of the moderator are light water (H 2 O) and heavy water (D 2 O). Also, it is well known that the slowing-down ratio of D 2 O is hundreds of times larger than that of H 2 O while the slowing-down power of H 2 O is several times larger than that of D 2 O. This means that the H 2 O sometimes plays a role of an absorber such as the liquid zone controller (LZC) in a CANDU-type reactor. It is thought that the flux spectrums in a different reactor can differ from each other. In this research, two representative assemblies (the Westinghouse (WH)-type fuel assembly of PWR and the CANDU-type fuel lattice of PHWR) are selected and the flux results for each group are extracted. Although there are many codes for the lattice transport calculation, the WIMS code and the HELIOS code are used for the calculation of the WH-type fuel lattice and the CANDU-type fuel lattice. A clear difference in spectrum between the CANDU-type lattice and WH 16GD-type lattice is confirmed. Because of the superior moderating ratio of the heavy water, the thermal flux ratio of the CANDU-type lattice is almost 82%, while that of the WH 16 GD-type lattice is around 23%. Because of the large portion of the thermal flux in the CANDU-type lattice, the boron effect is maximized with the result from variations of boron. Thus it can be said that the spectrum largely depends on the moderator material, and the boron effect and sensitivity largely depends on the flux spectrum. Because of the dominant effect of the moderator material on the flux spectrum in a nuclear reactor, in the future, a comparison of the spectra of SFR, HTGR, PWR, and PHWR are also an interesting subject to study. Over-moderation in PHWR lattice and under-moderation in PWR lattice can be explained by the investigation about flux spectrums with variations of moderator density in each lattice

  14. The Difference between Flux Spectrums of WH-type Assembly and CANDU-type Lattice

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Eun Hyun; Song, Yong Mann [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

    2014-10-15

    The nuclear reactors are categorized by the material of the moderator because of its importance. The representative materials of the moderator are light water (H{sub 2}O) and heavy water (D{sub 2}O). Also, it is well known that the slowing-down ratio of D{sub 2}O is hundreds of times larger than that of H{sub 2}O while the slowing-down power of H{sub 2}O is several times larger than that of D{sub 2}O. This means that the H{sub 2}O sometimes plays a role of an absorber such as the liquid zone controller (LZC) in a CANDU-type reactor. It is thought that the flux spectrums in a different reactor can differ from each other. In this research, two representative assemblies (the Westinghouse (WH)-type fuel assembly of PWR and the CANDU-type fuel lattice of PHWR) are selected and the flux results for each group are extracted. Although there are many codes for the lattice transport calculation, the WIMS code and the HELIOS code are used for the calculation of the WH-type fuel lattice and the CANDU-type fuel lattice. A clear difference in spectrum between the CANDU-type lattice and WH 16GD-type lattice is confirmed. Because of the superior moderating ratio of the heavy water, the thermal flux ratio of the CANDU-type lattice is almost 82%, while that of the WH 16 GD-type lattice is around 23%. Because of the large portion of the thermal flux in the CANDU-type lattice, the boron effect is maximized with the result from variations of boron. Thus it can be said that the spectrum largely depends on the moderator material, and the boron effect and sensitivity largely depends on the flux spectrum. Because of the dominant effect of the moderator material on the flux spectrum in a nuclear reactor, in the future, a comparison of the spectra of SFR, HTGR, PWR, and PHWR are also an interesting subject to study. Over-moderation in PHWR lattice and under-moderation in PWR lattice can be explained by the investigation about flux spectrums with variations of moderator density in each

  15. Computing effects for correspondence types

    OpenAIRE

    Hüttel, Hans

    2010-01-01

    We show that type and effect inference is possible for a type and  effect system for authenticity using non-injective correspondences, opponent  types and a spi-calculus with symmetric encryption. We do this by a general  account of how effects can be computed given knowledge of how and where they  appear in type judgments. 

  16. Computing effects for correspondence types

    DEFF Research Database (Denmark)

    Hüttel, Hans

    2010-01-01

    We show that type and effect inference is possible for a type and  effect system for authenticity using non-injective correspondences, opponent  types and a spi-calculus with symmetric encryption. We do this by a general  account of how effects can be computed given knowledge of how and where they......  appear in type judgments. ...

  17. Facts about Type 2

    Medline Plus

    Full Text Available ... Know Your Risk Diabetes Basics Symptoms Type 1 Type 2 Gestational Myths Statistics Common Terms Genetics Living With Diabetes Recently Diagnosed Treatment & Care Complications ...

  18. Comparative analysis of type 1 and type 2 cassava peeling machines

    African Journals Online (AJOL)

    A comparative analysis of type 1 and type 2 cassava peeling machines with nail lengths of 26mm and 20mm were investigated in order to improve peeling techniques of cassava tubers. The machines were evaluated at four (4) different speeds; (80rev/min, 90rev/min, 100rev/min, and 110rev/min), with 750HP petrol engine.

  19. Type I and type II interferons upregulate functional type I interleukin-1 receptor in a human fibroblast cell line TIG-1.

    Science.gov (United States)

    Takii, T; Niki, N; Yang, D; Kimura, H; Ito, A; Hayashi, H; Onozaki, K

    1995-12-01

    The regulation of type I interleukin-1 receptor (IL-1R) expression by type I, interferon (IFN)-alpha A/D, and type II IFN, IFN-gamma, in a human fibroblast cell line TIG-1 was investigated. After 2 h stimulation with human IFN-alpha A/D or IFN-gamma, the levels of type I IL-1R mRNA increased. We previously reported that IL-1 upregulates transcription and cell surface molecules of type I IL-1R in TIG-1 cells through induction of prostaglandin (PG) E2 and cAMP accumulation. However, indomethacin was unable to inhibit the effect of IFNs, indicating that IFNs augment IL-1R expression through a pathway distinct from that of IL-1. The augmentation was also observed in other fibroblast cell lines. Nuclear run-on assays and studies of the stability of mRNA suggested that the increase in IL-1R mRNA was a result of the enhanced transcription of IL-1R gene. Binding studies using 125I-IL-1 alpha revealed that the number of cell surface IL-1R increased with no change in binding affinity by treatment with these IFNs. Pretreatment of the cells with IFNs enhanced IL-1-induced IL-6 production, indicating that IFNs upregulate functional IL-1R. IL-1 and IFNs are produced by the same cell types, as well as by the adjacent different cell types, and are concomitantly present in lesions of immune and inflammatory reactions. These results therefore suggest that IFNs exhibit synergistic effects with IL-1 through upregulation of IL-1R. Augmented production of IL-6 may also contribute to the reactions.

  20. Type III CRISPR-Cas systems can provide redundancy to counteract viral escape from type I systems.

    Science.gov (United States)

    Silas, Sukrit; Lucas-Elio, Patricia; Jackson, Simon A; Aroca-Crevillén, Alejandra; Hansen, Loren L; Fineran, Peter C; Fire, Andrew Z; Sánchez-Amat, Antonio

    2017-08-17

    CRISPR-Cas-mediated defense utilizes information stored as spacers in CRISPR arrays to defend against genetic invaders. We define the mode of target interference and role in antiviral defense for two CRISPR-Cas systems in Marinomonas mediterranea . One system (type I-F) targets DNA. A second system (type III-B) is broadly capable of acquiring spacers in either orientation from RNA and DNA, and exhibits transcription-dependent DNA interference. Examining resistance to phages isolated from Mediterranean seagrass meadows, we found that the type III-B machinery co-opts type I-F CRISPR-RNAs. Sequencing and infectivity assessments of related bacterial and phage strains suggests an 'arms race' in which phage escape from the type I-F system can be overcome through use of type I-F spacers by a horizontally-acquired type III-B system. We propose that the phage-host arms race can drive selection for horizontal uptake and maintenance of promiscuous type III interference modules that supplement existing host type I CRISPR-Cas systems.

  1. The preventive role of type 2 NKT cells in the development of type 1 diabetes.

    Science.gov (United States)

    Sørensen, Jakob Ørskov; Buschard, Karsten; Brogren, Carl-Henrik

    2014-03-01

    In the last two decades, natural killer T (NKT) cells have emerged as an important factor in preventing type 1 diabetes (T1D) when investigated in the experimental non-obese diabetic (NOD) mouse model. So far, investigations have largely focused on type 1 NKT cells with invariant T-cell receptors, whereas the role of type 2 NKT cells with diverse T-cell receptors is less well understood. However, there have been several findings which indicate that in fact type 2 NKT cells may regulate the progression of type 1 diabetes in NOD mice, including a fraction of these cells which recognize β-cell-enriched sulfatide. Therefore, the focus for this review is to present the current evidence of the effect of type 2 NKT cells on the development of T1D. In general, there is still uncertainty surrounding the mechanism of activation and function of NKT cells. Here, we present two models of the effector mechanisms, respectively, Th1/Th2 polarization and the induction of tolerogenic dendritic cells (DC). In conclusion, this review points to the importance of immunoregulation by type 2 NKT cells in preventing the development of T1D and highlights the induction of tolerogenic DC as a likely mechanism. The possible therapeutic role of type 1 and type 2 NKT cells are evaluated and future experiments concerning type 2 NKT cells and T1D are proposed. © 2013 APMIS. Published by John Wiley & Sons Ltd.

  2. Play the Blood Typing Game

    Science.gov (United States)

    ... Nobel's Life and Work Teachers' Questionnaire The Blood Typing Game What happens if you get a blood ... learn about human blood types! Play the Blood Typing Game 28 September 2017 The mission based game ...

  3. Facts about Type 2

    Medline Plus

    Full Text Available ... Know Your Risk Diabetes Basics Symptoms Type 1 Type 2 Gestational Myths Statistics Common Terms Genetics Living With Diabetes Recently Diagnosed Treatment & Care Complications Health Insurance For ...

  4. Isolation and molecular characterization of type I and type II feline coronavirus in Malaysia.

    Science.gov (United States)

    Amer, Alazawy; Siti Suri, Arshad; Abdul Rahman, Omar; Mohd, Hair Bejo; Faruku, Bande; Saeed, Sharif; Tengku Azmi, Tengku Ibrahim

    2012-11-21

    Feline infectious peritonitis virus (FIPV) and feline enteric coronavirus (FECV) are two important coronaviruses of domestic cat worldwide. Although FCoV is prevalent among cats; the fastidious nature of type I FCoV to grow on cell culture has limited further studies on tissue tropism and pathogenesis of FCoV. While several studies reported serological evidence for FCoV in Malaysia, neither the circulating FCoV isolated nor its biotypes determined. This study for the first time, describes the isolation and biotypes determination of type I and type II FCoV from naturally infected cats in Malaysia. Of the total number of cats sampled, 95% (40/42) were RT-PCR positive for FCoV. Inoculation of clinical samples into Crandell feline kidney cells (CrFK), and Feline catus whole fetus-4 cells (Fcwf-4), show cytopathic effect (CPE) characterized by syncytial cells formation and later cell detachment. Differentiation of FCoV biotypes using RT-PCR assay revealed that, 97.5% and 2.5% of local isolates were type I and type II FCoV, respectively. These isolates had high sequence homology and phylogenetic similarity with several FCoV isolates from Europe, South East Asia and USA. This study reported the successful isolation of local type I and type II FCoV evident with formation of cytopathic effects in two types of cell cultures namely the CrFK and Fcwf-4 , where the later cells being more permissive. However, the RT-PCR assay is more sensitive in detecting the antigen in suspected samples as compared to virus isolation in cell culture. The present study indicated that type I FCoV is more prevalent among cats in Malaysia.

  5. The phenotypic spectrum of organic acidurias and urea cycle disorders. Part 2: the evolving clinical phenotype.

    Science.gov (United States)

    Kölker, Stefan; Valayannopoulos, Vassili; Burlina, Alberto B; Sykut-Cegielska, Jolanta; Wijburg, Frits A; Teles, Elisa Leão; Zeman, Jiri; Dionisi-Vici, Carlo; Barić, Ivo; Karall, Daniela; Arnoux, Jean-Baptiste; Avram, Paula; Baumgartner, Matthias R; Blasco-Alonso, Javier; Boy, S P Nikolas; Rasmussen, Marlene Bøgehus; Burgard, Peter; Chabrol, Brigitte; Chakrapani, Anupam; Chapman, Kimberly; Cortès I Saladelafont, Elisenda; Couce, Maria L; de Meirleir, Linda; Dobbelaere, Dries; Furlan, Francesca; Gleich, Florian; González, Maria Julieta; Gradowska, Wanda; Grünewald, Stephanie; Honzik, Tomas; Hörster, Friederike; Ioannou, Hariklea; Jalan, Anil; Häberle, Johannes; Haege, Gisela; Langereis, Eveline; de Lonlay, Pascale; Martinelli, Diego; Matsumoto, Shirou; Mühlhausen, Chris; Murphy, Elaine; de Baulny, Hélène Ogier; Ortez, Carlos; Pedrón, Consuelo C; Pintos-Morell, Guillem; Pena-Quintana, Luis; Ramadža, Danijela Petković; Rodrigues, Esmeralda; Scholl-Bürgi, Sabine; Sokal, Etienne; Summar, Marshall L; Thompson, Nicholas; Vara, Roshni; Pinera, Inmaculada Vives; Walter, John H; Williams, Monique; Lund, Allan M; Garcia-Cazorla, Angeles; Garcia Cazorla, Angeles

    2015-11-01

    The disease course and long-term outcome of patients with organic acidurias (OAD) and urea cycle disorders (UCD) are incompletely understood. To evaluate the complex clinical phenotype of OAD and UCD patients at different ages. Acquired microcephaly and movement disorders were common in OAD and UCD highlighting that the brain is the major organ involved in these diseases. Cardiomyopathy [methylmalonic (MMA) and propionic aciduria (PA)], prolonged QTc interval (PA), optic nerve atrophy [MMA, isovaleric aciduria (IVA)], pancytopenia (PA), and macrocephaly [glutaric aciduria type 1 (GA1)] were exclusively found in OAD patients, whereas hepatic involvement was more frequent in UCD patients, in particular in argininosuccinate lyase (ASL) deficiency. Chronic renal failure was often found in MMA, with highest frequency in mut(0) patients. Unexpectedly, chronic renal failure was also observed in adolescent and adult patients with GA1 and ASL deficiency. It had a similar frequency in patients with or without a movement disorder suggesting different pathophysiology. Thirteen patients (classic OAD: 3, UCD: 10) died during the study interval, ten of them during the initial metabolic crisis in the newborn period. Male patients with late-onset ornithine transcarbamylase deficiency were presumably overrepresented in the study population. Neurologic impairment is common in OAD and UCD, whereas the involvement of other organs (heart, liver, kidneys, eyes) follows a disease-specific pattern. The identification of unexpected chronic renal failure in GA1 and ASL deficiency emphasizes the importance of a systematic follow-up in patients with rare diseases.

  6. Band-type microelectrodes for amperometric immunoassays

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ga-Yeon; Chang, Young Wook; Ko, Hyuk [Department of Materials Science and Engineering, Yonsei University, 134 Shinchon-dong, Seodaemun-gu, Seoul, 120-749 (Korea, Republic of); Kang, Min-Jung [Korea Institute of Science and Technology (KIST), Seoul (Korea, Republic of); Pyun, Jae-Chul, E-mail: jcpyun@yonsei.ac.kr [Department of Materials Science and Engineering, Yonsei University, 134 Shinchon-dong, Seodaemun-gu, Seoul, 120-749 (Korea, Republic of)

    2016-07-20

    A band-type microelectrode was made using a parylene-N film as a passivation layer. A circular-type, mm-scale electrode with the same diameter as the band-type microelectrode was also made with an electrode area that was 5000 times larger than the band-type microelectrode. By comparing the amperometric signals of 3,5,3′,5′-tetramethylbenzidine (TMB) samples at different optical density (OD) values, the band-type microelectrode was determined to be 9 times more sensitive than the circular-type electrode. The properties of the circular-type and the band-type electrodes (e.g., the shape of their cyclic voltammograms, the type of diffusion layer used, and the diffusion layer thickness per unit electrode area) were characterized according to their electrode area using the COMSOL Multiphysics software. From these simulations, the band-type electrode was estimated to have the conventional microelectrode properties, even when the electrode area was 100 times larger than a conventional circular-type electrode. These results show that both the geometry and the area of an electrode can influence the properties of the electrode. Finally, amperometric analysis based on a band-type electrode was applied to commercial ELISA kits to analyze human hepatitis B surface antigen (hHBsAg) and human immunodeficiency virus (HIV) antibodies. - Highlights: • A band-type microelectrode was made using a parylene-N film as a passivation layer. • The band-type microelectrode was 14-times more sensitive than circular-type electrode. • The influence of geometry on microelectrode properties was simulated using COMSOL. • The band-type electrode was applied to ELISA kits for hHBsAg and hHIV-antibodies.

  7. A correspondence between type checking via reduction and type checking via evaluation

    DEFF Research Database (Denmark)

    Sergey, Ilya; Clark, Dave

    2012-01-01

    We describe a derivational approach to proving the equivalence of different representations of a type system. Different ways of representing type assignments are convenient for particular applications such as reasoning or implementation, but some kind of correspondence between them should be proven...

  8. Facts about Type 2

    Medline Plus

    Full Text Available ... Type 1 Diabetes Enroll in the Living WIth Type 2 Diabetes Program In It Together We Can Help Center for Information Legal Assistance Success Stories ... Tips for Caregivers ...

  9. Type 1 diabetes

    OpenAIRE

    Atkinson, Mark A; Eisenbarth, George S; Michels, Aaron W

    2013-01-01

    Over the past decade, knowledge of the pathogenesis and natural history of type 1 diabetes has grown substantially, particularly with regard to disease prediction and heterogeneity, pancreatic pathology, and epidemiology. Technological improvements in insulin pumps and continuous glucose monitors help patients with type 1 diabetes manage the challenge of lifelong insulin administration. Agents that show promise for averting debilitating disease-associated complications have also been identifi...

  10. Lectin typing of Campylobacter concisus

    DEFF Research Database (Denmark)

    Aabenhus, Rune Munck; Hynes, Sean O; Permin, Henrik

    2002-01-01

    A total of 44 clinical isolates and the type strain of the putative pathogen Campylobacter concisus were grouped based on their reactions with plant lectins. The optimized lectin typing system used C. concisus strains proteolytically pretreated and subsequently typed by using a panel of four...... lectins. The system grouped all 45 strains into 13 lectin reaction patterns, leaving no strain untypeable due to autoagglutination. Lectin types were both stable and reproducible....

  11. Treatment of type II and type III open tibia fractures in children.

    Science.gov (United States)

    Bartlett, C S; Weiner, L S; Yang, E C

    1997-07-01

    To determine whether severe open tibial fractures in children behave like similar fractures in adults. A combined retrospective and prospective review evaluated treatment protocol for type II and type III open tibial fractures in children over a ten-year period from 1984 to 1993. Twenty-three fractures were studied in children aged 3.5 to 14.5 (18 boys and 5 girls). There were six type II, eight type IIIA, and nine type IIIB fractures. Type I fractures were not included. Seven fractures were comminuted with significant butterfly fragments or segmental patterns. Treatment consisted of adequate debridement of soft tissues, closure of dead space, and stabilization with external fixation. Bone debridement only included contaminated devitalized bone or devitalized bone without soft tissue coverage. Bone that could be covered despite periosteal stripping was preserved. Clinical and roentgenographic examinations were used to determine time to union. All fractures in this series healed between eight and twenty-six weeks. Wound coverage included two flaps, three skin grafts, and two delayed primary closures. No bone grafts were required. There were no deep infections, growth arrests, or malunions. Follow-up has ranged from six months to four years. Open tibia fractures in children differ from similar fractures in adults in the following ways: soft tissues have excellent healing capacity, devitalized bone that is not contaminated or exposed can be saved and will become incorporated, and external fixation can be maintained until the fracture has healed. Periosteum in young children can form bone even in the face of bone loss.

  12. Usher syndrome type III can mimic other types of Usher syndrome.

    Science.gov (United States)

    Pennings, Ronald J E; Fields, Randall R; Huygen, Patrick L M; Deutman, August F; Kimberling, William J; Cremers, Cor W R J

    2003-06-01

    Clinical and genetic characteristics are presented of 2 patients from a Dutch Usher syndrome type III family who have a new homozygous USH3 gene mutation: 149-152delCAGG + insTGTCCAAT. One individual (IV:1) is profoundly hearing impaired and has normal vestibular function and retinitis punctata albescens (RPA). The other individual is also profoundly hearing impaired, but has well-developed speech, vestibular areflexia, and retinitis pigmentosa sine pigmento (RPSP). These findings suggest that Usher syndrome type III can be clinically misdiagnosed as either Usher type I or II; that Usher syndrome patients who are profoundly hearing impaired and have normal vestibular function should be tested for USH3 mutations; and that RPA and RPSP can occur as fundoscopic manifestations of pigmentary retinopathy in Usher syndrome.

  13. Psychological Type of Person-Centered Counselors.

    Science.gov (United States)

    Robbins, Mandy; Turley, Joanne

    2016-02-01

    There are various models and approaches to counseling and psychotherapy. Important characteristics of therapists include psychological type. This study aimed to investigate the psychological type profile of person-centered counselors. The psychological type of 85 counselors (63 women, 22 men) was measured with the Francis Psychological Type Scales (FPTS). Results indicate that the FPTS can reliably measure psychological type among counselors, and the most common psychological type was introvert, intuitive, feeling, and judging (INFJ). The relation of these psychological types with a person-centered approach is further discussed.

  14. Memoization in Type-Directed Partial Evaluation

    DEFF Research Database (Denmark)

    Balat, Vincent; Danvy, Olivier

    2002-01-01

    We use a code generator—type-directed partial evaluation— to verify conversions between isomorphic types, or more precisely to verify that a composite function is the identity function at some complicated type. A typed functional language such as ML provides a natural support to express the funct......We use a code generator—type-directed partial evaluation— to verify conversions between isomorphic types, or more precisely to verify that a composite function is the identity function at some complicated type. A typed functional language such as ML provides a natural support to express...... originate in the handling of sums, which uses delimited continuations. We successfully eliminate these redundancies by extending type-directed partial evaluation with memoization capabilities. The result only works for pure functional programs, but it provides an unexpected use of code generation...... and it yields orders-of-magnitude improvements both in time and in space for type isomorphisms. Basic Research in Computer Science (www. brics. dk), funded by the Danish National Research Foundation....

  15. Biocompatible choline based ionic salts: Solubility in short-chain alcohols

    International Nuclear Information System (INIS)

    Lopes, Joana M.; Paninho, Ana B.; Môlho, Marta F.; Nunes, Ana V.M.; Rocha, Angelo; Lourenço, Nuno M.T.; Najdanovic-Visak, Vesna

    2013-01-01

    Highlights: • Biocompatible ionic liquids based on choline esters were synthesized in this work. • Solubility of choline and choline esters based ionic salt in alcohols were measured. • Activity coefficients were calculated. • Experimental data were correlated by means of the semi-empirical Grant equation. -- Abstract: In this work, we report data on solubility of choline chloride and choline acetate in short-chain linear alcohols (ethanol, 1-propanol and 1-butanol) at various temperatures. Furthermore, we synthesize two choline derivatives: hydrogen choline chloride glutarate ([CholGlut][Cl]) and hydrogen choline chloride succinate ([CholSucc][Cl]). Their characterization and solubility in short-chain alcohols as a function of temperature are also included. Activity coefficients were calculated and their comparisons with ideal solutions were discussed. The experimental data were correlated successfully by means of the semi-empirical Grant equation

  16. Assay for methadone

    International Nuclear Information System (INIS)

    1980-01-01

    An improved radioimmunoassay for methadone is described using a novel antigen, antibody and labelled methadone derivatives. The preparation of a hemi-ester antigen is described by reacting a methadone derivative with succinic anhydride or glutaric anhydride; this hapten is then covalently bonded through the carboxyl group to bovine serum albumin. An antibody specific to methadone is produced by inoculating a host animal with the above antigen. The unknown amount of methadone in a sample is then determined by mixing the sample with a known amount of radiolabelled methadone derivative and the above antibody and comparing the degree of binding to a standard curve obtained by mixing the antibody with known amounts of methadone and fixed amounts of labelled methadone derivative. The radioimmunoassay was used to measure methadone levels in urine from individuals attending a methadone clinic. (U.K.)

  17. Extinction efficiencies of coated absorbing aerosols measured by cavity ring down aerosol spectrometry

    Directory of Open Access Journals (Sweden)

    E. Segre

    2008-03-01

    Full Text Available In this study, we measure the extinction efficiency at 532 nm of absorbing aerosol particles coated with a non-absorbing solid and liquid organic shell with coating thickness varying between 5 and 100 nm using cavity ring down aerosol spectrometry. For this purpose, we use nigrosin, an organic black dye, as a model absorbing core and two non-absorbing organic substances as shells, glutaric acid (GA and Di-Ethyl-Hexyl-Sebacate (DEHS. The measured behavior of the coated particles is consistent with Mie calculations of core-shell particles. Errors between measured and calculated values for nigrosin coated with GA and DEHS are between 0.5% and 10.5% and between 0.5% and 9%, respectively. However, it is evident that the calculations are in better agreement with the measured results for thinner coatings. Possible reasons for these discrepancies are discussed.

  18. Preparation of Conductive Organometallic Complexes and Their Pastes or Inks Using the Electron Beam Apparatus

    International Nuclear Information System (INIS)

    Gu, Ja Min; Lee, Hyosun; Lee, Byung Cheol; Park, Ji Hyun

    2011-01-01

    We have synthesized the silver and copper complexes using the ligands, amine derivatives Ν-methylhydantoin amine, Ν-triethanol amine, the copper complexes are prepared depending on the equivalents of starting materials, however the silver complexes failed. In case of terephthalic acid, glutaric acid, we have synthesized silver and copper complexes successfully. We have measured conductivity of silver and copper complexes paste and ink themselves by thermal reduction using PULSE UV method. A couple of synthesized copper complex's paste have shown some resistance which is not enough for the conductive materials. Commercially silver pastes composed of silver oxide and silver salt of carboxylic acid, applied to the printed transistor circuits with suitable process, i. e. thermal reduction. This process substituted for electron beam brings a simplification of process, economical, environmental friendly process and a development of in the application of flexible substrate

  19. Horseradish Peroxidase (HRP Immobilized Poly(aniline-co-m-aminophenol Film Electrodes–fabrication and Evaluation as Hydrogen Peroxide Sensor

    Directory of Open Access Journals (Sweden)

    Seong-Ho Choi

    2007-05-01

    Full Text Available Enzyme modified electrodes were fabricated with poly(aniline-co-m-aminophenol. Electrochemical polymerization of aniline and m-aminophenol wasperformed to get the film of copolymer on the surface of gold electrode. Modifiedelectrodes were fabricated by two methods, physical entrapment and covalent cross-linking.In one of the method, gold nanoparticles were loaded into the copolymer film andhorseradish peroxidase (HRP was immobilized into the Au nanoparticle loaded copolymerfilm through physical entrapment. In the other method, the amino and -OH groups in thecopolymer are utilized to form covalent functionalization with HRP via glutaric dialdehydeas cross-linker/mediator. The conducting copolymer/enzyme modified electrodes preparedby physical entrapment/covalent functionalization of enzyme were tested forelectrocatalytic activities towards sensing of H2O2. Amperometric results indicate thatenzyme modified electrode via physical entrapment possesses better electrocatalyticperformance over covalent functionalized enzyme electrode.

  20. Isolation and molecular characterization of type I and type II feline coronavirus in Malaysia

    Directory of Open Access Journals (Sweden)

    Amer Alazawy

    2012-11-01

    Full Text Available Abstract Background Feline infectious peritonitis virus (FIPV and feline enteric coronavirus (FECV are two important coronaviruses of domestic cat worldwide. Although FCoV is prevalent among cats; the fastidious nature of type I FCoV to grow on cell culture has limited further studies on tissue tropism and pathogenesis of FCoV. While several studies reported serological evidence for FCoV in Malaysia, neither the circulating FCoV isolated nor its biotypes determined. This study for the first time, describes the isolation and biotypes determination of type I and type II FCoV from naturally infected cats in Malaysia. Findings Of the total number of cats sampled, 95% (40/42 were RT-PCR positive for FCoV. Inoculation of clinical samples into Crandell feline kidney cells (CrFK, and Feline catus whole fetus-4 cells (Fcwf-4, show cytopathic effect (CPE characterized by syncytial cells formation and later cell detachment. Differentiation of FCoV biotypes using RT-PCR assay revealed that, 97.5% and 2.5% of local isolates were type I and type II FCoV, respectively. These isolates had high sequence homology and phylogenetic similarity with several FCoV isolates from Europe, South East Asia and USA. Conclusions This study reported the successful isolation of local type I and type II FCoV evident with formation of cytopathic effects in two types of cell cultures namely the CrFK and Fcwf-4 , where the later cells being more permissive. However, the RT-PCR assay is more sensitive in detecting the antigen in suspected samples as compared to virus isolation in cell culture. The present study indicated that type I FCoV is more prevalent among cats in Malaysia.

  1. Preventing Type 2 Diabetes

    Science.gov (United States)

    ... Sexual, & Bladder Problems Clinical Trials Preventing Type 2 Diabetes Perhaps you have learned that you have a ... I lower my chances of developing type 2 diabetes? Research such as the Diabetes Prevention Program shows ...

  2. Different Types of Lupus

    Science.gov (United States)

    ... Twitter Facebook Pinterest Email Print Different types of lupus Lupus Foundation of America September 18, 2017 Resource ... lupus. Learn more about each type below. Systemic lupus erythematosus Systemic lupus is the most common form ...

  3. Beyond PrPres type 1/Type 2 dichotomy in Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    Uro-Coste, E.; Cassard, H.; Simon, S.; Lugan, S.; Bilheude, J.M.; Perret-Liaudet, A.; Ironside, J.E.; Haik, S.; Basset-Leobon, C.; Lacroux, C.; Peoch, K.; Streichenberger, N.; Langeveld, J.P.M.; Head, M.W.; Grassi, J.; Hauw, J.J.; Schelcher, F.; Delisle, M.B.; Andreoletti, O.

    2008-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct

  4. Amnioinfusion in term labor with low amniotic fluid due to rupture of membranes: a new indication.

    Science.gov (United States)

    Miño, M; Puertas, A; Miranda, J A; Herruzo, A J

    1999-01-01

    The null hypothesis was that the use of intrapartum amnioinfusion to induce term labor because of premature rupture of membranes when labor was complicated by low amniotic fluid volume due to vaginal loss would not improve fetal heart rate patterns, decrease the incidence of operative delivery, or improve neonatal acid-base status. 200 term pregnancies with low amniotic fluid due to vaginal loss were randomly chosen to receive intrapartum amnioinfusion or standard obstetric care without amnioinfusion. Fetal heart rate pattern, method of delivery and neonatal acid-base status were compared with Student's t test, chi-squared analysis, Mann-Whitney U- or Fisher's exact test. When amnioinfusion was used, the fetuses had lower rates of variable (74 vs. 91%, Pamnioinfusion, and babies in this group had lower rates of neonatal acidemia of arterial (22 vs. 36%, PAmnioinfusion improved fetal heart rate pattern, lowered the incidence of operative delivery, and improved neonatal acid-base status in term labor complicated by low amniotic fluid due to vaginal loss.

  5. Neonatal metabolic acidosis at birth: In search of a reliable marker.

    Science.gov (United States)

    Racinet, C; Ouellet, P; Charles, F; Daboval, T

    2016-06-01

    A newborn may present acidemia on the umbilical artery blood which can result from respiratory acidosis or metabolic acidosis or be of mixed origin. Currently, in the absence of a satisfactory definition, the challenge is to determine the most accurate marker for metabolic acidosis, which can be deleterious for the neonate. We reviewed the methodological and physiological aspects of the perinatal literature to search for the best marker of NMA. Base deficit and pH have been criticized as the standard criteria to predict outcome. The proposed threshold of pathogenicity is not based on convincing studies. The algorithms of various blood gas analyzers differ and do not take into account the specific neonatal acid-base profile. Birth-related neonatal eucapnic pH is described as the most pertinent marker of NMA at birth. The various means of calculating this value and the level below which it seems to play a possible pathogenic role are presented. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  6. Revista de tesis de la Facultad de Medicina de Bogotá

    Directory of Open Access Journals (Sweden)

    Facultad de Medicina Revista

    1943-08-01

    Full Text Available Estudio estadístico y radiológico de la esteomielitis infantil en Bogotá. Tesis de grado. Declarada meritoria. 1943. Por Alejandro Isaza Botero / Estudio experimental sobre la acción rítmica de la progesterona y la autodefensa antitumoral. Tesis de grado, 1943. Declarada meritoria. Por Guillermo Nieto Cano / La piroterapia de la corea de Sidenham. Tesis de grado. 1943. Por Servio Tulio Acuña A. / Las anquilosis traumáticas del codo y su tratamiento. Tesis de grado. 1943. Por Hernando Ordóñez F. / Reacción de Vogel para el diagnóstico precoz del embarazo y sero-amino-acidemia (Contribución a su estudio. Tesis de grado. 1943. Por Víctor Dávila González / Algunas consideraciones sobre protección infantil. Tesis de grado. 1943. - Por Juan Pablo Botero R.

  7. [What you should know of the arterial blood gases during the watch].

    Science.gov (United States)

    Márquez-González, Horacio; Pámanes-González, Jesús; Márquez-Flores, Horacio; Gómez-Negrete, Alonso; Muñoz-Ramírez, Mireya C; Villa-Romero, Antonio Rafael

    2012-01-01

    Gasometry is the measurement of dissolved gases in the blood, by measuring pH, carbon dioxide pressure (pCO(2)), serum bicarbonate (HCO(3-)), and lactate and serum electrolytes: sodium, potassium and chlorine you can make a diagnosis, etiology and treatment in the critically ill patient. The aim is to provide five steps for the interpretation of blood gases by: 1. The definition of acidemia or acidosis, or alkalemia or alkalosis. 2. Defining the metabolic component or respiratory. 3. To determine the anion gap; levels above 15 ± 2 determine other likely causes of excess anions (methanol, uremia, diabetic ketoacidosis, paraldehyde, ionized, lactic acidosis, ethylene glycol and salicylates. 4. Compensation, using the Winter formula. 5. The delta gap, with the formula for determining intrinsic and metabolic alkalosis. When anion gap is normal, is calculated urinary anion gap; the value is negative if the loss is extrarenal, contrary to the positive result is renal etiology.

  8. Automated Screening for Three Inborn Metabolic Disorders: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Kavitha S

    2006-12-01

    Full Text Available Background: Inborn metabolic disorders (IMDs form a large group of rare, but often serious, metabolic disorders. Aims: Our objective was to construct a decision tree, based on classification algorithm for the data on three metabolic disorders, enabling us to take decisions on the screening and clinical diagnosis of a patient. Settings and Design: A non-incremental concept learning classification algorithm was applied to a set of patient data and the procedure followed to obtain a decision on a patient’s disorder. Materials and Methods: Initially a training set containing 13 cases was investigated for three inborn errors of metabolism. Results: A total of thirty test cases were investigated for the three inborn errors of metabolism. The program identified 10 cases with galactosemia, another 10 cases with fructosemia and the remaining 10 with propionic acidemia. The program successfully identified all the 30 cases. Conclusions: This kind of decision support systems can help the healthcare delivery personnel immensely for early screening of IMDs.

  9. Cerebral oximetry monitoring in the management of severe hypoxaemia associated with transposition of the great arteries with balloon atrial septostomy.

    Science.gov (United States)

    Pérez Moreno, J C; Nájera Losada, D C; Sanabria Carretero, P; Paredes Lacave, Á; Benito Bartolomé, F

    2018-05-01

    Transposition of the great arteries (D-TGA) is one of the most common congenital heart diseases requiring neonatal surgical intervention. In the desperately ill neonate with TGA and the resultant hypoxaemia, acidemia, and congestive heart failure, improvement is often obtained with balloon atrial septostomy (BAS). Current methods employed to evaluate oxygen delivery and tissue consumption are frequently nonspecific. Near infrared spectroscopy (NIRS) allows a continuous non-invasive measurement of tissue oxygenation which reflects perfusion status in real time. Because little is known about the direct effect of BAS on the neonatal brain and on cerebral oxygenation, we measured the effectiveness of BAS in two patients with D-TGA using NIRS before and after BAS. We concluded BAS improves cerebral oxygen saturation in neonates with D-TGA. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Influence of gas stunning and halal slaughter (no stunning) on rabbits welfare indicators and meat quality.

    Science.gov (United States)

    Nakyinsige, K; Sazili, A Q; Zulkifli, I; Goh, Y M; Abu Bakar, F; Sabow, A B

    2014-12-01

    This study assessed the effect of gas stunning which has not been conducted until now in comparison with slaughter without stunning on the welfare and meat quality of rabbits. Eighty male New Zealand White rabbits were divided into two groups of 40 animals and subjected to either halal slaughter without stunning (HS) or gas stunning using 61.4% CO2, 20.3% oxygen and 18.3 % nitrogen (GS). Analysis of the sticking blood revealed that both slaughter procedures caused a substantial increase in the levels of catecholamines, hypercalcemia, hyperglycemia, lactic acidemia and an increase in enzyme activities. The ultimate pH of the Longissimus lumborum muscle did not differ between treatments. GS exhibited higher lightness and cooking loss, and lower glycogen and MFI than HS. This indicates that both GS and HS can be significant stressors although the amount of stress may be below the threshold to negatively affect rabbit meat quality. Copyright © 2014 Elsevier Ltd. All rights reserved.

  11. A case of multiple organ failure induced by postoperative radiation therapy probably evoking oxidative stress

    International Nuclear Information System (INIS)

    Soejima, Akinori; Ishizuka, Shynji; Suzuki, Michihiko; Minoshima, Shinobu; Nakabayashi, Kimimasa; Kitamoto, Kiyoshi; Nagasawa, Toshihiko

    1995-01-01

    In recent years, several laboratories have suggested that serum levels of antioxidant activity and redox balance are reduced in patients with chronic renal failure. Some clinical reports have also proposed that defective serum antioxidative enzymes may contribute to a certain uremic toxicity through peroxidative cell damage. A 48-year-old woman was referred to us from the surgical department of our hospital because of consciousness disturbance, panctytopenia and acute acceleration of chronic azotemia after postoperative radiation therapy. We diagnosed acute acceleration of chronic renal failure with severe acidemia and started hemodialysis therapy immediately. Two days after admission to our department, she developed upper abdominal sharp pain and bradyarrhythmia. Serum amylase activity was elevated markedly and the ECG finding showed myocardial ischemia. On the 24th hospital day these complications were treated successfully with conservative therapy and hemodialysis. We considered that radiation therapy in this patient with chronic renal failure evoked marked oxidative stress and that deficiency of transferrin played an important role in peroxidative cell damage. (author)

  12. Facts about Type 2

    Medline Plus

    Full Text Available ... About Type 2 Recently Diagnosed Treatment and Care Blood Glucose Control Complications Medication Doctors, Nurses & More Enroll in the Living With Type 2 Diabetes Program donate en -- A Future Without Diabetes - a- ...

  13. Comparison of a newly developed binary typing with ribotyping and multilocus sequence typing methods for Clostridium difficile.

    Science.gov (United States)

    Li, Zhirong; Liu, Xiaolei; Zhao, Jianhong; Xu, Kaiyue; Tian, Tiantian; Yang, Jing; Qiang, Cuixin; Shi, Dongyan; Wei, Honglian; Sun, Suju; Cui, Qingqing; Li, Ruxin; Niu, Yanan; Huang, Bixing

    2018-04-01

    Clostridium difficile is the causative pathogen for antibiotic-related nosocomial diarrhea. For epidemiological study and identification of virulent clones, a new binary typing method was developed for C. difficile in this study. The usefulness of this newly developed optimized 10-loci binary typing method was compared with two widely used methods ribotyping and multilocus sequence typing (MLST) in 189 C. difficile samples. The binary typing, ribotyping and MLST typed the samples into 53 binary types (BTs), 26 ribotypes (RTs), and 33 MLST sequence types (STs), respectively. The typing ability of the binary method was better than that of either ribotyping or MLST expressed in Simpson Index (SI) at 0.937, 0.892 and 0.859, respectively. The ease of testing, portability and cost-effectiveness of the new binary typing would make it a useful typing alternative for outbreak investigations within healthcare facilities and epidemiological research. Copyright © 2018 Elsevier B.V. All rights reserved.

  14. Typing Style and the Use of Different Sources of Information during Typing: An Investigation Using Self-Reports.

    Science.gov (United States)

    Rieger, Martina; Bart, Victoria K E

    2016-01-01

    We investigated to what extent different sources of information are used in typing on a computer keyboard. Using self-reports 10 finger typists and idiosyncratic typists estimated how much attention they pay to different sources of information during copy typing and free typing and how much they use them for error detection. 10 finger typists reported less attention to the keyboard and the fingers and more attention to the template and the screen than idiosyncratic typists. The groups did not differ in attention to touch/kinaesthesis in copy typing and free typing, but 10 finger typists reported more use of touch/kinaesthesis in error detection. This indicates that processing of tactile/kinaesthetic information may occur largely outside conscious control, as long as no errors occur. 10 finger typists reported more use of internal prediction of movement consequences for error detection than idiosyncratic typists, reflecting more precise internal models. Further in copy typing compared to free typing attention to the template is required, thus leaving less attentional capacity for other sources of information. Correlations showed that higher skilled typists, regardless of typing style, rely more on sources of information which are usually associated with 10 finger typing. One limitation of the study is that only self-reports were used. We conclude that typing task, typing proficiency, and typing style influence how attention is distributed during typing.

  15. Early onset type 2 diabetes

    DEFF Research Database (Denmark)

    Bo, A; Thomsen, R W; Nielsen, J S

    2018-01-01

    was more frequent and meeting physical activity recommendations less likely in persons with early-onset type 2 DM. CONCLUSIONS: We found a clear age-gradient, with increasing prevalence of clinical and behavioural risk factors the younger the onset age of type 2 DM. Younger persons with early-onset type 2......AIM: To examine the association between early onset of type 2 diabetes (DM) and clinical and behavioural risk factors for later diabetes complications. METHODS: We conducted a cross-sectional study of 5115 persons with incident type 2 DM enrolled during 2010-2015 in the Danish Centre for Strategic...... Research in Type 2 Diabetes-cohort. We compared risk factors at time of diagnosis among those diagnosed at ≤45 years (early-onset) with diagnosis age 46-55, 56-65 (average-onset = reference), 66-75, and >75 years (late-onset). Prevalence ratios (PRs) were computed using Poisson regression. RESULTS: Poor...

  16. Types of Stem Cells

    Science.gov (United States)

    ... Stem Cell Glossary Search Toggle Nav Types of Stem Cells Stem cells are the foundation from which all ... Learn About Stem Cells > Types of Stem Cells Stem cells Stem cells are the foundation for every organ ...

  17. Study of araldite in edge protection of n-type and p-type surface barrier detectors

    International Nuclear Information System (INIS)

    Alencar, M.A.V.; Jesus, E.F.O.; Lopes, R.T.

    1995-01-01

    The aim of this work is the realization of a comparative study between the surface barrier detectors performance n and type using the epoxy resin Araldite as edge protection material with the purpose of determining which type of detector (n or p) the use of Araldite is more indicated. The surface barrier detectors were constructed using n and p type silicon wafer with resistivity of 3350Ω.cm and 5850 Ω.cm respectively. In the n type detectors, the metals used as ohmic and rectifier contacts were the Al and Au respectively, while in the p type detectors, the ohmic and rectifier contacts were Au and Al. All metallic contacts were done by evaporation in high vacuum (∼10 -4 Torr) and with deposit of 40 μm/cm 2 . The obtained results for the detectors (reverse current of -350nA and resolution from 21 to 26 keV for p type detectors and reserve current of 1μA and resolution from 44 to 49 keV for n type detectors) tend to demonstrate that use of epoxy resin Araldite in the edge protection is more indicated to p type surface barrier detectors. (author). 3 refs., 4 figs., 1 tab

  18. Typing for HLA-D/DR associated DP-antigens with the primed lymphocyte typing (PLT) technique

    DEFF Research Database (Denmark)

    Morling, N; Jakobsen, B K; Platz, P

    1980-01-01

    A total of 74 healthy unrelated random individuals and 36 patients with juvenile rheumatoid arthritis (JRA) were typed for HLA-D antigens with the homozygous typing cell technique and typed for HLA-D/DR associated DP-antigens with the primed lymphocyte typing (PLT) technique. All patients and some...... of the controls were also HLA-DR typed with a limited battery of anti-DR sera. Selected PLT-cells, specific for the HLA-D/DR antigens D/DRw1-8 and the local specificity D"H" were used. The results of the PLT-experiments were evaluated with the Normalized Median Response (NMR) method and the further procedure...

  19. Facts about Type 2

    Medline Plus

    Full Text Available ... Enroll in the Living WIth Type 2 Diabetes Program Gestational What is Gestational Diabetes? How to Treat ... Enroll in the Living WIth Type 2 Diabetes Program In It Together We Can Help Center for ...

  20. Facts about Type 2

    Medline Plus

    Full Text Available ... and Type 2 Diabetes Know Your Rights Employment Discrimination Health Care Professionals Law Enforcement Driver's License For ... 1 Type 2 Gestational Myths Statistics Common Terms Genetics Living With Diabetes Recently Diagnosed Treatment & Care Complications ...

  1. Facts about Type 2

    Medline Plus

    Full Text Available ... People Working to Stop Diabetes Common Terms Diabetes Statistics Infographics Living with Diabetes Home Recently Diagnosed Where ... Basics Symptoms Type 1 Type 2 Gestational Myths Statistics Common Terms Genetics Living With Diabetes Recently Diagnosed ...

  2. Facts about Type 2

    Medline Plus

    Full Text Available ... Your Risk Alert Day Diabetes Basics Home Symptoms Diagnosis America's Diabetes Challenge Type 1 Type 2 Facts ... Online Community Site Menu Are You at Risk? Diagnosis Lower Your Risk Risk Test Alert Day Prediabetes ...

  3. Facts about Type 2

    Medline Plus

    Full Text Available ... Type 1 Diabetes Get Started Safely Get And Stay Fit Types of Activity Weight Loss Assess Your Lifestyle Getting Started Food Choices In My Community Home Find Your Local Office Find your local diabetes ...

  4. Facts about Type 2

    Medline Plus

    Full Text Available ... 2 Type 2 diabetes is the most common form of diabetes. In type 2 diabetes, your body ... Sign Up Thank you for signing up ' + ' '); $('.survey-form').show(); }, success: function (data) { $('#survey-errors').remove(); $('.survey- ...

  5. Facts about Type 2

    Medline Plus

    Full Text Available ... Us in the Fight for a Cure Your tax-deductible gift today can fund critical diabetes research ... Care of Type 2 Diabetes This two-page introduction to type 2 diabetes is in PDF format ...

  6. Inborn Errors of Metabolism with Acidosis: Organic Acidemias and Defects of Pyruvate and Ketone Body Metabolism.

    Science.gov (United States)

    Schillaci, Lori-Anne P; DeBrosse, Suzanne D; McCandless, Shawn E

    2018-04-01

    When a child presents with high-anion gap metabolic acidosis, the pediatrician can proceed with confidence by recalling some basic principles. Defects of organic acid, pyruvate, and ketone body metabolism that present with acute acidosis are reviewed. Flowcharts for identifying the underlying cause and initiating life-saving therapy are provided. By evaluating electrolytes, blood sugar, lactate, ammonia, and urine ketones, the provider can determine the likelihood of an inborn error of metabolism. Freezing serum, plasma, and urine samples during the acute presentation for definitive diagnostic testing at the provider's convenience aids in the differential diagnosis. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Facts about Type 2

    Medline Plus

    Full Text Available ... Infographics Living with Diabetes Home Recently Diagnosed Where Do I Begin With Type2? Living With Type 1 ... Diabetes at School Tour de Cure Women's Series Do-It-Yourself Fundraising Become a Volunteer American Diabetes ...

  8. Multilocus Sequence Typing

    OpenAIRE

    Belén, Ana; Pavón, Ibarz; Maiden, Martin C.J.

    2009-01-01

    Multilocus sequence typing (MLST) was first proposed in 1998 as a typing approach that enables the unambiguous characterization of bacterial isolates in a standardized, reproducible, and portable manner using the human pathogen Neisseria meningitidis as the exemplar organism. Since then, the approach has been applied to a large and growing number of organisms by public health laboratories and research institutions. MLST data, shared by investigators over the world via the Internet, have been ...

  9. Making Type Inference Practical

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Oxhøj, Nicholas; Palsberg, Jens

    1992-01-01

    We present the implementation of a type inference algorithm for untyped object-oriented programs with inheritance, assignments, and late binding. The algorithm significantly improves our previous one, presented at OOPSLA'91, since it can handle collection classes, such as List, in a useful way. Abo......, the complexity has been dramatically improved, from exponential time to low polynomial time. The implementation uses the techniques of incremental graph construction and constraint template instantiation to avoid representing intermediate results, doing superfluous work, and recomputing type information....... Experiments indicate that the implementation type checks as much as 100 lines pr. second. This results in a mature product, on which a number of tools can be based, for example a safety tool, an image compression tool, a code optimization tool, and an annotation tool. This may make type inference for object...

  10. Case 22:Type II diabetes

    Science.gov (United States)

    Diabetes mellitus is characterized by elevated blood glucose levels. It is composed of two types depending on the pathogenesis. Type I diabetes is characterized by insulin deficiency and usually has its onset during childhood or teenage years. This is also called ketosis-prone diabetes. Type II diab...

  11. Research into basic rocks types

    International Nuclear Information System (INIS)

    1993-06-01

    Teollisuuden Voima Oy (TVO) has carried out research into basic rock types in Finland. The research programme has been implemented in parallel with the preliminary site investigations for radioactive waste disposal in 1991-1993. The program contained two main objectives: firstly, to study the properties of the basic rock types and compare those with the other rock types under the investigation; secondly, to carry out an inventory of rock formations consisting of basic rock types and suitable in question for final disposal. A study of environmental factors important to know regarding the final disposal was made of formations identified. In total 159 formations exceeding the size of 4 km 2 were identified in the inventory. Of these formations 97 were intrusive igneous rock types and 62 originally extrusive volcanic rock types. Deposits consisting of ore minerals, industrial minerals or building stones related to these formations were studied. Environmental factors like natural resources, protected areas or potential for restrictions in land use were also studied

  12. Diabetes mellitus type 1

    OpenAIRE

    Tøraasen, Lisa Vangen; Al-Sultan, Zainab

    2014-01-01

    Bacheloroppgave i sykepleie, 2014 Hvert år blir rundt 600 nordmenn diagnostisert med sykdommen diabetes type 1, og Norge er et av landene i verden med størst andel av barnediabetes. I dag er det 15 000- 20 000 personer i Norge som har diabetes type 1, og antall barn som får diabetes har fordoblet seg de siste 30 årene (Diabetesforbundet, 2014). Problemstillingen vår gikk ut på hvordan sykepleiere kan veilede og undervise ungdom med nyoppdaget diabetes type på sykehus. Ut i fra litteraturst...

  13. Diabetes mellitus type 1

    OpenAIRE

    Tøraasen, Lisa Vangen; Al-Sultan, Zainab

    2014-01-01

    Hvert år blir rundt 600 nordmenn diagnostisert med sykdommen diabetes type 1, og Norge er et av landene i verden med størst andel av barnediabetes. I dag er det 15 000- 20 000 personer i Norge som har diabetes type 1, og antall barn som får diabetes har fordoblet seg de siste 30 årene (Diabetesforbundet, 2014). Problemstillingen vår gikk ut på hvordan sykepleiere kan veilede og undervise ungdom med nyoppdaget diabetes type på sykehus. Ut i fra litteraturstudiet har vi arbeidet oss frem for å ...

  14. Parametric compositional data types

    DEFF Research Database (Denmark)

    Bahr, Patrick; Hvitved, Tom

    2012-01-01

    In previous work we have illustrated the benefits that compositional data types (CDTs) offer for implementing languages and in general for dealing with abstract syntax trees (ASTs). Based on Swierstra's data types \\'a la carte, CDTs are implemented as a Haskell library that enables the definition...... of recursive data types and functions on them in a modular and extendable fashion. Although CDTs provide a powerful tool for analysing and manipulating ASTs, they lack a convenient representation of variable binders. In this paper we remedy this deficiency by combining the framework of CDTs with Chlipala...

  15. Action Type Deontic Logic

    DEFF Research Database (Denmark)

    Bentzen, Martin Mose

    2014-01-01

    A new deontic logic, Action Type Deontic Logic, is presented. To motivate this logic, a number of benchmark cases are shown, representing inferences a deontic logic should validate. Some of the benchmark cases are singled out for further comments and some formal approaches to deontic reasoning...... are evaluated with respect to the benchmark cases. After that follows an informal introduction to the ideas behind the formal semantics, focussing on the distinction between action types and action tokens. Then the syntax and semantics of Action Type Deontic Logic is presented and it is shown to meet...

  16. Type 2 diabetes models

    DEFF Research Database (Denmark)

    Gram, Dorte Xenia

    2012-01-01

    This chapter deals with type 2 diabetes in vivo models and techniques suitable for testing new anti-diabetic compounds. In particular, the testing of TRP antagonist for beneficial effects against type 2 diabetes is considered. There are many choices of both in vitro techniques and in vivo models......, impaired glucose tolerance, impaired insulin secretion, and insulin resistance in vivo and should, thus, be sufficient to demonstrate preclinical proof of concept of a TRP antagonist in type 2 diabetes in rodents. The experiments are suggestions and could be replaced or supplemented by others....

  17. Facts about Type 2

    Medline Plus

    Full Text Available ... org > Diabetes Basics > Type 2 Share: Print Page Text Size: A A A Listen En Español Facts ... Type 2 Education Series Hear audio clips and full recordings of past Q&A events at your ...

  18. Facts about Type 2

    Medline Plus

    Full Text Available ... En Español Type 1 Type 2 About Us Online Community Meal Planning Sign In Search: Search More ... Generation of Brilliant Researchers Diabetes Pro: Professional Resources Online We Support Your Doctor Clinical Practice Guidelines Patient ...

  19. Facts about Type 2

    Medline Plus

    Full Text Available ... Type 2 Diabetes Risk Test Lower Your Risk Healthy Eating Overweight Smoking High Blood Pressure Physical Activity High ... type 2 can control their blood glucose with healthy eating and being active. But, your doctor may need ...

  20. Dopplervelocimetria dos Compartimentos Arterial e Venoso da Circulação Fetal e Umbilical em Gestação de Alto-Risco: Análise dos Resultados Perinatais Dopplervelocimetry of the Arterial and Venous Compartments of the Fetal and Umbilical Circulation in High-Risk Pregnancy: Perinatal Results

    Directory of Open Access Journals (Sweden)

    Joelma Queiroz Andrade

    2002-01-01

    Full Text Available Objetivos: estudar o perfil hemodinâmico fetal em gestantes de alto risco e avaliar a relação das suas alterações com os resultados perinatais. Métodos: realizou-se estudo prospectivo transversal em 108 gestantes atendidas no ambulatório de pré-natal da Clínica Obstétrica do HCFMUSP. Tais gestantes foram encaminhadas ao Setor de Avaliação de Vitalidade Fetal, onde foram submetidas à dopplervelocimetria das artérias umbilicais, cerebral média, aorta, ducto venoso e veia cava inferior. Os critérios de inclusão foram gestantes que apresentavam qualquer doença ou intercorrência na gravidez encaminhadas para avaliação da vitalidade fetal, nas quais a resolução da gestação ocorreu nas próximas 24 horas após os exames. Foram excluídas as pacientes com gestação gemelar ou com malformação fetal. Resultados: as repercussões hemodinâmicas na circulação fetal foram demonstradas pela observação de alterações na dopplervelocimetria das artérias umbilicais, aorta, artéria cerebral média, ducto venoso e veia cava inferior. Valores alterados do índice de pulsatilidade (IP da artéria umbilical foram observados em 25,9% dos casos, da aorta em 24,0% e da artéria cerebral média em 34,2%. O IPV do ducto venoso estava alterado em 18,2% dos casos e o da veia cava inferior em 46,6%. Os segmentos da circulação fetal que melhor se correlacionaram com os resultados perinatais foram a artéria umbilical e o ducto venoso. O IP alterado da artéria umbilical correlacionou-se significativamente com índices de Apgar de 1º minuto inferior a 7 em 42,8% e com necessidade de UTI neonatal em 50,0% dos casos. O IPV (indice de pulsatilidade para veias do ducto venoso alterado apresentou associação significativa com o índice de Apgar de 1º minuto inferior a 7 (52,6%, Apgar de 5º minuto inferior a 7 (15,7%, acidemia no nascimento (60,0%, necessidade de UTI neonatal (52,6% e óbito neonatal (21,1%. Os valores de predição da altera

  1. Distinct clinical characteristics and therapeutic modalities for diabetic ketoacidosis in type 1 and type 2 diabetes mellitus.

    Science.gov (United States)

    Kamata, Yuji; Takano, Koji; Kishihara, Eriko; Watanabe, Michiko; Ichikawa, Raishi; Shichiri, Masayoshi

    2017-02-01

    Patients with type 1 diabetes often develop diabetic ketoacidosis (DKA). Reportedly, DKA in type 2 diabetes has higher mortality despite its limited occurrence. The exact clinical characteristics and therapeutic modalities yielding successful outcomes in DKA type 2 diabetes remain unknown. This retrospective study compared the clinical features and detailed treatment of consecutive type 1 and type 2 diabetes patients hospitalized with DKA between January 2001 and December 2014. We report on 127 patients with type 1 and 74 patients with type 2 diabetes whose DKA was successfully treated. The most frequent precipitating cause for DKA was infectious disease for patients with type 1 diabetes and consumption of sugar-containing beverages for those with type 2 diabetes. Type 2 diabetes patients showed higher mean plasma glucose levels than those with type 1 diabetes (48.4±21.6, vs. 37.1±16.4mmol/l, P1) and higher serum creatinine, blood urea nitrogen, and hemoglobin levels, which normalized after DKA resolution. Compared with type 1 diabetes patients, those with type 2 diabetes required distinctly higher daily total insulin dosage (35.9±37.0U, vs. 20.2±23.3U, P1), larger replacement fluid volumes (4.17±2.69L, vs. 2.29±1.57L, P1) and greater potassium supplementation (23.9±36.5mEq, vs. 11.2±17.9mEq, P1) to resolve DKA and reduce plasma glucose level to ≤16.7mmol/l. DKA patients with type 2 diabetes required management with a modified treatment protocol to resolve their profound hyperglycemia and dehydration compared with those with type 1 diabetes. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. Type-Directed Partial Evaluation

    DEFF Research Database (Denmark)

    Danvy, Olivier

    1998-01-01

    Type-directed partial evaluation uses a normalization function to achieve partial evaluation. These lecture notes review its background, foundations, practice, and applications. Of specific interest is the modular technique of offline and online type-directed partial evaluation in Standard ML...

  3. Type-Directed Partial Evaluation

    DEFF Research Database (Denmark)

    Danvy, Olivier

    1998-01-01

    Type-directed partial evaluation uses a normalization function to achieve partial evaluation. These lecture notes review its background, foundations, practice, and applications. Of specific interest is the modular technique of offline and online type-directed partial evaluation in Standard ML of ...

  4. Computable Types for Dynamic Systems

    NARCIS (Netherlands)

    P.J. Collins (Pieter); K. Ambos-Spies; B. Loewe; W. Merkle

    2009-01-01

    textabstractIn this paper, we develop a theory of computable types suitable for the study of dynamic systems in discrete and continuous time. The theory uses type-two effectivity as the underlying computational model, but we quickly develop a type system which can be manipulated abstractly, but for

  5. Different Causes of Death in Patients with Myocardial Infarction Type 1, Type 2, and Myocardial Injury.

    Science.gov (United States)

    Lambrecht, Sascha; Sarkisian, Laura; Saaby, Lotte; Poulsen, Tina S; Gerke, Oke; Hosbond, Susanne; Diederichsen, Axel C P; Thygesen, Kristian; Mickley, Hans

    2018-05-01

    Data outlining the mortality and the causes of death in patients with type 1 myocardial infarction, type 2 myocardial infarction, and those with myocardial injury are limited. During a 1-year period from January 2010 to January 2011, all hospitalized patients who had cardiac troponin I measured on clinical indication were prospectively studied. Patients with at least one cardiac troponin I value >30 ng/L underwent case ascertainment and individual evaluation by an experienced adjudication committee. Patients were classified as having type 1 myocardial infarction, type 2 myocardial infarction, or myocardial injury according to the criteria of the universal definition of myocardial infarction. Follow-up was ensured until December 31, 2014. Data on mortality and causes of death were obtained from the Danish Civil Registration System and the Danish Register of Causes of Death. Overall, 3762 consecutive patients were followed for a mean of 3.2 years (interquartile range 1.3-3.6 years). All-cause mortality differed significantly among categories: Type 1 myocardial infarction 31.7%, type 2 myocardial infarction 62.2%, myocardial injury 58.7%, and 22.2% in patients with nonelevated troponin values (log-rank test; P causes, vs 42.6% in patients with type 2 myocardial infarction (P = .015) and 41.2% in those with myocardial injury (P causes of death did not differ substantially between patients with type 2 myocardial infarction and those with myocardial injury. Patients with type 2 myocardial infarction and myocardial injury exhibit a significantly higher long-term mortality compared with patients with type 1 myocardial infarction . However, most patients with type 1 myocardial infarction die from cardiovascular causes in contrast to patients with type 2 myocardial infarction and myocardial injury, in whom noncardiovascular causes of death predominate. Copyright © 2018 Elsevier Inc. All rights reserved.

  6. Linear contextual modal type theory

    DEFF Research Database (Denmark)

    Schack-Nielsen, Anders; Schürmann, Carsten

    Abstract. When one implements a logical framework based on linear type theory, for example the Celf system [?], one is immediately con- fronted with questions about their equational theory and how to deal with logic variables. In this paper, we propose linear contextual modal type theory that gives...... a mathematical account of the nature of logic variables. Our type theory is conservative over intuitionistic contextual modal type theory proposed by Nanevski, Pfenning, and Pientka. Our main contributions include a mechanically checked proof of soundness and a working implementation....

  7. DNA typing by capillary electrophoresis

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, N.

    1997-10-08

    Capillary electrophoresis is becoming more and more important in nucleic acid analysis including DNA sequencing, typing and disease gene measurements. This work summarized the background of DNA typing. The recent development of capillary electrophoresis was also discussed. The second part of the thesis showed the principle of DNA typing based on using the allelic ladder as the absolute standard ladder in capillary electrophoresis system. Future work will be focused on demonstrating DNA typing on multiplex loci and examples of disease diagnosis in the on-line format of PCR-CE. Also capillary array electrophoresis system should allow high throughput, fast speed DNA typing. Only the introduction and conclusions for this report are available here. A reprint was removed for separate processing.

  8. Type A and type behaviors and factors related to job satisfaction among male white-collar workers.

    Science.gov (United States)

    Hagihara, A; Tarumi, K; Morimoto, K

    1998-01-01

    Numerous studies have examined the health effects of Type A behavior and job satisfaction/dissatisfaction, but we know very little about the relationship between aspects of the work environment related to job satisfaction and the Type A behavior pattern. In the present study, we analyzed data concerning work Stressors, private aspects of life, and job satisfaction among male white-collar workers (n=657) in a large steel company, and identified the respective aspects of the work environment related to job satisfaction among groups divided by Type A/B behavior patterns.We found that the nature of predictors for job satisfaction varied with the behavior type. "Being not busy at work" (pjob satisfaction among the Type A workers while "working less than 10 hours per day" (pjob satisfaction among the Type workers. "Work performance is evaluated" was a significant predictor of job satisfaction among all three behavior types (A, B, and A/B).Our results can provide information useful for the creation of programs to lower the level of job dissatisfaction and mental stress depending upon the behavior type of employees.

  9. No Association of Blood Type O With Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1

    NARCIS (Netherlands)

    Nell, Sjoerd; van Leeuwaarde, Rachel S.; Pieterman, Carolina R. C.; de Laat, Joanne M.; Hermus, Ad R.; Dekkers, Olaf M.; de Herder, Wouter W.; van der Horst-Schrivers, Anouk N.; Drent, Madeleine L.; Bisschop, Peter H.; Havekes, Bas; Borel Rinkes, Inne H. M.; Vriens, Menno R.; Valk, Gerlof D.

    2015-01-01

    An association between ABO blood type and the development of cancer, in particular, pancreatic cancer, has been reported in the literature. An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested. Therefore, blood

  10. No Association of Blood Type O With Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1

    NARCIS (Netherlands)

    Nell, S.; Leeuwaarde, R.S. van; Pieterman, C.R.; Laat, J.M. de; Hermus, A.R.M.M.; Dekkers, O.M.; Herder, W.W. de; Horst-Schrivers, A.N. van der; Drent, M.L.; Bisschop, P.H.; Havekes, B.; Rinkes, I.H.; Vriens, M.R.; Valk, G.D.

    2015-01-01

    CONTEXT: An association between ABO blood type and the development of cancer, in particular, pancreatic cancer, has been reported in the literature. An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested. Therefore,

  11. No Association of Blood Type O With Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1

    NARCIS (Netherlands)

    Nell, Sjoerd; Van Leeuwaarde, Rachel S.; Pieterman, Carolina R. C.; de Laat, Joanne M.; Hermus, Ad R.; Dekkers, Olaf M.; de Herder, Wouter W.; van der Horst-Schrivers, Anouk N.; Drent, Madeleine L.; Bisschop, Peter H.; Havekes, Bas; Rinkes, Inne H. M. Borel; Vriens, Menno R.; Valk, Gerlof D.

    2015-01-01

    Context: An association between ABO blood type and the development of cancer, in particular, pancreatic cancer, has been reported in the literature. An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested. Therefore,

  12. An Imperative Type Hierarchy with Partial Products

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Schmidt, Erik Meineche

    1989-01-01

    notation for defining recursive types, that is superior to traditional type sums and products. We show how the ordering on types extends to an ordering on types with invariants. We allow the use of least upper bounds in type definitions and show how to compute upper bounds of invariants.......A type hierarchy for a programming language defines an ordering on the types such that any application for small types may be reused for all larger types. The imperative facet makes this non-trivial; the straight-forward definitions will yield an inconsistent system. We introduce a new type...... constructor, the partial product, and show how to define a consistent hierarchy in the context of fully recursive types. A simple polymorphism is derived by introducing a notion of placeholder types. By extending the partial product types to include structural invariants we obtain a particularly appropriate...

  13. Type extension trees

    DEFF Research Database (Denmark)

    Jaeger, Manfred

    2006-01-01

    We introduce type extension trees as a formal representation language for complex combinatorial features of relational data. Based on a very simple syntax this language provides a unified framework for expressing features as diverse as embedded subgraphs on the one hand, and marginal counts...... of attribute values on the other. We show by various examples how many existing relational data mining techniques can be expressed as the problem of constructing a type extension tree and a discriminant function....

  14. Creatine Kinase Activity in Patients with Diabetes Mellitus Type I and Type II

    Directory of Open Access Journals (Sweden)

    Adlija Jevrić-Čaušević

    2006-08-01

    Full Text Available Diabetes mellitus can be looked upon as an array of diseases, all of which exhibit common symptoms. While pathogenesis of IDDM (insulin dependant diabetes mellitus is well understood, the same is not true for diabetes mellitus type II. In the latter case, relative contribution of the two factors (insulin resistance or decreased insulin secretion varies individually, being highly increased in peripheral tissues and strictly dependant on insulin for glucose uptake. Moreover, in patients with diabetes mellitus type II, disbalance at the level of regulation of glucose metabolism as well as lipid metabolism has been noted in skeletal muscles. It is normal to assume that in this type of diabetes, these changes are reflected at the level of total activity of enzyme creatine kinase. This experimental work was performed on a group of 80 regular patients of Sarajevo General Hospital. Forty of those patients were classified as patients with diabetes type I and forty as patients with diabetes type II. Each group of patients was carefully chosen and constituted of equal number of males and females. The same was applied for adequate controls. Concentration of glucose was determined for each patient with GOD method, while activity of creatine kinase was determined with CK-NAC activated kit. Statistical analysis of the results was performed with SPSS software for Windows. Obtained results point out highly expressed differences in enzyme activity between two populations examined. Changes in enzyme activity are more expressed in patients with diabetes type II. Positive correlation between concentration of glucose and serum activity of the enzyme is seen in both categories of diabetic patients which is not the case for the patients in control group. At the same time, correlation between age and type of diabetes does exist . This is not followed at the level of enzyme activity or concentration of glucose.

  15. Facts about Type 2

    Medline Plus

    Full Text Available ... 2383) Give by Mail Close www.diabetes.org > Diabetes Basics > Type 2 Share: Print Page Text Size: A A A ... 2 diabetes is the most common form of diabetes. In type 2 diabetes, your body does not use insulin properly. ...

  16. Molecular Typing and Differentiation

    Science.gov (United States)

    In this chapter, general background and bench protocols are provided for a number of molecular typing techniques in common use today. Methods for the molecular typing and differentiation of microorganisms began to be widely adopted following the development of the polymerase chai...

  17. Facts about Type 2

    Medline Plus

    Full Text Available ... Honor Donation Donate by phone at 1-800-DIABETES (1-800-342-2383) Donate by mail Why Give? ... My Health Advisor Tools to Know Your Risk Diabetes Basics Symptoms Type 1 Type 2 Gestational Myths Statistics Common Terms Genetics ...

  18. Fine typing of methicillin-resistant Staphylococcus aureus isolates using direct repeat unit and staphylococcal interspersed repeat unit typing methods.

    Science.gov (United States)

    Ho, Cheng-Mao; Ho, Mao-Wang; Li, Chi-Yuan; Lu, Jang-Jih

    2015-08-01

    Methicillin-resistant Staphylococcus aureus (MRSA) typing is an important epidemiologic tool for monitoring trends and preventing outbreaks. However, the efficiency of various MRSA typing methods for each SCCmec MRSA isolate is rarely evaluated. A total of 157 MRSA isolates from four different regions in Taiwan were typed with five different molecular methods, including SCCmec typing, multilocus sequence typing (MLST), spa typing, mec-associated direct repeat unit (dru) copy number determination, and staphylococcal interspersed repeat unit (SIRU) profiling. There were four SCCmec types, eight MLST types, 15 spa types, 11 dru types, and 31 SIRU profiles. The most common type determined by each molecular typing method was SCCmec III (115 isolates, 73.2%), ST239 (99 isolates, 63.1%), t037 (107 isolates, 68.2%), 14 dru copies (76 isolates, 48.4%), and SIRU profile 3013722 (102 isolates, 65%), respectively. When using the combination of MLST, spa typing, and dru copy number, ST5-t002-4 (n = 8), ST239-t037-14 (n = 68), ST59-t437-9 (n = 9), and ST59-t437-11 (n = 6) were found to be the most common types of SCCmec types II (n = 9), III (n = 115), IV (n = 21), and VT (n = 11) isolates, respectively. SCCmec type III isolates were further classified into 11 dru types. Of the 21 SCCmec type IV isolates, 14 SIRU profiles were found. Seven SIRU patterns were observed in the 11 SCCmec type VT isolates. Different typing methods showed a similar Hunter-Gaston discrimination index among the 157 MRSA isolates. However, dru and SIRU typing methods had a better discriminatory power for SCCmec type III and SCCmec types IV and VT isolates, respectively, suggesting that dru and SIRU can be used to further type these isolates. Copyright © 2013. Published by Elsevier B.V.

  19. Compactly Supported Curvelet-Type Systems

    DEFF Research Database (Denmark)

    Rasmussen, Kenneth Niemann; Nielsen, Morten

    2012-01-01

    We study a flexible method for constructing curvelet-type frames. These curvelet-type systems have the same sparse representation properties as curvelets for appropriate classes of smooth functions, and the flexibility of the method allows us to give a constructive description of how to construct...... curvelet-type systems with a prescribed nature such as compact support in direct space. The method consists of using the machinery of almost diagonal matrices to show that a system of curvelet molecules which is sufficiently close to curvelets constitutes a frame for curvelet-type spaces. Such a system...

  20. S-TYPE AND P-TYPE HABITABILITY IN STELLAR BINARY SYSTEMS: A COMPREHENSIVE APPROACH. II. ELLIPTICAL ORBITS

    International Nuclear Information System (INIS)

    Cuntz, M.

    2015-01-01

    In the first paper of this series, a comprehensive approach has been provided for the study of S-type and P-type habitable regions in stellar binary systems, which was, however, restricted to circular orbits of the stellar components. Fortunately, a modest modification of the method also allows for the consideration of elliptical orbits, which of course entails a much broader range of applicability. This augmented method is presented here, and numerous applications are conveyed. In alignment with Paper I, the selected approach considers a variety of aspects, which comprise the consideration of a joint constraint including orbital stability and a habitable region for a possible system planet through the stellar radiative energy fluxes ( r adiative habitable zone ; RHZ). The devised method is based on a combined formalism for the assessment of both S-type and P-type habitability; in particular, mathematical criteria are deduced for which kinds of systems S-type and P-type habitable zones are realized. If the RHZs are truncated by the additional constraint of orbital stability, the notation of ST-type and PT-type habitability applies. In comparison to the circular case, it is found that in systems of higher eccentricity, the range of the RHZs is significantly reduced. Moreover, for a considerable number of models, the orbital stability constraint also reduces the range of S-type and P-type habitability. Nonetheless, S-, P-, ST-, and PT-type habitability is identified for a considerable set of system parameters. The method as presented is utilized for BinHab, an online code available at The University of Texas at Arlington

  1. S-TYPE AND P-TYPE HABITABILITY IN STELLAR BINARY SYSTEMS: A COMPREHENSIVE APPROACH. II. ELLIPTICAL ORBITS

    Energy Technology Data Exchange (ETDEWEB)

    Cuntz, M., E-mail: cuntz@uta.edu [Department of Physics, University of Texas at Arlington, Arlington, TX 76019-0059 (United States)

    2015-01-10

    In the first paper of this series, a comprehensive approach has been provided for the study of S-type and P-type habitable regions in stellar binary systems, which was, however, restricted to circular orbits of the stellar components. Fortunately, a modest modification of the method also allows for the consideration of elliptical orbits, which of course entails a much broader range of applicability. This augmented method is presented here, and numerous applications are conveyed. In alignment with Paper I, the selected approach considers a variety of aspects, which comprise the consideration of a joint constraint including orbital stability and a habitable region for a possible system planet through the stellar radiative energy fluxes ({sup r}adiative habitable zone{sup ;} RHZ). The devised method is based on a combined formalism for the assessment of both S-type and P-type habitability; in particular, mathematical criteria are deduced for which kinds of systems S-type and P-type habitable zones are realized. If the RHZs are truncated by the additional constraint of orbital stability, the notation of ST-type and PT-type habitability applies. In comparison to the circular case, it is found that in systems of higher eccentricity, the range of the RHZs is significantly reduced. Moreover, for a considerable number of models, the orbital stability constraint also reduces the range of S-type and P-type habitability. Nonetheless, S-, P-, ST-, and PT-type habitability is identified for a considerable set of system parameters. The method as presented is utilized for BinHab, an online code available at The University of Texas at Arlington.

  2. A note on tilted Bianchi type VIh models: the type III bifurcation

    Science.gov (United States)

    Coley, A. A.; Hervik, S.

    2008-10-01

    In this note we complete the analysis of Hervik, van den Hoogen, Lim and Coley (2007 Class. Quantum Grav. 24 3859) of the late-time behaviour of tilted perfect fluid Bianchi type III models. We consider models with dust, and perfect fluids stiffer than dust, and eludicate the late-time behaviour by studying the centre manifold which dominates the behaviour of the model at late times. In the dust case, this centre manifold is three-dimensional and can be considered a double bifurcation as the two parameters (h and γ) of the type VIh model are varied. We therefore complete the analysis of the late-time behaviour of tilted ever-expanding Bianchi models of types I VIII.

  3. Parameters in pure type systems

    NARCIS (Netherlands)

    Bloo, C.J.; Kamareddine, F.; Laan, T.D.L.; Nederpelt, R.P.; Rajsbaum, S.

    2002-01-01

    In this paper we study the addition of parameters to typed ¿-calculus with definitions. We show that the resulting systems have nice properties and illustrate that parameters allow for a better fine-tuning of the strength of type systems as well as staying closer to type systems used in practice in

  4. Effects of different computer typing speeds on acceleration and peak contact pressure of the fingertips during computer typing.

    Science.gov (United States)

    Yoo, Won-Gyu

    2015-01-01

    [Purpose] This study showed the effects of different computer typing speeds on acceleration and peak contact pressure of the fingertips during computer typing. [Subjects] Twenty-one male computer workers voluntarily consented to participate in this study. They consisted of 7 workers who could type 200-300 characteristics/minute, 7 workers who could type 300-400 characteristics/minute, and 7 workers who could type 400-500 chracteristics/minute. [Methods] This study was used to measure the acceleration and peak contact pressure of the fingertips for different typing speed groups using an accelerometer and CONFORMat system. [Results] The fingertip contact pressure was increased in the high typing speed group compared with the low and medium typing speed groups. The fingertip acceleration was increased in the high typing speed group compared with the low and medium typing speed groups. [Conclusion] The results of the present study indicate that a fast typing speed cause continuous pressure stress to be applied to the fingers, thereby creating pain in the fingers.

  5. Comparison of imaging properties of direct-type and indirect-type of flat-panel detector

    International Nuclear Information System (INIS)

    Matsumoto, Masao; Suekane, Koji; Ichimaru, Yasunobu; Ogata, Yuji; Inamura, Kiyonari; Kanai, Kouzou; Kanamori, Hitoshi

    2002-01-01

    A Flat-Panel Detector (FPD) has many advantages such as eliminating cassette handling and being able to display a preview image immediately in addition to the digital image processing and the networking. Thus, the FPD has ability to innovate the radiology department. We measured and evaluated the digital and over-all imaging properties (characteristic curves, modulation Transfer Functions, Wiener spectra and Noise Equivalent Quanta (NEQ) for the direct-type and indirect-type of FPD. The pre-sampling and overall NEQ of the indirect-type of FPD were better than the NEQ of the direct-type of FPD at lower spatial frequencies, but were worse at higher spatial frequencies. The FPD can take image data at real-time and be easy to digitalize. From these results, Screen/Film system and Computed Radiography system will be replaced by the FPD system, together with diffusion of CAD, cone beam Computed Tomography (CT) system and open-type Magnetic Resonance Imagining (MRI) system. (T. Tanaka)

  6. The Ultracool Typing Kit - An Open-Source, Qualitative Spectral Typing GUI for L Dwarfs

    Science.gov (United States)

    Schwab, Ellianna; Cruz, Kelle; Núñez, Alejandro; Burgasser, Adam J.; Rice, Emily; Reid, Neill; Faherty, Jacqueline K.; BDNYC

    2018-01-01

    The Ultracool Typing Kit (UTK) is an open-source graphical user interface for classifying the NIR spectral types of L dwarfs, including field and low-gravity dwarfs spanning L0-L9. The user is able to input an NIR spectrum and qualitatively compare the input spectrum to a full suite of spectral templates, including low-gravity beta and gamma templates. The user can choose to view the input spectrum as both a band-by-band comparison with the templates and a full bandwidth comparison with NIR spectral standards. Once an optimal qualitative comparison is selected, the user can save their spectral type selection both graphically and to a database. Using UTK to classify 78 previously typed L dwarfs, we show that a band-by-band classification method more accurately agrees with optical spectral typing systems than previous L dwarf NIR classification schemes. UTK is written in python, released on Zenodo with a BSD-3 clause license and publicly available on the BDNYC Github page.

  7. Pregnancy outcomes in type 2 diabetic patients as compared with type 1 diabetic patients and nondiabetic controls.

    Science.gov (United States)

    Knight, Kristin M; Thornburg, Loralei L; Pressman, Eva K

    2012-01-01

    To characterize the neonatal and maternal outcomes of type 2 diabetic patients as compared with type 1 diabetic patients and nondiabetic controls. We performed a retrospective cohort study reviewing perinatal outcomes of type 1 and type 2 diabetic patients and nondiabetic controls from July 2000 to August 2006. Analysis of variance, t testing and chi2 analysis were used to compare groups. Post hoc power analysis indicated 80% power was necessary to detect a 15% difference in composite poor neonatal outcomes. A total of 64 type 2 and 64 type 1 diabetic patients were compared with 256 controls. Type 1 diabetic patients had higher incidences of composite poor neonatal outcome and congenital anomalies than did type 2 diabetic and control patients. Both diabetic groups had similarly higher incidences of cesarean delivery, preeclampsia, preterm delivery, polyhydramnios and macrosomia than did controls. Type 2 diabetic patients have a decreased incidence of adverse neonatal outcomes when compared with that of type 1 diabetic patients. No difference was observed between the diabetic groups in the incidence of a majority of the adverse maternal outcomes examined, however both diabetic groups had overall worse outcomes that did nondiabetic controls.

  8. ER Stress and β-Cell Pathogenesis of Type 1 and Type 2 Diabetes and Islet Transplantation

    OpenAIRE

    Kataoka, Hitomi Usui; Noguchi, Hirofumi

    2013-01-01

    Endoplasmic reticulum (ER) stress affects the pathogenesis of diabetes. ER stress plays important roles, both in type 1 and type 2 diabetes, because pancreatic β-cells possess highly developed ER for insulin secretion. This review summarizes the relationship between ER stress and the pathogenesis of type 1 and type 2 diabetes. In addition, the association between islet transplantation and ER stress is discussed.

  9. Object-Oriented Type Inference

    DEFF Research Database (Denmark)

    Schwartzbach, Michael Ignatieff; Palsberg, Jens

    1991-01-01

    We present a new approach to inferring types in untyped object-oriented programs with inheritance, assignments, and late binding. It guarantees that all messages are understood, annotates the program with type information, allows polymorphic methods, and can be used as the basis of an op...

  10. Facts about Type 2

    Medline Plus

    Full Text Available ... as well as the aged population. Patient Education Materials — Taking Care of Type 2 Diabetes This two-page introduction to type 2 diabetes is in PDF format so you can download it, print it, and hand it out to patients. You ...

  11. Type I supernova models

    International Nuclear Information System (INIS)

    Canal, Ramon; Labay, Javier; Isern, Jordi

    1987-01-01

    We briefly describe the characteristics of Type I supernova outbursts and we present the theoretical models so far advanced to explain them. We especially insist on models based on the thermonuclear explosion of a white dwarf in a close binary system, even regarding the recent division of Type I supernovae into the Ia and Ib subtypes. Together with models assuming explosive thermonuclear burning in a fluid interior, we consider in some detail those based on partially solid interiors. We finally discuss models that incorporate nonthermonuclear energy contributions, suggested in order to explain Type Ib outbursts. (Author)

  12. Vein-type uranium deposits

    International Nuclear Information System (INIS)

    Rich, R.A.; Holland, H.D.; Petersen, U.

    1975-01-01

    A critical review is presented of published data bearing on the mineralogy, paragenesis, geochemistry, and origin of veiw-type uranium deposits. Its aim is to serve as a starting point for new research and as a basis for the development of new exploration strategies. During the formation of both vein and sandstone types of deposits uranium seems to have been dissolved by and transported in rather oxidized solutions, and deposited where these solutions encountered reducing agents such as carbon, sulfides, ferrous minerals and hydrocarbons. Granitic rocks abnormally enriched in uranium have apparently been the most common source for uranium in vein-type deposits. Oxidizing solutions have been derived either from the surface or from depth. Surface solutions saturated with atmospheric oxygen have frequently passed through red bed or clean sandstone conduits on their way to and from uranium source rocks. Deep solutions of non-surface origin have apparently become sufficiently oxidizing by passage through and equilibration with red beds. The common association of clean sandstones or red beds with uranium-rich granites in the vicinity of vein-type uranium deposits is probably not fortuitous, and areas where these rock types are found together are considered particularly favorable targets for uranium exploration

  13. The relationship between C-type natriuretic peptide and cognitive impairment in older patients with Type 2 diabetes

    International Nuclear Information System (INIS)

    Li Xinling; Zhu Xiangyang; Huang Huaiyu; Jin Yan

    2011-01-01

    Objective: To investigate the relationship between C-type natriuretic peptide and cognitive impairment in older patients with type 2 diabetes, and to explore the pathogenesis of diabetic cognitive impairment. Methods: According to the Montreal Cognitive Assessment (MoCA) scores, 80 type 2 diabetic patients over the age of 60 years were divided into two groups, one group including 31 cases with cognitive impairment, the other 49 patients with non-cognitive impairment. And 80 normal participants were selected as the control group. Plasma level of C-type natriuretic peptide was measured by radio-immunity assay in all subjects. The changes and associations of the plasma C-type natriuretic peptide level among three groups was analyzed. Result: In the non-cognitive impairment group, plasma level of C-type natriuretic peptide was higher than that in the control group (P<0.01). But the plasma level of C-type natriuretic peptide in the cognitive impairment group was degraded, significantly deferent with those in the control group and the non-cognitive impairment group (P<0.01). MoCA scores of the cognitive impairment group positively correlated with plasma level of C-type natriuretic peptide (r=0.513, P<0.01). Conclusion: In the early period of type 2 diabetes,the secretion of C-type natriuretic peptide was increased. When diabetic cognitive impairment complicated,the secretion of C-type natriuretic peptide was decompensated. Then plasma level of C-type natriuretic peptide become low. The level of C-type natriuretic peptide closely correlated with diabetic cognitive impairment. It was suggested that diabetic angiopathies may act an important role in the pathogenesis of diabetic cognitive impairment. (authors)

  14. Memorization in Type-Directed Partial Evaluation

    DEFF Research Database (Denmark)

    Balat, Vincent; Danvy, Olivier

    2002-01-01

    We use a code generator—type-directed partial evaluation— to verify conversions between isomorphic types, or more precisely to verify that a composite function is the identity function at some complicated type. A typed functional language such as ML provides a natural support to express the funct...

  15. Facts about Type 2

    Medline Plus

    Full Text Available ... to help you meet your target blood glucose levels. Type 2 usually gets worse over time – even if you don’t need medications at first, you may need to later on. Some groups have a higher risk for developing type 2 diabetes than ...

  16. Beyond Type D personality

    DEFF Research Database (Denmark)

    Pelle, Aline J; Pedersen, Susanne S.; Szabó, Balázs M

    2009-01-01

    Type D personality has been associated with impaired health status in chronic heart failure (CHF), but other psychological factors may also be important.......Type D personality has been associated with impaired health status in chronic heart failure (CHF), but other psychological factors may also be important....

  17. Facts about Type 2

    Medline Plus

    Full Text Available ... or insulin to help you meet your target blood glucose levels. Type 2 usually gets worse over time – even if you don’t need medications at first, you may need to later on. Some groups have a higher risk for developing type 2 ...

  18. Type I vs type II spiral ganglion neurons exhibit differential survival and neuritogenesis during cochlear development

    Directory of Open Access Journals (Sweden)

    Housley Gary D

    2011-10-01

    Full Text Available Abstract Background The mechanisms that consolidate neural circuitry are a major focus of neuroscience. In the mammalian cochlea, the refinement of spiral ganglion neuron (SGN innervation to the inner hair cells (by type I SGNs and the outer hair cells (by type II SGNs is accompanied by a 25% loss of SGNs. Results We investigated the segregation of neuronal loss in the mouse cochlea using β-tubulin and peripherin antisera to immunolabel all SGNs and selectively type II SGNs, respectively, and discovered that it is the type II SGN population that is predominately lost within the first postnatal week. Developmental neuronal loss has been attributed to the decline in neurotrophin expression by the target hair cells during this period, so we next examined survival of SGN sub-populations using tissue culture of the mid apex-mid turn region of neonatal mouse cochleae. In organotypic culture for 48 hours from postnatal day 1, endogenous trophic support from the organ of Corti proved sufficient to maintain all type II SGNs; however, a large proportion of type I SGNs were lost. Culture of the spiral ganglion as an explant, with removal of the organ of Corti, led to loss of the majority of both SGN sub-types. Brain-derived neurotrophic factor (BDNF added as a supplement to the media rescued a significant proportion of the SGNs, particularly the type II SGNs, which also showed increased neuritogenesis. The known decline in BDNF production by the rodent sensory epithelium after birth is therefore a likely mediator of type II neuron apoptosis. Conclusion Our study thus indicates that BDNF supply from the organ of Corti supports consolidation of type II innervation in the neonatal mouse cochlea. In contrast, type I SGNs likely rely on additional sources for trophic support.

  19. Attitudes in patients with diabetes mellitus type 1 and type 2

    Directory of Open Access Journals (Sweden)

    Oleg Gennad'evich Motovilin

    2012-12-01

    Full Text Available Aims. To compare disease attitudes in patients with type 1 and type 2 diabetes mellitus (T1DM and T2DM and to evaluate relationship between attitudes and psychological welfare of these groups. Materials and Methods. We examined 140 patients with T1DM and 70 patients with T2DM on insulin therapy (mean age 22.6?3.2 and 60.1?7.8 years; male/female ratio 47/93 and 15/55; duration of diabetes 12.1?5.7 and 11.4?6.5 years, HbA1c 9.3?2.2 и 9.0?1.4%, respectively. Psychological parameters were assessed by following methods: Bekhterev Disease Attitude Typing (DAT, Colour Attitude Test (CAT, SF-36 (36-Item Short Form Health Survey, Spielberger Anxiety Inventory (SAI, CES-D Depression Scale, Dembo-Rubinstein (DR technique for self-esteem assessment. Results. DAT showed increased sensitive attitude to their disease in patients with T1 and T2DM, being significantly higher in T2DM. According to CAT, T2DM patients perceive DM as a disease, associated with severe manifestations and complications, while T1DM patients tend to incorporate the notion of diabetes with lifestyle. Cluster analysis showed negative disease attitude to be associated independently of diabetes type with decrease in quality of life and emotional deterioration (higher anxiety and depression score, as measured by SF-36, SAI and CES-D. Conclusion. Disease attitude typing and correction is important in management of DM. Emotional acceptance allows improvement in quality of life and promotes psychological welfare. Also, despite the absence of direct relationship between HbA1c and disease attitudes (which, is plausibly non-linear, emotional acceptance may favour glycemic compensation due to increase in compliance.

  20. Effectiveness of a regional prepregnancy care program in women with type 1 and type 2 diabetes

    DEFF Research Database (Denmark)

    Murphy, Helen R.; Roland, Jonathan M.; Skinner, Timothy C.

    2010-01-01

    of 680 pregnancies in women with type 1 and type 2 diabetes was performed. Primary outcomes were adverse pregnancy outcome (congenital malformation, stillbirth, or neonatal death), congenital malformation, and indicators of pregnancy preparation (5 mg folic acid, gestational age, and A1C). Comparisons...... with improved pregnancy preparation and reduced risk of adverse pregnancy outcome in type 1 and type 2 diabetes. Prepregnancy care had benefits beyond improved glycemic control and was a stronger predictor of pregnancy outcome than maternal obesity, ethnicity, or social disadvantage.......OBJECTIVE - To implement and evaluate a regional prepregnancy care program in women with type 1 and type 2 diabetes. RESEARCH DESIGN AND METHODS - Prepregnancy care was promoted among patients and health professionals and delivered across 10 regional maternity units. A prospective cohort study...

  1. Intersection Types and Related Systems

    Directory of Open Access Journals (Sweden)

    Paweł Parys

    2017-02-01

    Full Text Available We present a new approach to the following meta-problem: given a quantitative property of trees, design a type system such that the desired property for the tree generated by an infinitary ground lambda-term corresponds to some property of a derivation of a type for this lambda-term, in this type system. Our approach is presented in the particular case of the language finiteness problem for nondeterministic higher-order recursion schemes (HORSes: given a nondeterministic HORS, decide whether the set of all finite trees generated by this HORS is finite. We give a type system such that the HORS can generate a tree of an arbitrarily large finite size if and only if in the type system we can obtain derivations that are arbitrarily large, in an appropriate sense; the latter condition can be easily decided.

  2. Blood Types

    Science.gov (United States)

    ... positive or Rh-negative blood may be given to Rh-positive patients. The rules for plasma are the reverse: ... ethnic and racial groups have different frequency of the main blood types in their populations. Approximately ...

  3. PIDs, Types and the Semantic Web

    Science.gov (United States)

    Schwardmann, Ulrich

    2017-04-01

    PID Information Types are becoming a crucial role in scientific data management because they can provide state (what) and binding (where) information about digital objects as attributes of the PID. This is a similar but much more flexible approach than the well known mime type characterization, because both of these types concepts allow to decide about preconditions for processes in advance and before touching the data. One aspect of this is the need for standards and correctness of the used types to ensure reliability for the processes operating on the digital objects. This requires registries and schemas for PID InfoTypes and suggests an automated schema generation process. Such a process in combination with data type registries will be described in more detail in the intended talk. Another aspect of PID InfoTypes is its intrinsic grammar as subject-predicate-object triple, with the PID as subject, the type as predicate and its value (often again a PID) as object in this relation. Given the registration of types and the proposed syntactical rigidness of the value, guaranteed by the schema, together with the use of PIDs in subject and predicate, the type concept has the ability to overcome the fuzziness and lack of reliability of semantic web categories with its URL references and possibly changing locations and content. The intended talk will also describe this approach in more detail, discusses the differences to linked data and describes some necessary technological developments for the type concept to keep up with the possibilities currently provided by the semantic web.

  4. 29 CFR 779.329 - Effect of type of customer and type of goods or services.

    Science.gov (United States)

    2010-07-01

    ... 29 Labor 3 2010-07-01 2010-07-01 false Effect of type of customer and type of goods or services... FAIR LABOR STANDARDS ACT AS APPLIED TO RETAILERS OF GOODS OR SERVICES Exemptions for Certain Retail or Service Establishments ârecognizedâ As Retail âin the Particular Industryâ § 779.329 Effect of type of...

  5. Denotational semantics for guarded dependent type theory

    DEFF Research Database (Denmark)

    Bizjak, Aleš; Møgelberg, Rasmus Ejlers

    2018-01-01

    We present a new model of Guarded Dependent Type Theory (GDTT), a type theory with guarded recursion and multiple clocks in which one can program with, and reason about coinductive types. Productivity of recursively defined coinductive programs and proofs is encoded in types using guarded recursion......, crucial for programming with coinductive types, types must be interpreted as presheaves orthogonal to the object of clocks. In the case of dependent types, this translates to a unique lifting condition similar to the one found in homotopy theoretic models of type theory. Since the universes defined...... by inclusions of clock variable contexts commute on the nose with type operations on the universes....

  6. Type-I and type-II topological nodal superconductors with s -wave interaction

    Science.gov (United States)

    Huang, Beibing; Yang, Xiaosen; Xu, Ning; Gong, Ming

    2018-01-01

    Topological nodal superconductors with protected gapless points in momentum space are generally realized based on unconventional pairings. In this work we propose a minimal model to realize these topological nodal phases with only s -wave interaction. In our model the linear and quadratic spin-orbit couplings along the two orthogonal directions introduce anisotropic effective unconventional pairings in momentum space. This model may support different nodal superconducting phases characterized by either an integer winding number in BDI class or a Z2 index in D class at the particle-hole invariant axes. In the vicinity of the nodal points the effective Hamiltonian can be described by either type-I or type-II Dirac equations, and the Lifshitz transition from type-I nodal phases to type-II nodal phases can be driven by external in-plane magnetic fields. We show that these nodal phases are robust against weak impurities, which only slightly renormalizes the momentum-independent parameters in the impurity-averaged Hamiltonian, thus these phases are possible to be realized in experiments with real semi-Dirac materials. The smoking-gun evidences to verify these phases based on scanning tunneling spectroscopy method are also briefly discussed.

  7. Florence Nightingale: her personality type.

    Science.gov (United States)

    Dossey, Barbara M

    2010-03-01

    This article casts new and refreshing light on Florence Nightingale's life and work by examining her personality type. Using the theory-based Myers-Briggs Type Indicator (MBTI), the author examines Nightingale's personality type and reveals that she was an introverted-intuitive-thinking-judging type. The merit of using the MBTI is that it allows us to more clearly understand three major areas of Nightingale's life that have been partially unacknowledged or misunderstood: her spiritual development as a practicing mystic, her management of her chronic illness to maintain her prodigious work output, and her chosen strategies to transform her visionary ideas into new health care and social realities.

  8. 14 CFR 21.31 - Type design.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Type design. 21.31 Section 21.31... PROCEDURES FOR PRODUCTS AND PARTS Type Certificates § 21.31 Type design. The type design consists of— (a) The... configuration and the design features of the product shown to comply with the requirements of that part of this...

  9. Prevalence of Type 1 and Type 2 Diabetes Among Children and Adolescents From 2001 to 2009

    Science.gov (United States)

    Dabelea, Dana; Mayer-Davis, Elizabeth J.; Saydah, Sharon; Imperatore, Giuseppina; Linder, Barbara; Divers, Jasmin; Bell, Ronny; Badaru, Angela; Talton, Jennifer W.; Crume, Tessa; Liese, Angela D.; Merchant, Anwar T.; Lawrence, Jean M.; Reynolds, Kristi; Dolan, Lawrence; Liu, Lenna L.; Hamman, Richard F.

    2015-01-01

    IMPORTANCE Despite concern about an “epidemic,” there are limited data on trends in prevalence of either type 1 or type 2 diabetes across US race and ethnic groups. OBJECTIVE To estimate changes in the prevalence of type 1 and type 2 diabetes in US youth, by sex, age, and race/ethnicity between 2001 and 2009. DESIGN, SETTING, AND PARTICIPANTS Case patients were ascertained in 4 geographic areas and 1 managed health care plan. The study population was determined by the 2001 and 2009 bridged-race intercensal population estimates for geographic sites and membership counts for the health plan. MAIN OUTCOMES AND MEASURES Prevalence (per 1000) of physician-diagnosed type 1 diabetes in youth aged 0 through 19 years and type 2 diabetes in youth aged 10 through 19 years. RESULTS In 2001, 4958 of 3.3 million youth were diagnosed with type 1 diabetes for a prevalence of 1.48 per 1000 (95% CI, 1.44–1.52). In 2009, 6666 of 3.4 million youth were diagnosed with type 1 diabetes for a prevalence of 1.93 per 1000 (95% CI, 1.88–1.97). In 2009, the highest prevalence of type 1 diabetes was 2.55 per 1000 among white youth (95% CI, 2.48–2.62) and the lowest was 0.35 per 1000 in American Indian youth (95% CI, 0.26–0.47) and type 1 diabetes increased between 2001 and 2009 in all sex, age, and race/ethnic subgroups except for those with the lowest prevalence (age 0–4 years and American Indians). Adjusted for completeness of ascertainment, there was a 21.1% (95% CI, 15.6%–27.0%) increase in type 1 diabetes over 8 years. In 2001, 588 of 1.7 million youth were diagnosed with type 2 diabetes for a prevalence of 0.34 per 1000 (95% CI, 0.31–0.37). In 2009, 819 of 1.8 million were diagnosed with type 2 diabetes for a prevalence of 0.46 per 1000 (95% CI, 0.43–0.49). In 2009, the prevalence of type 2 diabetes was 1.20 per 1000 among American Indian youth (95% CI, 0.96–1.51); 1.06 per 1000 among black youth (95% CI, 0.93–1.22); 0.79 per 1000 among Hispanic youth (95% CI, 0

  10. Liver/kidney microsomal antibody type 1 and liver cytosol antibody type 1 concentrations in type 2 autoimmune hepatitis.

    Science.gov (United States)

    Muratori, L; Cataleta, M; Muratori, P; Lenzi, M; Bianchi, F B

    1998-05-01

    Liver/kidney microsomal antibody type 1 (LKM1) and liver cytosol antibody type 1 (LC1) are the serological markers of type 2 autoimmune hepatitis (AIH). Since LKM1 and LC1 react against two distinct liver specific autoantigens (cytochrome P450IID6 (CYP2D6) and a 58 kDa cytosolic polypeptide respectively), the aim was to see whether LKM1 and LC1 concentrations correlate with liver disease activity. Twenty one patients with type 2 AIH were studied. All sera were tested by indirect immunofluorescence, counterimmunoelectrophoresis, and immunoblotting visualised by enhanced chemiluminescence. To evaluate LKM1 and LC1 levels, the 50 kDa microsomal reactivity (corresponding to CYP2D6) and the 58 kDa cytosolic reactivity were quantified by densitometric analysis. Seven patients were positive for LKM1, nine for LC1, and five for both. Serial serum samples at onset and during immunosuppressive treatment were analysed in 13 patients (four positive for LKM1, six positive for LC1 and three positive for both). During remission, LKM1 concentration remained essentially unchanged in six of seven patients, and decreased in only one. Conversely, in two of nine patients, LC1 was completely lost, and, in the remaining seven, LC1 concentration was reduced by more than 50%. After immunosuppression tapering or withdrawal, flare ups of liver necrosis ensued with increasing LC1 concentration, but not LKM1. LC1 concentration, at variance with that of LKM1, parallels liver disease activity, and its participation in the pathogenic mechanisms of liver injury can be hypothesised.

  11. Diabetic ketoacidosis in type 1 and type 2 diabetes mellitus – clinical and biochemical differences

    Directory of Open Access Journals (Sweden)

    N. Krdžalić

    2007-02-01

    Full Text Available The goal of this retrospective study was to establish differences in clinical picture, biochemical parametres and precipitating factors in patiens with diabetic ketoacidosis in type 1 and type 2 diabetes. A total number of 25 patients was hospitalised in the Intensive Case Unit of the Department for Internal Diseases of the Cantonal Hospital in Zenica in the period of 14 months. Most patients had type 1 diabetes whose ketoacidosis showed symptoms of vomiting, stomachache and it was most often caused by a discontinued application of insulin or an infection. In patients with type 2 diabetes an inadequate regulation of glycemia had been noticed before hospitalisation and diabetic ketoacidosis was manifested by polyuria, polyphagia, polydipsia and weight loss. Precipitating factor in most patients with type 2 diabetes was an infection. In addition, a significant number of patients were newly discovered diabetics whose precipitating factor in most cases could not be found and the symptoms of the disease correspond to insulin dependent patients. The observed biochemical parameters did not show statistically significant differences between the groups of patients suffering from different types of diabetes. This study has shown that diabetic ketoacidosis can occur in type 2 diabetes. DKA can be prevented by education of patients, improvement of health care as well as improved communication between patients and doctors of family medicine.

  12. Sandstone-type uranium deposits

    International Nuclear Information System (INIS)

    Finch, W.I.; Davis, J.F.

    1985-01-01

    World-class sandstone-type uranium deposits are defined as epigenetic concentrations of uranium minerals occurring as uneven impregnations and minor massive replacements primarily in fluvial, lacustrine, and deltaic sandstone formations. The main purpose of this introductory paper is to define, classify, and introduce to the general geologic setting for sandstone-type uranium deposits

  13. Types of electric field distribution and corresponding types of convection in the polar ionosphere. Model

    International Nuclear Information System (INIS)

    Uvarov, V.M.; Barashkov, P.D.

    1989-01-01

    All types of distributions, known due to the experiment, for Ee-m electric field evening-morning component along morning-evening meridian are reproduced and corresponding types of convection in polar ionosphere are calculated on the basis of model of continuous distribution of E large-scale electric fields. Two-, three- and four-whirl types of convection are realized depending on conditions in interplanetary medium

  14. Sexual dysfunction in diabetic women: prevalence and differences in type 1 and type 2 diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Mazzilli R

    2015-02-01

    Full Text Available Rossella Mazzilli, Norina Imbrogno, Jlenia Elia, Michele Delfino, Olimpia Bitterman, Angela Napoli, Fernando Mazzilli Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Unit of Diabetology and Endocrinology, University of Rome Sapienza, Rome, Italy Background: The aim of this work was to evaluate the impact of diabetes on female sexuality and to highlight any differences between sexuality in the context of type 1 and type 2 diabetes mellitus (DM. Methods: The subjects selected were 49 women with type 1 DM, 24 women with type 2 DM, and 45 healthy women as controls. Each participant was given the nine-item Female Sexual Function Index questionnaire to complete. The metabolic profile was evaluated by body mass index and glycosylated hemoglobin assay. Results: The prevalence of sexual dysfunction (total score ≤30 was significantly higher in the type 1 DM group (25/49, 51%; 95% confidence interval [CI] 18–31 than in the control group (4/45, 9%; 95% CI 3–5; P=0.00006; there were no significant variations in the type 2 DM group (4/24, 17%; 95% CI 3–4 versus the control group (P=0.630, not statistically significant. The mean total score was significantly lower in the type 1 DM group (30.2±6.9 versus the control group (36.5±4.9; P=0.0003, but there was no significant difference between the type 2 DM group and the control group (P=0.773. With regard to specific questionnaire items, the mean values for arousal, lubrication, dyspareunia, and orgasm were significantly lower only in the type 1 DM group versus the control group. The mean values for desire were reduced in type 1 and type 2 DM groups versus control group. Conclusion: Type 1 DM is associated with sexual dysfunction. This may be due to classic neurovascular complications or to the negative impact of the disease on psychosocial factors. Larger and ideally longitudinal studies are necessary to better understand the relationship between DM and sexual dysfunction. Keywords

  15. THE ENVIRONMENTAL DEPENDENCE OF THE FRACTION OF 'UNCONVENTIONAL' GALAXIES: RED LATE TYPES AND BLUE EARLY TYPES

    International Nuclear Information System (INIS)

    Deng Xinfa; He Jizhou; Wu Ping; Ding Yingping

    2009-01-01

    From the Main galaxy sample of the Sloan Digital Sky Survey Data Release 6, we construct two volume-limited samples with the luminosity -20.0 ≤ M r ≤ -18.5 and -22.40 ≤ M r ≤ -20.16, respectively, to explore the environmental dependence of the fraction of 'unconventional' galaxies: red late types and blue early types. We use the density estimator within the distance to the fifth nearest neighbor, and construct two samples at both extremes of density and perform comparative studies between them for each volume-limited sample. Results of two volume-limited samples show the same conclusions: the fraction of red late-type galaxies rises considerably with increasing local density, and that one of the blue early-type galaxies declines substantially with increasing local density. In addition, we note that bluer galaxies preferentially are late types, but the red galaxies are not dominated by early types.

  16. Diabetic Nephropathy and Microalbuminuria in Pregnant Women With Type 1 and Type 2 Diabetes

    DEFF Research Database (Denmark)

    Damm, Julie Agner; Asbjörnsdóttir, Björg; Callesen, Nicoline Foged

    2013-01-01

    To evaluate the prevalence of diabetic nephropathy and microalbuminuria in pregnant women with type 2 diabetes in comparison with type 1 diabetes and to describe pregnancy outcomes in these women following the same antihypertensive protocol....

  17. Critical Imperative for the Reform of British Interpretation of Fetal Heart Rate Decelerations: Analysis of FIGO and NICE Guidelines, Post-Truth Foundations, Cognitive Fallacies, Myths and Occam’s Razor

    Science.gov (United States)

    Sholapurkar, Shashikant L.

    2017-01-01

    Cardiotocography (CTG) has disappointingly failed to show good predictability for fetal acidemia or neonatal outcomes in several large studies. A complete rethink of CTG interpretation will not be out of place. Fetal heart rate (FHR) decelerations are the most common deviations, benign as well as manifestation of impending fetal hypoxemia/acidemia, much more commonly than FHR baseline or variability. Their specific nomenclature is important (center-stage) because it provides the basic concepts and framework on which the complex “pattern recognition” of CTG interpretation by clinicians depends. Unfortunately, the discrimination of FHR decelerations seems to be muddled since the British obstetrics adopted the concept of vast majority of FHR decelerations being “variable” (cord-compression). With proliferation of confusing waveform criteria, “atypical variables” became the commonest cause of suspicious/pathological CTG. However, National Institute for Health and Care Excellence (NICE) (2014) had to disband the “typical” and “atypical” terminology because of flawed classifying criteria. This analytical review makes a strong case that there are major and fundamental framing and confirmation fallacies (not just biases) in interpretation of FHR decelerations by NICE (2014) and International Federation of Gynecology and Obstetrics (FIGO) (2015), probably the biggest in modern medicine. This “post-truth” approach is incompatible with scientific practice. Moreover, it amounts to setting oneself for failure. The inertia to change could be best described as “backfire effect”. There is abundant evidence that head-compression (and other non-hypoxic mediators) causes rapid rather than shallow/gradual decelerations. Currently, the vast majority of decelerations are attributed to unproven cord compression underpinned by flawed disproven pathophysiological hypotheses. Their further discrimination based on abstract, random, trial and error criteria remains

  18. Bauhinia variegata (Caesalpiniaceae) leaf extract: An effective treatment option in type I and type II diabetes.

    Science.gov (United States)

    Kulkarni, Yogesh A; Garud, Mayuresh S

    2016-10-01

    Among various metabolic disorders, diabetes mellitus is one of the most common disorder. Present study was designed to evaluate the effectiveness of aqueous extract of Bauhinia variegata leaves (AE) in animal models of type I and type II diabetes. Type I diabetes was induced by streptozotocin at the dose of 55mg/kg (i.p.) in male Sprague Dawley rats while type II diabetes was induced by high fat diet and streptozotocin at the dose of 35mg/kg (i.p.). Diabetic animals were treated with AE at the dose of 250, 500 and 1000mg/kg. Glipizide (5mg/kg) was used as standard treatment drug. Treatment was given for 28days. Parameters evaluated were body weight, plasma glucose, cholesterol, triglyceride, aspartate aminotransferase, alanine transaminase, alkaline phosphatase, total proteins, albumin, creatinine and bun urea nitrogen. In type II diabetes, high density lipoprotein levels in plasma and plasma insulin level were also evaluated. Histopathological study of pancreases were carried out in type I study. AE showed significant decrease in plasma glucose significantly. AE was also found to decrease cholesterol, triglyceride, creatinine and blood urea nitrogen level in both types of diabetes. AE did not show any significant effect on plasma levels of aspartate aminotransferase, alanine transaminase, alkaline phosphatase. AE was found to increase the albumin and total protein levels. Histopathological study showed that AE decreases the necrotic changes in the pancreatic tissue. Aqueous extract of B. variegata leaves was found effective in treatment of both type I and type II diabetes. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  19. Blood typing South American camelids.

    Science.gov (United States)

    Miller, W J; Hollander, P J; Franklin, W L

    1985-01-01

    Preliminary blood typing tests were made on New World camelids, guanacos, llamas, and two hybrids. Erythrocyte samples were tested against a battery of cattle blood typing reagents. Three different reagents were prepared from rabbit anti-erythrocyte sera. Transferrin variation and lectin polymorphism also were observed. No naturally occurring isoantibodies were found. Blood typing tests of New World camelids were shown to be feasible for studies of taxonomic relationships.

  20. Tank type reactor

    International Nuclear Information System (INIS)

    Otsuka, Fumio.

    1989-01-01

    The present invention concerns a tank type reactor capable of securing reactor core integrity by preventing incorporation of gases to an intermediate heat exchanger, thgereby improving the reliability. In a conventional tank type reactor, since vortex flows are easily caused near the inlet of an intermediate heat exchanger, there is a fear that cover gases are involved into the coolant main streams to induce fetal accidents. In the present invention, a reactor core is suspended by way of a suspending body to the inside of a reactor vessel and an intermediate heat exchanger and a pump are disposed between the suspending body and the reactor vessel, in which a vortex current preventive plate is attached at the outside near the coolant inlet on the primary circuit of the intermediate heat exchanger. In this way vortex or turbulence near the inlet of the intermediate heata exchanger or near the surface of coolants can be prevented. Accordingly, the cover gases are no more involved, to insure the reactor core integrity and obtain a tank type nuclear reactor of high reliability. (I.S.)